Ann Thorac Surg 2006;81:739-740
© 2006 The Society of Thoracic Surgeons
Case report
Total Anomalous Pulmonary Venous Connection With Intact Interatrial Septum
Amit Mishra, MCh, FPCS,
Rajnish Garg, MD,
Raghunath CN, MD,
Sunita Maheswari, ABP, ABPC,
Rajesh Sharma, MCh
*
Division of Pediatric Cardiac Surgery, Department of Cardiothoracic and Vascular Surgery, Narayana Hrudayalaya, Bangalore, India
Accepted for publication December 17, 2004.
* Address correspondence to Dr Sharma, Division of Pediatric Cardiac Surgery, Department of Cardiothoracic & Vascular Surgery, Narayana Hrudayalaya, No. 258/A, Bommasandra Industrial Area, Anekal Taluk, 560 099 Bangalore, India (Email: rsharmacvs{at}hotmail.com).
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Abstract
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The presence of an interatrial communication is considered an integral part of the diagnosis of total anomalous pulmonary venous connection and is believed to be essential for survival. We report a 9-month-old infant with obstructed supracardiac total anomalous pulmonary venous connection without an interatrial communication.
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Introduction
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Restrictive atrial septal defect in total anomalous pulmonary venous connection (TAPVC) is a well-known cause of obstruction to pulmonary return. The extreme situation of an intact interatrial septum thus far was believed to have been incompatible with life.
A 9-month-old male child was referred to our hospital with a history of recurrent cough and bluish discoloration since 1 month of age. On examination the child was thinly built, weighing 4 kg. There was mild cyanosis. Auscultation revealed conducted sounds in both lung fields. There was mild hepatomegaly. Chest skiagram showed cardiomegaly with pulmonary venous congestion. An electrocardiogram showed right axis deviation and right ventricle hypertrophy.
Transthoracic echocardiography revealed situs solitus with atrioventricular and ventriculo-arterial concordance. There was obstructed supracardiac total anomalous pulmonary venous connection with a vertical vein draining to the right superior vena cava. The right atrium and right ventricle were dilated with poor right ventricular function. The left ventricle was compressed and pancaked. The mitral valve annulus was 9 mm (normal for body surface area, 11 mm), and the tricuspid valve annulus was 16 mm. There was a large patent ductus arteriosus (PDA) with bi-directional shunting and severe pulmonary artery hypertension. No right to left shunting could be demonstrated at the atrial level.
The child was prepared for surgery. Echocardiographic findings of a supra cardiac type of TAPVC with a vertical vein draining to the superior vena cava right atrial junction were confirmed. The PDA was looped. Trial occlusion of the PDA resulted in complete loss of pressure in both the ascending aorta and femoral arterial lines with bradycardia. Absence of an atrial septal defect with right ventricle dependent systemic circulation through a PDA and nonfunctional left ventricle (LV) was postulated. A transesophageal echocardiogram was then conducted in the operating room to confirm the findings. The transesophageal echocardiogram showed a small left atrium and LV with trivial flow across the mitral valve. There was no flow across the aortic valve with leaflets not opening in systole.
It was therefore concluded that the LV was not contributing to systemic output and the right ventricle was supporting both pulmonary and systemic circulations. Treatment options considered were as follows: (1) treatment on lines of a single ventricle type of repair (ie, pulmonary veins to left atrium connection with excision of the interatrial septum along with a Stansel type of aortopulmonary connection leaving the distal pulmonary arteries dependent on a systemic to pulmonary artery shunt); and (2) TAPVC repair with postoperative extracorporeal membrane oxygenation if the LV did not sustain systemic output. The second option was chosen, because it would lead to a biventricular repair with a better chance of a good long-term result.
Under cardiopulmonary bypass, with aorto bi-caval cannulation and moderate hypothermia, the PDA was ligated. The right atrium was opened, and absence of interatrial communication was confirmed. The interatrial septum was incised. The pulmonary venous chamber was opened transversely and anastomosed to the posterior wall of the left atrium. The atrial septum was reconstructed using a Dacron patch. The patient was taken off bypass with dobutamine and milrinone infusions, with acceptable hemodynamics and left atrium pressures of 10 mm Hg. Postoperative transesophageal echocardiogram showed a fair sized left atrium and LV with normal flow across the mitral and aortic valves with good biventricular function.
The postoperative course was characterized by episodic rise in the left atrial pressure to 20 to 30 mm Hg over the next 10 to 15 days. During this period, extracorporeal membrane oxygenation was always a possibility, but in view of a good systemic arterial pressure, the left atrial pressure came down and stabilized at 13 to 16 mm Hg. A tracheostomy was performed on postoperative day 20 in view of the long-term intubation. The patient was gradually weaned off the ventilator, and the tracheostomy tube was subsequently removed. Transthoracic echocardiography at discharge showed no pulmonary venous obstruction with drainage into the left atrium, intact interatrial septum, and normal biventricular function. Pulmonary arterial pressure had dropped to 30% of systemic pressure 1 month after discharge.
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Comment
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An intracardiac right-to-left shunt usually at the atrial level is obligatory for survival in a patient with TAPVC [1]. Rarely a ventricular septal defect without an atrial septal defect has been reported to sustain life in TAPVC [2]. Literature search revealed a single case of postmortem detection of the absence of an atrial septal defect in a case of infradiaphragmatic TAPVC with PDA, which did not survive beyond 6 weeks [3].
In our patient, the PDA was large and resulted in both pulmonary and systemic circulations being right ventricle dependent. We believe this is the first reported case of the absence of an atrial septal defect in presence of obstructed TAPVC with survival into late infancy. It is also notable that the LV could be trained after incorporation in the systemic circulation after a prolonged recovery period with return to completely normal hemodynamics on follow up.
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References
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- Kouchoukos NT, Blackstone EH, Doty DB, Hanley FL, Karp RB, Boyes KB. Cardiac surgery. Churchill: Livingstone; 2003.
- Delisle G, Ando M, Calder AL, Zuberbuhler JR, Rochenmacher S, Alday LE. Total anomalous pulmonary venous connectionreport of 93 autopsied cases with emphasis on diagnostic and surgical consideration. Am Heart J 1976;91:99.[Medline]
- Hastreiter AR, Paul MH, Molthan ME, Miller RA. Total anomalous pulmonary venous correction with severe pulmonary venous obstruction Circulation 1962;25:916-917.[Abstract/Free Full Text]
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Abstract
Introduction
