Article Text

Survival outcomes in severe congenital versus non-congenital pulmonary hypertension
  1. Clare Arnott1,2,
  2. Christiane Boehm1,
  3. Edmund Lau3,
  4. David S Celermajer1,2
  1. 1Department of Cardiology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia
  2. 2Faculty of Medicine, University of Sydney, Camperdown, New South Wales, Australia
  3. 3Department of Respiratory, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia
  4. Correspondence to Professor David S Celermajer, Department of Cardiology, Royal Prince Alfred Hospital, Missenden Road, Camperdown, NSW 2050, Australia; david.celermajer{at}


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.


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