Surgical Results in Patients With Pulmonary Atresia-Major Aortopulmonary Collaterals in Association With Total Anomalous Pulmonary Venous Connection

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1 Surgical Results in Patients With Pulmonary Atresia-Major Aortopulmonary Collaterals in Association With Total Anomalous Pulmonary Venous Connection Richard D. Mainwaring, MD, V. Mohan Reddy, MD, Olaf Reinhartz, MD, Rajesh Punn, MD, Theresa Tacy, MD, and Frank L. Hanley, MD Divisions of Pediatric Cardiac Surgery and Pediatric Cardiology, Lucile Packard Children s Hospital/Stanford University, Stanford, California Background. Pulmonary atresia and major aortopulmonary collaterals (PA/MAPCAs) is a complex form of congenital heart disease. One to two percent of patients with PA/MAPCAs will also have total anomalous pulmonary venous connection (TAPVC). This study summarizes our surgical experience with this rare combination of life-threatening congenital heart defects. Methods. A retrospective review was performed to identify patients who had surgery for PA/MAPCAs in association with TAPVC. From November 2001 to March 2011, 9 patients presented with this combination of defects. Eight of the 9 patients had heterotaxy with an unbalanced atrioventricular canal and functional single ventricle. The ninth patient had double outlet right ventricle (two ventricles). Timing of surgical intervention was typically predicated on the degree of pulmonary venous obstruction. The median age at surgery was 14 days. All nine patients had surgical correction of TAPVC, unifocalization of MAPCA s into a central confluence, and placement of a shunt. Results. There was one early mortality (< 30 days) and two late mortalities. For the 6 survivors, 5 have subsequently undergone a bidirectional Glenn procedure, and 3 had completion of their Fontan. Two patients are currently at the bidirectional Glenn stage; one is a good candidate for Fontan completion while the other is not suitable. The sixth patient is awaiting further assessment. Conclusions. The PA/MAPCAs, in association with TAPVC, is a challenging combination of defects. The data suggest that the combination of PA/MAPCAs and TAPVC can be undertaken with a reasonable midterm prognosis. (Ann Thorac Surg 2011;92: ) 2011 by The Society of Thoracic Surgeons Pulmonary atresia with major aortopulmonary collateral arteries (PA/MAPCAs) is a relatively rare congenital heart defect. Untreated, the prognosis for patients with PA/MAPCAs is rather bleak, with half of the patients dying within 1 year of birth [1, 2]. The relative efficacy of surgical treatment was a point of debate for several decades [3]. However, the introduction of the unifocalization procedure through a midline approach has resulted in a vast improvement in outcomes for this patient population [4]. The subsequent standardization of protocols at many congenital heart centers throughout the world has provided evidence that the outlook for patients with PA/MAPCAs has been substantively enhanced [5 7]. The PA/MAPCAs, in association with total anomalous pulmonary venous connection (TAPVC), is an extremely rare subset of patients, accounting for only 1% to 2% of all patients with PA/MAPCAs. This combination of two Accepted for publication June 1, Address correspondence to Dr Mainwaring, Stanford University School of Medicine, 300 Pasteur Dr, Falk CVRC, Stanford, CA 94305; mainwaring@stanford.edu. separate life-threatening congenital heart defects further complicates the management of these patients, as the timing of surgery is frequently dictated by the presence or development of pulmonary venous obstruction as opposed to the standardized protocols that apply to the majority of patients. Thus, the entity of PA/MAPCAs in association with TAPVC is one of the most challenging in all of congenital heart surgery. The purpose of this study was to review our institutional experience with PA/MAPCAs in association with TAPVC to evaluate the features of this patient population and to determine their outcomes. It is our goal that this analysis will identify some important principles in the management of these complex patients. Material and Methods This study was approved by the Institutional Review Board at Stanford University. Medical records were reviewed and a written questionnaire was sent to the families of those children surviving the repair of PA/ MAPCAs and TAPVC. Current follow-up was obtained 2011 by The Society of Thoracic Surgeons /$36.00 Published by Elsevier Inc doi: /j.athoracsur

2 Ann Thorac Surg MAINWARING ET AL 2011;92: PULMONARY ATRESIA patients had bilateral superior venae cavae. While these findings did not influence the initial procedure, they did play a role in subsequent operations. Fig 1. Echocardiogram from a four-chamber view demonstrating the presence of a complete atrioventricular septal defect (AVSD) with hypoplasia of the right ventricle (RV). (AVV atrioventricular valve; LA left atrium; LV left ventricle; VSD ventricular septal defect.) in all of the surviving patients through these mechanisms. The study summarizes our surgical experience with 9 patients from November 2001 through November There were 5 males and 4 females. The median age at first surgery was 14 days, with a range of 2 hours to 8 years. The median weight of the patients at surgery was 3.4 kg, with a range of 1.1 to 28.5 kg. Eight patients had heterotaxy with right atrial isomerism and asplenia, an unbalanced atrioventricular (AV) canal, and functional single ventricle (see Fig 1). The ninth patient had double outlet right ventricle with two ventricles. Four patients had supracardiac TAPVC, 3 patients had infradiaphragmatic TAPVC, and 2 patients had mixed TAPVC. Two patients had obstructed infradiaphragmatic TAPVC at birth and required emergency surgery within hours of delivery. Six patients (4 with supracardiac, 1 with infradiaphragmatic, and 1 with mixed TAPVC) initially did not have evidence of pulmonary venous obstruction at birth, but subsequently developed clinical signs of obstruction between 2 weeks and 2.5 months of age. There was a single patient with mixed TAPVC who never did develop pulmonary venous obstruction and did not undergo any surgery until 8 years of age. In addition to the anatomic diagnosis of PA/MAPCAs and TAPVC, 2 patients had cor triatriatum (see Fig 2) and Results The nine patients in this study had a median of three major aortopulmonary collateral arteries (range of 1 to 5). A complete bilateral unifocalization procedure was performed in all patients through a midline approach utilizing cardiopulmonary bypass. This unifocalization procedure entailed identification of the major aortopulmonary collaterals within the mediastinum, ligation of the collaterals at their origin, and then reconstruction of the collaterals with homograft augmentation to construct a central confluence [4]. Four of the 9 patients had evidence of a true pulmonary artery system. In these circumstances, the native pulmonary arteries were incorporated into the central confluence, particularly when they supplied segments of the lung that were not otherwise supplied by the MAPCAs. All 9 patients underwent placement of a shunt as a source for their pulmonary blood flow. Eight of these shunts were central shunts from the aorta to central confluence (ranging in size from 3.5 in the smallest patient to 8 mm in the largest) and 1 patient (weighing 1.1 Kg) had a 5-mm right ventricle to central confluence conduit constructed. The median cross-clamp time for repair of the anomalous pulmonary veins was 38 minutes (range, 22 to 60 minutes), and the median cardiopulmonary bypass time was 328 minutes (range, 208 to 451 minutes). There was one early mortality ( 30 days) and two late mortalities in this cohort of patients with PA/MAPCAs in association with TAPVC. The early death was caused by a sudden cardiac arrest in the cardiac intensive care unit. One late death was related to recurrent pulmonary vein stenosis. Surgical re-repair of the pulmonary veins was performed but was not successful. The other late death occurred in the patient with double outlet right ventricle and two ventricles who demonstrated persistent pulmonary hypertension despite evidence of a satisfactory anatomic repair. This patient eventually died from a combination of hypoxemia and respiratory failure. Two patients had evidence of cor triatriatum by echocardiography. Cardiac catheterization subsequently documented that this anatomic feature had adverse physiologic consequences. The cor triatriatum anatomy was Fig 2. Echocardiogram of the left atrial (LA) anatomy in the same patient as shown in Figure 1. (A) The cor triatriatum membrane is demonstrated and labeled. (B) The color flow Doppler signal demonstrates acceleration across the cor triatriatum membrane.

3 1758 MAINWARING ET AL Ann Thorac Surg PULMONARY ATRESIA 2011;92: Table 1. Summary of the Bidirectional Glenn and the Fontan Procedure Data for the Six Eligible Candidates After Initial Repair of Pulmonary Atresia-Major Aortopulmonary Collateral Arteries and Total Anomalous Pulmonary Venous Connection Patient No. BDG Bilat Off-Pump Fontan Off-Pump 1 Yes Yes Yes Yes Yes 2 Yes Yes No Yes Yes 3 Yes No Yes Yes Yes 4 Yes Yes No No 5 Yes Yes Yes No 6 No BDG bidirectional Glenn procedure; Bilat bilateral. repaired at the second stage in one patient and at the third stage in the other. Four of the 5 patients who have undergone a bidirectional Glenn procedure had bilateral superior venae cavae, in which case a bilateral bidirectional Glenn was performed. The details relating patient anatomy and operations performed are shown in Table 1. The 6 survivors have been followed for a median duration of 4.5 years (range, 17 months to 9 years) after their primary procedures. The current stage of management for these patients is illustrated in Figure 3. Five of the 6 patients have undergone a bidirectional Glenn procedure, and of these, 3 have subsequently had a successful completion of their Fontan procedure (see Fig 4). The three Fontan procedures were performed offpump using a nonfenestrated extracardiac conduit. Two of the patients are currently at the bidirectional Glenn stage, with one considered a good candidate for a Fontan and the other deemed not suitable. The sixth patient is Fig 4. Pulmonary angiogram of the same patient as shown in Figures 1 and 2; this study was performed in preparation for the bidirectional Glenn procedure. The patient has undergone complete unifocalization, repair of total anomalous pulmonary venous connection, and repair of cor triatriatum. The angiogram demonstrates a reasonably normal pulmonary arterial arborization, and the hemodynamic measurements resulted in a calculated pulmonary vascular resistance of 1.2 Woods units. (LPA left pulmonary artery; MPA main pulmonary artery; RPA right pulmonary artery.) awaiting further assessment after pulmonary vein revision. The families of the 6 surviving patients received and answered written questionnaires regarding the health status of their children. Five of the 6 children were perceived to be doing well by their families, with minimal or no limitations. The one family that did perceive a more significant problem was that of the child who has undergone a bidirectional Glenn procedure, but is not a viable candidate for a Fontan. Fig 3. Patient flow diagram for all 9 patients in this series and the current stage of management demonstrated for the 6 surviving patients. (BDG bidirectional Glenn procedure.) Comment This retrospective review was performed to evaluate the efficacy of surgical treatment for patients with PA/ MAPCAs in association with TAPVC. We were able to identify 9 patients who had this rare combination of congenital heart defects. Eight of the 9 patients had heterotaxy (right atrial isomerism) with an unbalanced atrioventricular canal and functional single ventricle. There were three deaths in this cohort of patients, all of which occurred within the first 6 months after presentation. Among the survivors, the majority of patients have successfully progressed along a single-ventricle management pathway. These results suggest that the combination of PA/MAPCAs and TAPVC can be undertaken surgically with a reasonable midterm prognosis. An estimated 4% of patients with MAPCAs have a

4 Ann Thorac Surg MAINWARING ET AL 2011;92: PULMONARY ATRESIA 1759 Table 2. Literature Summary of the Types of Total Anomalous Pulmonary Venous Connection Author Supracardiac Intracardiac Infradiaphragmatic Mixed Mainwaring et al [current study] Hsu et al [11] Miyaji et al [12] 1 Found in Association With Pulmonary Atresia and Major Aortopulmonary Collateral Arteries. functional single ventricle. It was initially uncertain whether patients with major MAPCAs could be suitable candidates for a bidirectional Glenn or Fontan procedure. By definition, all patients with MAPCAs begin with markedly abnormal pulmonary arterial arborization, and some will never achieve the physiologic mandate of a low pulmonary vascular resistance. Reinhartz and colleagues [8] summarized an experience with 14 patients who underwent unifocalization for major aortopulmonary collaterals in single-ventricle patients. Half (7 of 14) of the patients progressed to a bidirectional Glenn procedure and 3 out of 14 underwent successful completion of their Fontan. The authors concluded that in selected patients the pulmonary vascular bed can be reconstructed sufficiently to allow for a cavopulmonary connection and that the life expectancy and quality of life for these patients would be enhanced due to the decrease in cardiac volume load. Several other case reports in the literature support the notion that some patients with major aortopulmonary collaterals and single ventricle can achieve separation of their circulations through application of the bidirectional Glenn and Fontan procedures [9, 10]. The association between heterotaxy and MAPCAs has been previously described in one small series, and one case report, that we were able to locate in the literature. Hsu and colleagues [11] reported that 9 out of 138 patients with right isomerism had MAPCAs, for an overall incidence of 6.5%. All 9 of their patients had an unbalanced atrioventricular canal with functionally single ventricle in association with TAPVC (4 supracardiac, 4 intracardiac, 1 mixed). However, none of their patients were able to progress through to a successful Fontan. Miyaji and colleagues [12] did report a successful Fontan procedure for asplenia with PA/MAPCAs in association with intracardiac TAPVC. These cases are similar in every respect to the majority of cases in our study, with the caveat that we have not observed the intracardiac form of TAPVC (see Table 2). Of the 10 patients described in previous studies, only one patient has been able to achieve Fontan completion. Thus, the current series, with its contribution of three new patients, provides a substantive increase in the number of patients who have been able to achieve separation of their systemic and pulmonary circulations through Fontan completion for this specific entity. A review of the literature also produces a few case reports describing anatomic features closely related to those summarized in this study. Ban and colleagues [13] described a successful Kawashima procedure for asplenia syndrome and MAPCAs. This patient did not have TAPVC, and it is not known whether the patient will be a suitable candidate for Fontan completion in the future. Uemura and colleagues [14] reported a rare case of a patient with right atrial isomerism and MAPCAs who had a balanced atrioventricular canal and was able to undergo a two-ventricle (anatomic) repair. This patient had a normal pulmonary venous confluence draining to the left atrium. Cor triatriatum is another rare form of congenital heart defect [15]. Two of the 9 patients in this series were discovered to have cor triatriatum along with PA/ MAPCAs and TAPVC. Cor triatriatum is frequently associated with other congenital heart defects, including atrioventricular septal defect and either partial or complete anomalous pulmonary venous drainage [16 18]. This association would suggest that the genetic coding and subsequent embryologic development of PA/ MAPCAs and TAPVC must be linked in some fashion with the development of cor triatriatum. In conclusion, PA/VSD/MAPCAs in association with TAPVC is a rare combination of two life-threatening congenital heart defects. This combination of defects is typically found in conjunction with heterotaxy, right atrial isomerism, and functional single ventricle. The timing of surgery is almost always dictated by the presence at birth or subsequent development of pulmonary venous obstruction, and thus requires much earlier surgery than would otherwise be chosen on an elective basis. Despite these alterations in the standard PA/ MAPCAs management algorithm, it is evident that a significant percentage of patients with this rare combination of congenital heart defects can achieve satisfactory palliation. References 1. Bull K, Somerville J, Spiegelhalter D. Presentation and attrition in complex pulmonary atresia. JACC 1995;25: Leonard H, Derrick G, O Sullivan J, Wren C. Natural and unnatural history of pulmonary atresia. Heart 2000;84: D Uedekem Y, Alphonso N, Norgaard MA, et al. Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries: Unifocalization brings no longterm benefits. J Thorac Cardiovasc Surg 2005;130: Reddy VM, McElhinney DB, Amin Z, et al. Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Circulation 2000;101: Carotti A, Albanese SB, Filleppelli S, et al. Determinants of outcome after surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary colla-

5 1760 MAINWARING ET AL Ann Thorac Surg PULMONARY ATRESIA 2011;92: teral arteries. J Thorac Cardiovasc Surg 2010;140: Amark KM, Karamlou T, O Carroll A, et al. Independent factors associated with mortality, reintervention, and achievement of complete repair in children with pulmonary atresia with ventricular septal defect. JACC 2006;47: Davies B, Mussa S, Davies P, et al. Unifocalization of major aortopulmonary collateral arteries in pulmonary atresia with ventricular septal defect is essential to achieve excellent outcomes irrespective of native pulmonary artery morphology. J Thorac Cardiovasc Surg 2009;138: Reinhartz O, Reddy VM, Petrossian E, Suleman S, et al. Unifocalization of major aortopulmonary collaterals in single-ventricle patients. Ann Thorac Surg 2006;82: Shinkawa T, Yamagishi M, Shuntoh K, Yaku H. One-stage unifocalization followed by staged Fontan operation. Interact CardioVasc Thorac Surg 2007;6: Nagashima M, Hibino N, Yamamoto E, Higaki T. Total cavopulmonary connection for functionally single ventricle with pulmonary atresia and abnormal arborization of pulmonary arteries- exclusion of overwhelmed area by collateral arteries from Fontan circulation. Interact CardioVasc Thorac Surg 2008;7: Hsu J-Y, Wang J-K, Lin M-T, et al. Clinical implications of major aortopulmonary collateral arteries in patients with right isomerism. Ann Thorac Surg 2006;82: Miyaji K, Nagata N, Matsui H, Miyamoto T, Kitahori K. Successful Fontan procedure for asplenia with pulmonary atresia and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 2003;126: Ban Y, Noma M, Horigome H, et al. Kawashima procedure after staged unifocalizations in asplenia with major aortopulmonary collateral arteries. Ann Thorac Surg 2010;89: Uemura H, Yagihara T, Kawahira Y, Yoshikawa Y. Staged unifocalization and anatomic repair in a patient with right isomerism. Ann Thorac Surg 2001;71: Alphonso N, Norgaard MA, Newcomb A, d Udekem Y, Brizard CP, Cochrane A. Cor triatriatum: presentation, diagnosis, and long-term surgical results. Ann Thorac Surg 2005;80: Varma PK, Warrier G, Ramachandran P, et al. Partial atrioventricular canal defect with cor triatriatum sinister: report of three cases. J Thorac Cardiovasc Surg 2004;127: Kirk AJ, Pollock JC. Concomitant cor triatriatum and coronary sinus total anomalous pulmonary venous connection. Ann Thorac Surg 1987;44: Imachi T, Arimitsu K, Minami M, Hayakawa M, Kawaguchi A. Cor triatriatum dexter with anomalous pulmonary venous drainage and sinus venosus atrial septal defect. J Thorac Cardiovasc Surg 1988;95: INVITED COMMENTARY The association of pulmonary atresia major aortopulmonary collaterals with total anomalous pulmonary venous drainage combines two severe congenital malformations. This association is usually found in conjunction with heterotaxy syndrome and single ventricle physiology. Patients with this combination are rare and the information regarding natural history as well as surgical outcome is very scarce. The prognosis in the absence of surgical treatment is probably extremely poor. The only chance to provide a satisfactory late outcome is to achieve a Fontan circulation by a staged surgical approach. Two goals must therefore be reached: (1) to reconstruct a normal low resistance pulmonary arterial bed and (2) to provide an unobstructed pulmonary venous drainage. This clearly represents a major surgical challenge. The data reported by Mainwaring and associates [1] show that this goal can be achieved in a reasonable number of patients by an aggressive initial approach which includes repair of anomalous pulmonary venous drainage and complete unifocalization procedure through a midline incision. In a series of 9 patients, 3 had successful Fontan completion, 1 (with cavopulmonary shunt) was a suitable candidate for Fontan and 1 (after initial palliation) might become a good candidate. These encouraging results deserve some comments. The current study is coming from a surgical center with exceptional expertise in the management of pulmonary atresia major aortopulmonary collaterals and, particularly, with one-stage complete unifocalization procedure. Achieving such outstanding results, particularly when complete unifocalization must be performed very early in life because of associated obstructed total anomalous pulmonary venous drainage, is most probably very difficult to reproduce. Even if they are encouraging, the results are far from optimal. A significant number of patients (at least 4 of 9 in the present series) still have a poor outcome. Further information is needed to identify potential subsets with incremental risk factors. The current data should be kept in mind and taken into consideration at the time of prenatal counseling or postnatal information of the parents. Pascal R. Vouhe, MD Service de Chirurgie Cardiaque Hopital Necker - Enfants Malades 149, Rue de Sevres Paris Cedex, France pascal.vouhe@nck.ap-hop-paris.fr Reference 1. Mainwaring RD, Mohan Reddy V, Reinhartz O, Punn R, Tacy T, Hanley FL. Surgical results in patients with pulmonary atresia major aortopulmonary collaterals in association with total anomalous pulmonary venous connection. Ann Thorac Surg 2011;92: by The Society of Thoracic Surgeons /$36.00 Published by Elsevier Inc doi: /j.athoracsur