Percutaneous closure of isolated ostium secundum-type atrial septal defect in a patient with Mayer-Rokitansky-Küster-Hauser syndrome

• Main Authors: Murat Akcay, Okan Gulel, Korhan Soylu, Murat Meric, Muzaffer Elmali
• Format: Article
• Language: English
• Published: Elsevier 2016-12-01
• Series: Revista Portuguesa de Cardiologia
• Online Access: http://www.sciencedirect.com/science/article/pii/S0870255116302736

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare congenital anomaly characterized by complete or partial aplasia of the uterus and the upper part of the vagina. It is reported to be associated with cardiovascular disorders including atrial septal defect, anomalous pulmonary venous return, aortopulmonary window, pulmonary valve stenosis, mitral valve prolapse, tetralogy of Fallot, truncus arteriosus, and patent ductus arteriosus. Herein, for the first time in the medical literature, we present percutaneous closure of an isolated ostium secundum atrial septal defect in this syndrome. Resumo: A síndrome de Mayer-Rokitansky-Kuster-Hauser (MRKH) é uma anomalia congénita rara, caraterizada por uma aplasia completa ou parcial do útero e da parte superior da vagina. Tem sido associado a problemas cardiovasculares, incluindo comunicação interauricular, retorno venoso pulmonar anómalo, janela aorto-pulmonar, estenose valvular pulmonar, prolapso da válvula mitral, tetralogia de Fallot, tronco arterial e persistência do canal arterial. Pela primeira vez na literatura médica, apresentamos um caso de encerramento percutâneo de comunicação interauricular tipo ostium secundum isolado nesta síndrome. Keywords: Atrial septal defect, Mayer-Rokitansky-Küster-Hauser syndrome, Percutaneous closure, Palavras-chave: Comunicação intra-auricular, Síndrome de Mayer-Rokitansky-Kuster-Hauser, Encerramento percutâneo