ABSTRACT
SESSION TITLE: Anatomical Cardiovascular Disease Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 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
ABSTRACT
Case Report Sinus venosus ASDs are usually associated with one or more anomalous right sided pulmonary veins. Diagnosis by transthoracic echocardiogram (TTE) and confirmation with transesophageal echocardiogram (TEE) and right heart catherization can lead to a multidisciplinary approach for appropriate surgical correction. Case A 21-year-old male with no PMH presented to clinic for a routine physical to return to collegiate athletics post COVID-19 infection. His only complaint during this time was residual dyspnea (NYHA Class 1). An ECG was obtained and showed an incomplete right bundle branch block and TTE revealed an ASD with moderate RV dilation. Repeat TTE at our institution showed an interatrial shunt on injection of agitated saline via the right arm within three beats after injection. Subsequent right heart catheterization with shunt series revealed a step in oxygen saturation from 75% in the superior vena cava (SVC) to 88% in the right atrium. Additional imaging obtained with TEE confirmed a sinus venosus ASD. Cardiovascular surgery was engaged and further imaging with computed tomography angiography (CTA) of the chest confirmed a large superior sinus venosus ASD measuring 16 mm in diameter as well as partial anomalous right pulmonary venous drainage into the SVC. The heart team decided on a minimally invasive robotic approach and performed an autologous pericardial patch repair of the ASD with redirection of the right and superior pulmonary veins into the left atrium. Intra-op TEE showed no residual shunt across the interatrial septum. The patient had an uncomplicated post-operative course and was discharged home on day 4. Decision-Making Sinus venosus ASDs and associated anomalous pulmonary veins are often missed on TTE. In our patient, TEE and CTA assisted in the detection of anomalous pulmonary venous connection. A multidisciplinary heart team approach helped determine and tailor the best option for surgical correction in our patient's case. Conclusion Sinus venosus defects account for up to 10% of ASDs and can lead to pulmonary hypertension if left uncorrected. TTE remains the first imaging modality in assessing for ASDs, but TEE, RHC, and CTA can assist in comprehensive diagnosis and planning for procedural correction. Surgical closure in patients less than 25 years old without pulmonary hypertension is associated with low postoperative mortality, and a multidisciplinary approach can help ensure the most optimal method of surgical correction. (Figure Presented).