Determinants of functional mitral regurgitation severity in patients with ischemic cardiomyopathy versus nonischemic dilated cardiomyopathy.

AIMS: Functional mitral regurgitation (MR) is prevalent among patients with left ventricular (LV) dysfunction and is associated with a poorer prognosis. Our aim was to assess the primary determinants of MR severity in patients with ischemic cardiomyopathy (ICM) and nonischemic dilated cardiomyopathy (DCM). METHODS AND RESULTS: Patients with functional MR secondary to ICM (n = 55) and DCM (n = 48) were prospectively enrolled. Effective regurgitant orifice (ERO) area, global LV remodeling, regional wall-motion abnormalities, and mitral apparatus deformity indices were assessed utilizing conventional and tissue Doppler echocardiography. ICM patients had more severe MR compared with DCM patients despite similar ejection fraction and functional status (ERO = 0.16 ± 0.08 cm(2) vs. ERO = 0.12 ± 0.70 cm(2) , respectively, P = 0.002). Regional myocardial systolic velocities in mid-inferior and mid-lateral wall were negatively correlated with ERO in ICM and DCM patients, respectively. Multivariate analysis identified coaptation height as the only independent determinant of ERO in both groups. In a subset of ICM patients (n = 9) with relatively high ERO despite low coaptation height, a higher prevalence of left bundle branch block was detected (88.9% vs. 46.7%, P = 0.02). CONCLUSIONS: Functional MR severity was chiefly determined by the extent of mitral apparatus deformity, and coaptation height can provide a rapid estimation of MR severity in heart failure patients. Additional contributory mechanisms in ICM patients include depressed myocardial systolic velocities in posteromedial papillary muscle attaching site and evidence of global LV dyssynchrony.

Chronotropic incompetence and its relation to exercise intolerance in hypertrophic cardiomyopathy.

BACKGROUND: Diminished functional capacity is common in hypertrophic cardiomyopathy (HCM), although the underlying mechanisms are complicated. We studied the prevalence of chronotropic incompetence and its relation to exercise intolerance in patients with HCM. METHODS: Cardiopulmonary exercise testing was performed in 68 patients with HCM (age 44.8 ± 14.6 years, 45 males). Chronotropic incompetence was defined by chronotropic index (heart rate reserve)/(220-age-resting heart rate) and exercise capacity was assessed by peak oxygen consumption (peak Vo(2)). RESULTS: Chronotropic incompetence was present in 50% of the patients and was associated with higher NYHA class, history of atrial fibrillation, higher fibrosis burden on cardiac MRI, and treatment with ß-blockers, amiodarone and warfarin. On univariate analysis, male gender, age, NYHA class, maximal wall thickness, left atrial diameter, peak early diastolic myocardial velocity of the lateral mitral annulus, history of atrial fibrillation, presence of left ventricular outflow tract obstruction (LVOTO) at rest, and treatment with beta-blockers were related to peak Vo(2). Peak heart rate during exercise, heart rate reserve, chronotropic index, and peak systolic blood pressure were also related to peak Vo(2). On multivariate analysis male gender, atrial fibrillation, presence of LVOTO and heart rate reserve were independent predictors of exercise capacity (R(2) = 76.7%). A cutoff of 62 bpm for the heart rate reserve showed a negative predictive value of 100% in predicting patients with a peak Vo(2) <80%. CONCLUSIONS: Blunted heart rate response to exercise is common in HCM and represents an important determinant of exercise capacity.

Left ventricular outflow tract obstruction as a risk factor for sudden cardiac death in hypertrophic cardiomyopathy.

The effect of left ventricular outflow tract obstruction (LVOTO) at rest on the incidence of sudden death (SD) in patients with hypertrophic cardiomyopathy is rather conflicting. The aim of this study was the evaluation of LVOTO at rest as a new potential risk factor for SD in hypertrophic cardiomyopathy. A total of 166 patients (112 men, 51.8 +/- 15.6 years) were studied; 50 patients (30.1%) had peak instantaneous LVOTO gradients of > or = 30 mm Hg at rest. During the follow-up period (median 32.4 months, range 1 to 209), 13 patients either died suddenly, or had cardiac arrest, documented sustained ventricular tachycardia, or implantable cardioverter defibrillator discharge. The cumulative event-free survival rate was 92% in patients with LVOTO, and 92% in patients without obstruction (p = NS). LVOTO at rest was associated with a particularly low positive predictive value for SD (8%), although a high negative predictive value (92%) was recorded. Patients having syncope or presenting with a maximum wall thickness > or =3 cm in echocardiography were more sensitive to SD emergence because they had a 13.07 (95% confidence interval 4.00 to 46.95, p <0.0001) and a 10.07 (95% confidence interval 2.92 to 34.79, p = 0.003) greater relative risk, respectively. In conclusion, our cohort study results do not support LVOTO as an independent risk factor for SD in patients with hypertrophic cardiomyopathy.

Identification of high risk patients with hypertrophic cardiomyopathy in a northern Greek population.

BACKGROUND: The percentage of hypertrophic cardiomyopathy (HCM) patients who are in high risk for Sudden Death (SD) constitutes only a minority of all HCM population but the incidence of SD in this subset is high (at least 5% annually). The identification of this small but important proportion of high risk HCM patients has been the clue in the clinical evaluation of these patients. METHODS: Our study cohort consisted from 123 patients with HCM who are currently followed up in our Institution. Five clinical risk factors were assessed: a family history of premature SD, unexplained syncope, Non Sustained Ventricular Tachycardia (NSVT) on 24-h ECG monitoring, Abnormal Blood Pressure Response (ABPR) during upright exercise testing and Maximum left ventricular Wall Thickness (MWT) > or =30 mm. The purpose of our study was the identification of high risk HCM patients coming from Northern Greece. RESULTS: Fifteen patients (12.2%) of the whole cohort had MWT > or = 30 mm, 30 patients (24.4%) had an ABPR to exercise, 17 patients (13.8%) had episodes of NSVT in 24-h Holter monitoring, 17 patients (13.8%) suffered from syncope, and 8 patients (6.5%) had a positive family history of premature SD. Data analysis revealed that 74 patients (60.1%) had none risk factor. Twenty four patients (19.5%) had 1 risk factor, 17 patients (13.8%) had 2 risk factors, 4 patients (3.25%) had 3 risk factors, and 4 patients (3.25%) had 4 risk factors, while none patient had 5 risk factors. Twenty five patients (20.3%) had 2 or more risk factors. CONCLUSION: This study for the first time confirms that, although a 60% of patients with HCM coming from a regional Greek population are in low risk for SD, a substantial proportion (almost 20%) carries a high risk for SD justifying prophylactic therapy with amiodaron or ICD implantation.

Hypertrophic cardiomyopathy with midventricular obstruction and apical aneurysm formation in a single family: case report.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is an extremely heterogeneous disease. An under recognized and very often missed subgroup within this broad spectrum concerns patients with left ventricular (LV) apical aneurysms in the absence of coronary artery disease. CASE PRESENTATION: We describe a case of HCM with midventricular obstruction and apical aneurysm formation in 3 patients coming from a single family. This HCM pattern was detected by 2D-echocardiography and confirmed by cardiac magnetic resonance imaging. A cardioverter defibrillator was implanted in one of the patients because of non-sustained ventricular tachycardia detected in 24-h Holter monitoring and an abrupt drop in systolic blood pressure during maximal exercise test. The defibrillator activated 8 months after implantation by suppression of a ventricular tachycardia providing anti-tachycardia pacing. The patient died due to refractory heart failure 2 years after initial evaluation. The rest of the patients are stable after a 2.5-y follow-up period. CONCLUSION: The detection of apical aneurysm by echocardiography in HCM patients may be complicated. Ventricular tachycardia arising from the scarred aneurysm wall may often occur predisposing to sudden death.

Hypertrophic cardiomyopathy with late enhancement of the non-hypertrophied left ventricular segments.

A 56-year-old asymptomatic man was referred for further evaluation because he displayed a rapid T-wave change on the ECG, from positive T waves to giant negative T waves in the anterolateral precordial leads, within 2 years. Transthoracic echocardiography revealed mild left ventricular apical hypertrophy without obstruction. Cardiac magnetic resonance imaging showed apical hypertrophic cardiomyopathy with focal hyperenhancement of the non-hypertrophic basal lateral segment of the left ventricle and absence of hyperenhancement of the hypertrophied apical segments.

Right ventricular function in ischemic or idiopathic dilated cardiomyopathy.

BACKGROUND: Differentiation between ischemic (ICM) and dilated cardiomyopathy (DCM) has important therapeutic implications because the former may benefit from coronary revascularization. The aim of this study was to investigate right ventricular (RV) function using tissue Doppler echocardiography (TDE) and compare the TDE parameters of the RV among patients with ICM and DCM. METHODS AND RESULTS: Forty-two patients with ICM and 40 patients with DCM were studied with conventional echocardiography and TDE. The 2 groups did not differ in terms of New York Heart Association class, left ventricular ejection fraction and pharmacological treatment. Patients with ICM had higher pulmonary artery systolic pressure (44.4 mmHg vs 34.7 mmHg, p=0.006) and lower tricuspid annular motion systolic (RV Sa 0.06 m/s vs 0.09 m/s, p<0.0001), and diastolic velocities (RV Ea 0.05 m/s vs 0.07 m/s, p=0.0003, RV Aa 0.075 m/s vs 0.11 m/s, p=0.0016). They also exhibited a higher ratio of early transtricuspid filling velocity to early diastolic velocity of the tricuspid annulus (RV E/Ea 8.2 vs 5.7, p=0.0008). Age, pulmonary artery systolic pressure and tricuspid Sa were significant independent predictors of the diagnosis of ICM. CONCLUSIONS: RV dysfunction is more pronounced in patients with ICM than in patients with DCM. The RV TDE parameters can be used to complement clinical and conventional echocardiographic findings in the assessment of patients with ICM and DCM.

Clinical significance of tissue Doppler imaging in patients with hypertrophic cardiomyopathy.

BACKGROUND: A transmitral E/septal Ea ratio > or =15 is a predictor of adverse outcome in cardiac disease, so it was hypothesized that a septal E/Ea >/=15 would predict the risk of adverse outcome, including sustained ventricular tachycardia (VT), cardiac arrest, implantable cardioverter defibrillator (ICD) discharge, or sudden death (SD) in patients with hypertrophic cardiomyopathy (HCM). METHODS AND RESULTS: The study group comprised 96 consecutive patients with HCM (median age 53 years) who completed all noninvasive tests for risk stratification. The endpoint of the study was defined as death, cardiac arrest, documented sustained VT, or ICD-discharge. The median follow-up was 20.6 months. All patients were alive at the end of follow-up, although 8 patients had reached the endpoint during the study period. The variables that were predictive of adverse clinical outcome were family history of premature SD (p=0.03), syncope (p<0.001), maximum wall thickness > or =3 cm (p=0.02), and septal E/Ea > or =15 (p<0.001). In a stepwise multivariable model the only independent prognostic indicator was a septal E/Ea > or =15 (relative risk 0.26, 95% confidence interval 0.2-0.58, p<0.001). The cumulative event-free survival rate was 78.9% in patients with septal E/Ea > or =15, and 100% in patients with septal E/Ea <15 (p=0.0003). CONCLUSIONS: Septal E/Ea > or =15 predicts patients with HCM who are at risk of sustained VT, cardiac arrest, ICD-discharge, or SD. (Circ J 2007; 71: 897 - 903).

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).