Case report
Percutaneous closure of isolated ostium secundum-type atrial septal defect in a patient with Mayer-Rokitansky-Küster-Hauser syndrome
Encerramento percutâneo da comunicação interauricular tipo ostium secundum isolado em doente com síndrome de Mayer-Rokitansky-Kuster-Hauser (MRKH)
Murat Akcaya,, , Okan Gulelb, Korhan Soylub, Murat Mericb, Muzaffer Elmalic
a Cardiology Clinic, Terme State Hospital, Samsun, Turkey
b Department of Cardiology, Faculty of Medicine, Ondokuz Mayis University, Samsun, Turkey
c Department of Radiology, Faculty of Medicine, Ondokuz Mayis University, Samsun, Turkey
Recebido 06 Dezembro 2015, Aceitaram 02 Fevereiro 2016
Abstract

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

Métricas

  • Impact Factor: 1.195(2016)
  • SCImago Journal Rank (SJR):0,24
  • Source Normalized Impact per Paper (SNIP):0,398