Table 2

Clinical outcomes of unrepaired Ebstein’s anomaly patients at median 3.4 (range 0.4–10.9) years follow-up

All cardiac events during follow-upPatients
Death (sudden in all), n (%)*4 (5%)
Cardiac transplantation1
Congestive heart failure, n (%)†3 (4%)
New-onset VT after CMR, n (%)12 (15%)
 Sustained VT, n3
 Non-sustained VT, n9
First-onset atrial tachyarrhythmia after CMR, n (%)17 (22%)
 Atrial tachycardia10
 Atrial fibrillation7
  • Patients experiencing death/sustained VT/heart failure/cardiac transplantation; NYHA class ≥3 n=3, chronic atrial fibrillation n=2, accessory pathway n=3, QRS duration ≥120 ms n=5, exercise intolerance n=2, decreased right ventricular ejection fraction n=6, decreased left ventricular ejection fraction n=4.

  • Following a MACE, three patients went on for tricuspid valve surgery, one for cardiac transplantation.

  • *Two confirmed cardiac deaths, two unknown causes but sudden.

  • †One patient required hospital admission for diuresis and two patients deteriorated to NYHA class 4.

  • CMR, cardiovascular magnetic resonance; MACE, major adverse cardiovascular event; NYHA, New York Heart Association; VT, ventricular tachycardia.