[Pulmonary atresia with open septum: analysis of therapeutic management apropos of 47 cases].
Pulmonary atresia with ventricular septal defect (VSD) accounts for 2% of all congenital heart disease. The prognosis is poor (30% survival at 1 year) and early medico-surgical management is essential. A natural history of retrospective analysis of 47 consecutive patients with this abnormality born between 1962 and 1992, hospitalised between 1971 and 1992 and followed up for an average of 9.6 years (range: 1 month to 24 years) allowed identification of several anatomical groups: Group I (N = 19, 40.4%) comprised patients who underwent complete correction; Group II comprised patients who had undergone one or more palliative procedures. In Group II, there were 13 survivors at the end of the study (Group IIA); 14 patients died (Group IIB) and one was lost tot follow-up. The global mortality was 38% (21% in Group I and 52% in Group II). Overall management was intense: 166 cardiac catheter studies, 109 surgical operations including 48 systemico-pulmonary anastomoses, 34 right ventricular outflow reconstructions, I Ilbawi procedure. The survival was higher in Group I (79%) than in Group II (48%) indicating a clear advantage in reestablishing as normal as possible anatomic and haemodynamic status. Patients in Group I had a higher incidence of pulmonary artery confluence, patent ductus and an anatomically simple form of pulmonary atresia with VSD, all good surgical prognostic factors. The first palliative procedure was performed on average at 35 months in Group I, 46 months in Group IIA and 24 months in Group IIB; complete correction, when possible, was performed at about the 10th year, after an average of 3 operative procedures per patient.(ABSTRACT TRUNCATED AT 250 WORDS)
Service de cardiologie et néphrologie pédiatrique, CHR Toulouse, hôpital Purpan, Toulouse.
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Lambert
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Martinez
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Vahdat
D000206:Actuarial Analysis; D000293:Adolescent; D000328:Adult; D000790:Angiocardiography; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000379:methods; D002648:Child; D002675:Child, Preschool; D006345:Heart Septal Defects, Ventricular / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D006801:Humans; D007223:Infant; D011379:Prognosis; D018633:Pulmonary Atresia / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D012189:Retrospective Studies; D015996:Survival Rate
L'atrésie pulmonaire à septum ouvert: analyse de la prise en charge thérapeutique à partir de 47 observations.