Calendar | For Your Patients | PHA Main Site | Contact Us | About Us | Not a registered user? Sign up here.

Resource Library

REVEAL Registry: Two Year Outcome of Patients with Congenital Heart Disease-Associated Pulmonary Arterial Hypertension (CHD-APAH) Compared with Idiopathic PAH Patients

Robyn Barst


A. J. Foreman

Michael McGoon


Reviews

  Sign in to add a review

0 comments
Leave a Comment

Conference: 2010 International PHA Conference and Scientific Sessions

Release Date: 06.24.2010

Presentation Type: Abstracts

Barst RJ1, Foreman AJ2, Ivy DD3, McGoon MD4.
1. Columbia University College of Physicians and Surgeons, New York, NY, USA

2. ICON Clinical Research, Ellicott City, MD, USA
3. Children's Hospital, Aurora, CO, USA
4. Mayo Clinic, Rochester, MN, USA

BACKGROUNDThe REVEAL Registry is a 54 center, observational, US study evaluating 3500 PAH patients (pts) followed ≥5 yrs from enrollment (ENR). REVEAL objectives include outcomes in PAH subgroups including CHD-APAH pts.

METHODSOutcomes (survival and all-cause hospitalization) for CHD-APAH pts were compared to idiopathic PAH (IPAH) pts. Group I subgroups are from the time of study enrollment.

RESULTSOf 2967 pts entered (3/06-9/07), 1378 had IPAH and 343 had CHD-APAH at ENR: 92 complete repair (CHD-COM), 189 unrepaired/48 partial repair (CHD-U/P, n=237), and 14 unclassified. Demographics, treatments and outcomes are in the Table. FC and hemodynamics were worse in CHD-U/P than CHD-COM pts. 2 yr survival from ENR and 5 yr survival from diagnosis (dx; i.e., date of confirmatory RHC) were similar for IPAH, all CHD-APAH, CHD-COM and CHD-U/P. There were also no differences in all-cause hospitalizations 2 yr from ENR between the groups.

CONCLUSIONS: Historically, unrepaired CHD-APAH (e.g., Eisenmenger Syndrome) pts had a much better long-term outcome vs IPAH. However, in REVEAL, CHD-APAH pts have similar outcomes as IPAH pts at 2 yrs after ENR and 5 yrs from dx. Longer follow-up is needed to discern whether these similarities will continue. Furthermore, although the CHD-U/P pts were worse by FC at ENR, survival and freedom from hospitalization CHD-COM vs CHD-U/P were similar at 2 yrs from ENR and 5 yr survival from dx was similar. Longer follow-up is important to determine if complete repair is appropriate for all APAH-CHD pts.

 

Data are mean (SD)
unless specified otherwise

CHD
n=343 
CHD-COM
n=92 
CHD-U/P
n=237 
IPAH
n=1378 
P-value
CHD vs
IPAH 
P-value 
CHD-COM
vs U/P 
P-value
CHD-COM
vs IPAH 
P-value 
CHD-U/P 
vs IPAH 
Age at ENR, yrs  39 (18)
 37 (20)
 40 (17)
 51 (18)
<0.001 0.14 
<0.001 
<0.001
Time from dx to ENR, mos 
 61 (72)
 63 (76)
 61 (71)
 40 (44)
<0.001  0.80 <0.001 
<0.001 
≤18y at dx, %  
 21  29 19 
 7 <0.001 
 0.033 <0.001 
<0.001 
Female, %  74  74  75  78 0.093 
 0.89 0.36 
0.26 
Newly Dx*, %  7  9  6  15 <0.001  0.45  0.11 <0.001
NYHA FC I/II, %  49  61  45  46 0.35 
 0.016  0.012  0.73
ETRAs at ENR, % 
 55  45  60  46  0.006  0.020  0.86 <0.001
Prostacyclins at ENR, % 
 34  24  37  47  <0.001  0.025  <0.001  0.005
PVRI at ENR, Wood units•m2 
 17 (10)
 18 (10)
 N/A  19 (11)
0.12 
 N/A  0.21  N/A
mPAP at ENR, mmHg 
 58 (19)
 52 (17)
 61 (19)
 50 (14)
 <0.001  <0.001  0.32 <0.001
2-year survival from ENR, % 
 87  89  86  87 0.63 
 0.60  0.50 0.97 

2-year freedom from
hospitalization from ENR, %

 63  64  61  61  0.84  0.59  0.74  0.76
5-year survival from dx, % 
 70  67  71  67  0.83 0.77 
 0.67 0.91 

N/A: Incomplete data for CHD-U/P pts at time of analysis. 
*Newly dx are diagnosed within 3 months of enrollment in REVEAL.