Total right pulmonary venous return to the inferior vena cava: a rare variant of Scimitar syndrome
Marmorato-Rivera, R.; Banchs-Pieretti, H.; Parrilla-Quinones, F.; Altieri-Nieto, P.; Carrion, E.; Quintana-Rodríguez, C.; Maldonado-Vargas, J.
Boletin de la Asociacion Medica de Puerto Rico 103(4): 46-49
2011
ISSN/ISBN: 0004-4849 PMID: 22737830 Document Number: 702656
Scimitar syndrome is a rare congenital disorder characterized by a partial, or rarely total, unilateral anomalous pulmonary venous return to the inferior vena cava. This anomaly has a distinctive bimodal presentation with the infantile form having a higher incidence, severity and mortality than the adult form, which is usually asymptomatic on diagnosis. We present the case of a 36-year-old-male transferred to our institution due to incidental anomalous vascular findings on contrast enhanced chest tomography while being evaluated for dyspnea on exertion. Patient underwent right and left heart catheterization with evidence of left-to-right shunt secondary to complete anomalous right pulmonary venous return. Patient was referred to a cardiovascular surgeon with expertise in congenital heart disease for definitive surgical correction.