Sacubitril/valsartan for heart failure in adults with complex congenital heart disease

Maurer, Sabine; Pujol, C.; Schiele, Sandra; Hager, Alfred; Ewert, Peter; Tutarel, Oktay

doi:10.1016/j.ijcard.2019.06.031

Cited by 38 publications

(15 citation statements)

References 14 publications

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“…As opposed to the recent work of Maurer et al, our findings show that this specific group of patients can indeed benefit from treatment with sacubitril/ valsartan. 21 Previous studies with smaller cohorts or populations mixed with other types of congenital heart defects show neutral or tentatively positive results but do not perform formal statistics or do not concern patients with systemic RV specifically. 21 22 Our findings provide new insight into the pharmacological possibilities of heart failure treatment in the patients with systemic RV and justify assessment in larger prospective cohorts.…”

Section: Discussionmentioning

confidence: 97%

Sacubitril/valsartan in the treatment of systemic right ventricular failure

Zandstra

Nederend

Jongbloed

et al. 2021

Heart

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ObjectivePharmacological options for patients with a failing systemic right ventricle (RV) in the context of transposition of the great arteries (TGA) after atrial switch or congenitally corrected TGA (ccTGA) are not well defined. This study aims to investigate the feasibility and effects of sacubitril/valsartan treatment in a single-centre cohort of patients.MethodsData on all consecutive adult patients (n=20, mean age 46 years, 50% women) with a failing systemic RV in a biventricular circulation treated with sacubitril/valsartan in our centre are reported. Patients with a systemic RV ejection fraction of ≤35% who were symptomatic despite treatment with β-blocker and ACE-inhibitor/angiotensin II receptor-blockers were started on sacubitril/valsartan. This cohort underwent structural follow-up including echocardiography, exercise testing, laboratory investigations and quality of life (QOL) assessment.ResultsSix-month follow-up data were available in 18 out of 20 patients, including 12 (67%) patients with TGA after atrial switch and 6 (33%) patients with ccTGA. N-terminal pro-B-type natriuretic peptide (NT-pro-BNP) decreased significantly (950–358 ng/L, p<0.001). Echocardiographic systemic RV fractional area change and global longitudinal strain showed small improvements (19%–22%, p<0.001 and −11% to −13%, p=0.014, respectively). The 6 min walking distance improved significantly from an average of 564 to 600 m (p=0.011). The QOL domains of cognitive function, sleep and vitality improved (p=0.015, p=0.007 and p=0.037, respectively).ConclusionsWe describe the first patient cohort with systemic RV failure treated with sacubitril/valsartan. Treatment appears feasible with improvements in NT-pro-BNP and echocardiographic function. Our positive results show the potential of sacubitril/valsartan for this patient population.

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Section: Discussionmentioning

confidence: 97%

Sacubitril/valsartan in the treatment of systemic right ventricular failure

Zandstra

Nederend

Jongbloed

et al. 2021

Heart

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“…[ 32 33 ] Sacubitril-valsartan should augment this endogenous defence mechanism and could be beneficial in heart failure with both reduced and preserved ejection fraction. [34] …”

Section: Discussionmentioning

confidence: 99%

Efficacy and safety of sacubitril-valsartan in patients with heart failure: a systematic review and meta-analysis of randomized clinical trials

Lin¹,

Zhou²,

Xie³

et al. 2021

Medicine

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Background: To investigate the efficacy and safety of sacubitril-valsartan in patients with heart failure, relevant randomized clinical trials (RCTs) were analyzed. Methods: We used Cochrane Library, PubMed web of science, CNKI, VIP, Medline, ISI Web of Science, CBMdisc, and Wanfang database to conduct a systematic literature research. A fixed-effects model was used to evaluate the standardized mean differences (SMDs) with 95% confidence intervals. We conducted sensitivity analysis and analyzed publication bias to comprehensively estimate the efficacy and safety of sacubitril-valsartan in patients with heart failure. Results: Among 132 retrieved studies, 5 relevant RCTs were included in the meta-analysis. The result showed that left ventricular ejection fraction (LVEF) was improved after sacubitril-valsartan in patients with heart failure, with an SMD (95% CI of 1.1 [1.01, 1.19] and P < .00001 fixed-effects model). Combined outcome indicators showed that, combined outcome indicators showed that, compared with control group, the left ventricular volume index (LAVI) (WMD = −2.18, 95% CI [−3.63, −0.74], P = .003), the E/e’ (WMD = −1.01, 95% CI [−1.89, −0.12], P = .03), the cardiovascular death (RR = 0.89, 95% CI [0.83, 0.96], P = .003], and the rehospitalization rate of heart failure (RR = 0.83, 95% CI [0.78, 0.88], P < .01) decreased more significantly, but it had no effect on renal function (WMD = 0.74, 95% CI [0.54, 1.01], P = .06). Conclusions: The present meta-analysis suggested that sacubitril-valsartan may improve the cardiac function of heart failure. Given the limited number of included studies, additional large sample-size RCTs are required to determine the long-term effect of cardiac function of sacubitril-valsartan in patients with heart failure.

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“…Although large trials evaluating the effect of sacubitril/valsartan in the systemic right ventricle are lacking, recently a small study in 23 patients with ACHD of moderate and severe complexity, including 12 patients with a systemic right ventricle, was reported. During a median follow-up of 221 days, there was no improvement in systemic ventricular function or functional status [71].…”

Section: Heart Failure In Systemic Right Ventriclementioning

confidence: 90%

“…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. In four patients, treatment was discontinued due to side effects [71].…”

Section: Novel Therapies: Angiotensin Receptor Neprilysin Inhibitorsmentioning

confidence: 99%

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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Sacubitril/valsartan for heart failure in adults with complex congenital heart disease

Cited by 38 publications

References 14 publications

Sacubitril/valsartan in the treatment of systemic right ventricular failure

Sacubitril/valsartan in the treatment of systemic right ventricular failure

Efficacy and safety of sacubitril-valsartan in patients with heart failure: a systematic review and meta-analysis of randomized clinical trials

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

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