Cardiology in the Young

Original Article

Individualized approach to the surgical treatment of tetralogy of Fallot with pulmonary atresia*

Ahmed Farouka1, Kenneth Zahkaa2, Ernest Siwika2, Francine Erenberga2, Yasser Al-Khatiba2, Alex Goldena2, Mohsen Karimia1, Minhaz Uddina1 and Hani A. Henneina1 c1

a1 Department of Pediatric Cardiothoracic Surgery, Rainbow Babies and Children’s Hospital, Case Western Reserve University, Cleveland, Ohio, United States of America

a2 Department of Pediatric Cardiology, Rainbow Babies and Children’s Hospital, Case Western Reserve University, Cleveland, Ohio, United States of America


Background Tetralogy of Fallot with pulmonary atresia is a heterogeneous group of defects, characterised by diverse sources of flow of blood to the lungs, which often include multiple systemic-to-pulmonary collateral arteries. Controversy surrounds the optimal method to achieve a biventricular repair with the fewest operations while basing flow to the lungs on the native intrapericardial pulmonary arterial circulation whenever possible. We describe an individualized approach to this group of patients that optimizes these variables.

Methods Over a consecutive 10-year period, we treated 66 patients presenting with tetralogy of Fallot and pulmonary atresia according to the source of the pulmonary arterial flow. Patients were grouped according to whether the flow of blood to the lungs was derived exclusively from the intrapericardial pulmonary arteries, as seen in 29 patients, exclusively from systemic-to-pulmonary collateral arteries, as in 5 patients, or from both the intrapericardial pulmonary and collateral arteries, as in the remaining 32 patients. We divided the latter group into 9 patients deemed simple, and 23 considered complex, according to whether the pulmonary arterial index was greater than or less than 90 millimetres squared per metre squared, and whether the number of collateral arteries was less than or greater than 2, respectively.

Results We achieved complete biventricular repair in 58 patients (88%), with an overall mortality of 3%. Repair was accomplished in a single stage in all patients without systemic-to-pulmonary collateral arteries, but was staged, with unifocalization, in the patients lacking intrapericardial pulmonary arteries. Complete repair without unifocalization was achieved in all patients with the simple variant of the mixed morphology, and in 56% of patients with the complex variant. The average number of procedures per patient to achieve complete repair was 1, 2.2, 3.8, and 2.6 in patients with exclusively native intrapericardial, simple and mixed, complex and mixed and exclusively collateral pulmonary arterial flow, respectively.

Conclusions An individualized approach based on the morphology of the pulmonary arterial supply permits achievement of a high rate of complete intracardiac repairs, basing pulmonary arterial flow on the intrapericardial pulmonary arteries in the great majority of cases, and has a low rate of reoperation and mortality.

(Accepted September 03 2008)


c1 Correspondence to: Hani A. Hennein, MD, Chief, Pediatric Cardiothoracic Surgery, Rainbow Babies and Children’s Hospital, Associate Professor, Case Western Reserve University, 11100 Euclid Avenue, Suite 380, Cleveland, Ohio 44106, United States of America. Tel: 216-844-3058; Fax: 216-844-3517; E-mail:


* The presentation on which this work is based was given at the Inaugural Meeting of the World Society for Pediatric and Congenital Heart Surgery, held in Washington, District of Columbia, May 3 and 4, 2007.