A central shunt to rehabilitate diminutive pulmonary arteries in patients with pulmonary atresia with ventricular septal defect

Objectives Pulmonary atresia (PA) with ventricular septal defect (VSD) and systemic-pulmonary collateral arteries (SPCA’s) has a variable anatomy with regard to the pulmonary vasculature, asking for an individualized surgical treatment. A protocol was applied consisting of staged unifocalization and correction. Methods Since 1989 39 consecutive patients were included (median age at first operation 13 months). In selected cases a central aorto-pulmonary shunt was performed as first procedure. Unifocalization procedures were performed through a lateral thoracotomy. Correction consisted of shunt takedown, VSD closure and interposition of an allograft between the right ventricle and the reconstructed pulmonary artery. Postoperatively and at follow up echocardiographic data were obtained. Results In 39 patients 66 unifocalization procedures were performed. Early mortality was 5%. Seven patients were considered not suitable for correction, four of them died. One patient is awaiting further correction. Correction was done successfully in 28 patients. Operative mortality was 3% and late mortality 11%. Median follow-up after correction was 19 years. Eleven patients needed homograft replacement. Freedom from conduit replacement was 88%, 73% and 60% at 5, 10 and 15 years respectively. Right ventricular function was reasonable or good in 75 % of the patients. Conclusions After complete unifocalization 30/37 patients (81%) were considered correctable. The main reasons for palliative treatment without correction were pulmonary hypertension and/or inadequate outgrowth of pulmonary arteries. Staged approach of PA, VSD and SPCA’s results in adequate correction and good functional capacity. RV function after correction remains reasonable or good in the majority of patients.

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Surgical repair of pulmonary atresia with ventricular septal defect and hypoplastic pulmonary arteries: A different approach

Journal of Thoracic and Cardiovascular Surgery ◽

10.1016/s0022-5223(19)39305-5 ◽

1981 ◽

Vol 82 (4) ◽

pp. 638-639 ◽

Cited By ~ 2

Author(s):

Kishan C. Agarwal

Keyword(s):

Ventricular Septal Defect ◽

Septal Defect ◽

Surgical Repair ◽

Pulmonary Arteries ◽

Pulmonary Atresia

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Velo-Cardio-Facial Syndrome Associated With Ventricular Septal Defect, Pulmonary Atresia, and Hypoplastic Pulmonary Arteries

PEDIATRICS ◽

10.1542/peds.89.5.915 ◽

1992 ◽

Vol 89 (5) ◽

pp. 915-919

Author(s):

Kerry B. Jedele ◽

Virginia V. Michels ◽

Francisco J. Puga ◽

Robert H. Feldt

Keyword(s):

Ventricular Septal Defect ◽

Septal Defect ◽

Pulmonary Arteries ◽

Pulmonary Atresia ◽

Severe Form ◽

Arterial Tree ◽

Complete Repair ◽

Pulmonary Arterial ◽

Ear Anomalies ◽

Velo Cardio Facial Syndrome

We report on 15 patients with velo-cardio-facial syndrome who had a severe form of tetralogy of Fallot (pulmonary atresia, ventricular septal defect, and hypoplastic pulmonary arteries). Noncardiac anomalies in these patients included typical facial and ear anomalies in 15, nasal speech in 13, palate anomalies in 10, and developmental delay in 10. Seven patients had significant bronchospasm, which has not been reported in association with the velo-cardio-facial syndrome. All 15 patients had severe abnormalities of the arborization of the pulmonary arterial tree, which also has not been reported in velo-cardio-facial syndrome. All patients underwent staging operations to prepare the true pulmonary vascular tree for complete repair of the defect (five underwent complete repair and three survived). Of the remaining 10 patients, 6 are awaiting further operation, 3 are not candidates for complete repair, and 1 has died.

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