Temporal Evolution of the Heart Failure Phenotype in Barth ...

Temporal evolution of the heart failure phenotype in Barth syndrome ...

Cardiomyopathy presents in most patients with BTHS, typically appearing as dilated cardiomyopathy (DCM) in infancy and evolving to hypertrophic ...

Abnormalities in all of these proteins were observed in the failing LV myocardium of dogs as well as in LV myocardium from explanted failed human hearts [ ...

Temporal Evolution of the Heart Failure Phenotype in Barth ...

Cardiomyopathy presents in most patients with BTHS, typically appearing as dilated cardiomyopathy (DCM) in infancy and evolving to hypertrophic cardiomyopathy ( ...

"Temporal evolution of the heart failure phenotype in Barth ...

Barth syndrome (BTHS) is a rare genetic disorder caused by pathogenic variants in TAFAZZIN leading to reduced remodeled cardiolipin (CL), a phospholipid ...

Temporal evolution of the heart failure phenotype in Barth syndrome ...

Barth syndrome (BTHS) is a rare genetic disorder caused by pathogenic variants in TAFAZZIN leading to reduced remodeled cardiolipin (CL), a phospholipid ...

(PDF) Temporal Evolution of the Heart Failure Phenotype in Barth ...

progression of cardiomyopathy-related disease symptomatology in patients with Barth Syndrome: A TAZPOWER analysis. Presented at: American ...

Temporal evolution of the heart failure phenotype in Barth syndrome ...

Fingerprint. Dive into the research topics of 'Temporal evolution of the heart failure phenotype in Barth syndrome and treatment with elamipretide'.

Temporal evolution of the heart failure phenotype in Barth syndrome ...

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Barth Syndrome / Heart Failure Limits: Humans Language: En Journal: Future Cardiol ...

Barth syndrome - PMC - PubMed Central

Cardiomyopathy and Cardiac Arrhythmias ... Cardiac disease is common in BTHS. BTHS Registry data describe 69 of 73 subjects reporting a history of CMP [Roberts et ...

Cardiac and Clinical Phenotype in Barth Syndrome - AAP Publications

... Barth syndrome has not been characterized systematically, and the condition ... development of cardiomyopathy in this disease. There may be some ...

Barth syndrome: MedlinePlus Genetics

In people with Barth syndrome, the heart problems can lead to heart failure. ... Barth syndrome is associated with a cognitive phenotype. J ...

Elamipretide for Barth syndrome cardiomyopathy: gradual rebuilding of a failed ... Temporal Evolution of the Heart Failure Phenotype in Barth ...

Natural history of Barth syndrome: a national cohort study of 22 ...

The 20 patients (91%) with clinical evidence of cardiomyopathy were each hospitalised at least once for heart failure episodes, and there were a ...

Expanded-access use of elamipretide in a critically ill patient with ...

Barth syndrome (BTHS; OMIM #302060) is a rare disease characterized by cardiolipin abnormalities and cardiomyopathy, intermittent neutropenia and skeletal ...

Barth syndrome cardiomyopathy - Oxford Academic

Barth syndrome (BTHS) is an x-linked autosomal recessive disease characterized ... development and progression of various forms of heart failure.14,142 ...

Long-term efficacy and safety of elamipretide in patients with Barth ...

The dilated cardiomyopathy may follow an undulating pattern in surviving patients with eventual transition in some to a phenotype resembling heart failure with ...

Elamipretide for Barth syndrome cardiomyopathy: gradual rebuilding ...

Treatment of cardiomyopathy in patients with Barth syndrome generally follows that for heart failure patients, including angiotensin-converting ...

Cardiac and Clinical Phenotype in Barth Syndrome | Request PDF

Family history was positive for confirmed or suspected Barth syndrome in 63%. Ninety percent of patients had a clinical history of cardiomyopathy (mean age at ...

Clinical Characteristics and Outcomes of Cardiomyopathy in Barth ...

Temporal evolution of the heart failure phenotype in Barth syndrome and treatment with elamipretide. · Medicine. Future cardiology · 2023.

Expanded-access use of elamipretide in a critically ill patient with ...

improve over time with standard of care (SOC) heart failure ... Temporal evolution of the heart failure phenotype in Barth syndrome and treatment with ...