Prognostic value of right ventricular diastolic function indices in hypertrophic cardiomyopathy.

AIMS: Despite the fact that the role of left ventricular diastolic dysfunction in hypertrophic cardiomyopathy (HCM) patients' prognosis has been established, the effect of increased right ventricular (RV) diastolic filling pressures still remains unclear. The purpose of this study was to determine the prognostic significance of transthoracic echocardiographic indices of RV diastolic function (tricuspid inflow and tricuspid annulus tissue Doppler imaging) in HCM patients. METHODS AND RESULTS: We followed up 386 patients diagnosed with HCM (aged 49.3 ± 17.2 years; 65% male) for a median period of 67 months (interquartile range 26-189 months). Primary endpoints were considered mortality due to heart failure (HF) (13 patients) and total cardiovascular (TC) mortality [HF, sudden cardiac death and its equivalents (35 patients)]. Patients presenting with an increased RV E/E(r) ratio (ratio of tricuspid in flow E wave to E(r) wave obtained by tissue Doppler imaging at the lateral tricuspid annulus) had a 1.6 times greater risk for HF mortality [hazard ratio (HR): 1.6, 95% confidence interval (CI): 1.1-2.4, P = 0.03] while patients with shortened tricuspid E wave deceleration time (DTE) had a 1.1 greater risk for SCD (HR: 1.1, 95% CI: 1.01-1.2, P = 0.03). Following ROC analysis, the optimal RV indices' cut-off values for the recognition of our study endpoints were assessed [E/E(r) = 6.88, sensitivity 75%, specificity 77.4%, area under curve (AUC) 0.847, P = 0.017 for HF mortality and DTE < 239 ms, sensitivity 62.5%, specificity 56.7%, AUC 0.642, P = 0.05 for TC mortality]. CONCLUSION: The establishment of RV restrictive physiology appears to have significant predictive value in HCM, regardless of the presence of other detrimental risk factors.

Subaortic and midventricular obstructive hypertrophic cardiomyopathy with extreme segmental hypertrophy.

BACKGROUND: Subaortic and midventricular hypertrophic cardiomyopathy in a patient with extreme segmental hypertrophy exceeding the usual maximum wall thickness reported in the literature is a rare phenomenon. CASE PRESENTATION: A 19-year-old man with recently diagnosed hypertrophic cardiomyopathy (HCM) was referred for sudden death risk assessment. The patient had mild exertional dyspnea (New York Heart Association functional class II), but without syncope or chest pain. There was no family history of HCM or sudden death. A two dimensional echocardiogram revealed an asymmetric type of LV hypertrophy; anterior ventricular septum = 49 mm; posterior ventricular septum = 20 mm; anterolateral free wall = 12 mm; and posterior free wall = 6 mm. The patient had 2 types of obstruction; a LV outflow obstruction due to systolic anterior motion of both mitral leaflets (Doppler-estimated 38 mm Hg gradient at rest); and a midventricular obstruction (Doppler-estimated 43 mm Hg gradient), but without apical aneurysm or dyskinesia. The patient had a normal blood pressure response on exercise test and no episodes of non-sustained ventricular tachycardia in 24-h ECG recording. Cardiac MRI showed a gross late enhancement at the hypertrophied septum. Based on the extreme degree of LV hypertrophy and the myocardial hyperenhancement, an implantation of a cardioverter-defibrillator was recommended prophylactically for primary prevention of sudden death. CONCLUSION: Midventricular HCM is an infrequent phenotype, but may be associated with an apical aneurysm and progression to systolic dysfunction (end-stage HCM).

Left ventricular Doppler characteristics in first-degree relatives of patients with hypertrophic cardiomyopathy.

Left ventricular (LV) diastolic function may be affected early in patients with hypertrophic cardiomyopathy (HCM), regardless of the phenotypic expression of the disease. The aim of the present study was to detect whether LV diastolic performance, evaluated by conventional Doppler echocardiography, is impaired in first-degree relatives of patients with phenotypically expressed HCM, who had no clinical, electrocardiographic, or echocardiographic signs of the disease. Twenty-two young adults having the previously described characteristics comprised the study population and 22 sex- and age-matched healthy individuals served as controls. The 2 groups were compared according to several echocardiographic parameters and the following diastolic function indices: peak velocity of E wave, representing early filling; peak velocity of A wave, representing late filling; ratio of peak early to peak late velocity (E/A); deceleration time of E wave; and LV isovolumic relaxation time. Slower deceleration time of transmitral early filling in first-degree relatives of patients with HCM (192 +/- 31 vs 149 +/- 31 msec, p < 0.001) was the only variable that significantly differentiated the 2 groups. This study shows that in healthy persons with a family history of HCM, Doppler-derived mitral filling pattern shifted toward that observed in HCM and the slower deceleration time may serve as an early sign of disease development.

Doppler echocardiographic evaluation of right ventricular diastolic function in patients with mitral regurgitation.

BACKGROUND AND AIM OF THE STUDY: The effect of left ventricular (LV) pressure overload on right ventricular (RV) diastolic function has been extensively studied. In contrast, no data are available concerning the influence of LV volume overload on RV diastolic function. Accordingly, RV diastolic function was studied in patients with mitral regurgitation (MR) using Doppler echocardiography. METHODS: RV diastolic indices were calculated, using pulsed Doppler echocardiography, in 30 patients (mean age 56.87 +/- 8.58 years) with severe MR, and in 30 healthy control subjects (mean age 56.67 +/- 8.52 years). RESULTS: Compared with controls, MR patients had a significantly lower RV E/A ratio (0.85 +/- 0.12 versus 1.21 +/- 0.16, p <0.001), a significantly prolonged RV isovolumic relaxation time (70 +/- 20 versus 30 +/- 10 ms, p <0.001), a significantly prolonged deceleration time of the transtricuspid E wave (210 +/- 20 versus 140 +/- 10 ms, p <0.001), and a significantly greater right atrial filling fraction (38.58 +/- 4.59 versus 32.58 +/- 3.14%, p <0.001). There was no statistically significant correlation between RV diastolic indices and LV mass index and interventricular septum thickness. CONCLUSION: RV diastolic function in patients with MR is impaired, reflecting prolonged relaxation and redistribution of RV filling into late diastole. Ventricular interdependence constitutes the most likely mechanism of this action.

Right ventricular diastolic function in patients with chronic aortic regurgitation.

Right ventricular diastolic function was evaluated using Doppler echocardiography in 40 patients with aortic regurgitation. Abnormal right ventricular relaxation and filling were the main findings of our study.