Mitral stenosis and aortic atresia in hypoplastic left heart syndrome: survival analysis after stage I palliation.
BACKGROUND
Mitral stenosis with aortic atresia (MS-AA) has been implicated as a risk factor for decreased survival after stage 1 palliation for hypoplastic left heart syndrome. Conflicting results were reported in the literature evaluating the association of anatomic subtypes and mortality. Our objective was to determine whether MS-AA is associated with increased mortality after stage 1 palliation.


METHODS
Between January 2005 and May 2009 100 consecutive neonates with hypoplastic left heart syndrome underwent stage 1 palliation. Echocardiograms were reviewed for patency of the mitral and aortic valves as well as presence of ventriculocoronary connections (VCC). Patients were divided into (1) mitral and aortic atresia (MA-AA), (2) MS-AA, and (3) mitral and aortic stenosis groups. Survival analysis was performed, and impact of MS-AA and VCC on early and midterm survival was assessed.


RESULTS
Of the 100 patients, 31 had MA-AA, 42 had mitral stenosis and aortic stenosis, and 27 had MS-AA (15 with VCC and 12 without). Stage 1 palliation 30-day survival was 90% for the entire cohort and 88.9% for the MS-AA subtype (not significantly different). Six-month survival was 70% overall and 70.4% for MS-AA (not significantly different). When VCC was taken into account, MS-AA with VCC reached 93% survival (versus 50% for MS-AA without VCC; p < 0.01).


CONCLUSIONS
Stage 1 palliation for hypoplastic left heart syndrome is associated with satisfactory hospital and midterm survival regardless of anatomic subtype. The variants MS-AA and MA-AA are associated with smaller ascending aorta. Mitral stenosis with aortic atresia or VCC did not adversely influence survival.
Division of Pediatric Cardiovascular Surgery, The Heart Institute for Children at Advocate Hope Children's Hospital, Oak Lawn, Illinois, USA.
Shyam K
SK
Sathanandam
Anastasios C
AC
Polimenakos
David A
DA
Roberson
Chawki F
CF
elZein
Andrew
A
Van Bergen
Tarek S
TS
Husayni
Michel N
MN
Ilbawi
D001021:Aortic Valve / Q000002:abnormalities*; D006348:Cardiac Surgical Procedures; D005260:Female; D017052:Hospital Mortality; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000401:mortality / Q000601:surgery*; D007231:Infant, Newborn; D008297:Male; D008946:Mitral Valve Stenosis / Q000401:mortality*; D010166:Palliative Care