Introduction
Patients with adult congenital heart disease (ACHD) who have an anatomic right ventricle
supporting the systemic circulation have increased mortality and morbidity from heart
failure (HF). Angiotensin receptor–neprilysin inhibitors (ARNI) have emerged as a
standard of therapy for adults with HF. However, the effects of this therapy have
not been extensively studied in ACHD patients, especially those with systemic right
ventricle (SRV).
Hypothesis
ARNIs are associated with subjective and objective improvement in SRV patients.
Methods
Eighteen (18) SRV patients were prescribed ARNI at our institution in the last 5 years.
Data before and during treatment, including demographics, medical history, New York
Heart Association functional class (NYHA FC), labs, cardiac computed tomography (CT)
or magnetic resonance imaging (MRI), echocardiographic measurements, cardiopulmonary
stress test (CPET), and hospitalisation for HF were obtained by review of the electronic
medical record. Statistical analysis was performed using paired t and Wilcoxon rank
sum tests.
Results
Eighteen (18) SRV patients (mean age 40 yrs, 72% male) were treated with ARNI (median
duration 13 mo) in addition to other HF medications. All patients tolerated ARNI without
symptomatic or asymptomatic hypotension or worsening kidney function. High ARNI dose
(97/103 mg) was achieved in three (17%) patients, and moderate (49/51 mg) in three
(17%). At baseline, nine patients were NYHA FC 2, seven FC 3, and two FC 4. Mean baseline
cardiopulmonary exercise testing (CPET) and echocardiographic data were: oxygen uptake
(VO2) 18 mL/kg/min, minute ventilation/carbon dioxide (VE/VCO2) 38, right ventricular ejection fraction (RVEF) 32%, fractional area change (FAC)
21%. Significant tricuspid regurgitation was present in 33% (28% moderate, and 5%
severe) and mean tricuspid annular plane systolic excursion (TAPSE) was 9.4 mm. With
treatment, there was no statistically significant difference in blood pressure, labs,
testing, or imaging. There was a statistically significant improvement in median NYHA
FC (2 vs 2.5, p=0.005). When compared to an equal pre-ARNI median timeframe, there
was a noted decrease in cardiac hospitalisation (4 vs 9) that did not reach statistical
significance (p=0.313).
Conclusion
In adult patients with failing systemic right ventricle, ARNI is safe and well tolerated.
Their use is associated with improvement in functional status. Prospective studies
on a larger group of patients are warranted to better understand the causes of this
improvement.
Keywords
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Article info
Publication history
Published online: March 09, 2022
Accepted:
February 9,
2022
Received in revised form:
November 14,
2021
Received:
July 13,
2021
Identification
Copyright
© 2022 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.
