Abstract
Pulmonary vein stenosis (PVS) is a rare, but high mortality and resource intensive disease caused by mechanical obstruction or intraluminal myofibroproliferation, which can be post-surgical or idiopathic. There are increasing options for management including medications, cardiac catheterization procedures, and surgery. We queried the International Quality Improvement Collaborative for Congenital Heart Disease (IQIC) database for cases of PVS and described the cohort including additional congenital lesions and surgeries as well as infectious and mortality outcomes. IQIC is a quality improvement project in low-middle-income countries with the goal of reducing mortality after congenital heart surgery. Three cases were described in detail with relevant images. We identified 57 cases of PVS surgery, with similar mortality to higher income countries. PVS should be recognized as a global disease. More research and collaboration are needed to understand the disease, treatments, and outcomes, and to devise treatment approaches for low resource environments.
Highlights
Pulmonary vein stenosis (PVS) is a rare, but frequently fatal disease caused by physical obstruction or intraluminal myofibroproliferation, either post-surgical or idiopathic [1]
It is known that the rates of congenital heart disease (CHD) and deaths associated with CHD have increased in low-and-middle income countries (LMIC) [9]
Scans performed on described cases from Malaysia (Figure 2) showing repaired total anomalous pulmonary venous return (TAPVR)
Summary
Pulmonary vein stenosis (PVS) is a rare, but frequently fatal disease caused by physical obstruction or intraluminal myofibroproliferation, either post-surgical or idiopathic [1]. Intraluminal PVS is frequently associated with total anomalous pulmonary venous return (TAPVR) repair, but can be related to prematurity or be idiopathic [2]. The disease burden for PVS, and even TAPVR, in low-and-middle income countries (LMIC) is unknown. It is known that the rates of congenital heart disease (CHD) and deaths associated with CHD have increased in LMIC [9]. LMIC are performing complex repairs of TAPVR and managing idiopathic forms of PVS necessitating an understanding of the disease burden
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