Myocardial glucose and fatty acid metabolism is altered and associated with lower cardiac function in young adults with Barth syndrome

AbstractBackground Barth syndrome (BTHS) is a rare X-linked condition resulting in cardiomyopathy, however; the effects of BTHS on myocardial substrate metabolism and its relationships with cardiac high-energy phosphate metabolism and left ventricular (LV) function are unknown. We sought to characterize myocardial glucose, fatty acid (FA), and leucine metabolism in BTHS and unaffected controls and examine their relationships with cardiac high-energy phosphate metabolism and LV function.Methods/ResultsYoung adults with BTHS (n = 14) and unaffected controls (n = 11, Control, totaln = 25) underwent bolus injections of15O-water and 1-11C-glucose, palmitate, and leucine and concurrent positron emission tomography imaging. LV function and cardiac high-energy phosphate metabolism were examined via echocardiography and31P magnetic resonance spectroscopy, respectively. Myocardial glucose extraction fraction (21  ± 14% vs 10 ± 8%,P  =  .03) and glucose utilization (828.0 ± 470.0 vs 393.2 ± 361.0 μmol·g−1·min−1,P = .02) were significantly higher in BTHS vs Control. Myocardial FA extraction fraction (31 ± 7% vs 41 ± 6%,P 
Source: Journal of Nuclear Cardiology - Category: Nuclear Medicine Source Type: research

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Condition:   Cardiomyopathy, Hypertrophic Intervention:   Other: PET Scan Sponsors:   Massachusetts General Hospital;   Brigham and Women's Hospital;   National Heart, Lung, and Blood Institute (NHLBI) Recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
We present not ‐yet‐seen multimodal images of a 55‐year‐old female patient with isolated atrial amyloidosis (IAA) who clinically suffered from multiple atrial arrhythmias and heart failure symptoms with preserved left ventricular ejection fraction. We aim to show structural and functional abnormalities det ected by electrophysiological voltage mapping, cardiac magnetic resonance imaging (MRI) [cMRI; atrial strain measurements, late gadolinium enhancement (LGE) visualization], and99mTc ‐DPD scintigraphy. Bipolar voltage mapping performed during two electrophysiological procedures showed diffuse left atrial low‐v...
Source: ESC Heart Failure - Category: Cardiology Authors: Tags: Case Report Source Type: research
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Source: Japanese Journal of Infectious Diseases - Category: Infectious Diseases Authors: Tags: Jpn J Infect Dis Source Type: research
AbstractMyocardial ischemia constitutes one of the most important pathophysiological features in hypertrophic cardiomyopathy. Chronic and recurrent myocardial ischemia leads to fibrosis, which may culminate in myocardial dysfunction. Since the direct visualization of coronary microcirculation in vivo is not possible, its function must be studied indirectly. Invasive and noninvasive techniques allow microcirculatory dysfunction to be evaluated, including echocardiography, magnetic resonance, positron emission tomography, and cardiac catheterization. Blunted myocardial blood flow and coronary flow reserve have been suggested...
Source: Microcirculation - Category: Research Authors: Tags: REVIEW ARTICLE Source Type: research
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Source: General Thoracic and Cardiovascular Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research
AbstractExclusion of cardiac abnormalities should be performed at the beginning of the athlete ’s career. Myocarditis, right ventricular remodeling and coronary anomalies are well-known causes of life-threatening events of athletes, major cardiovascular events and sudden cardiac death. The feasibility of an extended comprehensive echocardiographic protocol for the detection of structural ca rdiac abnormalities in athletes should be tested. This standardized protocol of transthoracic echocardiography includes two- and three-dimensional imaging, tissue Doppler imaging, and coronary artery scanning. Post processing was ...
Source: The International Journal of Cardiovascular Imaging - Category: Radiology Source Type: research
AbstractMyocardial ischemia constitutes one of the most important pathophysiological features in hypertrophic cardiomyopathy. Chronic and recurrent myocardial ischemia leads to fibrosis, which may culminate in myocardial dysfunction. Since the direct visualization of coronary microcirculationin vivo is not possible, its function must be studied indirectly. Invasive and non ‐invasive techniques allow microcirculatory dysfunction to be evaluated, including echocardiography, magnetic resonance, positron emission tomography and cardiac catheterization. Blunted myocardial blood flow and coronary flow reserve have been suggest...
Source: Microcirculation - Category: Research Authors: Tags: REVIEW Source Type: research
AbstractPurpose of reviewHypertrophic cardiomyopathy, an inherited autosomal dominant disease, is the most common familial cardiovascular disorder and presents with heterogeneous pathobiological, clinical, and phenotypic features. This review will explore how multimodality cardiovascular imaging offers insight into understanding and identifying these heterogeneous features which are important to reliably diagnose and risk stratify patients with HCM.Recent findingsBeyond the left ventricular (LV) hypertrophy, multimodality imaging is able to fully characterize a number of structural and functional abnormalities seen in HCM ...
Source: Current Treatment Options in Cardiovascular Medicine - Category: Cardiology Source Type: research
This report described a 53-year-old male who was diagnosed as PCNSL 17 months after heart transplantation. Diagnoses: The patient was admitted to the local hospital presenting with dizziness, headache, and reduced left-sided power and sensation for 1 week. He had a medical history of heart transplantation because of the dilated cardiomyopathy 17 months ago and had a 17-month history of immunosuppressive therapy with tacrolimus. A computed tomography scan of the brain revealed a bulky mass in the right temporal lobe. The emergency intracranial mass resection and cerebral decompression were performed in our hospital. The ...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
Shaloo Garg, Archana Singh, Mukesh Kalita, Ayesha Zafar Siddiqui, Mukul Chandra KapoorJournal of Anaesthesiology Clinical Pharmacology 2020 36(5):44-47 A pregnant patient presented with fever and desaturation, without breathlessness. She was suspected to have COVID-19 but SARS-CoV-2 was negative. She developed fetal distress and underwent an uneventful Cesarean section. Postoperatively, she developed respiratory distress and needed mechanical ventilation support. The clinical features suggested COVID-19 infection and antiviral treatment were empirically initiated. Repeat SARS-CoV-2 was negative. Echocardiography, computed...
Source: Journal of Anaesthesiology Clinical Pharmacology - Category: Anesthesiology Authors: Source Type: research
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