Table 1

Baseline characteristics of patients with severe PAH (n=19)

Age (years)31.5±9.1
Female, No (%)16 (84.2)
Body mass index, kg/m2*21.5±3.0
Aetiology of PAH, No (%)
 Idiopathic PAH10 (52.6)
 PAH related to repaired CHD9 (47.4)
Symptoms, No (%)
 Exertional dyspnoea19 (100)
 Syncope11 (57.9)
 Lower limb oedema14 (73.7)
WHO functional class, median (IQR)†3 (3, 4)
 III, No (%)11 (57.9)
 IV, No (%)8 (42.1)
6MWD (metre)252.9±98.3
NT-proBNP (pg/mL), median (IQR)†1478 (859, 2331)
Haemoglobin (g/dL)14.9±2.0
Cardiothoracic ratio (%)56.4±4.4
Electrocardiography
 Heart rate (beats/min)84.9±12.3
 Atrial tachycardia, No (%)2 (10.5)
 Paroxysmal atrial flutter, No (%)1 (5.3)
 First-degree atrioventricular block, No (%)1 (5.3)
TTE
 RAD (mm)56.0±7.9
 RVD (mm)43.4±6.4
 LAD (mm)28.1±4.0
 LVD (mm)33.2±4.7
 LVEF (%)65.4±4.2
 TAPSE (mm)17.7±3.1
 Tricuspid regurgitation (moderate/severe), No (%)14 (73.7)
 Pericardial effusion, No (%)8 (42.1)
Targeted medical therapy, No (%)
 Ambrisentan14 (73.7)
 Tadalafil16 (82.1)
 Bosentan3 (15.8)
 Sildenafil3 (15.8)
 Macitentan2 (10.5)
 Subcutaneous treprostinil5 (26.3)
History of balloon atrial septostomy, No (%)3 (15.8)
  • Values expressed as mean±SD for normally distributed variables, median (IQR) for non-normally distributed variables and No (%) for categorical variable.

  • *Calculated as weight in kilograms divided by height in meters squared.

  • †Non-normally distributed variables.

  • ‡Thirteen patients performed the 6-minute walk test before the procedure.

  • CHD, congenital heart disease; LAD, left atrial dimension; LVD, left ventricular dimension; LVEF, left ventricular ejection fraction; 6MWD, 6 min walk distance; NT-proBNP, N-terminal pro–brain natriuretic peptide; PAH, pulmonary arterial hypertension; RAD, right atrial dimension; RVD, right ventricular dimension; TAPSE, tricuspid annular plane systolic excursion; TTE, transthoracic Doppler echocardiography.