scholarly journals Heart failure in single right ventricle congenital heart disease: physiological and molecular considerations

2020 ◽  
Vol 318 (4) ◽  
pp. H947-H965 ◽  
Author(s):  
Anastacia M. Garcia ◽  
Jonathan-Thomas Beatty ◽  
Stephanie J. Nakano
Keyword(s):  
Risk Factors ◽  
Heart Failure ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Right Ventricle ◽  
Congenital Heart ◽  
Molecular Mechanisms ◽  
Therapeutic Interventions ◽  
Anatomy And Physiology ◽  
Molecular Alterations

Because of remarkable surgical and medical advances over the past several decades, there are growing numbers of infants and children living with single ventricle congenital heart disease (SV), where there is only one functional cardiac pumping chamber. Nevertheless, cardiac dysfunction (and ultimately heart failure) is a common complication in the SV population, and pharmacological heart failure therapies have largely been ineffective in mitigating the need for heart transplantation. Given that there are several inherent risk factors for ventricular dysfunction in the setting of SV in addition to probable differences in molecular adaptations to heart failure between children and adults, it is perhaps not surprising that extrapolated adult heart failure medications have had limited benefit in children with SV heart failure. Further investigations into the molecular mechanisms involved in pediatric SV heart failure may assist with risk stratification as well as development of targeted, efficacious therapies specific to this patient population. In this review, we present a brief overview of SV anatomy and physiology, with a focus on patients with a single morphological right ventricle requiring staged surgical palliation. Additionally, we discuss outcomes in the current era, risk factors associated with the progression to heart failure, present state of knowledge regarding molecular alterations in end-stage SV heart failure, and current therapeutic interventions. Potential avenues for improving SV outcomes, including identification of biomarkers of heart failure progression, implications of personalized medicine and stem cell-derived therapies, and applications of novel models of SV disease, are proposed as future directions.

scholarly journals Alteration of cardiolipin biosynthesis and remodeling in single right ventricle congenital heart disease

2020 ◽  
Vol 318 (4) ◽  
pp. H787-H800
Author(s):  
Anastacia M. Garcia ◽  
Jessica C. McPhaul ◽  
Genevieve C. Sparagna ◽  
Danielle A. Jeffrey ◽  
Raleigh Jonscher ◽  
...  
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Right Ventricle ◽  
Copy Number ◽  
Congenital Heart ◽  
Molecular Mechanisms ◽  
Single Ventricle ◽  
Ionization Mass ◽  
Mitochondrial Copy Number

Despite advances in both medical and surgical therapies, individuals with single ventricle heart disease (SV) remain at high risk for the development of heart failure (HF). However, the molecular mechanisms underlying remodeling and eventual HF in patients with SV are poorly characterized. Cardiolipin (CL), an inner mitochondrial membrane phospholipid, is critical for proper mitochondrial function, and abnormalities in CL content and composition are known in various cardiovascular disease etiologies. The purpose of this study was to investigate myocardial CL content and composition in failing and nonfailing single right ventricle (RV) samples compared with normal control RV samples, to assess mRNA expression of CL biosynthetic and remodeling enzymes, and to quantitate relative mitochondrial copy number. A cross-sectional analysis of RV myocardial tissue from 22 failing SV (SVHF), 9 nonfailing SV (SVNF), and 10 biventricular control samples (BVNF) was performed. Expression of enzymes involved in CL biosynthesis and remodeling were analyzed using RT-qPCR and relative mitochondrial DNA copy number determined by qPCR. Normal phase high-pressure liquid chromatography coupled to electrospray ionization mass spectrometry was used to quantitate total and specific CL species. While mitochondrial copy number was not significantly different between groups, total CL content was significantly lower in SVHF myocardium compared with BVNF controls. Despite having lower total CL content however, the relative percentage of the major tetralinoleoyl CL species is preserved in SVHF samples relative to BVNF controls. Correspondingly, expression of enzymes involved in CL biosynthesis and remodeling were upregulated in SVHF samples when compared with both SVNF samples and BVNF controls. NEW & NOTEWORTHY The mechanisms underlying heart failure in the single ventricle (SV) congenital heart disease population are largely unknown. In this study we identify alterations in cardiac cardiolipin metabolism, composition, and content in children with SV heart disease. These findings suggest that cardiolipin could be a novel therapeutic target in this unique population of patients.

Abstract 14610: Renal Dysfunction in Adults With Congenital Heart Disease in Vietnam

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Ngoc Thanh Kim ◽  
Thanh Tung Le ◽  
Doan Loi Do ◽  
Thanh Huong Truong
Keyword(s):  
Risk Factors ◽  
Heart Failure ◽  
Atrial Fibrillation ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Renal Dysfunction ◽  
Congenital Heart ◽  
Pulmonary Arterial

Introduction: In Vietnam, knowledge about renal function in adults with congenital heart disease (CHD) is limited. Hypothesis: This study aims to estimate incidence of renal dysfunction in adults with congenital heart disease and risk factors. Methods: This is a cross-sectional study, including 365 CHD patients more than 16 years old. We collected clinical and para-clinical information, estimated glomerular filtration rate (GFR) and calculated the odds ratio (OR) for reduced GFR. Results: Totally, 52.8% patients had GFR < 90 ml/phút/1.73 m 2 . Logistic regression had confirmed the OR for GFR < 90 ml/phút/1.73 m 2 in the group > 60-years-old, the group with atrial fibrillation, the group with heart failure (based on NT-proBNP > 125 pmol/L), and the group with pulmonary arterial hypertension (based on pulmonary artery systolic pressure > 50 mmHg by echocardiography) were 6.46 (95% CI: 1.37 - 30.41), 7.58 (95% CI: 1.66 - 34.56), 2.98 (95% CI: 1.49 - 5.98) and 1.84 (95% CI: 1.02 - 3.33), respectively. Conclusions: Renal dysfunction is common in adults with CHD. Age > 60 years-old, atrial fibrillation, heart failure, and pulmonary arterial hypertension were risk factors for renal dysfunction in adults with CHD.

Heart failure admissions in adults with congenital heart disease; risk factors and prognosis

2013 ◽  
Vol 168 (3) ◽  
pp. 2487-2493 ◽  
Author(s):  
A.C. Zomer ◽  
I. Vaartjes ◽  
E.T. van der Velde ◽  
H.M.Y. de Jong ◽  
T.C. Konings ◽  
...  
Keyword(s):  
Risk Factors ◽  
Heart Failure ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Disease Risk ◽  
Heart Disease Risk Factors ◽  
Heart Disease Risk

Prevalence and risk factors of sleep apnea in adult patients with congenital heart disease

2019 ◽  
Vol 29 (5) ◽  
pp. 576-582 ◽  
Author(s):  
Gen Harada ◽  
Daiji Takeuchi ◽  
Kei Inai ◽  
Tokuko Shinohara ◽  
Toshio Nakanishi
Keyword(s):  
Risk Factors ◽  
Heart Failure ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Sleep Apnea ◽  
Congenital Heart ◽  
Adult Patients ◽  
Respiratory Disturbance Index ◽  
Disturbance Index ◽  
Respiratory Disturbance

AbstractBackground:Although sleep apnea is an important disorder associated with cardiac events, data regarding its prevalence and risk factors in adult patients with congenital heart disease are limited.Methods:In this study, patients underwent a sleep study in the hospital. Indications for admission were classified as heart failure, diagnostic catheterisation, interventional catheterisation, or arrhythmia. The prevalence, characteristics, and risk factors of sleep apnea using a type-3 portable overnight polygraph in adult patients with congenital heart disease were evaluated.Results:There were 104 patients [median age: 36 (interquartile range: 28–48) years] who were admitted for heart failure 34% (n = 36), diagnostic catheterisation 26% (n = 27), interventional catheterisation 18% (n = 19), or arrhythmia 22% (n = 23). The prevalence of sleep apnea, defined as a respiratory disturbance index ≥5, was 63% (n = 63), with a distribution of 37%, 16%, and 10% for mild (5≤ respiratory disturbance index <15), moderate (15≤ respiratory disturbance index <30), and severe (respiratory disturbance index ≥30) sleep apnea, respectively. A large majority of the sleep apnea cases were categorised as obstructive sleep apnea (92%, n = 58). The respiratory disturbance index ≥15 group had a significantly higher proportion of male patients and higher body mass index, noradrenaline level, and aortic blood pressure than the group without sleep apnea (respiratory disturbance index <5). Multivariable analysis showed that New York Heart Association class ≥II (OR, 4.36; 95% CI, 1.09–20.87) and body mass index ≥25 (OR, 4.29; 95% CI, 1.32–15.23) were independent risk factors for a respiratory disturbance index ≥15.Conclusion:Our results showed a high prevalence of sleep apnea in adult patients with congenital heart disease. Its unique haemodynamics may be associated with a high prevalence of sleep apnea. Congestive heart failure and being overweight are important risk factors for sleep apnea. Management of heart failure and general lifestyle improvements will be important for controlling sleep apnea symptoms in these patients.

scholarly journals Heart failure related to adult congenital heart disease: prevalence, outcome and risk factors

2021 ◽  
Author(s):  
Stijn Arnaert ◽  
Pieter De Meester ◽  
Els Troost ◽  
Walter Droogne ◽  
Lucas Van Aelst ◽  
...  
Keyword(s):  
Risk Factors ◽  
Heart Failure ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Adult Congenital Heart Disease ◽  
Disease Prevalence ◽  
Adult Congenital

Pregnancy and lactation

2019 ◽  
pp. 343-366
Author(s):  
Anita Banerjee ◽  
Debasish Banerjee ◽  
Vivekanand Jha
Keyword(s):  
Risk Factors ◽  
Heart Failure ◽  
Cardiovascular Disease ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Pregnancy And Lactation ◽  
Developed World ◽  
Rheumatic Heart ◽  
In Pregnancy

Cardiovascular disease in pregnancy remains a significant cause of morbidity and mortality worldwide. In the developed world, congenital heart disease remains the most frequently encountered presentation, and in the developing world, it is rheumatic heart disease. However, with ageing of the maternal population and cardiovascular risk factors, women are presenting with familiar cardiac conditions such as heart failure. Pre-pregnancy counselling and appropriate and timely management lead to an optimal outcome for the mother and baby. This chapter describes the physiology and provides up-to-date guidance on drug therapy of cardiovascular disease in pregnancy.

Heart failure risk predictions in adult patients with congenital heart disease: a systematic review

Heart ◽  
2019 ◽  
Vol 105 (21) ◽  
pp. 1661-1669 ◽  
Author(s):  
Fei Wang ◽  
Lee Harel-Sterling ◽  
Sarah Cohen ◽  
Aihua Liu ◽  
James M Brophy ◽  
...  
Keyword(s):  
Risk Factors ◽  
Heart Failure ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Risk Prediction ◽  
Congenital Heart ◽  
Patient Characteristics ◽  
Adverse Outcomes ◽  
Adult Patients ◽  
Population Characteristics

To summarise existing heart failure (HF) risk prediction models and describe the risk factors for HF-related adverse outcomes in adult patients with congenital heart disease (CHD). We performed a systematic search of MEDLINE, EMBASE and Cochrane databases from January 1996 to December 2018. Studies were eligible if they developed multivariable models for risk prediction of decompensated HF in adult patients with CHD (ACHD), death in patients with ACHD-HF or both, or if they reported corresponding predictors. A standardised form was used to extract information from selected studies. Twenty-five studies met the inclusion criteria and all studies were at moderate to high risk of bias. One study derived a model to predict the risk of a composite outcome (HF, death or arrhythmia) with a c-statistic of 0.85. Two studies applied an existing general HF model to patients with ACHD but did not report model performance. Twenty studies presented predictors of decompensated HF, and four examined patient characteristics associated with mortality (two reported predictors of both). A wide variation in population characteristics, outcome of interest and candidate risk factors was observed between studies. Although there were substantial inconsistencies regarding which patient characteristics were predictive of HF-related adverse outcomes, brain natriuretic peptide, New York Heart Association class and CHD lesion characteristics were shown to be important predictors. To date, evidence in the published literature is insufficient to accurately profile patients with ACHD. High-quality studies are required to develop a unique ACHD-HF prediction model and confirm the predictive roles of potential risk factors.

scholarly journals Clinical use of percutaneous mechanical circulatory assistance in a patient with end-stage right-sided heart failure and massive tricuspid insufficiency due to congenital heart disease: first-in-the-world case report

2021 ◽  
Vol 5 (8) ◽  
Author(s):  
Mario García Gómez ◽  
Aitor Uribarri ◽  
José Alberto San Román Calvar ◽  
Alexander Stepanenko
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Right Ventricle ◽  
Heart Transplantation ◽  
Right Ventricular ◽  
Congenital Heart ◽  
Ebstein’S Anomaly ◽  
Exclusion Criteria ◽  
End Stage

Abstract Background Due to improvement in the management of patients with congenital heart disease (CHD), the likelihood of their survival to adulthood is increasing. A relevant population suffers end-stage right ventricular failure (RVF) in their 3rd–4th decade of life. Hence, heart transplantation is still gold standard of treatment of end-stage heart failure, mechanical circulatory assistance has become a valuable tool in the bridging to heart transplant or definitive therapy. Use of implantable short-term or long-term devices is reported by others. However, within this clinical context, presence of significant tricuspid regurgitation (TR) or CHD is used as exclusion criteria for insertion of a percutaneous right ventricular circulatory support. Case summary We described a 36-year-old patient diagnosed with Ebstein's anomaly and severe TR who is admitted to hospital due to RVF refractory to standard medical treatment. After case presentation to the heart team, an Impella RP device insertion was scheduled, in spite of the presence of TR or CHD after evaluation of pulmonary valve competency and 3D reconstruction with virtual device implantation. During support, the patient improved clinically and haemodynamically. Due to device displacement to the right ventricle, it was bedside explanted after 30 days of support. After mechanical unloading during 30 days patients’ right ventricle recovered partially, permitting patient to improve his functional class. Discussion Although TR and CHD are exclusion criteria for the implantation of the Impella RP device, we report clinical experience in patient with Ebstein's anomaly and severe TR supported with percutaneous device as bridge to heart transplantation during 30 days.

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