Comparison of clinical, morphological, and prognostic features in hypertrophic cardiomyopathy between Japanese and western patients.

Apical hypertrophic cardiomyopathy appears to be more common in Japan than in the West. Explanations for this difference include variable methods and criteria for the diagnosis. To assess morphological, clinical, and prognostic differences, 45 consecutive Japanese and 45 age- and gender-matched Western patients with hypertrophic cardiomyopathy were evaluated in two referral institutions by the same individuals. The diagnosis of hypertrophic cardiomyopathy was based on the echocardiographic demonstration of unexplained left ventricular hypertrophy. Patients were aged 8 to 64 years (mean 50); there were 66 males and 24 females. The pattern of left ventricular hypertrophy was similar in Japanese and Western patients: asymmetric septal 64 vs. 76%, concentric 22 vs. 13%, and apical 13 vs. 11% (p = NS). The incidence of an echocardiographic or Doppler calculated left ventricular gradient of > 30 mmHg was similar (11 vs. 18%; p = NS). The maximal left ventricular wall thickness was greater in Western patients (23 +/- 7 vs. 20 +/- 4 mm; p = 0.03), but was not different when adjusted for body surface area. Clinical features including incidence of family history and ventricular tachycardia during 24-h ambulatory electrocardiography were similar. During follow-up (4.9 +/- 4.0 years for Western vs. 4.4 +/- 2.0 years for Japanese), disease-related mortality was worse in Western patients (p < 0.05; 10 versus 2 patients). This evaluation, using the same diagnostic methods and criteria, reveals a worse prognosis in Western patients despite a similar clinical and morphological spectrum of hypertrophic cardiomyopathy.

[Prognostic significance of perfusion defect and its extension in dilated cardiomyopathy: assessment with thallium-201 myocardial scintigraphy].

To determine whether a change in the size of a scintigraphic perfusion defect is of prognostic significance in dilated cardiomyopathy (DCM), we performed thallium-201 myocardial scintigraphy twice for each of 13 patients with mean intervals of 45 +/- 18 months. Seven patients showed clinical deterioration (Group D) during the follow-up period of 58 +/- 22 months; whereas, 6 patients improved clinically as well as echocardiographically (LVDd < or = 5.5 cm and %FS > or = 20%) (Group I). 1. The initial scintigraphy showed that 3 patients had large defects (LD), 2 had multiple small defects (MSD) and 2 had no defects (NL) in Group D. Among 6 patients in Group I, one had LD, 3 had MSD and 2 had NL. 2. The second scintigraphy showed that in 5 patients in Group D, the defects were progressively enlarged and in the remaining 2 who had no defects at the initial scintigraphy, new defects developed (MSD). Among 6 patients in Group I, no patient had enlargement of defects. In 4 patients with defects (1LD, 3MSD), the sizes in their defects reduced. 3. At the initial study, all 7 patients in Group D fitted NYHA functional class III-IV; while, in Group I, 4 of the 6 patients fitted class III-IV and 2 patients class II. In the second study, all 7 patients in Group D remained in class III-IV; whereas, all 6 patients in Group I fitted class I-II. Five of the 7 patients in Group D died of heart failure after the second study.(ABSTRACT TRUNCATED AT 250 WORDS)