Inferior vena cava return in left atrium: a rare presentation with severe mitral regurgitation

Abstract

ÖZBu yazıda, klinik tabloyu değiştiren şiddetli mitral yetersizlik ile ilişkili interatriyal iletişimli sol atriyumda inferior vena kavanın anormal bağlantısı olan nadir bir olgu sunuldu. Mitral kapak tamiri ve inferior cava akımının yeniden sağ atriyuma yönlendirilmesinin ardından başarılı bir düzeltme sağlandı.Anah tar söz cük ler: Kardiyak septal defekt; mitral kapak yetersizliği; sinüs venozus atriyal septal defekt; sistemik venöz dönüş. ABSTRACTHerein, we present a rare case of abnormal connection of the inferior vena cava to the left atrium with an interatrial communication associated with severe mitral regurgitation which altered clinical presentation. Successful correction was achieved after mitral valve repair and re-routing of the inferior cava flow to the right atrium.Keywords: Cardiac septal defect; mitral valve insufficiency; sinus venosus atrial septal defect; systemic venous return.Inferior vena cava (IVC) draining into the left atrium is a rare anomaly of systemic venous return. [1][2][3] In the literature, several cases with various anatomical associations have been published. [1][2][3][4][5][6] Herein, we report a female case with a Marfanoid morphotype, presenting with an abnormal return of the IVC into the left atrium associated with an interatrial communication and severe mitral regurgitation. CASE REPORTA 16-year-old schoolgirl was referred for surgical treatment of severe mitral regurgitation associated with an interatrial communication. Physical examination revealed a good overall condition with dyspnea New York Heart Association (NYHA) Class II without cyanosis. Blood pressure was 107/65 mmHg, oxygen saturation 96%, weight 42 kg and height 166 cm. The patient suffered from several phenotypic features mimicking Marfan syndrome; however, she did not fulfill the Ghent's criteria for the definite diagnosis of Marfan syndrome. She presented with skeletal malformations including moderate pectus excavatum, arachnodactyly, joint hypermobility, an arm span to height ratio greater than 1.05; and a decrease in visual acuity. The cardiac examination showed a 3/6 systolic ejection murmur at the apex, split and fixed S2. There was no sign of the right heart failure and all findings were non-specific. Electrocardiography showed a regular sinus rhythm at 8/bpm and biatrial hypertrophy. Chest radiography revealed a cardiothoracic ratio of 0.5 with a prominent pulmonary artery segment and increased pulmonary vascular markings.Echocardiography demonstrated a moderately dilated left ventricle with normal systolic functions and dilated right cavities; an aortic root diameter of 20 mm without regurgitation, Grade 3 mitral regurgitation by A2 and P2 prolapse and trivial tricuspid regurgitation. A low-situated interatrial communication was also diagnosed with significant left to right shunting (QP/QS>2). Computed tomography scan demonstrated a large IVC connecting directly to the floor of left atrium (Figure 1).The patient was operated on by median sternotomy under cardiopulmonary bypass and aortic