MANIFESTATIONS OF SCIMITAR SYNDROME IN AN ADULT

ABSTRACT

SESSION TITLE Anatomical Cardiovascular Disease Case Posters SESSION TYPE Case Report Posters PRESENTED ON 10/19/2022 1245 pm - 0145 pm INTRODUCTION: Scimitar Syndrome is a rare congenital disorder characterized by partial or complete anomalous pulmonary venous drainage to the inferior vena cava, right lung hypoplasia and displacement of the cardiac structures into the right hemi-thorax (1) The name Scimitar Syndrome is derived from the resemblance of the anomalous pulmonary vein to a curved Middle Eastern sword known as a scimitar (1). Some patients experience no symptoms while others may have severe pulmonary hypertension and dyspnea (2) (3). Cases may be discovered in infancy although some patients are not diagnosed until early adulthood. Here we present a case of a woman who was discovered to have symptomatic scimitar syndrome in her teenage years and opted to forego correction. CASE PRESENTATION Patient was a 38 year old female with past medical history of Scimitar Syndrome who presented to the pulmonary clinic after a short hospitalization with mild COVID-19. Prior to contracting COVID-19 she had experienced worsening dyspnea over one year and now becomes short of breath after walking one half block. Open surgical correction was offered at diagnosis but was not pursued due to patient preference. A CT Angiogram of the chest was performed while she was hospitalized which showed an anomalous right pulmonary vein draining to the hepatic IVC, minimal bilateral patchy opacities and displacement of the heart into the right hemi-thorax. Echocardiography showed normal right ventricular size and function and did not show evidence of pulmonary hypertension. She was referred to the adult congenital heart disease clinic and continued to have severe dyspnea and fatigue. 6 months later, repeat echocardiography and cardiac MRI demonstrated borderline dilation of the right ventricle and atrium but no echocardiographic evidence of pulmonary hypertension. The patient remains hesitant to undergo invasive procedures or interventions. She has been offered a right heart catheterization to better characterize her volume status and obtain direct measurement of her pulmonary artery pressures. DISCUSSION: Although generally discovered in infancy and childhood, Scimitar syndrome may not be discovered until adulthood. Various treatments are available for correction of scimitar syndrome including open surgical approaches with direct implantation of the scimitar vein into the left atrium, trans-catheter occlusion of aorto-pulmonary collaterals and re-routing of the anomalous vein into the left atrium via endoscopic graft placement (4) (6) (7) (8). If left uncorrected worsening left to right shunting and pulmonary hypertension may occur (5). Our patient has developed borderline right ventricular dilation and has experienced severe functional limitation. A right heart catheterization is indicated to determine her pulmonary artery pressures. This case illustrates the potential consequences of deferring early treatment for Scimitar Syndrome CONCLUSIONS:. Reference #1 Frydrychowicz A, Landgraf B, Wieben O, François CJ. Images in Cardiovascular Medicine. Scimitar syndrome added value by isotropic flow-sensitive four-dimensional magnetic resonance imaging with PC-VIPR (phase-contrast vastly undersampled isotropic projection reconstruction). Circulation. 2010 Jun 15;121(23)e434-6. doi 10.1161/CIRCULATIONAHA.109.931857. PMID 20547935 Reference #2 Abdullah A. Alghamdi, Mansour Al-Mutairi, Fahad Alhabshan, Scimitar syndrome restoration of native pulmonary venous connection, European Heart Journal Supplements, Volume 16, Issue suppl_B, November 2014, Pages B41–B43, https//doi.org/10.1093/eurheartj/suu025 Reference #3 Khan A, Ring NJ, Hughes PD. Scimitar syndrome (congenital pulmonary venolobar syndrome). Postgrad Med J. 2005 Apr;81(954)216. doi 10.1136/pgmj.2004.027813. PMID 15811882;PMCID PMC1743239 DISCLOSURES No relevant relationships by John Prudenti No relevant relationships by Anthony Smith