Abstract

FONTE, Miguel et al. Acute respiratory failure: when time matters.... Nascer e Crescer [online]. 2011, vol.20, n.1, pp.45-48. ISSN 0872-0754.

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease in which all the pulmonary veins from both lungs fail to connect to the left atrium. The classification of TPAVC is related with the local of connection. The obstructive variant of TAPVC represents a severe cause of pulmonary hyper­tension and refractory cyanosis, with rapidly fatal evolution in the absence of emergent surgical repair. We present the clinical case of a newborn that initiated respiratory difficulty with continuous grunting and need for supplemental oxygen at  fifteen minutes of life. He was transferred to the Neonatal Intensive Care Unit presenting acute hypoxic respiratory failure, initiated mechanical ventilation with subsequent installation of persistent pulmonary hypertension. Prostaglandin E1 was then added to the treatment. The chest X-ray showed pulmonary edema. The echocardiogram revealed a severe obstructive supracardiac TAPVC. He was transferred to a specialized neonatal heart surgical center and was submitted to corrective surgery within the first 24 hours of life. Today, he is a twelve months’ healthy child, with normal growth and development. Obstructive TAPVC is one of the rare true surgical cardiac emergencies, and should be considered in any newborn with precocious refractory cyanosis, signs of pulmonary hypertension and low cardiac output.

Keywords : newborn; respiratory failure; cyanosis; congenital heart disease; cardiac surgery.

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