Table 1:
Baseline Clinical Indices (n=4,977)
| Age, years | 35 (24–47) |
| Male sex | 2,589 (52%) |
| Severe CHD | 2,080 (42%) |
| Non-severe CHD | 2,897 (58%) |
| Congenital heart lesions | |
| *Coarctation of aorta | 805 (16%) |
| **Tetralogy of Fallot | 744 (15%) |
| Ebstein anomaly | 683 (14%) |
| ***Congenital aortic stenosis (isolated) | 639 (13%) |
| Fontan palliation | 367 (7%) |
| Atrioventricular canal defect | 304 (6%) |
| ASD/PAPVR (repaired) | 281 (6%) |
| Valvular pulmonic stenosis | 240 (5%) |
| Congenitally corrected TGA | 205 (4%) |
| TGA status post atrial switch operation | 179 (4%) |
| TGA status post arterial switch operation | 166 (3%) |
| Subaortic stenosis (isolated) | 130 (3%) |
| Eisenmenger syndrome/unrepaired single ventricle | 75 (2%) |
| Pulmonary atresia-intact ventricular septum | 43 (0.9%) |
| Truncus arteriosus | 40 (0.8%) |
| Double chambered RV | 33 (0.6%) |
| ASD/APVR (unrepaired) | 24 (0.4%) |
| Others | 19 (0.9%) |
| Comorbidities | |
| Hypertension | 1,216 (24%) |
| Hyperlipidemia | 1,027 (21%) |
| Diabetes | 324 (6%) |
| Atrial fibrillation | 738 (15%) |
| Atrial flutter/tachycardia | 582 (12%) |
| Hepatitis B/C | 6 (0.1%) |
| Medications | |
| Beta blockers | 1,303 (26%) |
| ACEI/ARB | 1,280 (26%) |
| Spironolactone | 270 (6%) |
| Loop diuretics | 930 (19%) |
| Warfarin | 849 (17%) |
| Direct oral anticoagulant | 84 (2%) |
| Antiplatelet therapy | 1,136 (23%) |
| ¶ Echocardiography (n=4,610) | |
| RA reservoir strain, % | 36±11 |
| RV (non-systemic) global longitudinal strain | −21±5 |
| Estimated RA pressure, mmHg | 8±4 |
| ≥Moderate TR severity | 926 (20%) |
| Estimated RVSP, mmHg | 34 (28–46) |
| LA reservoir strain, % | 36±13 |
| LV cardiac index, L/min/m2 | 3.2 ±0.7 |
| LV ejection fraction, % | 61±8 |
| LV (systemic) global longitudinal strain, % | −21±3 |
TGA: Transposition of great arteries; ASD/PAPVR: atrial septal defect/partial anomalous pulmonary venous return; ACEI/ARB: angiotensin converting enzyme inhibitor/angiotensin-II receptor blocker; RA: Right atrium; RV: Right ventricle; RVSP: right ventricular systolic pressure; LV: Left ventricle; CHD: congenital heart disease
Coarctation of aorta included patients with isolated coarctation and those with associated left ventricular inflow and outflow disease
Tetralogy of Fallot includes the entire disease spectrum including pulmonary atresia and ventricular septal defect and absent pulmonary valve syndrome
Congenital aortic stenosis defined as having >moderate aortic stenosis prior to 18 years of age regardless of aortic valve morphology
Echocardiographic data presented in the tables were derive from all patients except those with Fontan palliation