Neonatal Ebstein's Management - Annotation

How I Manage Neonatal Ebstein's Anomaly. Bove et al. (Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 2009;12:63-65)

Algorithm:

U Mich Newborn Experience:

1988 - 2008 (20 yrs).

n=40 consecutive pts.

No intervention - 16

Neonatal intervention - 24

Mean age at surgery 6 days (1-7 days)

Mean weight 3.2 kg (2.5 - 4.1 kg)

24 surgeries:

TV closure - 11

Shunt only - 9 > 2 TV closure (one 2 days after & another during Glenn)

TV repair 4

Overall, 6 hospital deaths - 3 in TV closure group, 3 in TV repair group.

18 early survivors - 4 late deaths in 7.2 yrs mean f-up.

14 late survivors - 7 have had Fontan.

Outcome by surgery:

1) Shunt only (n=9)

All survived hospital discharge.

1 needed ECMO, had TV closure 2 days later.

2 - Fontan

1 - late TV repair & RV-PA conduit

1 - 1.5 ventricle repair

1 - TV closure at the time of Glenn.

2 - late deaths (1 died after Glenn due to hypoxia and low cardiac output and 1 died at Fontan from ventricular dysfunction).

2 - lost to f-up.

2) TV repair (n=4)

3 hospital deaths, 1 survivor is doing well at 12.5 yrs.

3) TV closure (n=11)

3 hospital deaths.

1 late death at 3 months (sudden death; unknown cause)

Remaining 7 had Fontan.

Overall survival:

All neonatal surgeries:

66.7% at 1 yr

62.2% at 5, 10 yrs

51.9% at 15 yrs

TV closure:

63.6% at 1, 5, 10 yrs

47.7% at 15 yrs

Shunt only:

88.9% at 1 yr

76.2% at 5, 10 yrs

TV repair:

25% at 1, 5, 10, yrs.

Comments: Review article. Not randomized. No clear indication as to equal disease severity of Ebsteins who underwent different interventions. Therefore, this should not be viewed as comparison of surgeries for the same severity of disease. Each patient would have been evaluated and the surgical option applied depending on disease features and institutional experience.

Also see Indications for surgery in Ebstein Anomaly