TABLE 2.

COMPARISON OF RIGHT HEART CATHETERIZATION REPORTS OF PULMONARY HYPERTENSION AMONG PATIENTS WITH SICKLE CELL DISEASE

	Castro et al., 2003 (71)	Anthi et al., 2007 (57)	Parent et al., 2011 (77)	Fonseca et al., 2012 (81)	Mehari et al., 2012 (80)
No. subjects screened	34	43	445	80	531
Exclusion criteria	None	None	Renal insufficiency	None	None
			Restrictive lung disease
			Liver disease
Criteria for RHC	Clinical suspicion*	TRV ≥ 2.5 m/s	TRV ≥ 2.5 m/s	TRV ≥ 2.5 m/s	TRV ≥ 2.5 m/s and/or clinical suspicion
Number RHCs	39†	35	96	26	84
Number of subjects with PH‡	20	26	24	8	55
% RHC with PH	Not reported	74	25	31	65.5
% of population screened	Not reported	Not reported	6	10	10.5
Mean age of subjects with PH	37	43	45	46	41
mPAP, mm Hg	36 ± 8	37 ± 1.5	30 ± 6	33 ± 9	Not reported
PVR, dyn⋅s/cm5	271 ± 90§	206 ± 23	138 ± 58	179 ± 120‖	Not reported
CO, L/min	7 ± 2§	9 ± 0.5	9 ± 2	5 ± 2¶	Not reported
6MWD, m	Not reported	320 ± 20	404 ± 94	460 ± 152	358 ± 113
Duration of follow-up, yr	1.9**	Not reported	2.2	2.8††	4.4**
Mortality in PH group	55%	Not reported	23%	38%	37%

Definition of abbreviations: 6MWD = 6-min walk distance; CO = cardiac output; mPAP = mean pulmonary artery pressure; PH = pulmonary hypertension; PVR = pulmonary vascular resistance; RHC = right heart catheterization.

^*Clinical suspicion reported as: increased intensity of second heart sound in pulmonic area, right side cardiac chamber enlargement by chest X-ray or electrocardiogram, or abnormal echocardiography.

^†Five of 34 patients had one or more follow-up procedures.

^‡Pulmonary hypertension defined as mPAP ≥ 25 mm Hg.

^§Data only reported for subgroup of eight patients.

^‖Converted mean value: PVR measured in Wood units as 2.24 ± 1.5.

^¶Cardiac index (L/min/m²).

^**Median.

^††Estimated from figure in Reference 81.