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Lifelong Management of Right Ventricular Outflow Tract Dysfunction in Adults with Congenital Heart Disease Cover

Lifelong Management of Right Ventricular Outflow Tract Dysfunction in Adults with Congenital Heart Disease

Open Access
|Jun 2026

Figures & Tables

Table 1

Formal factors supporting RVOT intervention in repaired tetralogy of Fallot. RVOTO: right ventricular outflow tract obstruction; PVR: pulmonary valve replacement; RVEDVi: right ventricular end-diastolic volume index; RVESVi: right ventricular end-systolic volume index; RVEF: right ventricular ejection fraction; LVEF: left ventricular ejection fraction; LVEDVi: left ventricular end-diastolic volume index; RVSP: right ventricular systolic pressure; TR: tricuspid regurgitation

FORMAL FACTORS SUPPORTING RVOT INTERVENTION IN RTOF2020 ESC ACHD GUIDELINES32025 ACC/AHA ACHD GUIDELINES1
SymptomsPVR is recommended in symptomatic patients with severe PR and/or moderate RVOTO (Class l-C)PVR is recommended in symptomatic patients with at least moderate PR (Class 1)
Right ventricleAsymptomatic patients should be considered for PVR if PR/RVOTO and (Class II-C):
  • RVEDVi > 160ml/mq

  • RVESVi > 80 ml/mq

  • Progressive RV dysfunction

  • RVOTO with RVSP > 80 mm Hg

Asymptomatic patients should be considered for PVR if at least moderate PR and at least two among (Class 2a):
  • RVESVi > 80 ml/mq

  • RVEDVi ≥ 2x LVEDVi

  • RVEF < 46%

Left ventricleNoneAsymptomatic patients should be considered for PVR if at least moderate PR and LVEF < 50% along with at least one additional factor (Class 2a)
ArrhythmiasNoneAsymptomatic patients should be considered for PVR if at least moderate PR and ventricular arrhythmia (Class 2b)
Functional capacityAsymptomatic patients should be considered for PVR if PR/RVOTO and decrease in objective exercise capacity (Class II-C)Asymptomatic patients should be considered for PVR if at least moderate PR and decrease in objective exercise capacity with at least one additional factor (Class 2a)
Additional factorsAsymptomatic patients should be considered for PVR if PR/RVOTO and progressive TR to at least moderate (Class ll-C)Asymptomatic patients should be considered for PVR if PR/RVOTO and progressive functional TR to at least moderate (Class 2b)
Type of interventionIn patients with no native outflow tract, catheter intervention should be preferred if anatomically feasible (Class l-C)No preference between surgical or transcatheter approach is provided
Figure 1

Surgical RV-PA conduits and prosthetic valves for PVR in ACHD: (A) Pulmonary homograft, (B) Contegra™ pulmonary valved conduit, (C) Hancock™ II bioprosthesis, (D) ePTFE valved conduit. Contegra pulmonary valved conduit and Hancock II bioprosthesis images reprinted with permission from Medtronic.

Figure 2

Transcatheter pulmonary valves for right ventricular outflow tract (RVOT) dysfunction in adult congenital heart disease: (A) Melody™ transcatheter pulmonary valve, (B) Sapien™ 3 Ultra-Resilia valve, (C) Harmony™ transcatheter pulmonary valve, (D) Venus P-Valve™ System, (E) Pulsta™ valve, (F) Alterra™ Adaptive Prestent. Reprinted with permission from Medtronic (Melody, Harmony valves), Edwards Lifesciences (Sapien 3 Ultra Resilia, Alterra Adaptive Prestent), Venus MedTech (VenusP-valve) and Taewoong Medical (Pulsta valve).

DOI: https://doi.org/10.14797/mdcvj.1797 | Journal eISSN: 1947-6108
Language: English
Page range: 67 - 77
Submitted on: Feb 9, 2026
Accepted on: May 21, 2026
Published on: Jun 30, 2026
Published by: Houston Methodist DeBakey Heart & Vascular Center
In partnership with: Paradigm Publishing Services

© 2026 Maurizio Brighenti, Gabriele Egidy Assenza, Elisabetta Mariucci, Andrea Donti, published by Houston Methodist DeBakey Heart & Vascular Center
This work is licensed under the Creative Commons Attribution-NonCommercial 4.0 License.