Ann Thorac Surg 2010;89:635-637. doi:10.1016/j.athoracsur.2009.07.068
© 2010 The Society of Thoracic Surgeons
Case Reports
Successful Staged Fontan Completion for Truncus Arteriosus With Hypoplastic Left Ventricle
Takaya Hoashi, MD,
Edward L. Bove, MD,
Richard G. Ohye, MD*
Division of Pediatric Cardiovascular Surgery, Department of Surgery, University of Michigan Medical School, Ann Arbor, Michigan
Accepted for publication July 21, 2009.
* Address correspondence to Dr Ohye, 5144 Cardiovascular Center/SPC 5864 1500 E Medical Center Dr, Ann Arbor, MI 48109-5864 (Email: ohye{at}umich.edu).
 |
Abstract
|
|---|
We report a case of truncus arteriosus type II with a large outlet ventricular septal defect and a hypoplastic left ventricle. The first-stage palliation was the removal of both branch pulmonary arteries from the ascending aorta, patch augmentation of the pulmonary artery confluence, and modified Blalock-Taussig shunt at age 1 month. The second stage palliation was a bidirectional Glenn at 6 months. The final stage was an extracardiac Fontan at age 3 years. Eight years later, the patient is doing well, with an unobstructed Fontan pathway and mild-to-moderate truncal valve insufficiency.
|
Introduction
|
|---|
Truncus arteriosus communis (TAC) combined with a functionally single ventricle is a rare combination. Successful surgical intervention in this unique anatomic group has only been reported in 4 patients [1–4]. In this report we describe a patient with TAC and a functionally single right ventricle who has been staged to a Fontan-type operation.
A 2-week-old boy with a diagnosis of suspected congenital heart disease was transferred to our hospital for further evaluation. Echocardiography showed a solitary, quadricuspid semiluminar valve with mild-to-moderate insufficiency. Branch pulmonary arteries (PAs) originated separately from the posterior of the single arterial trunk. There was a large outlet ventricular septal defect (VSD). The left ventricle (LV) was hypoplastic with a small mitral valve annulus of 8.6 mm (Z value, –4). The right pulmonary veins drained to the superior vena cava. Biventricular function was normal. There was a right aortic arch and a large atrial septal defect (ASD).
A preoperative cineangiogram showed a solitary semiluminar valve (truncal valve) overriding both ventricles with moderate insufficiency. The presence of the large outlet VSD resulted in an unobstructed LV outflow tract from the LV to the truncal valve. The LV was thought to be too hypoplastic to tolerate supporting the systemic circulation (Fig 1).
View larger version (75K):
[in this window]
[in a new window]
|
Fig 1. Cardiac cine angiograms show the common atrial trunk with separately originating (A) branch pulmonary arteries and (B) hypoplastic left ventricle.
|
|
The patient underwent a placement of a central 4-mm shunt, atrial septectomy, division of the PAs from the common atrial trunk, and homograft patch augmentation of the posterior truncus where the PAs were removed. The patient tolerated the procedure well and was discharged on postoperative day 6 with an oxygen saturation of 86% on room air.
An elective bidirectional Glenn procedure was performed as the second-stage palliation when the patient was 5 months old. The superior vena cava was divided just above the entrance of the anomalous right pulmonary veins, which entered at the junction of the superior vena cava and right atrium. The patient's postoperative course was significant for asymmetric upper extremity and trunk edema, which slowly resolved with diuretic therapy over the ensuing days. A perfusion scan to rule out PA occlusion revealed 49% flow to the right and 51% to the left.
An extracardiac Fontan operation was attempted at age 1.5 year with a 16-mm stretch polytetrafluoroethylene graft. Immediately postoperatively, however, low cardiac output developed secondary to junctional tachycardia. This was treated both with medications and with cooling, with some improvement. By postoperative day 2, however, he persisted in a low cardiac output requiring increasing pressor support. The compression of the right upper pulmonary vein was also suspected due to evidence from a Doppler echocardiogram and radiographic changes in the right upper lung field. Because of this issue and his borderline hemodynamic status, the decision was made to take down the Fontan.
A redo extracardiac Fontan procedure was successfully performed with an 18-mm stretch polytetrafluoroethylene graft when he was 3 years old. Eight years later, he is doing well, with an unobstructed Fontan pathway and mild-to-moderate truncal valve insufficiency.
|
Comment
|
|---|
Few TAC cases combined with a hypoplastic LV, with or without VSD have been reported [2, 5–7], but none of these reported patients reached to a Fontan-type operation. The addition of patients with TAC with right ventricular hypoplasia results in finding reports of only 4 patients undergoing successful surgical intervention (Table 1). This report describes a patient with the rare combination of TAC and a functionally single right ventricle who successfully underwent a Fontan-type operation.
View this table:
[in this window]
[in a new window]
|
Table 1 Summary of Truncus Arteriosus With Functional Single Ventricle That Received Successful Surgical Treatment
|
|
|
References
|
|---|
- Shaddy RE, McGough EC. Successful diagnosis and surgical treatment of single ventricle, truncus arteriosus Ann Thorac Surg 1989;48:298-300.[Abstract/Free Full Text]
- Michelfelder EC, Zales VR, Jacobs ML. Surgical palliation of truncus arteriosus with mitral atresia and hypoplastic left ventricle Ann Thorac Surg 1998;65:260-263.[Abstract/Free Full Text]
- Malec E, Mroczek T, Pajak J, Kordon Z. Operative treatment of truncus arteriosus communis coexisting with tricuspid atresia Ann Thorac Surg 2000;69:278-280.[Abstract/Free Full Text]
- Numata S, Uemura H, Kagisaki K, Yagihara T. Tricuspid atresia with common arterial trunk: successful treatment using Fontan procedure Interact Cardiovasc Thorac Surg 2004;3:161-162.[Abstract/Free Full Text]
- Alves PM, Ferrari AH. Common arterial trunk arising exclusively from the right ventricle with hypoplastic left ventricle and intact ventricular septum Int J Cardiol 1987;16:99-102.[Medline]
- Rice MJ, Andrilenas K, Reller, MD, McDonald RW. Truncus arteriosus associated with mitral atresia and a hypoplastic left ventricle Pediatr Cardiol 1991;12:128-130.[Medline]
- Murdison KA, McLean DA, Carpenter B, Duncan WJ. Truncus arteriosus communis associated with mitral valve and left ventricular hypoplasia without ventricular septal defect: unique combination Pediatr Cardiol 1996;17:322-326.[Medline]
This article has been cited by other articles:
|
|
|
|
 
M. L. Jacobs and K. K. Pourmoghadam
Fontan Procedure for Truncus Arteriosus With Functionally Univentricular Heart
Ann. Thorac. Surg.,
November 1, 2010;
90(5):
1746 - 1746.
[Full Text]
[PDF]
|
|
|
Top
Abstract
Introduction
