ABSTRACT
The patient was a 4-month-old infant, who underwent persistent ductus arteriosus interruption with titanium clips at the age of 13 days and, since the age of 2 months, had crises of hypoxia and hypertonicity. After clinical investigation, the presence of pulmonary hypertension was confirmed and left ventricular inflow tract obstruction was suspected. The patient underwent surgical treatment at the age of 4 months, during which right and left ventricular endocardial fibrosis was identified. The fibrosis was resected, but the infant had an unfavorable clinical evolution with significant diastolic restriction and died on the sixth postoperative day. Anatomicopathological and surgical findings suggested endomyocardial fibrosis, although that pathology is very rare at the patient's age
Subject(s)
Humans , Male , Infant , Endomyocardial Fibrosis , Fatal Outcome , Heart Ventricles , Hypertension, PulmonaryABSTRACT
Três recém-natos dentro do 1§ dia de vida, portadores de cianose, em decorrência de anomalia de Ebstein de importante repercussäo hemodinâmica, apresentavam aspecto de valva pulmonar imperfurada. Na evoluçäo clínica, verificou-se que a atresia era do tipo funcional, decorrente da baixa pressäo ventricular direita e hipertensäo no território pulmonar
Three newborn children with cyanosis due to Ebstein's anomaly of tricuspid valve with an important hemodynamic repercusssion presented features of imperforation of pulmonary valve. On the follow-up, clinical and laboratorial findings verified that pulmonary atresia was functional resulting from a combination of low right ventricular pressure and high pulmonary vascular resistance
Subject(s)
Humans , Infant, Newborn , Pulmonary Valve/abnormalities , Alprostadil/therapeutic use , Echocardiography, Doppler , Ebstein Anomaly/complications , Electrocardiography , VectorcardiographyABSTRACT
A angioplastia transluminal por cater-baläo tem sido utilizada de maneira promissora no tratamento de diversas cardiopatias congênitas, especialmente na estenose pulmonar valvar. Descrevemos o caso de um lactente de 3 meses de idade com diagnóstico de drenagem anômala total de veias pulmonares (DATVP) em veia cava superior direita revelando obstruçäo na sua desembocadura. Foi submetida à dilataçäo da obstruçäo utilizando-se dois cateteres-baläo de 4mm de diâmetro (Simpson Ultra Low Profile), com evidências manométricas, agngiográficas e clínicas de bom resultado do procedimento. A pressäo sistólica do ventrículo direito diminuiu de 68 par 49 mmHg e a pressäo média da veia pulmonar comum de 41 para 17 mmHg. Houve melhora evidente da cianose e dos sinais congestivos. Embora seja uma experiência isolada no Srviço, os autores sugerem que a angioplastia transluminal por cateter-baläo possa ser opçäo terapêutica inicial em crianças com DATVP na forma obstrutiva, consideradas de alto risco para correçäo cirúrgica