Background
Pulmonary arterial hypertension (PAH) has a progressive, unremitting clinical course.
Vasoreactivity testing (VdT) during right heart catheterisation (RHC) identifies a
subgroup with excellent long-term response to calcium channel blockade (CCB). Reporting
on these patients is limited. Established in 2011, the Pulmonary Hypertension Society
of Australia and New Zealand (PHSANZ) registry offers the opportunity to assess the
frequency of VdT during RHC, treatment and follow up of PAH patients.
Methods
Registry data from 3,972 PAH patients with index RHC revealed 1,194 VdT appropriate
patients. Data was analysed in three groups: 1) VdT+CCB+: VdT positive, CCB treated;
2) VdT+CCB-: VdT positive, no CCB prescribed, 3) VdT-/noVdT: VdT negative, or VdT
not tested. Data was reviewed for adherence to guidelines, clinical response (World
Health Organization functional class [WHO FC], 6-minute-walk-distance [6MWD], RHC),
and outcomes (survival or lung transplantation).
Results
Patients included had idiopathic (IPAH=1,087), heritable (HPAH=67) and drug or toxin-induced
PAH (DPAH=40). A VdT was performed in 22% (268/1,194), with incomplete data in 26%
(70/268); 28% (55/198) were VdT+. Analysis group allocation was: VdT+CCB+ (33/55),
VdT+CCB- (22/55), VdT- (143)/noVdT (996). From patients with 1-year data VdT+CCB+
and VdT-/noVdT patients improved WHO FC, 6MWD and cardiac index (CI); VdT+CCB- data
remained similar. Within the VdT+CCB+ group, 30% (10/33) were long-term CCB responders
with a 100% 5-year survival; non-responders had a 61% survival at 5.4 years. Long-term
responders were younger at diagnosis (40 yrs vs 54 yrs).
Conclusion
Use of VdT testing and documentation is poor in this contemporary patient cohort.
Nonetheless, survival in VdT+CCB+ patients from the PHSANZ registry is excellent,
supporting guidelines promoting VdT testing. Strategies to promote the use of VdT
are warranted.
Keywords
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Article info
Publication history
Published online: December 08, 2022
Accepted:
September 1,
2022
Received in revised form:
August 14,
2022
Received:
January 27,
2022
Identification
Copyright
© 2022 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.
