Congenital heart disease

Feasibility of Treat and Repair Strategy in Congenital Heart Defects With Pulmonary Arterial Hypertension

JACC AdvPublished Cohort study

Authors' conclusion

In this small cohort, a treat and repair strategy was successfully used in a significant proportion of the patients with congenital heart defects with moderate to severe PAH.

Study context

Population
Patients with atrial or ventricular septal defect and pulmonary arterial hypertension (PAH) (PVR ≥ 5 Wood units) eligible for treat and repair strategy
Participants
25 patients
Exposure
Repaired vs unrepaired after treat and repair strategy
  • Repairedn = 20
  • Unrepairedn = 5reference
Outcome
Pretreatment variables associated with successful defect repair

Main finding

As reported in the abstract

Pretreatment variables associated with defect repair
  • Pretreatment mean pulmonary artery pressure, PVR, Qp/Qs and arterial oxygen saturations associated with undergoing defect repair

Observational data: associations, not effects.

Further findings

PVR, repaired group (pretreatment vs pre-repair) · significant decrease

ß coefficient −4.6 (−5.9 to −3.3)

OutcomePretreatmentPre-repairß coefficient (95% CI)
Qp/Qs, repaired group (pretreatment vs pre-repair)1.5 ± 0.62.4 ± 1.30.9 (0.4–1.38)

PVR and Qp/Qs, unrepaired group

PVR decreased with treatment but remained elevated
Qp/Qs did not change between pretreatment and post-treatment

Pretreatment variables associated with defect repair

Pretreatment mean pulmonary artery pressure, PVR, Qp/Qs and arterial oxygen saturations associated with undergoing defect repair

Original publication

Feasibility of Treat and Repair Strategy in Congenital Heart Defects With Pulmonary Arterial Hypertension. JACC Adv. Published 6 March 2024. doi:10.1016/j.jacadv.2024.100887

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