Aortopulmonary window with pumonary atresia with ventricular septal defect with D-transposition of great arteries: extremely rare anomaly

BMJ Case Rep. 2018 Jun 13:2018:bcr2018224401. doi: 10.1136/bcr-2018-224401.

Abstract

Aortopulmonary window (APW) is rare a congenital heart disease accounting for 0.1%-0.2% of all congenital heart defects. The 35% of the APW has been associated with wide variety of other structural heart diseases such as ventricular septal defect, persistent ductus arteriosus, arch anomalies and coronary artery anomalies. To the best of our knowledge, only six cases of APW with pulmonary atresia with ventricular septal defect has been described in the literature. It resembles the type 1 truncus arteriosus, and differentiation from this condition is important prior to surgical correction. We present a case of 14-year-old girl child; she was diagnosed with APW with pulmonary atresia with ventricular septal defect and D transposition of great arteries with the help of echocardiography, cardiac catheterisation and cardiac CT.

Keywords: cardiovascular Medicine; interventional cardiology; radiology (diagnostics).

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple / diagnostic imaging
  • Abnormalities, Multiple / drug therapy
  • Abnormalities, Multiple / pathology*
  • Abnormalities, Multiple / physiopathology
  • Adolescent
  • Aftercare
  • Aortopulmonary Septal Defect / diagnostic imaging
  • Aortopulmonary Septal Defect / drug therapy
  • Aortopulmonary Septal Defect / pathology*
  • Aortopulmonary Septal Defect / physiopathology
  • Cardiac Catheterization / methods
  • Echocardiography / methods
  • Electrocardiography / methods
  • Female
  • Heart Septal Defects, Ventricular / diagnostic imaging
  • Heart Septal Defects, Ventricular / drug therapy
  • Heart Septal Defects, Ventricular / pathology*
  • Heart Septal Defects, Ventricular / physiopathology
  • Humans
  • Pulmonary Atresia / diagnostic imaging
  • Pulmonary Atresia / drug therapy
  • Pulmonary Atresia / pathology*
  • Pulmonary Atresia / physiopathology
  • Rare Diseases
  • Transposition of Great Vessels / diagnostic imaging
  • Transposition of Great Vessels / drug therapy
  • Transposition of Great Vessels / pathology*
  • Transposition of Great Vessels / physiopathology
  • Treatment Outcome