Double outlet right ventricle

Sergey Zaets, MD(*); Mark Ruzmetov, MD; Bagrat Alekyan, MD; Irina Lepikhova, MD; Mikhail Chiaureli, MD and Vladimir Podzolkov, MD. Department of Congenital Heart Disease, Bakulev Scientific Center for Cardiovascular Surgery, Moscow, Russia and Section of Cardiothoracic Surgery, Indiana University School of Medicine, Indianapolis, IN.

PURPOSE: The creation of a fenestration has already become a generally acknowledged procedure in high-risk Fontan candidates. However, further tactics in this group of patients remains questionable. The aim of this work was to estimate late functional status, as well as central hemodynamics in fenestrated Fortran patients, and elaborate rational interventional approach.

METHODS: One-hundred-fifteen patients with tricuspid atresia (n=46), double-inlet left ventricle (n=29), hypoplastie right ventricle (n= 15), atrioventricular discordance (n=9), double-outlet right ventricle (n=8), and other complex congenital heart defects (n=8) underwent Fontan operation between 1989 and December 1999. Fenestration was performed selectively in 48 patients (42%). 40 (83%) of them had 1-4 (mean, 1.4 [+ or -] 0.7) various risk factors. Mean baffle fenestration was 3.4 mm (range, 2.5-5.0 mm). 23 fenestrated patients were examined 1-5 (2.0 [+ or -] 1.0) years after surgery. Examination included echocardiography, determination of rest and exercise of arterial blood oxygen saturation (SaO2), as well as cardiac catheterization.

RESULTS: Among 23 patients 18 (78%) were in New York Heart Association Class I or II, and 5 (22%) - in Class III. Spontaneous closure of the fenestration was noted in 5 (22%) patients only (group I). The latter had normal SaO2, which averaged 93.3 [+ or -] 1.5%, and remained stable in exercise. Postoperative systemic venous pressure ranged from 14 to 21 mmHg (mean, 16.7 [+ or -] 3.0 mmHg). In one case, when elevated systemic venous ,pressure accompanied by the signs of significant heart failure transcatheter fenestration was performed. Rest SaO2 in 18 (78%) patients with functioning fenestration (group II) averaged 90.1 [+ or -] 3.2 mmHg, and was significantly less, compared with the first group (p [is less than] 0.05). Besides, SaO2 in group II dropped to 84.6 [+ or -] 3.2% after exercise (p [is less than] 0.01). All patients with existing fenestration had favourable hemodynaics for transcatheter fenestration closure, and their mean systemic venous pressure averaged 15.2 [+ or -] 3.9 mmHg. No embolizations (early or late), clinical hemolysis, thromboembolic events, deteriorations occurred among patients during 1- to 11-month follow-up periods.

CONCLUSION: The majority of Fontan patients with fenestration need its subsequent fenestration transcatheter closure. If a spontaneous closure of fenestration leads to an increase of venous pressure and deterioration of a patient, repeated transcatheter fenestration is indicated.

CLINICAL IMPLICATIONS: The aim of this work was to estimate late functional status, as well as central hemodynamics in fenestrated Fontan patients, and elaborate rational interventional approach. Fenestration was performed selectively in 48 our patients. The majority of Fontan patients with fenestration need its subsequent fenestration transcatheter closure.

COPYRIGHT 2000 American College of Chest Physicians
COPYRIGHT 2001 Gale Group