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Original research
Prevalence of pulmonary hypertension in adults after atrial switch and role of ventricular filling pressures
  1. William R Miranda1,
  2. C Charles Jain1,
  3. Heidi M Connolly1,
  4. Hilary M DuBrock2,
  5. Frank Cetta3,
  6. Alexander C Egbe1,
  7. Donald J Hagler1,3
  1. 1Department of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA
  2. 2Division of Pulmonary and Critical Care Medicine, Mayo Clinic Minnesota, Rochester, Minnesota, USA
  3. 3Department of Pediatric and Adolescent Medicine/Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota, USA
  1. Correspondence to Dr William R Miranda, Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, USA; miranda.william{at}mayo.edu

Abstract

Objective To assess the prevalence of elevated systemic right ventricular (sRV) end-diastolic pressure and pulmonary arterial hypertension in adults with transposition of the great arteries (TGA) who have undergone atrial switch operation.

Methods Forty-two adults (aged ≥18 years) with complete TGA and atrial switch palliation undergoing cardiac catheterisation between 2004 and 2018 at Mayo Clinic, MN, were identified. Clinical, echocardiographic and invasive haemodynamic data were abstracted from the medical charts and procedure logs.

Results Mean age was 37.6±7.9 years; 28 were male (67%). The Mustard operation was performed in 91% of individuals. Mean estimated sRV ejection fraction by echocardiography was 33.3%±10.9% and ≥moderate tricuspid (systemic atrioventricular valve) regurgitation was present in 15 patients (36%). Mean sRV end-diastolic pressure was 13.2±5.4 mm Hg. An sRV end-diastolic pressure >15 mm Hg was present in 35% of individuals whereas a pulmonary artery wedge pressure (PAWP) >15 mm Hg was seen in 59%. Mean pulmonary artery pressure ≥25 mm Hg was seen in 47.5% of patients with PAWP being >15 mm Hg in all but one patient.

Conclusion In adults after atrial switch, elevated sRV end-diastolic pressure was present in only one-third of patients whereas increased PAWP was seen in almost 60%. These findings are most likely related to a combination of decreased pulmonary atrial (functional left atrium) compliance and, in a subset of patients, pulmonary venous baffle obstruction. Elevation in pulmonary pressures was highly prevalent with concomitant elevation in PAWP being present in essentially all patients.

  • congenital heart disease
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Footnotes

  • Contributors WRM: study design, data abstraction/analysis, and drafting of the manuscript. HMC: data abstraction and critical review of the manuscript. CCJ, HMD, FC, ACE, DJH: critical review of the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

  • Patient consent for publication Not required.

  • Ethics approval Mayo Clinic (20-000660). The Institutional Review Board approved the study and only patients providing authorisation for the use of their medical records were included.

  • Provenance and peer review Commissioned; externally peer reviewed.

  • Data availability statement Data are available upon reasonable request. The data supporting the findings of the study are available from the corresponding author upon reasonable request.

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