Abstract 17043: Severe Pulmonary Hypertension in Young Adults with Coarctation of the Aorta Repaired in Infancy.
Background: Endocardial fibroelastosis is an independent predictor for time-related mortality after neonatal intervention for aortic valve stenosis or coarctation of the aorta (CoA).
Methods: A retrospective review of adults operated on for CoA before 16 years of age was performed. Patients with RV outflow tract obstruction, mitral valve disease (Shone's complex), LV systolic dysfunction, significant shunts or complex heart defects were excluded. According to the tricuspid regurgitation Doppler peak velocity (TRV) patients were classified as PH (TRV > 2.8 m/s) or control (TRV ≤ 2.8 m/s) groups.
Results: Control group included 113 and PH group consisted of 18 patients (14%) with a mean systolic pulmonary pressure (SPP) of 71±36 mmHg (range 43-165). In multivariate analysis using the Cox proportional hazards model, SPP was the only independent predictor for death, transplantation or clinical deterioration during adult life (hazard ratio 1.9 for each 10 mmHg of rise in SPP; 95% confidence interval 1.3 to 2.7; p<0.001). There were not significant differences between groups with regard to age, transCoA pressure gradient, systolic blood pressure, arm-to-leg pressure gradient, LV outflow tract pressure gradient, aortic regurgitation degree and LV dimensions, mass or ejection fraction. However, female prevalence (61% vs. 29%; p=0.017), need for early repair of CoA in neonatal period (31% vs. 7%; p=0.04) and reoperation in childhood due to valvular or subvalvular aortic stenosis (69% vs. 17%; p < 0.001) were significantly greater in PH group. A restrictive LV filling pattern by Doppler was present in 82% of PH group compared with 5% in control group (p<0.001). Cardiac catheterization in PH group showed severe elevation of LV end-diastolic pressure and MRI showed a patchy LV subendocardial late gadolinium enhancement. Pathological examination of the one explanted heart confirmed extensive left ventricular subendocardial fibrosis.
Conclusions: Pulmonary hypertension as a result of severe LV diastolic stiffness is a frequent and serious complication in young adults with complex or severe forms of CoA that required early repair. Late PH might be related with persistent LV subendocardial fibrosis.
- © 2010 by American Heart Association, Inc.