Table 2.
Fontan Clinical Characteristics
| Variable | Value |
|---|---|
| Anatomy, n (%) | |
| HLHS | 14 (33) |
| Tricuspid atresia | 7 (16) |
| Unbalanced AV canal defect | 7 (16) |
| Other single ventricle variants | 7 (16) |
| DORV | 6 (14) |
| PA/IVS | 2 (5) |
| Heterotaxy syndrome, n (%) | 4 (9) |
| Ventricular morphology, n (%) | |
| RV | 18 (42) |
| LV | 15 (35) |
| Mixed | 10 (23) |
| Type of Fontan, n (%) | |
| Extracardiac conduit | 25 (58) |
| Lateral tunnel | 18 (42) |
| Fenestration, n (%)a | 35 (81) |
| Medications, n (%) | |
| Aspirin | 40 (93) |
| ACEI | 19 (44) |
| Sildenafil | 3 (7) |
| Supplements, n (%) | |
| Calcium | 9 (21) |
| Vitamin D | 20 (47) |
| Laboratory values | |
| Albumin (g/dL) | 4.7 (3.4–5.3) |
| Ionized calcium | 1.22 (1.08–1.31) |
| Intact PTH (pg/mL) | 47.8 (12.3–140) |
| 25 (OH) Vitamin D (ng/mL) | 29.9 (4.5–64.2) |
| BNP (pg/mL) | 18.1 (10–246.8) |
| IGF-1 Z-score | 0.06 (−2.72 to 3.00) |
| Echocardiographic assessment, n (%)b | |
| Normal or low normal ventricular function | 29 (78) |
| Absent or mild AV valve regurgitation | 32 (86) |
| Indexed systemic flow on CMR, (L/min/m2)c | 2.3 (1.9–3.3) |
Results expressed as median (range) or n (%).
a
at time of initial operation;
b
N=37;
c
N=19
HLHS, hypoplastic left heart syndrome; AV, atrioventricular valve; DORV, double outlet right ventricle; PA/IVS, pulmonary atresia/intact ventricular septum; RV, right ventricle; LV, left ventricle; ACEI, angiotensin-converting enzyme inhibitor; PTH, parathyroid hormone; BNP, brain type natriuretic peptide; IGF-1, insulin-like growth factor-1; CMR, cardiac magnetic resonance; L/min/m2, liters per minute per meters squared