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Echocardiography was used to evaluate the incidence, flow dynamics and morphological characteristics of right ventricular obstruction in 91 patients with hypertrophic cardiomyopathy. Color flow mapping was used to define the sites of obstruction in the left and right ventricles. Ventricular obstruction was considered to be present if the flow velocity was less than 2.0 m/s as measured by continuous wave Doppler. The thickness of both the right ventricular free wall and anterior ventricular septum was measured to assess the magnitude and extent of hypertrophy. Right ventricular obstruction was present in 14 patients of whom 6 (43%) had left ventricular obstruction also. The right ventricular obstructions were found in the outflow tract (9 patients), mid-base septal bulge (2 patients) and apical trabecular region (3 patients). Doppler waveform was confined to systole in all patients with obstruction in the outflow tract and in one of the patients with mid-base septal bulge. Moreover, the flow wave persisted into early diastole in 4 patients, including 2 with apical trabecular obstruction. The thickness of both the right ventricular free wall and anterior ventricular septum suggested that these hypertrophied regions were the sites of right ventricular obstruction. Thus, echocardiography was useful in evaluating right ventricular obstruction in hypertrophic cardiomyopathy.

Background and Hypothesis: Supraventricular tachycardia and ventricular tachycardia are often observed in patients with hypertrophic cardiomyopathy (HCM) and they often alter the clinical features of HCM. We examine the influence of supraventricular tachycardia on cardiac function and assess the clinical characteristics of patients with HCM., Methods: We studied 32 patients with HCM and 8 normal volunteers using echocardiography under transesophageal rapid atrial pacing., Results: Presyncope-associated hypotension was observed during rapid atrial pacing in 8 HCM patients, but in none of the normal controls. During rapid atrial pacing (144 +/- 8 beats/min in HCM, 146 +/- 5 beats/min in controls), systolic blood pressure (SBP), the product of left ventricular filling volume (FV) and heart rate, and fractional shortening (%FS) in the HCM patients decreased significantly compared with the basal values (138 +/- 19 mmHg vs. 99 +/- 24 mmHg, 5.0 +/- 1.2 l/min vs. 2.9 +/- 0.9 l/min, 41.7 +/- 6.2% vs. 35.2 +/- 6.0%, respectively), but these decreases were not observed in normal controls. The decrement of SBP during rapid atrial pacing in HCM patients with a history of syncope was more marked than that in those without such history. The decrement correlated positively with the indices of left ventricular hypertrophy (maximal wall thickness and wall thickness index) and with %FS, and correlated negatively with the endsystolic left ventricular diameter at rest., Conclusions: In some patients with HCM, supraventricular tachycardia causes marked hemodynamic deterioration that may be related to a history of syncope, marked hypertrophy, hyperkinesis, small cavity size, and small filling volume of the left ventricle.

The flow dynamics of midventricular obstruction in hypertrophic cardiomyopathy were evaluated with intravenous injection of sonicated albumin in 14 patients with vague velocity profiles on conventional continuous wave Doppler echocardiography. Nine patients (64%) demonstrated a clear envelope after the injection. The following three patterns of continuous Doppler profiles were obtained : 1) a monophasic flow profile with a brief and sharp increase in peak velocity in late systole, 2) a late-peaking systolic flow and a diastolic flow during isovolumic relaxation time emerging after sonicated albumin injection (biphasic flow profile), 3) a biphasic flow profile with systolic and diastolic flow waves having a notch around the second heart sound. The calculated peak pressure gradients from the modified Bernoulli equation did not differ significantly with the presence of diastolic wave or the site of obstruction. Therefore, these are independent parameters for estimating the severity of midventricular obstruction. Therefore, these are independent parameters for estimating the severity of midventricular obstruction. The sonicated albumin method was useful in delineating the flow profile, and thus evaluating the hemodynamics of midventricular obstruction in hypertrophic cardiomyopathy.

Background: During atrial contraction, the left atrium produces both forward ejection to the left ventricle and backward ejection to pulmonary veins., Purpose: To determine quantitatively left atrial ejection performance in symptomatic hypertrophic cardiomyopathy (HCM) patients by using left atrial systolic time intervals., Method: Thirty-one patients with symptomatic HCM and sinus rhythm and 31 normal subjects were studied with transthoracic and transesophageal Doppler echocardiography. Left atrial pre-ejection period (APEP), corrected atrial pre-ejection period (APEPc), and the duration (AET) and the time velocity integral (IA) of the atrial contraction wave in transmitral flow were determined. The duration (PV-AET) and the time velocity integral (PV-IA) of reverse pulmonary venous flow during atrial contraction were also determined., Results: For normal subjects, significant correlations were found between APEP and electrocardiographic P wave duration (r = 0.78, p < 0.001), and between APEPc and left atrial forward ejection indices (AET: r = -0.42, p = 0.01, IA: r = -0.54, p < 0.05, respectively), but not between APEP and either of the two latter indices. These findings indicate that, for normal subjects, APEPc is a better index of left atrial systolic function than APEP. In contrast, for HCM patients, significant correlations were found between APEPc and left atrial backward ejection indices (PV-AET: r = -0.49; p < 0.005, PV-IA: r = -0.66; p < 0.001, respectively), but not between APEPc and left atrial forward ejection indices., Conclusion: Augmentation of left atrial afterload may result in decreased left atrial forward ejection and increased left atrial backward ejection. Thus, both left atrial forward ejection and left atrial backward ejection must be considered in the assessment of left atrial ejection performance in patients with symptomatic HCM.

The pathomorphologic features of hypertrophic cardiomyopathy simulating dilated cardiomyopathy in the late stage (HCM-DCM) were compared with those of ordinary hypertrophic cardiomyopathy (HCM). Seven autopsied hearts with HCM-DCM and 11 with HCM were assessed quantitatively using an image analyzer. Unlike HCM, significant left ventricular enlargement and wall thinning were observed in HCM-DCM, and the percentage areas of massive fibrosis and disarray were significantly greater. In HCM-DCM, the disarray was distributed diffusely, whereas massive fibrosis was distributed more intensively in the ventricular septum and anterior wall than in the lateral and posterior wall. Narrowing of intramyocardial small arteries was observed more frequently in HCM-DCM, especially in the ventricular septum and anterior wall, than in HCM. These results suggest that the enlargement and wall thinning of the left ventricle in HCM-DCM are attributable to non-uniform progression of massive fibrosis, which is closely related to small-arterial lesions.

To investigate the significance of augmentation of left ventricular slow filling, trans-mitral flow (TMF) was echocardiographically observed through pulsed Doppler method in 116 patients with hypertrophic cardiomyopathy (HCM), 74 with dilated cardiomyopathy (DCM), 27 with mitral regurgitation (MR), 86 with old myocardial infarction (OMI) and 80 normal controls (C). The slow filling-wave (SFW) in TMF was defined as the filling wave with obvious peak velocity during slow filling phase. The peak velocities (E, A, S) of the early filling, the atrial contraction and the slow filling waves were measured. The SFW was divided into two patterns according to the S and E ratio: large SFW (S/E greater than or equal to 1/2) and small SFW (S/E less than 1/2). 1) The small SFW was more frequently, but not significantly, observed patients with MR (37%) than in normal subjects (18%), patients with HCM (10%), DCM (4%) and OMI (7%). However large SFW was observed in 16 patients (14%) with HCM, but not in normal subjects and patients with other cardiac diseases excluding one patient with DCM. 2) In normal subjects and patients with DCM, OMI and MR, those with small SFW had larger E and smaller A/E than those without small SFW. However in patients with HCM, there was no difference in these indices (E and A/E) according to whether the patients were with or without SFW. 3) In HCM, patients with large SFW had significantly smaller E and significantly larger isovolumic relaxation time than those without SFW. Thus, appearance of the large slow filling wave, which might be caused by abnormal relaxation of the left ventricle, was frequently observed in patients with HCM.

To elucidate the effects of atrial defibrillation in patients with idiopathic cardiomyopathy, we clinically and echocardiographically assessed 6 patients with hypertrophic cardiomyopathy (HCM) and 7 patients with dilated cardiomyopathy (DCM). Their mean age was 57 +/- 14 years and the mean duration of their atrial fibrillation (Af) was 47 +/- 29 days. There were no differences in age and the duration of Af between the HCM and DCM groups. We assessed the effects of defibrillation on the NYHA functional classification, heart rate (HR), systolic blood pressure (S-BP), M-mode echocardiographic data (LVDd, LVDs, %FS, LAD) and transmitral pulsed Doppler echocardiographic findings (peak velocity, time-velocity integral of rapid and atrial filling waves). These indices were obtained before and 52 +/- 22 days after defibrillation, and were compared with each other. 1. HR decreased (HCM: 87 +/- 16-->58 +/- 7/min, DCM: 93 +/- 19-->70 +/- 14/min) and total left ventricular filling increased (HCM: 6 +/- 1-->11 +/- 4 cm, DCM: 6 +/- 1-->10 +/- 2 cm) after defibrillation, and the increment of %FS (HCM: 36 +/- 6-->41 +/- 6%, DCM: 16 +/- 6-->25 +/- 11%) was observed. Four of 6 HCM patients and 5 of 7 DCM patients also improved with regard to the NYHA classification. 2. After defibrillation, LVDd increased in HCM (42 +/- 4-->47 +/- 4 mm), but not in DCM. However, LVDs decreased in DCM (52 +/- 9-->44 +/- 12 mm), but not in HCM. We concluded that atrial defibrillation had a beneficial effect on the recovery of the left ventricular function both in HCM and DCM due to the reduction in HR and increase in left ventricular filling. The mode of LV functional improvement after defibrillation varied depending on the state of patient's basal pathophysiology .

The effects of verapamil on the diastolic function and regional asynchrony of the left ventricle were echocardiographically investigated in 37 patients with hypertrophic non-obstructive cardiomyopathy (HCM). Before and 20 min after the intravenous administration of verapamil (0.1 mg/kg), blood pressure (BP), heart rate (HR), transmitral pulsed Doppler indices (R, A, A/R), M-mode echo indices (Dd, Ds, %FS) and the echo-phonocardiographic index (isovolumic relaxation time; IRT) were measured. We also measured the rapid extension times of the posterior walls at the chordae and papillary muscle level [Tc(pw), Tp(pw)] as the indices of regional diastolic function, and their difference [Tp-c(pw)] was used as the index of diastolic asynchrony. Before verapamil administration, the HCM patients had significantly larger A, A/R, IRT and Tp-c(pw) and smaller R than did 20 healthy controls, but showed no significant differences in HR, BP, Dd, Ds and %FS. After verapamil injection, R increased and A, A/R, IRT, Tp-c(pw) decreased significantly without any changes in BP and HR. The increment of R (delta R) correlated significantly with the decrement of Tp-c(pw) (r = -0.66, p less than 0.001). According to the value of delta R/R, 37 patients with HCM were categorized in 2 groups (19 responders with delta R/R greater than or equal to 10% and 18 non-responders with delta R/R less than 10%). The responders had smaller R and larger A/R, IRT and Tp-c(pw) before verapamil administration than did the non-responders. Verapamil increased R, Dd, Ds and decreased A, A/R and Tp-c(pw) in the responders, but not in the non-responders. These results suggested that verapamil might improve left ventricular diastolic function in HCM patients through the improvement of left ventricular diastolic asynchrony.

To investigate the cardiac reserve of hypertrophic nonobstructive cardiomyopathy (HNCM), an echocardiographic study was performed before and during angiotensin II loading in 22 HNCM patients without left ventricular (LV) systolic dysfunction at rest and 8 normal subjects. LV end-diastolic dimension (LVDd) and time velocity integral (Ie) at aortic annulus were obtained as parameters of preload and forward ejection respectively. Responses of these indices to afterload stress were compared with clinical findings and right ventricular biopsy findings. Based on the reserve of forward ejection characteristics to afterload stress, 22 HNCM patients were divided into 2 groups; Group I: 9 cases with more than 15% decrease of Ie which corresponded to the value of mean - 1SD in normal subjects, Group II: 13 cases with a decrease of Ie less than 15%. There were no significant differences in echocardiographic findings between Group I and II before angiotensin II loading. On the other hand, Group I had a smaller increase of LVDd (p less than 0.05) to afterload stress, a higher incidence of ventricular tachycardia on a Holter electrocardiogram (p less than 0.05), lower maximal oxygen consumption with treadmil exercise test (p less than 0.01), and a larger value of LV end-diastolic pressure (p less than 0.05) than those in Group II, respectively. Although no difference in myocyte diameter was found between the 2 groups, myocardial fibrosis was more severe (p less than 0.001) and the incidence of moderate or severe disarray was higher (p less than 0.05) in Group I than in Group II. Thus, some HNCM patients cannot maintain forward ejection to afterload stress because of impaired preload reserve attributable to a greater stiffness of the left ventricle with severe myocardial lesion.

To determine the effects of aging and concentric hypertrophy on the reserve of the left ventricle (LV), 36 patients with hypertension (HT), 22 with hypertrophic cardiomyopathy (HCM) and 25 age-matched normal subjects (N) were studied by isoproterenol (ISP) infusion echocardiography. The end-diastolic LV relative wall thickness (RWT), fractional shortening (FS), peak mitral flow velocity during the rapid filling phase (R) and atrial systole (A), and the A/R ratio were measured. At rest, the RWT and A/R were larger in groups HT and HCM than in group N. There were no significant differences in age, heart rate (HR), and FS among the groups. The A/R of all groups showed a good positive correlation with increasing age. The A/R of the HT group was also positively correlated with RWT. During ISP infusion, the HR, R, A, A/R and FS increased in all groups. The changes in R and FS were smaller in groups HT and HCM than in group N. The change in HR in groups N and HT, and the change in FS in all groups during ISP infusion were all inversely correlated with increasing age. The change in FS during ISP infusion was inversely correlated with RWT in the HT group, but not in HCM. Thus, it was suggested that the impairment of LV reserve in patients with HCM is not only caused by concentric hypertrophy and the aging process, but also by other myocardial factors.

A 64-year-old man was hospitalized in March 1983 for recurrent episodes of dyspnea and palpitation despite medical treatment. At 48 years of age (1967), hypertension was diagnosed, but well controlled by propranolol. At the age of 59 years (1979), his first episode of dyspnea and palpitation was noted, with electrocardiographic (ECG) evidence of left ventricular hypertrophy (LVH), and abnormal Q waves in leads III and a VF. The echocardiogram, however, showed mild LVH with neither asymmetric septal hypertrophy (ASH) nor systolic anterior movement (SAM). On admission (1983), his blood pressure was 130/70 mmHg, and a grade III systolic murmur was heard along the left sternal border and over the apex. On ECG, the left precordial voltage (Sv1 + Rv5) was diminished and the depth of the Q wave in lead III increased compared with that of 1978. On echocardiography, we observed prominent septal hypertrophy (IVST: 20 mm) with ASH (IVST/PWT = 2.0), SAM, an enlarged left atrium, and a diminished left ventricular (LV) cavity. Left ventricular catheterization showed a pressure gradient of 90 mmHg between the LV apex and outflow tract and histological examination of the bi-ventricular endomyocardial biopsy material showed markedly hypertrophied and mildly disarranged myocardial fibers with bizarre nuclei. We considered that this was an interesting case in which ASH and SAM developed in the progression of LVH during the follow-up period.

In a total of 114 cases of hypertrophic cardiomyopathy (HCM), four cases were regarded to have dilated left ventricle and congestive heart failure. History of myocardial infarction was not present, and significant narrowing of coronary arteries was not detected in each case. The clinical manifestations were analyzed in the light of the differentiation from postmyocarditis cardiomyopathy (PMC: three cases) and hypertensive heart disease ( HHD : five cases). Representative case was a 47-year-old man with HCM, who showed congestive heart failure developed gradually over the last 20 years. The cardiothoracic ratio increased from 57% to 64% and high voltage of the precordial leads noted initially decreased gradually with normalization of ST-T changes. Meanwhile, a new abnormal Q wave appeared in aVL. The echocardiographic findings at the age of 47 were asymmetric septal hypertrophy and left ventricular enlargement. Myocardial biopsy of the right ventricle done at the same period revealed the marked progression of interstitial fibrosis as compared with that of 15 years ago. Clinical pictures demonstrated that PMC patients were younger than HCM or HHD patients. Abnormal Q waves were observed in three cases of HCM. In the remaining one, the Q wave was not discernible due to superimposed complete left bundle branch block. No abnormal Q waves were observed in cases of PMC and HHD . A high voltage of the left precordial leads with marked ST-T changes suggesting left ventricular hypertrophy was not seen in HCM with dilated left ventricle and HHD . Echocardiographically, the systolic anterior motion was absent in all cases. The left ventricular posterior wall in PMC and HHD was equally hypertrophied both at the apex and base, whereas in three of four cases of HCM, the apical region of the posterior wall was disproportionately hypertrophied. All cases of HCM and PMC showed asymmetric septal hypertrophy, while all the HHD cases except one showed concentric hypertrophy. In HCM, left ventricular end-diastolic dimension and left atrial dimension were increased to 60.0 +/- 6.1 mm and 45.3 +/- 9.4 mm, respectively, which were not different from those of PMC and HHD . Isometric relaxation time was prolonged to 100 msec or more in all cases of HCM, while in only one case of PMC it was prolonged and no prolongation was observed in HHD . Left ventricular wall motion revealed asynergy mainly at the hypertrophic apical area in HCM and PMC, whereas asynergy was found at the hypertrophic area, which was mostly apical, and also in the area without hypertrophy in HHD .(ABSTRACT TRUNCATED AT 400 WORDS)

A dipyridamole-induced Tl-201 perfusion abnormality was evaluated from its clinical features, echocardiography and myocardial histopathology in 39 patients with hypertrophic cardiomyopathy (HCM). From the findings of Tl-201 emission computed tomography (ECT), subjects were divided into three groups: group 1 (n = 16) which did not show a perfusion abnormality in the hypertrophic region; group 2 (n = 12) which showed a perfusion defect on the initial image with complete redistribution on the delayed image; and group 3 (n = 11) which showed a persistent perfusion defect--this group included most patients who revealed partial and/or incomplete redistribution. Echocardiography revealed that group 2 showed a marked asymmetrical septal hypertrophy and an incidental obstructive pattern, and that group 3 had a significantly dilated left ventricular diastolic dimension and a decreased percentage of fractional shortening. Group 3 also showed frequent ventricular tachycardia and a familial history of cardiomyopathy. As for the myocardial biopsy findings, group 3 had significantly advanced myocardial fibrosis, the percentage being 6.0 +/- 3.1% in group 1; 5.5 +/- 2.5% in group 2; and 11.9 +/- 3.4% in group 3. Thus, it was concluded that the persistent perfusion defect on dipyridamole stress Tl-201 ECT testing is an important finding corresponding to the advanced clinical and pathological aspects of HCM.

To clarify the influence of cardiac function of myocardial histologic lesions on the prognosis of hypertrophic cardiomyopathy (HCM), most recent echocardiographic findings of 30 deceased patients were reviewed. In 12 patients, histological sections from the left ventricular transverse plane made at necropsy were examined. According to the main causes of death, patients were categorized into four groups as sudden death (17 cases), heart failure death (four cases), embolic death (four cases), and non-cardiac death (five cases). 1. Clinically, atrial fibrillation and heart failure (NYHA III, IV) were frequently observed in heart failure and embolic groups. 2. Echocardiographically, left ventricular wall thickness did not differ among the four groups, but percent fractional shortening was significantly smaller in heart failure group than in non-cardiac group, and left ventricular end-diastolic dimension (LVDd) and left atrial dimension were significantly greater in heart failure group than in non-cardiac group. The normalized rapid filling rate was less in heart failure, embolic, sudden death and non-cardiac groups in that order. 3. Histopathologically, the mean myocyte diameter (MD) and percent area of disarray (%D) did not differ among the four groups, but % area of massive fibrosis (MF) greater than 2 mm2 was larger in heart failure, embolic, sudden death and non-cardiac groups in that order, especially in heart failure group. 4. Comparison of echocardiographic and histopathological findings: Though MD and %D did not correlate with any echocardiographic indices, %MF correlated significantly with LVDd (r = 0.83, p less than 0.005), and correlated inversely with the normalized rapid filling rate (r = -0.80, p less than 0.005).(ABSTRACT TRUNCATED AT 250 WORDS)

Twenty-one members of a family were traced for three generations including the propositus, who had typical dilated cardiomyopathy (DCM)-like features. Clinical, radiological, electrocardiographic and echocardiographic examinations were performed for 13 of 16 still living. Five of 21 members died including three of sudden death. Among living 16 members, five had congestive heart failure, greater than grade III of the NYHA, and cardiomegaly on chest radiography in five. Left ventricular hypertrophy, abnormal Q waves, and ST-T changes were observed on electrocardiography in two, five and eight members, respectively. Echocardiography revealed asymmetric septal hypertrophy (ASH) in four, two of whom had systolic anterior motion (SAM) of the mitral valve. Two had features simulating DCM, and five had mitral valve prolapse. Among six patients with ASH or features simulating DCM, four had cardiac catheterization, coronary angiography and endomyocardial biopsy. None had significant coronary stenosis, but all had high left ventricular end-diastolic pressure (LVEDP) (23 +/- 7 mmHg). Endomyocardial biopsy showed moderate myocardial cellular hypertrophy, interstitial myocardial fibrosis, nuclear changes and myocardial disarrangement, with features characteristic of HCM. In this family with malignant HCM, patients had various clinical features including typical HCM, simulated DCM, DCM coexisted with mitral valve prolapse, and sudden death occurred frequently.

To investigate the influence of wall hypertrophy and myocardial lesions on the regional contractile dynamics of the left ventricle (LV) in patients of hypertrophic cardiomyopathy (HCM), we obtained the thicknesses of the ventricular septum (VS) and posterior wall at end-diastole and systole (E.Td, E.Ts) from echocardiograms made before death for 11 patients of HCM and 6 patients of secondary concentric hypertrophic heart disease (SHH), and the percent regional systolic wall thickening normalized by that of 15 normal controls (%NRWT). We then compared the %NRWT with the pathological wall thickness (P.T.), myocyte diameter (MD), and percent areas of fibrosis (%F) and disarray (%D) in specimens of the corresponding portion from each autopsied heart. The %NRWT in the VS of HCM patients was significantly less than that in SHH patients. All of the MD, %F and %D in the LV wall of HCM patients were significantly greater than those in SHH patients. The %NRWT in the LV wall of HCM patients and SHH patients correlated inversely with the E.Td (r = -0.53, p less than 0.02 and r = -0.70, p less than 0.02, respectively), but not with the E.Ts or P.T. The %NRWT in the LV wall of HCM patients had an inverse correlation with the %F (r V -0.59, p less than 0.005). Furthermore, that in the VS had an inverse relationship with the %D (r = -0.63, p less than 0.05). Thus, it is suggested that not only LV hypertrophy, but also LV myocardial lesions affect the abnormality of the regional LV contractile dynamics in HCM patients. In addition, observation of the regional contractile dynamics by echocardiography may be useful for speculating on myocardial lesions associated with this disease.

Thallium-201 (Tl) single photon emission computed tomography (SPECT) after dipyridamole infusion (0.56 mg/kg) was performed in 23 patients with hypertrophic non-obstructive cardiomyopathy (HNCM) and in seven patients with HCM simulating dilated cardiomyopathy (HCM-DCM) to clarify the mechanism and clinical significance of decreased coronary vasodilatory reserve. The coronary vasodilatory reserve in the hypertrophied area assessed by SPECT was compared with the findings of echocardiography, left ventriculography and endomyocardial biopsy. 1. Eleven patients with HNCM had no perfusion defects in the hypertrophied area (group I), but the other 12 patients (52.2%) had such defects (group II). All seven patients with HCM-DCM had perfusion defects in the anterior or septal walls (group III). Redistribution was observed in 11 of the 12 patients in group II and in three of the seven patients in group III. 2. The regional washout rate was relatively low in the upper septum in group II and in the anterior wall and upper septum in group III. Thus, coronary vasodilatory reserve in the hypertrophied area was decreased in groups II and III. 3. Echocardiographically, the degree of hypertrophy did not differ between groups I and II, but the latter had significantly greater left ventricular diastolic dimension (42.3 mm vs 49.5 mm: p less than 0.05) and lower percent fractional shortening (%FS) (43.7% vs 35.6%: p less than 0.05). However, group III showed thinner left ventricular wall, much greater diastolic dimension (60.9 mm vs 49.5 mm: p less than 0.05), and lower %FS (24.0% vs 35.6%: p less than 0.05) than did those of group II. 4. On left ventriculography, those in group II showed larger left ventricular end-diastolic volume index (93.9 ml/m2 vs 79.7 ml/m2: p less than 0.05) than that of group I. Left ventricular ejection fraction showed the same tendency, but this was not statistically significant. 5. On endomyocardial biopsy, the specimens of the patients in group II had significantly higher percent fibrosis than did those of group I (11.4% vs 6.8%: p less than 0.05). These findings suggest that the mechanism of decreased coronary vasodilatory reserve in the hypertrophied area may be related to myocardial fibrosis, and this decrease may induce left ventricular dysfunction and compensatory dilatation.

To investigate the relationship between regional contractile dynamics and regional myocardial lesions of the left ventricular wall in patients with hypertrophic cardiomyopathy (HCM), autopsy findings of 11 patients were compared with their ante mortem echocardiographic findings. The regional systolic wall thickenings (%RWT) of the interventricular septum (IVS) and left ventricular posterior wall (LVPW) obtained using M-mode echocardiography were converted into % normalized RWT (%NRWT) by the averaged %RWT in 15 normal subjects. The %NRWT was compared with the wall thickness obtained by echocardiography and/or autopsy, and histological findings, such as the myocardial fibrosis ratio, disarray area ratio, and mean myocyte diameter. 1. There were no significant correlations among wall thickness of the left ventricle, the myocardial fibrosis ratio, the disarray area ratio, and the mean myocyte diameter of each segment. 2. The %NRWT in 22 segments of the 11 patients with HCM was not significantly related to the echocardiographic wall thickness at end-systole, the autopsy wall thickness, the mean myocyte diameter and the disarray area ratio, but that correlated significantly with the echocardiographically-determined wall thickness at end-diastole (r = -0.53, p less than 0.02), and with the myocardial fibrosis ratios (r = -0.59, p less than 0.005). 3. The %NRWT in the IVS was significantly less than that in the LVPW. The %NRWT in all segments of the LVPW was significantly related to the myocardial fibrosis ratios (r = -0.80, p less than 0.005), but was not related to the wall thicknesses or the disarray area ratios.(ABSTRACT TRUNCATED AT 250 WORDS)

In 30 consecutive hypertrophic cardiomyopathy (HCM) patients who eventually died and 50 who survived, morphologic and functional changes in the heart during a follow-up period of an average of 3.9 years were echocardiographically evaluated. Echocardiographic indices were compared among 5 groups of patients with HCM, consisting of 4 groups of patients who eventually died (SD: 17 patients who suffered sudden death, ED: 4 who died of embolism, HF: 4 who died of congestive heart failure, NC: 5 who died of a noncardiac event) and 1 group of patients who survived with nondilated left ventricle (S: 50 patients). These indices at the last evaluation before death were compared with histopathological findings of left ventricles in 12 autopsied patients. At the initial evaluation ED and HF patients had a larger left ventricular end-diastolic dimension (LVDd), and HF patients had a smaller percent fractional shortening (%FS) and a slower normalized rapid filling rate (nRFR: mean rapid filling rate/LVEDV) than S patients, but these indices showed no differences between SD and S patients. During follow-up, no echocardiographic indices changed in S patients, but LVDd was increased and %FS and nRFR were decreased in SD and HF patients. Mean myocyte diameter and % area of disarray showed no differences among the 4 death groups. However, compared with NC, the other 3 groups, especially HF, had larger % area of massive fibrosis. The % area of massive fibrosis was correlated with LVDd (r = 0.80, p less than 0.005), %FS (r = -0.64, p less than 0.05), and nRFR (r = -0.95, p less than 0.001) at the last evaluation. These results suggested that echocardiographic follow-up was useful in predicting the progression of the myocardial lesion and the prognosis of HCM.

In order to understand the mechanism of development of asymmetric septal hypertrophy (ASH) in hypertension, 290 patients with essential hypertension (HT) were examined echocardiographically. Out of them 84 cases of advanced left ventricular hypertrophy (LVH) [37 cases of symmetric hypertrophy (HT-SH group) and 47 cases of ASH (HT-ASH group)] were compared in their clinical and echocardiographic findings with hypertrophic cardiomyopathy (HCM). In the 290 HT cases, the highest systolic pressure in each patient's history was found to correlate with left ventricular (LV) posterior wall thickness (PWT), but not with the septal wall thickness (IVST). There were no differences in LV thickness (IVST + PWT) among patients in the HT-ASH, HT-SH and HCM groups. While the HCM group patients showed no significant differences in IVST and PWT from those in the HT-ASH group, they did have greater IVST and smaller PWT than HT-SH group patients. The rapid filling rate (RFR) was also not much different in the HCM and HT-ASH groups, but was significantly lower in the HCM group than in the HT-SH group. Furthermore, HT-ASH group patients has a milder degree of hypertension and a higher incidence of familial occurrence of HCM than did those in the HT-SH group. After treatment for HT, the HT-SH group showed a significant decrease in wall thickness during long-term observation, while the HT-ASH and HCM groups, failed to exhibit such changes. Moreover, the degree of myocardial disarrangement in the HT-ASH group did not differ significantly from that in the HCM group. These results suggested that LVH in HT is related not only to pressure load but also to genetic factors similar to that in HCM.

The mode of right ventricular hypertrophy was assessed by two-dimensional echocardiography (2DE) for 24 patients with hypertrophic cardiomyopathy (HCM), and the results were compared with those of 51 patients with hypertension (HT). The patients with HT were categorized in four groups depending on the thickness of the interventricular septum (IVST) and left ventricular posterior wall (PWT): HT-ASH with both left ventricular hypertrophy (LVH) (IVST greater than or equal to 13 mm) and asymmetric septal hypertrophy (ASH) (IVST/PWT greater than or equal to 1.3), severe HT with LVH and without ASH, and mild HT without LVH and ASH. Anterior wall thickness (AWT), posterior wall thickness (PWT), and diaphragmatic wall thickness (DWT) of the right ventricle were obtained from 2DE in the parasternal long-axis view, the short-axis view and subxiphoid view, respectively. These were recorded on video tape, and the measurements were made on the stop frames. Right ventricular hypertrophy (RVH) was estimated by the maximal right ventricular wall thickness (max RVWT), and the ratio of the maximal and minimal thickness (max RVWT/min RVWT) was calculated to evaluate asymmetrical hypertrophy (AH) of the right ventricle (RV). The incidence of RVH (Max RVWT greater than or equal to 5 mm) and asymmetrical hypertrophy (AH) (max RVWT/min RVWT greater than or equal to 1.3) of the RV in HCM, HT-ASH and mild HT were 67% and 41%, 57% and 45%, and 15% and 11%, respectively. The incidence of RVH with AH was more frequent in patients with HCM as well as HT with ASH than in patients with HT without ASH.(ABSTRACT TRUNCATED AT 250 WORDS)

This is a report of a patient with mitral valve prolapse (MVP) and myocardial abnormalities on endomyocardial biopsy in whose relatives hypertrophic cardiomyopathy (HCM) was identified. A 19-year-old woman was admitted to our hospital for evaluation of a heart murmur. A systolic ejection murmur was audible in the third intercostal space at the left sternal border, and a standard 12-lead electrocardiogram showed ST-T wave changes in leads II, III and aVF. Echocardiography revealed prolapse of the anterior leaflet of the mitral valve, but no left ventricular hypertrophy. Endomyocardial biopsy disclosed mild hypertrophy and disarrangement of the myocardium. The family study revealed asymmetrical septal hypertrophy in her mother, who had no history of hypertension. Her younger sister had mild hypertrophy of the interventricular septum on echocardiography, and her histopathological findings suggested a diagnosis of HCM. This case was clinically regarded as MVP, but development of left ventricular hypertrophy as noted in her mother may occur in the future.

To assess pathophysiological changes and prognoses in hypertrophic cardiomyopathy (HCM), the clinical courses of 190 patients with HCM between 1968 and 1985 were reviewed and analyzed. Their average follow-up period was 4.0 years. During the course of follow-up, 24 patients died of heart disease at annual mortality of 3.3%; 22 sudden death, and two congestive heart failure. Among the 190 cases, 70 were studied by echocardiography with an average follow-up period of 4.4 years. The subjects were categorized in three groups: 10 who died suddenly (SD group), 16 with ventricular tachycardia (VT group) and the remaining 44 without ventricular tachycardia (non-VT group). Initially, the VT group had significantly greater left ventricular end-diastolic dimension (LVDd), smaller % fractional shortening (FS), and normalized rapid felling rate (RFR) compared with the other two groups. However, there were no significant differences in the echocardiographic parameters between the SD and non-VT groups. During the follow-up period, no changes in echocardiographic parameters were observed in the non-VT group. However, LVDd was increased in both the SD and VT groups, and normalized RFR and atrial filling rate (AFR) were decreased in the SD group. These results suggested that close observations using echocardiography are useful for evaluating the natural history and prognoses of HCM.