General principles of heart failure management in adult congenital heart disease

Tompkins, Rose; Romfh, Anitra

doi:10.1007/s10741-019-09895-x

Cited by 9 publications

(19 citation statements)

References 91 publications

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“…Refinement of surgical correction methods for TOF improved the survival in the short term but the elevated cardiovascular long-term risk of TOF patients did not change over the past decades 7 – 10 . Besides surgery, specific treatment options for TOF patients are not available, mostly because the pathomechanisms underlying the cardiac remodeling process triggered by TOF are unknown 7 – 10 .…”

Section: Mainmentioning

confidence: 98%

“…Despite greatly improved surgical procedures in infancy and early childhood, long-term complications of corrected TOF remain a major problem 7 – 10 . Besides coronary artery disease, long-term cardiac complications of TOF include cardiac hypertrophy and cardiac remodeling, which predispose to an increased risk of cardiac death due to arrhythmias and heart failure 7 – 10 . Refinement of surgical correction methods for TOF improved the survival in the short term but the elevated cardiovascular long-term risk of TOF patients did not change over the past decades 7 – 10 .…”

Section: Mainmentioning

confidence: 99%

“…Besides coronary artery disease, long-term cardiac complications of TOF include cardiac hypertrophy and cardiac remodeling, which predispose to an increased risk of cardiac death due to arrhythmias and heart failure 7 – 10 . Refinement of surgical correction methods for TOF improved the survival in the short term but the elevated cardiovascular long-term risk of TOF patients did not change over the past decades 7 – 10 . Besides surgery, specific treatment options for TOF patients are not available, mostly because the pathomechanisms underlying the cardiac remodeling process triggered by TOF are unknown 7 – 10 .…”

Section: Mainmentioning

confidence: 99%

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BBLN triggers CAMK2D pathology in mice under cardiac pressure overload and potentially in unrepaired hearts with tetralogy of Fallot

Abd Alla,

Langer,

Wolf

et al. 2023

Nat Cardiovasc Res

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Tetralogy of Fallot (TOF) is one of the most prevalent congenital heart defects, with adverse cardiac remodeling and long-term cardiac complications. Here, searching for pathomechanisms, we find upregulated bublin coiled-coil protein (BBLN) in heart specimens of TOF patients with cyanosis, which positively correlates with cardiac remodeling pathways. Human BBLN, a protein with largely unknown function, promoted heart failure features, with increased mortality when overexpressed in mice, in a protein dosage-dependent manner. BBLN enhanced cardiac inflammation, fibrosis and necroptosis by calcium/calmodulin-dependent protein kinase II delta (CAMK2D) activation, whereas a BBLN mutant with impaired CAMK2D binding was inert. Downregulation of CAMK2D by an interfering RNA retarded BBLN-induced symptoms of heart failure. Endogenous BBLN was induced by hypoxia as a major TOF feature in human patients and by chronic pressure overload in mice, and its downregulation decreased CAMK2D hyperactivity, necroptosis and cardiovascular dysfunction. Thus, BBLN promotes CAMK2D-induced pathways to pathological cardiac remodeling, which are triggered by hypoxia in TOF.

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Section: Mainmentioning

confidence: 98%

Section: Mainmentioning

confidence: 99%

Section: Mainmentioning

confidence: 99%

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BBLN triggers CAMK2D pathology in mice under cardiac pressure overload and potentially in unrepaired hearts with tetralogy of Fallot

Abd Alla,

Langer,

Wolf

et al. 2023

Nat Cardiovasc Res

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“…Therefore, there is a limit to applying HF management due to traditional left ventricular systolic dysfunction. 26,27) Active efforts are being made to prepare for the increase in the number of GUCH patients and the medical burden due to their HF.…”

Section: Increased Hf and Arrhythmiasmentioning

confidence: 99%

Environmental changes surrounding congenital heart disease

Choi¹

2023

Clin Exp Pediatr

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As the outcomes of patients with congenital heart disease (CHD) improve, the number of patients is accumulating and the proportion of adult CHD patients is gradually increasing.Accordingly, the proportion of typical adult diseases such as coronary arterial disease, diabetes, dyslipidemia, and hypertension among patients with CHD is increasing, as is the medical burden due to residual cardiac problems, heart failure, and arrhythmias. Due to increasing genetic knowledge, efforts are being made to provide active medical support through risk stratification. Surgical techniques and prostheses are also developing, the instruments used for transcatheter intervention are diversifying, and the range of therapeutic options is expanding. In addition, owing to the development of electronic devices, heart rhythm and blood pressure monitoring instruments have been miniaturized and diversified. In this process, a vast amount of information is collected; therefore, the current systematic review examined their clinical usefulness. A team approach consisting of experts from various fields is essential for the treatment of patients with CHD and advanced heart failure.

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“…Drs. Vaikunth and Lui begin the edition by covering general principles of heart failure in ACHD [14], and this is built upon by a review of the general approach to heart failure management to patients with ACHD [15]. Following on, Mueller et al cover heart failure in the largest such group of patients with ACHD, that being tetralogy of Fallot [16].…”

mentioning

confidence: 99%

The success and failure of the adult congenital heart

Collins

2020

Heart Fail Rev

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General principles of heart failure management in adult congenital heart disease

Cited by 9 publications

References 91 publications

BBLN triggers CAMK2D pathology in mice under cardiac pressure overload and potentially in unrepaired hearts with tetralogy of Fallot

BBLN triggers CAMK2D pathology in mice under cardiac pressure overload and potentially in unrepaired hearts with tetralogy of Fallot

Environmental changes surrounding congenital heart disease

The success and failure of the adult congenital heart

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