Intraoperative customized double-patch device with twin sutures for multiple muscular septal defects

Intraoperative customized double-patch device with twin sutures for multiple muscular septal defects Abstract OBJECTIVES Closure of multiple muscular ventricular septal defects (VSDs) remains a challenge because of anatomical complexity. METHODS We mapped all the VSDs using en face reconstruction of the right ventricular septal surface through echocardiography and then performed an ‘Intraoperative Customized Double-Patch Device’ technique to surgically close them in 39 patients (male:female = 25:14). The median age of the patients was 6 months (2 months–10 years), and mean weight was 5.98 ± 4.21 kg. A patch of polytetrafluoroethylene was placed on the left ventricular side of the defect and another on the right ventricular side, and they were anchored to each other using 2 polypropylene sutures. Residual shunts were evaluated using intraoperative echocardiography and measurements of right atrial-pulmonary arterial saturation were taken in all patients. RESULTS The distribution of muscular VSDs was as follows: anterior muscular 12, posterior muscular 18, mid-muscular 11 and apical 9. The associated lesions included perimembranous VSD (n = 28), tetralogy of Fallot (n = 6), double-outlet right ventricle (n = 2) and supramitral membrane (n = 2). Mean clamp time and bypass time were 93 ± 19 min and 147 ± 26 min, respectively. Mean hospital stay was 11 ± 3.39 days with no in-hospital mortality. Five patients with significant residual shunts needed concomitant PA banding. All patients remained in New York Heart Association Class I. There was either no residual shunt (n = 3) or trivial shunt (n = 2) among the banded patients. All patients remained symptom-free and continued to thrive well at the most recent follow-up (3.48 ± 1.51 years). CONCLUSIONS Muscular VSDs can be mapped through en face reconstruction and closed using intraoperative customized double-patch device technique in a variety of situations with satisfactory immediate and short-term results. Intraoperative customized double-patch device, En face reconstruction INTRODUCTION Multiple ventricular septal defects (VSDs) pose a technical challenge for precise surgical management. Various techniques have been published to attempt closure of these multiple VSDs [1–7]. There are few reports on a ventriculotomy approach [1, 3, 7], whereas others approach it through atriotomy exclusively [2, 4–6]. Nonetheless, no single technique has emerged as superior to others. Residual shunts and postoperative ventricular dysfunction (due to ventriculotomy, large patches) contribute to postoperative morbidity and mortality [1, 2, 4–7]. We approached these multiple defects in 2 steps: first, we profiled all the defects on the right ventricular septal surface through systematic 2 dimensional echocardiography and pictorially plotted them using the technique of en face reconstruction (EFR) [8]. Second, we closed all the defects through the right atrium using an ‘Intraoperative Customized Double-Patch Device’ (ICDPD)—1 patch on the left ventricular (LV) side and another on the right ventricular (RV) side of the defect. Both these patches were anchored to each other across the defect using 2 sutures. This article describes the immediate and short-term results in 39 patients with multiple VSDs. MATERIALS AND METHODS From June 2011 to December 2016, 39 patients (Table 1) with multiple VSDs underwent transatrial surgical repair by a single surgeon at our institution. The ‘ICDPD’ was used in all patients following preoperative echocardiography and EFR. There were 24 males and 15 females. The median age of the patients was 6 months (range 2 months–10 years). The mean weight was 5.98 ± 4.21 kg. There were 23 infants. Table 1: Details of individual patients Serial number  Age  Sex  Weight (kg)  Morphology  Number of defects  LV approach               Across MV  Across larger defect  Both  1  3 months  Male  2.7  PM VSD, multiple MM VSDs  4    +    2  2 years  Female  7  TOF, MM VSD  2    +    3  4 years  Male  10  PM VSD, AM VSD  2    +    4  1.5 years  Male  5  TOF, PoM VSD  2    +    5  1.5 years  Female  4.5  PM VSD, PoM VSDs  3      +  6  1 years  Male  4  PM VSD, PoM VSDs  3    +    7  8 month  Female  3.5  PM VSD, PoM VSDs  3      +  8  2 years  Male  8  PM VSD, AM VSDs  3    +    9  2.5 years  Female  9  PM VSD, AM VSDs  3    +    10  6 years  Male  14  PM VSD, PoM VSDs  3      +  11  3 years  Male  9.9  PM VSD, Ap VSDs, S/P PAB  5  +      12  3 months  Male  3  PM VSD, Ap VSDs, PoM VSD  3      +  13  11 months  Male  4.3  PM VSD, Ap VSDs, AM VSD  4    +    14  10 years  Female  19  TOF, AM VSD  2    +    15  4 months  Male  3.8  DORV, PoM VSD, AM VSD, SMM  3      +  16  5 months  Male  5.1  AM VSDs, PoM VSD  3  +      17  6 months  Male  2.35  PM VSD, AM VSD, Ap VSD  3      +  18  2 months  Female  2.4  PM VSD, PoM VSD  2    +    19  3 years  Male  11  PoM VSD, MM VSDs  4  +      20  2 months  Male  2.3  PM VSD, MM VSD  2    +    21  2 months  Male  2.5  PM VSD, MM VSDs  3    +    22  5 months  Male  3.4  PM VSD, MM VSDs  5    +    23  4 months  Female  2.9  PM VSD, MM VSDs  3    +    24  1.5 years  Male  5  TOF, MM VSD  2    +    25  6 months  Male  3.8  PM VSD, PoM VSDs  3      +  26  6 months  Female  3.6  PM VSD, PoM VSDs  3    +    27  5 years  Female  11  TOF, PoM VSD  2      +  28  2.5 years  Female  9  PM VSD, MM VSDs, Ap VSDs  6      +  29  6 months  Male  3.5  PM VSD, PoM VSDs  3    +    30  5 months  Female  5  PM VSD, MM VSD  2    +    31  6 months  Female  2.6  PM VSD, MM VSD  2    +    32  6 months  Female  2.35  PM VSD, AM VSD, Ap VSD  5      +  33  2 months  Male  2.4  PM VSD, PoM VSD  2    +    34  5 months  Male  5.1  AM VSD, PoM VSDs  3  +      35  6 months  Male  5.5  DORV, PoM VSD  2    +    36  10 years  Female  19  TOF, AM VSD  2    +    37  11 months  Male  4.3  PM VSD, AM VSD, Ap VSD, SMM  5      +  38  3 months  Male  3  PM VSD, PoM VSD, Ap VSD  3      +  39  3.6 years  Female  8.5  PM VSD, Ap VSDs, S/P PAB  6    +    Serial number  Age  Sex  Weight (kg)  Morphology  Number of defects  LV approach               Across MV  Across larger defect  Both  1  3 months  Male  2.7  PM VSD, multiple MM VSDs  4    +    2  2 years  Female  7  TOF, MM VSD  2    +    3  4 years  Male  10  PM VSD, AM VSD  2    +    4  1.5 years  Male  5  TOF, PoM VSD  2    +    5  1.5 years  Female  4.5  PM VSD, PoM VSDs  3      +  6  1 years  Male  4  PM VSD, PoM VSDs  3    +    7  8 month  Female  3.5  PM VSD, PoM VSDs  3      +  8  2 years  Male  8  PM VSD, AM VSDs  3    +    9  2.5 years  Female  9  PM VSD, AM VSDs  3    +    10  6 years  Male  14  PM VSD, PoM VSDs  3      +  11  3 years  Male  9.9  PM VSD, Ap VSDs, S/P PAB  5  +      12  3 months  Male  3  PM VSD, Ap VSDs, PoM VSD  3      +  13  11 months  Male  4.3  PM VSD, Ap VSDs, AM VSD  4    +    14  10 years  Female  19  TOF, AM VSD  2    +    15  4 months  Male  3.8  DORV, PoM VSD, AM VSD, SMM  3      +  16  5 months  Male  5.1  AM VSDs, PoM VSD  3  +      17  6 months  Male  2.35  PM VSD, AM VSD, Ap VSD  3      +  18  2 months  Female  2.4  PM VSD, PoM VSD  2    +    19  3 years  Male  11  PoM VSD, MM VSDs  4  +      20  2 months  Male  2.3  PM VSD, MM VSD  2    +    21  2 months  Male  2.5  PM VSD, MM VSDs  3    +    22  5 months  Male  3.4  PM VSD, MM VSDs  5    +    23  4 months  Female  2.9  PM VSD, MM VSDs  3    +    24  1.5 years  Male  5  TOF, MM VSD  2    +    25  6 months  Male  3.8  PM VSD, PoM VSDs  3      +  26  6 months  Female  3.6  PM VSD, PoM VSDs  3    +    27  5 years  Female  11  TOF, PoM VSD  2      +  28  2.5 years  Female  9  PM VSD, MM VSDs, Ap VSDs  6      +  29  6 months  Male  3.5  PM VSD, PoM VSDs  3    +    30  5 months  Female  5  PM VSD, MM VSD  2    +    31  6 months  Female  2.6  PM VSD, MM VSD  2    +    32  6 months  Female  2.35  PM VSD, AM VSD, Ap VSD  5      +  33  2 months  Male  2.4  PM VSD, PoM VSD  2    +    34  5 months  Male  5.1  AM VSD, PoM VSDs  3  +      35  6 months  Male  5.5  DORV, PoM VSD  2    +    36  10 years  Female  19  TOF, AM VSD  2    +    37  11 months  Male  4.3  PM VSD, AM VSD, Ap VSD, SMM  5      +  38  3 months  Male  3  PM VSD, PoM VSD, Ap VSD  3      +  39  3.6 years  Female  8.5  PM VSD, Ap VSDs, S/P PAB  6    +    AM: anterior muscular; Ap: apical; DORV: double-outlet right ventricle; LV: left ventricle; MM: mid-muscular; MV: mitral valve; PAB: pulmonary artery banding; PM: perimembranous; PoM: posterior muscular; S/P: status post; SMM: supramitral membrane; TOF: tetralogy of Fallot; VSD: ventricular septal defect. Table 1: Details of individual patients Serial number  Age  Sex  Weight (kg)  Morphology  Number of defects  LV approach               Across MV  Across larger defect  Both  1  3 months  Male  2.7  PM VSD, multiple MM VSDs  4    +    2  2 years  Female  7  TOF, MM VSD  2    +    3  4 years  Male  10  PM VSD, AM VSD  2    +    4  1.5 years  Male  5  TOF, PoM VSD  2    +    5  1.5 years  Female  4.5  PM VSD, PoM VSDs  3      +  6  1 years  Male  4  PM VSD, PoM VSDs  3    +    7  8 month  Female  3.5  PM VSD, PoM VSDs  3      +  8  2 years  Male  8  PM VSD, AM VSDs  3    +    9  2.5 years  Female  9  PM VSD, AM VSDs  3    +    10  6 years  Male  14  PM VSD, PoM VSDs  3      +  11  3 years  Male  9.9  PM VSD, Ap VSDs, S/P PAB  5  +      12  3 months  Male  3  PM VSD, Ap VSDs, PoM VSD  3      +  13  11 months  Male  4.3  PM VSD, Ap VSDs, AM VSD  4    +    14  10 years  Female  19  TOF, AM VSD  2    +    15  4 months  Male  3.8  DORV, PoM VSD, AM VSD, SMM  3      +  16  5 months  Male  5.1  AM VSDs, PoM VSD  3  +      17  6 months  Male  2.35  PM VSD, AM VSD, Ap VSD  3      +  18  2 months  Female  2.4  PM VSD, PoM VSD  2    +    19  3 years  Male  11  PoM VSD, MM VSDs  4  +      20  2 months  Male  2.3  PM VSD, MM VSD  2    +    21  2 months  Male  2.5  PM VSD, MM VSDs  3    +    22  5 months  Male  3.4  PM VSD, MM VSDs  5    +    23  4 months  Female  2.9  PM VSD, MM VSDs  3    +    24  1.5 years  Male  5  TOF, MM VSD  2    +    25  6 months  Male  3.8  PM VSD, PoM VSDs  3      +  26  6 months  Female  3.6  PM VSD, PoM VSDs  3    +    27  5 years  Female  11  TOF, PoM VSD  2      +  28  2.5 years  Female  9  PM VSD, MM VSDs, Ap VSDs  6      +  29  6 months  Male  3.5  PM VSD, PoM VSDs  3    +    30  5 months  Female  5  PM VSD, MM VSD  2    +    31  6 months  Female  2.6  PM VSD, MM VSD  2    +    32  6 months  Female  2.35  PM VSD, AM VSD, Ap VSD  5      +  33  2 months  Male  2.4  PM VSD, PoM VSD  2    +    34  5 months  Male  5.1  AM VSD, PoM VSDs  3  +      35  6 months  Male  5.5  DORV, PoM VSD  2    +    36  10 years  Female  19  TOF, AM VSD  2    +    37  11 months  Male  4.3  PM VSD, AM VSD, Ap VSD, SMM  5      +  38  3 months  Male  3  PM VSD, PoM VSD, Ap VSD  3      +  39  3.6 years  Female  8.5  PM VSD, Ap VSDs, S/P PAB  6    +    Serial number  Age  Sex  Weight (kg)  Morphology  Number of defects  LV approach               Across MV  Across larger defect  Both  1  3 months  Male  2.7  PM VSD, multiple MM VSDs  4    +    2  2 years  Female  7  TOF, MM VSD  2    +    3  4 years  Male  10  PM VSD, AM VSD  2    +    4  1.5 years  Male  5  TOF, PoM VSD  2    +    5  1.5 years  Female  4.5  PM VSD, PoM VSDs  3      +  6  1 years  Male  4  PM VSD, PoM VSDs  3    +    7  8 month  Female  3.5  PM VSD, PoM VSDs  3      +  8  2 years  Male  8  PM VSD, AM VSDs  3    +    9  2.5 years  Female  9  PM VSD, AM VSDs  3    +    10  6 years  Male  14  PM VSD, PoM VSDs  3      +  11  3 years  Male  9.9  PM VSD, Ap VSDs, S/P PAB  5  +      12  3 months  Male  3  PM VSD, Ap VSDs, PoM VSD  3      +  13  11 months  Male  4.3  PM VSD, Ap VSDs, AM VSD  4    +    14  10 years  Female  19  TOF, AM VSD  2    +    15  4 months  Male  3.8  DORV, PoM VSD, AM VSD, SMM  3      +  16  5 months  Male  5.1  AM VSDs, PoM VSD  3  +      17  6 months  Male  2.35  PM VSD, AM VSD, Ap VSD  3      +  18  2 months  Female  2.4  PM VSD, PoM VSD  2    +    19  3 years  Male  11  PoM VSD, MM VSDs  4  +      20  2 months  Male  2.3  PM VSD, MM VSD  2    +    21  2 months  Male  2.5  PM VSD, MM VSDs  3    +    22  5 months  Male  3.4  PM VSD, MM VSDs  5    +    23  4 months  Female  2.9  PM VSD, MM VSDs  3    +    24  1.5 years  Male  5  TOF, MM VSD  2    +    25  6 months  Male  3.8  PM VSD, PoM VSDs  3      +  26  6 months  Female  3.6  PM VSD, PoM VSDs  3    +    27  5 years  Female  11  TOF, PoM VSD  2      +  28  2.5 years  Female  9  PM VSD, MM VSDs, Ap VSDs  6      +  29  6 months  Male  3.5  PM VSD, PoM VSDs  3    +    30  5 months  Female  5  PM VSD, MM VSD  2    +    31  6 months  Female  2.6  PM VSD, MM VSD  2    +    32  6 months  Female  2.35  PM VSD, AM VSD, Ap VSD  5      +  33  2 months  Male  2.4  PM VSD, PoM VSD  2    +    34  5 months  Male  5.1  AM VSD, PoM VSDs  3  +      35  6 months  Male  5.5  DORV, PoM VSD  2    +    36  10 years  Female  19  TOF, AM VSD  2    +    37  11 months  Male  4.3  PM VSD, AM VSD, Ap VSD, SMM  5      +  38  3 months  Male  3  PM VSD, PoM VSD, Ap VSD  3      +  39  3.6 years  Female  8.5  PM VSD, Ap VSDs, S/P PAB  6    +    AM: anterior muscular; Ap: apical; DORV: double-outlet right ventricle; LV: left ventricle; MM: mid-muscular; MV: mitral valve; PAB: pulmonary artery banding; PM: perimembranous; PoM: posterior muscular; S/P: status post; SMM: supramitral membrane; TOF: tetralogy of Fallot; VSD: ventricular septal defect. The majority of the patients (n = 26) had more than 2 VSDs. The morphological spectrum has been illustrated in Fig. 1. We followed the anatomical classification of VSDs put forth by Alsoufi et al. [7]. Associated lesions along with multiple VSDs in this subset of patients were supramitral membrane (n = 2) and previous pulmonary artery banding (n = 2). Primary lesions of tetralogy of Fallot (n = 6) and double-outlet right ventricle (n = 2) associated with multiple muscular VSDs were also included in this study. ‘Swiss-cheese septum’ (n = 7) was diagnosed according to the definition stated in the Congenital Heart Surgery nomenclature by Jacobs et al. [9]. Two patients had undergone pulmonary artery banding elsewhere in infancy. We performed redo-sternotomy with closure of all defects and pulmonary artery debanding procedure for both the patients. Figure 1: View largeDownload slide A sketch of the en face view of the right ventricular septum. AM defects are located anterior to the TSM. PoM defects are located in the inlet (infratricuspid) area of the muscular septum. Defects proximal to the MB are MM defects, and those distal to the MB are Ap defects. AM: anterior muscular; Ap: apical defects; MB: moderator band; ML: muscle of Lancisi; MM: mid-muscular; PM: perimembranous; PoM: posterior muscular; TSM: trabecula septomarginalis. Figure 1: View largeDownload slide A sketch of the en face view of the right ventricular septum. AM defects are located anterior to the TSM. PoM defects are located in the inlet (infratricuspid) area of the muscular septum. Defects proximal to the MB are MM defects, and those distal to the MB are Ap defects. AM: anterior muscular; Ap: apical defects; MB: moderator band; ML: muscle of Lancisi; MM: mid-muscular; PM: perimembranous; PoM: posterior muscular; TSM: trabecula septomarginalis. Echocardiography and en face reconstruction EFR [8] is routinely done at our centre for profiling ventricular septum in children with multiple VSDs. All the studies are performed on Siemens Acuson SC2000 machine using either an 8-Hz (8V3) or a 4-Hz (4V1c) probe. In this technique, a comprehensive echocardiography to evaluate the entire cardiac anatomy is followed by detecting the number, location and size of the defects. The dimensions of each defect and the distance between the margins of the defects and various septal landmarks are determined from subcostal, apical 4-chamber and parasternal views (Fig. 2A and B). The dimensions in the orthogonal planes help to specify size and shape of the defects. The expected course of the conduction bundle and the relationship of the defects with the tricuspid valve apparatus are also mapped. Finally, this information is illustrated on a paper in the form of a pictorial reconstruction (Fig. 2C). This information is deliberated in detail during the presurgical meeting among the paediatric cardiologists and paediatric cardiac surgeons. Figure 2: View largeDownload slide (A and B) Two-dimensional echocardiography images with colour Doppler study in an orthogonal view. (C) The en face reconstruction. ‘A’ axis in (C) traverses 1 moderate size anterior muscular ventricular septal defect (VSD) and then 1 large posterior muscular VSD (as seen in the colour Doppler study in A). ‘B’ axis in (C) traverses through 2 tiny apical VSDs and then through the same large posterior muscular VSD (as seen in the colour Doppler study in B). Figure 2: View largeDownload slide (A and B) Two-dimensional echocardiography images with colour Doppler study in an orthogonal view. (C) The en face reconstruction. ‘A’ axis in (C) traverses 1 moderate size anterior muscular ventricular septal defect (VSD) and then 1 large posterior muscular VSD (as seen in the colour Doppler study in A). ‘B’ axis in (C) traverses through 2 tiny apical VSDs and then through the same large posterior muscular VSD (as seen in the colour Doppler study in B). Surgical technique The surgical technique is diagrammatically demonstrated in Fig. 3. The enface view of the RV septum is displayed in the operating room during surgery. Cardiopulmonary bypass is established by standard aortobicaval cannulation. Under moderate hypothermia, the ascending aorta is clamped, and intermittent antegrade cold blood cardioplegia is administered. It is repeated at regular intervals and is assisted by topical cooling. The right atrium is opened from the tip of its appendage towards the inferior vena cava, parallel to the atrioventricular groove. The left heart is vented through the interatrial septum or through the right superior pulmonary vein. The interior of the RV is inspected in greater details. This is greatly facilitated by tricuspid valve retraction at the mid-point of each leaflet. Figure 3: View largeDownload slide A diagrammatic representation of the surgical steps during intraoperative customized double-patch device technique. (A) Introduction of right-angled clamps, 1 in the left ventricle (LV) and 1 in the right ventricle (RV). (B) The LV clamp has fed the vessel loop into the tip of the RV clamp. (C) The RV clamp has been withdrawn. One RV limb and 1 LV limb of the vessel loop are observed. The LV limb is then threaded with 2 fine polypropylene sutures that already have been passed through the LV patch at poles apart. (D) The RV limb of the vessel loop is gradually withdrawn out of the right atrium. The LV patch now is placed onto the LV side of the defect. (E) The polypropylene sutures are then slipped out of the vessel loop and passed through the RV patch, which is lowered into the RV. (F) The intraoperative customized double-patch device has been constructed. Figure 3: View largeDownload slide A diagrammatic representation of the surgical steps during intraoperative customized double-patch device technique. (A) Introduction of right-angled clamps, 1 in the left ventricle (LV) and 1 in the right ventricle (RV). (B) The LV clamp has fed the vessel loop into the tip of the RV clamp. (C) The RV clamp has been withdrawn. One RV limb and 1 LV limb of the vessel loop are observed. The LV limb is then threaded with 2 fine polypropylene sutures that already have been passed through the LV patch at poles apart. (D) The RV limb of the vessel loop is gradually withdrawn out of the right atrium. The LV patch now is placed onto the LV side of the defect. (E) The polypropylene sutures are then slipped out of the vessel loop and passed through the RV patch, which is lowered into the RV. (F) The intraoperative customized double-patch device has been constructed. With the help of the road map provided by the pictorial reconstruction, all the VSDs were defined. This is accomplished by carefully introducing a right-angled clamp from the RV side of the septum as guided by the illustration. Its tip is made to protrude from the LV side of the defect, seen through either the larger VSD at the basal area of the septum or through a pre-existing atrial septal defect/incised interatrial septum. Thereafter, ensuring that the mitral valve chordae are not entangled, a vessel loop is guided into the right-angled clamp and brought out of the RV cavity across the septum (Fig. 3A and B). Thus, the vessel loop now has 2 limbs, 1 LV and 1 RV, thereby saddling the inter-ventricular septum across the defect (Figs 3C and 4A). Two fine polypropylene sutures are then passed at opposite poles of an appropriately fashioned circular expanded polytetrafluoroethylene patch, to be placed on the LV side of the defect. These sutures are then passed through the LV limb of the vessel loop (Fig. 3C). Next, the RV limb of the vessel loop is carefully and gradually pulled out of the atriotomy, so as to retrieve the vessel loop completely out and to place the patch against the LV side of the defect (Fig. 3D). The polypropylene sutures that are retrieved along with the vessel loop are slipped out from the loop and then passed through another appropriately tailored and slightly larger expanded polytetrafluoroethylene patch (Fig. 3E). This patch is then placed against the RV side of the defect (Fig. 4B). Care is taken not to entangle the sutures. These sutures are then tied on the RV patch so that both the patches get anchored like a device across the defect (Figs 3F and 5B). Figure 4: View largeDownload slide (A) A vessel loop with 1 left ventricular (LV) limb and another right ventricular (RV) limb (arrow). It is looping one of the muscular ventricular septal defect. (B) The LV limb of vessel loop (bold arrow) to which the LV patch has been anchored using 2 fine polypropylene sutures. The RV limb (arrow) will be pulled out gradually to place the LV patch onto the LV side of the defect. Figure 4: View largeDownload slide (A) A vessel loop with 1 left ventricular (LV) limb and another right ventricular (RV) limb (arrow). It is looping one of the muscular ventricular septal defect. (B) The LV limb of vessel loop (bold arrow) to which the LV patch has been anchored using 2 fine polypropylene sutures. The RV limb (arrow) will be pulled out gradually to place the LV patch onto the LV side of the defect. Figure 5: View largeDownload slide (A) A sketch of the RV septal view showing the large mid-muscular ventricular septal defect (VSD) (oblique arrow) surrounded by 3 tiny muscular VSDs. The dotted circle (bold arrow) is the LV patch. The RV patch (arrow) has been fixed with a layer of continuous polypropylene suture and reinforced with intermittent pledgetted sutures. Two sutures are seen traversing across the large defect, anchoring the 2 patches. A tiny posterior muscular VSD (vertical arrow) has been closed directly by pledgetted suture. (B, Coronal view of interventricular septum) A large mid-muscular VSD (fragmented by the RV trabeculations into multiple tiny defects surrounding it) closed from either sides by polytetrafluoroethylene patch anchored to each other by 2 sutures. The RV patch has been reinforced with a layer of continuous polypropylene suture (dotted lines on its edges). (C, Coronal view of interventricular septum) The possibility of distortion of patches due to a single anchoring stitch at the centre, without fixation of the RV patch by a layer of continuous suture. LV: left ventricular; RV: right ventricular. Figure 5: View largeDownload slide (A) A sketch of the RV septal view showing the large mid-muscular ventricular septal defect (VSD) (oblique arrow) surrounded by 3 tiny muscular VSDs. The dotted circle (bold arrow) is the LV patch. The RV patch (arrow) has been fixed with a layer of continuous polypropylene suture and reinforced with intermittent pledgetted sutures. Two sutures are seen traversing across the large defect, anchoring the 2 patches. A tiny posterior muscular VSD (vertical arrow) has been closed directly by pledgetted suture. (B, Coronal view of interventricular septum) A large mid-muscular VSD (fragmented by the RV trabeculations into multiple tiny defects surrounding it) closed from either sides by polytetrafluoroethylene patch anchored to each other by 2 sutures. The RV patch has been reinforced with a layer of continuous polypropylene suture (dotted lines on its edges). (C, Coronal view of interventricular septum) The possibility of distortion of patches due to a single anchoring stitch at the centre, without fixation of the RV patch by a layer of continuous suture. LV: left ventricular; RV: right ventricular. The RV side of the customized device is then sutured to the septum all around with continuous polypropylene suture. This layer is reinforced with few interrupted pledgetted sutures (Fig. 5A and B). Thereafter, any basal septal defects are closed in standard fashion. Saline is injected into the LV across the mitral valve (the saline injection test) to check for any residual defects. Those newly detected VSDs which are small and left out of the device are closed directly by pledgetted fine polypropylene sutures (Fig. 5A). Any residual leak is controlled with pledgetted fine polypropylene sutures. Whenever the LV approach to the defects is transmitral, competency of the mitral valve apparatus is ensured by the ‘saline injection test’. Once this is done, the interatrial septum is closed. The RA is closed, and cross-clamp is released in standard fashion. While rewarming, the main pulmonary artery is looped as a protocol for standby pulmonary artery banding. Once cardiopulmonary bypass is discontinued, either an epicardial echocardiography or a transoesophageal echocardiography is performed depending on the weight of the baby (≤7.5 kg, n = 28 and >7.5 kg, n = 11, respectively). Ventricular function and mitral valve competency are checked. Saturations in the right atrium and pulmonary artery are then checked. For any significant residual shunt on echocardiography associated with significant right atrial-pulmonary arterial saturation step-up (>7%), the main pulmonary artery is banded according to the Trusler’s rule [10] using a 3-mm strip of expanded polytetrafluoroethylene patch. The band is always anchored to the adventitia of the main pulmonary artery to avoid band migration. For pulmonary artery debanding, the main pulmonary artery at the banded portion is cut across longitudinally on bypass and vented. After VSD closure, the main pulmonary artery is reconstructed while rewarming, with an adequately tailored patch of bovine pericardium. RESULTS All patients underwent a 1-stage surgical repair. There were no revisions on bypass again. There were no hospital deaths. In 23 patients, the LV side of the septum was approached through the perimembranous VSD. In 4 patients, the approach was exclusively through the interatrial septum. Frequently (n = 12), both routes were utilized to approach the LV septum (Table 1). None had conduction-related abnormalities or the mitral valve injury. The mean aortic cross-clamp time was 93 ± 19 min, and the mean cardiopulmonary bypass time was 147 ± 26 min. In 5 patients (12.8%), significant residual shunt was confirmed (all with a pre-operative diagnosis of the ‘Swiss-cheese’ septum) by intraoperative echocardiography and by significant step-up in right atrial-pulmonary arterial saturations (>7%). All the residual shunts were found to be at the RV apical septum. None of them were found in relation to the constructed ‘ICDPD’. All 5 patients were banded according to the Trusler’s rule in the same sitting (Table 2). Table 2: Demographic findings Number of patients  39  Male:female ratio  24:15  Median age  6 months (range 2 months–10 years)  Mean weight (kg)  5.98 ± 4.21  Patients with >2 VSDs  26  VSD locations   Anterior muscular  12   Mid-muscular  11   Posterior muscular  18   Apical  9  Mean aortic clamp time (min)  93 ± 19  Mean CPB time (min)  147 ± 26  Mean hospital stay (days)  11 ± 3.39  Residual shunt with simultaneous PAB  5  Number of patients  39  Male:female ratio  24:15  Median age  6 months (range 2 months–10 years)  Mean weight (kg)  5.98 ± 4.21  Patients with >2 VSDs  26  VSD locations   Anterior muscular  12   Mid-muscular  11   Posterior muscular  18   Apical  9  Mean aortic clamp time (min)  93 ± 19  Mean CPB time (min)  147 ± 26  Mean hospital stay (days)  11 ± 3.39  Residual shunt with simultaneous PAB  5  CPB: cardiopulmonary bypass; PAB: pulmonary artery banding; VSD: ventricular septal defect. Table 2: Demographic findings Number of patients  39  Male:female ratio  24:15  Median age  6 months (range 2 months–10 years)  Mean weight (kg)  5.98 ± 4.21  Patients with >2 VSDs  26  VSD locations   Anterior muscular  12   Mid-muscular  11   Posterior muscular  18   Apical  9  Mean aortic clamp time (min)  93 ± 19  Mean CPB time (min)  147 ± 26  Mean hospital stay (days)  11 ± 3.39  Residual shunt with simultaneous PAB  5  Number of patients  39  Male:female ratio  24:15  Median age  6 months (range 2 months–10 years)  Mean weight (kg)  5.98 ± 4.21  Patients with >2 VSDs  26  VSD locations   Anterior muscular  12   Mid-muscular  11   Posterior muscular  18   Apical  9  Mean aortic clamp time (min)  93 ± 19  Mean CPB time (min)  147 ± 26  Mean hospital stay (days)  11 ± 3.39  Residual shunt with simultaneous PAB  5  CPB: cardiopulmonary bypass; PAB: pulmonary artery banding; VSD: ventricular septal defect. The mean hospital stay of this study group was 11 ± 3.39 days. All patients had an uneventful postoperative recovery except for 3 patients with pulmonary artery banding. They had a relatively prolonged intensive care unit recovery (mean of 15 days) in view of right ventricular dysfunction secondary to pulmonary artery banding. Postoperative echocardiography for rest of the patients showed good repair with normal biventricular function. All patients remained in sinus rhythm. The patients who underwent pulmonary artery banding showed minimal residual shunt in postoperative period. All patients were followed up on the 10th day after discharge and then at 1 month. Follow-up was then extended at 3-month interval for 1 year and then once in every 6 months for the next 5 years. Two-dimensional echocardiography was repeated at 1 month on follow-up (Fig. 6) and then whenever indicated—at least once in every 6 months. There was no late mortality in the study group. All patients continued to thrive well and remained symptom-free (mean follow-up of 3.48 ± 1.51 years). Out of the 5 banded patients, 2 had trivial residual shunt, whereas there was no residual shunt in the remaining 3 patients. One underwent successful pulmonary artery debanding with closure of the remaining defect. In the remaining 4 children, the RV pressure still remained subsystemic. They were awaiting debanding procedure at the time of the last follow-up. Figure 6: View largeDownload slide Postoperative 2-dimensional echocardiography image in apical 4-chamber view (zoomed) showing the intraoperative customized double-patch device well seated in the muscular septum, with no residual shunt on colour Doppler study. RV: right ventricle; LV: left ventricle. Figure 6: View largeDownload slide Postoperative 2-dimensional echocardiography image in apical 4-chamber view (zoomed) showing the intraoperative customized double-patch device well seated in the muscular septum, with no residual shunt on colour Doppler study. RV: right ventricle; LV: left ventricle. DISCUSSION The anterior muscular septum has been subdivided into anterior muscular septum and infundibular apex by Kumar et al. [11]. This helped in closing defects in some of the inaccessible areas of the septum [12, 13] more precisely. It is important to realize that the RV apex is not juxtaposed to the LV apex and remains the only portion that eludes better accessibility [11]. In our experience, defects in this portion of the septum remain most challenging to close. This is due to the densely trabeculated portion of the septum. In addition, the prevailing approaches of atrial/ventricular/apicular routes have never been able to access this portion of the trabecular septum. This can be aptly labelled as the ‘dark area of the heart’. We believe that prior EFR of the septum coupled with ICDPD technique has addressed both identification and accurate closure of all defects including the smaller ones that are in the perimeter of the larger defects. We are convinced that our approach of accurate identification, precise orientation and closure of all the defects advances the understanding of this morphology by few more steps. Various techniques have been described in the literature for the management of multiple muscular VSDs. Initially, a Dacron patch was used with right atriotomy and ventriculotomies (right and/or left) to close isolated multiple VSDs [1]. Murzi et al. [2] suggested the use of intraoperative VSD device (Rashkind double-umbrella device) for isolated or multiple defects especially located at the low or apical part of the septum. Kitagawa et al. [3] approached the muscular defects in 4 different ways according to their location as follows: right atriotomy with or without right ventriculotomy with division of septal and/or moderator band for all defects except apical and anterior muscular ones; right atrial approach with an oversized patch for trabecular VSDs with the patch placed on the LV side of the defect; apical left ventriculotomy for apical VSDs and the ‘Sandwich technique’ for closure of small anterior muscular defects by transfixing the muscular edge of the defects to the anterior free wall of the ventricle. Another technique by Mace et al. [4] suggested the use of a single large patch (extending to the apical portion of the trabecular septum) with intermediate fixings for the ‘Swiss-cheese’ septum. Re-endothelialization strategy was put forth by Alsoufi et al. [7], which relied on double-layer suturing of septal trabeculations to each other. All these techniques have been limited by varying morbidity and mortality rates due to the presence of significant residual shunt and postoperative myocardial dysfunction. The ‘Sandwich Technique’ was first presented by Kapoor et al. [14]. It was later described in the literature with various modifications and indications [5, 6, 15]. Our preoperative profiling of the ventricular septum and customized device technique for closure of the defects is unique in following ways: EFR helped in precise profiling of individual defects beforehand, which increased the level of detection of defects and to a greater extent defined the shapes of each defect, thereby increasing the level of surgical perfection. The use of 2 fine polypropylene sutures (instead of 1) at poles apart, near the periphery of the patches, helped in fixing them, giving more stability across the septum, while minimizing telescopic effect. This also minimized the buckling/distortion of the patches that may occur with a single anchoring stitch at the centre (Fig. 5C). We believe that these twin sutures gave a ‘tighter seal’ to the device, nullifying any residual shunts across it (Fig. 5B). The use of additional layer of continuous fine polypropylene suture fixing the patch to the RV side of the septum, adding further stability to the device. The repair was always assessed for adequacy by an intraoperative echocardiography—either transoesophageal or epicardial. Haemodynamic studies including right atrial-pulmonary arterial saturations were checked in all cases to supplement echocardiography findings.We believe that this novel technique of EFR followed by ICDPD has reduced the incidence of residual shunts in patients with multiple VSDs in our own experience. We have also observed that the LV function in our series has been remarkably preserved when compared with the reports in the ‘Sandwich Technique’ series [5, 6, 15]. We attribute this to few factors. First, our technique avoided unnecessary use of large patches by getting a precise estimate of VSD sizes by EFR prior to surgery, thus minimizing myocardial dysfunction. Second, the administration of inodilators as a drug of choice in the modern era has reduced the systemic vascular resistance, thereby minimizing the ventricular dysfunction. Third, the stitches that are being taken while securing the patch are no different from the conventional closure. This perhaps preserves the septal movement to the best extent possible. Fourth, we believe that our technique does not squash the septum between the patches. Rather it just eliminates the shunt keeping the septal function intact. Apart from this, the morbidity and mortality associated with myocardial dysfunction secondary to ventriculotomies and primary pulmonary artery banding (previous treatment strategy for a majority of these patients) was minimized in our subset of patients. We performed additional pulmonary artery banding for 5 patients probably because we extended the application of our technique to all types of multiple muscular defects, including those in the RV apex. We designed this customized device based on the proven philosophy of device closure of the defects in the catheterization laboratory by the cardiologists and the customized device closure of atrial septal defects by Warinsirikul et al. [16]. The major difference when compared with the technique proposed by Warinsirikul et al. is the anchoring point of the 2 patches, which has been shifted towards the periphery rather than the centre. This prevents the patch from ballooning, buckling and everting and also results in sealing the additional small defects in the vicinity of the larger defect. We would like to emphasize here that the suture placement should be approximately equidistant on the 2 patches, in both vertical and horizontal dimensions, for the patches to ‘line-up’ properly. The ICDPD technique results in closure of all the defects that are in the vicinity of the larger trabecular defects. This would not be possible if we close from the RV side alone as it is well known that the LV side of the septum usually has a single larger opening while having multiple openings on the RV side of the septum, largely due to the RV trabeculations (Fig. 5B). Thus, the twin patches with the twin sutures give stability and a tighter seal to the device. CONCLUSION In conclusion, echocardiographic EFR of multiple muscular defects helps in accurate profiling and closure of most of these VSDs. EFR also facilitates in customizing the devices that are prepared on table. The double-patch concept results in secure closure of the larger defects besides closing the smaller defects in the vicinity. We believe that the device design is a step ahead in terms of stability and accuracy in addressing multiple muscular defects with acceptable short-term outcomes. It also decreases the cost when compared with the VSD devices used in the operating room without compromising the safety and efficacy. Conflictofinterest: none declared. REFERENCES 1 Serraf A, Lacour-Gayet F, Bruniaux J, Ouaknine R, Losay J, Petit J et al.   Surgical management of isolated multiple ventricular septal defects (Logical approach in 130 cases). J Thorac Cardiovasc Surg  1992; 103: 437– 42. Google Scholar PubMed  2 Murzi B, Bonanomi GL, Giusti S, Luisi VS, Bernabei M, Carminati M et al.   Surgical closure of muscular ventricular septal defects using double umbrella devices (intraoperative VSD device closure). Eur J Cardiothorac Surg  1997; 12: 450– 5. Google Scholar CrossRef Search ADS PubMed  3 Kitagawa T, Durham LAIII, Mosca RS, Bove EL. Techniques and results in the management of multiple ventricular septal defects. J Thorac Cardiovasc Surg  1998; 115: 848– 56. Google Scholar CrossRef Search ADS PubMed  4 Mace L, Dervanian P, Bret EL, Folliguet TA, Lambert V, Losay J et al.   “Swiss cheese” septal defects: surgical closure using a single patch with intermediate fixings. Ann Thorac Surg  1999; 67: 1754– 9. Google Scholar CrossRef Search ADS PubMed  5 Ootaki Y, Yamaguchi M, Yoshimura N, Oka S, Yoshida M, Hasegawa T. Surgical management of trabecular ventricular septal defects: the sandwich technique. J Thorac Cardiovasc Surg  2003; 125: 508– 12. Google Scholar CrossRef Search ADS PubMed  6 Murakami H, Yoshimura N, Takahashi H, Matsuhisa H, Yoshida M, Oshima Y et al.   Closure of multiple ventricular septal defects by the felt sandwich technique: further analysis of 36 patients. J Thorac Cardiovasc Surg  2006; 132: 278– 82. Google Scholar CrossRef Search ADS PubMed  7 Alsoufi B, Karamlou T, Osaki M, Badiwala MV, Ching CC, Dipchand A et al.   Surgical repair of multiple muscular ventricular septal defects: the role of re-endocardialization strategy. J Thorac Cardiovasc Surg  2006; 132: 1072– 80. Google Scholar CrossRef Search ADS PubMed  8 Sivakumar S, Anil SR, Rao SG, Shivaprakash K, Kumar RK. Closure of muscular ventricular septal defects guided by en face reconstruction and pictorial representation. Ann Thorac Surg  2003; 76: 158– 66. Google Scholar CrossRef Search ADS PubMed  9 Jacobs JP, Burke RP, Quintessenza JA, Mavroudis C. Congenital Heart Surgery Nomenclature and Database Project: ventricular septal defect. Ann Thorac Surg  2000; 69: S25– 35. Google Scholar CrossRef Search ADS PubMed  10 Trusler GA, Mustard WT. A method of banding the pulmonary artery for large isolated ventricular septal defect with and without TGA. Ann Thorac Surg  1972; 13: 351– 5. Google Scholar CrossRef Search ADS PubMed  11 Kumar K, Lock JE, Geva T. Apical muscular ventricular septal defects between the left ventricle and the right ventricular infundibulum. Circulation  1997; 95: 1207– 13. Google Scholar CrossRef Search ADS PubMed  12 Myhre U, Duncan BW, Mee RB, Joshi R, Seshadri SG, Herrera-Verdugo O et al.   Apical right ventriculotomy for closure of apical ventricular septal defects. Ann Thorac Surg  2004; 78: 204– 8. Google Scholar CrossRef Search ADS PubMed  13 Chaturvedi RR, Shore DF, Yacoub M, Redington AN. Intraoperative apical ventricular septal defect closure using a modified Rashkind double umbrella. Heart  1996; 76: 367– 9. Google Scholar CrossRef Search ADS PubMed  14 Kapoor L, Gan MD, Das MB, Mukhopadhyay S, Bandhopadhyay A. Technique to repair multiple muscular ventricular septal defects. J Thorac Cardiovasc Surg  1999; 117: 402– 3. Google Scholar CrossRef Search ADS PubMed  15 Yamaguchi M, Yoshimura N, Oka S, Ootaki Y, Yoshida M. Closure of muscular VSD by a sandwiching method via a coexisting larger VSD or an interatrial septostomy. In: Proceedings of the 3rd World Congress of Pediatric Cardiology and Cardiac Surgery, Toronto, Canada, 2001. p.227. 16 Warinsirikul W, Sangchote S, Mokarapong P, Chaiyodsilp S, Tanamai S. Closure of atrial septal defects without cardiopulmonary bypass: the Sandwich operation. J Thorac Cardiovasc Surg  2001; 121: 1122– 9. Google Scholar CrossRef Search ADS PubMed  © The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices) http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Interactive CardioVascular and Thoracic Surgery Oxford University Press

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Oxford University Press
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© The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
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1569-9293
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Abstract

Abstract OBJECTIVES Closure of multiple muscular ventricular septal defects (VSDs) remains a challenge because of anatomical complexity. METHODS We mapped all the VSDs using en face reconstruction of the right ventricular septal surface through echocardiography and then performed an ‘Intraoperative Customized Double-Patch Device’ technique to surgically close them in 39 patients (male:female = 25:14). The median age of the patients was 6 months (2 months–10 years), and mean weight was 5.98 ± 4.21 kg. A patch of polytetrafluoroethylene was placed on the left ventricular side of the defect and another on the right ventricular side, and they were anchored to each other using 2 polypropylene sutures. Residual shunts were evaluated using intraoperative echocardiography and measurements of right atrial-pulmonary arterial saturation were taken in all patients. RESULTS The distribution of muscular VSDs was as follows: anterior muscular 12, posterior muscular 18, mid-muscular 11 and apical 9. The associated lesions included perimembranous VSD (n = 28), tetralogy of Fallot (n = 6), double-outlet right ventricle (n = 2) and supramitral membrane (n = 2). Mean clamp time and bypass time were 93 ± 19 min and 147 ± 26 min, respectively. Mean hospital stay was 11 ± 3.39 days with no in-hospital mortality. Five patients with significant residual shunts needed concomitant PA banding. All patients remained in New York Heart Association Class I. There was either no residual shunt (n = 3) or trivial shunt (n = 2) among the banded patients. All patients remained symptom-free and continued to thrive well at the most recent follow-up (3.48 ± 1.51 years). CONCLUSIONS Muscular VSDs can be mapped through en face reconstruction and closed using intraoperative customized double-patch device technique in a variety of situations with satisfactory immediate and short-term results. Intraoperative customized double-patch device, En face reconstruction INTRODUCTION Multiple ventricular septal defects (VSDs) pose a technical challenge for precise surgical management. Various techniques have been published to attempt closure of these multiple VSDs [1–7]. There are few reports on a ventriculotomy approach [1, 3, 7], whereas others approach it through atriotomy exclusively [2, 4–6]. Nonetheless, no single technique has emerged as superior to others. Residual shunts and postoperative ventricular dysfunction (due to ventriculotomy, large patches) contribute to postoperative morbidity and mortality [1, 2, 4–7]. We approached these multiple defects in 2 steps: first, we profiled all the defects on the right ventricular septal surface through systematic 2 dimensional echocardiography and pictorially plotted them using the technique of en face reconstruction (EFR) [8]. Second, we closed all the defects through the right atrium using an ‘Intraoperative Customized Double-Patch Device’ (ICDPD)—1 patch on the left ventricular (LV) side and another on the right ventricular (RV) side of the defect. Both these patches were anchored to each other across the defect using 2 sutures. This article describes the immediate and short-term results in 39 patients with multiple VSDs. MATERIALS AND METHODS From June 2011 to December 2016, 39 patients (Table 1) with multiple VSDs underwent transatrial surgical repair by a single surgeon at our institution. The ‘ICDPD’ was used in all patients following preoperative echocardiography and EFR. There were 24 males and 15 females. The median age of the patients was 6 months (range 2 months–10 years). The mean weight was 5.98 ± 4.21 kg. There were 23 infants. Table 1: Details of individual patients Serial number  Age  Sex  Weight (kg)  Morphology  Number of defects  LV approach               Across MV  Across larger defect  Both  1  3 months  Male  2.7  PM VSD, multiple MM VSDs  4    +    2  2 years  Female  7  TOF, MM VSD  2    +    3  4 years  Male  10  PM VSD, AM VSD  2    +    4  1.5 years  Male  5  TOF, PoM VSD  2    +    5  1.5 years  Female  4.5  PM VSD, PoM VSDs  3      +  6  1 years  Male  4  PM VSD, PoM VSDs  3    +    7  8 month  Female  3.5  PM VSD, PoM VSDs  3      +  8  2 years  Male  8  PM VSD, AM VSDs  3    +    9  2.5 years  Female  9  PM VSD, AM VSDs  3    +    10  6 years  Male  14  PM VSD, PoM VSDs  3      +  11  3 years  Male  9.9  PM VSD, Ap VSDs, S/P PAB  5  +      12  3 months  Male  3  PM VSD, Ap VSDs, PoM VSD  3      +  13  11 months  Male  4.3  PM VSD, Ap VSDs, AM VSD  4    +    14  10 years  Female  19  TOF, AM VSD  2    +    15  4 months  Male  3.8  DORV, PoM VSD, AM VSD, SMM  3      +  16  5 months  Male  5.1  AM VSDs, PoM VSD  3  +      17  6 months  Male  2.35  PM VSD, AM VSD, Ap VSD  3      +  18  2 months  Female  2.4  PM VSD, PoM VSD  2    +    19  3 years  Male  11  PoM VSD, MM VSDs  4  +      20  2 months  Male  2.3  PM VSD, MM VSD  2    +    21  2 months  Male  2.5  PM VSD, MM VSDs  3    +    22  5 months  Male  3.4  PM VSD, MM VSDs  5    +    23  4 months  Female  2.9  PM VSD, MM VSDs  3    +    24  1.5 years  Male  5  TOF, MM VSD  2    +    25  6 months  Male  3.8  PM VSD, PoM VSDs  3      +  26  6 months  Female  3.6  PM VSD, PoM VSDs  3    +    27  5 years  Female  11  TOF, PoM VSD  2      +  28  2.5 years  Female  9  PM VSD, MM VSDs, Ap VSDs  6      +  29  6 months  Male  3.5  PM VSD, PoM VSDs  3    +    30  5 months  Female  5  PM VSD, MM VSD  2    +    31  6 months  Female  2.6  PM VSD, MM VSD  2    +    32  6 months  Female  2.35  PM VSD, AM VSD, Ap VSD  5      +  33  2 months  Male  2.4  PM VSD, PoM VSD  2    +    34  5 months  Male  5.1  AM VSD, PoM VSDs  3  +      35  6 months  Male  5.5  DORV, PoM VSD  2    +    36  10 years  Female  19  TOF, AM VSD  2    +    37  11 months  Male  4.3  PM VSD, AM VSD, Ap VSD, SMM  5      +  38  3 months  Male  3  PM VSD, PoM VSD, Ap VSD  3      +  39  3.6 years  Female  8.5  PM VSD, Ap VSDs, S/P PAB  6    +    Serial number  Age  Sex  Weight (kg)  Morphology  Number of defects  LV approach               Across MV  Across larger defect  Both  1  3 months  Male  2.7  PM VSD, multiple MM VSDs  4    +    2  2 years  Female  7  TOF, MM VSD  2    +    3  4 years  Male  10  PM VSD, AM VSD  2    +    4  1.5 years  Male  5  TOF, PoM VSD  2    +    5  1.5 years  Female  4.5  PM VSD, PoM VSDs  3      +  6  1 years  Male  4  PM VSD, PoM VSDs  3    +    7  8 month  Female  3.5  PM VSD, PoM VSDs  3      +  8  2 years  Male  8  PM VSD, AM VSDs  3    +    9  2.5 years  Female  9  PM VSD, AM VSDs  3    +    10  6 years  Male  14  PM VSD, PoM VSDs  3      +  11  3 years  Male  9.9  PM VSD, Ap VSDs, S/P PAB  5  +      12  3 months  Male  3  PM VSD, Ap VSDs, PoM VSD  3      +  13  11 months  Male  4.3  PM VSD, Ap VSDs, AM VSD  4    +    14  10 years  Female  19  TOF, AM VSD  2    +    15  4 months  Male  3.8  DORV, PoM VSD, AM VSD, SMM  3      +  16  5 months  Male  5.1  AM VSDs, PoM VSD  3  +      17  6 months  Male  2.35  PM VSD, AM VSD, Ap VSD  3      +  18  2 months  Female  2.4  PM VSD, PoM VSD  2    +    19  3 years  Male  11  PoM VSD, MM VSDs  4  +      20  2 months  Male  2.3  PM VSD, MM VSD  2    +    21  2 months  Male  2.5  PM VSD, MM VSDs  3    +    22  5 months  Male  3.4  PM VSD, MM VSDs  5    +    23  4 months  Female  2.9  PM VSD, MM VSDs  3    +    24  1.5 years  Male  5  TOF, MM VSD  2    +    25  6 months  Male  3.8  PM VSD, PoM VSDs  3      +  26  6 months  Female  3.6  PM VSD, PoM VSDs  3    +    27  5 years  Female  11  TOF, PoM VSD  2      +  28  2.5 years  Female  9  PM VSD, MM VSDs, Ap VSDs  6      +  29  6 months  Male  3.5  PM VSD, PoM VSDs  3    +    30  5 months  Female  5  PM VSD, MM VSD  2    +    31  6 months  Female  2.6  PM VSD, MM VSD  2    +    32  6 months  Female  2.35  PM VSD, AM VSD, Ap VSD  5      +  33  2 months  Male  2.4  PM VSD, PoM VSD  2    +    34  5 months  Male  5.1  AM VSD, PoM VSDs  3  +      35  6 months  Male  5.5  DORV, PoM VSD  2    +    36  10 years  Female  19  TOF, AM VSD  2    +    37  11 months  Male  4.3  PM VSD, AM VSD, Ap VSD, SMM  5      +  38  3 months  Male  3  PM VSD, PoM VSD, Ap VSD  3      +  39  3.6 years  Female  8.5  PM VSD, Ap VSDs, S/P PAB  6    +    AM: anterior muscular; Ap: apical; DORV: double-outlet right ventricle; LV: left ventricle; MM: mid-muscular; MV: mitral valve; PAB: pulmonary artery banding; PM: perimembranous; PoM: posterior muscular; S/P: status post; SMM: supramitral membrane; TOF: tetralogy of Fallot; VSD: ventricular septal defect. Table 1: Details of individual patients Serial number  Age  Sex  Weight (kg)  Morphology  Number of defects  LV approach               Across MV  Across larger defect  Both  1  3 months  Male  2.7  PM VSD, multiple MM VSDs  4    +    2  2 years  Female  7  TOF, MM VSD  2    +    3  4 years  Male  10  PM VSD, AM VSD  2    +    4  1.5 years  Male  5  TOF, PoM VSD  2    +    5  1.5 years  Female  4.5  PM VSD, PoM VSDs  3      +  6  1 years  Male  4  PM VSD, PoM VSDs  3    +    7  8 month  Female  3.5  PM VSD, PoM VSDs  3      +  8  2 years  Male  8  PM VSD, AM VSDs  3    +    9  2.5 years  Female  9  PM VSD, AM VSDs  3    +    10  6 years  Male  14  PM VSD, PoM VSDs  3      +  11  3 years  Male  9.9  PM VSD, Ap VSDs, S/P PAB  5  +      12  3 months  Male  3  PM VSD, Ap VSDs, PoM VSD  3      +  13  11 months  Male  4.3  PM VSD, Ap VSDs, AM VSD  4    +    14  10 years  Female  19  TOF, AM VSD  2    +    15  4 months  Male  3.8  DORV, PoM VSD, AM VSD, SMM  3      +  16  5 months  Male  5.1  AM VSDs, PoM VSD  3  +      17  6 months  Male  2.35  PM VSD, AM VSD, Ap VSD  3      +  18  2 months  Female  2.4  PM VSD, PoM VSD  2    +    19  3 years  Male  11  PoM VSD, MM VSDs  4  +      20  2 months  Male  2.3  PM VSD, MM VSD  2    +    21  2 months  Male  2.5  PM VSD, MM VSDs  3    +    22  5 months  Male  3.4  PM VSD, MM VSDs  5    +    23  4 months  Female  2.9  PM VSD, MM VSDs  3    +    24  1.5 years  Male  5  TOF, MM VSD  2    +    25  6 months  Male  3.8  PM VSD, PoM VSDs  3      +  26  6 months  Female  3.6  PM VSD, PoM VSDs  3    +    27  5 years  Female  11  TOF, PoM VSD  2      +  28  2.5 years  Female  9  PM VSD, MM VSDs, Ap VSDs  6      +  29  6 months  Male  3.5  PM VSD, PoM VSDs  3    +    30  5 months  Female  5  PM VSD, MM VSD  2    +    31  6 months  Female  2.6  PM VSD, MM VSD  2    +    32  6 months  Female  2.35  PM VSD, AM VSD, Ap VSD  5      +  33  2 months  Male  2.4  PM VSD, PoM VSD  2    +    34  5 months  Male  5.1  AM VSD, PoM VSDs  3  +      35  6 months  Male  5.5  DORV, PoM VSD  2    +    36  10 years  Female  19  TOF, AM VSD  2    +    37  11 months  Male  4.3  PM VSD, AM VSD, Ap VSD, SMM  5      +  38  3 months  Male  3  PM VSD, PoM VSD, Ap VSD  3      +  39  3.6 years  Female  8.5  PM VSD, Ap VSDs, S/P PAB  6    +    Serial number  Age  Sex  Weight (kg)  Morphology  Number of defects  LV approach               Across MV  Across larger defect  Both  1  3 months  Male  2.7  PM VSD, multiple MM VSDs  4    +    2  2 years  Female  7  TOF, MM VSD  2    +    3  4 years  Male  10  PM VSD, AM VSD  2    +    4  1.5 years  Male  5  TOF, PoM VSD  2    +    5  1.5 years  Female  4.5  PM VSD, PoM VSDs  3      +  6  1 years  Male  4  PM VSD, PoM VSDs  3    +    7  8 month  Female  3.5  PM VSD, PoM VSDs  3      +  8  2 years  Male  8  PM VSD, AM VSDs  3    +    9  2.5 years  Female  9  PM VSD, AM VSDs  3    +    10  6 years  Male  14  PM VSD, PoM VSDs  3      +  11  3 years  Male  9.9  PM VSD, Ap VSDs, S/P PAB  5  +      12  3 months  Male  3  PM VSD, Ap VSDs, PoM VSD  3      +  13  11 months  Male  4.3  PM VSD, Ap VSDs, AM VSD  4    +    14  10 years  Female  19  TOF, AM VSD  2    +    15  4 months  Male  3.8  DORV, PoM VSD, AM VSD, SMM  3      +  16  5 months  Male  5.1  AM VSDs, PoM VSD  3  +      17  6 months  Male  2.35  PM VSD, AM VSD, Ap VSD  3      +  18  2 months  Female  2.4  PM VSD, PoM VSD  2    +    19  3 years  Male  11  PoM VSD, MM VSDs  4  +      20  2 months  Male  2.3  PM VSD, MM VSD  2    +    21  2 months  Male  2.5  PM VSD, MM VSDs  3    +    22  5 months  Male  3.4  PM VSD, MM VSDs  5    +    23  4 months  Female  2.9  PM VSD, MM VSDs  3    +    24  1.5 years  Male  5  TOF, MM VSD  2    +    25  6 months  Male  3.8  PM VSD, PoM VSDs  3      +  26  6 months  Female  3.6  PM VSD, PoM VSDs  3    +    27  5 years  Female  11  TOF, PoM VSD  2      +  28  2.5 years  Female  9  PM VSD, MM VSDs, Ap VSDs  6      +  29  6 months  Male  3.5  PM VSD, PoM VSDs  3    +    30  5 months  Female  5  PM VSD, MM VSD  2    +    31  6 months  Female  2.6  PM VSD, MM VSD  2    +    32  6 months  Female  2.35  PM VSD, AM VSD, Ap VSD  5      +  33  2 months  Male  2.4  PM VSD, PoM VSD  2    +    34  5 months  Male  5.1  AM VSD, PoM VSDs  3  +      35  6 months  Male  5.5  DORV, PoM VSD  2    +    36  10 years  Female  19  TOF, AM VSD  2    +    37  11 months  Male  4.3  PM VSD, AM VSD, Ap VSD, SMM  5      +  38  3 months  Male  3  PM VSD, PoM VSD, Ap VSD  3      +  39  3.6 years  Female  8.5  PM VSD, Ap VSDs, S/P PAB  6    +    AM: anterior muscular; Ap: apical; DORV: double-outlet right ventricle; LV: left ventricle; MM: mid-muscular; MV: mitral valve; PAB: pulmonary artery banding; PM: perimembranous; PoM: posterior muscular; S/P: status post; SMM: supramitral membrane; TOF: tetralogy of Fallot; VSD: ventricular septal defect. The majority of the patients (n = 26) had more than 2 VSDs. The morphological spectrum has been illustrated in Fig. 1. We followed the anatomical classification of VSDs put forth by Alsoufi et al. [7]. Associated lesions along with multiple VSDs in this subset of patients were supramitral membrane (n = 2) and previous pulmonary artery banding (n = 2). Primary lesions of tetralogy of Fallot (n = 6) and double-outlet right ventricle (n = 2) associated with multiple muscular VSDs were also included in this study. ‘Swiss-cheese septum’ (n = 7) was diagnosed according to the definition stated in the Congenital Heart Surgery nomenclature by Jacobs et al. [9]. Two patients had undergone pulmonary artery banding elsewhere in infancy. We performed redo-sternotomy with closure of all defects and pulmonary artery debanding procedure for both the patients. Figure 1: View largeDownload slide A sketch of the en face view of the right ventricular septum. AM defects are located anterior to the TSM. PoM defects are located in the inlet (infratricuspid) area of the muscular septum. Defects proximal to the MB are MM defects, and those distal to the MB are Ap defects. AM: anterior muscular; Ap: apical defects; MB: moderator band; ML: muscle of Lancisi; MM: mid-muscular; PM: perimembranous; PoM: posterior muscular; TSM: trabecula septomarginalis. Figure 1: View largeDownload slide A sketch of the en face view of the right ventricular septum. AM defects are located anterior to the TSM. PoM defects are located in the inlet (infratricuspid) area of the muscular septum. Defects proximal to the MB are MM defects, and those distal to the MB are Ap defects. AM: anterior muscular; Ap: apical defects; MB: moderator band; ML: muscle of Lancisi; MM: mid-muscular; PM: perimembranous; PoM: posterior muscular; TSM: trabecula septomarginalis. Echocardiography and en face reconstruction EFR [8] is routinely done at our centre for profiling ventricular septum in children with multiple VSDs. All the studies are performed on Siemens Acuson SC2000 machine using either an 8-Hz (8V3) or a 4-Hz (4V1c) probe. In this technique, a comprehensive echocardiography to evaluate the entire cardiac anatomy is followed by detecting the number, location and size of the defects. The dimensions of each defect and the distance between the margins of the defects and various septal landmarks are determined from subcostal, apical 4-chamber and parasternal views (Fig. 2A and B). The dimensions in the orthogonal planes help to specify size and shape of the defects. The expected course of the conduction bundle and the relationship of the defects with the tricuspid valve apparatus are also mapped. Finally, this information is illustrated on a paper in the form of a pictorial reconstruction (Fig. 2C). This information is deliberated in detail during the presurgical meeting among the paediatric cardiologists and paediatric cardiac surgeons. Figure 2: View largeDownload slide (A and B) Two-dimensional echocardiography images with colour Doppler study in an orthogonal view. (C) The en face reconstruction. ‘A’ axis in (C) traverses 1 moderate size anterior muscular ventricular septal defect (VSD) and then 1 large posterior muscular VSD (as seen in the colour Doppler study in A). ‘B’ axis in (C) traverses through 2 tiny apical VSDs and then through the same large posterior muscular VSD (as seen in the colour Doppler study in B). Figure 2: View largeDownload slide (A and B) Two-dimensional echocardiography images with colour Doppler study in an orthogonal view. (C) The en face reconstruction. ‘A’ axis in (C) traverses 1 moderate size anterior muscular ventricular septal defect (VSD) and then 1 large posterior muscular VSD (as seen in the colour Doppler study in A). ‘B’ axis in (C) traverses through 2 tiny apical VSDs and then through the same large posterior muscular VSD (as seen in the colour Doppler study in B). Surgical technique The surgical technique is diagrammatically demonstrated in Fig. 3. The enface view of the RV septum is displayed in the operating room during surgery. Cardiopulmonary bypass is established by standard aortobicaval cannulation. Under moderate hypothermia, the ascending aorta is clamped, and intermittent antegrade cold blood cardioplegia is administered. It is repeated at regular intervals and is assisted by topical cooling. The right atrium is opened from the tip of its appendage towards the inferior vena cava, parallel to the atrioventricular groove. The left heart is vented through the interatrial septum or through the right superior pulmonary vein. The interior of the RV is inspected in greater details. This is greatly facilitated by tricuspid valve retraction at the mid-point of each leaflet. Figure 3: View largeDownload slide A diagrammatic representation of the surgical steps during intraoperative customized double-patch device technique. (A) Introduction of right-angled clamps, 1 in the left ventricle (LV) and 1 in the right ventricle (RV). (B) The LV clamp has fed the vessel loop into the tip of the RV clamp. (C) The RV clamp has been withdrawn. One RV limb and 1 LV limb of the vessel loop are observed. The LV limb is then threaded with 2 fine polypropylene sutures that already have been passed through the LV patch at poles apart. (D) The RV limb of the vessel loop is gradually withdrawn out of the right atrium. The LV patch now is placed onto the LV side of the defect. (E) The polypropylene sutures are then slipped out of the vessel loop and passed through the RV patch, which is lowered into the RV. (F) The intraoperative customized double-patch device has been constructed. Figure 3: View largeDownload slide A diagrammatic representation of the surgical steps during intraoperative customized double-patch device technique. (A) Introduction of right-angled clamps, 1 in the left ventricle (LV) and 1 in the right ventricle (RV). (B) The LV clamp has fed the vessel loop into the tip of the RV clamp. (C) The RV clamp has been withdrawn. One RV limb and 1 LV limb of the vessel loop are observed. The LV limb is then threaded with 2 fine polypropylene sutures that already have been passed through the LV patch at poles apart. (D) The RV limb of the vessel loop is gradually withdrawn out of the right atrium. The LV patch now is placed onto the LV side of the defect. (E) The polypropylene sutures are then slipped out of the vessel loop and passed through the RV patch, which is lowered into the RV. (F) The intraoperative customized double-patch device has been constructed. With the help of the road map provided by the pictorial reconstruction, all the VSDs were defined. This is accomplished by carefully introducing a right-angled clamp from the RV side of the septum as guided by the illustration. Its tip is made to protrude from the LV side of the defect, seen through either the larger VSD at the basal area of the septum or through a pre-existing atrial septal defect/incised interatrial septum. Thereafter, ensuring that the mitral valve chordae are not entangled, a vessel loop is guided into the right-angled clamp and brought out of the RV cavity across the septum (Fig. 3A and B). Thus, the vessel loop now has 2 limbs, 1 LV and 1 RV, thereby saddling the inter-ventricular septum across the defect (Figs 3C and 4A). Two fine polypropylene sutures are then passed at opposite poles of an appropriately fashioned circular expanded polytetrafluoroethylene patch, to be placed on the LV side of the defect. These sutures are then passed through the LV limb of the vessel loop (Fig. 3C). Next, the RV limb of the vessel loop is carefully and gradually pulled out of the atriotomy, so as to retrieve the vessel loop completely out and to place the patch against the LV side of the defect (Fig. 3D). The polypropylene sutures that are retrieved along with the vessel loop are slipped out from the loop and then passed through another appropriately tailored and slightly larger expanded polytetrafluoroethylene patch (Fig. 3E). This patch is then placed against the RV side of the defect (Fig. 4B). Care is taken not to entangle the sutures. These sutures are then tied on the RV patch so that both the patches get anchored like a device across the defect (Figs 3F and 5B). Figure 4: View largeDownload slide (A) A vessel loop with 1 left ventricular (LV) limb and another right ventricular (RV) limb (arrow). It is looping one of the muscular ventricular septal defect. (B) The LV limb of vessel loop (bold arrow) to which the LV patch has been anchored using 2 fine polypropylene sutures. The RV limb (arrow) will be pulled out gradually to place the LV patch onto the LV side of the defect. Figure 4: View largeDownload slide (A) A vessel loop with 1 left ventricular (LV) limb and another right ventricular (RV) limb (arrow). It is looping one of the muscular ventricular septal defect. (B) The LV limb of vessel loop (bold arrow) to which the LV patch has been anchored using 2 fine polypropylene sutures. The RV limb (arrow) will be pulled out gradually to place the LV patch onto the LV side of the defect. Figure 5: View largeDownload slide (A) A sketch of the RV septal view showing the large mid-muscular ventricular septal defect (VSD) (oblique arrow) surrounded by 3 tiny muscular VSDs. The dotted circle (bold arrow) is the LV patch. The RV patch (arrow) has been fixed with a layer of continuous polypropylene suture and reinforced with intermittent pledgetted sutures. Two sutures are seen traversing across the large defect, anchoring the 2 patches. A tiny posterior muscular VSD (vertical arrow) has been closed directly by pledgetted suture. (B, Coronal view of interventricular septum) A large mid-muscular VSD (fragmented by the RV trabeculations into multiple tiny defects surrounding it) closed from either sides by polytetrafluoroethylene patch anchored to each other by 2 sutures. The RV patch has been reinforced with a layer of continuous polypropylene suture (dotted lines on its edges). (C, Coronal view of interventricular septum) The possibility of distortion of patches due to a single anchoring stitch at the centre, without fixation of the RV patch by a layer of continuous suture. LV: left ventricular; RV: right ventricular. Figure 5: View largeDownload slide (A) A sketch of the RV septal view showing the large mid-muscular ventricular septal defect (VSD) (oblique arrow) surrounded by 3 tiny muscular VSDs. The dotted circle (bold arrow) is the LV patch. The RV patch (arrow) has been fixed with a layer of continuous polypropylene suture and reinforced with intermittent pledgetted sutures. Two sutures are seen traversing across the large defect, anchoring the 2 patches. A tiny posterior muscular VSD (vertical arrow) has been closed directly by pledgetted suture. (B, Coronal view of interventricular septum) A large mid-muscular VSD (fragmented by the RV trabeculations into multiple tiny defects surrounding it) closed from either sides by polytetrafluoroethylene patch anchored to each other by 2 sutures. The RV patch has been reinforced with a layer of continuous polypropylene suture (dotted lines on its edges). (C, Coronal view of interventricular septum) The possibility of distortion of patches due to a single anchoring stitch at the centre, without fixation of the RV patch by a layer of continuous suture. LV: left ventricular; RV: right ventricular. The RV side of the customized device is then sutured to the septum all around with continuous polypropylene suture. This layer is reinforced with few interrupted pledgetted sutures (Fig. 5A and B). Thereafter, any basal septal defects are closed in standard fashion. Saline is injected into the LV across the mitral valve (the saline injection test) to check for any residual defects. Those newly detected VSDs which are small and left out of the device are closed directly by pledgetted fine polypropylene sutures (Fig. 5A). Any residual leak is controlled with pledgetted fine polypropylene sutures. Whenever the LV approach to the defects is transmitral, competency of the mitral valve apparatus is ensured by the ‘saline injection test’. Once this is done, the interatrial septum is closed. The RA is closed, and cross-clamp is released in standard fashion. While rewarming, the main pulmonary artery is looped as a protocol for standby pulmonary artery banding. Once cardiopulmonary bypass is discontinued, either an epicardial echocardiography or a transoesophageal echocardiography is performed depending on the weight of the baby (≤7.5 kg, n = 28 and >7.5 kg, n = 11, respectively). Ventricular function and mitral valve competency are checked. Saturations in the right atrium and pulmonary artery are then checked. For any significant residual shunt on echocardiography associated with significant right atrial-pulmonary arterial saturation step-up (>7%), the main pulmonary artery is banded according to the Trusler’s rule [10] using a 3-mm strip of expanded polytetrafluoroethylene patch. The band is always anchored to the adventitia of the main pulmonary artery to avoid band migration. For pulmonary artery debanding, the main pulmonary artery at the banded portion is cut across longitudinally on bypass and vented. After VSD closure, the main pulmonary artery is reconstructed while rewarming, with an adequately tailored patch of bovine pericardium. RESULTS All patients underwent a 1-stage surgical repair. There were no revisions on bypass again. There were no hospital deaths. In 23 patients, the LV side of the septum was approached through the perimembranous VSD. In 4 patients, the approach was exclusively through the interatrial septum. Frequently (n = 12), both routes were utilized to approach the LV septum (Table 1). None had conduction-related abnormalities or the mitral valve injury. The mean aortic cross-clamp time was 93 ± 19 min, and the mean cardiopulmonary bypass time was 147 ± 26 min. In 5 patients (12.8%), significant residual shunt was confirmed (all with a pre-operative diagnosis of the ‘Swiss-cheese’ septum) by intraoperative echocardiography and by significant step-up in right atrial-pulmonary arterial saturations (>7%). All the residual shunts were found to be at the RV apical septum. None of them were found in relation to the constructed ‘ICDPD’. All 5 patients were banded according to the Trusler’s rule in the same sitting (Table 2). Table 2: Demographic findings Number of patients  39  Male:female ratio  24:15  Median age  6 months (range 2 months–10 years)  Mean weight (kg)  5.98 ± 4.21  Patients with >2 VSDs  26  VSD locations   Anterior muscular  12   Mid-muscular  11   Posterior muscular  18   Apical  9  Mean aortic clamp time (min)  93 ± 19  Mean CPB time (min)  147 ± 26  Mean hospital stay (days)  11 ± 3.39  Residual shunt with simultaneous PAB  5  Number of patients  39  Male:female ratio  24:15  Median age  6 months (range 2 months–10 years)  Mean weight (kg)  5.98 ± 4.21  Patients with >2 VSDs  26  VSD locations   Anterior muscular  12   Mid-muscular  11   Posterior muscular  18   Apical  9  Mean aortic clamp time (min)  93 ± 19  Mean CPB time (min)  147 ± 26  Mean hospital stay (days)  11 ± 3.39  Residual shunt with simultaneous PAB  5  CPB: cardiopulmonary bypass; PAB: pulmonary artery banding; VSD: ventricular septal defect. Table 2: Demographic findings Number of patients  39  Male:female ratio  24:15  Median age  6 months (range 2 months–10 years)  Mean weight (kg)  5.98 ± 4.21  Patients with >2 VSDs  26  VSD locations   Anterior muscular  12   Mid-muscular  11   Posterior muscular  18   Apical  9  Mean aortic clamp time (min)  93 ± 19  Mean CPB time (min)  147 ± 26  Mean hospital stay (days)  11 ± 3.39  Residual shunt with simultaneous PAB  5  Number of patients  39  Male:female ratio  24:15  Median age  6 months (range 2 months–10 years)  Mean weight (kg)  5.98 ± 4.21  Patients with >2 VSDs  26  VSD locations   Anterior muscular  12   Mid-muscular  11   Posterior muscular  18   Apical  9  Mean aortic clamp time (min)  93 ± 19  Mean CPB time (min)  147 ± 26  Mean hospital stay (days)  11 ± 3.39  Residual shunt with simultaneous PAB  5  CPB: cardiopulmonary bypass; PAB: pulmonary artery banding; VSD: ventricular septal defect. The mean hospital stay of this study group was 11 ± 3.39 days. All patients had an uneventful postoperative recovery except for 3 patients with pulmonary artery banding. They had a relatively prolonged intensive care unit recovery (mean of 15 days) in view of right ventricular dysfunction secondary to pulmonary artery banding. Postoperative echocardiography for rest of the patients showed good repair with normal biventricular function. All patients remained in sinus rhythm. The patients who underwent pulmonary artery banding showed minimal residual shunt in postoperative period. All patients were followed up on the 10th day after discharge and then at 1 month. Follow-up was then extended at 3-month interval for 1 year and then once in every 6 months for the next 5 years. Two-dimensional echocardiography was repeated at 1 month on follow-up (Fig. 6) and then whenever indicated—at least once in every 6 months. There was no late mortality in the study group. All patients continued to thrive well and remained symptom-free (mean follow-up of 3.48 ± 1.51 years). Out of the 5 banded patients, 2 had trivial residual shunt, whereas there was no residual shunt in the remaining 3 patients. One underwent successful pulmonary artery debanding with closure of the remaining defect. In the remaining 4 children, the RV pressure still remained subsystemic. They were awaiting debanding procedure at the time of the last follow-up. Figure 6: View largeDownload slide Postoperative 2-dimensional echocardiography image in apical 4-chamber view (zoomed) showing the intraoperative customized double-patch device well seated in the muscular septum, with no residual shunt on colour Doppler study. RV: right ventricle; LV: left ventricle. Figure 6: View largeDownload slide Postoperative 2-dimensional echocardiography image in apical 4-chamber view (zoomed) showing the intraoperative customized double-patch device well seated in the muscular septum, with no residual shunt on colour Doppler study. RV: right ventricle; LV: left ventricle. DISCUSSION The anterior muscular septum has been subdivided into anterior muscular septum and infundibular apex by Kumar et al. [11]. This helped in closing defects in some of the inaccessible areas of the septum [12, 13] more precisely. It is important to realize that the RV apex is not juxtaposed to the LV apex and remains the only portion that eludes better accessibility [11]. In our experience, defects in this portion of the septum remain most challenging to close. This is due to the densely trabeculated portion of the septum. In addition, the prevailing approaches of atrial/ventricular/apicular routes have never been able to access this portion of the trabecular septum. This can be aptly labelled as the ‘dark area of the heart’. We believe that prior EFR of the septum coupled with ICDPD technique has addressed both identification and accurate closure of all defects including the smaller ones that are in the perimeter of the larger defects. We are convinced that our approach of accurate identification, precise orientation and closure of all the defects advances the understanding of this morphology by few more steps. Various techniques have been described in the literature for the management of multiple muscular VSDs. Initially, a Dacron patch was used with right atriotomy and ventriculotomies (right and/or left) to close isolated multiple VSDs [1]. Murzi et al. [2] suggested the use of intraoperative VSD device (Rashkind double-umbrella device) for isolated or multiple defects especially located at the low or apical part of the septum. Kitagawa et al. [3] approached the muscular defects in 4 different ways according to their location as follows: right atriotomy with or without right ventriculotomy with division of septal and/or moderator band for all defects except apical and anterior muscular ones; right atrial approach with an oversized patch for trabecular VSDs with the patch placed on the LV side of the defect; apical left ventriculotomy for apical VSDs and the ‘Sandwich technique’ for closure of small anterior muscular defects by transfixing the muscular edge of the defects to the anterior free wall of the ventricle. Another technique by Mace et al. [4] suggested the use of a single large patch (extending to the apical portion of the trabecular septum) with intermediate fixings for the ‘Swiss-cheese’ septum. Re-endothelialization strategy was put forth by Alsoufi et al. [7], which relied on double-layer suturing of septal trabeculations to each other. All these techniques have been limited by varying morbidity and mortality rates due to the presence of significant residual shunt and postoperative myocardial dysfunction. The ‘Sandwich Technique’ was first presented by Kapoor et al. [14]. It was later described in the literature with various modifications and indications [5, 6, 15]. Our preoperative profiling of the ventricular septum and customized device technique for closure of the defects is unique in following ways: EFR helped in precise profiling of individual defects beforehand, which increased the level of detection of defects and to a greater extent defined the shapes of each defect, thereby increasing the level of surgical perfection. The use of 2 fine polypropylene sutures (instead of 1) at poles apart, near the periphery of the patches, helped in fixing them, giving more stability across the septum, while minimizing telescopic effect. This also minimized the buckling/distortion of the patches that may occur with a single anchoring stitch at the centre (Fig. 5C). We believe that these twin sutures gave a ‘tighter seal’ to the device, nullifying any residual shunts across it (Fig. 5B). The use of additional layer of continuous fine polypropylene suture fixing the patch to the RV side of the septum, adding further stability to the device. The repair was always assessed for adequacy by an intraoperative echocardiography—either transoesophageal or epicardial. Haemodynamic studies including right atrial-pulmonary arterial saturations were checked in all cases to supplement echocardiography findings.We believe that this novel technique of EFR followed by ICDPD has reduced the incidence of residual shunts in patients with multiple VSDs in our own experience. We have also observed that the LV function in our series has been remarkably preserved when compared with the reports in the ‘Sandwich Technique’ series [5, 6, 15]. We attribute this to few factors. First, our technique avoided unnecessary use of large patches by getting a precise estimate of VSD sizes by EFR prior to surgery, thus minimizing myocardial dysfunction. Second, the administration of inodilators as a drug of choice in the modern era has reduced the systemic vascular resistance, thereby minimizing the ventricular dysfunction. Third, the stitches that are being taken while securing the patch are no different from the conventional closure. This perhaps preserves the septal movement to the best extent possible. Fourth, we believe that our technique does not squash the septum between the patches. Rather it just eliminates the shunt keeping the septal function intact. Apart from this, the morbidity and mortality associated with myocardial dysfunction secondary to ventriculotomies and primary pulmonary artery banding (previous treatment strategy for a majority of these patients) was minimized in our subset of patients. We performed additional pulmonary artery banding for 5 patients probably because we extended the application of our technique to all types of multiple muscular defects, including those in the RV apex. We designed this customized device based on the proven philosophy of device closure of the defects in the catheterization laboratory by the cardiologists and the customized device closure of atrial septal defects by Warinsirikul et al. [16]. The major difference when compared with the technique proposed by Warinsirikul et al. is the anchoring point of the 2 patches, which has been shifted towards the periphery rather than the centre. This prevents the patch from ballooning, buckling and everting and also results in sealing the additional small defects in the vicinity of the larger defect. We would like to emphasize here that the suture placement should be approximately equidistant on the 2 patches, in both vertical and horizontal dimensions, for the patches to ‘line-up’ properly. The ICDPD technique results in closure of all the defects that are in the vicinity of the larger trabecular defects. This would not be possible if we close from the RV side alone as it is well known that the LV side of the septum usually has a single larger opening while having multiple openings on the RV side of the septum, largely due to the RV trabeculations (Fig. 5B). Thus, the twin patches with the twin sutures give stability and a tighter seal to the device. CONCLUSION In conclusion, echocardiographic EFR of multiple muscular defects helps in accurate profiling and closure of most of these VSDs. EFR also facilitates in customizing the devices that are prepared on table. The double-patch concept results in secure closure of the larger defects besides closing the smaller defects in the vicinity. We believe that the device design is a step ahead in terms of stability and accuracy in addressing multiple muscular defects with acceptable short-term outcomes. It also decreases the cost when compared with the VSD devices used in the operating room without compromising the safety and efficacy. Conflictofinterest: none declared. REFERENCES 1 Serraf A, Lacour-Gayet F, Bruniaux J, Ouaknine R, Losay J, Petit J et al.   Surgical management of isolated multiple ventricular septal defects (Logical approach in 130 cases). J Thorac Cardiovasc Surg  1992; 103: 437– 42. Google Scholar PubMed  2 Murzi B, Bonanomi GL, Giusti S, Luisi VS, Bernabei M, Carminati M et al.   Surgical closure of muscular ventricular septal defects using double umbrella devices (intraoperative VSD device closure). Eur J Cardiothorac Surg  1997; 12: 450– 5. Google Scholar CrossRef Search ADS PubMed  3 Kitagawa T, Durham LAIII, Mosca RS, Bove EL. Techniques and results in the management of multiple ventricular septal defects. J Thorac Cardiovasc Surg  1998; 115: 848– 56. Google Scholar CrossRef Search ADS PubMed  4 Mace L, Dervanian P, Bret EL, Folliguet TA, Lambert V, Losay J et al.   “Swiss cheese” septal defects: surgical closure using a single patch with intermediate fixings. Ann Thorac Surg  1999; 67: 1754– 9. Google Scholar CrossRef Search ADS PubMed  5 Ootaki Y, Yamaguchi M, Yoshimura N, Oka S, Yoshida M, Hasegawa T. Surgical management of trabecular ventricular septal defects: the sandwich technique. J Thorac Cardiovasc Surg  2003; 125: 508– 12. Google Scholar CrossRef Search ADS PubMed  6 Murakami H, Yoshimura N, Takahashi H, Matsuhisa H, Yoshida M, Oshima Y et al.   Closure of multiple ventricular septal defects by the felt sandwich technique: further analysis of 36 patients. J Thorac Cardiovasc Surg  2006; 132: 278– 82. Google Scholar CrossRef Search ADS PubMed  7 Alsoufi B, Karamlou T, Osaki M, Badiwala MV, Ching CC, Dipchand A et al.   Surgical repair of multiple muscular ventricular septal defects: the role of re-endocardialization strategy. J Thorac Cardiovasc Surg  2006; 132: 1072– 80. Google Scholar CrossRef Search ADS PubMed  8 Sivakumar S, Anil SR, Rao SG, Shivaprakash K, Kumar RK. Closure of muscular ventricular septal defects guided by en face reconstruction and pictorial representation. Ann Thorac Surg  2003; 76: 158– 66. Google Scholar CrossRef Search ADS PubMed  9 Jacobs JP, Burke RP, Quintessenza JA, Mavroudis C. Congenital Heart Surgery Nomenclature and Database Project: ventricular septal defect. Ann Thorac Surg  2000; 69: S25– 35. Google Scholar CrossRef Search ADS PubMed  10 Trusler GA, Mustard WT. A method of banding the pulmonary artery for large isolated ventricular septal defect with and without TGA. Ann Thorac Surg  1972; 13: 351– 5. Google Scholar CrossRef Search ADS PubMed  11 Kumar K, Lock JE, Geva T. Apical muscular ventricular septal defects between the left ventricle and the right ventricular infundibulum. Circulation  1997; 95: 1207– 13. Google Scholar CrossRef Search ADS PubMed  12 Myhre U, Duncan BW, Mee RB, Joshi R, Seshadri SG, Herrera-Verdugo O et al.   Apical right ventriculotomy for closure of apical ventricular septal defects. Ann Thorac Surg  2004; 78: 204– 8. Google Scholar CrossRef Search ADS PubMed  13 Chaturvedi RR, Shore DF, Yacoub M, Redington AN. Intraoperative apical ventricular septal defect closure using a modified Rashkind double umbrella. Heart  1996; 76: 367– 9. Google Scholar CrossRef Search ADS PubMed  14 Kapoor L, Gan MD, Das MB, Mukhopadhyay S, Bandhopadhyay A. Technique to repair multiple muscular ventricular septal defects. J Thorac Cardiovasc Surg  1999; 117: 402– 3. Google Scholar CrossRef Search ADS PubMed  15 Yamaguchi M, Yoshimura N, Oka S, Ootaki Y, Yoshida M. Closure of muscular VSD by a sandwiching method via a coexisting larger VSD or an interatrial septostomy. In: Proceedings of the 3rd World Congress of Pediatric Cardiology and Cardiac Surgery, Toronto, Canada, 2001. p.227. 16 Warinsirikul W, Sangchote S, Mokarapong P, Chaiyodsilp S, Tanamai S. Closure of atrial septal defects without cardiopulmonary bypass: the Sandwich operation. J Thorac Cardiovasc Surg  2001; 121: 1122– 9. Google Scholar CrossRef Search ADS PubMed  © The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices)

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Interactive CardioVascular and Thoracic SurgeryOxford University Press

Published: Mar 26, 2018

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