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PubMed2000

Metabolic aspects of myocardial disease and a role for L-carnitine in the treatment of childhood cardiomyopathy.

OBJECTIVES: A multicenter retrospective study was conducted to investigate the possible metabolic causes of pediatric cardiomyopathy and evaluate the outcome of patients treated with L-carnitine. METHODS: Seventy-six patients diagnosed with cardiomyopathy were treated with L-carnitine in addition to conventional cardiac treatment, and 145 patients were treated with conventional treatment only. There were 101 males and 120 females between 1 day and 18 years old. Cardiomyopathy diagnoses included dilated (148 patients), hypertrophic (42 patients), restrictive (16 patients), mixed diagnosis (11 patients), and 4 with an unknown type. Of 76 L-carnitine-treated patients, 29 (38%) had evidence to suggest a disorder of metabolism, and of 145 control patients, 15 (10%) were suspected to have a disorder of metabolism. These metabolic disorders were thought to be the cause for the cardiomyopathy of the patients. The duration of L-carnitine treatment ranged from 2 weeks to >1 year. Information was collected on length of survival (time-to-event), clinical outcome, echocardiogram parameters, and clinical assessments. Data were collected at intervals from baseline to study endpoint, death, transplant, or last known follow-up visit. RESULTS: L-Carnitine-treated patients were younger than control patients and had poorer clinical functioning at baseline, yet they demonstrated lower mortality and a level of clinical functioning and clinical severity comparable to control patients on conventional therapy by the end of the study. An analysis of the interaction between clinical outcome and concomitant medications unexpectedly revealed that the population of patients treated with angiotensin-converting enzyme (ACE) inhibitors (40% of patients) had significantly poorer survival (although their greater likelihood for poor survival may possibly have made them more likely to receive ACE inhibitors). CONCLUSION: Results suggest that L-carnitine provides clinical benefit in treating pediatric cardiomyopathy. There is a need for further exploration of potential explanatory factors for the higher mortality observed in the population of patients treated with ACE inhibitors.

Cardiomyopathies↗
PubMed1992

Pulmonary and cardiac manifestations of polymyositis-dermatomyositis.

Polymyositis-dermatomyositis (PM-DM) is an inflammatory disease of muscle and skin mediated by autoimmune and cellular events. Most typically, muscle weakness is the usual presentation. This review emphasizes that often the systemic components of this disease may mask the usual presentation and actually may be the presenting and only manifestations; more often than not they are the causes of increased morbidity and mortality. In particular, the cardiopulmonary manifestations may dominate the disease course. Cardiac complications include congestive heart failure resulting from a primary cardiomyopathy, disrhythmias and atrioventricular conduction disturbances, sick sinus syndrome, and cor pulmonale either secondary to interstitial lung disease (ILD) or primary pulmonary artery hypertension. Recurrent aspiration pneumonia results from pharyngeal muscle involvement by the myositic process. Several histologic patterns of ILD can emerge with varying outcomes and responses to immunosuppresive therapy. Involvement of the muscles of respiration can lead to hypercapnic respiratory failure, diaphragmatic dysfunction, hypostatic pneumonia, and restrictive lung disease.

Cardiomyopathies↗
PubMed2003

Left ventricular assist device as bridge to heart transplantation--lessons learned with the MicroMed DeBakey axial blood flow pump.

OBJECTIVE: The MicroMed DeBakey left ventricular assist device (LVAD) axial blood flow pump was used as bridge to heart transplantation (HTx) in patients with terminal heart failure. The aim was to evaluate this novel mechanical circulatory support system in regard to overall outcome. METHODS: Prospective study in 15 HTx candidates (mean age 40+/-7 years) with terminal heart failure and maximal medical treatment due to ischemic cardiomyopathy (CMP, n=5), dilated CMP (n=3), restrictive CMP (n=2), unclassified CMP (n=1), metabolic CMP (n=1), valvular CMP (n=1) and congenital CMP (n=2). All patients were implanted with a MicroMed DeBakey LVAD. A rescue procedure was necessary in eight critical patients, while seven underwent elective LVAD implantation. Procedures were performed via median sternotomy, in normotherm femoro-femoral CPB (mean duration 59+/-1 min). Oral Marcoumar (INR 2.0-3.0) and Aspirin (100 mg daily) were started as soon as possible. Patients were discharged into a specialized rehabilitation clinic from which it was possible to release them home after a few weeks. RESULTS: Successful implantation and discharge from ICU (mean stay 10+/-7 days) was possible in 11 patients. Seven were transplanted (mean support 50.7 days) and one is awaiting HTx (support >310 days) in the comfort of his home (NYHA I). Survival was 100% among the transplanted patients. Of the seven elective implants, five, and of the eight rescue procedures three patients underwent successful HTx. Four patients died early, while three patients died late on pump support due to intracranial hemorrhage (n=2, 73 and 76 days) and chest infection (n=1, 124 days). All survivors were discharged from hospital, with significant decrease in NYHA class (mean 3.8-2.4 (n=11)). Treadmill testing showed increased exercise tolerance, from 35 to 71W (n=4). Plasma BNP values (mean 950-162 ng/l (n=4)) and pulmonary resistance (mean 316-194.5 dyne s/cm(5) (n=3)) decreased significantly during LVAD support. CONCLUSIONS: The MicroMed DeBakey LVAD is simple to implant; outpatient treatment is safe and efficient. Patients' condition and pulmonary resistances normalize within 6 weeks, making previously considered inoperable patients amenable for HTx. HTx can be performed in low-risk situation, allowing better donor-recipient matching and improving overall outcome.

Adolescent↗
PubMed1979

Regulation of respiration in Friedreich's ataxia.

Friedreich's Ataxia (F.A.) is a degenerative disease which commonly leads to premature death of cardiorespiratory origin. To explain the early death of these patients, previous investigations have established the existence of 1) a cardiomyopathy in nearly 100% of cases, 2) a restrictive pulmonary syndrome of scoliotic origin and 3) a mild hypoxemia associated with slight respiratory alkalosis and a normal oxyhemoglobin dissociation curve. To further assess the cause of early death in patients with such neuromyopathy, we evaluated, in eleven F.A. patients, the sensitivity of the respiratory centers to hypercapnia, hypoxia, and hyperoxia. Ventilatory (VE, VT, F, VT/Ti) and occlusion pressure (P0.1) responses were taken as indices of the respiratory centers output during progressive hypercapnia (Read's method) and isocarbic hypoxia (Weil's method). We studied 11 Friedreich's Ataxia patients and 11 age, sex, and armspan matched controls. The responses of patients to hypercapnia were significantly greater than controls but their responses to hypoxia were similar to controls. Our study establishes that the respiratory centers are functioning adequately in early Friedreich's Ataxia and do not contribute to cardio-respiratory insufficiency in such neuromyopathy.

Adolescent↗
PubMed2004

Essential role for mitochondrial thioredoxin reductase in hematopoiesis, heart development, and heart function.

Oxygen radicals regulate many physiological processes, such as signaling, proliferation, and apoptosis, and thus play a pivotal role in pathophysiology and disease development. There are at least two thioredoxin reductase/thioredoxin/peroxiredoxin systems participating in the cellular defense against oxygen radicals. At present, relatively little is known about the contribution of individual enzymes to the redox metabolism in different cell types. To begin to address this question, we generated and characterized mice lacking functional mitochondrial thioredoxin reductase (TrxR2). Ubiquitous Cre-mediated inactivation of TrxR2 is associated with embryonic death at embryonic day 13. TrxR2(TrxR2(-/-)minus;/TrxR2(-/-)minus;) embryos are smaller and severely anemic and show increased apoptosis in the liver. The size of hematopoietic colonies cultured ex vivo is dramatically reduced. TrxR2-deficient embryonic fibroblasts are highly sensitive to endogenous oxygen radicals when glutathione synthesis is inhibited. Besides the defect in hematopoiesis, the ventricular heart wall of TrxR2(TrxR2(-/-)minus;/TrxR2(-/-)minus;) embryos is thinned and proliferation of cardiomyocytes is decreased. Cardiac tissue-restricted ablation of TrxR2 results in fatal dilated cardiomyopathy, a condition reminiscent of that in Keshan disease and Friedreich's ataxia. We conclude that TrxR2 plays a pivotal role in both hematopoiesis and heart function.

Animals↗
PubMed1993

Hermansky-Pudlak syndrome in a Swiss population.

Tyrosinase-positive albinism, previously diagnosed as Hermansky-Pudlak Syndrome (HPS), has been examined in four generations from a village of the canton Valais, Switzerland. Homozygotes, obligate heterozygotes and putative heterozygotes in this geneology yielded lower than normal membrane-associated thioredoxin reductase (TR) activities compared with normal family members and controls. All of the homozygotes and 50% of each the obligate and putative heterozygotes showed an increase in bleeding time associated with storage-pool-deficient platelets lacking dense bodies. The TR activity profile and the platelet-dense body deficiency in the Swiss albinos was the same as that in the HPS population from Puerto Rico. However, in albinos from Puerto Rico, there is an accumulation of ceroid/lipofuscin-like pigment in lysosomal structures causing tissue damage, and, upon kidney involvement, this leads to increased urinary dolichol excretion. Approximately half of the Puerto Rican HPS cases had clinical evidence of storage disease with restrictive lung disease, granulomatous colitis, kidney failure and cardiomyopathy. By comparison, the Swiss HPS geneology had a normal life expectancy with no significant evidence for ceroid accumulation or urinary dolichol excretion. An examination of antioxidant enzymes, catalase, TR and glutathione reductase in epidermal suction blisters from Swiss HPS homozygotes showed a similar result for catalase and TR levels to the depigmented epidermis of patients with vitiligo, except that intracellular TR was found to be calcium free in HPS compared with vitiligo. Intracellular glutathione reductase levels were highest in HPS. Both the Swiss and Puerto Rican HPS homozygotes and heterozygotes have giant melanosomes in skin melanocytes.

Adolescent↗
PubMed1982

[Therapy of cardiomyopathies].

The treatment of congestive cardiomyopathy is dependent on clinical findings, reduced ejection fraction and presence of congestion. Diet, digitalis and vasodilator drugs: prazosin or dihydralazine are used in congestive cardiomyopathy class I or II (NYHA). In the presence of congestion diuretics are added, in chronic congestion of the lung nitrate. Congestive cardiomyopathy class III or IV (NYHA) is treated with diet, digitalis and the converting enzyme inhibitor captopril. In the therapy of HOCM or HNCM the calcium antagonist verapamil is now widely used and seems to be replacing the beta-blocker treatment with propranolol. If treatment with beta-blocker and calcium antagonists in patients with HOCM class III or IV (NYHA) ist ineffective surgical therapy is necessary.

Adrenergic beta-Agonists↗
PubMed1994

[Meadows syndrome: puerperal cardiomyopathy].

This retrospective study concerns six cases of gravido-puerperal cardiomyopathy. These six cases account for 18% of all cases of non-obstructive cardiomyopathy (NOCM) affecting women in general and 67% of NOCM in women of childbearing age. Mean age was 28.5 with a range of 18 to 39. Reported risk factors include poor socio-economic conditions, multiparity, twin pregnancies and anemia. The clinical picture is that of cardiac failure, most often congestive (83.3%), with a systolic murmur of functional mitral incompetence (50%) and atypical chest pain (50%). The ECG is invariably abnormal, without specific signs. The high incidence of repolarisation disturbances is a fairly special feature. Echocardiography is the technique of choice, enabling detection, diagnosis and study of ventricular function, which is an important factor in prognostic evaluation. Medical treatment is based upon a combination of rest, salt-free diet, diuretics, digitalis and vasodilators. The etiology remains unknown and the prognosis is severe despite cases of complete recovery.

Adolescent↗
PubMed1981

The echocardiographic assessment of cardiomyopathy: diagnosis, classification and problems.

Two-hundred-and-sixty-three patients with cardiomyopathy were studied by M-mode echocardiography. Measurements of left ventricular cavity size, wall thickness and myocardial contraction were used to classify cardiomyopathy into "congestive" (212 patients) and "hypertrophic" (50 patients) groups; the "hypertrophic" group was further divided into asymmetric septal hypertrophy (37) and symmetric (concentric) mural thickening (13). Using clinical and electrocardiographic information as well as echocardiographic data, the latter group could then be further classified into "concentric infiltrative cardiomyopathy" (9) and "concentric hypertrophic cardiomyopathy" (4). The former either presented with signs of restriction or were known to have systemic amyloidosis; the electrocardiograph showed low voltage and myocardial contraction was impaired in advanced cases. The latter had evidence of severe left ventricular hypertrophy and resembled asymmetric septal hypertrophy clinically. Problems encountered with the echocardiographic diagnosis of congestive cardiography were mainly concerned with proper clinical interpretation of the echocardiographic data whilst technical difficulties in recording the echocardiogram and in interpretation of tracings were a common problem in hypertrophic cardiomyopathy.

Cardiomyopathies↗
PubMed2006

Phenotypic and genotypic heterogeneity in transthyretin-related cardiac amyloidosis: towards tailoring of therapeutic strategies?

Transthyretin-related hereditary amyloidosis (ATTR) is genotypically/phenotypically heterogeneous. We investigated myocardial involvement in ATTR in a cohort of patients with a wide range of mutations. Clinical/echocardiographic follow-up of 41 consecutive symptomatic ATTR patients from a single referral center was analyzed according to TTR mutation. Diagnosis was based on histology, immunohistochemistry and genotyping. Median follow up was 40 months (range 8-120). Among the 12 different mutations identified, Val30Met was found in 10 patients and Glu89Gln in seven. Compared with Val30Met, Glu89Gln was associated with higher LV mass index, lower left ventricular ejection fraction and shorter E-wave deceleration time. All Glu89Gln carriers had cardiomyopathy, which was more severe (for left ventricular thickness, left ventricular mass and restrictive pathophysiology) than in the six affected Val30Met patients. Glu89Gln was independently associated with higher risk of major cardiovascular events among cardiomyopathy patients. This follow-up study of ATTR patients carrying a wide range of mutations indicates that (1) cardiac involvement is a very important component of phenotypic expression; and (2) genotype is an important source of heterogeneity in myocardial involvement, with Glu89Gln being associated with a severe, heart-driven prognosis. We think that combined heart-liver transplantation could be considered for Glu89Gln carriers with established, morphologically severe cardiomyopathy.

Adult↗
PubMed1993

Prognosis of patients with hypertrophic obstructive cardiomyopathy after transaortic myectomy. Late results up to twenty-five years.

A complete clinical study was performed for 364 patients with hypertrophic obstructive cardiomyopathy who were operated on in the years 1963 to 1991 (217 male, 146 female, mean age 40 years, range 5 months to 76 years). Transaortic subvalvular myectomy was performed in 272 patients (hospital mortality 2.9%), and 92 patients needed additional cardiac procedures simultaneously (hospital mortality 10.9%). A complete follow-up study (100%) included 346 patients who survived the operation. The shortest follow-up time was 2 months and the longest 25.2 years (mean 8.2 years). Most of the patients improved clinically by one to three classes (New York Heart Association). During the observation period 38 patients (10.4%) died. The death of 17 patients was closely related to the original disease (4.9%). Other causes, unrelated to hypertrophic obstructive cardiomyopathy, were responsible for the death of 21 patients (5.8%). In consideration of these data, the yearly total death rate was 2.2%; in close relation to hypertrophic obstructive cardiomyopathy it was about 0.6%. The cumulative survivals were 88% after 10 years and 72% after 20 years. In our long-term clinical experience it is increasingly evident, despite the restrictions of a retrospective study, that patients with symptomatic hypertrophic obstructive cardiomyopathy and failing medical therapy benefit from transthoracic subvalvular myectomy.

Adolescent↗
PubMed2003

A critical role for PPARalpha-mediated lipotoxicity in the pathogenesis of diabetic cardiomyopathy: modulation by dietary fat content.

To explore the role of peroxisome proliferator-activated receptor alpha (PPARalpha)-mediated derangements in myocardial metabolism in the pathogenesis of diabetic cardiomyopathy, insulinopenic mice with PPARalpha deficiency (PPARalpha(-/-)) or cardiac-restricted overexpression [myosin heavy chain (MHC)-PPAR] were characterized. Whereas PPARalpha(-/-) mice were protected from the development of diabetes-induced cardiac hypertrophy, the combination of diabetes and the MHC-PPAR genotype resulted in a more severe cardiomyopathic phenotype than either did alone. Cardiomyopathy in diabetic MHC-PPAR mice was accompanied by myocardial long-chain triglyceride accumulation. The cardiomyopathic phenotype was exacerbated in MHC-PPAR mice fed a diet enriched in triglyceride containing long-chain fatty acid, an effect that was reversed by discontinuing the high-fat diet and absent in mice given a medium-chain triglyceride-enriched diet. Reactive oxygen intermediates were identified as candidate mediators of cardiomyopathic effects in MHC-PPAR mice. These results link dysregulation of the PPARalpha gene regulatory pathway to cardiac dysfunction in the diabetic and provide a rationale for serum lipid-lowering strategies in the treatment of diabetic cardiomyopathy.

Adenosine Triphosphate↗
PubMed2001

Current perspectives of partial left ventriculectomy in the treatment of dilated cardiomyopathy.

OBJECTIVES: Partial left ventriculectomy has been performed as an alternative to heart transplantation in the treatment of severe cardiomyopathies. This investigation documents the clinical and left ventricular (LV) function effects of this procedure, associated, when necessary, with mitral insufficiency correction, in 43 patients with idiopathic dilated cardiomyopathy. METHODS: Eighteen patients were in New York Heart Association class III and 25 in class IV. Seven of them were operated in cardiogenic shock. The procedure was associated with mitral annuloplasty in 32 patients and mitral replacement in three. RESULTS: Nine patients (20.9%) died during the hospital period and the cause of death was associated with ventricular failure in seven patients. The other patients were followed up from 2 to 57 months (mean, 28.3 months). At 6 months of follow-up, eight patients were in functional class I, 13 in class II, three in class III and one patient was in class IV (P<0.001). On the other hand, nine patients died during the first 6 months and another six in the later postoperative period. The cause of late death was progressive heart failure in eight patients, and seven patients died because of arrhythmia related events. The actuarial survival was 58.1+/-7.5% at 1 year and 43.9+/-8.1% at 4 years of follow-up. Regarding ventricular function modifications, the LV diastolic volume decreased by around 25% and the LV ejection fraction increased from 17.8+/-4.7 to 22.3+/-7.9% (P<0.001), whereas significant changes in the cardiac index, stroke index and pulmonary pressures were also found 1 month after the operation. In the later follow-up, despite the maintenance of hemodynamic improvement, the LV diastolic volume tended to increase and returned to preoperative levels at 4 years, while a concomitant decrease in the LV ejection fraction was also observed. CONCLUSION: Partial left ventriculectomy associated with mitral insufficiency correction improves LV function and ameliorates congestive heart failure in patients with idiopathic cardiomyopathy. Otherwise, the LV function benefits seem to be restricted by the possibility of progressive LV redilatation. Furthermore, the clinical application of this procedure is limited by the high mortality observed in the first postoperative months and by the possibility of heart failure progression and arrhythmia related events at late follow-up.

Adult↗
PubMed1971

Ventricular hypertrophy in cardiomyopathy.

Semantic difficulties arise when hypertrophic obstructive cardiomyopathy is seen without obstruction and with congestive failure, and also when congestive cardiomyopathy is seen with gross hypertrophy but without heart failure. Retention of a small left ventricular cavity and a normal ejection fraction characterizes hypertrophic cardiomyopathy at all stages of the disorder. Congestive cardiomyopathy is recognized by the presence of a dilated left ventricular cavity and reduced ejection fraction regardless of the amount of hypertrophy and the presence or not of heart failure. Longevity in congestive cardiomyopathy seems to be promoted when hypertrophy is great relative to the amount of pump failure as measured by increase in cavity size. Conversely, death in hypertrophic cardiomyopathy is most likely when hypertrophy is greatest at a time when outflow tract obstruction has been replaced by inflow restriction caused by diminishing ventricular distensibility. Hypertrophy is thus beneficial and compensatory in congestive cardiomyopathy, whereas it may be the primary disorder and eventual cause of death in hypertrophic cardiomyopathy. Reasons are given for believing that hypertension may have been the original cause of left ventricular dilatation in some case of congestive cardiomyopathy in which loss of stroke output thenceforward is followed by normotension. Development of severe hypertension in these patients after recovery from a prolonged period of left ventricular failure with normotension lends weight to this hypothesis. No fault has been found in the large or small coronary arteries in either hypertrophic cardiomyopathy or congestive cardiomyopathy when they have been examined in life by selective coronary angiography, or by histological methods in biopsy or post-mortem material. Coronary blood supply may be a limiting factor in the compensatory hypertrophy of congestive cardiomyopathy, and the ability to hypertrophy may explain the better prognosis of some patients. In hypertrophic cardiomyopathy excessive metabolic demand may not be met, and inadequacy of blood flow may contribute both to sudden death and to progressive replacement fibrosis in this disease. Histochemical and ultrastructural methods have failed to show any fundamental differences between hypertrophic cardiomyopathy and congestive cardiomyopathy, whereas conventional histology permits recognition of hypertrophic cardiomyopathy and distinction both from congestive cardiomyopathy and from ;normal' secondary hypertrophy in organic aortic stenosis.

Angiocardiography↗
PubMedDate not supplied

[Cardiomyopathy in children].

Cardiomyopathies (CMP) are rare diseases in childhood. There are three different types (dilated, hypertrophic and restrictive). Exact epidemiologic drawing are not well-known, and the numerous nosologic problems still exist. The basic classification distinguishes primary (not well-knows cause) and secondary CMP (cardiac changes have been developed as any influence of the well-known diseases of the cardiovascular system). Dilated forms make 55%, hypertrophic 40% and restrictive only 5% of all CMP. The tendency of this paper is introduce the knowledge of new approaches to cardiomyopathies, the definition of which was taken at 1983. The mean consideration includes etiology and therapeutic approach of different types of CMP. The chapter on dilated CMP was mainly aimed to discuss their development after virus myocarditis, immunologic processes and complicated therapeutic treatment (different in acute, subacute and chronic phase). The purpose of this paper is to call the attention on the CMP in children, as the main problem in pediatric cardiology after congenital heart diseases.

Cardiomyopathies↗
PubMed2002

Continuous spinal analgesia for labor and delivery in a parturient with hypertrophic obstructive cardiomyopathy.

Induction of labor under analgesia was planned for a 30-year-old-primiparous patient with hypertrophic obstructive cardiomyopathy (HOCM), as her fetal evaluation revealed intrauterine growth restriction at 38 weeks' gestation. However, regional analgesia during labor may present a potential risk for hemodynamic instability in patients with HOCM due to the possibility of a sympathetic block, as a result of vasodilation associated with the administration of local anesthesia. This case report demonstrates the successful management of the patient with analgesia provided by a continuous spinal catheter dosed with a continuous infusion of fentanyl and supplemental meperidine. Fetal surveillance monitoring included fetal pulse oximetry in addition to conventional cardiotocography, on the basis of which cesarean section was avoided.

Adult↗
PubMed1995

Restricted usage of T cell receptor V alpha-V beta genes in infiltrating cells in the hearts of patients with acute myocarditis and dilated cardiomyopathy.

Prolonged myocardial cell damage initiated by acute myocarditis is thought to be one of the most important etiology of dilated cardiomyopathy. To investigate the immunological mechanisms involved in the pathogenesis of dilated cardiomyopathy, we analyzed the phenotypes of infiltrating cells and examined the expression of perforin in infiltrating cells in the hearts of patients with dilated cardiomyopathy as well as acute myocarditis. We also examined the expression of HLA and intercellular adhesion molecule-1 (ICAM-1) in myocardial tissue of these patients. Furthermore, to evaluate the antigen specificity of infiltrating T cells and persistence of viral genomes in the myocardial tissue, we analyzed the expression of T cell receptor (TCR) V alpha and V beta genes as well as enterovirus genomes by PCR. We found infiltration of perforin-expressing killer cells and enhanced expression of HLA class I and ICAM-1 in the myocardial tissue. We also found that the repertoires of TCR V alpha as well as V beta gene transcripts were restricted, indicating that a specific antigen in the hearts was targeted. Because no enterovirus genomes were detected in all patients, it is strongly suggested that a cell-mediated autoimmune mechanism triggered by virus infection may play a critical role in the pathogenesis of dilated cardiomyopathy. However, we could not exclude the possibility that viruses other than enteroviruses could be pathogenic in these patients.

Acute Disease↗