Hypertrophic nonobstructive cardiomyopathy with giant negative T waves (apical hypertrophy): ventriculographic and echocardiographic features in 30 patients

In 30 of 1,002 consecutive patients who had left heart catheterization and cineangiography for evaluation of either ischemic heart disease or cardiomyopathy the electrocardiogram showed giant negative T waves (greater than 10 mm) associated with high QRS voltage (R wave greater than 26 mm in lead V₅ or the sum of the S wave in lead V₁ and the R wave in lead V₅ 35 mm or more) in the precordial leads despite absence of hypertension or significant coronary artery disease. In all 30 patients a characteristic spade-like configuration (concentric apical hypertrophy) was observed in the right anterior oblique ventriculogram at end-diastole as well as in the long axis two dimensional echocardiogram.The average apical thickness in these patients (24.8 ± 6.6 mm) was significantly greater than that in normal subjects (9.4 ± 3.1 mm) (P < 0.001) or in patients with hypertrophic obstructive cardiomyopathy (14.7 ± 5.0 mm) (P < 0.001). Values for both the mid anterior free wall thickness (13.9 ± 4.1 mm) and the mid posterior free wall thickness (14.3 ± 3.0 mm) were greater than values in normal subjects (8.9 ± 1.8 mm and 8.2 ± 2.0 mm, respectively) (P < 0.001). However, the ratio between the apical and the mid anterior free wall thickness in these 30 patients (1.86 ± 0.53) was significantly greater than the ratio in normal subjects (1.05 ± 0.24), patients with hypertrophic obstructive cardiomyopathy (0.96 ± 0.15) (P < 0.001) and patients with types of nonobstructive hypertrophic cardiomyopathy (1.26 ± 0.24) (P < 0.005) other than apical concentric hypertrophy. Obstruction of the tract did not occur because the upper half of the septem remained rather thin in systole and did not bulge into the left ventricle during systole. Pressure study with proper provocations as well as two dimensional echocardiograms revealed no peak systolic pressure gradient or obstruction within the outflow tract of the left ventricle.It is concluded that these 30 patients have nonobstructive hypertrophic cardiomyopathy with marked concentric hypertrophy in the apex (apical hypertrophic type) and with a different septal shape and contraction pattern from those seen in the obstructive type. This type of hypertrophy appears to be a fairly common type of hypertrophic cardiomyopathy in Japan.     

ECG pattern of left ventricular hypertrophy in nonobstructive hypertrophic cardiomyopathy: the significance of the mid-precordial changes.

A review of electrocardiograms from 33 patients with nonobstructive hypertrophic cardiomyopathy was made. In 22 patients there was noted a high QRS voltage, depression of the ST segment, and inversion of the T wave, satisfying the diagnostic criteria of left ventricular hypertrophy with the abnormal changes not only extending to the midprecordial leads but showing the most striking abnormal changes in Lead V4 in 20 patients. The frontal plane electrical axis was normal (around 60 degrees), with the most remarkable changes in Lead II. In the VCG, the magnitude of the QRS loop was increased and directed anteriorly and to the left, and the T loop was deviated posteriorly and to the right opposite the QRS loop. The asymmetric septal and apical hypertrophy was noted on echocardiography and/or angiocardiography. The coronary arteries were normal without significant obstruction in selective coronary angiography. It was postulated that the asymmetric septal and apical hypertrophy was reflected in this ECG pattern. The recognition of this ECG pattern provides pertinent information in the clinical detection of nonobstructive HCM.     

Anterior QRS loop in hypertrophic cardiomyopathy.

Frank vectorcardiograms (VCGs) were reviewed in 45 patients with hypertrophic cardiomyopathy (HCM), 26 with obstruction and 19 without obstruction. Twelve of the 19 patients without obstruction and five of the 26 patients with obstruction were found to have predominantly anterior QRS loops. Fourteen patients had a large left anterior QRS loop with increased anterior and leftward force; the posterior and terminal rightward force were within the normal range, and the T loops were displaced posteriorly and to the right opposite to the QRS loop. Asymmetric septal and apical hypertrophy were noted echocardiographically and/or angiographically. Increased electrical force from the asymmetric hypertrophy of the septal and apical area is proposed to explain this large left anterior loop. Three patients had a QRS loop located anteriorly and to the right with electrocardiograms (ECGs) resembling those of posterolateral myocardial infarction or right ventricular hypertrophy. These finding suggest that (1) hypertrophic cardiomyopathy may be another cause of an anterior QRS loop; (2) the recognition of the large left anterior loop in the VCG in patients with a left ventricular hypertrophy pattern in the ECG is helpful in the diagnosis of HCM, especially the nonobstructive form; and (3) hypertrophic cardiomyopathy should be considered in the differential diagnosis of myocardial infarction or right ventricular hypertrophy.     

Significant X-ray patterns in cardiomyopathy--an approach improving noninvasive diagnosis

To identify their characteristic patterns, the X-ray dorso-ventral chest images of 118 patients with cardiomyopathy (63--dilated cardiomyopathy, 25--hypertrophic obstructive cardiomyopathy, 30--hypertrophic nonobstructive cardiomyopathy) were analyzed and compared with those of 22 patients without cardiac abnormalities. All 140 patients underwent comprehensive invasive cardiac examinations. In dilated and hypertrophic nonobstructive cardiomyopathy transvasal endomyocardial biopsy was performed. Irrespective of the type and the aetiology of cardiomyopathy, 4 characteristic patterns of X-ray findings could be identified: a class of left heart abnormality (class L) and 3 types of bilateral cardiac abnormalities with various stages of left heart failure (class L + R). An association between the image patterns and haemodynamic parameters (left ventricular end-diastolic pressure, wall mass index, pulmonary mean pressure) was demonstrated which was most obvious in dilated and less evident in hypertrophic obstructive cardiomyopathy. Mitral insufficiency in all types and classes frequently corresponded with the X-ray findings of left atrial enlargement. The X-ray classification method proved to be an efficient approach and superior to the heart-to-lung ratio.     

HLA-DR3 antigen linkage in patients with hypertrophic obstructive cardiomyopathy

In order to investigate if genetic factors could be involved in the pathogenesis of hypertrophic obstructive cardiomyopathy, we determined HLA-A, HLA-B, HLA-C, and HLA-DR specificities in 12 Italian patients affected with the disease and in healthy family members of one of them. HLA-DR3 was found in 50% of patients as compared to 17.1% of normal control subjects (p = 0.023, relative risk = 4.82). The two relatives also had HLA-DR3 antigen and, in addition, showed equivocal signs of hypertrophy at echocardiographic examination. Thus hypertrophic obstructive cardiomyopathy is associated with genes in the HLA-DR region, and immunogenetic factors could be involved in the pathogenesis of the disease. Furthermore, the minimal target organ abnormalities in "healthy" relatives could represent a subclinical stage of the disease.     

The importance of echocardiography for the diagnosis of cardiomyopathies in comparison to cardio-computer tomography and to complex invasive heart diagnostics

112 patients with various forms of cardiomyopathy (typical-subaortic--hypertrophic obstructive; atypical--medioventricular--hypertrophic obstructive; hypertrophic nonobstructive; apical hypertrophic; dilatative) were subjected to echocardiographic examination of the morphological and functional state of the heart. The results were compared with the results of computer heart tomography and comprehensive invasive examination. The high diagnostic reliability of ultrasonic diagnostics has been confirmed. Invasive examination is necessary in hypertrophic nonobstructive and in dilatative cardiomyopathies. Computer heart tomography is suitable for complementing findings and for clarifying contradictory results of examination, especially in apical forms of cardiomyopathy.     

静脉注射美托洛尔对肥厚型心肌病左心室功能的影响

目的:应用频谱多普勒超声心动图技术,定量观测肥厚型梗阻性心肌病和肥厚型非梗阻性心肌病患者经静脉注射美托洛尔前后左心室功能和左心室流出道压力阶差的变化,并观察血流动力学的变化,探讨静脉注射美托洛尔对肥厚型心肌病左心室功能的影响。方法:应用PHILIPS-SONOS7500型彩色多普勒超声诊断仪,测量用药前和用药后10分钟肥厚型梗阻性心肌病组(n=33)和肥厚型非梗阻性心肌病组(n=26)患者左心室功能各指标,并监测用药过程中的血流动力学变化。结果:肥厚型梗阻性心肌病组患者用药后较用药前左心室舒张功能明显改善,左心室流出道(LVOT)明显增宽(P<0.05),左心室流出道压力阶差(LVOTPG)明显下降(P<0.05),EF值无明显变化(P>0.05);肥厚型非梗阻性心肌病组患者用药后较用药前上述各指标无明显变化(P>0.05)。两组的心率、收缩压、舒张压用药后较用药前均明显降低(P<0.05),有显著差异。结论:静脉注射美托洛尔能够快速改善肥厚型梗阻性心肌病组患者的左心室舒张功能,改善临床症状,明显减轻左心室流出道梗阻,降低压力阶差,明显降低两组的血压、心率,影响其血流动力学;而对肥厚型非梗阻性心肌病组患者无明显作用,对两组的收缩功能均无明显影响。     

Idiopathic dilated cardiomyopathy: familial prevalence and HLA distribution.

OBJECTIVES: To compare HLA distribution in familial and non-familial dilated cardiomyopathy, because a serum marker that could identify families at risk of developing dilated cardiomyopathy should be of use in screening for the disease. PATIENTS: 100 patients with dilated cardiomyopathy. METHODS: 200 first degree relatives from 56 of the proband families were screened for dilated cardiomyopathy by echocardiography. The HLA profile of the patients with dilated cardiomyopathy, as well as of the familial and non-familial subgroups, was compared with that of 9000 normal controls. RESULTS: The familial prevalence of dilated cardiomyopathy in this patient group was "definite" in 14 of 56 (25%) and "possible" in 25 of 56 (45%). The HLA-DR4 frequency in the 100 patients with dilated cardiomyopathy was similar to that in the 9000 controls (39% v 32%). However, the DR4 subtype was significantly more common in the 25 probands with a familial tendency to dilated cardiomyopathy than in the 31 probands with non-familial dilated cardiomyopathy (68% v 32%; P < 0.05). CONCLUSIONS: The present finding supports an HLA linked predisposition to familial dilated cardiomyopathy. The HLA type DR4 was significantly more common in familial than in non-familial cases. The DR4 halotype was associated with two thirds of the families at risk for dilated cardiomyopathy.     

Lack of correlation between HLA haplotypes and familial hypertrophic cardiomyopathy

In order to confirm the recent observations of the particular incidence of some HLA antigens in the familial hypertrophic cardiomyopathy we have determined the HLA-A, B and C antigens in the components of a family with high prevalence of hypertrophic cardiomyopathy (10 males and 7 females, age from 10 to 75 years). Six patients were affected by the disease. In one case (sudden death) the diagnosis was established by necroscopy; in the other living subjects by the characteristic echocardiographic features after having ruled out the conditions which can result in secondary myocardial hypertrophy. HLA typing showed the A9, Bw35, Cw4 aplotype in 4 of the 5 living patients affected by hypertrophic cardiomyopathy (2 with obstruction). This aplotype however was not shared by the other patient and was found in one healthy member of the family. Two patients affected by hypertrophic cardiomyopathy without obstruction did not share any aplotype while the 2 subjects with obstructive hypertrophic cardiomyopathy had the same aplotype of a healthy member of the family. Accordingly the genetic study of this family does not allow to establish a steady correlation between hypertrophic cardiomyopathy and HLA aplotypes.     

Human leukocyte antigens in hypertrophic cardiomyopathy patients in South India

Hypertrophic cardiomyopathy is characterized by massive ventricular hypertrophy, reduced diastolic function, and excessive ventricular contraction. The human leukocyte antigens HLA-A, HLA-B, and HLA-DR were studied in 14 hypertrophic cardiomyopathy patients with left ventricular obstruction from South India. They were compared with 81 normal age- and sex-matched individuals from the same ethnic background. The human leucocyte antigens were identified using the standard serological assay with a longer incubation for DR antigens. The odds ratio, frequency, chi-squared value, p-value, etiological fraction, preventive fraction, and haplotype frequency estimates were calculated. The HLA-B51 and HLA-DR2 levels were significantly increased in hypertrophic cardiomyopathy patients compared to controls, whereas HLA-A19, HLA-B7, and HLA-DR4 were decreased when compared to the controls. It was noticed that haplotype B51-DR2-DQ3 was significantly associated with hypertrophic cardiomyopathy patients from South India. Hypertrophic cardiomyopathy may be associated with genes in the human leukocyte antigen region, and immunogenetic factors linked to human leukocyte antigens appear to play a major role in the pathogenesis.     

M mode echocardiography in hypertrophic cardiomyopathy: Diagnostic criteria and prediction of obstruction

To assess the reliability of the classic echocardiographic features in the heart with hypertrophic cardiomyopathy as criteria that differentiate it from normal heart and as predictors of outflow tract obstruction versus nonobstruction, 70 patients with clinical and angiographic evidence of hypertrophic cardiomyopathy were studied with M mode echocardiography. The diagnostic sensitivity and specificity of the classic features were assessed: ventricular septal thickness, 83 and 94 percent (sensitivity and specificity, respectively); ventricular septal amplitude of movement, 71 and 89 percent; ventricular septal thickness to left ventricular posterior wall ratio, 79 and 94 percent; left ventricular end-systolic dimension, 54 and 86 percent; septal-mitral valve distance at the onset of systole, 29 and 100 percent; systolic anterior motion of the mitral valve, 61 and 100 percent; and mid systolic closure of the aortic valve, 61 and 100 percent.No single M mode echocardiographic feature was consistently abnormal in hypertrophic cardiomyopathy. In nonobstructive hypertrophic cardiomyopathy, ventricular septal thickness greater than or equal to 13 mm (sensitivity 68 percent and specificity 94 percent) and ventricular septal thickness to posterior wall ratio greater than or equal to 1.5 (sensitivity 82 percent and specificity 94 percent) were the individual features with the greatest diagnostic value from the norm. Patients with obstruction at rest and labile obstruction (gradient only on provocation) had echocardiographically identical features. Ventricular septal thickness greater than or equal to 13 mm plus systolic anterior motion of the mitral valve or mid systolic closure of the aortic valve were the features that in combination best differentiated obstructive (resting and labile) from nonobstructive hypertrophic cardiomyopathy (sensitivity 82 percent and specificity 68 percent) and the heart with obstructive hypertrophic cardiomyopathy from the normal heart (sensitivity 82 percent and specificity 100 percent).     

Anti-beta-receptor antibodies in human dilated cardiomyopathy and correlation with HLA-DR antigens

The mechanisms responsible for the decline in the density of beta-adrenoceptors in the failing myocardium have not been adequately defined. It is a possibility that the nature of the process leading to heart failure may determine, in large part, the pathogenesis of this decline. Sera of some patients with dilated cardiomyopathy contain antibodies directed against the beta-adrenoceptor, as judged by ligand binding inhibition, immunoprecipitation and immunoblotting assays. Because deranged immune function is thought to play a role in dilated cardiomyopathy, immunogenetic markers of the propensity to develop anti-beta-receptor antibodies were sought. The prevalence of HLA-DR4 was significantly higher in dilated cardiomyopathy patients (40 vs 24% in 511 normal subjects, pc less than 0.001). In contrast, no association was found between HLA phenotypes and alcoholic cardiomyopathy. Furthermore, 72% (13 of 18) of the HLA-DR4 dilated cardiomyopathy patients had anti-beta-receptor antibodies compared to 22% (7 of 33) HLA-DR4-negative patients; in the latter, presence of antibody was linked to the HLA-DR1 phenotype. Conversely, 67% (15 of 23) of the antibody-positive patients were typed as HLA-DR4 compared to only 10% of the antibody-negative patients. Interestingly, none of the 23 antibody-positive patients were typed as HLA-DR3 while 37% of the antibody-negative did. Only 25% of alcoholic cardiomyopathy patients had anti-beta-receptor antibodies and no preponderant HLA association could be demonstrated. These results suggest that the presence of anti-beta-receptor antibodies in patients with idiopathic dilated cardiomyopathy may be under the control of the major histocompatibility locus.     

HL-A and hypertrophic cardiomyopathy.

HL-A antigens were determined in 26 unrelated Japanese patients with hypertrophic cardiomyopathy. Several antigens were more common in patients compared with controls, but statistically significant differences were not evidenced. We also studied two families in which many had a hypertrophic cardiomyopathy. All the affected individuals revealed HL-A-A9 and B7, while none among the unaffected family members had HL-A-B7. Our findings suggest that the HLA-A system may play some role in the pathogenesis of hypertrophic cardiomyopathy with familial occurrence.     

Do specific HLA antigens predispose to ischaemic heart disease or idiopathic dilated cardiomyopathy?

BACKGROUND--The aetiology of idiopathic dilated cardiomyopathy is believed to have an immunological component. Association with human leucocyte antigens (HLAs) has been previously reported, particularly with HLA-DR4. AIM--To determine the association of HLA type with diagnosis in a group of patients assessed for heart transplantation. METHODS--A comparison was made of frequencies of HLA types in patients with a diagnosis of idiopathic dilated cardiomyopathy or (n = 98) ischaemic heart disease (n = 170) and in controls from the North Western region (n = 857). RESULTS--Neither the patients with idiopathic dilated cardiomyopathy nor those with ischaemic heart disease showed a significant increase or decrease in any HLA frequency compared with the controls. CONCLUSION--These results suggest that there is no HLA association with idiopathic dilated cardiomyopathy or ischaemic heart disease. This conflicts with the results of some previous studies.     

Comparison of coronary flow velocities between patients with obstructive and nonobstructive type hypertrophic cardiomyopathy: noninvasive assessment by transthoracic Doppler echocardiography

BACKGROUND: We aimed to compare coronary flow velocity (CFV) measurements of patients with nonobstructive (NHCM) and obstructive hypertrophic cardiomyopathy (HOCM) by using transthoracic Doppler echocardiography (TTDE). METHODS AND RESULTS: In 11 patients with NHCM and 26 with HOCM, CFV in the distal left anterior descending (LAD) coronary was measured by TTDE (3.5 MHz) under the guidance of color Doppler flow mapping in addition to standard 2D and Doppler echocardiography. The results were compared with 24 normal participants who had no evidence of cardiac disease. Peak diastolic velocity of LAD was also higher in NHCM and HOCM than controls (52 +/- 14 cm/sec and 54 +/- 20 cm/sec vs 41 +/- 11 cm/sec, respectively, P < 0.01). The analysis of systolic velocities revealed abnormal flow patterns in 16 (61%) patients with HOCM (12 systolic-reversal flow and 4 no systolic flow) and 6 (54%) (5 reversal flow and 1 zero flow) patients with NHCM (-11 +/- 30 cm/sec and -13 +/- 38 cm/sec, vs 24 +/- 9 cm/sec, respectively, P < 0.001). Linear regression analysis demonstrated no correlation between intraventricular pressure gradient and coronary flow velocities in HOCM patients. However, there were significant positive and negative correlations between septal thickness and diastolic and systolic velocities, respectively (r = 0.50, P < 0.002, and r =-0.43, P < 0.005). CONCLUSION: We conclude that the coronary flow velocity abnormalities are independent from the type of hypertrophic cardiomyopathy.     

Antiarrhythmic drug, cibenzoline, can directly improve the left ventricular diastolic function in patients with hypertrophic cardiomyopathy.

The effect of cibenzoline on left ventricular diastolic function was investigated in patients with hypertrophic cardiomyopathy (HCM). Before and 2 h after an oral administration of 200 mg of cibenzoline, echocardiographic, apexcardiographic and gated radionuclide angiographic studies were performed in 12 patients with hypertrophic obstructive cardiomyopathy (HOCM) and 7 with hypertrophic nonobstructive cardiomyopathy (HNCM). After administration of cibenzoline, the left ventricular pressure gradient decreased from 96+/-33 mmHg to 29+/-22 mmHg (<0.0001). Fractional shortening decreased from 53.3+/-7.5 to 45.4+/-6.2% (p=0.0008) in patients with HOCM and from 49.9+/-8.7 to 40.9+/-7.5% (p=0.0039) in patients with HNCM. On the other hand, E-wave velocity increased and A-wave velocity decreased in both groups. The time between the second heart sound and O point was shortened from 253+/-53 to 176+/-21 ms (p<0.0001) in patients with HOCM and from 245+/-54 to 185+/-44 ms (p=0.0050) in patients with HNCM. The time to peak filling rate was shortened from 248+/-79 to 190+/-40 ms (p=0.0072) in patients with HOCM and from 218+/-33 to 163+/-26 ms (p=0.0052) in patients with HNCM. These results indicate that in patients with HCM, cibenzoline suppresses left ventricular systolic function, but can markedly improve left ventricular diastolic dysfunction through its direct action.     

Relation of electrocardiographic abnormalities and patterns of left ventricular hypertrophy identified by 2-dimensional echocardiography in patients with hypertrophic cardiomyopathy

Distribution of left ventricular (LV) hypertrophy was assessed by wide-angle, 2-dimensional (2-D) echocardiography in 153 patients with hypertrophic cardiomyopathy and compared with the scalar electrocardiogram in the same patients. The most common electrocardiographic alterations were S-T segment changes and T-wave inversion (61%), LV hypertrophy (47%), abnormal Q waves (25%), and left atrial enlargement (24%). LV hypertrophy on the electrocardiogram was significantly more common in patients with the most extensive distribution of LV hypertrophy on 2-D echocardiogram involving substantial portions of both the ventricular septum and LV free wall (type III; 51 of 69, 74%) than in those with more limited distribution of LV hypertrophy (21 of 84, 25%; p less than 0.001). Most patients with hypertrophic cardiomyopathy and normal electrocardiograms (13 of 23) had localized (type I) hypertrophy, but only 4 had the extensive type III pattern of hypertrophy. Abnormal Q waves were significantly more common in those patients without hypertrophy of the anterior, basal septum (type IV; 15 of 27, 56%) than in those with basal septal hypertrophy (23 of 126, 18%; p less than 0.001); abnormal Q waves were uncommon in extensive type III distribution of hypertrophy (13 of 69, 19%). Thus, although no single electrocardiographic abnormality is characteristic of hypertrophic cardiomyopathy, 2-D echocardiography clarifies the significance of certain electrocardiographic patterns: (1) LV hypertrophy on the electrocardiogram, although present in only about half of the study group, was a relatively sensitive (74%) marker for extensive (type III) LV hypertrophy; (2) abnormal Q waves cannot be explained by ventricular septal hypertrophy alone; and (3) a normal electrocardiogram was most commonly a manifestation of localized LV hypertrophy.     

Vector Flow Mapping in Obstructive Hypertrophic Cardiomyopathy to Assess the Relationship of Early Systolic Left Ventricular Flow and the Mitral Valve

Background

The hydrodynamic cause of systolic anterior motion of the mitral valve (SAM) is unresolved.

Objectives

This study hypothesized that echocardiographic vector flow mapping, a new echocardiographic technique, would provide insights into the cause of early SAM in obstructive hypertrophic cardiomyopathy (HCM).

Methods

We analyzed the spatial relationship of left ventricular (LV) flow and the mitral valve leaflets (MVL) on 3-chamber vector flow mapping frames, and performed mitral valve measurements on 2-dimensional frames in patients with obstructive and nonobstructive HCM and in normal patients.

Results

We compared 82 patients (22 obstructive HCM, 23 nonobstructive HCM, and 37 normal) by measuring 164 LV pre- and post-SAM velocity vector flow maps, 82 maximum isovolumic vortices, and 328 2-dimensional frames. We observed color flow and velocity vector flow posterior to the MVL impacting them in the early systolic frames of 95% of obstructive HCM, 22% of nonobstructive HCM, and 11% of normal patients (p < 0.001). In both pre- and post-SAM frames, we measured a high angle of attack >60° of local vector flow onto the posterior surface of the leaflets whether the flow was ejection (59%) or the early systolic isovolumic vortex (41%). Ricochet of vector flow, rebounding off the leaflet into the cul-de-sac, was noted in 82% of the obstructed HCM, 9% of nonobstructive HCM, and none (0%) of the control patients (p < 0.001). Flow velocities in the LV outflow tract on the pre-SAM frame 1 and 2 mm from the tip of the anterior leaflet were low: 39 and 43 cm/s, respectively.

Conclusions

Early systolic flow impacts the posterior surfaces of protruding MVL initiating SAM in obstructive HCM.     

Clinical aspects, course and prognosis of various forms of hypertrophic cardiomyopathy

To characterize the different types of hypertrophic cardiomyopathy [typical (subaortic) hypertrophic obstructive cardiomyopathy (HOCM) (n = 235), atypical (midventricular or apical) HOCM (n = 33), and hypertrophic nonobstructive cardiomyopathy (HNCM) (n = 85)], studies of the clinical picture, course, and prognosis were performed in 353 patients. Clinical picture. There were found to be distinct differences between the diseases in terms of incidence, symptoms, findings at auscultation, carotid pulse tracings, ECG (incidence of abnormal negative T-waves), and echocardiography. Echochardiography proved to be diagnostically less specific than invasive methods, however, and in particular often failed to distinguish between atypical HOCM and HNCM. Clinical course. There was no evidence of a change from one form of hypertrophic cardiomyopathy to the other. The Sokolow-Lyon index in the ECG did not increase in any group. The rate of complications (endocarditis, systemic emboli) varied between 0.61 and 1.28 events per 100 patient years. Only 7.4%-23.5% of patients with HOCM improved as a result of conservative treatment, compared to 83%-87.5% of surgical patients. In addition, the rate of postoperative syncope was reduced by 90%. Prognosis. 90 patients were operated on. Operative mortality in typical HOCM was 4.5% over the last 5 years. If this is disregarded, the cumulative survival rates are significantly higher in surgical patients with typical HOCM than in those treated conservatively. The data confirm surgical treatment to be the therapy of choice in patients with HOCM refractory to conservative treatment. The prognosis seems to be improved by operation.     

Left ventricular function in patients with hypertrophic nonobstructive cardiomyopathy

From 1970 to 1977 the diagnosis of hypertrophic, nonobstructive cardiomyopathy was made by clinical, echocardiographic and hemodynamic findings in 13 patients (2 females and 11 males). Echocardiography showed asymmetric septal hypertrophy in 10 of the 12 evaluated patients (83%) and symmetric hypertrophy of the septum and the posterior wall in 2 patients (17%). Systolic anterior motion of the anterior mitral leaflet was found in 5 patients (42%) although there was no obstruction of the outflow tract. Significantly higher angiographic ejection parameters were present in the 13 patients with cardiomyopathy compared to 13 control patients and the angiographic lateral wall thickness was significantly larger in patients with cardiomyopathy than in controls. The clinical course was followed during 36 mth; a stable course was found in 8 (group A) and a deterioration in 5 patients (group B). Physical working capacity and left ventricular systolic ejection fraction were significantly higher in group A than in group B. Ejection fraction in group B was similar to that of the controls whereas physical working capacity was depressed. Thus, in nonobstructive cardiomyopathy a higher than normal ejection fraction is needed to maintain a normal physical working capacity. Th increased ejection performance seems, however, not to be related to an increase in muscle fiber shortening, but can be explained by geometric reasons.