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The role of myocardial biopsy in cardiac diagnosis.

With the advent of cardiac catheterization, cardioangiography, and selective coronary arteriography, specific types of cardiac disease can be recognized and clearly defined. This is appropriate because myocardial biopsy alone rarely plays a major role in cardiac diagnosis. Excluding Aschoff's nodules in patients with rheumatic valve disease, the light microscopic findings in patients with rheumatic heart disease, congenital heart disease, pericardial disease, hypertensive and arteriosclerotic heart disease are similar and nonspecific. In these, interstitial fibrosis and/or myocardial hypertrophy is the dominant tissue diagnosis. Occasionally a pericardial and myocardial specimen is helpful to distinguish constrictive pericarditis and restrictive cardiomyopathy. Myocardial biopsy has provided the only method for diagnosis in a small number of patients with normal hemodynamics, normal coronary arteriograms and normal ventriculograms. The patients were studied because of chest pain and/or cardiac arrhythmias. Supraventricular and/or ventricular arrhythmias were encountered. In these patients the tissue diagnosis was interstital fibrosis and/or myocardial hypertrophy. These findings are consistent with primary myocardial disease which was not recognized clinically or by angiographic studies. The procedure seems to play a major role in the diagnosis of specific types of primary myocardial disease. It is valuable in the recognition of glycogen storage disease, amyloidosis, hemochromatosis, and myocarditis. On the basis of current experience, the indications for myocardial biopsies depend on the need for a tissue diagnosis in determining the management of the patient and the availability of adequately trained personnel to perform the procedure and manage the complications.

Angiocardiography↗

Symptomatic sinus node disease: natural history after permanent ventricular pacing.

Fifty-nine patients between the ages of 13 and 88 with sinus node disease, who received a permanent ventricular pacemaker between 1965 and 1976 at one institution, were followed to determine the natural history of the disorder after permanent pacing. Nineteen had ischemic heart disease, six had primary myocardial disease, and eight valvular heart disease. In 26, no etiology for the arrhythmia was apparent. The one- and five-year survival was 85.5% and 73.1%, respectively. Patients with underlying heart disease had a significantly poorer survival when compared to those without (58% versus 94% at 36 months) and all but 3 of 13 deaths in the first 36 months were in those with ischemic heart disease. There was a distinct trend toward poor survival in those with heart failure prior to pacemaker implant and those over age 65. Patients with sinus bradycardia alone did best (91% survival three years after implant), while those with bradycardia-tachycardia syndrome and those with sinoatrial arrest alone did distinctly worse (76% and 65% survival at three years, respectively). Twelve of 18 deaths were due to progression of underlying heart disease. The long-term prognosis with symptomatic sinus node disease can be predicted in part by (1) etiology of the underlying heart disease, (2) pre-implant arrhythmia, and (3) ventricular function prior to implant.

Adolescent↗

Extracorporeal life support in heart and lung transplantation.

Extracorporeal life support is a technology that is being increasingly used to support children with cardiac and respiratory failure who fail conventional measures. Both extracorporeal membrane oxygenation and ventricular assist devices have been used in cardiac failure with the opportunity to support patients who fail repair of congenital heart disease and in primary myocardial disease. Outcomes in the latter are superior to the surgical patients which has lead to increasing use of extracorporeal life support as a bridge to transplantation. Experience with membrane oxygenation as a support mode after lung transplantation is limited to adult series with similar outcomes.

Catheterization↗

[Scintigraphy of the myocardium. Comparative study between thallium and perfusion scintigrams (author's transl)].

In a total of 51 patients (26 with coronary heart diseases, 25 with primary myocardial diseases), the myocardial scintigrams after intracoronary particle injection (perfusion scintigraphy) and after intravenous nuclide injection with accumulation in the myocardium (thallium scintigraphy) were compared with each other and set against the coronary angiographic and levocardiographic findings. It was shown that extensive myocardial failures (aneurysms) can be represented by both nuclear medical procedures, but that perfusion scintigraphy is more sensitive and correlates more closely to the levocardiogram findings. Smaller abnormalities of contraction of the left ventricle (hypokinetic and akinetic areas) were always shown in the perfusion scintigram, more seldom in the thallium scintigram. The outstanding advantage of thallium scintigraphy is that it is non-invasive, it can be repeated as often as desired and is easily performed during ergometric stress.

Adult↗

Myocardial diseases in Thai children.

UNLABELLED: Myocardial diseases are among the important causes of mortality and morbidity in children. This drew the authors attention to the study of myocardial diseases in children to find out the outcome, factors affecting the outcome, and management strategies. The authors retrospectively studied children who had been diagnosed with primary myocardial diseases at six university hospitals in Thailand from January 1996 to December 2000. The total number of cases was 209 which accounted for 1.2 per cent of cardiovascular diseases in children. The patients' ages ranged from 0.1-15 years. These myocardial diseases included dilated cardiomyopathy (DCM) 45 per cent, acute myocarditis 27.3 per cent, hypertrophic cardiomyopathy (HCM) 18.2 per cent, hypertrophic obstructive cardiomyopathy (HOCM) 8.1 per cent and restrictive cardiomyopathy (RCM) 1.4 per cent. Fifty-six per cent of the patients were female. Congestive heart failure was the most common presenting symptom (75%). Median ejection fraction (EF) of acute myocarditis was 42 per cent (15-79%) which was significantly higher than DCM (33.5%, 10-57%). Serum cardiac troponin T (cTnT) was also significantly higher in acute myocarditis than in DCM (0.08 ng/ml, 0.01-0.16 vs 0.01 ng/ml, 0.01-0.10). Within the follow-up period of 1 year (0.1-5.5 years), the mortality rates were 18.8 per cent, 17.0 per cent, 5.4 per cent and 33.3 per cent in DCM, acute myocarditis, HCM and RCM respectively. Factors associated with the mortality rate in acute myocarditis were admission to ICU and low left ventricular EF at presentation while IVIG administration and cTnT level did not. CONCLUSION: Primary myocardial diseases are uncommon. Most of the patients had compromised cardiovascular reserve. Admission to ICU and low EF were factors that affected the mortality in acute myocarditis while intravenous immunoglobulin administration did not. Mortality rate in the subacute follow-up period was high in all groups.

Adolescent↗

Appearance of atrial sound after reversion of atrial fibrillation.

To determine the incidence and time of onset of the atrial sound after reversion of atrial fibrillation in patients with myocardial disease, 12 patients with arteriosclerotic heart disease and 4 patients with primary myocardial disease were followed with serial auscultation as well as daily simultaneous apical phonocardiogram, apex cardiogram, and lead II electrocardiogram. Their average age was 52.3 years. Each patient had been in atrial fibrillation for 6 months to 2 years' duration; 13 were reverted to sinus rhythm with DC capacitor discharge and 3 with quinidine sulphate. All 16 patients developed an atrial sound when in sinus rhythm, but only 2 on the day of reversion; the other 14 patients developed an atrial sound on the first to the sixth day after reversion (average third day). The atrial sound persisted in each patient. This study indicates that the atrial sound as a manifestation of diminished ventricular compliance may be absent in patients with myocardial disease immediately after reversion of atrial fibrillation and may later appear as left atrial function improves.

Adult↗

Idiopathic left ventricular aneurysm: a clinical and pathological study of a new entity in the spectrum of cardiomyopathies.

In a prospective study on 340 cases with primary myocardial disease, eight patients (six males, two females, mean age 36 years, range 24-47) with an idiopathic left ventricular aneurysm were observed. All patients had normal coronary arteries, no angina or history of myocardial infarction; all but one had no risk factors for ischaemic heart disease; all had normal right ventricles; one patient had a history of familial dilated cardiomyopathy, two of 'flu-like' syndrome at the time of first symptom and two of alcohol abuse. All patients had ventricular tachycardia (VT), five sustained (of right bundle branch block morphology in three, and of different morphologies in two), three non-sustained. Patients with sustained VT had inducible VT (resembling the clinical one) on electrophysiological study. Electrocardiogram (ECG) showed an infarction pattern in three cases. Aneurysms were of limited size (2.1 +/- 1/11 segments on echocardiography) and were located in the septum, apex or posterior wall. Left ventricular ejection fraction (LVEF) was reduced (< 0.50) in six patients and was not correlated with the aneurysm size. The duration of illness was inversely correlated with LVEF (P < 0.05). Endomyocardial biopsy showed evidence of diffuse pathological changes in all cases (cell hypertrophy, myofibrillar lysis, mitochondriosis). During follow-up (64 +/- 32 months), patients were successfully treated with anti-arrhythmic drugs: no patients required surgical treatment to control ventricular arrhythmia. Considering the clinical and pathological features of idiopathic left ventricular aneurysm, this primary myocardial disease could be classified as a novel peculiar form of cardiomyopathy.

Adult↗

The clinical spectrum of chronic His bundle block.

This report details our total experience with documented chronic His bundle block in 24 patients. Ten patients had second-degree block (eight with 2:1 block and two with type-1 block), and 14 patients had complete heart block. There were 16 women (67 percent) and eight men (33 percent) with ages ranging from 17 to 87 years. Diagnoses were as follows: hypertensive cardiovascular disease, nine patients (38 percent); arteriosclerotic heart disease, six patients (25 percent); aortic valvular disease, three patients (13 percent); primary conduction disease, two patients (8 percent); primary myocardial disease, two patients (8 percent); congenital heart block, one patient (4 percent); and traumatic heart block, one patient (4 percent). Pacing was instituted in 20 patients because of the following; congestive heart failure, seven patients; syncope, seven patients; fatigue, four patients; and recurrent dizziness, two patients. Permanent pacing was indicated within ten days of initial diagnosis in 13 patients, from 20 to 80 days in four patients, and later than 100 days in three patients. An additional two asymptomatic patients were treated with prophylactic pacing.

Adolescent↗

Heart disease screening of school children using abbreviated electrocardiogram and phonocardiogram.

By recording the electrocardiogram and phonocardiogram simultaneously using three abbreviated leads, 23,216 (6 and 7 years of age) elementary school children were screened for heart diseases. One hundred and one cases (0.44%) with congenital heart diseases, ten cases (0.04%) with acquired heart diseases and 100 cases (0.43%) with electrocardiographic abnormalities were discovered. From these observations, we conclude that this screening method applied on school children is useful in discovering the previously unrecognized heart diseases, especially atrial septal defect, primary myocardial disease and arrhythmia.

Arrhythmias, Cardiac↗

Visualization of right atrial appendix by thallium-201 myocardial scintigraphy: concise communication.

The atrial myocardium has been barely visible with thallium-201 myocardial scintigraphy. This is probably related to the difference in size, distance from anterior chest wall, and small coronary blood flow of the atrium, compared with the ventricle. We have encountered eight cases of visualization of the right atrial appendix (RAA). All had disease involving the right side of the heart, such as mitral stenosis (four cases), congenital heart disease (two), cor pulmonale (one), and primary myocardial disease (one). The RAA was identified from multiple projections in all cases and sometimes confirmed by radionuclide angiocardiography. The RAA was seen at the right upper aspect of the ventricles and was distinguishable from them. Evidence derived from ECG, chest radiographs, and cardiac catheterization, indicated that the most important factor in the visualization might be the displacement of the RAA to a more anterior position.

Adult↗

Sudden cardiac death in the young: a strategy for prevention by targeted evaluation.

The annual incidence of sudden cardiac death (SCD) in the general population is estimated as 1 in a 1,000. Since survival rates from out-of-hospital cardiac arrests are poor, primary prevention is key to reducing the burden of SCD in the community. Prominent causes of SCD include ischaemic heart disease, anomalous coronary arteries, and the primary myocardial diseases: hypertrophic cardiomyopathy, dilated cardiomyopathy, and ar rhythmogenic right ventricular cardiomyopathy (ARVC). In 4% of sudden deaths in the 16-64 age group, post-mortem examination fails to identify a cause, yielding a default diagnosis of sudden arrhythmic death syndrome (SADS). The inherited arrhythmia syndromes (long QT, short QT, and Brugada syndromes, and familial catecholaminergic polymorphic ventricular tachycardia) may be implicated in SADS, owing to their propensity for producing ventricular tachyarrhythmia in the structurally normal heart. Monogenic disorders therefore predominate as causes of SCD in the young. The advent of effective therapies for these diseases, particularly implantable cardioverter defibrillators, has prompted calls for universal screening to enable timely diagnosis of occult cardiac disease. Since prospective cardiac assessment of the general population is not feasible, the solution may be to target high-risk subgroups, namely, patients with cardiac symptoms, relatives of SCD victims, and competitive athletes. The recommended preliminary work-up includes a 12-lead ECG, signal-averaged ECG, transthoracic echocardiogram, exercise test, and ambulatory ECG monitoring. Cardiovascular magnetic resonance is a useful adjunct in patients with suspected ARVC or anomalous coronary arteries. Provocative challenge with a sodium challenge blocker may be of value in unmasking the Brugada syndrome. Identification of disease-causing mutations in affected individuals facilitates cascade screening of families.

Adolescent↗

111Indium antimyosin antibody imaging of primary myocardial involvement in systemic diseases.

OBJECTIVE: The diagnosis of primary myocardial involvement in systemic diseases is clinically relevant but difficult in the absence of specific criteria. Whatever the underlying disease, myocytes degeneration is observed during the active phase of myocardial damage. The aim of this study was to assess the diagnostic value of scintigraphic imaging with 111Indium antimyosin antibody (AM), a specific marker of the damaged myocyte, for ongoing myocardial damage related to systemic diseases. METHODS: 40 patients with histologically confirmed systemic diseases were studied. They were classified into two groups according to the presence (group 1, n = 30), or the absence (group 2, n = 10) of clinical, electrocardiographic (ECG) or echocardiographic signs suggestive of myocardial involvement. Planar and tomographic acquisitions were obtained 48 hours after injection of AM (90 MBq). Rest 201thallium (T1) scintigraphy was also performed to assess myocardial perfusion and scarring. Clinical, ECG, and echocardiographic +/- scintigraphic evaluations were repeated during follow up (17 +/- 19 months) in 36 of 40 patients. RESULTS: In group 1, 13 of 30 patients (43%) showed diffuse significant AM uptake throughout the left ventricle (LV), and no or mild T1 abnormality. Two of these were asymptomatic, four had normal ECG, and two had no clinical or echographic LV dysfunction. All patients in group 2 had negative AMA scintigraphy and normal T1 scintigraphy. During follow up of 12 AM positive patients, cardiac status improved after immunosuppressive treatment was intensified in nine cases, worsened in two cases, and remained stable in one. During follow up of 24 AM negative patients, cardiac status remained stable in 23 cases despite treatment not being increased in 20, including two patients with sequellary myocardial involvement. The last patient developed mild LV dysfunction after 36 months. CONCLUSION: AM scintigraphy allows detection of active myocardial damage related to systemic diseases, with increased specificity compared with conventional methods, and increased sensitivity in some cases. Further studies are needed to assess the potential value of AM scintigraphy as a therapeutic guide.

Adult↗

Spontaneously occurring arrhythmogenic right ventricular cardiomyopathy in the domestic cat: A new animal model similar to the human disease.

BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a primary myocardial disease of incompletely resolved pathogenesis and is a largely unappreciated cause of sudden death in the young. METHODS AND RESULTS: Clinical features of 12 domestic cats with ARVC (7 male; 1 to 20 years old, mean 7.3+/-5.2 years) were right-sided congestive heart failure (n=8), supraventricular tachyarrhythmias (n=5), ventricular tachycardia (n=3), polymorphic ventricular arrhythmias (n=6), and right bundle-branch block (n=5). ARVC was suspected in all 8 cats examined with echocardiography by marked enlargement of the right ventricle (RV) and right atrium and tricuspid regurgitation. Eight died of cardiovascular disease and 4 died of noncardiac conditions. At autopsy, hearts of ARVC cats were characterized grossly by moderate-to-severe RV cavity enlargement and wall thinning (n=12) and apical aneurysm formation (n=6). Histology demonstrated pronounced RV lesions in all 12 ARVC cats, including marked myocardial injury (myocyte death and atrophy) and repair (fibrous and/or fatty replacement). Injury and repair were also evident in the left ventricle (LV) in 10 cats, and 2 had involvement of both atria. Myocarditis was present in 10 of the 12 ARVC cats. Apoptosis was detected in 9 ARVC cats (mean apoptotic index, 28+/-23% RV, 21+/-19% LV, and 17+/-15% ventricular septum) but not in controls. CONCLUSIONS: In the common domestic cat, we identified a clinically relevant cardiomyopathy that closely mimics ARVC in humans. This unique feline model of human disease will be relevant to defining pathogenesis and investigating mechanisms responsible for disease progression in ARVC.

Animals↗

[Studies of enzyme histochemistry and ultrastructure of the myocardium in rats with streptozotocin-induced diabetes].

Experimental diabetes was induced in wistar rats by intraperitoneal streptozotocin in a single dose of 60-65 mg per kg body weight. The changes of myocardium enzyme histochemistry and ultrastructure were observed during the 2nd, 4th, 6th, 12th week of diabetic state. The glucose metabolism enzyme activities such as ICDH, SDH, MDH, LDH, all decreased. The results indicated that glucose oxidation and glycolysis reduced. In the ventricular myocardium of diabetic rats, varying degrees of ultrastructural change were apparent. Swelling of mitochondria was observed. Focal areas showed myofibrillar degeneration, and cardiac muscle muscle cells showed condensation of nuclear chromatin. Lipid droplets could be seen in the cytoplasm of cardiac myocytes. The ultrastructural changes in the cardiac muscle cells were not accompanied by any changes in the endothelial cells and smooth muscle cells of the small vessels or capillaries. This study provides a strong evidence for the occurrence of a primary myocardial disease in the model of streptozotocin-induced diabetes. The primary cardiomyopathy was not dependent on vascular pathological changes.

Animals↗

Nitroglycerin-induced improvement in exercise tolerance and hemodynamics in patients with chronic rheumatic heart valve disease.

Nitroglycerin reduces elevated left ventricular filling and pulmonary arterial pressures in resting patients with rheumatic valve disease and reduces symptoms when given over long periods to patients with primary myocardial disease. To determine whether nitroglycerin may prove effective therapeutically in ambulatory patients with heart valve disease, its effects on hemodynamics and exercise capacity were studied in 11 severely symptomatic adults who were already receiving optimal treatment with digitalis and diuretic agents. Seven had predominant mitral valve disease, one had predominant aortic insufficiency and three had equally severe mitral and aortic valve disease. Maximal exercise capacity was assessed with graded treadmill exercise after placebo and after nitroglycerin (0.5 mg sublingually) administered in random sequence to each patient. Exercise capacity (exercise time to limiting fatigue or dyspnea) increased from a mean of 8.3 minutes after placebo to 9.8 minutes after nitroglycerin (P less than 0.005). Eight patients were studied hemodynamically during further intense treadmill exercise. Pulmonary arterial pressure was significantly lower (P less than 0.05) after nitroglycerin than after placebo (mean 44 versus 56 mm Hg), but cardiac output was greater after nitroglycerin (5.0 versus 4.6 liters/min, P less than 0.005). Thus, nitroglycerin appears to increase exericse tolerance and improve the hemodynamic response to exercise in patients with heart valve disease and may be valuable in the long-term pharmacologic therapy of such patients.

Administration, Oral↗

Measurements of plasma endothelin immunoreactivity in healthy cats and cats with cardiomyopathy.

Plasma concentrations of endothelin-1 (ET-1), the most potent endogenous pressor substance discovered to date, are abnormally high in humans with congestive heart failure (CHF), and they correlate with the degree of functional impairment. We sought first to validate a human sandwich ELISA kit that targets that portion of the amino acid sequence that is identical in cats. The assay demonstrated linearity (R2 = .9968) and parallelism (P = .5339), recovery of spiked human ET-1 in cat plasma averaged 98.7%, and intraassay precision had a coefficient of variation <10%. We subsequently determined ET-1 immunoreactivity in healthy cats and in cats with myocardial disease with and without CHF, systemic thromboembolism (STE), or both. Plasma ET-1 immunoreactivity was measured in 12 healthy cats and in 28 cats with primary myocardial disease, including hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), or restrictive or unclassified cardiomyopathy (RCM and UCM), respectively. Plasma ET mean (95% CI) concentrations were 0.777 (0.6536-0.924) fmol/mL in the control cats, 1.427 (0.922-2.209) fmol/mL in 12 cats with cardiomyopathy (HCM = 11, RCM/UCM = 1) but without CHF or evidence of STE, and 2.360 (1.666-3.343) fmol/mL in 16 cats with cardiomyopathy (HCM = 8, RCM/UCM = 7, DCM = 1) and CHF (n = 15) or STE (n = 4). Plasma immunoreactivity of ET-1 was significantly higher in cats with myocardial disease without CHF/STE versus normal cats (P < .05) and in cats with myocardial disease with CHF/STE versus normal cats (P < .001).

Animals↗

A clinicopathological study on 25 cases of complete left bundle branch block.

A total of 25 cases (12 men, 13 women) of complete left bundle branch block (LBBB) were found among 1,400 consecutive autopsy in the aged. Their ages ranged from 70 to 86 years (average 78.9). ECG was analyzed as for the occurrence of LBBB and myocardial infarction (MI). Pathological examinations included observations of the conduction system by serial sections. They were divided into group A with MI and group B without MI. Duration of LBBB was 1 to 3 days in 4 cases, more than 1 month in 7, and more than 1 year in 14. From the temporal sequence of LBBB and MI in group A, cases were classified into (1) MI preceding LBBB in 5, (2) both coexistent in 5, and (3) LBBB preceding MI in 1. There were 8 cases of normal electrical axis, 17 left axis deviation, 7 first degree A-V block, and 2 atrial fibrillation. Various heart diseases were underlying in 21 cases, including hypertension, MI, mitral and aortic regurgitation, and primary myocardial disease, and there were 4 cases with no cardiac diseases. Cause of death was cardiac in 12; MI, congestive heart failure, and sudden death. Heart weight was 410 Gm on the average (240 to 550 Gm). MI was found in 11, with stenotic index of 12/15, while it was 9/15 in group B. Lesions of the conduction system were slight to moderate (1.5 to 2.4) except left bundle branch, which showed marked changes in posterior (4.9) and anterior (4.8) fascicles. Site of interruption of the left bundle branch was the junction between the branching portion of the A-V bundle and the left bundle branch (Junctional type) in 17, and peripheral portion of the left bundle branch about 10 mm or more below the junction in 8 (Peripheral type). In conclusion, 2/3 of cases of LBBB belonged to the junctional type and most of them were not related to MI, but to the lesions caused by mechanical injuries at the septal summit. One third of the cases were as peripheral type, which was mainly related to the various types of lesions including septal ischemia (necrosis and fibrosis).

Aged↗