Table 1.

Clinical information. Main clinical characteristics of the patients and results obtained in the tests performed from diagnosis to the present time.

Number Patient	Patient 1	Patient 2
Etiology	Group 1: PAH associated with HHT	Group 1: idiopathic PAH
Sex	Male	Female
Age at diagnosis (years)	5	4
Current age (years)	13	12
NYHA functional class	II	I
Last known PAH therapies	Bosentan: 125 mg BID Tadalafil: 40 mg/day	Macitentan: 10 mg/day Tadalafil: 40 mg/day
Cardiac catheterization (mPAP and cardiac index)	5 y.o. 1: 55 mmHg; 8.7 L/min/m2 8 y.o.: 20 mmHg	4 y.o.: 33 mmHg; 4.2 L/min/m2 9 y.o.: 44 mmHg; 3.6 L/min/m2
Abdominal and Thoracic computed tomography	5 y.o.: No radiologic abnormalities of significance	4 y.o.: No radiologic abnormalities of significance
Cranial nuclear magnetic resonance	6 y.o.: No significant abnormalities 11 y.o.: No significant abnormalities	Not performed
Gastrointestinal endoscopy	8 y.o.: No telangiectasias detected	Not performed

¹ y.o.: years old.