ABSTRACT
BACKGROUND: There is some controversy concerning the prognosis of patients with left ventricular hypertrabeculation/noncompaction (LVHT). LVHT is frequently associated with neuromuscular disorders (NMDs). The aim of this study was to assess cardiac and neurological findings as predictors of mortality in patients with LVHT. PATIENTS AND METHODS: The study included patients with LVHT diagnosed between June 1995 and January 2014 in one echocardiographic laboratory. They underwent a baseline cardiologic examination and were invited for a neurological examination. Between January and February 2014, their survival status was assessed. RESULTS: LVHT was diagnosed in 220 patients (68 female, aged 52 ± 17 years) with a prevalence of 0.35 %/year. During a follow-up of 72 ± 61 months, 65 patients died. The mortality was 5 %/year. A neurological investigation was performed on 173 patients (79 %) and revealed specific NMDs in 31 (14 %), NMD of unknown etiology in 103 (47 %), and normal findings in 39 (18 %) patients. In multivariate analysis, the predictors of mortality were increased age (p = 0.0001), presence of a specific NMD (p = 0.0062) or NMD of unknown etiology (p = 0.0062), heart failure NYHA III (p = 0.0396), atrial fibrillation (p = 0.0022), and sinus tachycardia (p = 0.0395). CONCLUSIONS: LVHT patients should undergo systematic neurological examinations. Whether an optimal therapy of heart failure and atrial fibrillation will improve the prognosis of LVHT patients needs to be addressed in further studies.
Subject(s)
Atrial Fibrillation/mortality , Heart Defects, Congenital/mortality , Heart Failure/mortality , Neuromuscular Diseases/mortality , Tachycardia, Sinus/mortality , Adolescent , Adult , Age Distribution , Aged , Aged, 80 and over , Atrial Fibrillation/diagnosis , Austria/epidemiology , Comorbidity , Female , Heart Defects, Congenital/diagnostic imaging , Heart Failure/diagnosis , Humans , Incidence , Male , Middle Aged , Neuromuscular Diseases/diagnosis , Prognosis , Reproducibility of Results , Risk Factors , Sensitivity and Specificity , Sex Distribution , Survival Analysis , Tachycardia, Sinus/diagnosis , Ultrasonography , Young AdultABSTRACT
Left ventricular hypertrabeculation/noncompaction (LVHT) is diagnosed in all ages and is frequently associated with neuromuscular disorders (NMDs). The aim of the study was to compare patients with LVHT depending on age at diagnosis. Included were 232 patients with LVHT (72 women, mean age 52±17 years) diagnosed from 1995 to 2014 at 1 echocardiographic laboratory. In 2014, their survival was assessed. Seventy-six percent of the patients were neurologically investigated, revealing specific NMDs in 18%, unspecific NMDs in 60%, and normal findings in 22%. Forty-five patients (19%) received electronic devices: implantable cardioverter-defibrillators in 26 patients, combined with cardiac resynchronization systems (n=14) or an antibradycardic pacemaker (n=1); antibradycardic pacemakers (n=8); cardiac resynchronization systems (n=4); implantable loop recorders (n=4); life vests (n=2); and a left ventricular assist device as a bridge to transplantation (n=1). During 72-month follow-up, mortality was 4.9% per year. In younger age groups, more patients were referred for syncope or palpitations, whereas in older age groups, more patients were referred for heart failure. Classic cardiovascular risk factors such as hypertension and diabetes, as well as coronary artery stenosis, were rare in the young age groups but were more prevalent in older age groups. Differences between age groups were found regarding cardiac symptoms, NMDs, electrocardiographic findings, rate of device implantation, and mortality but not in location and extension of LVHT. None of the neurologically investigated patients≥70 years of age was neurologically normal. Prevalence of heart failure, electrocardiographic abnormalities, and mortality were highest in the oldest age group. In conclusion, LVHT must be considered as an echocardiographic diagnosis in all age groups. The morphologic pattern of LVHT is similar, whereas clinical manifestations and prognosis are variable among age groups.
Subject(s)
Age Factors , Heart Defects, Congenital/epidemiology , Neuromuscular Diseases/epidemiology , Adult , Age Distribution , Aged , Aged, 80 and over , Cardiac Resynchronization Therapy Devices , Cohort Studies , Defibrillators, Implantable , Female , Heart Defects, Congenital/complications , Heart Defects, Congenital/therapy , Humans , Male , Middle Aged , Neuromuscular Diseases/diagnosis , Prevalence , Survival Rate , Young AdultABSTRACT
PURPOSE: Chemotherapy regimens based on anthracycline (doxorubicin) are well established in lymphoma therapy. The purpose of this study was to examine the effects of L-carnitine with a view to reducing cytotoxic side-effects. METHODS: 20 patients were scheduled to receive 3 g L-carnitine before each chemotherapy cycle, followed by 1 g L-carnitine/day during the following 21 days, while 20 patients received a placebo (randomized controlled trial). The plasma lipid profile and relative mRNA levels of key enzymes of oxidative metabolism (carnitine acyltransferases) were measured at three points of time. In addition to the clinical parameters we used the mRNA of white blood cells to evaluate the toxic effects on cardiomyocytes. RESULTS: In the present study no cardiotoxicity of anthracycline therapy was detected. Carnitine treated patients showed a rise in plasma carnitine which led to an increase of relative mRNA levels from CPT1A (liver isoform of carnitine palmitoyltransferase) and OCTN2 (carnitine transporter). Following chemotherapy, an activation of carnitine acyltransferases was associated with a stimulation of OCTN2 in both groups. CONCLUSION: Biochemical and molecular analyses indicated a stimulation of oxidative metabolism in white blood cells through carnitine uptake.
