Pulmonary atresia – unfavorable prognostic factor in tetralogy of Fallot with neonatal onset

Revista Romana de PEDIATRIE | Volumul LXI, Nr. 1, An 2012

ISSN 1454-0398 | e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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Aniela Luminita Rugina, A.G. Dimitriu, N. Nistor and Doina Mihaila

ABSTRACT

Infundibular or valvular pulmonary atresia occurs in 15-25% of tetralogy of Fallot cases, is manifested clinically in the neonatal period, is included in the ductdepedent congenital heart disease (CHD), requires prostaglandin E1 infusion and early surgical treatement. Tetralogy of Fallot may associate more anatomical variants, some with poor prognosis in the first year of life, if the cardiovascular surgery is not performed in time, most commonly in the neonatal period. The aim of this article is presenting a clinical case of tetralogy of Fallot and pulmonary atresia, diagnosed in the neonatal period, with severe cyanosis and had a progression to death whithin 6 months of life. Pathologic examination revealed malformative association: renal left hypoplasia, with left megaureter and hidrocalicosis without urinary tract infection.

Key words: infant, cyanosis, tetralogy of Fallot, pulmonary artery atresia, pathological examination

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