TY - JOUR
AU1 - Kabbani, Mohamed S.
AU2 - Munshi, Farid
AU3 - Alhabshan, Fahad
AU4 - Mutairi, Mansour Al
AU5 - Alghamdi, Abdullah A.
AB - Abstract An Amplatzer device can be used for peri-membranous ventricular septal defect (VSD) closure. Late complication presenting after device closure is rare and its management is subject of debate. In this report, we describe a child who underwent Amplatzer device occlusion of peri-membranous VSD with late development of life-threatening complete heart block presenting many days post-procedure. We discuss the case and summarize our successful surgical management with review of different approaches reported in the literature. Amplatzer device, Complete heart block, VSD Introduction Complete atrioventricular (AV) heart block is a complication that can occur after surgical repair or device closure of ventricular septal defect (VSD). It has been reported in 1–22% of cases after transcatheter device occlusion of peri-membranous VSD.1,2 Usually, it occurs immediately after device deployment. A delayed presentation, however, is rare and its management is unclear (I) Case history A 4-year-old girl was diagnosed with peri-membranous VSD associated with mild tricuspid valve insufficiency. She underwent uneventful percutaneous closure of the VSD using a transcatheter Amplatzer device, which was satisfactorily positioned. One week after the procedure, she developed sudden syncope with cardiogenic shock. The father performed cardiac massage while she was rushed to emergency room (ER). On arrival to the ER, she was unresponsive with severe bradycardia (15–20 b.p.m.) and cardiogenic shock (Figure 1). She was immediately resuscitated, i.v. atropine was provided, and emergency transthoracic pacing was started. Soon after cardiopulmunary resuscitation (CPR) initiation, she returned to normal sinus rhythm with gradual stabilization of the haemodynamic status. Echocardiography assessment showed the device being in good position (Figure 2). However, with the potential risk of recurrence of life-threatening heart block, the child was immediately referred for surgical removal of the Amplatzer device and repair of VSD. At surgery, it became obvious that the Amplatzer device was compressing the septal leaflet of tricuspid valve. The device was removed, the VSD was patched, and the tricuspid septal leaflet was repaired (Figure 3). Intra-operative transoesophageal ECHO showed complete elimination of the left to right shunt, no patch leak, and improvement in tricuspid valve regurgitation. The patient remained in normal sinus rhythm thereafter and is doing well. Periodic follow-ups over 2 years with serial ECG and ECHO documented persistent normal sinus rhythm without any further incidence of heart block or arrhythmia and satisfactory surgical correction. During 48 months of follow-up, the patient needed no pacemaker insertion and was free from any episode of life-threatening arrhythmia. With good recovery and no residual disease, no further electrophysiological study was required (Figure 4). Figure 1 View largeDownload slide Electrocardiogram showing complete heart block with severe bardycardia upon arrival to emergency room. Figure 1 View largeDownload slide Electrocardiogram showing complete heart block with severe bardycardia upon arrival to emergency room. Figure 2 View largeDownload slide Echocardiography of the right ventricle outflow tract showing the Amplatzer device with good positioning. Figure 2 View largeDownload slide Echocardiography of the right ventricle outflow tract showing the Amplatzer device with good positioning. Figure 3 View largeDownload slide Post-operative echocardiography of the right ventricle outflow tract showing patch occlusion of VSD after device removal. Figure 3 View largeDownload slide Post-operative echocardiography of the right ventricle outflow tract showing patch occlusion of VSD after device removal. Figure 4 View largeDownload slide Post-operative electrocardiogram showing complete resolution of heart block and return of normal sinus rhythm. Figure 4 View largeDownload slide Post-operative electrocardiogram showing complete resolution of heart block and return of normal sinus rhythm. Discussion While device occlusion of VSD is a valid alternative to surgical repair, the best option for the peri-membranous VSD is not known. Device occlusion of this particular form of VSD is aggravated with 5–22% of heart block as the peri-membranous VSD is in close proximity to AV node and conduction system.1–5 Heart block may not appear immediately after device insertion and on some occasions, it occurs after discharge like in the case presented here. Literature data on how to handle this late complication are scanty. Butera et al.6 reported two patients who developed late (1 and 4 months) AV block requiring pacemaker implantation. The European Registry reports an incidence of 3.7% over a total of 430 cases.7 Out of the 3.7%, only four cases were classified as late presentation, all requiring pacemaker implant. It follows that occurrence of complete AV block is rare and quite unpredictable, and it is usually related to the proximity of the conduction system to the margins of the peri-membranous VSD. Different mechanisms have been suggested for AV Block development after device closure: (i) the presence of the device disturbs AV conduction system by direct traumatic compression; (ii) the device is oversized and reaches with its bulky metal border the conduction system; (iii) the device induces an inflammatory reaction or scar formation in the conduction tissue. The management options are also diverse and debatable. Some suggested that development of second- or third-degree heart block during advancement of the introducer in the VSD before deploying the device is a potential predictor for future heart block development. Under these circumstances, which was not the case of our patient, it is recommended to abandon the procedure altogether and refer to surgery.8 Others recommended the use of steroidal and non-steroidal anti-inflammatory medications to reduce inflammation and local oedema in the AV node as well as conduction system.9 The temporary use of pace-maker to treat heart block has also been suggested as an alternative option. In our patient, we elected to remove the device by surgery, to close the VSD by patch, and to explore the tricuspid valve leaflets aiming at a possible repair. This approach is not new.7 We believe that the risk of recurrence of life-threatening AV block and of the progression of tricuspid regurgitation after device implantation make the surgical option the more appealing. The immediate self-recovery of normal sinus rhythm following CPR encouraged our decision. Conclusion Peri-membranous VSD device occlusion can be associated with life-threatening late AV block. Removal of the device or insertion of pacemaker might be a necessary urgent management to save patient life. Considering this unpredictable complication, the decision to occlude peri-membranous VSD with Amplatzer device should be carefully considered. Conflict of interest: none declared. References 1 Predescu D,  Chaturvedi RR,  Friedberg MK,  Benson LN,  Ozawa A,  Lee KJ.  Complete heart block associated with device closure of perimembranous ventricular septal defects,  J Thorac Cardiovasc Surg ,  2008, vol.  136 (pg.  1223- 1228) Google Scholar CrossRef Search ADS PubMed  2 Erdem S,  Kızıltaş A,  Küçükosmanoğlu O,  Özbarlas N.  Temporary atrioventricular complete block that develops following the transcatheter closure of ventricular septal defect,  Turk J Pediatr ,  2012, vol.  54 (pg.  80- 82) Google Scholar PubMed  3 Carminati M,  Butera G,  Chessa M,  De Giovanni J,  Fisher G,  Gewillig M,  Peuster M,  Piechaud JF,  Santoro G,  Sievert H,  Spadoni I,  Walsh K.  Investigators of the European VSD Registry Transcatheter closure of congenital ventricular septal defects: results of the European Registry,  Eur Heart J ,  2007, vol.  28 (pg.  2361- 2368) Google Scholar CrossRef Search ADS PubMed  4 Szkutnik M,  Qureshi SA,  Kusa J,  Rosenthal E,  Bialkowski J.  Use of the Amplatzer muscular ventricular septal defect occluder for closure of perimembranous ventricular septal defects,  Heart ,  2007, vol.  93 (pg.  355- 358) Google Scholar CrossRef Search ADS PubMed  5 Butera G,  Carminati M,  Chessa M,  Piazza L,  Micheletti A,  Negura DG,  Abella R,  Giamberti A,  Frigiola A.  Transcatheter closure of perimembranous ventricular septal defects: early and long-term results,  J Am Coll Cardiol ,  2007, vol.  50 (pg.  1189- 1195) Google Scholar CrossRef Search ADS PubMed  6 Butera G,  Massimo C,  Mario C.  Late complete arteriovenous block after percutaneous closure of perimembanous ventricular septal defect,  Catheter Cardiovasc Interv ,  2006, vol.  67 (pg.  938- 941) Google Scholar CrossRef Search ADS PubMed  7 Clark J,  Chowdhury D,  Pauliks L,  Weber H.  Resolution of heart block after surgical removal of an Amplatzer device,  Ann Thorac Surg ,  2010, vol.  89 (pg.  1631- 1633) Google Scholar CrossRef Search ADS PubMed  8 Walsh MA,  Bialkowski J,  Szkutnik M,  Pawelec-Wojtalik M,  Bobkowski W,  Walsh KP.  Atrioventricular block after transcatheter closure of perimembranous ventricular septal defects,  Heart ,  2006, vol.  92 (pg.  1295- 1297) Google Scholar CrossRef Search ADS PubMed  9 Yip WC,  Zimmerman F,  Hijazi ZM.  Heart block and empirical therapy after transcatheter closure of perimembranous ventricular septal defect,  Catheter Cardiovasc Interv ,  2005, vol.  66 (pg.  436- 441) Google Scholar CrossRef Search ADS PubMed  Published on behalf of the European Society of Cardiology. 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TI - Unusual delayed presentation of life-threatening complete heart block after ventricular septal defect (VSD) closure with Amplatzer Device
JF - European Heart Journal Supplements
DO - 10.1093/eurheartj/suu028
DA - 2014-11-01
UR - https://www.deepdyve.com/lp/oxford-university-press/unusual-delayed-presentation-of-life-threatening-complete-heart-block-sA24CRSAor
SP - B72
EP - B74
VL - 16
IS - suppl_B
DP - DeepDyve
ER -