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Influence of fenestration on long-term Fontan survival
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- Ondřej Materna, Vojtěch Illinger, Denisa Jičínská, Karel Koubský, Jan Kovanda, Miroslav Ložek, Petr Tax, Oleg Reich, Václav Chaloupecký, Jan Janoušek
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- Journal:
- Cardiology in the Young / Volume 32 / Issue 7 / July 2022
- Published online by Cambridge University Press:
- 31 August 2021, pp. 1021-1026
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- Article
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Introduction:
Fenestration in the total cavopulmonary connection system may improve the outcome of patients with significant risk factors for Fontan haemodynamics. Our study aims to analyse the difference in long-term survival between non-fenestrated and fenestrated patients.
Methods:All consecutive patients (n = 351) who underwent total cavopulmonary connection between 1992 and 2016 were identified. Six early deaths were excluded resulting in a group of 345 patients. Median (interquartile range,) length of follow-up was 14.4 (7.1–19.7) years. Freedom from the composite endpoint of death, total cavopulmonary connection take-down or indication for a heart transplant was analysed.
Results:Fenestration was absent in 237 patients (68.7%, Group 1), was created and closed later in 79 patients (22.9%, Group 2), and remained open in 29 patients (8.4%, Group 3). Mean survival probability until composite endpoint was 97.1 and 92.9% at 10 and 20 years, respectively. Patients with patent fenestration had worse survival (p < 0.001) as compared to both the non-fenestrated and fenestration closure groups. Despite a similar outcome, exercise capacity was lower in Group 2 than 1 (p = 0.013). In 58 patients with interventional fenestration closure, Nakata index was lower at the time of closure than pre-operatively, and both the pressure in the circuit and oxygen saturation in the aorta increased significantly (p < 0.001).
Conclusions:Patients with persisting risk factors preventing fenestration closure are at higher risk of reaching the composite endpoint. Patients after fenestration closure have the worse functional outcome; their survival is, however, not different from the non-fenestrated group.
Repair of tetralogy of Fallot associated with atrioventricular septal defect
- Tomás̆ Tláskal, Bohumil Huc̆ín, Martin Kostelka, Václav Chaloupecký, Jan Marek, Petr Tax, Jan Janouàek, Vladimír Kuèera, Jaroslav Hruda, Oleg Reich, Jan S̆kovránek
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- Journal:
- Cardiology in the Young / Volume 8 / Issue 1 / January 1998
- Published online by Cambridge University Press:
- 19 August 2008, pp. 105-112
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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.