Early Experience With Sacubitril/Valsartan in Adult Patients With Congenital Heart Disease

Lluri, Gentian; Lin, Jeannette; Reardon, L.; Miner, Pamela D.; Whalen, Katrina; Aboulhosn, Jamil

doi:10.1177/2150135119825599

Cited by 37 publications

(9 citation statements)

References 12 publications

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“…7 Initial experience of sacubitril/valsartan in ACHD patients included small cohorts and reported conflicting results. [8][9][10] The only prospective study in this specific subset included 20 patients followed for 6 months. 11 Therefore, robust data supporting the use of sacubitril/valsartan in sRV are still lacking.…”

mentioning

confidence: 99%

Safety and Efficacy of Sacubitril/Valsartan in Patients With a Failing Systemic Right Ventricle: A Prospective Single-Center Study

Fusco

Scognamiglio

Merola

et al. 2023

Circ: Heart Failure

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BACKGROUND: Sacubitril/valsartan was demonstrated to reduce hospitalization rate and mortality in patients with heart failure with reduced ejection fraction. Data on the effects of sacubitril/valsartan in patients with a systemic right ventricle are still lacking. METHODS: Patients with transposition of the great arteries following Senning/Mustard procedure or congenitally corrected transposition of the great arteries with impaired systemic right ventricle systolic function were prospectively included. Primary end points included sacubitril/valsartan safety and efficacy. Primary efficacy end points were NT-proBNP (N-terminal pro-B-type natriuretic peptide) and systolic function improvement. Secondary end points included New York Heart Association class, 6-minute walking distance, and quality of life change. RESULTS: Fifty patients (38±12 years, 60% male, 35% congenitally corrected transposition of the great arteries) were included and followed for 1 year. No major adverse events occurred. Two (4%) patients ceased treatment due to hypotension and 1 (2%) developed a nephrotic syndrome. The target dose was reached in 20 (42%) patients. NT-proBNP values decreased significantly immediately after treatment initiation, while returned to baseline at 1 year. Echocardiography showed progressive fractional area change increase (29.2±5.8 versus 34.9±5.1%; P <0.001), and right ventricle global longitudinal strain (−13.9 [−15.1, −11.8] versus −15.3 [−17.2, −13.4]%; P <0.001) and free-wall global longitudinal strain (−14.3 [−17.3, −12.3] versus −17.2 [−19.3, −15.8]%; P <0.001) raise, whereas tricuspid regurgitation severity improved only in transposition of the great arteries patients ( P =0.006). Moreover, 3-dimensional echocardiography demonstrated right ventricle volumes reduction (end-diastolic volume: 181±63 versus 156±50 mL; P =0.002; end-systolic volume: 117±48 versus 89±33 mL; P <0.001), and significantly increased systemic right ventricle ejection fraction (35.6±8.1 versus 41.5±7.5%; P <0.001). Clinical improvement was suggested by New York Heart Association class change ( P <0.001), increased 6-minute walking distance (425 [333, 480] versus 500 [443, 560] m; P <0.001) as well as improved quality of life at 1-year follow-up. Beneficial effects were observed irrespective of the underlying anatomy and were more pronounced in those on target dose. CONCLUSIONS: Our data showed that sacubitril/valsartan is well tolerated and is associated with systemic right ventricle remodeling and improved systolic function as well as improved clinical status, supporting its use in this complex population.

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confidence: 99%

Safety and Efficacy of Sacubitril/Valsartan in Patients With a Failing Systemic Right Ventricle: A Prospective Single-Center Study

Fusco

Scognamiglio

Merola

et al. 2023

Circ: Heart Failure

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“…No clinical deterioration was reported. In four patients in NYHA class III with complex ACHD, pulmonary hypertension, and cyanosis, a significant improvement to NYHA class II was reported [70]. In a study of 23 patients with moderate/severe complexity ACHD, no improvement in functional class or systemic ventricular function, nor decrease in proBNP was described.…”

Section: Novel Therapies: Angiotensin Receptor Neprilysin Inhibitorsmentioning

confidence: 94%

Heart failure in congenital heart disease: management options and clinical challenges

Leusveld

Kauling

Geenen

et al. 2020

Expert Review of Cardiovascular Therapy

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Introduction: The population of adults with congenital heart disease (ACHD) is rapidly expanding and one of the major complications is heart failure. Timely diagnosis and treatment are crucial, but strong evidence for effectiveness of heart failure treatment in ACHD is currently lacking. Components of the medical history, physical examination, and further diagnostic tests including ECG, echocardiography, cardiac magnetic resonance imaging, exercise testing, and biomarkers can identify patients at risk for early mortality or heart failure. Areas covered: Although the number of studies guiding evidence-based treatment are expanding, many clinical questions have not been completely answered yet. Therefore, in this review we provide an overview of current available insights in epidemiology, diagnosis, risk stratification, and treatment options in ACHD patients, including non-medical therapies and advanced care planning. Expert opinion: We strongly advocate expanding current use of biomarkers in the diagnostic process and timely initiation of discussing advanced treatment options and advanced care planning with patients and their loved ones. More research in multi-center collaborations is needed to study all aspects of care of adult congenital heart disease patients.

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“…In the case of sacubitril-valsartan, there exists some evidence to suggest its safety and tolerability amongst ACHD patients, with a signal of potential efficacy with respect to functional class particularly in patients with complex congenital heart disease, pulmonary hypertension, and/or cyanosis [13,14]. In one additional retrospective study, however, treatment with this agent was not associated with any improvement in systemic ventricular function, NT-proBNP levels, or functional class but did lead to increases in creatinine [15].…”

Section: Medical Managementmentioning

confidence: 99%

Chronic heart failure management in adult patients with congenital heart disease

Dhingra¹,

Mazer

Connelly

et al. 2023

Current Opinion in Cardiology

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Purpose of reviewA growing number of adult patients with congenital heart disease (ACHD) are entering the healthcare system as a result of advances in the diagnosis and management of congenital heart defects. Heart failure is a common final pathway for this diverse patient population, representing the leading cause of mortality in ACHD patients. Herein, we review present guideline-directed management of heart failure in ACHD patients. Recent findingsThere exists a dearth of data to guide management of ACHD-related heart failure. Given this gap, recent guidelines have been limited in the recommendations they can provide for this patient population, with practitioners being consequently forced to generalize findings from studies of acquired heart disease patients based on mechanistic plausibility. The small number of studies directly assessing ACHD patients have been largely limited in their clinical relevance through being negative, small, observational, limited to specific subsets of ACHD patients or assessing nonvalidated outcomes. SummaryDespite the prevalence and impact of ACHD-related heart failure, there are limited evidence-based therapies for its management. Given the rising burden of this clinical problem, definitive trials assessing newer therapies are required to establish their potential role in heart failure amongst ACHD patients.

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Early Experience With Sacubitril/Valsartan in Adult Patients With Congenital Heart Disease

Cited by 37 publications

References 12 publications

Safety and Efficacy of Sacubitril/Valsartan in Patients With a Failing Systemic Right Ventricle: A Prospective Single-Center Study

Safety and Efficacy of Sacubitril/Valsartan in Patients With a Failing Systemic Right Ventricle: A Prospective Single-Center Study

Heart failure in congenital heart disease: management options and clinical challenges

Chronic heart failure management in adult patients with congenital heart disease

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