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The progression of hypertrophic cardiomyopathy: dilatation of the left ventricle with supernormal systolic function
We performed cardiac catheterisation in a man who had been diagnosed as having hypertrophic cardiomyopathy 7 years earlier. The repeat angiogram showed the maintenance of a "supernormal" systolic function (ejection fraction: 87%) although there was an increase of left ventricular end-diastolic volume (from 65 to 132 ml/m2). This case suggests that progressive left ventricular dilatation should not necessarily be considered a marker of the progression of hypertrophic cardiomyopathy into a hypokinetic left ventricle
Sudden death during pregnancy in hypertrophic cardiomyopathy
A 27-year-old asymptomatic woman became pregnant 6 months after a diagnosis of hypertrophic cardiomyopathy and was well until 28 weeks' gestation when she died suddenly while running up stairs. The potential pathophysiological mechanisms of sudden death during pregnancy in hypertrophic cardiomyopathy, as well as the diagnostic and therapeutic implications, are discussed
Natural history of hypertrophic cardiomyopathy in the elderly
To assess the natural history of hypertrophic cardiomyopathy (HC) in the elderly, we reviewed clinical, electrocardiographic and hemodynamic data from 21 patients over 60 years of age at diagnosis who were studied since 1970. Comparison with 95 younger patients (less than 60 years) showed that a greater proportion of older patients had exertional angina and atrioventricular conduction delay at time of diagnosis. During a mean follow-up of 9 +/- 6 years, mortality from cardiac causes was 33% (7 out of 21) in patients over 60 years of age and 23% (22 out of 95) in younger patients (NS). Univariate analysis showed that functional class and hemodynamic indices of right- and left-sided heart impairment were associated with a poor prognosis in the elderly. We conclude that: (1) presenting features and outcome of patients with HC over 60 years of age do not differ significantly from those of younger patients; (2) functional class and measurements of cardiac function are significant predictors of death in the elderly
Tolerance to continous transdermal nitroglycerin therapy in patients with effort and mixed angina: Its assessment and relevance [STUDIO SULLO SVILUPPO DI TOLLERANZA ALLA TERAPIA CONTINUA CON NITROGLICERINA TRANSDERMICA IN PAZIENTI CON ANGINA DA SFORZO E MISTA]
The present multicentre study, conducted in patients with angina pectoris on chronic therapy with continuous 24-hour transdermal nitroglycerin was aimed at investigating: (1) the actual incidence of nitrate tolerance; (2) the clinical features that characterize those who do not develop tolerance; (3) whether the evidence of clinical and ergometer benefits at 1-month assessment predicts their long-term maintenance. Eligible patients (n = 110; average age 56 +/- 5 yrs) had stable angina pectoris (78 effort angina, 32 mixed angina) with symptom-limited, reproducible cycloergometer tests and were responsive to s.l. nitroglycerin. After a 7-day washout period and a placebo run-in week, all patients were assigned to continuous 10 mg/24-hour nitroglycerin patches (T0). Exercise tests were performed again after 1 month (T1) and 6 months (T6) of treatment. At T1, ergometric data in each patient were compared with those at T0 and showed an increase (> or = 15%) in exercise tolerance in only 61/110 patients: 38 (49%; Group A) of the 78 patients with effort angina and 23 (72%; Group B) of the 32 patients with mixed angina (p < 0.05). Those patients with no significant change in exercise tolerance were assigned to conventional antianginal therapy and were excluded from the study. At T6, both group A and B patients maintained the favourable effects on total exercise duration, time and maximum workload at ischemic threshold and maximal ST depression recorded at T1 vs T0. The weekly attacks of angina and nitroglycerin s.I. consumption decreased significantly in both Group A and Group B patients from the beginning of therapy and throughout the study. These results show that: 1) tolerance to continuous 24-hour transdermal nitroglycerin therapy is not constant phenomenon, but occurs only in a subgroup of patients; 2) patients with mixed angina are more resistant to develop tolerance than those patients with effort angina; 3) the 1-month evidence of clinical and ergometric benefits predicts their maintenance during long-term treatment, as well. These results allow one to hypothesize that the loss of nitrate efficacy on venous pooling capability, but not on arterial tone, might constitute the more frequent cause of tolerance
Electrocardiographic findings in patients with hypertrophic cardiomyopathy. Relation to presenting features and prognosis
The relation of ECG findings to presenting features and prognosis was evaluated in 125 consecutive patients with hypertrophic cardiomyopathy (HC). Seventy-nine men and 46 women (mean age, 34 +/- 7 years) were studied since 1970. Most ECG features were similar in patients with and without a left ventricular outflow tract gradient. Those with obstruction had a higher prevalence of left ventricular hypertrophy according to ECG voltage criteria (54% vs. 28%, p less than 0.01), whereas higher grade ventricular arrhythmias were more common in patients without an outflow gradient (20% vs. 7%, p less than 0.05). The prevalence of ECG abnormalities was also similar in younger (less than or equal to 14 years) and older patients (greater than 14 years), and only repolarization abnormalities were more frequently detected in the older age group (56% vs. 32%, p less than 0.025). Stratification of patients according to the clinical state revealed that those who had moderate to severe functional limitation had a higher prevalence of atrial fibrillation than asymptomatic or mildly symptomatic patients (24% vs. 1%, p less than 0.001). There were no significant differences in most hemodynamic variables among patients dichotomized according to any specific ECG abnormality. Only patients with atrial fibrillation had significantly higher right ventricular end-diastolic pressure (10 +/- 7 vs. 6 +/- 4 mmHg, p less than 0.01), lower systolic index (22 +/- 8 vs. 37 +/- 15 ml/m2; beat, p less than 0.02) and lower ejection fraction (53 +/- 8 vs. 64 +/- 10%, p less than 0.001) than those in sinus rhythm.(ABSTRACT TRUNCATED AT 250 WORDS
Hypertrophic cardiomyopathy: long-term effects of propranolol versus verapamil in preventing sudden death in "low-risk" patients
The aim of this study was to evaluate retrospectively the outcome of 101 patients who were assigned to long-term therapy with propranolol (55 patients) or verapamil (46 patients) between 1980 and 1988. Baseline clinical, electrocardiographic, and echocardiographic data were similar in both groups. Exclusion criteria were the evidence of complex ventricular arrhythmias, a family history of the disease and/or sudden death, previous syncopal episodes, or left ventricular dysfunction. During a mean follow-up of 4 +/- 3 years (range: 1-9 years), side effects were more commonly recorded in patients who were treated with verapamil rather than in propranolol-treated patients (8 vs. 3, respectively), though the difference was not statistically significant. Sixteen patients (13 propranolol-treated patients and three verapamil-treated ones, p less than 0.05) died suddenly while on treatment. In addition, three patients who stopped verapamil because of adverse reactions died from heart failure after withdrawal, but before the end of the follow-up period. The assessment of total mortality on the intention-to-treat basis showed that death due to cardiac causes occurred in 13 propranolol-treated patients and in six verapamil-treated patients (ns). Thus, verapamil was more effective than propranolol in preventing sudden death during long-term therapy of "low-risk" patients with hypertrophic cardiomyopathy, though its administration was associated with the occurrence of non-sudden cardiac deaths and a high incidence of side effects
Resting and ambulatory ECG predictors of mode of death in dilated cardiomyopathy
With the purpose of verifying whether the electrocardiogram (ECG) pattern alone can predict the mode of death in dilated cardiomyopathy, data from 12-lead ECGs and 48-hour arrhythmia monitoring were evaluated in 67 patients with dilated cardiomyopathy. During a mean follow-up period of 3 +/- 2 years, death from congestive heart failure occurred in 18 patients (27%), whereas 10 (15%) died suddenly (NS). Multivariate analysis showed that left bundle branch block (p < 0.001) and left atrial enlargement (p < 0.001) were independently related to death from congestive heart failure. Ventricular arrhythmias of Lown grade 4A or 4B (p < 0.001) and repolarization time, as assessed by QTc-QRS interval (p < 0.05), were independent predictors of sudden death. It is concluded that ECG features alone may be helpful for risk factor characterization of dilated cardiomyopathy patients, provided that multiple ECG criteria are utilized at time of diagnosis
Electrocardiographic correlates with left ventricular morphology in idiopathic dilated cardiomyopathy
The purpose of the present study was to verify whether the electrocardiographic pattern of patients with idiopathic dilated cardiomyopathy (IDC) might be useful in predicting measurements of left ventricular (LV) morphology. A total of 12 electrocardiographic criteria for LV enlargement were evaluated in 67 patients with IDC, aged 14 to 68 years (mean 48), and were correlated to LV wall thickness, volume and mass, as assessed at angiography (all patients) and echocardiography (50 patients). Linear regression analysis showed weak correlations between multiple electrocardiographic criteria and LV wall thickness, volume and mass. Multiple logistic regression analysis showed that total 12-lead QRS amplitude, voltage criteria of Sokolow and Lyon, overshoot and U-wave inversion were the variables significantly related to LV wall thickness, as assessed by angiography (r = 0.55, p less than 0.005) and echocardiography (r = 0.43, p less than 0.025). The sum of T/R-wave ratios, the RV6/RV5 ratio and the Romhilt-Estes score were predictors of LV end-diastolic volume, as determined by angiography (r = 0.83, p less than 0.001) and echocardiography (r = 0.77, p less than 0.005). Total 12-lead QRS amplitude and the sum of T/R-wave ratios were the only independent predictors of LV mass, either angiographically (r = 0.81, p less than 0.001) or echocardiographically measured (r = 0.71, p less than 0.025). It is concluded that a single electrocardiographic criterion for prediction of LV morphology in patients with IDC is barely effective. Multiple electrocardiographic criteria should be utilized to better predict LV mass and distinguish reliably between LV wall thickening and dilatation
Cumulative exercise-induced left ventricular systolic and diastolic dysfunction in hypertrophic cardiomyopathy.
The phenomenon of cumulative exercise-induced left ventricular function impairment was studied in 40 patients with non-obstructive hypertrophic cardiomyopathy with resting normal left ventricular function and no increase in ejection fraction on exercise. All patients underwent two symptom-limited exercise tests one-hour apart. Cumulative myocardial dysfunction was seen in 13 patients (group I) but not in the remaining 27 patients (group II). During follow-up, group I showed more commonly than group II a deterioration in symptoms (67% vs 22%, P=0.025) and left ventricular function (50% vs 9%, P=0.019). In conclusion, cumulative exercise-induced myocardial dysfunction can occur in hypertrophic cardiomyopathy and may be associated with clinical deterioration and worse outcome
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