Specific features and survival of French bulldogs with congenital pulmonic stenosis: a prospective cohort study of 66 cases.

ABSTRACT

INTRODUCTION: The objectives of this study were to characterize the epidemiological, clinical, and echocardiographic features of French bulldogs (FBs) with congenital pulmonic stenosis and document their survival times and risk factors for cardiac death (CD). ANIMALS This study included 66 FBs with congenital pulmonic stenosis. METHODS: Prospective cohort study including a survival analysis to assess time to CD. RESULTS: In most cases (53/66, 80%), at least two obstructive lesions were observed, most commonly valvular and supravalvular (42/66, 64%), with pulmonary trunk hypoplasia in 40/66 (61%) of cases. The median Doppler-derived peak trans-stenotic pressure gradient (ΔP) was very high 170 mmHg (range = 34-291 mmHg), with ΔP ≥ 200 mmHg in 33% of FBs. Among the 51 FBs with an available follow-up and that did not undergo surgical valvuloplasty, 21/51 (41%) died, 67% (14/21) of deaths being CD. The median survival time from diagnosis to CD was 2.8 years (interquartile range = 0.8-4.6 years). Univariate Cox proportional hazard analyses revealed that age (hazard ratio [HR] = 2.3 per 1 year increase; p = 0.02), clinical signs at presentation (HR = 3.7; p = 0.03), ΔP (HR = 1.2 per 10 mmHg increase; p = 0.01), right ventricular dilation (HR = 5.0; p = 0.04), severe tricuspid regurgitation (HR = 7.6; p = 0.001), and right-sided congestive heart failure (HR = 4.8; p = 0.05) were associated with time to CD. After adjustment for age and ΔP, tricuspid regurgitation remained significantly associated with time to CD (HR = 5.1; p = 0.02). CONCLUSIONS: Pulmonic stenosis in FBs is commonly severe and complex, with at least 2 obstructive lesions in most cases, a high incidence of pulmonary trunk hypoplasia and CD, and strong association between prognosis and tricuspid regurgitation severity.