Total anomalous pulmonary venous connection with ccTGA and VSD: Can pulmonary artery banding avert pulmonary venous obstruction?

Pediatr Int. 2015 Aug;57(4):714-6. doi: 10.1111/ped.12580. Epub 2015 Mar 25.

Abstract

We describe a rare case of infracardiac total anomalous pulmonary venous connection (TAPVC), associated with congenitally corrected transposition of the great arteries (ccTGA) and ventricular septal defect, in which the patient had undergone pulmonary artery banding (PAB) at 16 days of age. She began to have episodes of severe cyanosis while crying, 2 weeks after PAB. Cardiac catheterization at 34 days of age showed severe pulmonary hypertension and a transhepatic pressure gradient of 7 mmHg. The infant underwent TAPVC repair and conventional repair for ccTGA at 35 days of age. Although PAB might have the provisional effect of delaying the manifestation of pulmonary venous obstruction (PVO), it is unable to prevent the development of PVO due to the high resistance of the hepatic sinusoids. Signs of PVO should be closely monitored so that TAPVC can be repaired in a timely fashion.

Keywords: congenitally corrected transposition of the great arteries; pulmonary artery banding; pulmonary hypertension; pulmonary venous obstruction; total anomalous pulmonary venous connection.

MeSH terms

  • Adult
  • Angiography
  • Cardiac Surgical Procedures / methods
  • Congenitally Corrected Transposition of the Great Arteries
  • Echocardiography
  • Female
  • Follow-Up Studies
  • Heart Septal Defects, Ventricular / diagnosis*
  • Heart Septal Defects, Ventricular / surgery
  • Humans
  • Imaging, Three-Dimensional
  • Infant, Newborn
  • Ligation
  • Pregnancy
  • Pulmonary Artery / surgery*
  • Pulmonary Veno-Occlusive Disease / congenital*
  • Pulmonary Veno-Occlusive Disease / diagnosis
  • Pulmonary Veno-Occlusive Disease / surgery
  • Time Factors
  • Tomography, X-Ray Computed
  • Transposition of Great Vessels / diagnosis*
  • Transposition of Great Vessels / surgery
  • Vascular Surgical Procedures / methods*