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Arnott C, Boehm C, Lau E, et al. Survival outcomes in severe congenital versus non-congenital pulmonary hypertension. Heart Asia. 2016;8(1):3-7doi: 10.1136/heartasia-2015-010702.
Arnott, C., Boehm, C., Lau, E., & Celermajer, D. S. (2016). Survival outcomes in severe congenital versus non-congenital pulmonary hypertension. Heart Asia, 8(1), 3-7. https://doi.org/10.1136/heartasia-2015-010702
Arnott, Clare, et al. "Survival outcomes in severe congenital versus non-congenital pulmonary hypertension." Heart Asia vol. 8,1 (2016): 3-7. doi: https://doi.org/10.1136/heartasia-2015-010702
Arnott C, Boehm C, Lau E, Celermajer DS. Survival outcomes in severe congenital versus non-congenital pulmonary hypertension. Heart Asia. 2016 Jan 19;8(1):3-7. doi: 10.1136/heartasia-2015-010702. eCollection 2016. PMID: 27326222; PMCID: PMC4898627.
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Heart Asia. 2016 Jan 19;8(1):3-7. doi: 10.1136/heartasia-2015-010702. eCollection 2016.

Survival outcomes in severe congenital versus non-congenital pulmonary hypertension.

Heart Asia

Clare Arnott, Christiane Boehm, Edmund Lau, David S Celermajer

Affiliations

  1. Department of Cardiology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia; Faculty of Medicine, University of Sydney, Camperdown, New South Wales, Australia.
  2. Department of Cardiology , Royal Prince Alfred Hospital , Camperdown, New South Wales , Australia.
  3. Department of Respiratory , Royal Prince Alfred Hospital , Camperdown, New South Wales , Australia.

PMID: 27326222 PMCID: PMC4898627 DOI: 10.1136/heartasia-2015-010702

Abstract

OBJECTIVE: Since 2006, our institution has cared for 70 patients with pulmonary arterial hypertension (PAH) and severe right ventricular (RV) hypertension; 44 with congenital heart disease (CHD) had Eisenmenger syndrome (ES) and 26 had PAH without CHD. We sought to determine and compare 'real-world' survival outcomes and cause of death in these two distinct groups.

METHODS: An observational study of consecutive adult patients from our CHD or PAH clinics with RV systolic pressure ≥80 mm Hg (on echocardiography or right-heart catheter). Detailed patient demographics, investigations and outcomes at baseline and follow-up were reviewed.

RESULTS: Patients with ES were younger than non-CHD group (39±9 vs 64±14 years, p<0.001) with a lower proportion of women (61% vs 85%; p=0.04), and higher RV systolic pressure. Estimated 1-year, 3-year and 7-year survival were not significantly different between the groups (98%, 95% and 74% ES; 100%, 92% and 63% non-CHD, p=0.52). In patients with non-CHD, poorer survival was associated with a diagnosis of connective tissue disease (HR 6.90, 95% CI 1.21 to 39.3) and number of PAH therapies (HR 2.8, 95% CI 1.03 to 7.59). Mortality was directly attributed to PAH in 75% of non-CHD group compared with 31% in patients with ES (p=0.049), many of whom died from infection or bleeding.

CONCLUSIONS: We report favourable 7-year survival in adults with PAH and systemic RV pressures-equivalent in ES and non-CHD groups. Those with non-CHD predominately died of cardiac complications of PAH, as distinct from those with ES, many of whom died from complications of chronic cyanosis.

Keywords: CONGENITAL HEART DISEASE

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