(Circulation. 2000;101:1826.)
© 2000 American Heart Association, Inc.
Clinical Investigation and Reports |
Early and Intermediate Outcomes After Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries
Experience With 85 Patients
Presented at the 70th Scientific Sessions of the American Heart Association, Orlando, Fla, November 9–12, 1997.
From the Divisions of Cardiothoracic Surgery (V.M.R., D.B.M., A.J.P., Z.A., F.L.H.) and Pediatric Cardiology (P.M., D.F.T.), University of California, San Francisco.
Correspondence to V. Mohan Reddy, MD, Division of Cardiothoracic Surgery, UCSF Medical Center, 505 Parnassus Ave, M593, San Francisco, CA 94143-0118.
Background—Pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCAs) is a complex lesion with marked heterogeneity of pulmonary blood supply. Traditional management has involved staged unifocalization of pulmonary blood supply. Our approach has been to perform early 1-stage complete unifocalization in almost all patients.
Methods and Results—Since 1992, 85 patients with pulmonary atresia, VSD, and MAPCAs have undergone unifocalization (median age, 7 months). Complete 1-stage unifocalization and intracardiac repair were performed through a midline approach in 56 patients, whereas 23 underwent unifocalization in a single stage with the VSD left open, and 6 underwent staged unifocalization through sequential thoracotomies. There were 9 early deaths. During follow-up (1 to 69 months), there were 7 late deaths. Actuarial survival was 80% at 3 years. Among early survivors, actuarial survival with complete repair was 88% at 2 years. Reintervention on the neo–pulmonary arteries was performed in 24 patients.
Conclusions—Early 1-stage complete unifocalization can be performed in >90% of patients with pulmonary atresia and MAPCAs, even those with absent true pulmonary arteries, and yields good functional results. Complete repair during the same operation is achieved in two thirds of patients. There remains room for improvement; actuarial survival 3 years after surgery is 80%, and there is a significant rate of reintervention. These results must be appreciated within the context of the natural history of this lesion: 65% of patients survive to 1 year of age and slightly >50% survive to 2 years even with surgical intervention.
Key Words: survival • surgery • heart defects, congenital
This article has been cited by other articles:
 |
|
 |
|
  J. LeBlanc and M. Heran Decision-making in unoperated adults with congenital heart disease: a difficult task Interactive CardioVascular and Thoracic Surgery, December 1, 2007; 6(6): 820 - 822. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 N. Ishibashi, T. Shin'oka, M. Ishiyama, T. Sakamoto, and H. Kurosawa Clinical results of staged repair with complete unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries Eur. J. Cardiothorac. Surg., August 1, 2007; 32(2): 202 - 208. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. M. Blue, J. M. Mah, A. D. Cole, V. Lal, M. J. Wilson, R. B. Chard, G. F. Sholler, R. E. Hawker, M. C. Sherwood, and D. S. Winlaw The negative impact of Alagille syndrome on survival of infants with pulmonary atresia J. Thorac. Cardiovasc. Surg., April 1, 2007; 133(4): 1094 - 1096. [Full Text] [PDF]
|
|
|
|
 |
|
 Y. Kaneko, H. Yoda, and K. Tsuchiya Airway Compression by Major Aortopulmonary Collaterals with 22q11 Deletion Asian Cardiovasc Thorac Ann, February 1, 2007; 15(1): e9 - e11. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S Vesel, S Rollings, A Jones, N Callaghan, J Simpson, and G K Sharland Prenatally diagnosed pulmonary atresia with ventricular septal defect: echocardiography, genetics, associated anomalies and outcome Heart, October 1, 2006; 92(10): 1501 - 1505. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 O. Reinhartz, V. M. Reddy, E. Petrossian, S. Suleman, R. D. Mainwaring, D. N. Rosenthal, J. A. Feinstein, R. Gulati, and F. L. Hanley Unifocalization of major aortopulmonary collaterals in single-ventricle patients. Ann. Thorac. Surg., September 1, 2006; 82(3): 934 - 938. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. M. Amark, T. Karamlou, A. O'Carroll, C. MacDonald, R. M. Freedom, S.-J. Yoo, W. G. Williams, G. S. Van Arsdell, C. A. Caldarone, and B. W. McCrindle Independent Factors Associated With Mortality, Reintervention, and Achievement of Complete Repair in Children With Pulmonary Atresia With Ventricular Septal Defect J. Am. Coll. Cardiol., April 4, 2006; 47(7): 1448 - 1456. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
Y. d'Udekem, N. Alphonso, M. A. Norgaard, A. D. Cochrane, L. E. Grigg, J. L. Wilkinson, and C. P. Brizard Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries: Unifocalization brings no long-term benefits J. Thorac. Cardiovasc. Surg., December 1, 2005; 130(6): 1496 - 1502. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
T. Shinkawa, M. Yamagishi, K. Shuntoh, A. Takahashi, K. Hayashida, and N. Kitamura One-Stage Unifocalization and Palliative Right Ventricular Outflow Tract Reconstruction Ann. Thorac. Surg., March 1, 2005; 79(3): 1044 - 1047. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. Agnoletti, Y. Boudjemline, D. Bonnet, D. Sidi, and P. Vouhe Surgical Reconstruction of Occluded Pulmonary Arteries in Patients With Congenital Heart Disease: Effects on Pulmonary Artery Growth Circulation, May 18, 2004; 109(19): 2314 - 2318. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. J. Roche, R. Rivera, M. Argilla, N. R. Fefferman, L. P. Pinkney, H. Rusinek, and N. B. Genieser Assessment of Vasculature Using Combined MRI and MR Angiography Am. J. Roentgenol., April 1, 2004; 182(4): 861 - 866. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. K. Prasad, N. Soukias, T. Hornung, M. Khan, D. J. Pennell, M. A. Gatzoulis, and R. H. Mohiaddin Role of Magnetic Resonance Angiography in the Diagnosis of Major Aortopulmonary Collateral Arteries and Partial Anomalous Pulmonary Venous Drainage Circulation, January 20, 2004; 109(2): 207 - 214. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. F. Abella, T. De la Torre, G. Mastropietro, N. Morici, A. Cipriani, and C. Marcelletti Primary repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: A useful approach J. Thorac. Cardiovasc. Surg., January 1, 2004; 127(1): 193 - 202. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Griselli, S. P. McGuirk, D. S. Winlaw, O. Stumper, J. V. de Giovanni, P. Miller, R. Dhillon, J. G. Wright, D. J. Barron, and W. J. Brawn The influence of pulmonary artery morphology on the results of operations for major aortopulmonary collateral arteries and complex congenital heart defects J. Thorac. Cardiovasc. Surg., January 1, 2004; 127(1): 251 - 258. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Gupta, J. Odim, D. Levi, R.-K. Chang, and H. Laks Staged repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: Experience with 104 patients J. Thorac. Cardiovasc. Surg., December 1, 2003; 126(6): 1746 - 1752. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. C. Marshall, B. A. Love, P. Lang, R. A. Jonas, P. J. del Nido, J. E. Mayer, and J. E. Lock Staged repair of tetralogy of Fallot and diminutive pulmonary arteries with a fenestrated ventricular septal defect patch J. Thorac. Cardiovasc. Surg., November 1, 2003; 126(5): 1427 - 1433. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B. W. Duncan, R. B. B. Mee, L. R. Prieto, G. L. Rosenthal, C. I. Mesia, A. Qureshi, O. P. Tucker, J. F. Rhodes, and L. A. Latson Staged repair of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries J. Thorac. Cardiovasc. Surg., September 1, 2003; 126(3): 694 - 702. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
W. T. Mahle, J. Crisalli, K. Coleman, R. M. Campbell, V. K. H. Tam, R. N. Vincent, and K. R. Kanter Deletion of chromosome 22q11.2 and outcome in patients with pulmonary atresia and ventricular septal defect Ann. Thorac. Surg., August 1, 2003; 76(2): 567 - 571. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Carotti, S. B. Albanese, G. Minniti, P. Guccione, and R. M. Di Donato Increasing experience with integrated approach to pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries Eur. J. Cardiothorac. Surg., May 1, 2003; 23(5): 719 - 727. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. S. Mackie, K. Gauvreau, S. B. Perry, P. J. del Nido, and T. Geva Echocardiographic predictors of aortopulmonary collaterals in infants with tetralogy of fallot and pulmonary atresia J. Am. Coll. Cardiol., March 5, 2003; 41(5): 852 - 857. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
T. Geva, G. F. Greil, A. C. Marshall, M. Landzberg, and A. J. Powell Gadolinium-Enhanced 3-Dimensional Magnetic Resonance Angiography of Pulmonary Blood Supply in Patients With Complex Pulmonary Stenosis or Atresia: Comparison With X-Ray Angiography Circulation, July 23, 2002; 106(4): 473 - 478. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. Stamm, I. Friehs, D. Zurakowski, A. M. Scheule, A. M. Moran, J. E. Lock, J. E. Mayer Jr, P. J. del Nido, and R. A. Jonas Outcome after reconstruction of discontinuous pulmonary arteries J. Thorac. Cardiovasc. Surg., February 1, 2002; 123(2): 246 - 257. [Abstract] [Full Text] [PDF]
|
|