Table 1.
Baseline characteristics.
| PLE group (n = 19) | Control group (n = 190) | p | |
|---|---|---|---|
| Age, years | 54 ± 11 | 38 ± 9 | <0.001 |
| Female | 12 (63%) | 92 (48%) | 0.2 |
| Systemic left ventricle | 16 (84%) | 160 (84%) | 0.9 |
| Body mass index, kg/m2 | 24.4 ± 2.1 | 29.7 ± 2.6 | <0.001 |
| CHD Diagnosis | |||
| Tetralogy of Fallot | 7 (37%) | 70 (37%) | 0.9 |
| Ebstein anomaly | 1 (5%) | 10 (5%) | 0.9 |
| Congenitally corrected TGA | 1 (5%) | 10 (5%) | 0.9 |
| d-TGA s/p atrial switch operation | 2 (11%) | 20 (11%) | 0.9 |
| Pulmonic stenosis | 2 (11%) | 20 (11%) | 0.9 |
| Atrioventricular canal defect | 3 (16%) | 30 (16%) | 0.9 |
| Atrial septal defect | 1 (5%) | 10 (5%) | 0.9 |
| Palliated/unrepaired cyanotic heart disease | 2 (11%) | 20 (11%) | 0.9 |
| Comorbidities | |||
| Hypertension | 6 (32%) | 58 (31%) | 0.8 |
| Coronary artery disease | 2 (11%) | 11 (6%) | 0.7 |
| Atrial fibrillation/flutter | 9 (47%) | 15 (8%) | <0.001 |
| Laboratory data | |||
| Stool α-1 antitrypsin | 446 (291–502) | – | |
| eGFR, ml/min/1.73 m [2] | 63 (48–94) | 92 (72–121) | <0.001 |
| NT-proBNP, pg/ml | 2611 (988-5942) | 214 (88–615) | <0.001 |
| MELD-XI score | 12.4 (10.9–16.3) | 9.8 (9.4–10.9) | <0.001 |
| Total protein, mg/dl | 4.9 ± 2.4 | 6.8 ± 1.7 | <0.001 |
| Albumin, mg/dl | 2.8 ± 1.9 | 4.5 ± 0.8 | 0.007 |
| Echocardiogram | |||
| Systemic LV ejection fraction, % | 56 ± 8 | 62 ± 11 | 0.6 |
| Systemic RV fractional area change, % | 33 ± 4 | 38 ± 7 | 0.7 |
| Systemic AVVR | 2 (11%) | 8 (4%) | 0.5 |
| Non-systemic RV fractional area change, % | 31 ± 9 | 44 ± 8 | 0.02 |
| Non-systemic LV ejection fraction, % | 43 ± 5 | 49 ± 11 | 0.4 |
| Non-systemic AVVR | 9 (47%) | 11 (6%) | <0.001 |
| RA volume index, ml/m2 | 38 ± 11 | 22 ± 18 | <0.001 |
| Estimated RA pressure, mmHg | 15 (10–20) | 5 (5–10) | <0.001 |
| Non-systemic ventricular systolic pressure, mmHg | 59 ± 14 | 33 ± 7 | <0.001 |
Abbreviations: AVVR: Atrioventricular valve regurgitation; CHD: Congenital heart disease; eGFR: Estimated glomerular filtration rate; LV: Left ventricle; MELD-XI: Model for end-stage liver disease excluding international normalized ratio; NYHA: New York Heart Association; PLE: Protein-losing enteropathy; RA: Right atrium; RV: Right ventricle; TGA: Transposition of great arteries.