Abstract
BACKGROUND: Alagille syndrome is a multisystem disease that results in various vascular anomalies, commonly involving the cardiac and pulmonary systems. To the best of our knowledge, there is no literature regarding the cardiovascular outcomes of these patients in association with coronavirus disease 2019 (COVID-19). CASE SUMMARY: A 34-year-old woman with a history of Alagille syndrome who underwent successful atrial septal defect with partial anomalous pulmonary veins and patent ductus arteriosus repair, as well as left pulmonary artery catheterization and stenting in childhood due to pulmonary stenosis. The patient was without any respiratory symptoms and was a dancer prior to contracting COVID-19. Several weeks after her COVID-19 infection, she developed left pulmonary artery stent thrombosis and subsequent symptomatic pulmonary hypertension. A treatment strategy of anticoagulation alongside pharmacological agents for pulmonary hypertension for 3 months followed by balloon pulmonary artery angioplasty to reopen the stenosis was unsuccessful. CONCLUSION: In the era of COVID-19, patients with pulmonary vascular malformations and endovascular stents are at an increased risk for chronic thromboembolic disease. Patients may benefit from prophylactic antiplatelet or anticoagulation therapy. Stent thrombosis is a devastating phenomenon and should be treated urgently and aggressively with balloon pulmonary angioplasty, and/or a thrombolytic agent.