Atrial septostomy and disease targeting therapy in pulmonary hypertension secondary to neurofibromatosis

BMC Pulmonary Medicine

Table 1 Serial hemodynamic, functional and neurohormonal measurements

Parameter	Jan 2011	PreBAS Mar 2014	PostBAS	Trepostinil Mar 2015	Trepostinil Feb 2016
HR (beats/min)	76	91	90	71	70
RAP (mmHg)	(3)	(11)	(7)	(4)	(6)
PAP (mmHg)	68/26 (43)	130/57 (84)	135/56 (84)	106/36 (63)	77/30 (49)
PAWP/LAP* (mmHg)	(6)	(2)*	(7)*	(7)	(9)*
Ao (mmHg)	130/68 (93)	122/65 (84)	124/65 (84)	119/71 (87)	128/65 (85)
PVR (WU)	8.3	27.1	26.3	11.6	6.8
CI (l/min/m²)	2.9	1.9	2.0	3.1	3.7
SaO2 (%)	97 at rest 90 (during exercise)	98	93	95 at rest (70 during exercise)	98 at rest (75 during exercise)
6MWT (m)	489	230^a	335^b	345	399
NT-proBNP (pg/ml)	125	3402^a	1507^b	NA	161

Mean pressures are shown in parentheses
BAS: balloon atrial septostomy, HR: heart rate, RAP: Right atrial pressure, PAP: Pulmonary artery pressure, LAP: Left atrial pressure, PCWP: Pulmonary capillary wedge pressure, Ao: Aortic pressure, PVR: Pulmonary vascular resistance, CI: Cardiac index, WU: Wood Units, 6MWT: 6-min walking test, NT-proBNP: N-terminal pro-brain natriuretic peptide, NA: not available
*Asterisk indicates that the pressure was measured in the left atrium
^a2 weeks prior to the procedure
^b1 month after the procedure

ISSN: 1471-2466