Case Study

Total anomalous systemic with partial anomalous pulmonary venous connections

Asian Cardiovascular & Thoracic Annals 21(6) 720–723 ß The Author(s) 2012 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492312463567 aan.sagepub.com

Gopakumar Vallath, Trushar Gajjar and Neelam Desai

Abstract A 9-year-old girl with cyanosis, dyspnea, and grade II clubbing was diagnosed by contrast transthoracic echocardiography and angiocardiography to have an anomalous connection of the venae cavae to the physiologic left atrium with partial anomalous pulmonary venous connection. Successful surgical correction was achieved, and the patient’s recovery was uneventful.

Keywords Total anomalous systemic venous connection, partial anomalous pulmonary venous connection, left superior vena cava, heterotaxia

Introduction Total anomalous systemic venous connection (TASVC) is an exceptional form of congenital heart disease. All systemic venous flow, including the right superior vena cava (RSVC), persistent left superior vena cava (LSVC), inferior vena cava (IVC), and coronary sinus, drains abnormally into the heart.1–8 This disorder is usually associated with an atrioventricular canal, common atrium, atrial septal defect (ASD), ventricular septal defect, and heterotaxia.3–6 Approximately 20 such cases have been reported in the English literature hitherto.6–8 The association of TASVC with partial anomalous pulmonary venous connection (PAPVC) has not been reported. We discuss the successful surgical treatment of a case of TASVC with PAPVC.

Case report A 9-year-old girl presented with history of cyanosis, shortness of breath, and recurrent respiratory tract infection since birth. Physical examination revealed a heart rate of 102 beats  min1, regular rhythm, blood pressure of 140/82 mm  Hg, central cyanosis, grade II clubbing, a pansystolic murmur at the apex, and a loud pulmonary second sound with wide and fixed splitting. Room air oxygen saturation was 78% by pulse oximetry. Laboratory examinations showed hemoglobin of 17.1 g  dL1 with a hematocrit of

50.0%, and other investigations were within normal ranges. Chest radiography revealed cardiomegaly with a cardiothoracic ratio of 60%, pulmonary plethora, bilateral symmetrically long left bronchial anatomy, and a fundus gas shadow on the right side (Figure 1(a)). Electrocardiography showed normal sinus rhythm, left axis deviation with an axis of 100 degrees, inverted P waves in inferior leads with small Q waves in lead V1 and absent Q wave in V6. Abdominal ultrasound showed abdominal situs inversus, liver in the midline, the stomach and single spleen on the right side, hepatic veins draining directly into the left atrium, and a few veins from the left lobe of the liver draining into the IVC which was located to the left of the aorta (Figure 1(b)). Transthoracic echocardiography with a saline-contrast study revealed abdominal situs inversus, thoracic situs solitus, atrioventricular and ventriculoarterial concordance, a large ostium primum and a small ostium secundum ASD, a left-to-right shunt, cleft anterior mitral leaflet, mild to moderate mitral regurgitation, and right ventricular Department of Cardiothoracic and Vascular Surgery, Sri Sathya Sai Institute of Higher Medical Sciences, Prasanthigram, India Corresponding author: Trushar Gajjar, Department of Cardiothoracic and Vascular Surgery, Sri Sathya Sai Institute of Higher Medical Sciences, Prasanthigram 515134, District Anantapur, Andhra Pradesh, India. Email: [email protected]

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Figure 1. (a) Chest radiography showing thoracic situs solitus, pulmonary plethora, bilateral symmetrically long left bronchial anatomy (arrows), and a fundic gas shadow on the right side. (b) Ultrasound of the abdomen showing 2 separate hepatic veins draining into the left atrium (arrows).

Figure 2. Angiogram showing the venous catheter course: (a) right anterior oblique view revealing hemiazygous continuation of the inferior vena cava into the left superior vena cava; (b) left anterior oblique view demonstrating absence of the right superior vena cava, and the innominate vein draining to the left superior vena cava. Bilateral left bronchial anatomy is seen (arrows).

systolic pressure of 42 mm  Hg. The LSVC opened into the left atrium, the IVC was interrupted with hemiazygous continuation of the IVC and good biventricular function. Cardiac catheterization showed the IVC course hemiazygos to the LSVC (Figure 2(a)). Right and left injection indicated absent RSVC and the innominate vein opening into the LSVC (Figure 2(b)). Left ventricular angiography showed a closed perimembranous ventricular septal defect by an aneurysm of the membranous interventricular septum. Mean right atrial pressure was 9 mm  Hg, right ventricular pressure was 64/10 mm  Hg, pulmonary artery pressure was 58/24 mm  Hg with a mean of 40 mm  Hg, femoral artery pressure was 159/ 79 mm  Hg with a mean of 102 mm Hg, pulmonary capillary wedge pressure was 10 mm  Hg, left

ventricular pressure was 155/12 mm  Hg, and left atrial mean pressure was 9 mm  Hg. The patient was referred for surgery with a diagnosis of TASVC with partial atrioventricular canal defect. Pre-anesthetic concerns were polycythemia, altered coagulation profile, arrhythmia, and air embolism. Preoperatively, the patient was well hydrated and 10 mg  kg1 of tranexamic acid was injected before heparinization, on cardiopulmonary bypass (CPB), and after protamine administration, along with 1 unit of warm fresh blood, to prevent bleeding. Drugs inducing arrhythmia were avoided, and care was taken to avoid air embolism by giving all drugs through the chamber sets and avoiding direct bolus injections. A conventional median sternotomy approach was used. The intraoperative findings were left atrial isomerism, absent

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Figure 3. Intraoperative pictures taken from the head end of the patient. (a) Left atrial isomerism and both appendages with left atrial morphology. (b) Left atrial isomerism with cannulated left superior vena cava. (c) Left and right hepatic veins separately opening into the left atrium. (d) Cannulation of the left superior vena cava and both hepatic veins with angled venous cannulae. LSVC: left superior vena cava.

RSVC, LSVC draining to the left atrium, innominate vein draining to the LSVC, hemiazygos continuation of the IVC, and the opening of the hemiazygos was in the posterolateral aspect of the LSVC (Figure 3). Two large hepatic veins opened separately into the left atrium from the inferior aspect of the heart, the right atrium and right ventricle were dilated, there was a large ostium primum ASD with a cleft in the anterior mitral leaflet and moderate mitral regurgitation, a moderate-sized ostium secundum ASD with a septal bridge between the 2 defects, the right upper pulmonary vein drained to the right atrium (RA) and the other pulmonary veins drained to the left atrium. CPB was established by cannulation of the aorta with a 16F cannula, the LSVC with an angled 28F cannula, and the hepatic veins with 2 separate 24F and 20F angled cannulae. Myocardial protection was obtained with antegrade root cardioplegia and topical cooling. The RA was opened and the cleft anterior mitral leaflet was repaired with interrupted polypropylene sutures. The ostium secundum and primum ASD were coalesced into one large defect.

A large pericardial patch was used to partition the atria in such a way that the LSVC and hepatic veins drained to the RA, and all pulmonary veins to the left atrium. Weaning from CPB was carried out in the usual way. Intraoperative transesophageal echocardiography showed no residual shunt, trivial mitral regurgitation, all pulmonary veins draining to the left atrium and hepatic veins and LSVC to the RA. The postoperative course was uneventful, and the patient was well at the 3-month follow-up. Echocardiography showed normal systemic and pulmonary venous drainage, normal right ventricular systolic pressure, trivial mitral regurgitation, and good biventricular function.

Discussion TASVC is a very rare anomaly and anomalies of systemic venous return range from LSVC as the most common to TASVC as the rarest.1 Embryologically, TASVC probably results from the sinus venosus being incorporated into the left atrium rather than the RA.1

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However, in patients with a well-developed interatrial septum, an overgrowth of the valves of the sinus venosus is a possible alternative explanation. TASVC is usually associated with atrioventricular septal defect, common atrium, both primum as well as secundum types of ASD, and heterotaxia.1,6–8 Cyanosis and paradoxical embolic events are often the presenting features.1–8 Two-dimensional transthoracic and transesophageal echocardiography when combined with different bolus injections of contrast from the left and right peripheral arm and femoral veins, provides a reliable method of assessment of systemic venous connections.2,3 Angiocardiography is required only for confirmation of the diagnosis. Such cases require preanaesthetic management of polycythemia and altered coagulation profile along with prevention of arrhythmia and air embolism.7,8 The surgical challenges include the institution of CPB.4–6 Zhang and colleagues6 classified TASVC into 2 types according to the type of vena cava cannulation. In type I, the IVC is not interrupted and conventional CPB can be established by cannulating the superior vena cava and IVC. In type II, the IVC is interrupted, so single cannulation of the superior vena cava and conventional CPB can be achieved. In this case, the hepatic veins can be cannulated with a small cannula after excision of the atrial septum or an intracardiac extractor is used to manage the blood flow. Our case was type II TASVC with interrupted IVC and hemiazygos continuation of the IVC to the LSVC. We have achieved safe CPB with direct cannulation of the LSVC and both the hepatic veins separately, using angled cannulae. Various anastomotic techniques have been described to create the anastomosis between the LSVC and the RSVC and the LSVC to the right atrial appendage before atrial septation.6 In our case, we used an intracardiac baffling technique with a pericardial patch to reroute the LSVC and the hepatic veins to the RA and the pulmonary veins to the left atrium. The postoperative outcome and early follow-up result were satisfactory.

Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Conflicts of interest statement None declared.

References 1. Pearl WR and Spicer MJ. Total anomalous systemic venous return. South Med J 1980; 73: 259–261. 2. Foale R, Bourdillon PD, Somerville J and Rickards A. Anomalous systemic venous return: recognition by twodimensional echocardiography. Eur Heart J 1983; 4: 186–195. 3. Stumper O, Vargas-Barron J, Rijlaarsdam M, Romero A, Roelandt JR, Hess J, et al. Assessment of anomalous systemic and pulmonary venous connections by transoesophageal echocardiography in infants and children. Br Heart J 1991; 66: 411–418. 4. Krayenbuhl CU and Lincoln JC. Total anomalous systemic venous connection, common atrium, and partial atrioventricular canal. A case report of successful surgical correction. J Thorac Cardiovasc Surg 1977; 73: 686–689. 5. Danielson GK, McMullan MH, Kinsley RH and DuShane JW. Successful repair of complete atrioventricular canal associated with dextroversion, common atrium, and total anomalous systemic venous return. J Thorac Cardiovasc Surg 1973; 66: 817–822. 6. Zhang ZW, Duan QJ, Gao Z, Ru W and Ying LY. Total anomalous systemic venous drainage to the left atrium. Ann Thorac Surg 2009; 87: 1599–1601. 7. Moghadam MY, Omrani G, Zanjani KS, Tabae AS. Total anomalous systemic venous return to right-sided atrium with left atrium morphology: a case report. Iran Heart J 2004; 5: 61–64. Available at: http://www.iranmedex.com/ english/articles.asp?issueID¼16510. Accessed September 12, 2012. 8. Simha PP, Patel MD and Jagadeesh AM. Anesthetic implications of total anomalous systemic venous connection to left atrium with left isomerism. Ann Card Anaesth 2012; 15: 134–137.

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Total anomalous systemic with partial anomalous pulmonary venous connections.

A 9-year-old girl with cyanosis, dyspnea, and grade II clubbing was diagnosed by contrast transthoracic echocardiography and angiocardiography to have...
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