Prognosis in hypertrophic cardiomyopathy observed in a large clinic population

Overall annual cardiac mortality in hypertrophic cardiomyopathy (HC) has been reported to be between 2 and 4%, although these numbers are primarily from retrospective studies of patients referred to large research institutions. A clinic population of 113 patients with HC was prospectively studied to assess cardiac mortality in the overall group and in selected subgroups commonly thought to be at high risk for sudden death. The mean age at diagnosis was 37 ± 16 years. During follow-up, there were 11 cardiac and 2 noncardiac deaths. The annual cardiac mortality was 1% (95% confidence interval 0.2–1.8%). Because of the small number of deaths, relative risk for cardiac death was not significantly different in the presence of young age (≤30 years), family history of HC and sudden death, history of syncope or previous cardiac arrest, or both, ventricular tachycardia on 24-hour Holter monitoring, or septal myotomy/myectomy for refractory symptoms and outflow tract obstruction. It is concluded that HC has a relatively benign prognosis (1% annual cardiac mortality) that is 2 to 4 times less than that previously reported

Hypertrophic cardiomyopathy in a large community-based population: clinical outcome and identification of risk factors for sudden cardiac death and clinical deterioration

AbstractObjectivesThis study evaluates the clinical course and identifies risk factors for sudden cardiac death (SCD) and clinical deterioration in hypertrophic cardiomyopathy (HCM) in a large community-based population. Comparison was made with data from six tertiary referral and six nonreferral institutions.BackgroundHypertrophic cardiomyopathy is a disease with marked heterogeneity in clinical presentation and prognosis. Risk factors for SCD are not well defined in patients free of referral bias.MethodsBetween 1970 and 1999, 225 consecutive patients (mean age [±SD] 41±16 years) were examined and followed at yearly intervals.ResultsForty-four deaths were recorded of which 27 cases were cardiovascular. Fourteen patients died suddenly, six were successfully resuscitated, and seven patients died of congestive heart failure. The annual mortality, annual cardiac mortality, and annual mortality due to sudden death were 1.3%, 0.8%, and 0.6%, respectively. At least one New York Heart Association (NYHA) functional class deterioration was reported in 33% of the patients with a significant (≥50 mm Hg) left ventricular outflow tract (LVOT) gradient in contrast to 7% without obstruction. The presence of syncope was related to SCD (p < 0.05). Younger age and more severe functional limitation distinguishes patients from in hospital-based centers from the ones in community-based centers.ConclusionsHypertrophic cardiomyopathy is a benign disease in an unselected population with a low incidence of cardiac death. Syncope was associated with a higher incidence of SCD and patients with a significant LVOT obstruction were more susceptible to clinical deterioration

Angiotensin II type 2 receptors and cardiac hypertrophy in women with hypertrophic cardiomyopathy

The development of left ventricular hypertrophy in subjects with hypertrophic cardiomyopathy (HCM) is variable, suggesting a ro

Alterations in aortic elasticity in noncompaction cardiomyopathy

Background: Noncompaction cardiomyopathy (NCCM) is a recently recognized disorder frequently associated with systolic and diastolic heart failures. This study was designed to examine aortic stiffness in NCCM patients and to compare these results to age- and gender-matched controls. Methods: A total of 20 patients with typical echocardiographic features of NCCM (age 38 ± 16 years, eight males) were investigated. Their results were compared to 20 age- and gender-matched controls. All subjects underwent a complete two-dimensional transthoracic echocardiographic examination. Systolic (SD) and diastolic (DD) ascending aortic diameters were recorded in M-mode at a level of 3 cm above the aortic valve from a parasternal long-axis view. Aortic stiffness index (β) was calculated as a characteristic of aortic elasticity, as ln(SBP/DBP)/[(SD - DD)/DD], where SBP and DBP are the systolic and diastolic blood pressures, respectively, and ln is the natural logarithm. Results: The number of noncompacted segments in the NCCM patients was 4.6 ± 2.0. NCCM patients had significantly increased left ventricular dimensions and reduced left ventricular ejection fraction. Compared to controls, aortic stiffness index (β) was significantly increased in NCCM patients (8.3 ± 5.2 vs. 3.5 ± 1.1, p < 0.001). Conclusion: Increased aortic stiffness can be observed in patients with NCCM with moderate to severe heart failure. These alterations may be due to neurohormonal changes in heart failure

Alterations in aortic elasticity in noncompaction cardiomyopathy

BACKGROUND: Noncompaction cardiomyopathy (NCCM) is a recently recognized disorder frequently associated with systolic and diastolic heart failures. This study was designed to examine aortic stiffness in NCCM patients and to compare these results to age- and gender-matched controls. METHODS: A total of 20 patients with typical echocardiographic features of NCCM (age 38 +/- 16 years, eight males) were investigated. Their results were compared to 20 age- and gender-matched controls. All subjects underwent a complete two-dimensional transthoracic echocardiographic examination. Systolic (SD) and diastolic (DD) ascending aortic diameters were recorded in M-mode at a level of 3 cm above the aortic valve from a parasternal long-axis view. Aortic stiffness index (beta) was calculated as a characteristic of aortic elasticity, as ln(SBP/DBP)/[(SD - DD)/DD], where SBP and DBP are the systolic and diastolic blood pressures, respectively, and ln is the natural logarithm. RESULTS: The number of noncompacted segments in the NCCM patients was 4.6 +/- 2.0. NCCM patients had significantly increased left ventricular dimensions and reduced left ventricular ejection fraction. Compared to controls, aortic stiffness index (beta) was significantly increased in NCCM patients (8.3 +/- 5.2 vs. 3.5 +/- 1.1, p < 0.001). CONCLUSION: Increased aortic stiffness can be observed in patients with NCCM with moderate to severe heart failure. These alterations may be due to neurohormonal changes in heart failure

New Scores for the Assessment of Mitral Stenosis Using Real-Time Three-Dimensional Echocardiography

Nonsurgical management of patients with symptomatic mitral valve stenosis has been established as the therapeutic modality of choice for two decades. Catheter-based balloon dilation of the stenotic valvular area has been shown, at least, as effective as surgical interventions. Unfavorable results of catheter-based interventions are largely due to unfavorable morphology of the valve apparatus, particularly leaflets calcification and subvalvular apparatus involvement. A mitral valve score has been proposed in Boston, MA, about two decades ago, based on morphologic assessment of mitral valve apparatus by two-dimensional (2D) echocardiography to predict successful balloon dilation of the mitral valve. Several other scores have been developed in the following years in order to more successfully predict balloon dilatation outcome. However, all those scores were based on 2D echocardiography, which is limited by ability to distinguish calcification and subvalvular involvement. The introduction of new matrix-based ultrasound probe has allowed 3D echocardiography (3DE) to provide more detailed morphologic analysis of mitral valve apparatus including calcification and subvalvular involvement. Recently, a new 3DE scoring system has been proposed by our group, which represents an important leap into refinement of the use of echocardiography guiding mitral valve interventions

Value of assessment of tricuspid annulus: real-time three-dimensional echocardiography and magnetic resonance imaging

Aim: To detect the accuracy of real-time three-dimensional echocardiography (RT3DE) and two-dimensional echocardiography (2DE) for tricuspid annulus (TA) assessment compared with magnetic resonance imaging (MRI). Methods: Thirty patients (mean age 34 ± 13 years, 60% males) in sinus rhythm were examined by MRI, RT3DE, and 2DE for TA assessment. End-diastolic and end-systolic TA diameter (TAD) and TA fractional shortening (TAFS) were measured by RT3DE, 2DE, and MRI. End-diastolic and end-systolic TA area (TAA) and TA fractional area changes (TAFAC) were measured by RT3DE and MRI. End-diastolic and end-systolic right ventricular (RV) volumes and ejection fraction (RV-EF) were measured by MRI. Results: The TA was clearly delineated in all patients and visualized as an oval-shaped by RT3DE and MRI. There was a good correlation between TADMRIand TAD3D(r = 0.75, P = 0.001), while TAD2Dwas fairly correlated with TAD3Dand TADMRI(r = 0.5, P = 0.01 for both). There were no significant differences between RT3DE and MRI in TAD, TAA, TAFS, and TAFAC measurements, while TAD2Dand TAFS2Dwere significantly underestimated (P < 0.001). TAFS2Dwas not correlated with RV-EF, while TAFS3Dand TAFAC3Dwere fairly correlated with RV-EF (r = 0.49, P = 0.01, and r = 0.47, P = 0.02 respectively). Conclusion: RT3DE helps in accurate assessment of TA comparable to MRI and may have an important implication in the TV surgical decision-making processes. RT3DE analysis of TA function could be used as a marker of RV function