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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]
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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

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