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.
Sathanandam
Shyam K
SK
Division of Pediatric Cardiovascular Surgery, The Heart Institute for Children at Advocate Hope Children's Hospital, Oak Lawn, Illinois, USA.
Polimenakos
Anastasios C
AC
Roberson
David A
DA
elZein
Chawki F
CF
Van Bergen
Andrew
A
Husayni
Tarek S
TS
Ilbawi
Michel N
MN
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