Christopher P. Scott, Kamel Shibbani, Christopher A. Caldarone, Daniel McLennan
Children’s Wisconsin. University of Iowa Stead Family Children’s Hospital. Texas Children’s Hospital.
United States
European Heart Journal Case Reports
Eur Heart J Case Rep 2021; 5:
DOI: 10.1093/ehjcr/ytab429
Abstract
Background: Tetralogy of Fallot (TOF) is the most common congenital cyanotic cardiac lesion. Pulmonary vein stenosis occurs much less commonly and remains difficult to manage. It is exceedingly uncommon for a patient to have both lesions. This case highlights the diagnostic and management difficulties in an infant with these two lesions.
Case summary: The patient is a 4-month-old female infant with a history of TOF status post right ventricular outflow tract stent placement who presented after a hypoxaemic event at home to 40% SpO2. Computed tomography angiography demonstrated previously undiagnosed pulmonary vein stenosis of all four veins. She underwent multiple catheter-based palliations including balloon dilations and stent placements in each pulmonary vein in order to maximize her chances of successful definitive repair. She underwent successful repair of her TOF and pulmonary vein stenosis at ten months of age.
Discussion: The combination of TOF and pulmonary vein stenosis is not common, but when these lesions co-occur, they present a significant dilemma in determining the timing of surgery in order to optimize the odds of a successful outcome. This case demonstrates that serial catheter-based procedures can be valuable tools in minimizing pre-operative risk factors and highlights one strategy in determining timing of definitive surgical repair.
Category
Segmental Pulmonary Venous Disease. Without a Focus on Pulmonary Hypertension
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes