Shape Variations in Right Ventricular 3D Geometry Are Associated With Adverse Outcomes in Hypoplastic Left Heart Syndrome Patients: A Fontan Outcomes Registry Using CMR Examinations (FORCE) Study
Authors' conclusion
Our statistical shape modeling analyses provide novel insights into RV geometric remodeling in HLHS and identify specific shape phenotypes associated with dysfunction and adverse outcomes.
Study context
- Population
- Patients with hypoplastic left heart syndrome (HLHS) post-Fontan (FORCE registry CMR)mean age 14.7±6.3 years
- Participants
- 329 patients
- Outcome
- Composite:
- Adverse outcome including HF
- Mortality/transplant
Methodological detailsstatistical shape modeling
- Statistical shape modeling
Main finding
As reported in the abstract
Associations of RV shape/volume with adverse outcomes
- Right ventricle (RV) end-diastolic volume independently associated with composite adverse outcome, OR 6.50
- P=0.001
- Apical bulge phenotype independently associated with composite adverse outcome, OR 2.45
- P=0.047
- Apical bulge phenotype independently associated with mortality/transplant, OR 4.24
- P=0.004
Observational data: associations, not effects.
Further findings
Mean RV shape template
- Circumferentially dilated RV with loss of septal concavity
Apical bulge phenotype correlates
- Correlated with RV dilation, hypertrophy and impaired regional strain
Spheroidal shape
- Associated with ≥moderate tricuspid regurgitation and tricuspid annular dilation
Remnant LV morphology
- Influenced RV shape and function but not transplant-free survival
Original publication
Shape Variations in Right Ventricular 3D Geometry Are Associated With Adverse Outcomes in Hypoplastic Left Heart Syndrome Patients: A Fontan Outcomes Registry Using CMR Examinations (FORCE) Study. Circ Cardiovasc Imaging. Published 5 November 2025. doi:10.
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