Supravalvar Pulmonary Stenosis Research Articles

Simultaneous Repair of Supravalvar Aortic and Supravalvar Pulmonary Stenosis Using the 3-Patch Technique

Patients with Williams-Beuren syndrome may have bilateral outflow tract obstruction. We report a simultaneous repair of supravalvar aortic and supravalvar pulmonary stenosis using the 3-patch technique.

Long-Term Outcomes of Patients With Cardiovascular Abnormalities and Williams Syndrome

Williams syndrome (WS) is a congenital disorder affecting the vascular, connective tissue, and central nervous systems of 1 in 8,000 live births. Previous reports have reported high frequencies of cardiovascular abnormalities (CVAs) in small numbers of patients with WS. A retrospective review was undertaken of patients with WS evaluated at our institution from January 1, 1980 through December 31, 2007. WS was diagnosed by an experienced medical geneticist and/or by fluorescence in situ hybridization. CVAs were diagnosed using echocardiography, cardiac catheterization, or computed tomographic angiography. Freedom from intervention was determined using Kaplan-Meier analysis. The study group was 270 patients with WS. The age at presentation was 3.3 +/- 5.9 years with follow-up of 8.9 +/- 9.0 years (range 0 to 56.9). CVAs were present in 82% of the patients. The most common lesions were supravalvar aortic stenosis in 45% and peripheral pulmonary stenosis in 37%; 20% had both. Other common lesions included mitral valve prolapse and regurgitation in 15%, ventricular septal defect in 13%, and supravalvar pulmonary stenosis in 12%. Surgical or catheter-based interventions were performed in 21%. The rate of freedom from intervention was 91%, 81%, 78%, 72%, and 62% at 1, 5, 10, 20, and 40 years. Eight patients died. In conclusion, CVAs are common in patients with WS, but supravalvar aortic stenosis and peripheral pulmonary stenosis occurred less frequently in this large cohort than previously reported. In patients with WS and CVAs, interventions are common and usually occur by 5 years of age. Most patients with WS do not require intervention during long-term follow-up, and the overall mortality has been low.

Management of supravalvar aortic stenosis and severely depressed left ventricular function in a neonate with Williams syndrome

We report an interesting case of a patient with Williams syndrome who presented with moderate supravalvar aortic stenosis and bilateral pulmonary artery stenosis at one week of age. The supravalvar aortic stenosis became severe by the age of one month with severe depression of left ventricular function. The patient had a difficult postoperative course, developed an acquired aortic arch hypoplasia and required multiple interventions during the first two months of life with an excellent outcome. The management of this difficult patient is discussed with focus on the importance of close follow-up, early diagnosis and early surgical intervention in improving the outcome in this difficult group of patients.

Does the degree of preoperative mitral regurgitation predict survival or the need for mitral valve repair or replacement in patients with anomalous origin of the left coronary artery from the pulmonary artery?

Anomalous origin of the left coronary artery from the pulmonary artery causes severe myocardial ischemia, global left ventricular dysfunction, and annular dilatation producing varying degrees of mitral regurgitation. Mitral regurgitation secondary to the left ventricular or papillary muscle dysfunction in infants will usually improve in the absence of ongoing ischemia. The aim of this study is to determine the influence of the degree of preoperative mitral regurgitation on the early and late outcomes of patients with anomalous origin of the left coronary artery from the pulmonary artery who underwent coronary reimplantation. Twenty-five patients underwent coronary reimplantation and 1 early patient had ligation during a 30-year period (median age, 4 months; range, 1 month to 16 years), with a median follow-up of 7 years (range, 4 months to 25 years). Before repair, 7 infants (27%) presented in extremis requiring ventilatory and inotropic support, and 17 patients (65%) presented with heart failure. Mitral regurgitation was present in all patients: trivial in 6 patients, mild in 12 patients, moderate in 5 patients, and severe in 3 patients. No patient underwent mitral valve repair or replacement at the time of anomalous origin of the left coronary artery from the pulmonary artery repair. Hospital survival was 92%. Three patients underwent mitral valve repair or replacement at the mean time of 3.5 years (all with severe preoperative mitral regurgitation). The degree of mitral regurgitation gradually improved in all remaining patients with preoperative mild and moderate mitral regurgitation. Echocardiographic studies demonstrated improvement in left ventricular function in all children. None of the patients showed any evidence of supravalvar pulmonary stenosis as a result of their pulmonary artery reconstruction. Long-term clinical outcome and left ventricular function are good despite severe left ventricular dysfunction at presentation. Mitral valve repair or replacement is generally not necessary at the time of anomalous origin of the left coronary artery from the pulmonary artery repair. Significant residual mitral regurgitation is present in some patients and can usually be managed surgically at a later date depending on its degree of severity.

Análise da técnica de takeuchi usada para o tratamento da origem anômala da artéria coronária esquerda do tronco pulmonar

Evaluation of a case series of anomalous origin of the left coronary artery from the pulmonary artery corrected with the Takeuchi procedure, analyzing their immediate and late surgical outcomes as well as the related morbidity. To report a case series of anomalous origin of the left coronary artery from the pulmonary artery corrected with the Takeuchi procedure. The analysis was based on data collected from the medical records of 12 patients undergoing surgical correction between 1981 and 2003. The mean age of the patients was 5.9 +/- 1.7 months. The predominant manifestation was heart failure, especially in the infants. The baseline electrocardiogram showed a Q wave in DI and aVL and signs of myocardial ischemia in ten cases. Echocardiogram with a pattern of dilated cardiomyopathy and mitral regurgitation was found in six patients. The mean age at surgical correction was 31.8 +/- 14.3 months. The immediate surgical mortality was 16% (two patients), from cardiac dysfunction. In the outpatient follow-up we observed that the symptoms, ischemic electrocardiographic changes, cardiac function and mitral regurgitation improved. Three cases progressed with supravalvar pulmonary stenosis during follow-up. This heart disease should be suspected in every infant presenting with heart failure. Surgical correction with the Takeuchi procedure has proven efficient, with a low mortality rate. Cardiac function returns to normal and mitral regurgitation improves after surgical correction.

P157: Results of anomalous origin of the left coronary artery from the pulmonary artery in children

Background: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) causes severe myocardial ischemia, which can produce papillary muscle dysfunction, annular dilatation secondary to global left ventricular (LV) dysfunction, and frank myocardial infarction with papillary muscle rupture. Mitral regurgitation (MR) secondary to the LV and/or papillary muscle dysfunction will usually improve in the absence of continuing ischemia. The aim of this study is to determine the influence of mitral valve (MV) pathology on early and late outcomes of patients with ALCAPA who underwent a surgical repair. Methods: Twenty-five patients underwent operations over a 30-year period (median age, 4 months; range, 1 month-16 years) with a median follow-up of 7 years (range, 2 months-30 years). Before repair, 11 infants (44%) presented in extremis requiring ventilatory and inotropic support, and 16 infants (64%) presented in heart failure. MR was present in 18 patients (72%); mild in 10, moderate in 5, and severe in 3. None of the patients underwent MV repair at the same time as the ALCAPA repair. Results: Hospital survival was 96%. Three patients underwent MV repair at the mean time of 3.5 years (all with preoperative severe MR). The degree of MR gradually improved in all patients with preoperative mild and moderate MR. Echocardiographic studies demonstrated improvement in LV function in all children. None of the patients showed any evidence of supravalvar pulmonary stenosis. Conclusion: Long-term clinical outcome and LV function are good, despite severe LV dysfunction at presentation. Mitral valve repair or replacement is not generally necessary at the time of the initial operation. Residual or recurrent MR is present in some of patients and can be managed accordingly to its degree of severity.

Pathologic and Molecular Analysis in a Family with Rare Mixed Supravalvar Aortic and Pulmonic Stenosis

Nonsyndromic supravalvar aortic stenosis (SVAS) is an obstructive vascular disorder often inherited in an autosomal dominant manner. With pulmonary artery involvement, stenotic lesions are nearly always peripheral or downstream of the pulmonic valve. In rare cases when the supravalvar pulmonic region is affected, the stenoses usually improve over time and rarely affect prognosis. We evaluated a unique family in which 10 of 14 individuals have nonsyndromic SVAS and 7 of the 10 affected family members with SVAS have the rare finding of supravalvar pulmonic stenosis (SVPS). In at least 2 of these individuals, the severity of SVPS was so significant that it led to death in early infancy. Pathologic examination of stenotic lesions in this kindred group revealed concentrically organized smooth muscle cells separated by dense elastic fibers. In contrast, the arterial pathology reported for other individuals with nonsyndromic SVAS demonstrates increased numbers of hypertrophied smooth muscle cells separated by thin, fragmented elastin fibers. Molecular analysis identified a novel ELN mutation within the donor splice site of exon 16, which may be responsible for the unique phenotype and distinct elastin histopathology found in this kindred.

Invited commentary

From a broad perspective the arterial switch operation is among the most intellectually satisfying procedures commonly performed by congenital heart surgeons because it seems truly curative. Incorrectly connected structures are transformed into correctly connected structures by means of an elegant, conceptually simple procedure. On closer examination, as with most apparently simple procedures, the importance of the small details of the conduct of the operation becomes apparent, particularly in regard to the coronary artery transfer, late neoaortic valve function, and the degree of patency of the main and branch pulmonary arteries. In the evolution of the procedure, numerous technical modifications to address each of these aspects have been introduced with consequent ever-improving early and late results. One of the areas of greatest concern continues to be stenosis in the reconstructed neopulmonary artery, which has been observed in virtually every large series of arterial switch operations, with an incidence as high as 30%. Since the introduction of the arterial switch operation by Jatene and colleagues [1Jatene A.D. Fontes V.F. Paulista P.P. et al.Anatomic correction of transposition of the great vessels.J Thorac Cardiovasc Surg. 1976; 72: 364-370PubMed Google Scholar], and the addition of the Lecompte maneuver to place the pulmonary confluence anterior to the reconstructed aorta [2Lecompte Y. Zannini L. Hazan E. et al.Anatomic correction of transposition of the great arteries.J Thorac Cardiovasc Surg. 1981; 82: 629-631PubMed Google Scholar], a variety of techniques have been applied in an attempt to reduce the occurrence of supravalvar pulmonary stenosis, each based on a specific understanding of the cause of the stenosis. Commonly implicated factors in the development of stenosis include excessive tension on the suture line in the pulmonary artery and involution or shrinkage of the patches used to repair the defect left in the anterior great vessel trunk by removal of the coronary artery buttons. The study in the present issue by Ullman and colleagues [3Ullmann M.V. Gorenflo M. Bolenz C. et al.Late results after extended pulmonary artery reconstruction in the arterial switch operation.Ann Thorac Surg. 2006; 81: 2259-2266Abstract Full Text Full Text PDF PubMed Scopus (18) Google Scholar] describes a novel approach to prevention of supravalvar pulmonary stenosis, addressing the dual issues of excessive suture line tension and patch shrinkage, with very impressive mid-term and late results [3Ullmann M.V. Gorenflo M. Bolenz C. et al.Late results after extended pulmonary artery reconstruction in the arterial switch operation.Ann Thorac Surg. 2006; 81: 2259-2266Abstract Full Text Full Text PDF PubMed Scopus (18) Google Scholar]. Their technique, which is a literal extension of the two-patch technique introduced by Quaegebeur and colleagues [4Quaegebeur J.M. Rohmer J. Ottenkamp J. et al.The arterial switch operation. An eight-year experience.J Thorac Cardiovasc Surg. 1986; 92: 361-384PubMed Google Scholar], uses glutaraldehyde tanning to prevent shrinkage of the autologous pericardial patches used for pulmonary artery reconstruction. The novel feature of the present report is the use of an intentionally oversized triangular patch to repair the left neopulmonary sinus and simultaneously enlarge the posterior wall of the pulmonary bifurcation to minimize anastomotic traction. The technique is inventive, well described, and well illustrated, and the follow-up in the article is thorough. The fact that none of the patients described have yet required reintervention for supravalvar pulmonary stenosis is a testimony to the effectiveness of the technique, but as an endpoint must be said to be in part reflective of physician behavior, in addition to actual patient biology. Furthermore, although it is likely that most pulmonary stenosis after an arterial switch operation will occur early, ongoing assessment of this patient cohort will be required. The authors are to be congratulated for their success, but given the history of the arterial switch operation to date, it unlikely that this report represents the last word on the subject.

Late distortion of the original Palmaz stent implanted in postoperative lesions associated with congenital heart disease

The objective of this study was to report late distortion of a Palmaz stent. Late distortion of an original Palmaz stent, implanted in an extracardiac lesion, is rare. We completed a 1-year follow-up of 54 patients who had been implanted with 80 Palmaz stents in extracardiac lesions. Distortion of two stents was detected in two patients. For case 1, we implanted a P188 stent for supravalvar pulmonary stenosis complicating an arterial switch operation in a 14-year-old girl. Seven months later, we found compression of the stent. Although we implanted two P308 stents anterior to the distorted stent, distortion of both stents developed after 1 month. Two more P308 stents placed inside each stent were gradually recompressed. A CAT scan showed compression of the stent by a dilated sinus of valsalva. For case 2, we implanted a P308 stent for stenosis of the superior vena cava after Williams operation in an 11-year-old boy. A chest X-ray documented longitudinal compression of the stent 27 months after implantation and a CAT scan showed the ascending aorta was in contact with the stent. A Palmaz stent may be distorted when implanted in a lesion adjacent to a pulsating aorta.

Selective timing for the arterial switch operation

Background To determine outcomes for the arterial switch operation individualized according to the underlying anatomy and clinical status. Methods A retrospective review of a consecutive series of infants less than 90 days of age who underwent the arterial switch operation at a single institution. Results From July 1993–April 2001, 117 infants underwent an arterial switch operation before 90 days of age. Seventy-five patients (64%) had transposition of the great arteries with intact ventricular septum with the aim of operation before 14 days of age; however, 8 of these patients had delayed presentation (range 15–46 days). Thirty-five patients (30%) had transposition with a ventricular septal defect (30 patients) or double outlet right ventricle (5 patients) and normal arch anatomy and were repaired within the first 90 days of life depending on the severity of heart failure at a median of 12 days of age (range 3–83 days). Seven patients (6%) had associated aortic coarctation (5 patients) or interrupted aortic arch (2 patients). One patient died during hospitalization (0.85% hospital mortality) and one patient died from noncardiac causes during a median follow-up of 35 months (1.7% total mortality). Four patients required intervention during follow-up (3.4%) for new aortic coarctation (2 patients), supravalvar pulmonic stenosis (1 patient), or right hemi-diaphragm paralysis (1 patient). Conclusions Individualized timing for the arterial switch operation within the first ninety days of life produces excellent survival rates for all types of transposition physiology with the expectation of a satisfactory course during follow-up.

Autologous repair of supravalvar pulmonic stenosis

Native supravalvar pulmonary stenosis is a rare anomaly, but iatrogenic supravalvar pulmonary stenosis occurs after various repairs for congenital heart disease with relative frequency. Surgical techniques such as patching carry the risk of restenosis. We describe a technique of repair using only autologous tissues that can be applied to both native and iatrogenic supravalvar pulmonary stenosis. There were no complications and no patient developed restenosis at follow-up. Autologous repair of supravalvar pulmonary stenosis is an effective technique.

Experience with an alternative technique for the management of anomalous left coronary artery from the pulmonary artery

Background Several operative approaches are utilized for the management of anomalous origin of the left coronary artery from the pulmonary artery, each with some limitation. The long-term results of a technique that facilitates direct and tension-free implantation of the anomalous artery to the aorta in all patients are described. Methods From January 1, 1992 through August 30, 2000, 10 consecutive patients with anomalous left coronary artery underwent operation using this technique. It consists of isolating an anterior and posterior transverse segment of pulmonary artery in continuity with the origin of the anomalous coronary artery. The two segments are folded with the orifice of the coronary as its fulcrum, and the edges sutured together to form an extension tube of pulmonary artery tissue. This lengthens the coronary artery and allows direct aortic implantation (posterior to the pulmonary artery) without tension. The pulmonary artery is reconstructed with autologous pericardium, Results Patient age ranged from 3 weeks to 3 years old (median 8 weeks), with 80% of patients less than 11 weeks old. Median weight was 4.6 kg (3.7 to 23 kg). The left ventricle was dilated with an end-diastolic diameter z-value of +1 to +3, and the shortening fraction was markedly reduced to 16% ± 6% (7% to 28%), with 8 of 10 patients having a shortening fraction less than 20%. Mitral regurgitation was severe in 5 patients, moderate in 2 patients, and all patients were in congestive heart failure. After repair there were no hospital deaths. Inotropic support was needed in all patients, but none required mechanical assistance. At a follow-up of 4.3 ± 2.5 years (0.5 to 8.5 years), 9 patients are asymptomatic and 1 patient has intermittent chest pain. All patients (10/10) have echocardiographic documented patency of the reimplanted coronary artery, as well as marked improvement in the left ventricular shortening fraction (37% ± 5%; p > 0.05 versus preoperative) and decrease in the end-diastolic diameter z-value (−1 to +1; p > 0.05 versus preoperative). Mitral regurgitation was absent in 4 patients, mild in 4 patients, and moderate in 2 patients. severe in 1 patient. Four patients have evidence of mild supravalvar pulmonary stenosis (15 to 32 mm Hg), 1992 Conclusions This technique allows a tension-free direct aortic connection in all cases, has a low rate of coronary artery occlusion, and avoids significant pulmonary artery distortion or stenosis, making it an excellent alternative for the surgical management of anomalous origin of the coronary artery.

Invited commentary

Michielon and associates from Ospedale Pediatrico Bambino Gesu, Rome, Italy, have presented their experience with surgical repair of anomalous coronary artery from the pulmonary artery using three different techniques from 1987–2002. Five had subclavian-to-coronary artery grafts, 12 had an intrapulmonary artery tunnel, and 14 had direct reimplantation. In general, this is a nicely researched and written manuscript that is largely confirmatory of a number of extant papers dealing with this subject [1Dodge-Khatami A. Mavroudis C. Backer C.L. Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy.Ann Thorac Surg. 2002; 74: 946-955Google Scholar]. The conclusions are simple and in accordance with numerous previous reports. However, the authors include two interesting components that are not commonly found in the other literature reports. The first is that the authors present a comparison of three different techniques from the same institution that is very useful. The second is that the authors have analyzed their data to determine the left ventricular recovery time as it relates to age at operation. This is important information. The comparison of these three techniques brings to light the superiority of the aortic implantation method, which as the authors conclude, can be used in the great majority of cases. Coronary mobilization techniques, stemming from the arterial switch operation experience, have allowed for this significant trend. Through their long-term followup the authors also document the problems that are associated with the Takeuchi intrapulmonary tunnel technique; namely that tunnel stenosis and supravalvar pulmonary artery stenosis occur in a high percentage of patients. Even so, the authors “still consider the modified Takeuchi procedure a valid alternative in case of leftward location of the coronary ostium, especially when extensive collaterals surround the pulmonary sinuses.” The problem of extensive collaterals on the surface of the pulmonary artery in this disease has not been discussed to any great degree in literature reports. In our practice we take great care to ligate and divide these vessels and to assure hemostasis before the coronary button dissection. Failure to do this in one of our patients required repeat cardiopulmonary bypass to control the bleeding. We do not believe that extensive surface pulmonary artery collaterals should mitigate against the coronary reimplantation technique as long as careful hemostasis is achieved. This leads to a further conclusion that the Takeuchi operation should only be used rarely and only in the most unusual cases. We have used the Takeuchi operation only once, early in our series [2Backer C.L. Stout M.J. Zales V.R. Muster A.J. Weigel T.J. Idriss F.S. Mavroudis C. Anomalous origin of the left coronary artery a twenty-year review of surgical management.J Thorac Cardiovasc Surg. 1992; 103: 1049-1058Google Scholar] because of intellectual curiosity and not because of inability to transfer the artery to the aorta. This patient has supravalvar pulmonary stenosis, which we are following. Other than this patient, we have been able to transfer the anomalous coronary artery in all of our patients in the modern era [3Backer C.L. Hillman N.D. Dodge-Khatami A. Mavroudis C. Anomalous origin of the left main coronary from the pulmonary artery successful surgical strategy without assist devices.Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2000; 3: 165-172Abstract Full Text PDF Google Scholar]. The authors have analyzed their data in a comprehensive manner to compare the three techniques. It comes as no surprise that (1) an earlier operation (less than 6 months) is better for long-term recovery, (2) that mitral insufficiency regresses in most cases, and (3) that coronary artery transfer yields superior long-term survival and freedom from reoperation. Nevertheless, their analysis, although confirmatory, is well executed and is welcomed as a very important contribution.

Comprehensive management of branch pulmonary artery stenosis.

Branch pulmonary artery stenosis may occur as an isolated finding or in conjunction with other cardiac malformations; it may be congenital or acquired postoperatively. Transcatheter management with balloon pulmonary angioplasty (BPA) and/or endovascular stenting is generally considered the procedure of choice for most patients. With the introduction of high-pressure balloons, the results of BPA have improved significantly (75%). Stent implantation has increased the success rate to over 90%. However, BPA continues to be the initial procedure of choice for distal stenoses, or those associated with branching points, and in small children or infants. Stent implantation is preferred over BPA for central or proximal stenoses, and those due to kinking or tenting, external compression, intimal flaps, failed balloon dilation, and early postoperative procedures. Certain lesions, such as supravalvar pulmonary stenosis, or stenosis at the branch pulmonary arteries bifurcation, are better dealt with at surgery. High-risk situations for the development of postoperative pulmonary artery (PA) stenosis exist in small infants with hypoplastic branch PAs after a conotruncal procedure, after duct ligation, after shunt insertion, after PA band placement, or after pulmonary arteriotomies or anastomoses of any kind. A combined collaborative transcatheter and surgical approach is essential for many patients with PA stenosis, particularly in complex forms of tetralogy of Fallot.

Results of three to 10 year follow up of balloon dilatation of the pulmonary valve

BackgroundThe results of immediate and short term follow up of balloon dilatation of the pulmonary valve have been well documented, but there is limited information on long term follow up.ObjectiveTo...

Direct Reconstruction of the Pulmonary Artery During the Arterial Switch Operation: An Interesting Surgical Option With Excellent Hemodynamic Results

Background. In transposition of the great arteries, reconstruction of the neo-pulmonary artery is a challenging surgical detail during the arterial switch procedure. We present early and midterm clinical and hemodynamic results of a direct reconstruction of the pulmonary artery avoiding prosthetic and autologous material. Methods. Between 1990 and June 1996, a total of 189 patients underwent the arterial switch procedure because of d-transposition of the great vessels. Of them, 47 underwent direct pulmonary artery reconstruction. Mean age at operation was 5.2 ± 4.1 days and mean weight was 3.75 ± 0.85 kg. Simple transposition of the great arteries was present in 13, transposition of the great arteries plus ventricular septal defect in 27, and more complex forms of transposition of the great arteries in 7 patients. The great vessels were side-by-side in 4 patients and in the anteroposterior position in 43 patients. The technique of direct pulmonary reconstruction includes extensive mobilization of both pulmonary artery branches into the hilum, posterior incision of the mean pulmonary artery into the bifurcation, and resuspension of the posterior commissure of the neo-pulmonary valve. A large anastomosis without any tension is then performed, using the anterior remnant aortic sinus of Valsalva to fit out the expected size of the neo-pulmonary artery. Results. Early mortality was 8.5% (4/47) in this particular group of patients. Postoperative echocardiography was performed before hospital discharge, 3 to 6 months postoperatively, and after a mean follow-up of 24 months. Of the 43 survivors, 37 patients had a pressure gradient across the pulmonary valve of less than 15 mm Hg. Mild pulmonary stenosis (pressure gradient of 15 to 30 mm Hg) was present in 4 and more severe supravalvar stenosis (pressure gradient > 30 mm Hg) in 2 patients. After a mean follow-up of 36 months, there was one redo operation to enlarge the right ventricular outflow tract. Conclusions. Direct reconstruction of the neo-pulmonary artery—avoiding autologous pericardium and prosthetic material—may represent an interesting option during the arterial switch operation when the great vessels lie in the anteroposterior position. This technique is simple, and the hemodynamic midterm results are very favorable. The incidence of postoperative supravalvar pulmonary stenosis is low, and there may be considerable potential for unlimited tissue growth.

Anomalous Origin of Left Coronary Artery From Pulmonary Artery: Recovery of Left Ventricular Function After Dual Coronary Repair

Objectives. We reviewed our institutional experience with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) after dual coronary repair to assess preoperative variables predictive of outcome, the time course for postoperative recovery of cardiac function, the short- and long-term complications and our experience with left ventricular assist devices (LVAD) in these patients.Background. Outcome after surgical repair of ALCAPA remains incompletely defined.Methods. The surgical records and echocardiograms of 42 patients were reviewed. Left ventricular function was assessed by fractional shortening z-score (FSz) and stress-velocity index.Results. The overall survival rate was 86%. All six patients who died were <1 year old and died within 3 days of the operation. More severe preoperative mitral regurgitation (MR) was associated with increased mortality, but age, body surface area, preoperative FSz and end-diastolic dimension were not. We used an LVAD for 7 of 28 patients who underwent repair for ALCAPA since its introduction at our institution, with a survival of 5 of 7 patients. The degree of MR improved in 62% of patients and remained unchanged in 38%. Complications included supravalvar pulmonary stenosis (16 of 21 patients) and baffle leaks (11 of 21 patients) with the intrapulmonary baffling technique. Supravalvar pulmonary stenosis developed in 1 of 11 patients after direct coronary reimplantation. Left ventricular function became normalized in all 28 patients with follow-up past 1 year, regardless of preoperative FSz. Of 13 patients who underwent serial postoperative echocardiography, the average time to normalization of function was 2 to 7 months.Conclusions. The degree of preoperative MR was predictive of outcome, whereas the severity of preoperative cardiac dysfunction and ventricular dilation were not. Mild and moderate MR tended to improve without mitral valvuloplasty. Complete recovery from myocardial dysfunction is expected after dual coronary repair of ALCAPA.

Fetoscopic and open transumbilical fetal cardiac catheterization in sheep. Potential approaches for human fetal cardiac intervention.

Shortening the prenatal disease course of severe aortic and pulmonary stenoses by balloon valvuloplasty may diminish their postnatal expression. The purpose of this study in fetal sheep was to assess the feasibility of fetoscopic and open transumbilical fetal cardiac catheterization guided by fetal transesophageal echocardiography to provide alternative approaches for human fetal cardiac intervention. We studied a total of nine fetal sheep (95 to 103 days of gestation; term = 145 to 150 days) and performed transumbilical fetal cardiac catheterization by a minimally invasive fetoscopic (n = 6) or an open fetal surgical approach (n = 3). Monitored by fetal transesophageal echocardiography, with an 8F or 10F, 10-MHz intravascular ultrasound catheter we placed guidewires and interventional catheters via the umbilical arterial route into the fetal heart. In three of the fetuses, we created supravalvar pulmonary artery stenosis by open fetal cardiac surgery After fetal and maternal recovery, we exteriorized these fetuses and performed open transumbilical fetal cardiac catheterization with successful pulmonary arterial angioplasty in two. Three fetuses survived fetoscopic transumbilical catheterization for 1 or 2 days and died most likely of blood loss after sheath dislodgment (n = 1) or removal (n = 2). By securing the sheath insertion site with a suture, we prevented sheath dislodgment and minimized bleeding during sheath removal in three fetuses. These fetuses then survived fetoscopic transumbilical fetal cardiac catheterization for 1 to 2 weeks before being killed. This study in fetal sheep demonstrates that fetoscopic and open transumbilical fetal cardiac catheterization are feasible and, guided by fetal transesophageal echocardiography, provide potential alternative approaches for human fetal cardiac intervention.

Angioplastia de lesiones residuales tras cirugía correctora de transposición de grandes vasos

Surgical correction in infants born with transposition of the great arteries, venous rerouting procedure (Mustard or Senning operation) or an arterial switch operation, can be complicated by vascular narrowings. Balloon dilatation angioplasty may be the treatment for these stenotic lesions and is attempted to avoid a reoperation of these patients. Angioplasty was attempted in nine patients after the surgical correction of transposition of the great arteries. Senning procedure was performed in sixty-one patients; two patients (3.2%) were successfully balloon-dilated for systemic venous obstruction: superior vena caval obstruction (case 1) and inferior vena caval obstruction (case 2). Arterial switch operation was performed in forty patients, balloon angioplasty was attempted in seven patients (17.5%) who developed supravalvar pulmonary artery stenosis. Mean age at dilation was 32.5 +/- 20.7 months (range 11 to 67) in the switch arterial group. Age at dilation in the Senning group was 8 and 106 months. The balloon/stenosis diameter was 2 to 3 in the switch arterial operation and 3 to 8 in the Senning procedure. Balloon dilatation angioplasty was successful in three patients with supravalvar pulmonary stenosis postarterial switch operation (mean age was 16.6 months) and reduced the peak-to-peak gradient from 78.6 +/- 36.6 mmHg to 44 +/- 15 mmHg. There was no success after angioplasty in three patients and it was not possible to cross the stenosis with the guidewire in one (mean age was 46 months). Balloon dilatation angioplasty was successful in the obstruction following Senning procedure. These data indicate that although the success rate of balloon angioplasty for supravalvar pulmonary stenosis after the arterial switch operation is low, balloon angioplasty can be the first therapeutic choice owing to its low complication rate and the potential benefit of the procedure. The success rate should be higher if angioplasty is performed at a younger age after operating and a balloon of adequate size is used. Postoperative systemic venous obstructions can be successfully managed with balloon dilatation.

Supravalvar pulmonary stenosis following the arterial switch operation for complete transposition: aetiological and surgical considerations

AbstractFrom January 1988 through to July 1994, 54 consecutive infants underwent an arterial switch operation for simple or more complex forms of complete transposition (concordant atrioventricular and discordant ventriculo-arterial connections). They ranged in age from 2 to 180 days. The Lecompte maneuver was performed in all. In the first 19 patients the harvested sinuses of Valsalva were filled with two separate patches of autologous preserved pericardium, while, in the last 35 patients, a wide pantaloon patch of tanned pericardium was employed. Five babies died within 30 days after the procedure (operative mortality of 9.2%, 70% CL 4–17%). Four survivors were found to have developed a significant supravalvar pulmonary stenosis from 1 month to 21 months postoperatively. Echocardiographic data showed a transpulmonary peak systolic gradient from 60 mmHg to 101 mmHg, with a right-to-left ventricular systolic pressure ratio from 0.65 to 0.9. Reoperation was performed from 8 months to 39 months after the arterial switch procedure. Supravalvar pulmonary stenosis was located at the level of the pulmonary trunk, extending distally in two cases, due to the growth of fibrous scarring tissue with partial calcification. The pulmonary valvar leaflets were involved in two cases. Relief of the obstruction was obtained by insertion of a wide shield-tailored polytetrafluoroethylene patch after making an inverted Y-shaped longitudinal incision in the pulmonary trunk between the anterior sinuses of Valsalva. No mortality occurred at reoperation. Early and midterm echocardiographic measurements showed the effectiveness of this technique, with only trivial or mild residual transpulmonary gradients.