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Outcomes in Patients Receiving Treatment for Pulmonary Arterial Hypertension Associated With Repaired Congenital Heart Disease

RA. Krasuski, T. Tobore, S. Studer, P. Jansa, O. Sitbon, MM. Hoeper, R. Channick, S. Gaine, I. Lang, K. Chin, T. Pulido, S. Mehta, A. Torbicki, B. Sastry, X. Tang, V. McLaughlin, LC. Reardon

. 2025 ; 4 (3) : 101626. [pub] 20250224

Status not-indexed Language English Country United States

Document type Journal Article

Persistent link   https://www.medvik.cz/link/bmc25008310

BACKGROUND: Pulmonary arterial hypertension (PAH) is a common complication among patients with congenital heart disease (CHD). Despite advances in PAH treatment, evidence for the benefits of PAH therapies in CHD-PAH is limited. OBJECTIVES: This analysis aimed to evaluate outcomes in patients with repaired PAH-CHD receiving an approved PAH drug. METHODS: This was a pooled analysis including CHD-PAH patients whose CHD was repaired ≥1 year prior from 3 randomized, placebo-controlled, event-driven studies: GRIPHON (NCT01106014), SERAPHIN (NCT00660179), and COMPASS-2 (NCT00303459). The primary endpoint was time to first confirmed morbidity/mortality (M/M) event. HRs with 95% CIs were determined with random effects models. RESULTS: The analysis included 1,982 patients with PAH, 177 (8.9%) with CHD-PAH. In the overall PAH cohort, the mean age was 48 and 49 years in treatment and placebo groups; 80% and 77% were female. In the CHD-PAH cohort, the mean age was 41 and 39 years; 70% and 66% were female. Overall, ≥98% in each group were World Health Organization functional class II and III at baseline. There was a significant reduction in risk of M/M events vs placebo in the overall PAH and CHD-PAH cohorts: 37% reduction in the overall PAH cohort (HR: 0.63; 95% CI: 0.52-0.77) and 50% reduction in the CHD-PAH population (HR: 0.50; 95% CI: 0.26-0.94). CONCLUSIONS: Treatment with approved PAH drugs provided a similar reduction in M/M risk in patients with repaired CHD-PAH when compared with the overall PAH population. This pooled analysis provides important evidence to guide medical management in this patient population.

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$a BACKGROUND: Pulmonary arterial hypertension (PAH) is a common complication among patients with congenital heart disease (CHD). Despite advances in PAH treatment, evidence for the benefits of PAH therapies in CHD-PAH is limited. OBJECTIVES: This analysis aimed to evaluate outcomes in patients with repaired PAH-CHD receiving an approved PAH drug. METHODS: This was a pooled analysis including CHD-PAH patients whose CHD was repaired ≥1 year prior from 3 randomized, placebo-controlled, event-driven studies: GRIPHON (NCT01106014), SERAPHIN (NCT00660179), and COMPASS-2 (NCT00303459). The primary endpoint was time to first confirmed morbidity/mortality (M/M) event. HRs with 95% CIs were determined with random effects models. RESULTS: The analysis included 1,982 patients with PAH, 177 (8.9%) with CHD-PAH. In the overall PAH cohort, the mean age was 48 and 49 years in treatment and placebo groups; 80% and 77% were female. In the CHD-PAH cohort, the mean age was 41 and 39 years; 70% and 66% were female. Overall, ≥98% in each group were World Health Organization functional class II and III at baseline. There was a significant reduction in risk of M/M events vs placebo in the overall PAH and CHD-PAH cohorts: 37% reduction in the overall PAH cohort (HR: 0.63; 95% CI: 0.52-0.77) and 50% reduction in the CHD-PAH population (HR: 0.50; 95% CI: 0.26-0.94). CONCLUSIONS: Treatment with approved PAH drugs provided a similar reduction in M/M risk in patients with repaired CHD-PAH when compared with the overall PAH population. This pooled analysis provides important evidence to guide medical management in this patient population.
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$a Tobore, Tobore $u Johnson & Johnson, Titusville, New Jersey, USA. Electronic address: TTobore@ITS.JNJ.com
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$a Studer, Sean $u Johnson & Johnson, Titusville, New Jersey, USA
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$a Jansa, Pavel $u Charles University and General University Hospital, Prague, Czech Republic
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$a Sitbon, Olivier $u Université Paris-Saclay, APHP, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
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$a Hoeper, Marius M $u Hannover Medical School, Hannover, Germany; German Center of Lung Research (DZL), Hannover, Germany
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$a Channick, Richard $u David Geffen School of Medicine at University of California Los Angeles, Los Angeles, California, USA
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$a Gaine, Sean $u Mater Misericordiae University Hospital, Dublin, Ireland
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$a Lang, Irene $u Cardiology and Center of Cardiovascular Medicine, Medical University of Vienna, Vienna, Austria
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$a Chin, Kelly $u University of Texas, Southwestern Medical Center, Dallas, Texas, USA
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$a Pulido, Tomas $u Ignacio Chavez National Heart Institute, Mexico, Mexico
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$a Mehta, Sanjay $u London Health Sciences Centre, Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada
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$a Torbicki, Adam $u Centre of Postgraduate Medical Education, European Health Centre, Otwock, Poland
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$a Sastry, Bhagavatula $u CARE Hospitals, Hyderabad, India
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$a Tang, Xiaoqin $u Johnson & Johnson, Titusville, New Jersey, USA
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$a McLaughlin, Vallerie $u University of Michigan Health System, Ann Arbor, Michigan, USA
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$a Reardon, Leigh C $u Ahmanson/UCLA Adult Congenital Heart Disease Center University of California, Los Angeles, California, USA
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