Congenital heart disease

Outcomes After Development of Ventricular Arrhythmias in Single Ventricular Heart Disease Patients With Fontan Palliation

Circ Arrhythm ElectrophysiolPublished Retrospective cohort study

Main finding · Transplant or deathResult — go to the resultHazard ratio 9.2 95% CI 4.5–18.7

Authors' conclusion

Ventricular arrhythmias occurred in 11% of Fontan patients and was highly associated with transplant or death, with a transplant-free survival of <50% at 5-year post-VA diagnosis.

Study context

Population
Single ventricle patients with Fontan palliation seen at a single center, 2002–2019, without preFontan VA
Participants
431 patients
Exposure
Post-Fontan VA vs no VA
  • VAn = 48
Outcome
Death and heart transplantation
Methodological detailssingle-centre
  • Single-centre

Main finding

Transplant or death

Hazard ratio

9.2

95% CI 4.5–18.7

P < 0.001

By group

VA

48%

Transplant-free survival at 5 years post-VA diagnosis

VAvs No VA
HR 9.2 (95% CI 4.5–18.7); P < 0.001

As reported in the abstract

Overall primary outcome (death or transplant)
64 (15%)
Overall transplant-free survival at 15 years post-Fontan
82%
Death
16, 3.7%
Transplant
48, 11%

Observational data: associations, not effects.

Secondary outcomes

Incidence of VA

VA diagnosed
48 (11%)
Nonsustained VT among VA
90%
Sustained VT among VA
10%
Malignant VA
9, 2.0%
VA risk at 10 years
2.4%
VA risk at 20 years
19%

Risk factors for VA

Stage 1 surgery with RV to pulmonary artery conduit and older age at Fontan were significant risk factors for VA

Malignant VA associations

Malignant VA associated with younger age, worse systolic function and valvular regurgitation

Original publication

Outcomes After Development of Ventricular Arrhythmias in Single Ventricular Heart Disease Patients With Fontan Palliation. Circ Arrhythm Electrophysiol. Published 31 May 2023. doi:10.1161/circep.122.011143

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