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Repair of tetralogy of Fallot associated with atrioventricular septal defect

Published online by Cambridge University Press:  19 August 2008

Tomás̆ Tláskal ,
Bohumil Huc̆ín ,
Martin Kostelka ,
Václav Chaloupecký ,
Jan Marek ,
Petr Tax ,
Jan Janouàek ,
Vladimír Kuèera ,
Jaroslav Hruda  and
Oleg Reich
...Show all authors
Tomás̆ Tláskal*
Affiliation:
Kardiocentrum, University Hospital Motol, Prague, Czech Republic
Bohumil Huc̆ín
Affiliation:
Kardiocentrum, University Hospital Motol, Prague, Czech Republic
Martin Kostelka
Affiliation:
Kardiocentrum, University Hospital Motol, Prague, Czech Republic
Václav Chaloupecký
Affiliation:
Kardiocentrum, University Hospital Motol, Prague, Czech Republic
Jan Marek
Affiliation:
Kardiocentrum, University Hospital Motol, Prague, Czech Republic
Petr Tax
Affiliation:
Kardiocentrum, University Hospital Motol, Prague, Czech Republic
Jan Janouàek
Affiliation:
Kardiocentrum, University Hospital Motol, Prague, Czech Republic
Vladimír Kuèera
Affiliation:
Kardiocentrum, University Hospital Motol, Prague, Czech Republic
Jaroslav Hruda
Affiliation:
Kardiocentrum, University Hospital Motol, Prague, Czech Republic
Oleg Reich
Affiliation:
Kardiocentrum, University Hospital Motol, Prague, Czech Republic
Jan S̆kovránek
Affiliation:
Kardiocentrum, University Hospital Motol, Prague, Czech Republic
*
Tomás̆ Tláskal, MD, PhD, Kardiocentrum, University Hospital Motol, V úvalu 84, 150 18 Prague 5, Czech Republic Tel: 42-2/2443 2900; Fax: 42-2/2443 2920
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Abstract

Tetralogy of Fallot, when associated with atrioventricular septal defect permitting shunting at ventricular level, represents a complex cyanotic congenital malformation. Experience with surgical repair is limited, and results vary considerably. Between 1984 and 1996, we repaired 14 consecutive patients with this combination seen in our center. Their ages ranged from 8 months to 21 years (median 7.4 years). Six (42.9%) had Down's syndrome. In eight patients the correct diagnosis was made using echocardiogra phy alone. In the remaining six patients, who had previously-constructed arterial shunts and/or suspected pulmonary arterial stenosis, catheterization and angiocardiography were also performed. The repair consisted of double patch closure of the septal defect, reconstruction of two atrioventricular orifices, and relief of pulmonary stenosis at all levels. In five patients with a hypoplastic pulmonary trunk, a monocusp transannular patch (four patients) or an allograft (one patient) was used for restroration of continuity from the right ventricle to the pulmonary arteries. Patch enlargement of one or both pulmonary arteries was necessary in five patients. One patient (7.1%) died early, and another late. The twelve surviving (85.8%) patients have been followed for 1.2–12.5 years after surgery (median 4.9 years, mean 5.9 ± 3.9 years). During the follow-up, reoperation was necessary for repair of residual ventricular septal defect and pulmonary regurgitation in two patients, and closure of an atrial septal defect and alteration to left atrioventricular valvar regurgitation in one patient. Seven patients are in class I of the New York Heart Association, four in class II, and one in class III. Tetralogy of Fallot associated with atrioventricular septal defect can be corrected with low mortality and good long-term results. Residual lesions, however, have a tendency to progress, especially when seen in combination. After surgery, all patients need long-term close follow-up.

Keywords

Tetralogy of Fallotatrioventricular septal defectheart surgerycomplicationsresidual lesionslong-term results
Type
Original Articles
Information
Cardiology in the Young , Volume 8 , Issue 1 , January 1998 , pp. 105 - 112
Copyright
Copyright © Cambridge University Press 1998

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