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Janela Aortopulmonar - Uma Causa Rara de Insuficiência Cardíaca Congestiva em Lactente
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Advisor(s)
Abstract(s)
RESUMO
A janela aortopulmonar é uma cardiopatia
congénita rara, em que se verifica uma comunicação entre a aorta
ascendente e a artéria pulmonar, com
aparelhos valvulares distintos. Em 50%
dos casos coexistem outros defeitos cardíacos.
As manifestações clínicas são inespecíficas e geralmente precoces, traduzidas
por sinais e sintomas de insuficiência
cardíaca, comuns a outras situações de
shunt esquerdo-direito.
Apresenta-se o caso clínico de um
recém-nascido, sem antecedentes familiares
e pessoais relevantes, com quadro
de insuficiência cardíaca congestiva que
se manifestou na segunda semana de
vida. Inicialmente, foi efectuado por telemedicina
o diagnóstico de comunicação
interventricular não restritiva e persistência
de canal arterial pelo que iniciou terapêutica
diurética endovenosa. Por persistência
do quadro, apesar da terapêutica
médica instituída, foi transferido para um
centro de Cardiologia Pediátrica onde foi
efectuado o diagnóstico definitivo: janela
aortopulmonar com malposição dos grandes
vasos, associada a comunicação interventricular,
comunicação interauricular
e persistência de canal arterial. Foi submetido
a correcção cirúrgica total ao vigésimo
terceiro dia de vida, com evolução
favorável.
Este caso é relevante pela sua raridade
(encontrámos apenas um caso descrito
de janela aortopulmonar associado
a malposição dos grandes vasos) e por
alertar para a importância de considerar
hipóteses de diagnóstico mais raras perante
um quadro de insuficiência cardíaca
congestiva refractário à terapêutica
médica, ainda que coexistam outros defeitos
cardíacos.
ABSTRACT Aortopulmonary window is a rare form of congenital heart disease, consisting of a communication between the ascending aorta and the pulmonary artery. In 50% of the cases other cardiac defects are associated. The clinical manifestations are nonspecific and usually occur early, sharing the signs and symptoms of heart failure, with other more common situations with a significant left-right shunt. The case of a male newborn with special features is reported. He had an irrelevant family and personal history, and presented with congestive heart failure at two weeks of life. The first diagnosis was made by telemedicine and included persistent ductus arteriosus and large ventricular septal defect. However, due to the maintenance of symptoms after intra-venous medical therapy, he was transferred to a Department of Paediatric Cardiology. The final diagnosis was aortopulmonary window with malposition of the great vessels, associated with ventricular septal defect, persistent ductus arteriosus and atrial septal defect. He had corrective surgery with closure of all defects on the 23rd day of life, with a good outcome. This report is relevant due to the uncommon nature of the findings (only one previous report of aortopulmonary window with malposition of great vessels could be found in the literature); it also stresses the importance of a high degree of awareness for the diagnosis. Aortopulmonary window should always be considered in newborns and infants with heart failure, even in the presence of other cardiac defects.
ABSTRACT Aortopulmonary window is a rare form of congenital heart disease, consisting of a communication between the ascending aorta and the pulmonary artery. In 50% of the cases other cardiac defects are associated. The clinical manifestations are nonspecific and usually occur early, sharing the signs and symptoms of heart failure, with other more common situations with a significant left-right shunt. The case of a male newborn with special features is reported. He had an irrelevant family and personal history, and presented with congestive heart failure at two weeks of life. The first diagnosis was made by telemedicine and included persistent ductus arteriosus and large ventricular septal defect. However, due to the maintenance of symptoms after intra-venous medical therapy, he was transferred to a Department of Paediatric Cardiology. The final diagnosis was aortopulmonary window with malposition of the great vessels, associated with ventricular septal defect, persistent ductus arteriosus and atrial septal defect. He had corrective surgery with closure of all defects on the 23rd day of life, with a good outcome. This report is relevant due to the uncommon nature of the findings (only one previous report of aortopulmonary window with malposition of great vessels could be found in the literature); it also stresses the importance of a high degree of awareness for the diagnosis. Aortopulmonary window should always be considered in newborns and infants with heart failure, even in the presence of other cardiac defects.
Description
Keywords
Janela aortopulmonar cardiopatia congénita insuficiência cardíaca recém-nascido Aortopulmonary window congenital heart disease cardiac failure newborn
Citation
Nascer e Crescer 2009; 18(4): 283-287