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Systemic Artery Vasoconstrictor Therapy in Fontan Patients with High Cardiac Output-Heart Failure: A Single-Center Experience

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Abstract

Failed Fontan Patients with high cardiac output (CO) heart failure (HF) might have vasodilatory syndrome and markedly high mortality rates. The aim of this study was to review the clinical effects of vasoconstrictor therapy (VCT) for failed Fontan hemodynamics. We retrospectively reviewed 10 consecutive patients with Fontan failure (median age, 33 years) and high CO–HF who had received VCT. The hemodynamics were characterized by high central venous pressure (CVP: median, 16 mm Hg), low systolic blood pressure (median, 83 mm Hg), low systemic vascular resistance (median, 8.8 U·m2), high cardiac index (median, 4.6 L/min/m2), and low arterial oxygen saturation (median, 89%). VCT included intravenous noradrenaline infusion for five unstable patients, oral midodrine administration for nine stable patients, and both for four patients. After VCT introduction with a median interval of 1.7 months, the median systolic blood pressure (102 mm Hg, p = 0.004), arterial oxygen saturation (90%, p = 0.03), and systemic vascular resistance (12.1 U·m2, p = 0.13) increased without significant changes in CVP or cardiac index. After a median follow-up of 21 months, the number of readmissions per year decreased from 4 (1–11) to 1 (0–9) (p = 0.25), and there were no VCT-related complications; however, five patients (50%) developed hepatic encephalopathy, and six patients (60%) eventually died. VCT was safely introduced and could prevent the rapidly deteriorating Fontan hemodynamics. VCT could be an effective therapeutic strategy for failed Fontan patients with high CO–HF.

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The authors have no financial relationships relevant to this article to disclose.

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Contributions

HM Investigation, Data Curation, Writing—Original Draft, Visualization. Hideo Ohuchi: Conceptualization, Methodology, Writing, Review & Editing, Project administration. YH Resources. TI Resources. HS Resources. KK Resources. MN Statistic analysis, Supervision.

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Correspondence to Hideo Ohuchi.

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All the authors declared that they have no conflict of interest.

Ethical Approval

The Ethics Committee of the National Cardiovascular Center approved the study protocol (M23-002–4), and consent was waived due to the retrospective nature of the study.

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Miike, H., Ohuchi, H., Hayama, Y. et al. Systemic Artery Vasoconstrictor Therapy in Fontan Patients with High Cardiac Output-Heart Failure: A Single-Center Experience. Pediatr Cardiol 42, 700–706 (2021). https://doi.org/10.1007/s00246-020-02532-7

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  • DOI: https://doi.org/10.1007/s00246-020-02532-7

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