Intracardiac relation of extrahepatic persistent right umbilical vein
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Intracardiac relation of extrahepatic persistent right umbilical vein Sowmya Venkata Ramanan 1
&
Sameer Mohammed 1 & Baiju Sasi Dharan 1
Received: 13 September 2020 / Revised: 11 October 2020 / Accepted: 13 October 2020 # Indian Association of Cardiovascular-Thoracic Surgeons 2020
Abstract Persistent extrahepatic right umbilical vein without ductus venosus is a rare anomaly of systemic venous drainage and is reported to be associated with cardiac anomalies. We report the case of an 8-year-old boy diagnosed to have an ostium secundum atrial septal defect, in whom an abnormal vascular channel and its opening in the right atrium in close relationship to the inferior vena cava and coronary sinus opening was identified at operation. Post-operative evaluation of this vascular channel was diagnosed to be an extrahepatic persistent right umbilical vein. Keywords Congenital heart disease . Extrahepatic persistent umbilical vein . Persistent umbilical vein
Introduction The incidence of persistent right umbilical vein (PRUV) ranges from 0.19 to 0.46% [1]. The presence of PRUV is considered a marker for heart disease. In such cases, fetal echocardiography is recommended for identification of associated cardiovascular anomalies such as transposition of great arteries with pulmonary stenosis or tetralogy of Fallot [2, 3]. It has two variants: the intrahepatic (type 1, IH-PRUV) and an extrahepatic variant (type 2, EH-PRUV). In type 2, the umbilical vein connects directly to the right atrium or infracardiac portion of the inferior vena cava (IVC) without a ductus venosus (DV) [2]. Although the possible course of termination of PRUV to the right atrium is reported [3], we present the intracardiac relationship of type 2 PRUV: an unreported anatomical finding in the literature.
left to right shunt, was referred for surgical closure. There was also a 14-mm displacement of the septal leaflet of the tricuspid valve with adequate right ventricular volume and absent tricuspid regurgitation, with no hemodynamic consequences. During surgery, upon pericardiotomy, an aberrant vascular channel was seen entering the right atrium medial to the IVC (Fig. 1). The course of this channel was dissected below the diaphragm, the peritoneum was opened, and the patent course of the vascular channel in the falciform ligament of the liver identified. A diagnosis of EH-PRUV was made. On opening the right atrium, the vascular channel/EH-PRUV was found to
Case report An 8-year-old asymptomatic boy, incidentally diagnosed to have ostium secundum atrial septal defect (ASD) with a
* Sowmya Venkata Ramanan [email protected] 1
Department of Cardiovascular and Thoracic Surgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Medical College Campus, Thiruvananthapuram, Kerala 695011, India
Fig. 1 An aberrant vascular channel seen entering the right atrium medial to the inferior vena cava (yellow looped structure). Yellow rhombus pointing to PRUV
Indian J Thorac Cardiovasc Surg
Post-operatively, a contrast-enhanced compute
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