Primary Cardiomyopathy Research Articles

Labour analgesia guided by echocardiography in a parturient with primary dilated cardiomyopathy

To evaluate the effects of intrathecal analgesics on cardiac function during labour analgesia using echocardiography in a parturient with idiopathic dilated cardiomyopathy (DCM). Induction of labour was planned in a 35-yr-old primiparous woman suffering from DCM. In order to stabilize hemodynamics in this patient, we induced continuous spinal analgesia with an infusion of fentanyl and epinephrine. Although her analgesia was well maintained for three hours during the first stage of labour, the patient complained of pain towards the second stage of labour. At this point, we administered bupivacaine intrathecally to alleviate her pain. Transthoracic echocardiography showed that the left ventricular end-diastolic and systolic dimensions, as well as the ejection fraction were not impaired by use of these analgesic medications. Measurement of left ventricular dimensions by echocardiography allowed us to monitor the patient's response to intrathecal analgesic medications. In this patient with DCM, analgesia with intrathecal fentanyl and bupivacaine was well tolerated.

121 Recontruction of mitral and tricuspide valves in patients with primary dilated cardiomyopathy

121 Recontruction of mitral and tricuspide valves in patients with primary dilated cardiomyopathy

PKC-ε-dependent survival signals in diabetic hearts

Diabetes mellitus is complicated by the development of a primary cardiomyopathy, which contributes to the excess morbidity and mortality of this disorder. The protein kinase C (PKC) family of isozymes plays a key role in the cardiac phenotype expressed during postnatal development and in response to pathological stimuli. Hyperglycemia is an activating signal for cardiac PKC isozymes that modulate a myriad of cell events including cell death and survival. The ε-isozyme of the PKC family transmits a powerful survival signal in cardiac muscle cells. Accordingly, to test the hypothesis that endogenous activation of cardiac PKC-ε will protect against hyperglycemic cell injury and left ventricular dysfunction, diabetes mellitus was induced using streptozotocin in genetically engineered mice with cardiac-specific expression of the PKC-ε translocation activator [ψε-receptors for activated C kinase (ψε-RACK)]. The results demonstrate a striking PKC-ε cardioprotective phenotype in diabetic ψε-RACK (ε-agonist) mice that is characterized by inhibition of the hyperglycemia apoptosis signal, attenuation of hyperglycemia-mediated oxidative stress, and preservation of parameters of left ventricular pump function. Hearts of diabetic ε-agonist mice exhibited selective trafficking of PKC-ε to membrane and mitochondrial compartments, phosphorylation/inactivation of the mitochondrial Bad protein, and inhibition of cytochrome c release. We conclude that activation of endogenous PKC-ε in hearts of diabetic ε-agonist mice promotes the survival phenotype, attenuates markers of oxidative stress, and inhibits the negative inotropic properties of chronic hyperglycemia.

Molecular genetics of sudden cardiac death in small animals – A review

Sudden cardiac death in small animals is uncommon but often occurs due to cardiac conduction defects or myocardial diseases. Primary cardiac conduction defects are mainly caused by mutations in genes involved in impulse conduction processes (e.g., gap-junction genes and transcription factors) or repolarisation processes (e.g., ion-channel genes), whereas primary cardiomyopathies are mainly caused by defective force generation or force transmission due to gene mutations in either sarcomeric or cytoskeleton proteins. Although over 50 genes have been identified in humans directly or indirectly related to sudden cardiac death, no genetic aetiologies have been identified in small animals. Sudden cardiac deaths have been also reported in German Shepherds and Boxers. A better understanding of molecular genetic aetiologies for sudden cardiac death will be required for future study toward unveiling aetiology in sudden cardiac death in small animals.

Fazioskapulohumerale Muskeldystrophie mit Herzbeteiligung

Cardiac involvement is well known in a number of skeletomuscular diseases but not in facio-scapulohumeral muscular dystrophy (FSHD). We report on a 71 year old woman with progressive cardiac insufficiency in FSHD, which was also confirmed by molecular analysis in one of the two daughters affected by the disease. Autopsy of the deceased patient showed the typical changes in skeletal muscles including focal inflammatory infiltrates in the diaphragm and, in addition, cardiac muscular involvement. The histological changes resembled those seen in primary cardiomyopathy despite the normal muscle mass volume. Both clinically and morphologically, the cardiac disease was the cause of death in this patient with FSHD.

Der plötzliche Herztod bei familiärer hypertropher Kardiomyopathie

Hypertrophic cardiomyopathy (HCM) is a relatively frequent, genetically determined primary cardiomyopathy, characterized by most often asymmetric hypertrophy of the ventricular septum with or without systolic obstruction of the left ventricular outflow tract. HCM is a genetically heterogeneous disease, with 12 different disease-causing genes beeing indentified to date. Histologically the disease is characterized by hypertophy and disarray of myofibrils as well as by an increase in myocardial fibrosis. Clinically, these changes may lead to palpitations, dyspnoe on exertion, and/or angina pectoris. However, they also lead to an increased propensity to the development of severe ventricular tachyarrhythmias and sudden cardiac death. The incidence of sudden death is significantly increased in HCM, particularly in affected young subjects. Risk stratification in HCM should include a complete clinical-cardiological evaluation that should also consider new diagnostic features, e. g. MR imaging. Major risk factors for sudden cardiac death include a survived cardiac arrest (ventricular fibrillation), non-sustained and sustained ventricular tachycardia, a history of premature familial sudden death, unexplained syncope, an abnormal blood pressure response on exercise, and left ventricular thickness greater than or equal to 3 cm. Ideally, risk stratification should also include genetic testing, since some gene mutations seem to be associated with a higher risk for sudden cardiac death than others. However, genetic testing in HCM in not yet available on a routine basis. The implantation of a cardioverter/defibrillator is first-line therapy in patients with documented ventricular tachycardia/fibrillation or patients who have survived sudden cardiac death. These devices also play an important role in the primary prevention of sudden cardiac death in HCM. Algorithms and scores are available to estimate the risk of sudden death, however, the decision to implant a cardioverter/defibrillator remains an individual decision in every single patient.

Trasplante autólogo de médula ósea: propuesta basada en la evidencia, para un consenso en enfermedad cardiovascular

La falla cardíaca es un síndrome clínico heterogéneo que se desarrolla después de un "evento índice", el cual produce daño del tejido muscular cardíaco por la pérdida de cardiomiocitos o por la alteración de la capacidad del miocardio para generar una fuerza eficiente para la contracción ventricular. La noxa inicial puede ser de aparición súbita como en el síndrome coronario agudo o insidiosa y gradual como en la enfermedad coronaria crónica y las cardiomiopatías primarias y secundarias. Independiente del factor que genere el síndrome, se produce una serie de respuestas hemodinámicas y neurohormonales con cambios moleculares complejos, que conducen a la progresión de la disfunción ventricular y a mayor pérdida de células contráctiles funcionantes por necrosis o por apoptosis. Las estrategias terapéuticas desarrolladas para interrumpir este círculo vicioso de disfunción miocárdica y remodelación ventricular, cambian las condiciones en que se produce el trabajo miocárdico, con la consecuente mejoría de la enfermedad y de la tasa de supervivencia. Sin embargo, este enfoque terapéutico, conocido como la "aproximación Jurásica", no logra recuperar el tejido lesionado durante el proceso, lo que permite la progresión de la enfermedad hasta llegar a la falla cardíaca terminal y la muerte. Estudios experimentales y clínicos han demostrado que la implantación de células progenitoras derivadas de la médula ósea producen angiogénesis y recuperación de la función cardíaca en pacientes con cardiopatía isquémica aguda y crónica. Con esta terapia se ha demostrado mejoría significativa de la sintomatología, de la capacidad funcional, del estado funcional, del tamaño del defecto de perfusión miocárdica medido por pruebas de medicina nuclear y de la fracción de eyección al igual que disminución del volumen sistólico final del ventrículo izquierdo, sin complicaciones relacionadas con el procedimiento. En conclusión, el trasplante de células progenitoras derivadas de la médula ósea es una terapia efectiva y segura para promover la neovascularización y mejorar la contractilidad y la perfusión miocárdicas en los pacientes con cardiopatía isquémica aguda o crónica, que sean pobres candidatos para la cirugía.

Assessment of inotropic reserve during repetitive exercise in patients with primary dilated cardiomyopathy by radionuclide ambulatory monitoring of left ventricular function (II C-VEST)

Assessment of inotropic reserve during repetitive exercise in patients with primary dilated cardiomyopathy by radionuclide ambulatory monitoring of left ventricular function (II C-VEST)

Glycogen Storage Diseases Presenting as Hypertrophic Cardiomyopathy

Unexplained left ventricular hypertrophy often prompts the diagnosis of hypertrophic cardiomyopathy, a sarcomere-protein gene disorder. Because mutations in the gene for AMP-activated protein kinase gamma2 (PRKAG2) cause an accumulation of cardiac glycogen and left ventricular hypertrophy that mimics hypertrophic cardiomyopathy, we hypothesized that hypertrophic cardiomyopathy might also be clinically misdiagnosed in patients with other mutations in genes regulating glycogen metabolism. Genetic analyses performed in 75 consecutive unrelated patients with hypertrophic cardiomyopathy detected 40 sarcomere-protein mutations. In the remaining 35 patients, PRKAG2, lysosome-associated membrane protein 2 (LAMP2), alpha-galactosidase (GLA), and acid alpha-1,4-glucosidase (GAA) genes were studied. Gene defects causing Fabry's disease (GLA) and Pompe's disease (GAA) were not found, but two LAMP2 and one PRKAG2 mutations were identified in probands with prominent hypertrophy and electrophysiological abnormalities. These results prompted the study of two additional, independent series of patients. Genetic analyses of 20 subjects with massive hypertrophy (left ventricular wall thickness, > or =30 mm) but without electrophysiological abnormalities revealed mutations in neither LAMP2 nor PRKAG2. Genetic analyses of 24 subjects with increased left ventricular wall thickness and electrocardiograms suggesting ventricular preexcitation revealed four LAMP2 and seven PRKAG2 mutations. Clinical features associated with defects in LAMP2 included male sex, severe hypertrophy, early onset (at 8 to 17 years of age), ventricular preexcitation, and asymptomatic elevations of two serum proteins. LAMP2 mutations typically cause multisystem glycogen-storage disease (Danon's disease) but can also present as a primary cardiomyopathy. The glycogen-storage cardiomyopathy produced by LAMP2 or PRKAG2 mutations resembles hypertrophic cardiomyopathy but is distinguished by electrophysiological abnormalities, particularly ventricular preexcitation.

International Society for Heart and Lung Transplantation: Practice guidelines for management of heart failure in children

Heart failure (HF) in the United States is well recognized as a major public health problem, with over 900,000 hospital admissions annually in the United States, and greater than 250,000 deaths per year. The great majority of heart failure occurs in adults. In children, the scope of the problem is less well defined, but recent data from the Pediatric Cardiomyopathy Registry suggest an annual incidence of 1.13 cases of cardiomyopathy per 100,000 children. 1 While some of this represents asymptomatic disease, the burden of disease overall is nonetheless quite high. In the Pediatric Cardiomyopathy Registry, the majority of children with cardiomyopathy also had HF, with mortality rates of 13.6% at 2 years in dilated forms of cardiomyopathy. The etiology of heart failure differs greatly between children and adults. Children in the Pediatric Cardiomyopathy Registry had a recognizable syndrome or genetic diagnosis in 27% of cases, with an additional 5% of cases due to myocarditis. Furthermore, a large percentage of children with end-stage HF (between 25% and 75%, depending upon the age group) have an underlying diagnosis of congenital heart disease. 2 In contrast to adult patients, ischemic heart disease is rare in children. There is a large, and rapidly growing literature addressing HF treatment for adult patients, with a much smaller literature concerning HF therapy in children. Excellent guidelines for adult patients have recently been published, but given the significant differences between adult and pediatric patients with HF, there is little reason to believe that these guidelines are directly applicable to children. 3 Accordingly, in this document we have attempted to summarize the relevant literature and synthesize management guidelines for children with HF. The document that follows has been prepared in a consensus fashion, with input from pediatric cardiologists at multiple sites throughout the United States and Canada.

Myocardial perfusion imaging in pediatric cardiology

Myocardial perfusion imaging (MPI) is an important procedure in pediatric cardiology in terms of evaluating myocardial ischemia, infarction and damage associated with various congenital or acquired heart diseases, such as Kawasaki disease, anomalous origin of the left coronary artery from the pulmonary artery and complete transposition of the great arteries after arterial switch surgery. This type of imaging can detect myocardial damage in the morphological right ventricle when it functions as a systemic pumping chamber in patients with complex congenital heart diseases after intra-cardiac repair. Myocardial perfusion imaging can also evaluate myocardial damage associated with primary or secondary cardiomyopathy in children. The magnitude of increased right ventricular uptake on MPI is a useful noninvasive means of estimating right ventricular pressure overload due to congenital heart or pulmonary diseases. This article reviews myocardial perfusion tracers and pharmacological stress tests used to diagnose heart conditions in children, and the current clinical roles of MPI in pediatric cardiology.

Differentiation of constrictive pericarditis from restrictive cardiomyopathy using mitral annular velocity by tissue Doppler echocardiography

This study evaluated the diagnostic role of early diastolic mitral annular velocity (E′) by tissue Doppler echocardiography for differentiating constrictive pericarditis from restrictive cardiomyopathy (primary restrictive cardiomyopathy and cardiac amyloidosis). The study group consisted of 75 patients (53 men, 22 women; mean age 62 years, range 27 to 87). Of these, 23 patients had surgically confirmed constrictive pericarditis, 38 had biopsy-proved systemic amyloidosis and typical echocardiographic features of cardiac involvement, and 14 had primary restrictive cardiomyopathy. Standard mitral inflow characteristics were measured. Tissue Doppler echocardiography was used to measure E′ at the septal annulus. E′ was significantly higher in patients with constrictive pericarditis than in those with primary restrictive cardiomyopathy or cardiac amyloidosis (12.3 vs 5.1 cm/second, p <0.001). An E′ cut-off value ≥8 cm/second resulted in 95% sensitivity and 96% specificity for the diagnosis of constrictive pericarditis. There was no overlap of E′ between patients who had constrictive pericarditis and those who had cardiac amyloidosis. In a subgroup analysis of restrictive cardiomyopathy, E′ of patients who had cardiac amyloidosis was significantly lower than that of patients who had primary restrictive cardiomyopathy (4.6 vs 6.3 cm/second, p <0.001). Thus, E′ velocity can distinguish between constrictive pericarditis and restrictive cardiomyopathy with a specific cut-off value in patients with clinical and echocardiographic evidence of diastolic heart failure.

Transgenic rescue of neurogenic atrophy in the nmd mouse reveals a role for Ighmbp2 in dilated cardiomyopathy.

Immunoglobulin mu binding protein 2 (IGHMBP2) is a DNA/RNA helicase with a putative role in transcriptional regulation and splicing. A recessive mutation of the Ighmbp2 gene in neuromuscular degeneration (nmd) mice causes progressive neurogenic atrophy of limb muscles. Affected mice show significant loss of motor neurons with large caliber axons and a moderate reduction of neurons with small caliber axons in the ventral nerve roots of the spinal cord. To investigate the role of Ighmbp2 in the pathogenesis of neuromuscular degeneration, we generated two independent lines of transgenic mice expressing the full-length Ighmbp2 cDNA specifically in neurons. Histopathological evaluation of L4 ventral nerve roots revealed that transgenic expression of the Ighmbp2 cDNA prevented primary motor neuron degeneration, while restoring the normal axonal morphology and density in nmd mice. A similar neuronal improvement is found in mutant mice carrying the CAST/EiJ-derived modifier of nmd (Mnm(C)). Intriguingly, both the transgenic and modified nmd mice went on to develop a previously unobserved cardiac and skeletal myopathy. Necropsy of nmd mice in end-stage heart failure revealed a primary dilated cardiomyopathy with secondary respiratory failure that was confirmed by in vivo ECG and echocardiographic measures. Our results suggest that reduced levels of IGHMBP2 in nmd mice compromise the integrity and function not only of motor neurons but also of skeletal and cardiac myocytes. These findings highlight the important role of IGHMBP2 in the maintenance and survival of these terminally differentiated cell types.

Effects of amino acid supplementation on left ventricular remodeling in patients with chronic heart failure with decreased systolic function and diabetes mellitus: rationale and design of a magnetic resonance imaging study

A considerable number of patients with reduced systolic function caused by primary or ischemic cardiomyopathy have viable and noncontractile myocardium. This may be related to numerous and perhaps overlapping factors, such as chronic ischemia (stunning/hibernation), neurohormonal abnormalities, oxidative stress, metabolic imbalances, and/or nutritional depletion. Changes in myocardial substrate utilization have adverse effects on the metabolism of the viable but noncontractile myocardium. Shifting the energy substrate preference away from fatty acids and replenishing the tricarboxylic acid cycle components via amino acids rather than via fatty acids would increase adenosine triphosphate production, with positive effects on cellular metabolism. A proposed study design is described and will be piloted through the Effects of Diatrofen on Myocardial Function in Patients with Chronic Heart Failure trial (D-CHF), an evaluation of an oral amino acid supplementation treatment in outpatients with heart failure.

Effects of amino acid supplementation on left ventricular remodeling in patients with chronic heart failure with decreased systolic function and diabetes mellitus: rationale and design of a magnetic resonance imaging study

A considerable number of patients with reduced systolic function caused by primary or ischemic cardiomyopathy have viable and noncontractile myocardium. This may be related to numerous and perhaps overlapping factors, such as chronic ischemia (stunning/hibernation), neurohormonal abnormalities, oxidative stress, metabolic imbalances, and/or nutritional depletion. Changes in myocardial substrate utilization have adverse effects on the metabolism of the viable but noncontractile myocardium. Shifting the energy substrate preference away from fatty acids and replenishing the tricarboxylic acid cycle components via amino acids rather than via fatty acids would increase adenosine triphosphate production, with positive effects on cellular metabolism. A proposed study design is described and will be piloted through the Effects of Diatrofen on Myocardial Function in Patients with Chronic Heart Failure trial (D-CHF), an evaluation of an oral amino acid supplementation treatment in outpatients with heart failure.

Échocardiographie du sujet âgé

IN THE CONTEXT OF AGEING: The Doppler echocardiography is a non-invasive technique that permits assessment of the "physiological" ageing of the cardiac and vascular structures, notably including a concentric remodelling of the left ventricle associated with relaxation abnormalities, dilatation of the left atrium, valvular reorganisation and a modification in the large vessels. IN A PATHOLOGICAL CONTEXT: The Doppler echocardiography also detects the various cardiovascular affections related to ageing: valvulopathies, notably calcified aortic stenosis and mitral failure due to mitral anulus calcification or prolapsus of the valve; primary hypertrophic cardiomyopathy or secondary to arterial hypertension or an amyloidosis, and possibly leading to heart failure with spared systolic function, frequent in elderly patients; ischemic cardiopathies that have benefited, as in younger patient, from new echographical stress testing techniques, which safely study the variability in myocardial ischemia. Transoesophageal echography can also be performed in elderly patients, but the indications of this more invasive and less well-tolerated examination must be assessed case by case. It is very useful when an intra-parietal aortic hematoma is suspected or during aortic dissection or infectious endocarditis.

Fetal middle cerebral artery peak systolic velocity in the investigation of non-immune hydrops.

In some cases of non-immune hydrops there is congenital or acquired fetal anemia. The aim of this study was to investigate the potential value of fetal middle cerebral artery peak systolic velocity (MCA-PSV) in the assessment and management of non-immune hydrops due to anemia. Fetal MCA-PSV and fetal hemoglobin concentration, in blood obtained by cordocentesis, were measured in 16 singleton pregnancies referred to our unit for further investigations because of a diagnosis of non-immune hydrops fetalis. In all cases a detailed ultrasound examination demonstrated moderate or severe ascites, with or without skin edema, and pericardial or pleural effusions. Furthermore, there were no obvious malformations to account for the hydrops. In each fetus the measured MCA-PSV and hemoglobin concentration were expressed as delta values (the difference in SD from the normal mean for gestation). Regression analysis was used to determine the significance of the association between delta MCA-PSV and delta fetal hemoglobin concentration. In addition, we searched our database to identify the sonographic features and hemoglobin concentration of fetuses with congenital infection. In the 16 cases of non-immune hydrops there were seven with parvovirus B19 infection, one each of alpha-thalassemia and primary cardiomyopathy and seven with no obvious explanation for the hydrops. There was a significant association between delta MCA-PSV and delta hemoglobin concentration (delta hemoglobin = (delta MCA-PSV + 0.1437)/-0.4154; R(2) = 0.7202; P < 0.0001). In 10 of the cases the fetal hemoglobin concentration was more than 4 SD below the normal mean for gestation and in all these cases the MCA-PSV was more than 2 SD above the normal mean for gestation. Our computer search identified an additional nine fetuses with parvovirus B19 infection and in all cases the predominant sonographic finding was ascites and the hemoglobin concentration was more than 4 SD below the normal mean. In contrast, only 3/14 fetuses with cytomegalovirus, toxoplasmosis, coxsackie B or Treponema infection had ascites and only 2/14 had a hemoglobin deficit of 4-6 SD. In the management of non-immune hydrops, measurement of fetal MCA-PSV can help identify the subgroup with fetal anemia.

323 Assessment of mitral annulus dilatation in patients with primary dilated cardiomyopathy before and after posterior semicircular reductive annuloplasty

Aetiology of ischemic heart disease has been shown to be associated with worse prognosis than idiopathic aetiology in patients with chronic heart failure. Other reports showed that survival was worse for idiopathic dilated cardiomyopathy or was unrelated to aetiology. Due to these conflicting results, large therapeutic multicentre heart failure trials included patients regardless of aetiology. We hypothesized that patient group selection bias, for example the study of heart transplant candidates, may explain these conflicting results. To determine whether ischemic or idiopathic causes of cardiomyopathy were associated with prognosis, 287 patients with LV ejection fraction (EF) 6.0cm were followed prospectively. LVEF was assessed by visual estimation, M-mode echo (when there were no regional wall motion abnormalities or left bundle branch block), Simpson’s rule and systolic mitral annular velocity. Patients had invasively proven ischemic or idiopathic dilated cardiomyopathy. Advances in medical therapy were systematically implemented over a mean follow-up period of 3.7±3.3 years. The cause was idiopathic in 52% of patients and ischemic in 48%. There was no significant difference in age (45±13 versus 48±18years), sex (male 68 versus 71%), LVEF (24± 8v ersus 25±7%) and LV end diastolic diameter (7.0±1.0 versus 6.8±0.8cm) between idiopathic and ischemic cardiomyopathy patients. When patients with heart transplantation were considered as deaths (n=43), there was no significant difference in total mortality between the 2 groups (p= 0.65 logrank test). With multiple logistic regression analysis, NYHA functional class and LVEF were identified as the variables most closely associated with mortality (p=0.007 and 0.03 respectively) among patients with idiopathic cardiomyopathy. In ischemic patients, only peak oxygen consumption was related to mortality (p=0.01). Left bundle branch block or QRS duration >120ms failed to predict outcome. Conclusion: Aetiology of ischemic heart disease was not an independent predictor of mortality in patients with severe chronic heart failure in this prospective study.

“Demand” stimulation of latissimus dorsi heart wrap: experience in humans and comparison with adynamic girdling

Background Questionable systolic assistance and latissimus dorsi (LD) muscular degeneration as a result of continuous electrical stimulation constitute important drawbacks to dynamic cardiomyoplasty. To avoid full transformation of the LD and thereby cause better systolic assistance, a new stimulation protocol was developed. Fewer impulses per day are delivered so that the LD wrap has daily periods of rest (demand), based on a heart rate cutoff. We describe our experience of demand dynamic wrapping by discriminating between patients with active systolic assistance and those with a passive girdle effect (adynamic-girdling). Methods Fourteen patients with primary dilated cardiomyopathy (13 men, 1 woman; mean age, 58.2 ± 5.8 years; 12 sinus rhythm, 2 atrial fibrillation) underwent dynamic cardiomyoplasty between 1993 and 1996 as well as the demand protocol at different intervals. Clinical, echocardiographic, mechanographic, and cardiac invasive assessment records, as well as cardiovascular events (death and arrhythmias), were retrospectively reviewed. The patients were divided into two groups on the basis of the mechanographic measurement of speed of contraction of the heart wrap, as measured by tetanic fusion frequency analysis before starting demand stimulation: demand dynamic wrapping patients with fast LD (high tetanic fusion frequency, 7 patients), and adynamic-girdling patients with slow LD contraction times (low tetanic fusion frequency, 7 patients). It was assumed that in adynamic-girdling patients dynamic assistance was virtually absent, so the wrapping acted only as a passive constraint wall. Results The two groups were comparable for sex, age, dilated cardiomyopathy cause, New York Heart Association class, and left ventricular ejection fraction at the start of the demand protocol period. After a mean duration of follow-up of 41.4 ± 21.1 months (range, 23 to 69 months), the demand dynamic wrapping group showed improved New York Heart Association class (1.14 ± 0.34 versus 2.07 + 0.18; p = 0.0004), higher values of left ventricular ejection fraction (34.6 ± 8.0 versus 26.5 ± 3.1; p = 0.005) and LD wrap tetanic fusion frequency (38.3 ± 5.88 versus 24.3 ± 2.93; p = 0.002), and a better survival (85.7% versus 28.6%; p = 0.037) than the adynamic-girdling group. Conclusions Demand dynamic wrapping offers good results in terms of fewer cardiovascular events and greater survival. When compared with the passive constraint effect of LD muscle, demand dynamic wrapping proved to be more effective.

Usefulness of 1H MR spectroscopy in the evaluation of myocardial metabolism in patients with dilated idiopathic cardiomyopathy : pilot study 1

Rationale and Objectives. Primary dilated cardiomyopathy is a disease of unknown etiology, and it leads to severe heart failure. Abnormalities of the cardiac metabolism can play an important role in prognosis and influence the symptomatology in this group. The aim of this study was to assess cardiac metabolism using proton spectroscopy magnetic resonance ( 1H MRS) and to examine whether there is any correlation between cardiac metabolites and functional New York Heart Association (NYHA) class and left ventricular function parameters obtained in echocardiography. Materials and Methods. Fifteen patients with documented idiopathic dilated cardiomyopathy and 12 healthy volunteers were examined. The study protocol included clinical examination, 12-lead electrocardiogram, two-dimensional echocardiogram, and 1H MRS with voxel localized at interventricular septum area. The contents of total creatine (CR) ie, creatine + phosphocreatine, lipids (LIP), lactates (LAC) and their ratios (CR1A, CR2A, CR1/H20, CR2/H20, CR2/CR1, LIPA, LIP/H20, LIP/CR1, LACA, LAC/H20, LAC/CR1) were examined. Results. Patients with dilated cardiomyopathy had significantly lower levels of creatine CR1A (5.04 ± 0.88 vs 5.94 ± 1.15, P < .02) and ratios LIP/H20 (4.34 ± 2.3 vs 15.46 ± 20.39, P < .04) and LIP/CR1 (24.49 ± 21.26 vs 34.08 ± 13.36, P < .05) compared with healthy volunteers. Significant correlations between NYHA functional class and the ratios CR2/CR1, CR2/H20 ( r = 0.59, P < .038, r = 0.59, P < .02, respectively) and between %LVEF and LIP/CR1 ( r = 0.64, P < .036), as well as between the duration of the disease (TCH) and LIP/CR1 ( r = 0.67, P < .046) were found. Conclusion. A pilot study with proton spectroscopy magnetic resonance showed impairment of the cardiac metabolism in patients with idiopathic dilated cardiomyopathy. A trend to lower values of creatine and lipids, and to lower ratios of some of these metabolites was observed in the dilated cardiomyopathy group compared with healthy subjects. The results require further study.