Long term combination treatment for severe idiopathic pulmonary arterial hypertension

Table 1 Parameters at baseline (T0), after 1 year of therapy with tadalafil 40 mg/d (T1) and after 6 (T2) and 12 mo (T3) of a combination of tadalafil 40 mg/d plus sitaxentan 100 mg/d

	Patient 1				Patient 2				Patient 3
	F (74 yr)				F (44 yr)				F (45 yr)
RHC PAP S/M/D (mmHg)	97/52/31				95/53/34				116/68/44
RHC CI (L/min per m2)	2.25				2.00				2.94
RHC PVR (WU)	7.8				12.9				11.0
Timepoint	T0	T1	T2	T3	T0	T1	T2	T3	T0	T1	T2	T3
WHO functional class	III-IV	III	III	III	III-IV	II-III	II-III	IV	III	II	I	I
6MWT (m)	251	276	305	330	245	285	311	90	316	456	462	470
Borg score	8	8.5	8	7	5	7	6	9	2	0.5	0	0.5
SF-36	83	89	93	91	82	96	100	n/a	91	107	119	115
PAP (mmHg)	97	94	70	75	110	80	80	75	118	97	60	70
NT-proBNP (pg/mL)	989	951	785	654	2094	1650	1812	2834	503	172	106	102
TLCO-SB (%)	23	41	42	46	60	68	68	n/a	58	74	68	70
VO2max (mL/min per kilogram)	n/a	n/a	n/a	n/a	n/a	10.0	11.1	n/a	10.6	16.8	16.9	15.3
FMD (%)	6.6	7.3	7.6	n/a	6.6	11.2	12.6	n/a	7.35	13.3	14.3	n/a

RHC: Right heart catheterization data at baseline; PAP: Pulmonary artery pressure (systolic/diastolic/mean); CI: Cardiac index; PVR: Pulmonary vascular resistance; WHO: World Health Organization; 6MWT: 6 min walking test; SF-36: Short Form-36 questionnaire; PAP: Doppler estimate of systolic pulmonary arterial pressure; NT-proBNP: N-terminal pro-brain natriuretic peptide; TLCO-SB: Transfer factor of the lung for carbon monoxide; VO2max: Peak oxygen consumption on cardiopulmonary exercise testing; FMD: Flow mediated dilation.