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Improved Exercise Tolerance in an Adolescent Female After Failed Fontan and Subsequent Biventricular Conversion

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Abstract

We report a case of improved exercise tolerance in a single-ventricle patient following biventricular conversion. An 11 year old with a fenestrated extracardiac failing Fontan was accepted for a biventricular conversion repair at an out-of-town institution. The patient had multiple adverse cardiac events following Fontan surgery including recurrent pleural effusions, arteriovenous malformations, protein-losing enteropathy, and marked exercise intolerance. Serial cardiac catheterizations revealed chronic elevated pulmonary artery and Fontan pressures, normal left ventricular end-diastolic pressure and an adequately sized left ventricle. Cardiopulmonary exercise testing demonstrated severely reduced exercise tolerance due to ventilatory and cardiac limitations with significant arterial desaturations during exercise. Following a successful biventricular conversion, exercise tolerance improved remarkably, as evidenced by improved oxygen uptake and ventilatory efficiency. Our case demonstrates that biventricular conversion surgery may offer improvement in quality of life and exercise capacity in selected patients with failing Fontan physiology.

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Abbreviations

AVM:

Arteriovenous malformation

BiVC:

Biventricular conversion

CPET:

Cardiopulmonary exercise test

HR:

Heart rate

LV:

Left ventricle

LVEDP:

Left ventricular end-diastolic pressure

MRI:

Magnetic resonance imaging

O2P:

Oxygen pulse

PG:

Pressure gradient

PLE:

Protein-losing enteropathy

RER:

Respiratory exchange ratio

RV:

Right ventricle

RVP:

Right ventricular pressure

SV:

Single ventricle

VAT:

Ventilatory anaerobic threshold

VE/VCO2 slope:

Ventilatory requirements for carbon dioxide

VO2:

Oxygen consumption

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Funding

No funding was secured for this study.

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Authors and Affiliations

  1. Department of Pediatrics, Rush University Medical College, Chicago, IL, USA

    Brittany Tang, Angela Romme, Rima Dababneh & Sawsan Awad

  2. Department of Pediatrics/Division of Cardiology, Rush University Children’s Hospital, 1753 W. Congress Pkwy, Pavilion 654, Chicago, IL, 60612, USA

    Brittany Tang

Authors
  1. Brittany Tang
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  2. Angela Romme
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  3. Rima Dababneh
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  4. Sawsan Awad
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Contributions

BT conceptualized and designed the study, drafted the initial manuscript, and reviewed and revised the manuscript. AR conceptualized and designed the study, assisted in data collection, reviewed, and revised the manuscript. RD assisted in data collection and reviewed and revised the manuscript. SA conceptualized and designed the study, coordinated and supervised data collection, and critically reviewed the manuscript for important intellectual content. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

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Correspondence to Brittany Tang.

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Tang, B., Romme, A., Dababneh, R. et al. Improved Exercise Tolerance in an Adolescent Female After Failed Fontan and Subsequent Biventricular Conversion. Pediatr Cardiol 43, 1669–1673 (2022). https://doi.org/10.1007/s00246-022-02931-y

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  • DOI: https://doi.org/10.1007/s00246-022-02931-y

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