Fetal Aortic Valvuloplasty for Evolving Hypoplastic Left Heart Syndrome
Top Cited Papers
- 19 August 2014
- journal article
- clinical trial
- Published by Ovid Technologies (Wolters Kluwer Health) in Circulation
- Vol. 130 (8), 638-645
- https://doi.org/10.1161/circulationaha.114.009032
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. 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 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.This publication has 37 references indexed in Scilit:
- Outcomes after transplantation for “failed” Fontan: A single-institution experienceThe Journal of Thoracic and Cardiovascular Surgery, 2012
- Hypoplastic Left Heart Syndrome: Current Considerations and ExpectationsJournal of the American College of Cardiology, 2012
- Postnatal Left Ventricular Diastolic Function After Fetal Aortic ValvuloplastyThe American Journal of Cardiology, 2011
- Arrhythmias in a Contemporary Fontan Cohort: Prevalence and Clinical Associations in a Multicenter Cross-Sectional StudyJournal of the American College of Cardiology, 2010
- Primary left ventricular rehabilitation is effective in maintaining two-ventricle physiology in the borderline left heartThe Journal of Thoracic and Cardiovascular Surgery, 2009
- Functional state following the Fontan procedureCardiology in the Young, 2009
- Staged Surgical Palliation in Hypoplastic Left Heart Syndrome and Its VariantsJournal of Cardiac Surgery, 2009
- Aortic valvuloplasty in the fetus: Technical characteristics of successful balloon dilationThe Journal of Pediatrics, 2005
- Hypoplastic left heart syndrome: Progression of left ventricular dilation and dysfunction to left ventricular hypoplasia in uteroAmerican Heart Journal, 1992
- The natural history of the hypoplastic left heart syndromeInternational Journal of Cardiology, 1989