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: is a left ventricular outflow tract gradient a major prognostic determinant?

The relationship of left ventricular outflow tract gradient as well as of clinical, ECG and haemodynamic data to presenting features and prognosis was evaluated in 125 consecutive patients with hypertropic cardiomyopathy, 79 men and 46 women (mean age: 34 +/- 7 years) studied between January 1970 and December 1985. Most clinical, ECG and haemodynamic findings were similar in the 44 patients (35%) with a pressure gradient (greater than or equal to 30 mmHg) and in the 81 patients (65%) without. Those with obstruction had greater ECG voltage SV1 and RV5; however, higher grade ventricular arrhythmias were more common in patients without obstruction. During a mean follow-up period of 7.6 +/- 4.5 years (range 2-18 years), death from a cardiac cause occurred in 28 patients (21 died suddenly) and was significantly less common in patients with a pressure gradient than in those without (11% vs 28%, P = 0.039). Univariate analysis of survival curves showed that the most powerful predictors of a poor prognosis were ejection fraction P = 0.0001), mean pulmonary artery pressure (P = 0.0001), dyspnoea (P = 0.001), left ventricular end-diastolic pressure (P = 0.002), complex ventricular arrhythmias (P = 0.029) and severe mitral regurgitation (P = 0.037). Using multivariate analysis, a decreased ejection fraction (P = 0.006) and a raised mean pulmonary artery pressure (P = 0.022) were the only independent prognostic determinants. Thus, the presence of a left ventricular outflow tract gradient does not seem of adverse prognostic significance. Risk factor characterization in patients with hypertrophic cardiomyopathy may be improved by assessment of ventricular arrhythmias in the context of left ventricular function

Determinants of end-stage idiopathic dilated cardiomyopathy: a multivariate analysis of 104 patients

Our purpose in this study was to investigate the correlation of clinical, electrocardiographic, hemodynamic, and histopathologic features at diagnosis with the long-term prognosis in 104 patients with idiopathic dilated cardiomyopathy to determine which factors are the independent determinants of the end-stage disease. During a mean follow-up of 3.8 +/- 3.5 years, 35 patients (33%) died, 14 (13%) suddenly and 21 (20%) from congestive heart failure. Univariate analysis of survival curves disclosed that clinical and electrocardiographic variables at diagnosis were similar in survivors and non-survivors. On the contrary, patients who subsequently died had higher mean right atrial pressure (p = 0.0001), right ventricular end-diastolic pressure (p = 0.0061), mean pulmonary artery pressure (p = 0.0001), and left ventricular systolic (p = 0.0049) and end-diastolic (p = 0.0021) pressure than survivors. They also exhibited larger left ventricular end-diastolic (p = 0.0046) and end-systolic (p = 0.0027) volumes, lower ejection fraction (p = 0.0001), and a greater proportion had severe mitral regurgitation (p = 0.0095). Univariate analysis of histologic findings collected in a subgroup of patients referred since 1984 revealed a mild degree of myocellular hypertrophy to be associated with a poor prognosis (p = 0.0217). Multivariate analysis selected only mean right atrial pressure (p = 0.0022), ejection fraction (p = 0.0089), and end-systolic volume (p = 0.0265) as independent determinants of cardiac death. Our results suggest that cardiac catheterization is mandatory for risk stratification of patients with idiopathic dilated cardiomyopathy, since it allows the assessment of hemodynamic, angiographic, and histopathologic features helpful in identifying patients with a poor prognosis

Long-term prognosis in children with hypertrophic cardiomyopathy: an analysis of 37 patients aged less than or equal to 14 years at diagnosis

The relation of clinical, electrocardiographic, and hemodynamic findings at diagnosis to presenting features and prognosis of hypertrophic cardiomyopathy in childhood was evaluated in 37 consecutive patients below 14 years of age at time of diagnosis (24 males and 13 females, mean age 7 +/- 4 years). A left ventricular out-flow tract gradient (mean 42 +/- 27 mmHg) was detected at cardiac catheterization in 13 (35%) patients. Clinical, electrocardiographic, and hemodynamic features in patients with and without a pressure gradient were similar. Patients who had moderate to severe functional limitation had a higher incidence of syncopal episodes (p less than 0.001), lower ejection fraction (p less than 0.01), raised pulmonary artery pressure (p less than 0.001), and left ventricular end-diastolic pressure (p less than 0.01). During a follow-up of 9.2 +/- 5.1 years (range 2-18), 9 (24%) patients died suddenly (2 with a recorded left ventricular outflow tract gradient). Univariate analysis showed that reduced ejection fraction (p = 0.0001), syncopal episodes (p = 0.003), increased left ventricular end-diastolic pressure (p = 0.03), and severe dyspnea (p = 0.04) were associated with a poor prognosis. However, multivariate analysis revealed ejection fraction (p = 0.0001) and syncopal episodes (p = 0.0097) as independent predictors of survival. In conclusion, sudden cardiac death was common and was well predicted by the combination of left ventricular dysfunction and syncope at time of diagnosis