UNIVENTRICULAR HEART WITH SYSTEMIC OUTFLOW OBSTRUCTION - PALLIATION BY PRIMARY DAMUS PROCEDURE

In 24 consecutive infants (19 male and 5 female) with complex forms of single-ventricle physiology and systemic outflow obstruction, a modif ied Damus operation without the use of exogenous material was undertak en in conjunction with creation of an aortopulmonary shunt 3.5 mm in d iameter. The median age at operation was 6 days (range, 1 to 170 days) and the median weight, 3.4 kg (range, 2.6 to 4.6 kg). There were nine early deaths. All 15 survivors (median follow-up, 6.5 months) were cl inically well without major systemic ventricular dysfunction or atriov entricular or arterial valve regurgitation. Ten of them have undergone a superior vena cava-pulmonary shunt (one death), and 1 has required patch angioplasty of the aortic arch and innominate artery with revisi on of the aortopulmonary shunt The 4 other survivors are awaiting a ca vopulmonary shunt. Univariate analysis yielded the chronologic rank fo r an individual procedure (higher risk of death early in the series), presence of aortic arch atresia, and presence or absence of transposit ion of the great arteries as predictors of death. This aggressive surg ical approach provides excellent early palliation, and because the ope ration prevents abnormal ventricular hypertrophy from pressure or volu me overload, systemic ventricular function is optimally conserved for a future Fontan-type procedure.