Transcatheter Atrial Flow Regulator Device Implantations in Restrictive Cardiomyopathy: Bridge to Transplantation or Destination Therapy

Background: The PRELIEVE study reported atrial flow regulator (AFR) implantations in patients >65 years old with HFpEF and HFrEF. We published three first-in-human cases of AFR device implantation in restrictive cardiomyopathy (RCM) when LV dysfunction results in LA hypertension and postcapillary pulmonary hypertension (PH; doi: 10.1016/j.jaccas.2022.05.010). Here, we report 1 to 6 year-clinical follow-up of seven RCM post-AFR patients.

Methods: Transcatheter AFR implantations were performed in seven children with RCM (age 3.5–13 years) to create a restrictive LR shunt (fenestration 6 or 8 mm). No procedure-related complications were observed.

Results: Patient #1 (P1; 13 years old F in NYHA-FC 3) greatly benefited clinically, and she had a reduction of LA volume by −52% (CMR) 2 weeks after AFR implantation. Eight months later, her mPAP had decreased from 30 to 19 mm Hg (no PH). Twenty-seven months after AFR implantation, P1 is in excellent condition (NYHA-FC 2, interatrial dP = 8 mm Hg), on eplerenone, clopidogrel, and iron. P2 was an 11-year-old girl with RCM/genetic syndrome in NYHA-FC4. The AFR immediately reduced PAWP from 29 to 21 mm Hg, and the patient improved clinically. She was not a Tx candidate and died 25 months after the procedure. P3 (6-year-old F) had clinical and hemodynamic improvement after AFR implantation and converted from HLTx to heart transplantation (HTx) listing. She successfully underwent HTx 17 months after AFR implantation. P4 was a 4-year-old girl who decreased her mPAP from 38 to 33 mm Hg and PAWP from 29 to 23 mm Hg, 10 minutes after AFR implantation, and successfully underwent HTx 15 months thereafter. P5 (3.5-year-old F) had mPAP 27 and LVEDP 25 mm Hg at the time of AFR implantation and underwent successful HTx 2 months after AFR implantation. P6 was an 11-year-old girl who was on BVAD for 22 months. AFR decreased mLAP from 28 to 18 mm Hg. She underwent successful HTx 19 months later. P7 (15-year-old F) had clinical and hemodynamic improvement after AFR implantation. Re-dilation of AFR 3 years later reduced mLAP from 17 to 13 mm Hg. She is currently evaluated for HTx listing.

Conclusion: AFR device implantation in RCM is safe and can prevent progressive postcapillary PH/PVR elevation that would preclude HTx and require heart–lung–Tx listing. AFR device implantation can be considered a bridge to HTx in young patients with fatal RCM, and destination therapy in those who are not HTx candidates.

Publikationsverlauf

Artikel online veröffentlicht:
11. Februar 2025