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Importance of absent ductus arteriosus in tetralogy of Fallot with absent pulmonary valve syndrome.

ABSTRACT: Tetralogy of Fallot without pulmonary valve syndrome is almost always associated with an absent ductus arteriosus. Patients with right aortic arch and retroesophageal left subclavian artery have a vascular ring if the left ductus arteriosus or its remnant and the Kommerell diverticulum are present. We report the cases of 2 infants in whom the role of an absent ductus arteriosus or its remnant is noteworthy. Both patients had a combination of tetralogy of Fallot with absent pulmonary valve syndrome and right aortic arch with retroesophageal left subclavian artery without a vascular ring. The absence of the ductus arteriosus has a role in the pathogenesis of tetralogy of Fallot with absent pulmonary valve syndrome. The absence of a ductus arteriosus in the right aortic arch with retroesophageal left subclavian artery precludes a vascular ring.

SUBMITTER: Qureshi MY

PROVIDER: S-EPMC4251345 | biostudies-literature | 2014 Dec

REPOSITORIES: biostudies-literature

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Importance of absent ductus arteriosus in tetralogy of Fallot with absent pulmonary valve syndrome.

Qureshi Muhammad Yasir MY Burkhart Harold M HM Julsrud Paul P Cetta Frank F

Texas Heart Institute journal 20141201 6

Tetralogy of Fallot without pulmonary valve syndrome is almost always associated with an absent ductus arteriosus. Patients with right aortic arch and retroesophageal left subclavian artery have a vascular ring if the left ductus arteriosus or its remnant and the Kommerell diverticulum are present. We report the cases of 2 infants in whom the role of an absent ductus arteriosus or its remnant is noteworthy. Both patients had a combination of tetralogy of Fallot with absent pulmonary valve syndro ...[more]

PMID: 25593538

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Project description:Background Tetralogy of Fallot repair results in late occurrence of pulmonary regurgitation, which requires pulmonary valve replacement in a large proportion of patients. Both homografts and bioprostheses are used for pulmonary valve replacement as uncertainty remains on which prosthesis should be considered superior. We performed a long-term imaging and clinical comparison between these 2 strategies. Methods and Results We compared echocardiographic and clinical follow-up data of 209 patients with previous tetralogy of Fallot repair who underwent pulmonary valve replacement with homograft (n=75) or bioprosthesis (n=134) between 1995 and 2018 at a tertiary hospital. The primary end point was the composite of pulmonary valve replacement reintervention and structural valve deterioration, defined as a transpulmonary pressure decrease ?50 mm Hg or pulmonary regurgitation degree of ?2. Mixed linear model and Cox regression model were used for comparisons. Echocardiographic follow-up duration was longer in the homograft group (8 [interquartile range, 4-12] versus 4 [interquartile range, 3-6] years; P<0.001). At the latest echocardiographic follow-up, homografts showed a significantly lower transpulmonary systolic pressure decrease (16 [interquartile range, 12-25] mm Hg) when compared with bioprostheses (28 [interquartile range, 18-41] mm Hg; mixed model P<0.001) and a similar degree of pulmonary regurgitation (degree 0-4) (1 [interquartile range, 0-2] versus 2 [interquartile range, 0-2]; mixed model P=0.19). At 9 years, freedom from structural valve deterioration and reintervention was 81.6% (95% CI, 71.5%-91.6%) versus 43.4% (95% CI, 23.6%-63.2%) in the homograft and bioprosthesis groups, respectively (adjusted hazard ratio, 0.27; 95% CI, 0.13-0.55; P<0.001). Conclusions When compared with bioprostheses, pulmonary homografts were associated lower transvalvular gradient during follow-up and were associated with a significantly lower risk of reintervention or structural valve degeneration.

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Importance of absent ductus arteriosus in tetralogy of Fallot with absent pulmonary valve syndrome.

Publications

Importance of absent ductus arteriosus in tetralogy of Fallot with absent pulmonary valve syndrome.

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