# The Use of Vasodilator Therapy in Fontan Patients: A Single-Centre Experience

**Authors:** Alessia Faccini, Martina Avesani, Roberta Biffanti, Elettra Pomiato, Domenico Sirico, Alice Pozza, Alessia Cerutti, Elena Reffo, Biagio Castaldi, Giovanni Di Salvo

PMC · DOI: 10.3390/children12060751 · Children · 2025-06-10

## TL;DR

This study shares a hospital's experience using vasodilator therapy in patients who had a Fontan operation, highlighting how and why it was used.

## Contribution

The paper provides a detailed single-center analysis of vasodilator therapy use in Fontan patients without a standardized protocol.

## Key findings

- 17.1% of Fontan patients received pulmonary vasodilator therapy for various complications.
- Patients on vasodilators had higher rates of single right ventricle physiology and pulmonary hypertension.
- Vasodilator use was guided by clinical and hemodynamic factors rather than a standard protocol.

## Abstract

Background: The aim of this study was to describe our centre experience in the use of pulmonary vasodilator therapy in Fontan patients. Methods: We retrospectively enrolled patients that underwent Fontan operation between 2000 and 2024, reporting demographic and operative data and noting complications and the use of pulmonary vasodilators. Results: A total of 117 patients were followed for a median time of 150 months (90–207). In total, 36.7% were female, and the median age during the intervention was 50 months (37–64), and 53% had a single left ventricle physiology. In 20 of these 117 patients (17.1%), at least one pulmonary vasodilator drug was used during their life for the following reasons: 6 elevated pressures in the circuit, 3 low oxygen saturation, 2 plastic bronchitis, 2 pleural effusion, 1 chylothorax, 1 persistent pericardial effusion, 1 haemoptysis, 1 protein losing enteropathy, 1 poor exercise tolerance, 1 pulmonary arterial hypertension present since birth and 1 diastolic dysfunction. They had a significantly higher prevalence of single right ventricle physiology (65% vs. 37%, p = 0.03), pulmonary hypertension (60% vs. 0, p = 0.0001), plastic bronchitis (10% vs. 0, p = 0.03) and declivous oedema in the follow-up period (10% vs. 0, p = 0.03), with a higher assumption of warfarin (35% vs. 6.2%, p = 0.001). Conclusions: We found that in the absence of a standardise protocol, we usually use pulmonary vasodilator therapy in Fontan patients, as it is guided by clinical aspects and hemodynamic conditions, which lead us to start and stop this therapy.

## Linked entities

- **Diseases:** pulmonary arterial hypertension (MONDO:0015924), plastic bronchitis (MONDO:0018597), protein losing enteropathy (MONDO:0009174)

## Full-text entities

- **Diseases:** protein losing enteropathy (MESH:D011504), pulmonary hypertension (MESH:D006976), pericardial effusion (MESH:D010490), pulmonary arterial hypertension (MESH:D000081029), diastolic dysfunction (MESH:D018487), declivous oedema (MESH:C536897), pleural effusion (MESH:D010996), plastic bronchitis (MESH:D001991), chylothorax (MESH:D002916)
- **Chemicals:** oxygen (MESH:D010100), warfarin (MESH:D014859)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## References

21 references — full list in the complete paper: https://tomesphere.com/paper/PMC12191318/full.md

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Source: https://tomesphere.com/paper/PMC12191318