Trisomy-13 and 18 - Cardiac Surgery

Effectiveness of Cardiac Surgery in Trisomies 13 and 18 (From the Pediatric Cardiac Care Consortium).

Eric Graham, Scott Bradley, Girish Shirali, Christine Hills and Andrew Atz.
Am J Cardiol 2004;93:801-3

1982-2000.
Multicenter Pediatric Cardiac Care Consortium - Cardiac Registry
70 were identified, only 35 had genetic confirmation.
Study group: n=35

5 cardiac diagnostic categories (based on the most-significant lesion):
VSD - 20
TOF - 6
Coarctation - 4
PDA - 3
AVSD - 2.

Weight range: 2.1 - 16 kg (Median 3.7 kg).
Gender: 14/21
Age at surgery: 4 - 2479 days (Median 128 days)
Hospital stay: 3 - 48 days (Median 10 days)
Hospital survival: 32 (91%)

Morbidity: Among 26 patients who were either intubated for surgery or intubated for less than 2 days prior to surgery: Among 9 patients who were intubated for > 2 days prior to surgery, 3 were extubated prior to discharge. One died. 5 were still intubated at discharge.

Follow-up: None, after hospital discharge.

Justification for surgery: To improve quality of life...was not addressed in this study.

Other references:
Japan: Am J Med Genet 2008;146A(11):1372-80 (& Editorial on p.1369-71)
Gp A - 2000-2: Offered no treatment (n=13)
Gp B - 2002-3: Offered pharmacologic treatment for PDA only (n-9)
Gp C - After Nov 2003 - 2005: Pharmacologic and Surgical treatment offered (n=9)

Median length of survival:
Gp A - 7 days

Gp B - 24 days

Gp C - 243 days

Utah: Am J Med Genet 2006;140:937-44 & 1994;49:175-88