Author + information
- Michael Landzberg,
- Curtis Daniels,
- Paul Forfia,
- Anatoly Langer,
- Vallerie McLaughlin,
- Mona Selej,
- Marjan Sepassi,
- Mary Tan and
- Gary Webb
Background: Patients (pts) with repaired congenital heart disease (rCHD) remain at risk of developing PAH, which is diagnosed per established guidelines by right heart catheterization (RHC). ACHD-QuERI, a US-based, observational, multicenter, longitudinal screening registry (clinicaltrials.gov NCT01659411) developed to facilitate timely detection of PAH in adults with rCHD, is the first registry to prospectively follow high-risk rACHD pts. Baseline (BL) and 1-year (y) interim results are reported.
Methods: Pts aged ≥18 y with rCHD ≥1 y from enrollment, history of a large defect prior to closure and at high risk for PAH by ECHO measures were eligible. Physical exam, ECG, ECHO, WHO functional class (FC), 6 minute walk distance (6MWD), and SpO2 are prospectively collected to evaluate progression to PAH. Pop-up reminders of pt-specific evidence-based (clinical, ECG, ECHO) recommendations for PAH detection appeared to clinicians onscreen at data entry.
Results: Overall, 221 pts were eligible for analysis; mean (SD) age was 43.9 (15.9) y and 72.0% were women. At BL (n=219), 82.2% of pts were classified FC I and at 1 y 88.6% (n=140) remained in FC I. At BL, 49.8%, 28.8%, 24.7%, and 23.7% had arrhythmia (palpitations, atrial fibrillation or flutter), exertional dyspnea, were current/former smokers, and had hypertension, respectively. SpO2 (98±4%) did not change between BL (n=203) and 1 y (n=140). Pts who underwent a 6MWD test at BL (48/219) and 1 y (18/139) walked a mean (SD) 434 (109) m and 479 (115) m, respectively. Per pop-up recommendation, ECHO (per ECG high-risk PAH findings) was performed for majority of pts at BL (74.1%, 20/27) but not at 1 y (25.0%, 2/8). RHC (per ECHO high-risk PAH findings) was recommended for 83/216 (38.4%) pts at BL and 44/104 (42.3%) at 1 y; however, at BL and at 1 y, RHC was performed for 7/83 (8.4%) and 1/44 (2.3%), respectively. Primary reason for a clinician not performing nor planning to perform ECHO or RHC was belief that their management was appropriate.
Conclusions: In ACHD-QuERI, data up to 1 y suggest RHC is underutilized in rCHD pts who are at high-risk for PAH (per ECHO). Through reminders, ACHD-QuERI aims to improve PAH screening among ACHD patients at risk for PAH. 3-y follow-up is ongoing.
Moderated Poster Contributions
Congenital Heart Disease and Pulmonary Hypertension Moderated Poster Theater, Poster Hall, Hall C
Sunday, March 19, 2017, 12:30 p.m.-12:40 p.m.
Session Title: Finding Out What Works in Pediatric and Congenital Heart Disease
Abstract Category: 9. Congenital Heart Disease: Adult
Presentation Number: 1312M-03
- 2017 American College of Cardiology Foundation