Congenital Anomalies of Inferior Vena Cava, Review of Embryogenesis, Presentation, Associated Congenital Anomalies and Surgical Importance

Background: Congenital variation of inferior vena cava is caused by abnormal embryological development, they are associated with situs inversus, polysplenia and congenital heart diseases. Our aim is to evaluate embryogenesis, presentation, diagnostic modalities and its surgical importance in case of interrupted inferior vena cava with congenital heart disease. Materials and Methods: 14 cases of interrupted inferior vena cava have been reported till now. Out of these cases one case had congenital heart defect and underwent surgical correction. We are going to discuss two cases of interrupted inferior vena cava operated for congenital heart defects in our institute, embryogenesis of defect, the diagnostic modalities used, surgical technique followed and outcome of surgeries and compare them with previously reported cases. First case was a 10 year old boy diagnosed with Gerbode ventricular septal defect, large atrial septal defect, atrial isomerism, persistant left superior vena cava, heterotaxy, polysplenia, duplicated and interrupted inferior vena cava with azygos continuation .Second case was 17 year old boy diagnosed with tetra logy of fallot, interrupted inferior vena cava with azygous continuation, heterotaxy, polysplenia. Results: Out of previously reported cases two cases underwent surgery, one patient underwent partial anomalous pulmonary venous connection correction and the other patient underwent segmentectomy of carcinoma lung. Remaining previously reported cases were either detected incidentally or were detected during post mortem. Both our patients underwent surgical correction, first patient developed pericardial effusion post operatively, both patients discharged in stable condition, and pre-discharge echocardiography showed normal biventricular function, and awaiting follow up after three months. Conclusion: Interrupted inferior vena cava can be diagnosed with 2dimensional echocardiography, angiography and confirmed with computed tomography scan and magnetic resonance imaging Preoperative diagnosis of interrupted inferior vena cava helps in planning for surgeries involving cardiopulmonary bypass and surgeries involving thoracotomy. It also helps in management of associated diseases like deep vein thrombosis. Keywords: Interrupted Inferior Vena cava; Heterotaxy; Azygos; Hemiazygo