Treatment of Congenital and Acquired Pulmonary Vein Stenosis
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CONGENITAL HEART DISEASE (RA KRASUSKI AND G FLEMING, SECTION EDITORS)
Treatment of Congenital and Acquired Pulmonary Vein Stenosis Patcharapong Suntharos 1 & Lourdes R. Prieto 1
# Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose of Review Pulmonary vein stenosis (PVS) is a rare entity that until the last 2 decades was seen primarily in infants and children. Percutaneous and surgical interventions have limited success due to relentless restenosis, and mortality remains high. In adults, acquired PVS following ablation for atrial fibrillation has emerged as a new syndrome. This work will review these two entities with emphasis on current treatment. Recent Findings Greater emphasis on understanding and addressing the mechanism of restenosis for congenital PVS has led to the use of drug-eluting stents (DES) and systemic drug therapy to target neo-intimal growth. Frequent reinterventions are positively affecting outcomes. Longer-term outcomes of percutaneous treatment for acquired PVS are emerging. Summary Treatment of congenital PVS continues to be plagued by restenosis. DES show promise, but frequent reinterventions are required. Larger upstream vein diameter predicts success for congenital and acquired PVS interventions. Efforts to induce/ maintain vessel growth are important for future treatment strategies. Keywords Congenital pulmonary vein stenosis . Acquired pulmonary vein stenosis . Pulmonary vein isolation . Sutureless repair . Balloon angioplasty . Stent placement
Introduction Pulmonary vein stenosis (PVS) is a rare disease that in infants and children can occur as an isolated finding, associated with other forms of congenital heart disease not specifically involving the pulmonary veins, or following surgery for anomalous pulmonary venous return (APVR). It is also associated with prematurity, particularly in patients with severe chronic lung disease [1]. In adults, pulmonary vein stenosis was exceedingly rare until the widespread adoption of radiofrequency ablation to treat atrial fibrillation beginning in the late 1990s, known as pulmonary vein isolation (PVI) [2, 3]. Other adultThis article is part of the Topical Collection on Congenital Heart Disease Electronic supplementary material The online version of this article (https://doi.org/10.1007/s11886-020-01395-x) contains supplementary material, which is available to authorized users. * Lourdes R. Prieto [email protected] Patcharapong Suntharos [email protected] 1
Division of Pediatric Cardiology, Nicklaus Children’s Hospital, 3100 SW 62nd Avenue, Miami, FL 33155, USA
onset etiologies of PVS include fibrosing mediastinitis, mediastinal neoplasms, and other rare infiltrative processes. For the purpose of this review, congenital (pediatric) PVS will be referred to as either primary when occurring in patients with normally connected pulmonary veins or post-operative when arising after surgical repair of anomalous pulmonary venous return. Acquired PVS will refer to adult-onset PVS, m
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