ABSTRACT
BACKGROUND: The effect of gestational hypertension on left ventricular (LV) function in previously normotensive young women has not been evaluated. METHODS AND RESULTS: A total of 106 gestational hypertensive women (GHW, 32.3 ± 4.2 years) and 93 normotensive pregnant women (NPW, 30.2 ± 4.4 years) were enrolled. Transthoracic echocardiography, including 2-dimensional strain echocardiography, was done and myocardial performance (Tei index), LV mass index (LVMI), and relative wall thickness (RWT) were analyzed. GHW had significantly increased wall thickness (interventricular septum, 9.5 ± 0.9 mm vs. 8.8 ± 1.0 mm, P < 0.001; posterior wall, 9.0 ± 1.1 mm vs. 8.5 ± 1.1 mm, P = 0.007; and RWT, 0.39 ± 0.06 vs. 0.35 ± 0.05, P = 0.02), higher LVMI (95.6 ± 17.3g/m² vs. 86.1 ± 14.5g/m², P = 0.03), longer isovolumetric relaxation time (117.7 ± 18.2 ms vs. 82.3 ± 12.6 ms, P = 0.003), lower E/A ratio (1.00 ± 0.29 vs. 1.27 ± 0.33, P = 0.002), and higher Tei index (0.48 ± 0.23 vs. 0.33 ± 0.13, P = 0.003) compared to NPW. Global longitudinal LV strain, representing LV systolic function, was also significantly reduced in GHW compared with NPW (-17.6 ± 2.95% vs. -21.2 ± 2.14%, P = 0.02). A total of 62% of GHW (n = 66) had abnormal geometry, of whom, 42 (40%) had eccentric hypertrophy (EH). A total of 93% of NPW (n = 86) had normal geometry, and only 7 NPW (7%) had abnormal geometry. CONCLUSIONS: GHW had aggravated diastolic and longitudinal systolic dysfunction. GHW had increased LVMI with the abnormal geometric pattern of EH. The reversibility of these morphological and functional impairments after delivery needs to be clarified.
Subject(s)
Cardiomegaly/etiology , Hypertension, Pregnancy-Induced/pathology , Hypertension, Pregnancy-Induced/physiopathology , Ventricular Function, Left/physiology , Adult , Cardiomegaly/pathology , Diastole , Echocardiography , Echocardiography, Stress , Female , Heart Function Tests , Humans , Hypertension, Pregnancy-Induced/diagnostic imaging , Pregnancy , Systole , Young AdultABSTRACT
Aortic regurgitation is not so rare in patients with Fabry disease. Enzyme replacement therapy has become the standard medical care for Fabry disease in recent years. A 31-year-old man with Fabry disease, treated with recombinant alpha-galactosidase enzyme replacement for 19 months was admitted for evaluation of exertional dyspnea. Cardiac workup revealed left ventricular hypertrophy, increased left ventricular size, and moderate to severe aortic regurgitation. He underwent mechanical valvular replacement and heart biopsy. Histology of his aortic valve showed myxoid degeneration of valve leaflets. His heart muscle revealed extensive hypertrophy with vacuolization and the absence of lamellar bodies. We report a case of Fabry disease with aortic regurgitation in a man who underwent valvular replacement operation during enzyme replacement therapy.
