Table 3.

Targeted PAH treatment of the patients with congenital heart disease-associated pulmonary arterial hypertension. “Non-Eisenmenger-PAH treated” comprises patients with congenital heart disease-associated pulmonary arterial hypertension but without Eisenmenger syndrome who received targeted PAH treatment. Abbreviations: ERA, endothelin receptor antagonists; PAH, pulmonary arterial hypertension; PDE5-I, phosphodiesterase type-5 inhibitors; sGC-stimulator, soluble guanylate cyclase (sGC) stimulator. Comparative tests were only performed between treated Eisenmenger and non-Eisenmenger patients.

Treatment Group	Number of Patients Included (Total)	Targeted PAH Medication	n (%)	p-Value	Mono-/Combination-Therapy	p-Value
CHD-associated PAH - total	680	ERA PDE5-I Prostanoid sGC-stimulator Tyrosine kinase inhibitor	389 (57.2%) 353 (51.9%) 35 (5.1%) 17 (2.5%) 1 (0.1%)		408 (60.0%)/ 192 (28.3%)
Eisenmenger, treated	240	ERA PDE5-I Prostanoid sGC-stimulator Tyrosine kinase inhibitor	172 (71.7%) 134 (55.8%) 20 (8.3%) 7 (2.9%) 0		152 (63.3%)/ 88 (36.7%)
Non-Eisenmenger-PAH, treated	167	ERA PDE5-I Prostanoid sGC-stimulator Tyrosine kinase inhibitor	100 (59.9%) 97 (58.1%) 7 (4.2%) 6 (3.6%) 1 (0.6%)	0.013 0.652 0.099 0.703 0.410	121 (72.5%)/ 46 (27.5%)	0.054
Fontan, treated	7	ERA PDE5-I Prostanoid sGC-stimulator Tyrosine kinase inhibitor	1 (14.3%) 7 (100 %) 1 (14.3%) 0 0		6 (85.7%)/ 1 (14.3%)
Not categorized, treated	187	ERA PDE5-I Prostanoid sGC-stimulator Tyrosine kinase inhibitor	116 (62.4%) 115 (61.8%) 7 (3.8%) 4 (2.2%) 0		129 (69.4%)/ 57 (30.6%)