title: Fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome: postnatal outcomes of the first 100 patients.
abstract: 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.
  
  
  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). After a median follow-up of 5.4 years, freedom from cardiac death among all BV patients was 96±4% at 5 years and 84±12% at 10 years, which was better than HLHS patients (log-rank 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.
authors: From the Departments of Cardiology (L.R.F., D.B.M., A.C.M., G.R.M., K.G.F., T.L., J.E.L., W.T.) and Cardiac Surgery (P.J.d.N., S.M.E.), Boston Children's Hospital, Boston, MA; and the Departments of Obstetrics and Gynecology (V.S., L.E.W.-H.) and Radiology (C.B.B.), Brigham and Women's Hospital; Harvard Medical School, Boston, MA. lindsay.freud@cardio.chboston.org.
authors: Lindsay R
authors: LR
authors: Freud
authors: From the Departments of Cardiology (L.R.F., D.B.M., A.C.M., G.R.M., K.G.F., T.L., J.E.L., W.T.) and Cardiac Surgery (P.J.d.N., S.M.E.), Boston Children's Hospital, Boston, MA; and the Departments of Obstetrics and Gynecology (V.S., L.E.W.-H.) and Radiology (C.B.B.), Brigham and Women's Hospital; Harvard Medical School, Boston, MA.
authors: Doff B
authors: DB
authors: McElhinney
authors: From the Departments of Cardiology (L.R.F., D.B.M., A.C.M., G.R.M., K.G.F., T.L., J.E.L., W.T.) and Cardiac Surgery (P.J.d.N., S.M.E.), Boston Children's Hospital, Boston, MA; and the Departments of Obstetrics and Gynecology (V.S., L.E.W.-H.) and Radiology (C.B.B.), Brigham and Women's Hospital; Harvard Medical School, Boston, MA.
authors: Audrey C
authors: AC
authors: Marshall
authors: From the Departments of Cardiology (L.R.F., D.B.M., A.C.M., G.R.M., K.G.F., T.L., J.E.L., W.T.) and Cardiac Surgery (P.J.d.N., S.M.E.), Boston Children's Hospital, Boston, MA; and the Departments of Obstetrics and Gynecology (V.S., L.E.W.-H.) and Radiology (C.B.B.), Brigham and Women's Hospital; Harvard Medical School, Boston, MA.
authors: Gerald R
authors: GR
authors: Marx
authors: From the Departments of Cardiology (L.R.F., D.B.M., A.C.M., G.R.M., K.G.F., T.L., J.E.L., W.T.) and Cardiac Surgery (P.J.d.N., S.M.E.), Boston Children's Hospital, Boston, MA; and the Departments of Obstetrics and Gynecology (V.S., L.E.W.-H.) and Radiology (C.B.B.), Brigham and Women's Hospital; Harvard Medical School, Boston, MA.
authors: Kevin G
authors: KG
authors: Friedman
authors: From the Departments of Cardiology (L.R.F., D.B.M., A.C.M., G.R.M., K.G.F., T.L., J.E.L., W.T.) and Cardiac Surgery (P.J.d.N., S.M.E.), Boston Children's Hospital, Boston, MA; and the Departments of Obstetrics and Gynecology (V.S., L.E.W.-H.) and Radiology (C.B.B.), Brigham and Women's Hospital; Harvard Medical School, Boston, MA.
authors: Pedro J
authors: PJ
authors: del Nido
authors: From the Departments of Cardiology (L.R.F., D.B.M., A.C.M., G.R.M., K.G.F., T.L., J.E.L., W.T.) and Cardiac Surgery (P.J.d.N., S.M.E.), Boston Children's Hospital, Boston, MA; and the Departments of Obstetrics and Gynecology (V.S., L.E.W.-H.) and Radiology (C.B.B.), Brigham and Women's Hospital; Harvard Medical School, Boston, MA.
authors: Sitaram M
authors: SM
authors: Emani
authors: From the Departments of Cardiology (L.R.F., D.B.M., A.C.M., G.R.M., K.G.F., T.L., J.E.L., W.T.) and Cardiac Surgery (P.J.d.N., S.M.E.), Boston Children's Hospital, Boston, MA; and the Departments of Obstetrics and Gynecology (V.S., L.E.W.-H.) and Radiology (C.B.B.), Brigham and Women's Hospital; Harvard Medical School, Boston, MA.
authors: Terra
authors: T
authors: Lafranchi
authors: From the Departments of Cardiology (L.R.F., D.B.M., A.C.M., G.R.M., K.G.F., T.L., J.E.L., W.T.) and Cardiac Surgery (P.J.d.N., S.M.E.), Boston Children's Hospital, Boston, MA; and the Departments of Obstetrics and Gynecology (V.S., L.E.W.-H.) and Radiology (C.B.B.), Brigham and Women's Hospital; Harvard Medical School, Boston, MA.
authors: Virginia
authors: V
authors: Silva
authors: From the Departments of Cardiology (L.R.F., D.B.M., A.C.M., G.R.M., K.G.F., T.L., J.E.L., W.T.) and Cardiac Surgery (P.J.d.N., S.M.E.), Boston Children's Hospital, Boston, MA; and the Departments of Obstetrics and Gynecology (V.S., L.E.W.-H.) and Radiology (C.B.B.), Brigham and Women's Hospital; Harvard Medical School, Boston, MA.
authors: Louise E
authors: LE
authors: Wilkins-Haug
authors: From the Departments of Cardiology (L.R.F., D.B.M., A.C.M., G.R.M., K.G.F., T.L., J.E.L., W.T.) and Cardiac Surgery (P.J.d.N., S.M.E.), Boston Children's Hospital, Boston, MA; and the Departments of Obstetrics and Gynecology (V.S., L.E.W.-H.) and Radiology (C.B.B.), Brigham and Women's Hospital; Harvard Medical School, Boston, MA.
authors: Carol B
authors: CB
authors: Benson
authors: From the Departments of Cardiology (L.R.F., D.B.M., A.C.M., G.R.M., K.G.F., T.L., J.E.L., W.T.) and Cardiac Surgery (P.J.d.N., S.M.E.), Boston Children's Hospital, Boston, MA; and the Departments of Obstetrics and Gynecology (V.S., L.E.W.-H.) and Radiology (C.B.B.), Brigham and Women's Hospital; Harvard Medical School, Boston, MA.
authors: James E
authors: JE
authors: Lock
authors: From the Departments of Cardiology (L.R.F., D.B.M., A.C.M., G.R.M., K.G.F., T.L., J.E.L., W.T.) and Cardiac Surgery (P.J.d.N., S.M.E.), Boston Children's Hospital, Boston, MA; and the Departments of Obstetrics and Gynecology (V.S., L.E.W.-H.) and Radiology (C.B.B.), Brigham and Women's Hospital; Harvard Medical School, Boston, MA.
authors: Wayne
authors: W
authors: Tworetzky
mesh_terms: D001024:Aortic Valve Stenosis / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery*; D063126:Balloon Valvuloplasty / Q000379:methods*; D003326:Coronary Circulation; D018450:Disease Progression; D005260:Female; D005315:Fetal Diseases / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery*; D005500:Follow-Up Studies; D005865:Gestational Age; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000401:mortality / Q000517:prevention & control*; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D011247:Pregnancy; D011256:Pregnancy Outcome; D016216:Ultrasonography, Prenatal / Q000379:methods
keywords: aortic valve stenosis; balloon valvuloplasty; fetal heart; heart defects, congenital; hypoplastic left heart syndrome; pediatrics; treatment outcome