Predictors for Biventricular Repair in Pulmonary Atresia with Intact Ventricular Septum

Abstract

Background: Pulmonary atresia with intact ventricular septum (PA‐IVS) is a complex congenital heart defect with a large variety of right heart-sided morphologies. Methods: We undertook a retrospective review of 86 patients with PA‐IVS with a special emphasis on the angiographic findings. The aim of the study was to determine predictors for biventricular repair. Initial surgical procedures depended on the right ventricular morphology, the tricuspid valve size and coronary anomalies. Results: Fifty-five patients (64 %) underwent decompression of the right ventricle (RV) as an initial procedure; 16 of them required an additional systemic-to-pulmonary artery shunt. Twenty-six patients (30 %) had only a systemic-to-pulmonary artery shunt as their initial procedure. Five patients underwent interventional procedures performed by pediatric cardiologists. Biventricular repair was possible in 56 patients (65 %). Univentricular palliation was achieved in 16 patients. Fourteen patients had only palliation with a systemic-to-pulmonary artery shunt. Mean tricuspid valve size was significantly bigger in patients with biventricular repair (z-score −3.6 ± 2.6) than in patients who did not undergo biventricular repair (−5.2 ± 1.7, p = 0.003). Predictors for biventricular repair were right ventricular decompression with or without systemic-to-pulmonary artery shunt (p < 0.001), tripartite right ventricle (p < 0.001) and the absence of coronary fistulae (p < 0.001). Long-term survival was 80 % ± 13 % at 25 years for patients undergoing biventricular repair. Conclusions: Decompression of the RV as an initial surgical procedure improves the possibility of achieving biventricular repair with good long-term results. However, morphological factors such as right ventricular size and the absence of coronary fistulae are significant predictors for biventricular repair.

Key words

congenital heart disease - cyanotic - neonate - univentricular heart - tricuspid valve

References

• 1 Daubeney P E, Delany D J, Anderson R H, Sandor G G, Slavik Z, Keeton B R, Webber S A. Pulmonary atresia with intact ventricular septum. Range of morphology in a population-based study.  J Am Coll Cardiol. 2002;  39 1670-1679
• 2 Ashburn D A, Blackstone E H, Wells W J, Jonas R A, Pigula F A, Manning P B, Lofland G K, Williams W G, McCrindle B W. Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum.  J Thorac Cardiovasc Surg. 2004;  127 1000-1007
• 3 Jahangiri M, Zurakowski D, Bichell D, Mayer J E, del Nido P J, Jonas R A. Improved results with selective management in pulmonary atresia with intact ventricular septum.  J Thorac Cardiovasc Surg. 1999;  118 1046-1055
• 4 Yoshimura N, Yamaguchi M, Ohashi H, Oshima Y, Oka S, Yoshida M, Murakami H, Tei T. Pulmonary atresia with intact ventricular septum: strategy based on right ventricular morphology.  J Thorac Cardiovasc Surg. 2003;  126 1417-1426
• 5 Mair D D, Julsrud P R, Puga F J, Danielson G K. The Fontan procedure for pulmonary atresia with intact ventricular septum: operative and late results.  J Am coll Cardiol. 1997;  29 1359-1364
• 6 Najm H K, Williams W G, Coles J G, Rebeyka I M, Freedom R M. Pulmonary atresia with intact ventricular septum: results of the Fontan procedure.  Ann Thorac Surg. 1997;  63 669-675
• 7 Daubeney P E, Blackstone E H, Weintraub R G, Slavik Z, Scanlon J, Webber S A. Relationship of the dimension of cardiac structures to body size: an echocardiographic study in normal infants and children.  Cardiol Young. 1999;  9 402-410
• 8 Bull C, De Leval M R, Mercanti C, Macartney F J, Anderson R H. Pulmonary atresia and intact ventricular septum: a revised classification.  Circulation. 1982;  66 266-272
• 9 Concato J, Peduzzi P, Holford T R, Feinstein A R. Importance of events per independent variable in proportional hazards analysis. Background, goals and general strategy.  J Clin Epidemiol. 1995;  48 1495-1501
• 10 Dyamenahalli U, McCrindle B W, McDonald C, Trivedi K R, Smallhorn J F, Benson L N, Coles J, Williams W G, Freedom R M. Pulmonary atresia with intact ventricular septum: management of, and outcomes for, a cohort of 210 consecutive patients.  Cardiol Young. 2004;  14 299-308
• 11 Rychik J, Levy H, Gaynor J W, DeCampli W M, Spray T L. Outcome after operations for pulmonary atresia with intact ventricular septum.  J Thorac Cardiovasc Surg. 1998;  116 924-931
• 12 Hanley F L, Sade R M, Blackstone E H, Kirklin J W, Freedom R M, Nanda N C. Outcomes in neonatal pulmonary atresia with intact ventricular septum. A multiinstitutional study.  J Thorac Cardiovasc Surg. 1993;  105 406-423
• 13 de Leval M, Bull C, Hopkins R, Rees P, Deanfield J, Taylor J F N, Gersony W, Stark J, Macartney F J. Decision making in the definitive repair of the heart with a small right ventricle.  Circulation. 1985;  72 (Suppl.) II-52-II-60
• 14 Mainwaring R D, Lamberti J J. Pulmonary atresia with intact ventricular septum. Surgical approach based on ventricular size and coronary anatomy.  J Thorac Cardiovasc Surg. 1993;  106 733-738
• 15 Ovaert C, Qureshi S A, Rosenthal E, Baker E J, Tynan M. Growth of the right ventricle after successful transcatheter pulmonary valvotomy in neonates and infants with pulmonary atresia with intact ventricular septum.  J Thorac Cardiovasc Surg. 1998;  115 1055-1062
• 16 Giglia T M, Mandell V S, Connor A R, Mayer J E, Lock J E. Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum.  Circulation. 1992;  86 1516-1528