Get 20M+ Full-Text Papers For Less Than $1.50/day. Start a 14-Day Trial for You or Your Team.

Learn More →

Repair of congenital mitral valve dysplasia in infants and children: is it always possible?

Repair of congenital mitral valve dysplasia in infants and children: is it always possible? Abstract Objectives: Surgical management of congenital malformation of the mitral valve (MV) in the pediatric age group remains a therapeutic challenge for the wide spectrum of the morphological abnormalities and the high incidence of associated cardiac anomalies. We reviewed our experience so as to assess whether MV conservative surgery is always advisable and its results are superior to MV replacement. Methods: Thirty-four consecutive children (20 male and 14 female) with a mean age of 5.9 years (range 45 days–18 years) treated surgically for congenital MV disease between January 1987 and June 1999. Four patients (11.7%) were under 12 months of age, while 21 patients (62%) were younger than 5 years. Twenty-two patients presented with MV incompetence (or prevalent incompetence), while 12 presented with stenosis (or prevalent stenosis). Associated cardiac lesions were present in 22 patients (62.8%). Results: Mitral valve reconstruction was possible in all. There were no operative deaths. Three patients required reoperation for MV restenosis (a re-repair in one and MV replacement with mechanical prosthesis in two) 4 months, 27 months and 5.6 years after repair with no operative deaths. There was only one late death for prosthetic valve thrombosis. Follow-up data reveal that the 33 surviving patients are asymptomatic and well 4 months–12 years (mean 72 months) after surgery. At 12 years, actuarial survival and freedom from reoperation are 96.8 and 85.9%, respectively. Echocardiography performed in all of them shows no or mild incompetence or stenosis in 26 (78%), while residual moderate MV incompetence persists in six. Conclusions: Our experience indicates that MV reconstructive procedures in infants and children with congenital MV dysplasia may be effective and reliable with low mortality and low incidence of reoperation rate. Mitral valve repair should always be attempted, especially in infants, despite the frequent severity of MV dysplasia, to avoid the drawbacks of the currently available prostheses. Mitral valve dysplasia, Surgical repair, Early infancy 1 Introduction Congenital mitral valve (MV) dysplasia is a rare malformation characterized by a wide spectrum of morphologic abnormalities and a high incidence of associated cardiac anomalies [1–4]. Despite the recent advances in the treatment of complex congenital heart defects, surgical repair of congenital MV dysfunction still carries a considerable mortality rate [5,6]. Conservative surgery has been advocated and deemed possible in most of the patients [6–9], even in those with extremely severe MV disease [10]. Mitral valve replacement is still associated with considerable mortality at operation and a consistent permanent morbidity risk [11,12], while replacement at a younger age determines higher prevalence of reoperation [12]. The aim of this paper is to review our experience with conservative surgical treatment of congenital MV malformations in the pediatric age group over a 12-year period, so as to assess whether MV conservative surgery is always advisable and the result of this treatment is superior to MV replacement. 2 Material and methods Between January 1987 and June 1999, 34 consecutive children (20 male and 14 female), with a mean age of 5.9 years (range 45 days–18 years) underwent surgical treatment for congenital MV dysplasia. Four children (11.7%) were less than 12 months of age, and 21 (61.7%) were under 5 years of age. Mean weight at operation was 20.6 kg (range: 3.7–62.5 kg). Excluded from this study were patients with atrioventricular canal defects, left ventricle hypoplasia, atrio-ventricular and ventriculo-arterial discordance, and functional single ventricle. Preoperative symptoms consisted of exertional dyspnea, retarded growth, and repeated chest infections in 14 patients. Four children presented with congestive heart failure, two of them requiring endotracheal intubation, mechanical ventilation and inotropic agents infusion before surgery. The remaining 16 patients were asymptomatic. Electrocardiogram showed sinus rhythm in all. Mean cardio-thoracic ratio was 0.56, ranging from 0.41 to 0.71. All children were evaluated preoperatively by means of two dimensional echocardiography and color flow Doppler. Mitral valve incompetence (or prevalent incompetence) was the main dysfunction in 22 patients (64.7%). Mitral valve incompetence was quantified measuring the regurgitant volume below the orifice on color Doppler imaging (flow convergence region), according to Bargiggia et al. [13]. We have defined regurgitation as mild when maximal regurgitation flow volume (MRFV) is less than 100 ml/min per m2 (four cases), mild to moderate when MRFV ranges from 100 to 200 ml/min per m2 (five cases), moderate when MRFV ranges from 200 to 300 ml/min per m2 (11 cases), and severe when MRFV is more than 300 ml/min per m2 (two cases). In ten of these MV annulus was dilated with a mean increase of 5.7 mm (range from 2 to 9.6 mm), as compared to normal controls for body surface area [14]. Mitral valve stenosis (or prevalent stenosis) was the main valvar dysfunction in 12 patients (35.3 %), with a mean left atrium-left ventricle diastolic peak gradient of 24.1 mmHg. Preoperative cardiac catheterization was performed in 16 patients (47%) as a routine study, early in our experience. Pulmonary hypertension was severe (pulmonary pressure superior to 50% of systemic systolic pressure) in three patients and mild (mean pulmonary pressure inferior to 35 mmHg) in three. It was within normal limits in the remaining 28 patients [15]. Congenital MV malformations were classified according to Carpentier [1]. Mitral valve incompetence was divided in: type I (normal leaflet motion), 17 cases; type II (leaflet prolapse), three cases; type III (restricted leaflet motion), two cases. Mitral valve stenosis was subdivided into these categories: type A (normal papillary muscle), three cases; type B (abnormal papillary muscle), nine cases (Table 1) . Associated cardiac lesions requiring surgical maneuvers are listed in Table 2 . An aortic isthmic coarctation in 11 patients and a tetralogy of Fallot in one, were surgically corrected before MV treatment. In 12 cases (35%) MV malformation presented as an isolated anomaly. Table 1 Open in new tabDownload slide Classification according to Carpentier Table 1 Open in new tabDownload slide Classification according to Carpentier Table 2 Open in new tabDownload slide Associated cardiac malformations requiring surgical treatment in 22 patients (64.7%) Table 2 Open in new tabDownload slide Associated cardiac malformations requiring surgical treatment in 22 patients (64.7%) 2.1 Surgical technique Surgical management included MV evaluation by means of intraoperative transesophageal echocardiography (TEE) or epicardial echocardiography [16], in patients under 6 kg body weight. Particular attention was paid to understand the mechanism of MV dysfunction. Aorto-bicaval cardiopulmonary by-pass with systemic hypothermia was established in all cases; hypothermia was moderate (25°C) in 30 cases, while in patients whose weight was inferior to 4 kg (four cases) deep hypothermia (18°C rectal temperature) was reached to permit circulatory arrest; cold potassium cardioplegia was used in the first 25 patients, while more recently, cold potassium enriched blood cardioplegia was employed in the remaining nine patients. The MV was exposed either through a left atriotomy posterior to the interatrial groove (28 cases) or through the interatrial septum (six cases), depending on left atrium dimensions and the need for treating associated cardiac anomalies. The whole mitral valve apparatus was carefully inspected, with the aid of four everting pledget stitches placed at the annulus site (Fig. 1) . Valve function was hydro-dynamically assessed before and after reconstruction by means of rapid left ventricular cold saline infusion through the MV. Presence of a residual important leak at the leaflet closure site was considered as a poor result and a further treatment was attempted. Annular dilatation producing central valve incompetence was treated by means of a reductive annuloplasty at the mural leaflet starting at each commissure, similar to that described by Kay et al. [17]. Separated pledget U stitches have been employed to treat regurgitation at the commissural sites. Carpentier prosthetic ring was utilized in one 16-year-old boy who presented with pure annular dilatation. Details of the surgical techniques employed in each patient are summarized in Table 3 . Nineteen patients underwent simultaneous repair of associated intracardiac lesions, while in 11 patients an aortic isthmic coarctation was treated before MV repair (Table 3). Fig. 1 Open in new tabDownload slide Four everting pledget stitches are used to enhance exposure of the whole mitral valve apparatus. Fig. 1 Open in new tabDownload slide Four everting pledget stitches are used to enhance exposure of the whole mitral valve apparatus. Table 3 Open in new tabDownload slide Open in new tabDownload slide Surgical findings and operative techniques in patients undergoing surgical treatment of mitral valve dysplasiaa a AML, anterior mitral leaflet; ASD, atrial septal defect, ostium secundum type; AV, atrio-ventricular; F, female; M, male; MI, mitral incompetence; MS, mitral stenosis; MV, mitral valve; MVR, mitral valve replacement; PM, papillary muscle; PMCF, papillary muscle commissure fusion; PFO, patent foramen ovale; PV, pulmonary valve; VSD, ventricular septal defect. Table 3 Open in new tabDownload slide Open in new tabDownload slide Surgical findings and operative techniques in patients undergoing surgical treatment of mitral valve dysplasiaa a AML, anterior mitral leaflet; ASD, atrial septal defect, ostium secundum type; AV, atrio-ventricular; F, female; M, male; MI, mitral incompetence; MS, mitral stenosis; MV, mitral valve; MVR, mitral valve replacement; PM, papillary muscle; PMCF, papillary muscle commissure fusion; PFO, patent foramen ovale; PV, pulmonary valve; VSD, ventricular septal defect. 3 Results Mitral valve reconstruction was possible in all the children. There were no hospital deaths. There were no major postoperative surgical complications, apart from a complete atrioventricular block requiring permanent pace-maker implantation in one case (patient 14), who had undergone a subaortic stenosis resection and a conoventricular ventricular septal defect patch closure, in association to MV repair. Three children required reoperation 4, 27 and 66 months after MV repair respectively (Table 3). In all of them the MV appeared extremely malformed at the first operation (hammock valve in two cases, Shone's complex in one). Repeated conservative surgery was possible only in one child (patient 3, Table 3), after MV commissurotomy, in whom a further commissurotomy in adjunction to chordal fenestration associated to subaortic resection was performed to treat a residual functional stenosis. Two children (cases 16 and 23, Table 3) underwent successful MV replacement with mechanical prosthesis (Carbomedics size 16). One patient (case 11, Table 3), who was initially corrected for tetralogy of Fallot at the age of 7 months, required a modified Konno procedure for a recurrent tunnel-like subaortic stenosis after 73 months from MV repair and resection of discrete subaortic stenosis. 3.1 Follow-up One patient (case 16, Table 3) died late (10 months after MV repair, 6 months after MV replacement), because of valve thrombosis due to inadequate compliance to our anticoagulation program. A complete clinical and echocardiographic follow-up was obtained in all the survivors. Mean follow-up time was 72 months, ranging from 4 months to 12 years. All the patients remain asymptomatic and in sinus rhythm. None of them is under anticongestive oral therapy. Actuarial survival is 96.8%, freedom from reoperation rate is 85.9%, while freedom from MV prosthesis is 93% (Figs. 2–4) . Fig. 2 Open in new tabDownload slide Actuarial survival after mitral valve surgery. Fig. 2 Open in new tabDownload slide Actuarial survival after mitral valve surgery. Fig. 3 Open in new tabDownload slide Actuarial freedom from reoperation. Fig. 3 Open in new tabDownload slide Actuarial freedom from reoperation. Fig. 4 Open in new tabDownload slide Actuarial freedom from mitral valve prosthesis. Fig. 4 Open in new tabDownload slide Actuarial freedom from mitral valve prosthesis. Two-dimensional echocardiographic and color flow Doppler was performed in 32 patients treated conservatively. Seventeen of them have no or mild signs of mitral regurgitation or stenosis, while a moderate degree of incompetence is present in six. In nine patients a residual mild mitral stenosis is present, with a mean peak gradient of 12.6 mmHg across the MV. One surviving patient with a prosthetic valve is free of symptoms, on anticoagulation regimen with sodium warfarin. No embolic or hemorrhagic episodes are reported. 4 Conclusions Mitral valve repair for acquired disease in adults is, nowadays, a well established treatment and the long term follow up confirms its reliability [18–19]. Surgical results in children with congenitally malformed MV are reported to be not as good, because of the complex wide spectrum of morphological abnormalities, the frequent association with other cardiac anomalies, the lower incidence rate [1,2,5,6], and the consequent lesser experience with surgical repair in such a group of patients [20]. When MV malformation is diagnosed in early infancy, surgical treatment is usually delayed until symptoms develop. Technical problems at operation, such as difficulties to obtain a proper exposure of the valvar and subvalvar structures in small dimensioned hearts, and the fear for implanting a MV prosthesis which is usually oversized to the patient's MV annulus, seem to be the major concern. In our experience, we have employed deep hypothermic circulatory arrest in infants weighing less than 4 kg, and four everting stitches at the mitral annulus site, to enhance exposure of the whole valve apparatus (Fig. 1), which could always be carefully and thoroughly inspected before planning the proper surgical strategy. We have also been encouraged by Sousa Uva et al. [20], who, in their 13-year experience with MV repair in the first year of life, reported excellent results in such a difficult selected population, with low operative mortality, actuarial survival and actuarial rate of freedom from reoperation of 94 and 58%, respectively, at 7 years. We believe that a congenitally malformed MV is an active anatomical entity and the severity of its functional lesion may not be strictly related to its morphology. In our experience, the goal of surgical repair was to achieve an ‘ideal’ MV function rather than an ‘ideal’ anatomical surgical reconstruction. Good functional results can be achieved with minimal surgical maneuvers. Indeed some of our patients with severely dysplastic MV presented a residual degree of MV stenosis or regurgitation, after repair. However, an improvement in MV function can preserve the myocardial performance, alleviate clinical symptoms and delay an eventual MV replacement. Chavaud et al. [6,21] consider ring annuloplasty for MV incompetence mandatory in children older than 2 years. This statement is supported by their experience of 25% incidence rate of residual significant mitral regurgitation after repair without ring insertion [6]. Other recent reports [5,9,20] supports our initial statement that prosthetic rings, be they complete or partial, are not necessary to achieve favorable results in infants [8]. In our group of patients, ring annuloplasty has been employed only in a 16-year-old patient weighing 52 kg, early in our experience. Our current and previous results [8] have demonstrated the high effectiveness of mural leaflet annuloplasty to treat mitral incompetence, favoring any conservative procedure that does not require prosthetic material. Surgery for MV stenosis is usually associated to worse results compared to MV regurgitation [22]. In our experience, MV stenosis has been associated to a significantly higher reoperation rate (3/12 vs. 0/22, P-value ≪0.05). The goal of an effective repair is to restore an unobstructive pathway between left ventricular inflow and outflow, limiting as much as possible the potential risk of creating an incompetent MV. Surgical treatment needs to be individualized for each case, being the morphology of a stenotic MV ‘per se’ a variable [1,3]. Careful commissurotomy with or without a generous delicate splitting of papillary muscles deep into the myocardial left ventricular wall is often sufficient enough to relieve importantly the stenosis of most of the malformed valves. In parachute MV, the single papillary muscle was usually divided into two halves, as described by Carpentier [1], increasing in this way the excursion of the MV leaflets during the diastolic phase. The same principle was applied to the hammock MV and to the mitral arcade. According to Thiene et al. [23], we define the hammock valve as a very dysplastic MV, the tendinous chords are absent and the apex of the two papillary muscles is fused with the leaflet tissue at the expected areas of the commissures. From the atrial side, it looks like a hammock, having only one large leaflet with a central restrictive orifice due to lack of commissures. With the term of mitral arcade we intend a condition in which, instead of two well defined papillary muscles, there is a single ‘muscle arcade’ on which small tendinous chords attach. In agreement with other reports [1,22], a hammock MV is indeed the most difficult anomaly to correct, due to the amount of muscle that is found beneath the leaflets, which is impinging the left ventricular inflow; in this case, a generous splitting of the two huge papillary muscles has been performed in order to increase the leaflets motion, which is limited by the short and small chordae tendineae. In our experience, two patients out of six with a hammock valve required an eventual MV replacement 4 and 27 months after MV surgery (Table 3). Similarly, we have treated the mitral arcade by performing a generous longitudinal incision of the muscular structure, starting at each side of the chordal insertions and dividing the arcade into two halves. Mitral valve repair offers the advantage of avoiding the need for a lifelong anticoagulation in children. Furthermore, MV replacement in children, and particularly in infants, is associated with high mortality rate and high reoperation rate [11,12], and the maintenance of adequate anticoagulation regimen is often cumbersome [24]. The use of bioprosthetic valve substitutes in children is well known to be associated with reduced durability and early valve failure [25]. Recently, Plunkett et al. [26] have reported on four cases of MV replacement with mitral homograft in children. A long-term follow-up is not available, so as to evaluate the durability of such a homograft material in mitral position as compared to other biological substitutes. The importance of intraoperative echocardiography for functional evaluation of the surgical result soon after MV repair has been recognized elsewhere [5,6,9,10]. In addition, we believe that a further functional analysis of the malformed valve immediately before repair can provide the surgeon with important information about relevant anatomical details, leading to a better understanding of the valve dysfunction and, therefore, dictating the ideal surgical reconstruction. In conclusion, in our experience MV repair has developed into a reliable and preferable alternative to prosthetic MV replacement. Mitral valve repair in children is often a palliative procedure; however, every effort should be made to preserve the natural valve even though very dysplastic, by conservative repair techniques. Even when suboptimal, MV repair will allow a good quality of life to children, free from anticoagulation treatment. An eventual MV replacement can be possibly delayed to the adult age. ✩ Presented at the 13th Annual Meeting of the European Association for Cardio-thoracic Surgery, Glasgow, Scotland, UK, September 5–8, 1999. Dr M. Sousa Uva (Lisbon, Portugal): Dr Stellin, I want to congratulate you for these superb results, particularly to note the absent mortality and the very low reoperation rate. I wonder if you could comment on the techniques, because we reviewed the experience at Marie Lannelongue in children less than 1 year of age, and we had a higher, much higher reoperation rate, so I wonder if you could comment on the techniques that you used to treat patients with hammock or arcade mitral valve stenosis. Indeed this is a particularly difficult set of patients. We had one early valve replacement, that we had to do in one of these patients, and most of the reoperations were in these particularly difficult patients. Dr Stellin: I am aware of your recent publication of 20 cases under 1 year of age and with a very good result. There are no magic techniques in our hands. I think that our results have improved a lot since we have routinely employed intraoperative echocardiography. In addition, I think that to understand thoroughly the intrinsic mitral valve anatomy, you need very good exposure in the operative field. In small children, we shouldn't hesitate to employ deep hypothermic circulatory arrest to facilitate the visibility to the mitral valve apparatus. It is important to understand the anatomy and therefore the mechanism of dysfunction, in order to plan a proper surgical strategy. Secondly, in reconstructing a malformed valve, it is important to respect its basic anatomy. Our aim is not to achieve, at the end of reconstruction a nice-looking valve, but all we want is to improve its functional status. It is obviously a pallative procedure in most of the very dysplastic valve. Dr A. Urban (St. Augustin, Germany): Did you include mitral valves where the valve ring was hypoplastic or normally-sized valves which had pathology at the leaflet level and subvalvular apparatus? Dr Stellin: Well, as far as I am aware, in none of our cases we have found a hypoplastic ring. Obviously we had to discard those patients in which there was a hypoplasia of the left ventricle. All the cases with a functional single ventricle, are not included in our experience. Dr F. Ramirez (Guadalajara, Mexico): How much did you increase the valvular area in the parachute or arcade stenotic valves? Dr Stellin: This is a difficult question to answer. In parachute mitral valves, the annulus is usually of normal size. Obviously, the obstruction is caused by a malformed subvalvular apparatus. At that level, the valve area should be measured. It has not been measured in our patients. In parachute valves, what we have tried to achieve was to make the leaflets opening a little bit more, since they are anchored down to a single papillary muscle. A generous splitting of the papillary muscle down to the ventricular wall has been performed in all of them. Dr D. Metras (Marseille, France): One thing that did not appear very clear, to me at least, is how you made the difference between arcade and hammock valve? The other day at the postgraduate course there was a lot of talk about that. I did not understand exactly how you differed them. Dr Stellin: As far as the definition of hammock valve is concerned, we are following in Padova Dr Thiene's classification. For a hammock valve, we intend a very dysplastic mitral valve with two huge papillary muscles,and a very small cordhae attaching on these. If you look at the valve from above, it looks like a funnel. There are no defined commissures and the valve might look like a single-leaflet valve. As you remember yesterday during the post graduate course, I was asking Dr Anderson what he means for a mitral arcade and his answer was exactly what I said during my presentation. In mitral arcade instead of two well defined papillary muscles, there is a single ‘muscle arcade’ where small chordae attach on to it. References [1] Carpentier A. . Stark J. , de Leval M. . Congenital malformations of the mitral valve , Surgery for congenital heart defects , 1994 Philadelphia, PA W.B. Saunders (pg. 599 - 614 ) Google Scholar Google Preview OpenURL Placeholder Text WorldCat COPAC [2] Kirklin J.W. , Barratt-Boyes B.G. . , Cardiac surgery , 1993 New York Churchill-Livingstone (pg. 1343 - 1360 ) Google Scholar Google Preview OpenURL Placeholder Text WorldCat COPAC [3] Ruckman R.N. , Van Praagh R. . Anatomic types of congenital mitral stenosis: report of 49 autopsy cases with consideration of diagnosis and surgical implications , Am J Cardiol , 1978 , vol. 42 (pg. 592 - 601 ) Google Scholar Crossref Search ADS PubMed WorldCat [4] Shone J.D. , Sellers R.D. , Anderson R.C. , Adams P. , Lillehei C.W. , Edwards J.E. . The developmental complex of “parachute mitral valve”, supravalvular ring of left atrium, subaortic stenosis and coarctation of aorta , Am J Cardiol , 1963 , vol. 11 (pg. 714 - 725 ) Google Scholar Crossref Search ADS PubMed WorldCat [5] McCarthy J.F. , Neligan M.C. , Wood A.E. . Ten years’ experience of an aggressive reparative approach to congenital mitral valve anomalies , Eur J Cardio-thorac Surg , 1996 , vol. 10 (pg. 534 - 539 ) Google Scholar Crossref Search ADS WorldCat [6] Chauvaud S. , Fuzellier J.F. , Houel R. , Berrebi A. , Mihaileanu S. , Carpentier A. . Reconstructive surgery in congenital mitral valve insufficiency (Carpentier's techniques): long-term results , J Thorac Cardiovasc Surg , 1998 , vol. 115 (pg. 84 - 93 ) Google Scholar Crossref Search ADS PubMed WorldCat [7] Carpentier A. , Brachini B. , Cour J.C. , Asfaou E. , Villani M. , Deloche A. , Relland J. , D'Allaines C. , Blondeau P. , Piwnica A. , Parenzan L. , Brom G. . Congenital malformations of the mitral valve in children. Pathologic and surgical treatment , J Thorac Cardiovasc Surg , 1976 , vol. 6 (pg. 854 - 866 ) OpenURL Placeholder Text WorldCat [8] Stellin G. , Bortolotti U. , Mazzucco A. , Faggian G. , Guerra F. , Daliento L. , Livi U. , Gallucci V. . Repair of congenitally malformed mitral valve in children , J Thorac Cardiovasc Surg , 1988 , vol. 95 (pg. 480 - 485 ) Google Scholar PubMed OpenURL Placeholder Text WorldCat [9] Zias E.A. , Mavroudis C. , Backer C.L. , Kohr L.M. , Gotteiner N.L. , Rocchini A.P. . Surgical repair of the congenitally malformed mitral valve in infants and children , Ann Thorac Surg , 1998 , vol. 66 (pg. 1551 - 1559 ) Google Scholar Crossref Search ADS PubMed WorldCat [10] Aharon A.S. , Laks H. , Drinkwater D.C. , Chugh R. , Gates R.N. , Grant P.W. , Permut L.C. , Ardehali A. , Rudis E. . Early and late results of mitral valve repair in children , J Thorac Cardiovasc Surg , 1994 , vol. 107 (pg. 1262 - 1271 ) Google Scholar PubMed OpenURL Placeholder Text WorldCat [11] Williams W.G. , Pollock J.C. , Geiss D.M. , Trusler G.A. , Fowler R.S. . Experience with aortic and mitral valve replacement in children , J Thorac Cardiovasc Surg , 1981 , vol. 81 (pg. 326 - 333 ) Google Scholar PubMed OpenURL Placeholder Text WorldCat [12] Kadoba K. , Jonas R.A. , Mayer J.E. , Castaneda A.R. . Mitral valve replacement in the first year of life , J Thorac Cardiovasc Surg , 1990 , vol. 100 (pg. 762 - 768 ) Google Scholar PubMed OpenURL Placeholder Text WorldCat [13] Bargiggia G.S. , Tronconi L. , Sahn D.J. , Recusani F. , Raisaro A. , De Servi S. , Valdes-Cruz L.M. , Montemartini C. . A new method for quantitation of mitral regurgitation based on color flow Doppler imaging of flow convergence proximal to regurgitant orifice , Circulation , 1991 , vol. 84 (pg. 1481 - 1489 ) Google Scholar Crossref Search ADS PubMed WorldCat [14] Kirklin J.W. , Barratt-Boyes B.G. . Cardiac surgery , Churchill-Livingstone, New York , 1993 pg. 31 OpenURL Placeholder Text WorldCat [15] Fyler D.C. . , 1992 Philadelphia, PA Hanley & Belfus Inc (pg. 190 - 194 ) [16] Ungerleider R.M. , Greeley W.J. , Sheikh K.H. , Philips J. , Bennet Pearce F. , Kern F.H. , Kisslo J.A. . Routine use of intraoperative epicardial echocardiography and Doppler color flow imaging to guide and evaluate repair of congenital heart lesions. A prospective study , J Thorac Cardiovasc Surg , 1990 , vol. 100 (pg. 297 - 309 ) Google Scholar PubMed OpenURL Placeholder Text WorldCat [17] Kay J.H. , Zubiate P. , Mendez M.A. , Vanstrom N. , Yokoyama T. . Mitral valve repair for significant mitral insufficiency , Am Heart J , 1978 , vol. 96 2 (pg. 253 - 262 ) Google Scholar Crossref Search ADS PubMed WorldCat [18] Lessana A. , Tran Viet T. , Ades F. , Mostefa Kara S. , Ameur A. , Ruffenach A. , Guerin F. , Herreman F. , Degeorges M. . Mitral valve operations. A series of 130 consecutive cases , J Thorac Cardiovasc Surg , 1983 , vol. 86 (pg. 553 - 561 ) Google Scholar PubMed OpenURL Placeholder Text WorldCat [19] Spencer F.C. , Colvin S.B. , Culliford A.T. , Wayne Isom O. . Experiences with the Carpentier technique of mitral valve reconstruction in 103 patients (1980–1985) , J Thorac Cardiovasc Surg , 1985 , vol. 90 (pg. 341 - 350 ) Google Scholar PubMed OpenURL Placeholder Text WorldCat [20] Sousa Uva M. , Galletti L. , Lacour Gayet F. , Piot D. , Serraf A. , Bruniax J. , Comas J. , Roussin R. , Touchot A. , Binet J.P. , Planché C. . Surgery for congenital mitral valve disease in the first year of life , J Thorac Cardiovasc Surg , 1995 , vol. 109 1 (pg. 164 - 176 ) Google Scholar Crossref Search ADS PubMed WorldCat [21] Chauvaud S.M. , Milhaileanu S.A. , Gaer J.A.R. , Carpentier A.C. . Surgical treatment of mitral valvar insufficiency: “The Hopital Broussais” experience , Cardiol Young , 1997 , vol. 7 (pg. 5 - 14 ) OpenURL Placeholder Text WorldCat [22] Chauvaud S.M. , Milhaileanu S.A. , Gaer J.A.R. , Carpentier A.C. . Surgical treatment of mitral valvar stenosis: “The Hopital Broussais” experience , Cardiol Young , 1997 , vol. 7 (pg. 15 - 21 ) OpenURL Placeholder Text WorldCat [23] Thiene G. , Frescura C. , Daliento L. . Marcelletti C. , Anderson R.M. , Becker A.E. , Corno A. , Di Donato R.M. , Mazzera E. . The pathology of the congenitally malformed mitral valve , Pediatric cardiology , 1986 New York Churchill-Livingstone (pg. 225 - 239 ) Google Scholar Google Preview OpenURL Placeholder Text WorldCat COPAC [24] Bradley L.M. , Midgley F.M. , Watson D.C. , Getson P.R. , Scott L.P. . Anticoagulation in children with mechanical prosthetic cardiac valve , Am J Cardiol , 1985 , vol. 56 (pg. 533 - 555 ) Google Scholar Crossref Search ADS PubMed WorldCat [25] Gallucci V. , Bortolotti U. , Milano A. , Valfrè C. , Mazzucco A. , Thiene G. . Isolated mitral valve replacement with the Hancock bioprosthesis: a 13 years appraisal , Ann Thorac Surg , 1984 , vol. 38 (pg. 571 - 578 ) Google Scholar Crossref Search ADS PubMed WorldCat [26] Plunkett M.D. , Schneider D.J. , Shah J.J. , Bash S.E. , Bond L.M. , Geiss D.M. . Homograft replacement of mitral valve in children , Ann Thorac Surg; , 1998 , vol. 66 (pg. 649 - 652 ) Google Scholar Crossref Search ADS PubMed WorldCat © 2000 Published by Elsevier Science B.V. Elsevier Science B.V. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png European Journal of Cardio-Thoracic Surgery Oxford University Press

Repair of congenital mitral valve dysplasia in infants and children: is it always possible?

Download PDF
9 pages

Loading next page...
 
/lp/oxford-university-press/repair-of-congenital-mitral-valve-dysplasia-in-infants-and-children-is-w5NqoWw9DK

References (26)

Publisher
Oxford University Press
Copyright
© 2000 Published by Elsevier Science B.V.
Subject
Articles
ISSN
1010-7940
eISSN
1873-734X
DOI
10.1016/S1010-7940(00)00457-7
Publisher site
See Article on Publisher Site

Abstract

Abstract Objectives: Surgical management of congenital malformation of the mitral valve (MV) in the pediatric age group remains a therapeutic challenge for the wide spectrum of the morphological abnormalities and the high incidence of associated cardiac anomalies. We reviewed our experience so as to assess whether MV conservative surgery is always advisable and its results are superior to MV replacement. Methods: Thirty-four consecutive children (20 male and 14 female) with a mean age of 5.9 years (range 45 days–18 years) treated surgically for congenital MV disease between January 1987 and June 1999. Four patients (11.7%) were under 12 months of age, while 21 patients (62%) were younger than 5 years. Twenty-two patients presented with MV incompetence (or prevalent incompetence), while 12 presented with stenosis (or prevalent stenosis). Associated cardiac lesions were present in 22 patients (62.8%). Results: Mitral valve reconstruction was possible in all. There were no operative deaths. Three patients required reoperation for MV restenosis (a re-repair in one and MV replacement with mechanical prosthesis in two) 4 months, 27 months and 5.6 years after repair with no operative deaths. There was only one late death for prosthetic valve thrombosis. Follow-up data reveal that the 33 surviving patients are asymptomatic and well 4 months–12 years (mean 72 months) after surgery. At 12 years, actuarial survival and freedom from reoperation are 96.8 and 85.9%, respectively. Echocardiography performed in all of them shows no or mild incompetence or stenosis in 26 (78%), while residual moderate MV incompetence persists in six. Conclusions: Our experience indicates that MV reconstructive procedures in infants and children with congenital MV dysplasia may be effective and reliable with low mortality and low incidence of reoperation rate. Mitral valve repair should always be attempted, especially in infants, despite the frequent severity of MV dysplasia, to avoid the drawbacks of the currently available prostheses. Mitral valve dysplasia, Surgical repair, Early infancy 1 Introduction Congenital mitral valve (MV) dysplasia is a rare malformation characterized by a wide spectrum of morphologic abnormalities and a high incidence of associated cardiac anomalies [1–4]. Despite the recent advances in the treatment of complex congenital heart defects, surgical repair of congenital MV dysfunction still carries a considerable mortality rate [5,6]. Conservative surgery has been advocated and deemed possible in most of the patients [6–9], even in those with extremely severe MV disease [10]. Mitral valve replacement is still associated with considerable mortality at operation and a consistent permanent morbidity risk [11,12], while replacement at a younger age determines higher prevalence of reoperation [12]. The aim of this paper is to review our experience with conservative surgical treatment of congenital MV malformations in the pediatric age group over a 12-year period, so as to assess whether MV conservative surgery is always advisable and the result of this treatment is superior to MV replacement. 2 Material and methods Between January 1987 and June 1999, 34 consecutive children (20 male and 14 female), with a mean age of 5.9 years (range 45 days–18 years) underwent surgical treatment for congenital MV dysplasia. Four children (11.7%) were less than 12 months of age, and 21 (61.7%) were under 5 years of age. Mean weight at operation was 20.6 kg (range: 3.7–62.5 kg). Excluded from this study were patients with atrioventricular canal defects, left ventricle hypoplasia, atrio-ventricular and ventriculo-arterial discordance, and functional single ventricle. Preoperative symptoms consisted of exertional dyspnea, retarded growth, and repeated chest infections in 14 patients. Four children presented with congestive heart failure, two of them requiring endotracheal intubation, mechanical ventilation and inotropic agents infusion before surgery. The remaining 16 patients were asymptomatic. Electrocardiogram showed sinus rhythm in all. Mean cardio-thoracic ratio was 0.56, ranging from 0.41 to 0.71. All children were evaluated preoperatively by means of two dimensional echocardiography and color flow Doppler. Mitral valve incompetence (or prevalent incompetence) was the main dysfunction in 22 patients (64.7%). Mitral valve incompetence was quantified measuring the regurgitant volume below the orifice on color Doppler imaging (flow convergence region), according to Bargiggia et al. [13]. We have defined regurgitation as mild when maximal regurgitation flow volume (MRFV) is less than 100 ml/min per m2 (four cases), mild to moderate when MRFV ranges from 100 to 200 ml/min per m2 (five cases), moderate when MRFV ranges from 200 to 300 ml/min per m2 (11 cases), and severe when MRFV is more than 300 ml/min per m2 (two cases). In ten of these MV annulus was dilated with a mean increase of 5.7 mm (range from 2 to 9.6 mm), as compared to normal controls for body surface area [14]. Mitral valve stenosis (or prevalent stenosis) was the main valvar dysfunction in 12 patients (35.3 %), with a mean left atrium-left ventricle diastolic peak gradient of 24.1 mmHg. Preoperative cardiac catheterization was performed in 16 patients (47%) as a routine study, early in our experience. Pulmonary hypertension was severe (pulmonary pressure superior to 50% of systemic systolic pressure) in three patients and mild (mean pulmonary pressure inferior to 35 mmHg) in three. It was within normal limits in the remaining 28 patients [15]. Congenital MV malformations were classified according to Carpentier [1]. Mitral valve incompetence was divided in: type I (normal leaflet motion), 17 cases; type II (leaflet prolapse), three cases; type III (restricted leaflet motion), two cases. Mitral valve stenosis was subdivided into these categories: type A (normal papillary muscle), three cases; type B (abnormal papillary muscle), nine cases (Table 1) . Associated cardiac lesions requiring surgical maneuvers are listed in Table 2 . An aortic isthmic coarctation in 11 patients and a tetralogy of Fallot in one, were surgically corrected before MV treatment. In 12 cases (35%) MV malformation presented as an isolated anomaly. Table 1 Open in new tabDownload slide Classification according to Carpentier Table 1 Open in new tabDownload slide Classification according to Carpentier Table 2 Open in new tabDownload slide Associated cardiac malformations requiring surgical treatment in 22 patients (64.7%) Table 2 Open in new tabDownload slide Associated cardiac malformations requiring surgical treatment in 22 patients (64.7%) 2.1 Surgical technique Surgical management included MV evaluation by means of intraoperative transesophageal echocardiography (TEE) or epicardial echocardiography [16], in patients under 6 kg body weight. Particular attention was paid to understand the mechanism of MV dysfunction. Aorto-bicaval cardiopulmonary by-pass with systemic hypothermia was established in all cases; hypothermia was moderate (25°C) in 30 cases, while in patients whose weight was inferior to 4 kg (four cases) deep hypothermia (18°C rectal temperature) was reached to permit circulatory arrest; cold potassium cardioplegia was used in the first 25 patients, while more recently, cold potassium enriched blood cardioplegia was employed in the remaining nine patients. The MV was exposed either through a left atriotomy posterior to the interatrial groove (28 cases) or through the interatrial septum (six cases), depending on left atrium dimensions and the need for treating associated cardiac anomalies. The whole mitral valve apparatus was carefully inspected, with the aid of four everting pledget stitches placed at the annulus site (Fig. 1) . Valve function was hydro-dynamically assessed before and after reconstruction by means of rapid left ventricular cold saline infusion through the MV. Presence of a residual important leak at the leaflet closure site was considered as a poor result and a further treatment was attempted. Annular dilatation producing central valve incompetence was treated by means of a reductive annuloplasty at the mural leaflet starting at each commissure, similar to that described by Kay et al. [17]. Separated pledget U stitches have been employed to treat regurgitation at the commissural sites. Carpentier prosthetic ring was utilized in one 16-year-old boy who presented with pure annular dilatation. Details of the surgical techniques employed in each patient are summarized in Table 3 . Nineteen patients underwent simultaneous repair of associated intracardiac lesions, while in 11 patients an aortic isthmic coarctation was treated before MV repair (Table 3). Fig. 1 Open in new tabDownload slide Four everting pledget stitches are used to enhance exposure of the whole mitral valve apparatus. Fig. 1 Open in new tabDownload slide Four everting pledget stitches are used to enhance exposure of the whole mitral valve apparatus. Table 3 Open in new tabDownload slide Open in new tabDownload slide Surgical findings and operative techniques in patients undergoing surgical treatment of mitral valve dysplasiaa a AML, anterior mitral leaflet; ASD, atrial septal defect, ostium secundum type; AV, atrio-ventricular; F, female; M, male; MI, mitral incompetence; MS, mitral stenosis; MV, mitral valve; MVR, mitral valve replacement; PM, papillary muscle; PMCF, papillary muscle commissure fusion; PFO, patent foramen ovale; PV, pulmonary valve; VSD, ventricular septal defect. Table 3 Open in new tabDownload slide Open in new tabDownload slide Surgical findings and operative techniques in patients undergoing surgical treatment of mitral valve dysplasiaa a AML, anterior mitral leaflet; ASD, atrial septal defect, ostium secundum type; AV, atrio-ventricular; F, female; M, male; MI, mitral incompetence; MS, mitral stenosis; MV, mitral valve; MVR, mitral valve replacement; PM, papillary muscle; PMCF, papillary muscle commissure fusion; PFO, patent foramen ovale; PV, pulmonary valve; VSD, ventricular septal defect. 3 Results Mitral valve reconstruction was possible in all the children. There were no hospital deaths. There were no major postoperative surgical complications, apart from a complete atrioventricular block requiring permanent pace-maker implantation in one case (patient 14), who had undergone a subaortic stenosis resection and a conoventricular ventricular septal defect patch closure, in association to MV repair. Three children required reoperation 4, 27 and 66 months after MV repair respectively (Table 3). In all of them the MV appeared extremely malformed at the first operation (hammock valve in two cases, Shone's complex in one). Repeated conservative surgery was possible only in one child (patient 3, Table 3), after MV commissurotomy, in whom a further commissurotomy in adjunction to chordal fenestration associated to subaortic resection was performed to treat a residual functional stenosis. Two children (cases 16 and 23, Table 3) underwent successful MV replacement with mechanical prosthesis (Carbomedics size 16). One patient (case 11, Table 3), who was initially corrected for tetralogy of Fallot at the age of 7 months, required a modified Konno procedure for a recurrent tunnel-like subaortic stenosis after 73 months from MV repair and resection of discrete subaortic stenosis. 3.1 Follow-up One patient (case 16, Table 3) died late (10 months after MV repair, 6 months after MV replacement), because of valve thrombosis due to inadequate compliance to our anticoagulation program. A complete clinical and echocardiographic follow-up was obtained in all the survivors. Mean follow-up time was 72 months, ranging from 4 months to 12 years. All the patients remain asymptomatic and in sinus rhythm. None of them is under anticongestive oral therapy. Actuarial survival is 96.8%, freedom from reoperation rate is 85.9%, while freedom from MV prosthesis is 93% (Figs. 2–4) . Fig. 2 Open in new tabDownload slide Actuarial survival after mitral valve surgery. Fig. 2 Open in new tabDownload slide Actuarial survival after mitral valve surgery. Fig. 3 Open in new tabDownload slide Actuarial freedom from reoperation. Fig. 3 Open in new tabDownload slide Actuarial freedom from reoperation. Fig. 4 Open in new tabDownload slide Actuarial freedom from mitral valve prosthesis. Fig. 4 Open in new tabDownload slide Actuarial freedom from mitral valve prosthesis. Two-dimensional echocardiographic and color flow Doppler was performed in 32 patients treated conservatively. Seventeen of them have no or mild signs of mitral regurgitation or stenosis, while a moderate degree of incompetence is present in six. In nine patients a residual mild mitral stenosis is present, with a mean peak gradient of 12.6 mmHg across the MV. One surviving patient with a prosthetic valve is free of symptoms, on anticoagulation regimen with sodium warfarin. No embolic or hemorrhagic episodes are reported. 4 Conclusions Mitral valve repair for acquired disease in adults is, nowadays, a well established treatment and the long term follow up confirms its reliability [18–19]. Surgical results in children with congenitally malformed MV are reported to be not as good, because of the complex wide spectrum of morphological abnormalities, the frequent association with other cardiac anomalies, the lower incidence rate [1,2,5,6], and the consequent lesser experience with surgical repair in such a group of patients [20]. When MV malformation is diagnosed in early infancy, surgical treatment is usually delayed until symptoms develop. Technical problems at operation, such as difficulties to obtain a proper exposure of the valvar and subvalvar structures in small dimensioned hearts, and the fear for implanting a MV prosthesis which is usually oversized to the patient's MV annulus, seem to be the major concern. In our experience, we have employed deep hypothermic circulatory arrest in infants weighing less than 4 kg, and four everting stitches at the mitral annulus site, to enhance exposure of the whole valve apparatus (Fig. 1), which could always be carefully and thoroughly inspected before planning the proper surgical strategy. We have also been encouraged by Sousa Uva et al. [20], who, in their 13-year experience with MV repair in the first year of life, reported excellent results in such a difficult selected population, with low operative mortality, actuarial survival and actuarial rate of freedom from reoperation of 94 and 58%, respectively, at 7 years. We believe that a congenitally malformed MV is an active anatomical entity and the severity of its functional lesion may not be strictly related to its morphology. In our experience, the goal of surgical repair was to achieve an ‘ideal’ MV function rather than an ‘ideal’ anatomical surgical reconstruction. Good functional results can be achieved with minimal surgical maneuvers. Indeed some of our patients with severely dysplastic MV presented a residual degree of MV stenosis or regurgitation, after repair. However, an improvement in MV function can preserve the myocardial performance, alleviate clinical symptoms and delay an eventual MV replacement. Chavaud et al. [6,21] consider ring annuloplasty for MV incompetence mandatory in children older than 2 years. This statement is supported by their experience of 25% incidence rate of residual significant mitral regurgitation after repair without ring insertion [6]. Other recent reports [5,9,20] supports our initial statement that prosthetic rings, be they complete or partial, are not necessary to achieve favorable results in infants [8]. In our group of patients, ring annuloplasty has been employed only in a 16-year-old patient weighing 52 kg, early in our experience. Our current and previous results [8] have demonstrated the high effectiveness of mural leaflet annuloplasty to treat mitral incompetence, favoring any conservative procedure that does not require prosthetic material. Surgery for MV stenosis is usually associated to worse results compared to MV regurgitation [22]. In our experience, MV stenosis has been associated to a significantly higher reoperation rate (3/12 vs. 0/22, P-value ≪0.05). The goal of an effective repair is to restore an unobstructive pathway between left ventricular inflow and outflow, limiting as much as possible the potential risk of creating an incompetent MV. Surgical treatment needs to be individualized for each case, being the morphology of a stenotic MV ‘per se’ a variable [1,3]. Careful commissurotomy with or without a generous delicate splitting of papillary muscles deep into the myocardial left ventricular wall is often sufficient enough to relieve importantly the stenosis of most of the malformed valves. In parachute MV, the single papillary muscle was usually divided into two halves, as described by Carpentier [1], increasing in this way the excursion of the MV leaflets during the diastolic phase. The same principle was applied to the hammock MV and to the mitral arcade. According to Thiene et al. [23], we define the hammock valve as a very dysplastic MV, the tendinous chords are absent and the apex of the two papillary muscles is fused with the leaflet tissue at the expected areas of the commissures. From the atrial side, it looks like a hammock, having only one large leaflet with a central restrictive orifice due to lack of commissures. With the term of mitral arcade we intend a condition in which, instead of two well defined papillary muscles, there is a single ‘muscle arcade’ on which small tendinous chords attach. In agreement with other reports [1,22], a hammock MV is indeed the most difficult anomaly to correct, due to the amount of muscle that is found beneath the leaflets, which is impinging the left ventricular inflow; in this case, a generous splitting of the two huge papillary muscles has been performed in order to increase the leaflets motion, which is limited by the short and small chordae tendineae. In our experience, two patients out of six with a hammock valve required an eventual MV replacement 4 and 27 months after MV surgery (Table 3). Similarly, we have treated the mitral arcade by performing a generous longitudinal incision of the muscular structure, starting at each side of the chordal insertions and dividing the arcade into two halves. Mitral valve repair offers the advantage of avoiding the need for a lifelong anticoagulation in children. Furthermore, MV replacement in children, and particularly in infants, is associated with high mortality rate and high reoperation rate [11,12], and the maintenance of adequate anticoagulation regimen is often cumbersome [24]. The use of bioprosthetic valve substitutes in children is well known to be associated with reduced durability and early valve failure [25]. Recently, Plunkett et al. [26] have reported on four cases of MV replacement with mitral homograft in children. A long-term follow-up is not available, so as to evaluate the durability of such a homograft material in mitral position as compared to other biological substitutes. The importance of intraoperative echocardiography for functional evaluation of the surgical result soon after MV repair has been recognized elsewhere [5,6,9,10]. In addition, we believe that a further functional analysis of the malformed valve immediately before repair can provide the surgeon with important information about relevant anatomical details, leading to a better understanding of the valve dysfunction and, therefore, dictating the ideal surgical reconstruction. In conclusion, in our experience MV repair has developed into a reliable and preferable alternative to prosthetic MV replacement. Mitral valve repair in children is often a palliative procedure; however, every effort should be made to preserve the natural valve even though very dysplastic, by conservative repair techniques. Even when suboptimal, MV repair will allow a good quality of life to children, free from anticoagulation treatment. An eventual MV replacement can be possibly delayed to the adult age. ✩ Presented at the 13th Annual Meeting of the European Association for Cardio-thoracic Surgery, Glasgow, Scotland, UK, September 5–8, 1999. Dr M. Sousa Uva (Lisbon, Portugal): Dr Stellin, I want to congratulate you for these superb results, particularly to note the absent mortality and the very low reoperation rate. I wonder if you could comment on the techniques, because we reviewed the experience at Marie Lannelongue in children less than 1 year of age, and we had a higher, much higher reoperation rate, so I wonder if you could comment on the techniques that you used to treat patients with hammock or arcade mitral valve stenosis. Indeed this is a particularly difficult set of patients. We had one early valve replacement, that we had to do in one of these patients, and most of the reoperations were in these particularly difficult patients. Dr Stellin: I am aware of your recent publication of 20 cases under 1 year of age and with a very good result. There are no magic techniques in our hands. I think that our results have improved a lot since we have routinely employed intraoperative echocardiography. In addition, I think that to understand thoroughly the intrinsic mitral valve anatomy, you need very good exposure in the operative field. In small children, we shouldn't hesitate to employ deep hypothermic circulatory arrest to facilitate the visibility to the mitral valve apparatus. It is important to understand the anatomy and therefore the mechanism of dysfunction, in order to plan a proper surgical strategy. Secondly, in reconstructing a malformed valve, it is important to respect its basic anatomy. Our aim is not to achieve, at the end of reconstruction a nice-looking valve, but all we want is to improve its functional status. It is obviously a pallative procedure in most of the very dysplastic valve. Dr A. Urban (St. Augustin, Germany): Did you include mitral valves where the valve ring was hypoplastic or normally-sized valves which had pathology at the leaflet level and subvalvular apparatus? Dr Stellin: Well, as far as I am aware, in none of our cases we have found a hypoplastic ring. Obviously we had to discard those patients in which there was a hypoplasia of the left ventricle. All the cases with a functional single ventricle, are not included in our experience. Dr F. Ramirez (Guadalajara, Mexico): How much did you increase the valvular area in the parachute or arcade stenotic valves? Dr Stellin: This is a difficult question to answer. In parachute mitral valves, the annulus is usually of normal size. Obviously, the obstruction is caused by a malformed subvalvular apparatus. At that level, the valve area should be measured. It has not been measured in our patients. In parachute valves, what we have tried to achieve was to make the leaflets opening a little bit more, since they are anchored down to a single papillary muscle. A generous splitting of the papillary muscle down to the ventricular wall has been performed in all of them. Dr D. Metras (Marseille, France): One thing that did not appear very clear, to me at least, is how you made the difference between arcade and hammock valve? The other day at the postgraduate course there was a lot of talk about that. I did not understand exactly how you differed them. Dr Stellin: As far as the definition of hammock valve is concerned, we are following in Padova Dr Thiene's classification. For a hammock valve, we intend a very dysplastic mitral valve with two huge papillary muscles,and a very small cordhae attaching on these. If you look at the valve from above, it looks like a funnel. There are no defined commissures and the valve might look like a single-leaflet valve. As you remember yesterday during the post graduate course, I was asking Dr Anderson what he means for a mitral arcade and his answer was exactly what I said during my presentation. In mitral arcade instead of two well defined papillary muscles, there is a single ‘muscle arcade’ where small chordae attach on to it. References [1] Carpentier A. . Stark J. , de Leval M. . Congenital malformations of the mitral valve , Surgery for congenital heart defects , 1994 Philadelphia, PA W.B. Saunders (pg. 599 - 614 ) Google Scholar Google Preview OpenURL Placeholder Text WorldCat COPAC [2] Kirklin J.W. , Barratt-Boyes B.G. . , Cardiac surgery , 1993 New York Churchill-Livingstone (pg. 1343 - 1360 ) Google Scholar Google Preview OpenURL Placeholder Text WorldCat COPAC [3] Ruckman R.N. , Van Praagh R. . Anatomic types of congenital mitral stenosis: report of 49 autopsy cases with consideration of diagnosis and surgical implications , Am J Cardiol , 1978 , vol. 42 (pg. 592 - 601 ) Google Scholar Crossref Search ADS PubMed WorldCat [4] Shone J.D. , Sellers R.D. , Anderson R.C. , Adams P. , Lillehei C.W. , Edwards J.E. . The developmental complex of “parachute mitral valve”, supravalvular ring of left atrium, subaortic stenosis and coarctation of aorta , Am J Cardiol , 1963 , vol. 11 (pg. 714 - 725 ) Google Scholar Crossref Search ADS PubMed WorldCat [5] McCarthy J.F. , Neligan M.C. , Wood A.E. . Ten years’ experience of an aggressive reparative approach to congenital mitral valve anomalies , Eur J Cardio-thorac Surg , 1996 , vol. 10 (pg. 534 - 539 ) Google Scholar Crossref Search ADS WorldCat [6] Chauvaud S. , Fuzellier J.F. , Houel R. , Berrebi A. , Mihaileanu S. , Carpentier A. . Reconstructive surgery in congenital mitral valve insufficiency (Carpentier's techniques): long-term results , J Thorac Cardiovasc Surg , 1998 , vol. 115 (pg. 84 - 93 ) Google Scholar Crossref Search ADS PubMed WorldCat [7] Carpentier A. , Brachini B. , Cour J.C. , Asfaou E. , Villani M. , Deloche A. , Relland J. , D'Allaines C. , Blondeau P. , Piwnica A. , Parenzan L. , Brom G. . Congenital malformations of the mitral valve in children. Pathologic and surgical treatment , J Thorac Cardiovasc Surg , 1976 , vol. 6 (pg. 854 - 866 ) OpenURL Placeholder Text WorldCat [8] Stellin G. , Bortolotti U. , Mazzucco A. , Faggian G. , Guerra F. , Daliento L. , Livi U. , Gallucci V. . Repair of congenitally malformed mitral valve in children , J Thorac Cardiovasc Surg , 1988 , vol. 95 (pg. 480 - 485 ) Google Scholar PubMed OpenURL Placeholder Text WorldCat [9] Zias E.A. , Mavroudis C. , Backer C.L. , Kohr L.M. , Gotteiner N.L. , Rocchini A.P. . Surgical repair of the congenitally malformed mitral valve in infants and children , Ann Thorac Surg , 1998 , vol. 66 (pg. 1551 - 1559 ) Google Scholar Crossref Search ADS PubMed WorldCat [10] Aharon A.S. , Laks H. , Drinkwater D.C. , Chugh R. , Gates R.N. , Grant P.W. , Permut L.C. , Ardehali A. , Rudis E. . Early and late results of mitral valve repair in children , J Thorac Cardiovasc Surg , 1994 , vol. 107 (pg. 1262 - 1271 ) Google Scholar PubMed OpenURL Placeholder Text WorldCat [11] Williams W.G. , Pollock J.C. , Geiss D.M. , Trusler G.A. , Fowler R.S. . Experience with aortic and mitral valve replacement in children , J Thorac Cardiovasc Surg , 1981 , vol. 81 (pg. 326 - 333 ) Google Scholar PubMed OpenURL Placeholder Text WorldCat [12] Kadoba K. , Jonas R.A. , Mayer J.E. , Castaneda A.R. . Mitral valve replacement in the first year of life , J Thorac Cardiovasc Surg , 1990 , vol. 100 (pg. 762 - 768 ) Google Scholar PubMed OpenURL Placeholder Text WorldCat [13] Bargiggia G.S. , Tronconi L. , Sahn D.J. , Recusani F. , Raisaro A. , De Servi S. , Valdes-Cruz L.M. , Montemartini C. . A new method for quantitation of mitral regurgitation based on color flow Doppler imaging of flow convergence proximal to regurgitant orifice , Circulation , 1991 , vol. 84 (pg. 1481 - 1489 ) Google Scholar Crossref Search ADS PubMed WorldCat [14] Kirklin J.W. , Barratt-Boyes B.G. . Cardiac surgery , Churchill-Livingstone, New York , 1993 pg. 31 OpenURL Placeholder Text WorldCat [15] Fyler D.C. . , 1992 Philadelphia, PA Hanley & Belfus Inc (pg. 190 - 194 ) [16] Ungerleider R.M. , Greeley W.J. , Sheikh K.H. , Philips J. , Bennet Pearce F. , Kern F.H. , Kisslo J.A. . Routine use of intraoperative epicardial echocardiography and Doppler color flow imaging to guide and evaluate repair of congenital heart lesions. A prospective study , J Thorac Cardiovasc Surg , 1990 , vol. 100 (pg. 297 - 309 ) Google Scholar PubMed OpenURL Placeholder Text WorldCat [17] Kay J.H. , Zubiate P. , Mendez M.A. , Vanstrom N. , Yokoyama T. . Mitral valve repair for significant mitral insufficiency , Am Heart J , 1978 , vol. 96 2 (pg. 253 - 262 ) Google Scholar Crossref Search ADS PubMed WorldCat [18] Lessana A. , Tran Viet T. , Ades F. , Mostefa Kara S. , Ameur A. , Ruffenach A. , Guerin F. , Herreman F. , Degeorges M. . Mitral valve operations. A series of 130 consecutive cases , J Thorac Cardiovasc Surg , 1983 , vol. 86 (pg. 553 - 561 ) Google Scholar PubMed OpenURL Placeholder Text WorldCat [19] Spencer F.C. , Colvin S.B. , Culliford A.T. , Wayne Isom O. . Experiences with the Carpentier technique of mitral valve reconstruction in 103 patients (1980–1985) , J Thorac Cardiovasc Surg , 1985 , vol. 90 (pg. 341 - 350 ) Google Scholar PubMed OpenURL Placeholder Text WorldCat [20] Sousa Uva M. , Galletti L. , Lacour Gayet F. , Piot D. , Serraf A. , Bruniax J. , Comas J. , Roussin R. , Touchot A. , Binet J.P. , Planché C. . Surgery for congenital mitral valve disease in the first year of life , J Thorac Cardiovasc Surg , 1995 , vol. 109 1 (pg. 164 - 176 ) Google Scholar Crossref Search ADS PubMed WorldCat [21] Chauvaud S.M. , Milhaileanu S.A. , Gaer J.A.R. , Carpentier A.C. . Surgical treatment of mitral valvar insufficiency: “The Hopital Broussais” experience , Cardiol Young , 1997 , vol. 7 (pg. 5 - 14 ) OpenURL Placeholder Text WorldCat [22] Chauvaud S.M. , Milhaileanu S.A. , Gaer J.A.R. , Carpentier A.C. . Surgical treatment of mitral valvar stenosis: “The Hopital Broussais” experience , Cardiol Young , 1997 , vol. 7 (pg. 15 - 21 ) OpenURL Placeholder Text WorldCat [23] Thiene G. , Frescura C. , Daliento L. . Marcelletti C. , Anderson R.M. , Becker A.E. , Corno A. , Di Donato R.M. , Mazzera E. . The pathology of the congenitally malformed mitral valve , Pediatric cardiology , 1986 New York Churchill-Livingstone (pg. 225 - 239 ) Google Scholar Google Preview OpenURL Placeholder Text WorldCat COPAC [24] Bradley L.M. , Midgley F.M. , Watson D.C. , Getson P.R. , Scott L.P. . Anticoagulation in children with mechanical prosthetic cardiac valve , Am J Cardiol , 1985 , vol. 56 (pg. 533 - 555 ) Google Scholar Crossref Search ADS PubMed WorldCat [25] Gallucci V. , Bortolotti U. , Milano A. , Valfrè C. , Mazzucco A. , Thiene G. . Isolated mitral valve replacement with the Hancock bioprosthesis: a 13 years appraisal , Ann Thorac Surg , 1984 , vol. 38 (pg. 571 - 578 ) Google Scholar Crossref Search ADS PubMed WorldCat [26] Plunkett M.D. , Schneider D.J. , Shah J.J. , Bash S.E. , Bond L.M. , Geiss D.M. . Homograft replacement of mitral valve in children , Ann Thorac Surg; , 1998 , vol. 66 (pg. 649 - 652 ) Google Scholar Crossref Search ADS PubMed WorldCat © 2000 Published by Elsevier Science B.V. Elsevier Science B.V.

Journal

European Journal of Cardio-Thoracic Surgery – Oxford University Press

Published: Jul 1, 2000

Keywords: Mitral valve dysplasia Surgical repair Early infancy

There are no references for this article.