Table 1

Clinical data

Patient	Group	Diagnosis	Age (years)	Sex	Symptoms	Medications	NYHA
Group 1: primary or automimmune pulmonary arterial hypertension (PAH); group 2: PAH associated with congenital heart disease without complete mixing; group 3: PAH associated with congenital heart disease with complete mixing.
ASD, atrial septal defect; CREST syndrome: calcinosis, Raynaud’s phenomenon, oesophageal dysmotility, sclerodactyly, and telangiectasias; DOE, dyspnoea on exertion; F, female; M, male; NYHA, New York Heart Association functional class; PAH, pulmonary arterial hypertension; PDA, patent ductus arteriosus; VSD, ventricular septal defect.
1	1	Primary PAH	46	M	DOE	Nifedipine, warfarin	II
2	1	Primary PAH	24	F	DOE	Nifedipine, warfarin	II
3	1	Primary PAH	32	F	DOE	Furosemide, captopril	II
4	1	Primary PAH	27	F	DOE	Warfarin	II
5	1	Primary PAH	39	F	DOE	Nifedipine	II
6	1	Primary PAH	31	M	DOE, leg oedema	Nifedipine, warfarin, digoxin, furosemide	IV
7	1	Primary PAH	35	F	DOE, syncope	Furosemide	II
8	1	Primary PAH	26	M	DOE	Furosemide	II
9	1	PAH, cold agglutinin syndrome	50	M	DOE, syncope	Nifedipine, warfarin, furosemide	IV
10	1	PAH, CREST syndrome	71	M	DOE	Nifedipine, furosemide	II
11	1	PAH, systemic lupus erythematosus	38	F	DOE, leg oedema	Warfarin, furosemide, solumedrol	III
12	1	PAH, systemic lupus erythematosus	25	F	DOE	Warfarin, solumedrol, endoxan	II
13	2	PAH, secundum ASD	27	F	DOE, cyanosis	Nifedipine, warfarin, digoxin	III
14	2	PAH, secundum ASD	32	F	DOE, cyanosis	Warfarin	II
15	2	PAH, ASD repair at 7 years	17	F	DOE, cyanosis	Furosemide	III
16	2	PAH, VSD repair at 7 years, ASD	26	M	DOE, cyanosis, syncope, paroxysmal atrial flutter	Warfarin, digoxin, furosemide, amiodarone	III
17	2	PAH, arterial switch at 2 weeks	8	M	Mild DOE	None	II
18	2	PAH, PDA closure at 6 years	17	F	DOE, chest pain	Nifedipine, warfarin	III
19	2	PAH, PDA closure at 15 years	46	F	DOE, chest pain	Nifedipine, warfarin	IV
20	2	PAH, ASD repair at 45 years	46	M	DOE, oedema	Nifedipine, warfarin, digoxin, furosemide	III
21	3	PAH, double inlet single left ventricle	12	F	DOE, cyanosis	None	II
22	3	PAH, double inlet single left ventricle	5	F	DOE, cyanosis	Furosemide, digoxin, captopril	II
23	3	PAH, double outlet right ventricle	39	M	DOE, cyanosis	Furosemide, aspirin	II
24	3	PAH, truncus arteriosus, restrictive ASD	4	M	DOE, cyanosis	Digoxin, furosemide	II
25	3	PAH, aortopulmonary window	29	F	DOE	None	II
26	3	PAH, aortopulmonary window	12	M	DOE, cyanosis	None	II