Clinical features and survival in Takayasu’s arteritis-associated pulmonary hypertension: a nationwide study

Eur Heart JPublished Nationwide cohort study

Authors' conclusion

Patients with TA-PH were predominantly female and had severely compromised haemodynamics. More than 80% of patients in our cohort survived for at least 3 years.

Study context

Population
Patients with Takayasu's arteritis with pulmonary artery involvement and pulmonary hypertension, National Rare Diseases Registry System of China, 2007–201981% female
Participants
140 patients
Outcome
Time from TA-PH diagnosis to all-cause death
Methodological detailsregistry-based, multicentre
  • Registry-based
  • Multicentre

Main finding

As reported in the abstract

All-cause survival
Group1-year survival3-year survival5-year survival
TA-PH94.0%83.2%77.2%

TA-PH

1-year survival94.0%
3-year survival83.2%
5-year survival77.2%

Observational data: associations, not effects.

Further findings

Predictors of all-cause death

ComparisonAdjusted HR (95% CI)P value
Syncope5.38 (1.77–16.34)P = 0.003
NT-proBNP level1.04 (1.03–1.06)P < 0.001
Mean right atrial pressure1.07 (1.01–1.13)P = 0.015

NT-proBNP and haemodynamics with PAH-approved drugs

Significant improvements in NT-proBNP and haemodynamic profiles

Original publication

Clinical features and survival in Takayasu’s arteritis-associated pulmonary hypertension: a nationwide study. Eur Heart J. Published 10 September 2021. doi:10.1093/eurheartj/ehab599

Summarised from the published abstract; every number is checked against it

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