Hostname: page-component-586b7cd67f-t8hqh Total loading time: 0 Render date: 2024-11-28T03:27:39.606Z Has data issue: false hasContentIssue false
  • English
  • Français

An institutional approach to, and results for, patient with tetralogy with pulmonary atresia and major systemic-to-pulmonary collateral arteries

Published online by Cambridge University Press:  01 December 2010

William M. DeCampli ,
I. Ricardo Argueta-Morales ,
Jennifer Zabinsky ,
Robert L. Hannan  and
Redmond P. Burke
William M. DeCampli*
Affiliation:
The Congenital Heart Institute, Arnold Palmer Hospital for Children, Orlando, Florida and Miami Children’s Hospital, Miami, Florida, United States of America Department of Medical Education, The University of Central Florida College of Medicine, Orlando, Florida, United States of America
I. Ricardo Argueta-Morales
Affiliation:
The Congenital Heart Institute, Arnold Palmer Hospital for Children, Orlando, Florida and Miami Children’s Hospital, Miami, Florida, United States of America
Jennifer Zabinsky
Affiliation:
The Congenital Heart Institute, Arnold Palmer Hospital for Children, Orlando, Florida and Miami Children’s Hospital, Miami, Florida, United States of America
Robert L. Hannan
Affiliation:
The Congenital Heart Institute, Arnold Palmer Hospital for Children, Orlando, Florida and Miami Children’s Hospital, Miami, Florida, United States of America
Redmond P. Burke
Affiliation:
The Congenital Heart Institute, Arnold Palmer Hospital for Children, Orlando, Florida and Miami Children’s Hospital, Miami, Florida, United States of America
*
Correspondence to: W. M. DeCampli, MD, PhD, Congenital Heart Institute, Arnold Palmer Hospital for Children, 50 W. Sturtevant Street, Orlando, Florida 32806, United States of America. Tel: 321 843 3294; Fax: 321 841 4260; E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Background

Tetralogy of Fallot with pulmonary atresia and diminutive or absent intrapericardial pulmonary arteries is a rare congenital abnormality, with high morbidity and mortality. Despite great advances in surgical- and catheter-based therapies, management remains challenging and controversial. We describe the surgical methods and the results from our institution.

Methods

We performed a retrospective study of the medical records of patients included in our institutional database with tetralogy and pulmonary atresia, concentrating on those predominantly managed by our programme over their lifetime. We obtained demographics and records of all catheterisations and operations, and established mortality. We assessed the current state of those surviving in terms of clinical function at their most recent clinical evaluation and right ventricular function by echocardiography.

Results

We assessed 38 patients, with 89% follow-up. The mean number of catheterisations for each patients was 5, with a range from 1 to 15. The mean number of operations was 2.2, with a range from 1 to 6. Unifocalisation had been performed in 26 patients, with 12 undergoing procedures to recruit the native pulmonary vasculature. Of the overall cohort, eight patients died. The ventricular septal defect had been closed in all but two patients. Most patients have no or mild exercise intolerance. Right ventricle dysfunction has been a continuing hazard for 15 years.

Conclusions

An individualised approach, using unifocalisation as well as aggressive attempts to recruit the available native pulmonary vasculature, achieves outcomes in the intermediate term superior to the natural history of the lesions, and comparable with those of other studies.

Keywords

Pulmonary atresia with ventricular septal defecttetralogy of Fallottetralogy of Fallot with pulmonary atresiaunifocalisationoutcomes
Type
Original Article
Information
Cardiology in the Young , Volume 20 , Issue S3: Rare and Challenging Congenital Cardiac Lesions: an Interdisciplinary Approach , December 2010 , pp. 128 - 134
Copyright
Copyright © Cambridge University Press 2010

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Malhotra, SP, Hanley, FL. Surgical management of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a protocol-based approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2009: 145–151.CrossRefGoogle ScholarPubMed
2.Griselli, M, McGuirk, SP, Winlaw, DS, et al. 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 2004; 127: 251–258.CrossRefGoogle ScholarPubMed
3.Lofland, GK. Pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2004; 7: 85–94.CrossRefGoogle ScholarPubMed
4.Brizard, CP, Liava’a, M, d’Udekem, Y. Pulmonary atresia, VSD and Mapcas: repair without unifocalization. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2009: 139–144.CrossRefGoogle ScholarPubMed
5.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: 1269–1275.CrossRefGoogle ScholarPubMed
6.Reddy, VM, Liddicoat, JR, Hanley, FL. Midline one-stage complete unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. J Thorac Cardiovasc Surg 1995; 109: 832–845.CrossRefGoogle ScholarPubMed
7.Farouk, A, Zahka, K, Siwik, E, et al. Individualized approach to the surgical treatment of tetralogy of Fallot with pulmonary atresia. Cardiol Young 2009; 19: 76–85.CrossRefGoogle Scholar
8.Song, SW, Park, HK, Park, YH, Cho, BK. Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries – 18-year clinical experience and angiographic follow-up of major aortopulmonary collateral arteries. Circ J 2009; 73: 516–522.CrossRefGoogle Scholar
9.Carotti, A, Di Donato, RM, Squitieri, C, Guccione, P, Catena, G. Total repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: an integrated approach. J Thorac Cardiovasc Surg 1998; 116: 914–923.CrossRefGoogle ScholarPubMed
10.Bull, K, Somerville, J, Ty, E, Spiegelhalter, D. Presentation and attrition in complex pulmonary atresia. J Am Coll Cardiol 1995; 25: 491–499.CrossRefGoogle ScholarPubMed
11.Leonard, H, Derrick, G, O’Sullivan, J, Wren, C. Natural and unnatural history of pulmonary atresia. Heart 2000; 84: 499–503.CrossRefGoogle ScholarPubMed
12.d’Udekem, Y, Alphonso, N, Nørgaard, MA, et al. Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries: unifocalization brings no long-term benefits. J Thorac Cardiovasc Surg 2005; 130: 1496–1502.CrossRefGoogle ScholarPubMed
13.Nørgaard, MA, Alphonso, N, Cochrane, AD, Menahem, S, Brizard, CP, d’Udekem, Y. Major aorto-pulmonary collateral arteries of patients with pulmonary atresia and ventricular septal defect are dilated bronchial arteries. Eur J Cardiothorac Surg 2006; 29: 653–658.CrossRefGoogle ScholarPubMed
14.Brawn, WJ, Jones, T, Davies, B, Barron, D. How we manage patients with major aorta pulmonary collaterals. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2009: 152–157.CrossRefGoogle ScholarPubMed