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1/170. A novel form of familial congenital muscular dystrophy in two adolescents.

    We report on two brothers (the product of first-degree consanguineous marriage; aged 15 and 12 years) who presented with severe hypotonia at birth, proximal muscle weakness associated with delayed motor milestones but normal cognitive function. Investigations (at 4 years of age) revealed mildly elevated serum creatine kinase (CK) levels (300 and 824 IU/l; N < or = 210). Muscle biopsies showed minimal change myopathy, no neurogenic atrophy but remarkable type-1 fibre predominance (up to 85.5%) without fibre-type disproportion. Clinical examination at 12 and 9 years, respectively, showed mild facial weakness and high-arched palate in both patients. The younger sibling also had ptosis but otherwise normal external ocular muscles. They showed symmetric proximal muscle weakness and wasting associated with calf-muscle hypertrophy. They could walk independently. A repeat muscle biopsy showed advanced dystrophic changes in the younger patient at the age of 10 years. Virtually all the remaining fibres were type 1. immunohistochemistry revealed normal expression of the dystrophin-glycoprotein complex (DGC), including dystrophin, beta-dystroglycan, alpha-(adhalin), beta-, gamma-, and delta-sarcoglycan, laminin-alpha2 chain (merosin) and syntrophin. Mild dystrophic features and type-1 fibre predominance (92.5%) were seen in the biopsy of the older patient, whereas immunohistochemistry showed normal expression of the DGC. Both cases also showed clear expression of integrin alpha7 at the muscle fibre surface and in the blood vessels. Three years later, they could still walk, but with difficulty, and the older brother showed enlargement of the tongue and echocardiographic features of left ventricular dilated cardiomyopathy.
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ranking = 1
keywords = cardiomyopathy
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2/170. Rapid progression of cardiomyopathy in mitochondrial diabetes.

    Cardiac involvement and its clinical course in a diabetic patient with a mitochondrial tRNA(Leu)(UUR) mutation at position 3243 is reported in a 54-year-old man with no history of hypertension. At age 46, an electrocardiogram showed just T wave abnormalities. At age 49, it fulfilled SV1 RV5 or 6>35 mm with strain pattern. At age 52, echocardiography revealed definite left ventricular (LV) hypertrophy, and abnormally increased mitochondria were shown in biopsied endomyocardial specimens. He was diagnosed as having developed hypertrophic cardiomyopathy associated with the mutation. However, at age 54, SV1 and RV5,6 voltages were decreased, and echocardiography showed diffuse decreased LV wall motion and LV dilatation. Because he had mitochondrial diabetes, the patient's heart rapidly developed hypertrophic cardiomyopathy, and then it seemed to be changing to a dilated LV with systolic dysfunction. Rapid progression of cardiomyopathy can occur in mitochondrial diabetes.
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ranking = 7
keywords = cardiomyopathy
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3/170. Reversible catecholamine-induced cardiomyopathy in a heart transplant candidate without persistent or paroxysmal hypertension.

    BACKGROUND: Both dilated and hypertrophic cardiomyopathy have been reported in patients with pheochromocytoma, who were almost always hypertensive. The outcome frequently has been fatal, yet cardiac dysfunction can be reversible after medical or surgical therapy for the pheochromocytoma. methods: We report the case of a patient with dilated cardiomyopathy without persistent or paroxysmal hypertension, who was found to have a pheochromocytoma during initial medical evaluation. RESULTS: The identification and treatment of the pheochromocytoma led to significant improvement in cardiac function and cardiac transplantation was avoided. CONCLUSIONS: This case illustrates some unusual features in pheochromocytoma-induced cardiomyopathy: (1) absence of persistent or paroxysmal hypertension, (2) initial presentation with acute myocardial infarction and normal coronary arteries, and (3) recurrent episodes of nonsustained ventricular tachycardia.
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ranking = 7
keywords = cardiomyopathy
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4/170. Cardiac involvement in coffin-lowry syndrome.

    coffin-lowry syndrome is an X-linked recessive syndrome of mental retardation, characteristic facies and skeletal anomalies. In one patient with the syndrome, we observed early recurrent episodes of congestive heart failure with intercurrent normalization and the late development of mitral insufficiency due to annular dilation and congenital abnormalities of the valve apparatus. This unusual course of cardiac involvement, the non-adaptation of the left ventricular contractility to the aggravation of the mitral insufficiency and the postoperative persistence of the ventricular dysfunction, underline the possible role of an associated primary myocardial disease. This clinical observation demonstrates clearly that a mitral valve malformation can occur in patients with the syndrome, but also the role of a dilated cardiomyopathy, which can be secondary to the mitral regurgitation, but is more likely a myocardial disorder occurring as part of the syndrome.
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ranking = 1
keywords = cardiomyopathy
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5/170. Dynamic outflow obstruction due to the transient extensive left ventricular wall motion abnormalities caused by acute myocarditis in a patient with hypertrophic cardiomyopathy: reduction in ventricular afterload by disopyramide.

    A 65-year-old woman was admitted to the coronary care unit because of acute pulmonary edema. Immediate 2-dimensional and Doppler echocardiograms revealed extensive left ventricular wall motion abnormalities and left ventricular hypertrophy with extreme outflow obstruction. Although an ECG showed ST-segment elevation in the anterolateral leads, a coronary arteriogram revealed normal epicardial arteries. heart failure was relieved after diminishing the dynamic outflow obstruction with disopyramide administration. An endomyocardial biopsy from the right ventricle on the 8th hospital day showed borderline myocarditis. Wall motion abnormalities gradually normalized within 2 weeks. It is speculated that her pulmonary edema would not have been relieved so readily without the immediate reduction in ventricular afterload by disopyramide. These clinical changes over time were observed with serial echo-Doppler examinations.
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ranking = 4
keywords = cardiomyopathy
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6/170. DDD-pacing-induced cardiomyopathy following AV node ablation for persistent atrial tachycardia.

    Ventricular rate control by catheter ablation of the AV node and pacing in patients with persistent atrial tachycardia has been reported to improve left ventricular function. However, this approach requires careful selection of the pacing mode. We report a patient who underwent AV node ablation for persistent multiple atrial tachycardias, and who then had a non-mode-switching pacemaker implanted. Because of an inappropriately programmed relatively high upper rate limit, the patient developed left ventricular dysfunction after 6 years. This resolved after programming the pacemaker to VVI at 70 bpm.
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ranking = 4
keywords = cardiomyopathy
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7/170. Anthracycline-induced cardiomyopathy.

    Anthracycline cardiomyopathy is less frequently encountered nowadays, due to the well-recognised dose limitations and cardiac monitoring protocols used by chemotherapy centres. However, it is a condition that will persist due to the sensitivity of some patients to these drugs and the necessity for large doses to be used for certain individuals. We have demonstrated the benefit of angiotensin-converting enzyme inhibitor therapy and would consider introducing these compounds at the earliest opportunity. The use of probucol and vitamins as antioxidants capable of preventing the onset of cardiomyopathy in humans appears to require further investigation but may significantly reduce the incidence of this condition in the future.
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ranking = 6
keywords = cardiomyopathy
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8/170. A probable primary hiv infection associated with acute non-specific myocarditis causing severe dilated cardiomyopathy.

    A patient with a probable primary hiv infection and a biopsy proven non-specific myocarditis is reported. The patient developed a severe dilated cardiomyopathy and initially presented with global heartfailure and fever. The left ventricular function partially recovered. One week after discharge the patient was readmitted in a septic shock and died. Current hypotheses concerning the etiology of left ventricular dysfunction in hiv infection are discussed.
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ranking = 5
keywords = cardiomyopathy
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9/170. Tachycardiomyopathy--a case report.

    This case report describes a patient presenting with atrial tachyarrhythmia and severe left ventricular failure in which rate control was associated with restoration of normal contractile function.
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ranking = 4
keywords = cardiomyopathy
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10/170. Cardiac complications in pediatric patients on the ketogenic diet.

    Cardiac complications of the ketogenic diet, in the absence of selenium deficiency, have not been reported. Twenty patients on the ketogenic diet at one institution were investigated. Prolonged QT interval (QTc) was found in 3 patients (15%). There was a significant correlation between prolonged QTc and both low serum bicarbonate and high beta-hydroxybutyrate. In addition, three patients had evidence of cardiac chamber enlargement. One patient with severe dilated cardiomyopathy and prolonged QTc normalized when the diet was discontinued.
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ranking = 1
keywords = cardiomyopathy
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