RESUMO
Partial anomalous pulmonary venous return is a rare congenital heart defect that can escape detection until adulthood and can be misdiagnosed as pulmonary hypertension and managed with vasodilators before the anomaly is identified. This report describes the cases of 3 patients with pulmonary hypertension whose hemodynamics and symptoms improved after repair of anomalous veins. Anomalous pulmonary veins are difficult to identify on a transthoracic echocardiogram, so a high index of suspicion and early use of additional imaging modalities are important to avoid a delayed diagnosis and progression to irreversible disease.
Assuntos
Cardiopatias Congênitas , Hipertensão Pulmonar , Veias Pulmonares , Síndrome de Cimitarra , Humanos , Adulto , Síndrome de Cimitarra/complicações , Síndrome de Cimitarra/diagnóstico por imagem , Síndrome de Cimitarra/cirurgia , Hipertensão Pulmonar/diagnóstico , Veias Pulmonares/anormalidades , Cardiopatias Congênitas/diagnóstico , HemodinâmicaRESUMO
We report a combined heart-lung transplantation following seven prior sternotomies in a patient born with a transitional atrioventricular septal defect. Previous surgeries to repair and replace the mitral valve led to pulmonary vein stenosis and pulmonary vascular disease. Eighth-time sternotomy and significant vascular adhesions led to a prolonged operation and to placing the heart-lung block anterior to the phrenic nerves. Despite this, the patient was ready for discharge after two weeks and continues to do well over nine months later. As more patients survive multiple cardiac palliations with some developing pulmonary vascular disease, heart-lung transplantation may become relevant again.