Clinical aspects and diagnostic criteria in non-obstructive symmetric hypertrophic myocardiopathy

We describe the clinical data, the standard and Holter electrocardiographic observations, the carotid pulse tracings, the M-mode and B-mode echocardiographic findings and the left ventriculographic aspects of 21 patients with non-obstructive symmetric hypertrophic cardiomyopathy (NOSHCM). NOSHCM was diagnosed when there was echocardiographic and/or left ventriculographic evidence of septal and posterior wall hypertrophy without signs of left ventricular outflow destruction (LVOTO). Compared to the asymmetric hypertrophic obstructive cardiomyopathy (AHOCM), NOSHCM reveals reduced excursion of the posterior wall of the left ventricle, whereas patients with AHOCM have more frequently systolic murmurs and carotid pulse changes suggestive of LVOTO. Proper classification and treatment of NOSHCM are discussed.     

Parathyroid function in hypertrophic myocardiopathy]

It has been suggested that hypertrophic cardiomyopathy (HCM) may be associated with hyperparathyroidism. We evaluated parathyroid function in 15 patients with HCM and 14 patients with primary dilated cardiomyopathy (DCM), measuring different parameters of calcium metabolism (total serum calcium, ionized calcium, PTH, vitamin D metabolites, alkaline phosphatase, osteocalcin). As a group PTH levels were normal in HCM (intact PTH 3.9 +/- 1.6 pmol/l, midmolecular PTH 59 +/- 13 pmol/l and carboxyterminal PTH 0.6 +/- 0.4 ng/ml), but in 3 patients carboxyterminal PTH levels were persistently higher than normal, while all other parameters of calcium metabolism were normal. We conclude that parathyroid function is normal in patients with HCM, although some of them may have an abnormal secretion and/or metabolism of carboxyterminal PTH fragments, or a circulating substance the interferes with this PTH assay. Parathyroid function in DCM patients was normal, except in a patient who had hyperparathyroidism secondary to vitamin D deficiency.     

Obstructive hypertrophic myocardiopathy and coronary atherosclerosis

The purpose of this study was to determine the coexistence of idiopathic hypertrophic cardiomyopathy (IHC) and atheromatous coronary artery disease. We studied forty six patients with IHC documented by complete clinical study and also by left heart catheterization. The diagnosis was considered established, when the patient had a significant left intraventricular pressure gradient (LIPG) and by angiographic and or echocardiographic demonstration of systolic anterior movement of the mitral valve and asymmetric septal hypertrophy. In 15 patients a selective coronary angiography was performed because ischemic myocardial disease was suspected. There were eight patients with significant atherosclerotic obstruction (greater than 75% narrowing). There was one vessel disease in 13%, two vessels in 37% and three vessels in 50% of the patients. The LIPG was 43 +/- 20 mmHg, the left ventricular and diastolic pressure (LVEDP) was 18 +/- 11 mmHg in patients with atheromatous disease and there was no significant difference with the patients with normal coronary arteries with LIPG 52 +/- 31 mmHg and LVEDP 21 +/- 9 mmHg. Our data demonstrate that ischemic heart disease in patients with idiopathic hypertrophic cardiomyopathy is related not only to increase of the cardiac mass and/or the ventricular wall stress, but in some of them atherosclerosis plays a role in its pathogenesis. Significant differences were found between the aged in both groups. In the patients who had coronary atherosclerosis the mean age was 54 years; on the other hand in the patients with normal coronary arteries the mean age was 44 years. This last fact could explain the high frequency of atherosclerosis in the older patients.     

Non-obstructive hypertrophic myocardiopathy. Apropos of 5 cases

Out of a total of 1 470 cardiac catheterisations performed in one year, 5 were motivated by the chance finding of giant negative T waves, greater than 10 mm and maximal in leads V3, V4. The patients were all males with an average age of 50 +/- 3,2 years. Four were asymptomatic and the other had scleroderma. The changes partially regressed on exercise electrocardiography. M mode echocardiography showed normal septal (8,8 +/- 0,25 mm) and posterior wall (8,38 +/- 0,21 mm) thickness and a normal septum/posterior wall ratio (1,02 +/- 0,01). Coronary angiography was normal in all cases. Left ventricular and diastolic pressure was raised after ventriculography (21 +/- 1,34 mm Hg). There was no intraventricular pressure gradient. Left ventriculography in the 30 degrees right anterior oblique plane showed an end diastolic appearance similar to that of the ace of spades and the apex was obliterated in systole. End diastolic volumes (68 +/- 4,7 ml/m2) and ejection fractions (68,6 +/- 5,21) were normal. The wall thickness measured from the angiography was 10,4 +/- 0,81 mm at the mid zone of the anterior wall and 17,2 +/- 0,85 at the apex with a septum / posterior wall ratio of 1,67 +/- 0,08. In three cases, atrial pacing with coronary arterial and venous lactate sampling revealed abnormalities in myocardial metabolism. The final diagnosis was of non obstructive apical hypertrophic cardiomyopathy. The site of hypertrophy explains the electrical changes. The prognosis of this cardiomyopathy is still unknown, one regression and two aggravations of the electrical changes have been observed. continuous 24 hour ECG showed an episode of ventricular tachycardia in 1 patient, which would imply a reserved prognosis.     

Apical hypertrophic myocardiopathy. Apropos of 10 cases

Apical hypertrophic cardiomyopathy is a recently defined subgroup of primary hypertrophic cardiomyopathy. Ten new cases are studied. The electrical features (anomalies of repolarisation) were constant and giant negative T waves were observed in 3 out of 10 cases. Two-dimensional ultrasonography confirmed the diagnosis in every case; the optimal views consisted of 4 cavity scans obtained by the apical approach. When angiography was performed (7 cases out of 10), it confirmed the ultrasonographic findings and was able to eliminate any coronary artery lesions. The patients had a mean age of 48.3 years with a marked male predominance (sex ratio of 0.8) and all of the patients are alive with a mean follow-up of 3.4 years. The functional handicap was moderate (class III: 1 case, class II: 5 cases and class I: 4 cases) and did not progress during the period of follow-up. The family surveys performed revealed that apical hypertrophy can be integrated into the spectrum of segmental hypertrophic cardiomyopathy. In two cases, this survey revealed the coexistence in the same family of segmental hypertrophy with different topography. The long term prognosis of this disease is unknown and the authors consider that the risk of ventricular arrhythmia justifies routine rhythmological evaluation in order to guide subsequent treatment.     

Apical hypertrophic myocardiopathy with mid-ventricular obstruction and apical necrosis

In a 41-year old man hospitalized for investigation of a systolic ejection murmur, echocardiography revealed an apical (17 mm) and, chiefly, a mid ventricular (38 mm) myocardial hypertrophy resulting in a true stricture at that level. During systole the mid ventricular area became obstructed, dividing the left ventricule into an apical akinetic chamber and a proximal hyperkinetic chamber. Left ventriculography confirmed this abnormality and catheterization showed a left intraventricular pressure gradient of 60 mmHg. Although left coronary arteriography proved normal, myocardial radionuclide scanning at exercise displayed apical necrosis which resulted in a peculiar haemodynamic behaviour well demonstrated by doppler ultrasound. The apex of the left ventricle was filled in two stages: first, during atrial contraction, giving at doppler (mid ventricular sample) a positive flow with a speed of 1m/sec during 120 ms; then, during the isovolumetric period, giving a positive flow with a speed of 1m/sec during 60 ms. The apex drained during systole and protodiastole, giving a negative mid ventricular obstruction flow with a speed of 4 m/sec with protosystolic and mesodiastolic reinforcement.     

Intermediate-term trial treatment of arrhythmia in hypertrophic myocardiopathy with sotalol

Unlike amiodarone, beta-blockers have proved ineffective in the treatment of severe ventricular rhythm disorders encountered in hypertrophic myocardiopathy. We tried to evaluate the efficacy of sotalol, a betablocker with anti-arhythmic properties, like amiodarone, on the supraventricular and ventricular rhythm disorders of this disease. 13 patients (6 men and 7 women, mean age 53.5 +/- 26.5 years) were evaluated with a 48 hours electrocardiographic recording, before and 8 and 120 days after a sotalol treatment at a mean dose of 290 mg/day (160 to 640 mg). The diagnosis of hypertrophic myocardiopathy was established on standard clinical, phonomecanographic (13 cases), sonocardiographic (13 cases) and haemodynamic (9 cases) criteria. Five patients presented bursts of supraventricular tachycardia on DO. Their number decreased to 4 on D8 and to 3 to D120. Six patients presented Lown's class III or IV ventricular rhythm disorders on DO. Their number went from 2 on D8 to 4 on D120. Therefore, in this short series, sotalol has shown a non-negligible efficacy on the rhythm disorders encountered in hypertrophic myocardiopathy.     

Value of Doppler-echocardiography in the prognosis and follow up of hypertrophic myocardiopathy

INTRODUCTION AND OBJECTIVES. The aim of this study was to assess the value of Doppler-echocardiogram for the prognosis and follow up of a hospital-based series of adult patients with hypertrophic cardiomyopathy (HC). METHODS. One-hundred nineteen consecutive patients with HC (52 +/- 12 years, 60 women) were studied over a follow up period of 9.7 +/- 6.7 years. Echocardiographic evolution was analyzed in 104 patients (67 with dynamic obstruction) who had, at least, two echocardiograms performed within an interval of 3.7 +/- 3 years (1 to 7 years). RESULTS. Seven patients died during follow up and 31 patients developed severe complications (7 deaths, 15 syncopes, 4 class IV angina, 3 class IV dyspnea and 2 acute myocardial infarctions). The presence of mitral insufficiency (p = 0.001) and dynamic gradient > 50 mmHg (p = 0.02) were predictive of mortality and a left atrial index > 25 mm/m2 was predictive (p = 0.028) of severe complications. Fifteen percent of the patients without dynamic obstruction in the first Doppler-echo showed a gradient > 25 mmHg in the last echo. A greater number of patients with mitral insufficiency (80% vs 66%; p = 0.01) and an increase in its severity (p = 0.038) was observed during follow up. CONCLUSIONS. Mitral insufficiency, a dynamic gradient > 50 mmHg and a left atrial index > 25 mm/m2 are variables of a bad prognosis in adult patients with HC. An evolution to obstructive HC was observed in 15% of non obstructive HC, and a tendency to increased severity of mitral insufficiency was observed during follow up.     

Medical treatment of hypertrophic myocardiopathy. Comparison of propranolol and verapamil

It remains difficult to make a reasoned choice between betablockers and calcium antagonists in the medical treatment of hypertrophic cardiomyopathy. In order to help in making this choice, we compared the effects of 320 mg of propranolol and 480 mg of verapamil, prescribed in a random order for an average period of 2.5 months in 24 patients. In 15 patients the two therapeutic sequences were preceded and followed by a clinical examination and an exercise stress test after withdrawal of all treatment. The overall functional status correlated to oxygen consumption was unchanged by the two drugs, but patients felt better more often with verapamil. These was no significant change in maximal power developed during exercise testing although verapamil increased it by 9 p. 100 throughout with a corresponding increase in oxygen consumption. Two parameters were significantly changed by both drugs: the maximal heart rate fell from 161 +/- 22 to 122 +/- 18 with propranolol, and to 145 +/- 28 with verapamil; the oxygen pulse (equivalent to the systolic index) increased from 10.9 +/- 2.2 to 14.8 +/- 3.9 with propranolol, and to 13.7 +/- 2.4 with verapamil. There were no changes in the blood pressure profiles during exercise. Side effects were observed with both drugs; muscular weakness was the biggest problem with propranolol and sinus node dysfunction with verapamil.(ABSTRACT TRUNCATED AT 250 WORDS)     

Intravenous verapamil in hypertrophic myocardiopathy. A study using Doppler ultrasound

The effect of one intravenous dose of verapamil on left ventricular diastolic and systolic flow was studied by Doppler-echocardiography in 31 patients with hypertrophic cardiomyopathy. On diastolic flow, verapamil induced a decrease in "a" wave velocity (1.02 + 0.37 vs 0.91 + 0.29 m/seg, p less than 0.01), and in its relation with maximal protodiastolic velocity (1.08 + 0.56 vs 0.89 + 0.37, p less than 0.01), and a shortening in the isovolumic relaxation period (0.076 + 0.031 vs 0.068 + 0.02, p less than 0.05). On the ejection flow, verapamil decreased the peak velocity (2.82 + 1.28 vs 2.42 + 1.18 m/seg, p less than 0.001). Nor age, sex, ventricular mass, gradient, neither hypertrophic cardiomyopathy's classification relates with changes after intravenous verapamil. There were no adverse effects. This study by Doppler-echocardiography confirms the beneficial ++ effect of intravenous verapamil in patients with hypertrophic cardiomyopathy not only on gradient reduction but also in the improvement on left ventricular diastolic function.     

Apical bilateral hypertrophic myocardiopathy with mitral and greater tricuspid valve insufficiency

The case of a patient with non-obstructive apical hypertrophic cardiomyopathy diagnosed on criteria that are now conventional is reported. This case exhibited two special features: the apical hypertrophic cardiomyopathy was bilateral (i.e. the two ventricles were simultaneously involved) and it was associated with massive mitral and tricuspid valve regurgitation. These two features are frequent in other types of hypertrophic cardiomyopathy, but they have not yet been described in apical hypertrophic cardiomyopathy. They support the concept that there is no definite border between the different types of hypertrophic cardiomyopathy.     

Dobutamine stress echocardiography in hypertrophic cardiomyopathy

AIMS: Myocardial ischemia in the absence of coronary artery disease is common in patients with hypertrophic cardiomyopathy (HCM). Dobutamine stress echocardiography (DSE) induces left ventricular (LV) new wall motion abnormalities (NWMA) in some patients with HCM. We evaluated the effects of dobutamine on LV performance and hemodynamics in HCM. METHODS AND RESULTS: Eighteen patients with non-obstructive HCM underwent DSE. Dobutamine was administered at dosages of 5, 10, 20, 30 and 40 microg/kg/min with increments at intervals of 3 min. Seven patients developed NWMA, whereas the other 11 did not. During DSE, heart rate increased significantly more in NWMA patients,whereas LV outflow tract gradient (OTG) increased significantly and similarly in both groups. At peak dobutamine dose, NWMA patients had a significant increase in LV end-systolic diameter and volume and a significant decrease in LV fractional shortening and ejection fraction. Posterior wall thickening increased significantly, whereas septal thickening did not increase throughout DSE in both groups. CONCLUSIONS: In a subgroup of patients with HCM, DSE induces NWMA, associated with a greater increase in heart rate, irrespective of LVOTG. NWMA induce a depression of global LV systolic performance. The septum shows no contractile reserve, regardless of NWMA. These phenomena may be the result of induction of ischemia and/or impaired LV systolic function due to fast heart rate.