Cadiomyopathy

BACKGROUND: Fatty acid oxidation disorders (FAODs) include more than 15 distinct disorders with variable clinical manifestations. After the introduction of newborn screening using tandem mass spectrometry, early identification of FAODs became feasible. This study describes the clinical, biochemical and molecular characteristics of FAODs patients detected by newborn screening (NBS) compared with those of 9 patients with symptomatic presentations. METHODS: Clinical and genetic features of FAODs patients diagnosed by NBS and by symptomatic presentations were reviewed...

Objective: To observe the long-term prognosis and related outcome predictors for hypertrophic obstructive cadiomyopathy (HOCM) patients underwent alcohol septal ablation (ASA). Methods: A total of 227 consecutive patients(age: (47.8±11.7) years) treated by ASA from September 2005 to December 2013 in our hospital were included and followed-up for 4.42 years(range: ( 1.17-9.93) years). Follow up rate is 97.4%(221/227). General information, medical history, data of ASA and complications during hospitalization were obtained through access to medical records of patients...

Peripartum cardiomyopathy (PPCM) is defined as onset of acute heart failure without demonstrable cause in last trimester of pregnancy or within the first 6 months after delivery. We report a case of PPCM with left ventricular ejection fraction less than 25% who had reported to us at 38 weeks of gestation for emergency caesarean section managed with graded epidural anaesthesia. PPCM is a form of dilated cadiomyopathy with left ventricular systolic dysfunction that results in signs and symptoms of heart failure...

Pompe disease is characterized by deficiency or absence of activity of the lysosomal enzyme acid alpha-glucosidase. As a result of ineffective metabolism, glycogen progressively accumulates in muscle tissues. Patients with an aggressive classic infantile-onset form generally rapidly die of cardiorespiratory failure. A cross-reactive immunological material (CRIM)-negative status is predictive of high anti-alglucosidase alfa antibody titers and usually a poor clinical outcome of enzyme replacement therapy (ERT)...

BACKGROUND: Aneurysmal subarachnoid hemorrhage (aSAH) is an often devastating form of stroke. Aside from the initial hemorrhage, cardiac complications can occur resulting in neurogenic stress cardiomyopathy (NCM), leading to impaired cardiac function. We investigated whether aSAH patients with NCM had poorer long term functional outcomes than patients without NCM. Mortality, vasospasm, and delayed ischemic complications were also evaluated. METHODS: A retrospective study of all patients admitted for aneurysmal subarachnoid hemorrhage (aSAH) from January 2006 to June 2011 (n=299) was conducted...

Following Latin American migration, Chagas disease has inevitably appeared in non-endemic countries in Europe and elsewhere. New policies are necessary to prevent transmission in those countries but the long, often undetected chronic period of the early stages of the disease also renders epidemiological studies important. The main objective of our study was to determine the presence of clinical, electrocardiogram (ECG) and echocardiographic abnormalities in a population of Latin American migrants infected with Trypanosoma cruzi at the moment of diagnosis...

Portal hypertension (PHT) is associated with hemodynamic changes in intrahepatic, systemic, and portosystemic collateral circulation. Increased intrahepatic resistance and hyperdynamic circulatory alterations with expansion of collateral circulation play a central role in the pathogenesis of PHT. PHT is also characterized by changes in vascular structure, termed vascular remodeling, which is an adaptive response of the vessel wall that occurs in response to chronic changes in the environment such as shear stress...

Since 1971, in Italy every citizen engaged in competitive sports activity must periodically undergo preventive medical examinations to ascertain eligibility for sports participation. The medical examination includes rest and effort ECG. The Italian model has a very good sensitivity in identifying most pathologies at risk of sudden death particularly in the young (hypertrophic cadiomyopathy, arrhythmogenic right ventricular disease, chanelopathies). In this model, the ECG is very important, being able to disclose cardiac anomalies in most cases...

Cardiac troponin T (CTT) is elevated acutely in animal models of anthracycline cardiotoxicity. We assessed CTT release in children receiving anthracylines using a third generation assay. We measured CTT in 30 children receiving anthracycline chemotherapy. A total of 3 samples were taken from each child: 1) prior to, 2) immediately after and 3) between 24-48 hours after the infusion. The dose range given during the measurements of cardiac troponin T was 15-60 (median 25) mg/m(2) and the previous exposure ranged from 0-300 (median 150) mg/m(2)...

In this study, we investigate Nitric oxide synthase (NOS) expressions in adriamycin (ADR)-induced cadiomyopathy in rats. Sixty male Wistar rats were randomly divided into two main groups: control and ADR groups. Myocardial histopathological observation was performed; Expressions of 3 isoforms of NOS genes were examined by RT-PCR analysis; Expressions of 3 isoforms of NOS protein was assessed by Western blot analysis. Myocardium exhibited intensive morphological changes after 8 weeks of ADR treatment. The expression levels of inducible NOS (iNOS) gene and protein were significantly increased in ADRtreated rats after 8 weeks of treatment and then slightly increased at weeks 9 and 10...

Over the past several years interest regarding the causes of sudden deaths in young trained athletes has heightened in the medical community. 36% of sudden cardiac death (SCD) in young competitive athletes is due to hypertrophic cadiomyopathy (HCM), while 10% of SCD cases in sports are due to increased cardiac mass without signs of HCM. The goal of this study was to evaluate systolic and diastolic functions, and to determine the clinical significance of electrocardiographic patterns in highly trained football players with increased left ventricular mass index (LVMI) > 125g/m(2)...

Malonyl-CoA decarboxylase (E.C.4.1.1.9) catalyzes the conversion of malonyl-CoA to acetyl-CoA. Although the metabolic role of this enzyme has not been fully defined, it has been reported that its deficiency is associated with mild mental retardation, seizures, hypotonia, cadiomyopathy, developmental delay, vomiting, hypoglycemia, metabolic acidosis, and malonic aciduria. Here, we isolated a cDNA clone for malonyl CoA decarboxylase from a rat brain cDNA library, expressed it in E. coli, and characterized its biochemical properties...

A 70-year-old man who had been drinking a bottle of whisky each day was scheduled for laser resection of a tongue tumor. His electrocardiogram showed sinus bradycardia (heart rate was 35-40 bpm), and transient complete heart block was observed. Echocardiography showed hypertrophic cardiomyopathy. After a temporary transvenous pacemaker had been inserted, anesthesia was induced with thiopental and vecuronium bromide, and maintained with sevoflurane and fentanyl. Heart rate was 45.min-1 before the induction of anesthesia, and after the induction increased to 70-80...

UNLABELLED: A concept of volume reduction surgery for patients with end-stage dilated cardiomyopathy was introduced to the surgical aspect. We started consequently to employ this operation for patients with ischemic cadiomyopathy. Between October 1997 and April 1999, 14 patients (mean age 64 years; 12 men, 2 women) underwent endoventricular circular patch plasty (EVCPP) in our institute. TECHNIQUE: The left ventricle (LV) is opened by placing an incision parallel to the left anterior descending (LAD) coronary artery...

BACKGROUND: The aim of this study was to evaluate the clinical utility of using signal-averaged acoustic quantification (SAAQ) waveforms for improved assessment of left ventricular (LV) size and function. METHODS AND RESULTS: Four separate protocols were performed in 47 subjects. SAAQ waveforms were used to assess alterations in LV function induced by dobutamine (15 microg/kg per minute) and esmolol (200 microg/kg per minute) in eight normal subjects. Subsequently, we analyzed SAAQ waveforms obtained in 12 patients with LV dysfunction secondary to dilated cardiomyopathy and 12 age-matched normal subjects...

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The Nimbus pump is an indwelling, electromagnetic powered left ventricular (LV) assist device inserted transfemorally. The inflow cannula (7 mm) is positioned across the aortic valve and the pump in the descending aorta. Indications for insertion include cardiogenic shock despite maximal medical support and PCWP greater than 18 mmHg, MAP less than 90 mmHg, and Cl less than 2 L/min/m2. Twelve patients underwent the attempt at surgical insertion of the Nimbus pump. Diagnoses included eight acute myocardial infarctions (AMI), two ischemic cardiomyopathy, one postpartum cardiomyopathy, and one transplanted heart rejection...