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J Thorac Cardiovasc Surg 2003;125:731-733
© 2003 The American Association for Thoracic Surgery
Brief Communications |
From the Department of Cardiothoracic Surgery, Kanagawa Children's Medical Center, Yokohama, Japan.
Received for publication April 21, 2002. Accepted for publication May 16, 2002. Address for reprints: Takashi Miyamoto MD, Department of Cardiovascular Surgery, Kanagawa Children's Medical Center, 2-138-4 Mutsukawa, Minami-ku, Yokohama, Kanagawa, 232-8555, Japan (E-mail: miyamotot{at}tk3.speed.co.jp).
Patients with single-ventricle physiology in association with asplenia syndrome are common as candidates for the Fontan operation. A combination of nonconfluent pulmonary artery, bilateral patent ductus arteriosus, and total anomalous pulmonary venous connection (TAPVC), however, is rarely identified. We present here a case of heterotaxia in single ventricle in which a fenestrated total cavopulmonary connection (TCPC) was done successfully.
Clinical summary
The patient was a boy aged 4 years, 5 months who had a diagnosis of asplenia syndrome, heterotaxia syndrome, unbalanced atrioventricular canal defect, trivial common atrioventricular valve regurgitation, pulmonary atresia, TAPVC to the left superior vena cava, bilateral superior vena cava, right isomerism, and direct return of the right hepatic vein to the left-sided atrium. Cardiac catheterization revealed satisfactory mean pulmonary artery pressure (9 mm Hg), and pulmonary vascular resistance was 1.82 units.
We successively performed a right modified Blalock-Taussig shunt and pulmonary reconstruction for bilateral patent ductus arteriosus and pulmonary atresia at 1 month of age (Figure 1), exchange of right modified Blalock-Taussig shunt at the age of 3 years, 2 months, and a bilateral bidirectional Glenn shunt at 4 years of age. On completion of the bilateral bidirectional Glenn shunt, bilateral central venous pressure increased more than 30 mm Hg. We performed a pressure study and confirmed pulmonary venous obstruction because there was a significant pressure gradient (13 mm Hg) between the common pulmonary vein and the left-sided atrium. At that time we elected to do a patch augmentation for pulmonary venous obstruction because we wished to leave thedoor open to further intracardiac TCPC. Subsequently, pulmonary arterioplasty combined with repair of TAPVC was performed at 4 years, 1 month of age (Figure 2).
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) was prepared in a long boot-like structure (bottom wide), appending a 4-mm polytetrafluoroethylene fenestration tube.* With the patient under general anesthesia, the heart was exposed through a resternotomy. Cardiac cannulation was done, and pump perfusion was started. The patient was cooled to 28°C. After aortic crossclamping, the right atrium was vertically opened. A conduit of a long boot-like graft was connected to the atrial floor around the ostia of left-sided inferior vena cava and suprahepatic venous trunk. The other end of the conduit was sewn end to side to the left pulmonary artery. The right atriotomy was closed with the lower half of the conduit located in the atrium and another part in the extracardiac space. A fenestration was created between the extracardiac conduit and the pulmonary venous atrium. Finally, two plastic rings were placed the upper portion of the conduit to prevent potential occlusion through compression by surrounding tissue. Weaning from cardiopulmonary bypass was achieved without difficulty (Figure 3).
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Comment
Asplenia syndrome combined with complex single ventricle, which in this case comprised systemic and pulmonary venous connection, pulmonary atresia, and right isomerism patterns, may require specifically planned and timed technical adaptations to obtain an optimal Fontan procedure. 1-3 We believe that growth and maturation of the pulmonary vascular bed, with the subsequent fall in pulmonary vascular resistance, formed the substrate for successful right heart bypass operations.
Bove and colleagues 4 reported the case of a patient with heterotaxia and single-ventricle physiology successively undergoing pulmonary arterial banding at 8 months of age, repair of pulmonary vein stenosis at 15 months of age, and a fenestrated TCPC at 5 years of age. Clinically, management of that child's condition was troublesome because of pulmonary and systemic venous hypertension. In light of the grim prognosis of pulmonary vein stenosis in general, however, the case reports a long-term successful outcome of a TCPC after repair of pulmonary vein stenosis. Our patient underwent repair of pulmonary vein stenosis and also repair of nonconfluent pulmonaryartery-supplied bilateral patent ductus arteriosus. We consider our case to be unprecedented.
Footnotes
*Gore-Tex vascular graft, registered trademark of W. L. Gore & Associates, Inc, Flagstaff, Ariz. 
References
This article has been cited by other articles:
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  T. Nakata, Y. Fujimoto, K. Hirose, M. Osaki, Y. Tosaka, Y. Ide, M. Tachi, and K. Sakamoto Fontan completion in patients with atrial isomerism and separate hepatic venous drainage Eur J Cardiothorac Surg, June 1, 2010; 37(6): 1264 - 1270. [Abstract] [Full Text] [PDF]
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