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Perinatal Outcomes after Fetal Diagnosis of Single Ventricle Cardiac Defects.
MedLine Citation:
PMID:  25042627     Owner:  NLM     Status:  Publisher    
OBJECTIVES: We investigated perinatal outcomes after fetal diagnosis of single ventricle cardiac defects. 'Single ventricle' was defined as a dominant RV or LV, in which biventricular circulation was not possible.
METHODS: We reviewed patients with a fetal diagnosis of single ventricle cardiac defect at one institution from 1995-2008. Diagnoses such as double-inlet left ventricle, tricuspid atresia, pulmonary atresia with intact ventricular septum and severe RV hypoplasia, and hypoplastic left heart syndrome (HLHS) were included. HLHS patients were prenatally identified as 'standard risk' and 'high risk' groups (HLHS with highly restrictive or intact atrial septum, mitral stenosis with aortic atresia, and/or LV coronary artery sinusoids). Patients with an address outside the U.S., heterotaxy syndrome, and referrals for fetal intervention were excluded.
RESULTS: We identified 312 prenatally diagnosed single ventricle cardiac defects (208 dominant RV; 104 dominant LV). Most (96%) of dominant RV patients had HLHS. There were 98 (31%) elective pregnancy terminations, 12 (4%) spontaneous fetal demises, 12 (4%) prenatal lost to follow-up and 190 (61%) live born. Of the 199 patients with a fetal echocardiogram at <24 weeks, there were 97 (49%) elective pregnancy terminations. There were no differences in prenatal outcome between dominant RV vs. dominant LV (p = 0.9). Of 190 live born infants, 5 received comfort care. With ~7 average years of follow-up through Fontan completion, there was lower transplant free survival in dominant RV versus dominant LV defects ('standard risk' HLHS odds ratio 3.0, p = 0.01; 'high risk' HLHS odds ratio 8.8, p < 0.001).
CONCLUSIONS: Whereas the prenatal outcomes of single ventricle cardiac defects were similar, postnatal intermediate-term survival favored those with dominant LV. Prenatally identified 'high risk' HLHS was associated with the lowest transplant free survival.
Rebecca S Beroukhim; Kimberlee Gauvreau; Oscar J Benavidez; Christopher W Baird; Terra LaFranchi; Wayne Tworetzky
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-7-17
Journal Detail:
Title:  Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology     Volume:  -     ISSN:  1469-0705     ISO Abbreviation:  Ultrasound Obstet Gynecol     Publication Date:  2014 Jul 
Date Detail:
Created Date:  2014-7-21     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9108340     Medline TA:  Ultrasound Obstet Gynecol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
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This article is protected by copyright. All rights reserved.
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