In one of the most severe congenital heart defects, hypoplastic left heart syndrome (HLHS), the left ventricle is underdeveloped and the prognosis is worse than in most other heart defects. The underdevelopment can occur gradually during fetal growth caused by a narrowing of the aortic valve. At some international centers, such fetuses are treated with a balloon dilation of the narrowed valve, but there is no scientifically sound evidence that this treatment is effective. The aim of this study is: 1/ to evaluate whether balloon dilation during the fetal period of a narrowed aortic valve can reduce the risk of the left ventricle becoming underdeveloped and the baby being born with a so-called univentricular heart (HLHS); 2/ to investigate whether such treatment improves the prognosis for this group of children with a very complex and severe heart defect and 3/ to also describe side effects and risks in fetuses and mothers of the fetal procedure.
Routinely collected pre- and postnatal clinical data will be entered into a digital database and echocardiographic examinations will be uploaded to a server. A core lab will measure and analyze all echocardiographic examinations according to protocol. The growth of the left heart structures and the postnatal outcome will be compared between the intervention and non-intervention groups. The decision whether a fetal balloon dilatation shall be attempted is not part of the study protocol. The number of examinations of mother/fetus/infant in this study is not different from the number of examinations that will be recommended for someone choosing not to be part of this study. Participation in the study does not affect the care and treatment mothers and fetuses are receiving during pregnancy, nor how the infant is examined and treated after birth.
Study Type
OBSERVATIONAL
Enrollment
200
Fetal valvuloplasty will be performed as described in Arzt W, Wertaschnigg D, Veit I, Klement F, Gitter R, Tulzer G. Intrauterine aortic valvuloplasty in fetuses with critical aortic stenosis: experience and results of 24 procedures. Ultrasound Obstet Gynecol. 2011;37:689-695. with minor variations between centers involved. Technical success is defined as improved forward flow and/or new aortic regurgitation.
Fetal Cardiovascular Program, University of California San Francisco
San Francisco, California, United States
RECRUITINGCongenital Heart Collaborative, Nationwide Children's Hospital
Columbus, Ohio, United States
RECRUITINGKinderherzzentrum Linz
Linz, Austria
RECRUITINGThe Hospital for Sick Children Toronto
Toronto, Canada
RECRUITINGDepartment of Paediatric Cardiology, Helsinki University Children's Hospital
Helsinki, Finland
RECRUITINGPediatric Cardiology - University Hospital Bonn
Bonn, Germany
RECRUITINGDepartment of Pediatric and Congenital Cardiology, University of Heidelberg
Heidelberg, Germany
RECRUITINGUniversity hospital Technical university, mother- and-child center
Munich, Germany
RECRUITINGDepartment of Perinatal Cardiology and Congenital Anomalies, Centre of Postgraduate Medical Education.
Warsaw, Poland
RECRUITINGFetal Medicine Unit, Dept. Obstetrics & Gynecology University Hospital 12 de Octubre
Madrid, Spain
RECRUITING...and 3 more locations
Transplantation-free survival
Transplantation-free survival from fetal diagnosis to 2 years postnatal age with a biventricular circulation without pulmonary hypertension at that time. Absence of pulmonary hypertension is defined as a TR max velocity ≤ 2.8 m/s with no other echocardiographic signs of pulmonary hypertension and/or catheter data showing a mean pulmonary arterial pressure \<25 mmHg.
Time frame: Follow-up from study inclusion during fetal life until 2 years postnatal age
Intervention-related fetal death
Intervention-related fetal death (defined as fetal death within 24 hours of procedure)
Time frame: From the time of fetal intervention until 24 hours after the intervention
Fetal death not directly related to the intervention
Fetal death not directly related to the intervention, except termination of pregnancy
Time frame: From 24 hours after fetal intervention until fetal death, up to 20 weeks after study inclusion.
Maternal complications to fetal intervention procedure
Maternal complications to procedure requiring intensive care or resulting in maternal death
Time frame: From time of fetal intervention until 24 hours after the intervention
Preterm delivery
Preterm delivery before 37 weeks gestational age
Time frame: From time of fetal intervention until 37 weeks gestational age, maximum 14 weeks
Fetal left heart growth
Fetal left heart growth as measured with dimensions expressed a z-scores of the mitral valve diameter, left ventricular inlet length and aortic valve diameter
Time frame: From the date of study inclusion until just before the first postnatal catheter or surgical intervention, or death, whichever comes first, total time frame 7 months
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