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Eur J Cardiothorac Surg 2001;20:590-597
© 2001 Elsevier Science NL

Pulmonary atresia with ventricular septal defect, extremely hypoplastic pulmonary arteries, major aorto–pulmonary collaterals

^a Cardio-thoracic Unit, Children's Hospital La Timone, Marseille, France
^b Cardiology Unit, Children's Hospital La Timone, Marseille, France

Received 20 December 2000; received in revised form 15 May 2001; accepted 13 June 2001.

Corresponding author. Centre Hospitalier et Universitaire de la Timone, Boulevard Jean Moulin, 13005 Marseille Cedex 05, France. Tel.: +33-4-91-38-66-76; fax: +33-4-91-47-81-70
e-mail: dmetras{at}ap-hm.fr

Objective: Among 63 patients with pulmonary atresia and ventricular septal defect (VSD), 10 patients with extreme hypoplasia of the pulmonary arteries (PA) (mean Nakata index 20.6 mm²/m²), but with confluent arteries and a diminutive main PA, and major aorto–pulmonary collaterals (MAPCAS), have been submitted to a ‘rehabilitation’ of the PA with several stages: (i) connection between RV and PAs, (ii) interventional catheterizations, (iii) complete correction with or without unifocalisation. We report here the results of this approach. Methods: The RV–PA connection was direct (nine cases) or with an homograft conduit (one case), done under normothermic cardiopulmonary by-pass in patients aged 4.9 months (range 0.1–18 months). Subsequently, six underwent interventional catheterizations (dilations and stents in the PA, MAPCAS occlusion by coils). Complete correction was done in seven patients (mean age 30 months, range 8–49). One patient is awaiting correction. Results: One patient died after the first stage. All patients having had the third stage had a satisfactory development of the PA, had a complete closure of the VSD and a satisfactory reconstruction of the PA bifurcation. There was one death of severe pulmonary infection 6 months after repair. All other patients have been followed by catheterization and/or echocardiograms. With a follow-up of 83±65 months, all patients are improved, 50% have no cardiac medications, none has residual shunt, RV/LV pressure ratio is 0.6 (range 0.3–1). Conclusions: The strategy of ‘rehabilitation’ of PA allowing: (i) antegrade flow in the PA, (ii) interventional catheterizations, (iii) growth of the PA with possible angiogenesis, (iv) complete correction, is a logical approach to be undertaken in the young patient and is a valid alternative to strategies relying more on MAPCAS for pulmonary vascular supply. The therapeutic sequences depend upon the individual anatomy.

Key Words: Pulmonary atresia • Rehabilitation • Pulmonary arteries • Correction

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