Management of Heart Failure in Adult Congenital Heart Disease

Sabanayagam, Aarthi; Cavus, Omer; Williams, Jeremy C.; Bradley, Elisa A.

doi:10.1016/j.hfc.2018.06.005

Cited by 18 publications

(7 citation statements)

References 68 publications

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“…In our study, the difference between repaired Fontan patients with normal NT-proBNP levels and healthy controls yielded significance for the peak torsion. The reason for this is that low preload with chronic myocardial remodeling after the Fontan operation leads to altered ventricular geometry, decreased compliance, increased end-diastolic pressure, and possible high NT-proBNP plasma levels (1). In our study, LV function, cardiac mass, and AV valve regurgitation were not statistically different between the normal NT-proBNP level and high NT-proBNP level subjects.…”

Section: Discussioncontrasting

confidence: 49%

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Systemic ventricular strain and torsion are predictive of elevated serum NT-proBNP in Fontan patients: a magnetic resonance study

Hu¹,

Liu²,

Wang³

et al. 2020

Quant Imaging Med Surg

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Background: This study aimed to investigate the associations between cardiac strain, cardiac torsion, ventricular volumes, and ventricular ejection fraction, with N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels in Fontan patients who were age-and gender-matched with healthy control subjects.Methods: Cardiovascular magnetic resonance (CMR) studies performed in 22 (15 male, 7 female) patients with single-ventricle physiology (all morphological left ventricles) palliated with Fontan and 17 (10 male, 7 female) age-and gender-matched healthy children volunteers were retrospectively analyzed. Serum NT-proBNP levels were obtained in Fontan subjects. Standard post-processing of CMR images included systemic ventricular end-diastolic and end-systolic volumes, stroke volume, cardiac mass, atrioventricular regurgitation, and ejection fraction. CMR tissue tracking (TT) software was used to quantify global longitudinal strain (GLS), global radial strain (GRS), and global circumferential strain (GCS) and torsion of the systemic ventricle. Pearson and Spearman correlation coefficients were used in comparisons of correlations between NT-proBNP and functional parameters in repair Fontan patients. Intra-observer and inter-observer variability of CMR strain and torsion values were determined from 10 randomly selected Fontan subjects and 10 randomly selected control subjects.Results: GLS was significantly lower in Fontan patients than in control subjects (−15.19±2.94 vs. −19.97±1.70; P<0.001). GLS was not significantly different between normal NT-proBNP levels and high NT-proBNP levels in Fontan patients (−15.59±2.72 vs. −14.62±3.32; P=0.462). The GCS of repair Fontan patients was not significantly lower than that of the control group (−16.76±3.27 vs. −17.88±2.26; P=0.235). GCS was significantly different between normal and high NT-proBNP levels group in Fontan patients(−17.95±2.43 vs. −15.04±3.67; P=0.036). The peak systolic torsion and peak systolic torsion rates were significantly lower in Fontan patients than in control subjects (0.81±0.41 vs. 1.07±0.36, P=0.044; 7.36±3.41 vs. 9.85±2.61, P=0.017). Peak systolic torsion was significantly lower in Fontan patients with normal NT-proBNP levels than in high NT-proBNP subjects (0.67±0.43 vs. 1.01±0.29; P=0.036). GCS and torsion were more strongly correlated with NT-proBNP in the patient group (r=0.541 for GCS; r=0.588 for torsion, P<0.01). The parameters of strain and torsion could be reproduced with sufficient accuracy by intra-observer agreement(biases =0.04 for GLS; biases =0.66 for GCS; biases =1.03 for GRS; biases =0.04 for torsion) and inter−observer agreement (biases =0.32 for GLS; biases =0.85 for GCS; biases =1.52 for GRS; biases =0.18 for torsion).Conclusions: GLS is an earlier marker of contractile dysfunction in repair Fontan patients. Peak systolic torsion may be a biomarker for determining subclinical dysfunction, as it is more strongly correlated with serum biomarkers of ventricular function than ventricular size or ejection fraction.

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Section: Discussioncontrasting

confidence: 49%

“…Heart failure is a common complication in single-ventricle survivors with Fontan palliation that is a risk factor for mortality (1). N-terminal pro-B-type natriuretic peptide (NT-proBNP) is an important serum biomarker for pediatric patients with heart disease.…”

Section: Introductionmentioning

confidence: 99%

Systemic ventricular strain and torsion are predictive of elevated serum NT-proBNP in Fontan patients: a magnetic resonance study

Hu¹,

Liu²,

Wang³

et al. 2020

Quant Imaging Med Surg

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“…As the most prevalent type of human birth defect, congenital heart defect (CHD) occurs in about 1% of all live neonates, accounting for nearly a third of all forms of developmental abnormalities (Benjamin et al, 2019;Oliveira-Brancati et al, 2020). Although minor CHD may resolve spontaneously (Benjamin et al, 2019), serious CHD may lead to poor health-related quality of life (Amedro et al, 2018(Amedro et al, , 2019Boukovala et al, 2019), reduced exercise capacity (Müller et al, 2018;Abassi et al, 2019;Smith et al, 2019), abnormal nervous develop ment or brain injury (Peyvandi et al, 2018(Peyvandi et al, , 2019Khanna et al, 2019), hemorrhagic or ischemic stroke (Bokma et al, 2018;Giang et al, 2018;Pedersen et al, 2019), pulmonary hypertension (Brida and Gatzoulis, 2018;Dimopoulos et al, 2018;Kaemmerer et al, 2018;Pascall and Tulloh, 2018), acute kidney injury or renal dysfunction (Lui et al, 2017;Gist et al, 2018), infective endocarditis (Jortveit et al, 2018;Tutarel et al, 2018;Cahill et al, 2019), cardiac dysfunction or congestive heart failure (Gilbert et al, 2018;Lal et al, 2018;Sabanayagam et al, 2018;Chan et al, 2019), ventricular or supraventricular dysrhythmia (Labombarda et al, 2017;Barry et al, 2018;Hernández-Madrid et al, 2018;Fuchs et al, 2019), and death (Lynge et al, 2018;Yu C et al, 2018). Although vast advance in cardiac surgery allows over 90% of CHD newborns to survive into adulthood, it results i...…”

Section: Introductionmentioning

confidence: 99%

A novel TBX5 mutation predisposes to familial cardiac septal defects and atrial fibrillation as well as bicuspid aortic valve

Jiang

Zhao

et al. 2020

Genet. Mol. Biol.

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“…Interestingly, adult patients with heart failure due to CHD treated with ARBs showed a decrease in systolic blood pressure and tricuspid regurgitation, as well as an increase in exercise duration in those with great vessel transposition [ 57 ]. Additionally, ARBs have been shown to improve left ventricular ejection fraction in adults with heart failure [ 14 ].…”

Section: Drugs For Chd Treatmentmentioning

confidence: 99%

Congenital Heart Disease: The State-of-the-Art on Its Pharmacological Therapeutics

Varela-Chinchilla

Sánchez-Mejía

Trinidad-Calderón

2022

JCDD

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Congenital heart disease is one of the most common causes of death derived from malformations. Historically, its treatment has depended on timely diagnosis and early pharmacological and surgical interventions. Survival rates for patients with this disease have increased, primarily due to advancements in therapeutic choices, but mortality remains high. Since this disease is a time-sensitive pathology, pharmacological interventions are needed to improve clinical outcomes. Therefore, we analyzed the applications, dosage, and side effects of drugs currently used for treating congenital heart disease. Angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, beta-blockers, and potassium-sparing diuretics have shown a mortality benefit in most patients. Other therapies, such as endothelin receptor antagonists, phosphodiesterase-5 inhibitors, prostaglandins, and soluble guanylyl cyclase stimulators, have benefited patients with pulmonary artery hypertension. Likewise, the adjunctive symptomatic treatment of these patients has further improved the outcomes, since antiarrhythmics, digoxin, and non-steroidal anti-inflammatory drugs have shown their benefits in these cases. Conclusively, these drugs also carry the risk of troublesome adverse effects, such as electrolyte imbalances and hemodynamic compromise. However, their benefits for survival, symptom improvement, and stabilization outweigh the possible complications from their use. Thus, cases must be assessed individually to accurately identify interventions that would be most beneficial for patients.

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Management of Heart Failure in Adult Congenital Heart Disease

Cited by 18 publications

References 68 publications

Systemic ventricular strain and torsion are predictive of elevated serum NT-proBNP in Fontan patients: a magnetic resonance study

Systemic ventricular strain and torsion are predictive of elevated serum NT-proBNP in Fontan patients: a magnetic resonance study

A novel TBX5 mutation predisposes to familial cardiac septal defects and atrial fibrillation as well as bicuspid aortic valve

Congenital Heart Disease: The State-of-the-Art on Its Pharmacological Therapeutics

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