Fetal aortic valvuloplasty for evolving HLHS

Circulation 2014;130:638-45

Fetal Aortic Valvuloplasty for Evolving Hypoplastic Left Heart Syndrome

Postnatal Outcomes of the First 100 Patients

Linday R. Freud et al. from Boston

Background—Fetal aortic valvuloplasty can be performed for severe midgestation aortic stenosis in an attempt to prevent progression to hypoplastic left heart syndrome (HLHS). A subset of patients has achieved a biventricular (BV) circulation after fetal aortic valvuloplasty. The postnatal outcomes and survival of the BV patients, in comparison with those managed as HLHS, have not been reported.

Methods and Results—We included 100 patients who underwent fetal aortic valvuloplasty for severe midgestation aortic stenosis with evolving HLHS from March 2000 to January 2013. Patients were categorized based on postnatal management as BV or HLHS. Clinical records were reviewed. Eighty-eight fetuses were live-born, and 38 had a BV circulation (31 from birth, 7 converted after initial univentricular palliation). Left-sided structures, namely aortic and mitral valve sizes and left ventricular volume, were significantly larger in the BV group at the time of birth (P<0 .01="" 10="" 5.4="" 5="" 84="" 96="" a="" after="" all="" among="" and="" at="" better="" bv="" cardiac="" death="" em="" follow-up="" freedom="" from="" hlhs="" log-rank="" median="" of="" patients="" than="" was="" which="" years="">P

=0.04). There was no cardiac mortality in patients with a BV circulation from birth. All but 1 of the BV patients required postnatal intervention; 42% underwent aortic or mitral valve replacement. On the most recent echocardiogram, the median left ventricular end-diastolic volume z score was +1.7 (range, −1.3 to +8.2), and 80% had normal ejection fraction.

Conclusions—Short- and intermediate-term survival among patients who underwent fetal aortic valvuloplasty and achieved a BV circulation postnatally is encouraging. However, morbidity still exists, and ongoing assessment is warranted.