Multiple left heart obstructions (Shone's anomaly) with mitral valve involvement: long-term surgical outcome.
BACKGROUND
The outcome of children with multilevel left heart obstructions (Shone's anomaly) is generally poor. Literature is scarce, consisting mainly of case reports. The mitral disease may be the predominant factor affecting outcome.


METHODS
Surgical results in 19 consecutive patients are presented, with a median follow-up of 8 years. Mitral stenosis was present in all, with parachute deformity in 12 patients. Supramitral rings were found in 9 patients. Other features included subaortic stenosis (15 patients), valvar aortic stenosis (9), bicuspid aortic valve (16), and coarctation (13 patients). The patients underwent 46 surgical procedures, including 18 mitral operations (9 replacements, 9 repairs).


RESULTS
There were three in-hospital (16%) and two late (10.5%) deaths. Of the 5 nonsurvivors, 4 patients (80%) had predominant mitral disease and moderate to severe pulmonary hypertension, versus 4 (28.5%) and 5 (36%) survivors, respectively (p = not significant). Valve repair was the final procedure in 9 survivors. The other 5 patients had repeated valve replacements (1), aortoventriculoplasty with valve replacements (2), or no mitral operation (2). Freedom from mitral reoperation was 78% (7 of 9 patients) after repair procedures and 43% (3 of 7 patients) after replacement. At follow-up, 10 patients (71.4%) are in New York Heart Association functional class I and the other 4 in class II and III. Six (43%) await reoperation due to recurrent aortic (4) or subaortic (1) stenosis and recoarctation (2). Echocardiography reveals mild mitral stenosis or regurgitation in 3 patients after repair (33%). Four are considered free of residual disease (21% of all).


CONCLUSIONS
Late outcome in Shone's anomaly seems to correlate with the predominance of mitral valve involvement and the degree of pulmonary hypertension. Valve repair is indicated whenever feasible and should be considered before the occurrence of pulmonary hypertension.
Brauner
R A
RA
Division of Cardiothoracic Surgery, UCLA School of Medicine 90095, USA.
Laks
H
H
Drinkwater
D C
DC
Scholl
F
F
McCaffery
S
S
D001017:Aortic Coarctation / Q000601:surgery; D001021:Aortic Valve / Q000002:abnormalities / Q000601:surgery; D001024:Aortic Valve Stenosis / Q000151:congenital / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D015150:Echocardiography, Doppler; D005240:Feasibility Studies; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000601:surgery*; D006350:Heart Valve Prosthesis; D006352:Heart Ventricles / Q000601:surgery; D017052:Hospital Mortality; D006801:Humans; D006976:Hypertension, Pulmonary / Q000601:surgery; D007223:Infant; D007231:Infant, Newborn; D008137:Longitudinal Studies; D008297:Male; D008943:Mitral Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D008946:Mitral Valve Stenosis / Q000151:congenital / Q000601:surgery; D012008:Recurrence; D012086:Reoperation; D015996:Survival Rate; D016896:Treatment Outcome; D014694:Ventricular Outflow Obstruction / Q000601:surgery