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Insuficiência respiratória aguda: quando o tempo conta…
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Advisor(s)
Abstract(s)
RESUMO
A drenagem venosa pulmonar anómala total (DVPAT) é
uma cardiopatia congénita rara em que as veias pulmonares não
apresentam a normal conexão à aurícula esquerda. A sua classificação relaciona-se com o local de ligação. Na sua variante
obstrutiva, tem uma evolução rapidamente fatal na ausência de
tratamento cirúrgico emergente. Apresenta-se o caso clínico de
um recém-nascido que iniciou dificuldade respiratória com gemido
e necessidade de oxigénio suplementar aos quinze minutos
de vida. Foi transferido para a Unidade de Cuidados Intensivos
Neonatais por insuficiência respiratória com hipóxia aguda e instalação
de hipertensão pulmonar. Iniciou ventilação mecânica e
terapêutica com prostaglandina E1. A radiografia do tórax mostrou
sinais sugestivos de edema pulmonar. O ecocardiograma
revelou DVPAT para a veia cava superior com obstrução grave.
Foi transferido com carácter de emergência para Centro especializado
em cirurgia cardíaca neonatal, onde foi submetido a
correcção cirúrgica nas primeiras 24 horas de vida. Actualmente,
com doze meses, é uma criança saudável com crescimento e
desenvolvimento adequados. A DVPAT obstrutiva é uma das raras
verdadeiras emergências cirúrgicas cardíacas, salientando-se a importância da sua suspeição clínica perante um RN com
cianose precoce refractária associada a sinais de insuficiência
cardíaca de baixo débito e hipertensão pulmonar.
ABSTRACT 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.
ABSTRACT 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.
Description
Keywords
Recém-nascido insuficiência respiratória cianose cardiopatia congénita cirurgia cardíaca Newborn respiratory failure cyanosis congenital heart disease cardiac surgery
Citation
Nascer e Crescer 2011; 20(1): 45-48