Table 1.
Demographic and Clinical Characteristics
| All Patients (n=215) | Transplant Free Survival (n=191) | Death or Transplant (n=24) | P Value | |
|---|---|---|---|---|
| Gender (male) | 136 (63%) | 119 (62%) | 17 (71%) | 0.50 |
| Age at Fontan (years) | 3.6 [2.3, 7.1] | 3.5 [2.3, 6.6] | 4.2 [2.3, 13.0] | 0.31 |
| Age at CMR (years) | 18.3 [14, 26] | 18.3 [14, 26] | 18.1 [8, 33] | 0.98 |
| Time from Fontan to CMR (years) | 14.6 [10, 19] | 14.7 [10, 19] | 13.4 [5, 21] | 0.65 |
| Follow-up time after CMR (years) | 4.1 [2.6, 6.2] | 4.3 [2.7, 6.4] | 2.5 [1.1, 4.5] | <0.001 |
| Body surface area at CMR (m2) | 1.6 [1.4, 1.9] | 1.6 [1.4, 1.9] | 1.7 [1.0, 2.0] | 0.93 |
| Cardiac Diagnosis | 0.27 | |||
| Tricuspid atresia | 52 (24%) | 45 (24%) | 7 (29%) | |
| Double-inlet left ventricle | 36 (17%) | 35 (18%) | 1 (4%) | |
| HLHS | 34 (16%) | 26 (14%) | 8 (34%) | |
| Unbalanced AV canal | 21 (10%) | 18 (9%) | 3 (13%) | |
| Double-outlet right ventricle | 20 (9%) | 19 (10%) | 1 (4%) | |
| Complex 2 ventricle | 17 (8%) | 16 (8%) | 1 (4%) | |
| Hypoplastic TV/RV | 16 (7%) | 15 (8%) | 1 (4%) | |
| Pulmonary atresia/IVS | 10 (5%) | 9 (5%) | 1 (4%) | |
| Mitral atresia | 9 (4%) | 8 (4%) | 1 (4%) | |
| Heterotaxy | 20 (9%) | 17 (9%) | 3 (13%) | 0.47 |
| Ventricular morphology | 0.10 | |||
| Left ventricle | 96 (45%) | 90 (47%) | 6 (25%) | |
| Right ventricle | 66 (30%) | 55 (29%) | 11 (46%) | |
| Biventricular | 53 (25%) | 46 (24%) | 7 (29%) | |
| Surgical history | ||||
| Neonatal surgery | 108 (50%) | 99 (52%) | 9 (38%) | 0.20 |
| Bidirectional Glenn | 112 (52%) | 97 (51%) | 15 (63%) | 0.39 |
| Fontan revision (post CMR) | 19 (9%) | 15 (8%) | 4 (17%) | 0.24 |
| Fontan Type | 0.001 | |||
| Lateral tunnel | 151 (70%) | 140 (73%) | 11 (46%) | |
| RA-Pulmonary artery | 44 (21%) | 38 (20%) | 6 (25%) | |
| Extracardiac | 11 (5%) | 8 (4%) | 3 (12%) | |
| RA-RV | 9 (4%) | 5 (3%) | 4 (17%) | |
| Morbidity | ||||
| Liver disease | 34 (16%) | 25 (13%) | 9 (38%) | 0.005 |
| Congestive heart failure | 60 (28%) | 41 (22%) | 19 (79%) | <0.001 |
| Thrombus | 32 (15%) | 24 (13%) | 8 (33%) | 0.01 |
| Seizures | 22 (10%) | 19 (10%) | 3 (13%) | 0.72 |
| Stroke | 41 (19%) | 35 (18%) | 6 (25%) | 0.42 |
| Protein losing enteropathy | 14 (7%) | 6 (3%) | 8 (33%) | <0.001 |
| Atrial flutter/fibrillation | 65 (30%) | 53 (28%) | 12 (50%) | 0.03 |
| Nonsustained VT | 35 (16%) | 28 (15%) | 7 (29%) | 0.08 |
| Sustained VT | 8 (4%) | 5 (3%) | 3 (13%) | 0.05 |
| Resuscitated cardiac arrest | 2 (1%) | 1 (1%) | 1 (4%) | 0.21 |
| Defibrillator (post CMR) | 4 (2%) | 3 (2%) | 1 (4%) | 0.38 |
Values are expressed as n (%) or median [25th, 75th percentile].
Abbreviations: CMR = cardiac magnetic resonance; HLHS = hypoplastic left heart syndrome; IVS = intact ventricular septum; RA = right atrium; RV = right ventricle; TV = tricuspid valve; VT = ventricular tachycardia.