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Background: Primary determinants of pulmonary blood flow in the Fontan circulation are low transpulmonary gradient and pulmonary vascular resistance (PVR). Changes in intrathoracic pressure during intermittent positive pressure ventilation can influence the transpulmonary gradient, PVR, pulmonary blood flow, and cardiac output. The aim of this study was to evaluate the effect of low (5 mL/kg) versus high (10 mL/kg) tidal volume (V) ventilation on Fontan circulation hemodynamics.
Method: Postoperative patients with a Fontan circulation were enrolled in this single-center, randomized crossover trial. Patients, randomized to group 1 or 2, underwent a ventilation study sequence (baseline ventilation [7 mL/kg], then high V [10 mL/kg] or low V [5 mL/kg], then washout ventilation [7 mL/kg], followed by low [5 mL/kg] or high V [10 mL/kg]) in the operating room at the end of the cardiac surgical procedure. Respiratory, hemodynamic, and transesophageal (TEE) measurements were recorded after 5 min in each ventilation condition. The primary aim of this study was to evaluate the effect of low V ventilation (5 mL/kg) versus high V ventilation (10 mL/kg) on transpulmonary gradient (Fontan pressure minus left atrial pressure). The secondary aim was to compare TEE measurements of pulmonary blood flow, stroke volume, and Fontan flow between low and high V ventilation. We also compared standard hemodynamic and ventilation parameters for all ventilation conditions. Analysis was of paired data, calculating the between-treatment difference within participants across ventilation conditions.
Results: Eleven patients were included in the final data analysis with a median [IQR] age of 5 [4, 11] years and weight of 16.3 [13.8, 31.6] kg. The mean (±SD) peak inspiratory pressure during low and high V ventilation was 15.3 (±2.9) cmHO and 22.2 (±3.7) cmHO, respectively (difference -6.9, 95% CI -7.8, -5.9, p < 0.001). The mean airway pressure during low and high V ventilation was 7.3 ± 0.8 and 8.7 ± 0.9 (difference -1.5, 95% CI -2.1, -0.8, p = 0.001) with a mean inspiratory time of 0.62 (±0.22) s and 1.21 (±0.55) s (difference -0.59, 95% CI -0.84, -0.34, p < 0.001), respectively. During low V ventilation, the mean Fontan pressure was 13.3 (±1.8) mmHg compared to 12.3 (±2.5) mmHg for high V ventilation (difference 0.8, 95% CI -0.5, 2.1, p = 0.18). The mean transpulmonary gradient was 7.0 ± 1.3 mmHg compared to 6.8 ± 1.2 mmHg during low and high V ventilation, respectively (difference 0.2, 95% CI -0.2, 0.6, p = 0.21). We found no significant differences between low and high V ventilation in TEE measures of pulmonary blood flow, stroke volume, and Fontan flow.
Conclusions: This randomized, crossover pilot trial of Fontan patients showed that a low V ventilation (5 mL/kg) resulted in significantly lower peak and mean airway pressure compared with a high V ventilation (10 mL/kg). However, there were no significant changes in transpulmonary gradient, mean Fontan pressure, or TEE parameters of stroke volume, pulmonary blood flow, or Fontan flow.
Clinical Trials Registration Number: NCT04633343.
Principal Investigator: Manchula Navaratnam.
Date Of Registration: November 11, 2020. Clinical Trials Registration Registry URL: https://clinicaltrials.gov/study/NCT04633343?term=Fontan%20ventilation&rank=3.
Prior Presentations: Congenital Cardiac Anesthesia Society Annual Meeting Top Oral Abstract Presentation.
Presenter: Alexander R Schmidt, March 30th, 2023.
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http://dx.doi.org/10.1111/pan.15096 | DOI Listing |
Patients with cardiovascular compromise are likely to develop hypotension upon receiving even small doses of sedatives. On the other hand, patients with severe dental phobias or with intellectual disability who have a severe gag reflex often require deeper levels of anesthesia. Thus, achieving an optimal level of anesthesia can be difficult in patients with cardiovascular compromise because of the relatively narrow range of sedative dosing capable of providing sufficient sedation to prevent the gag reflex without compromising hemodynamics.
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September 2025
Texas Center for Pediatric and Congenital Heart Disease, The University of Texas at Austin Dell Medical School, Austin, TX, USA.
Pericardial effusion (PCE) represents a significant postoperative complication following congenital heart surgery (CHS), contributing to more complex postoperative care and heightened morbidity. In this study, we aim to elucidate the risk factors contributing to PCE development post-CHS through analysis of data from a nationwide, multi-institutional database. Review of the Pediatric Health Information System Database from January 1, 2004, to December 30, 2023.
View Article and Find Full Text PDFJTCVS Open
August 2025
Division of Cardiovascular Surgery, Children's National Heart Institute, Children's National Hospital, Washington, DC.
Objectives: We sought to review the outcomes of patients with Ebstein anomaly (EA) after the Fontan operation.
Methods: Patients with EA were identified from a large binational registry about the Fontan operation. Data were collected from hospital records, registry data, and clinical correspondence.
JTCVS Open
August 2025
Division of Cardiothoracic Surgery, Department of Surgery, University of Southern California, Los Angeles, Calif.
Objective: Patients with heterotaxy-associated congenital heart disease often require multiple operations, which may have a cumulative effect on their outcomes. This study aimed to define the cardiac surgical course in a large cohort and identify longitudinal risk factors for death/transplant.
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Int J Cardiol
September 2025
Department of Molecular and Cellular Sports Medicine, German Sport University Cologne, Am Sportpark Müngersdorf 6, 50933 Cologne, Germany. Electronic address:
Background: Patients with Fontan circulation are often advised to avoid hypoxic exposure due to presumed cardiopulmonary vulnerability. Low-grade inflammation has also been reported in this population and may be influenced by hypoxia and/or exercise. Based on the potential interaction between hypoxia and submaximal exercise in modulating inflammatory signaling, we hypothesized that this combination could exacerbate subclinical inflammation.
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