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Drainage of the Inferior Vena Cava Into the Left Atrium with Atrial Septal Defect and Partial Anomalous Pulmonary Venous Return: Initial and Postoperative Computed Tomography (CT) Findings

AUTHORS

Bum-Sung Choi 1 , Yeon Joo Jeong 2 , Geewon Lee 2 , Seunghwan Song 3 , Min-Jung Cho 4 , Miri Jeong 2 , Ji Won Lee 2 , *

AUTHORS INFORMATION

1 Pusan National University School of Medicine and Medical Research Institute, Pusan National University Yangsan Hospital, Yangsan, Republic of Korea

2 Department of Radiology, Pusan National University School of Medicine and Medical Research Institute, Pusan National University Hospital , Busan, Republic of Korea

3 Department of Thoracic and Cardiovascular Surgery, Pusan National University School of Medicine and Medical Research Institute, Pusan National University Hospital , Busan, Republic of Korea

4 Department of Pediatrics, Pusan National University School of Medicine and Medical Research Institute, Pusan National University Hospital , Busan, Republic of Korea

ARTICLE INFORMATION

Iranian Journal of Radiology: 15 (2); e13878
Published Online: March 10, 2018
Article Type: Case Report
Received: May 23, 2017
Revised: August 17, 2017
Accepted: November 3, 2017
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Abstract

Anomalous drainage of the inferior vena cava into the left atrium is a rare congenital vascular disorder. It can occur with atrial septal defect, anomalous pulmonary venous drainage, and pulmonary arteriovenous fistula. Hereby we describe the computed tomography (CT) findings of a rare combination of drainage of the inferior vena cava associated with atrial septal defect and partial anomalous pulmonary venous return.

CT angiography is useful for early detection of this combined anomaly and for proper surgical planning.

Keywords

Computed Tomography Angiography Atrial Septal Defect Partial Anomalous Pulmonary Venous Return Inferior Vena Cava

Copyright © 2018, Iranian Journal of Radiology. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (http://creativecommons.org/licenses/by-nc/4.0/) which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited
1. Introduction

Anomalous drainage of the inferior vena cava (IVC) into the left atrium is a rare congenital vascular disorder (1). It can occur with atrial septal defect (ASD), anomalous pulmonary venous drainage, and pulmonary arteriovenous fistula (2). This case report describes the computed tomography (CT) findings of a rare combination of drainage of the inferior vena cava associated with atrial septal defect and partial anomalous pulmonary venous return.

2. Case Presentation

A 7-year-old girl was referred to our hospital for evaluation of palpitations and chest discomfort she had for 3 years. Clinically, there was no auscultatory abnormality. Her blood pressure was 90/60 mmHg and heart rate was 91 beats/min. Oxygen saturation was 98% at rest. An electrocardiogram (ECG) showed normal sinus rhythm and right ventricular hypertrophy. A chest radiograph revealed mild cardiomegaly with clear lung fields. Transthoracic echocardiography (TTE) revealed a large ASD with no posterior rim. The right ventricle was dilated, with a left-to-right shunt. However, there was no evidence of partial anomalous pulmonary venous return (PAPVR) or other vascular anomaly on TTE. To evaluate the combined anomaly, retrospective ECG-gated CT angiography was performed. CT angiography showed S, D, S segmental anatomy with atrioventricular and ventriculoarterial concordance. A large ASD secundum, 3.1 cm × 3.8 cm in size (Figure 1A and B) was noted, and the right superior pulmonary vein was seen draining into the right atrium, suggestive of PAPVR (Figure 1C). Abnormal drainage of the IVC into the left atrium was likewise suspected (Figure 1D). There was no evidence of pulmonary arteriovenous fistula or other cardiac anomalies.

Axial reformatted image (A) and short axis image (B) of retrospective electrocardiogram-gated computed tomography (CT) angiography showed large atrial septal defect (ASD, secundum type) with posterior rim deficiency. The size of the ASD was 3.1 × 3.8 cm. Axial reformatted image (C) showed right superior pulmonary vein (arrow) draining into the right atrium, suggestive of partial anomalous pulmonary venous return (PAPVR). Oblique sagittal reformatted image (D) and short axis image (B) demonstrate the inferior vena cava (IVC) (arrow) draining directly into the left atrium. Maximum intensity projection images of postoperative follow-up CT angiography show successful correction of the ASD, PAPVR, and anomalous drainage of the IVC into the left atrium. Note the superior pulmonary vein (arrow) draining into the left atrium (E) and the IVC (arrow) draining into the right atrium without significant stenosis (F).
Figure 1. Axial reformatted image (A) and short axis image (B) of retrospective electrocardiogram-gated computed tomography (CT) angiography showed large atrial septal defect (ASD, secundum type) with posterior rim deficiency. The size of the ASD was 3.1 × 3.8 cm. Axial reformatted image (C) showed right superior pulmonary vein (arrow) draining into the right atrium, suggestive of partial anomalous pulmonary venous return (PAPVR). Oblique sagittal reformatted image (D) and short axis image (B) demonstrate the inferior vena cava (IVC) (arrow) draining directly into the left atrium. Maximum intensity projection images of postoperative follow-up CT angiography show successful correction of the ASD, PAPVR, and anomalous drainage of the IVC into the left atrium. Note the superior pulmonary vein (arrow) draining into the left atrium (E) and the IVC (arrow) draining into the right atrium without significant stenosis (F).

The operation was conducted via median sternotomy. Cardiopulmonary bypass was established with bicaval cannulation, and the heart was arrested using antegrade cold blood cardioplegia. The right atrium was opened, which revealed a large ASD secundum, the left-ward shift of IVC, and PAPVR. The defect was closed with a patch of fresh autologous pericardium. The right superior pulmonary vein was redirected to the left atrium and the IVC was redirected to the right atrium. The postoperative course was uneventful. Prospective ECG-gated CT angiography performed 2 years after the operation revealed successful correction of ASD, PAPVR, and anomalous drainage of the IVC into the left atrium (Figure 1E and F).

3. Discussion

Anomalous drainage of the IVC into the left atrium is a rare congenital vascular disorder, first described by Gardner in 1955 (1). Only 19 cases with anomalous drainage of the IVC into the left atrium including our case have been reported in the English literature (Table 1). An ASD occurs with the condition in approximately 70% of the reported cases. Anomalous pulmonary venous drainage and pulmonary arteriovenous fistula may also be associated (2). Although two patients had a combined PAPVR and ASD similar to our patient, invasive angiography was used for diagnosis.

Table 1. Reported Cases of Anomalous Drainage of the Inferior Vena Cava to the Left Atrium in English Literature
No.Author, YearGender/AgeClinical SymptomResting O2 SaturationImaging Modality UsedCombined Cardiovascular AnomalyOutcome
1Gardner, 1955 (1)F/32DOE, cyanosis, clubbing, frequent miscarriagesUnknownChest X-rayNoSudden death
2Meadows, 1961 (3)M/37Cyanosis, clubbing, polycythemia, frequent headache82Chest X-ray, Invasive angiographyNoNo treatment
3Gallaher, 1963 (4)M/26Cyanosis, clubbing, murmur94Chest X-ray, Invasive angiographySecudum ASD with prominent Eustachian valveUneventful after operation
4Black, 1964 (5)F/30Cyanosis, clubbing, dyspnea, fatigue, lower limb edema70Chest X-ray, Invasive angiographyPulmonary arteriovenous fistulaDeath 47 days after operation due to sepsis
5Guatam, 1968 (6)F/34Dyspnea, cyanosis, clubbing, murmur89Chest X-ray, Invasive angiographySecundum ASDUneventful after operation
6Kim, 1971 (7)M/12Minimal nail bed cyanosis, murmur93Chest X-ray, Invasive angiographyPDA, secundum ASDSeizure, hypoxia with brain injury after operation; improved
7Singh, 1976 (8)M/6Murmur99Invasive angiographySecundum ASD, sinus venosus ASD. PAPVRUneventful after operation
8Richardson, 1979 (9)Unknown/5 monMurmur92Invasive angiographyPFOUneventful after operation
9Sanchez, 1986 (10)M/6Murmur93Chest X-ray, Invasive angiographyTOF, ASDUneventful after operation
10Sanchez, 1986 (10)F/4Cyanosis, murmur95Chest X-ray, Echocardiography, Invasive angiographyASD, PAPVRUneventful after operation
11Lamb, 1987 (11)M/3Cyanosis, clubbing89Invasive angiographySecundum ASD, TAPVRUneventful after operation
12Mayers, 1987 (12)M/41Cyanosis, clubbing, hypoxemia, one episode of exertion, headache89Echocardiography, MRI, Invasive angiographySecundum ASD, PFOUneventful after operation
13Cabrera, 1994 (13)F/3Respiratory distress, murmur90Chest X-ray, Echocardiography, Invasive angiographyASDUneventful after operation
14Genoni, 1999 (14)F/7DOE, cyanosis, clubbing72Echocardiography, Invasive angiographyNoUneventful after operation
15Burri, 2003 (15)F/54DOE, cyanosis, clubbing, murmur, ankle edema, palpitation, cerebellar deficit81Echocardiography, MRI, Invasive angiographySecundum ASD, coronary sinus draining into left atriumUneventful after operation
16AL-Ammouri, 2010 (16)F/32Dyspnea, polycythemia, fatigue, cyanosis, clubbing, arthritis80Echocardiography, Invasive angiographyASDUneventful after operation
17Aliter, 2011 (2)F/17Cyanosis, clubbing95Echocardiography, CT, Invasive angiographySecundum ASDUneventful after operation
18Basquin, 2013 (17)F/4Dyspnea, cyanosisunknownEchocardiography, CT, Invasive angiographyASDUneventful after operation
19Present caseF/7Palpitation, chest discomfort98Chest X-ray, Echocardiography, CTSecundum ASD, PAPVRUneventful after operation

Abbreviations: ASD, atrial septal defect; DOE, dyspnea on exertion; O2, oxygen; PAPVR, partial anomalous pulmonary venous return; PDA, patent ductus arteriosus; PFO, patent foramen ovale; TAPVR, total anomalous pulmonary venous return; TOF, tetralogy of Fallot.

Anomalous drainage of the IVC into the left atrium results from persistence of the right valve of the sinus venosus. In the embryo, the sinus venosus receives the cardinal, umbilical, and vitelline veins. The sinus venosus communicates with the primitive atrium via an orifice that has a right and left valve. As the heart develops, the IVC formed then drains into the sinus venosus, which normally migrates to the right of the midline. The right valve of the sinus venosus usually atrophies, leaving only the crista terminalis, Eustachian valve, and Thebesian valves (Figure 2). If the right sinus venosus valve fails to regress and fuses with the superior part of the septum secundum, the IVC will drain into the left atrium (2, 5, 18).

Schema of the development of venous valves in 5th week of pregnancy (A) and fetal stage (B) [modified from (18)].
Figure 2. Schema of the development of venous valves in 5th week of pregnancy (A) and fetal stage (B) [modified from (18)].

We herein present a case of a 7-year-old girl with ASD combined with PAPVR and drainage of the IVC into the left atrium. In our case, the TTE failed to demonstrate the anomalous IVC drainage into the left atrium and the PAPVR in a patient with ASD because of the limited echocardiographic window. The diagnosis was eventually established using CT angiography. CT angiography can be useful for early detection of this complex anomaly and for proper surgical planning. To our knowledge, CT images of this rare combination and postoperative follow-up CT have not been reported in English literature.

Acknowledgements
Footnotes
References
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