SESSION TITLE: Pulmonary Vascular Disease Case Report Posters
SESSION TYPE: Affiliate Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: Scimitar syndrome is characterized by an anomalous venous return with the characteristic chest roentgenogram (CXR) appearance of the anomalous vein draining into the inferior vena cava (IVC). This appears as a curvilinear opacity paralleling the right border of the heart resembling a curved sword or "Scimitar".
CASE PRESENTATION: A 27 year old white woman with a reported history of dextrocardia was admitted after a drug overdose. Examination demonstrated an obtunded woman with tachycardia and right sided heart sounds. She was intubated upon hospitalization due to decreased level of consciousness. Her CXR revealed a right sided heart image with two curvilinear opacities in the retrocardiac area. Computed chest angiogram demonstrated that these opacities join to represent an anomalous vein draining into IVC. Additionally, there was aberrant systemic arterial supply to the right lower lobe, arising directly from the abdominal aorta. Other findings included cardiac dextroposition, hypoplastic right pulmonary artery, hypoplasia of the right lung and abnormal right lung lobation with a hyparterial right bronchus (left sided bronchial isomerism).
DISCUSSION: The patient was eventually diagnosed with Scimitar syndrome. This syndrome affects 1-3 in 100,000 live births, although it is believed that the true incidence may be higher as nearly half of the patients remain asymptomatic with some being misdiagnosed as dextrocadia, such as in our case. The need to correctly identify these patients is of paramount importance because some can develop severe pulmonary hypertension and right ventricular failure. Others may develop recurrent chest infections in the sequestered lung segments. Early identification with close echocardiographic and radiological monitoring can help identify those who will benefit from surgical interventions.
CONCLUSIONS: - Not all right sided hearts represent dextrocardia. - Patients with heart malpositioning must have a careful review of chest imaging and further confirmation of Scimitar syndrome can be made through echocardiography and heart catheterization. - Patients with Scimitar syndrome should be monitored closely for developing pulmonary hypertension, considering potential surgery to avert right heart failure. - Sequestered lung identification is important when assessing recurrent lung infections.
Reference #1: Dupuis C, Charaf LA, Brevière GM, Abou P, Rémy-Jardin M, Helmius G. The "adult" form of the scimitar syndrome. Am J Cardiol. 1992 Aug 15; 70(4):502-7.
DISCLOSURE: The following authors have nothing to disclose: Faisal Siddiqui, Edmundo Rubio, Vishal Patel, Sameh Aziz, Susanti Ie
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