Abstract
Fetal aortic valvuloplasty (FAV) aims at modifying the progression of aortic stenosis evolving to hypoplastic left heart syndrome (Donofrio et al. Circulation 129(21):2183–2242, 2014). In the last decades, the observation of mid-term outcome of the patients that have been intervened prenatally has allowed further understanding of the disease and the effects of FAV, as well as of postnatal treatment of these patients. FAV is also being studied with animal models, but still with limited results. Given the small number of cases, multicenter studies are ongoing. We present a review of the most recently reported data and central aspects of our experience in the procedure.
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Donofrio MT, Moon-Grady AJ, Hornberger LK, et al. Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association. Circulation. 2014;129(21):2183–242.
•• Freud LR, McElhinney DB, Marshall AC, et al. Fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome postnatal outcomes of the first 100 patients. Prenat Diagn. 2014;31:695–8. This study reports the postnatal outcome, in terms of achievement of a biventricular circulation and of short- and intermediate-term survival, of the actual largest series of patients who have undergone attempted fetal aortic valvuloplasty. It is a crucial contribution to the understanding of the role of fetal cardiac therapy.
McElhinney DB, Marshall AC, Wilkins-Haug LE, et al. Predictors of technical success and postnatal biventricular outcome after in utero aortic valvuloplasty for aortic stenosis with evolving hypoplastic left heart syndrome. Circulation. 2009;120(15):1482–90.
Arzt W, Wertaschnigg D, Veit I, et al. Intrauterine aortic valvuloplasty in fetuses with critical aortic stenosis: experience and results of 24 procedures. Ultrasound Obstet Gynecol. 2011;37(6):689–95.
• Pedra SR, Peralta CF, Crema L, et al. Fetal interventions for congenital heart disease in Brazil. Pediatr Cardiol. 2014;35(3):399–405. Fetal aortic valvuloplasty was performed in 14 fetuses, with a high rate of technical success (93 %), supporting feasibility of fetal cardiac intervention. Fetuses with aortic stenosis and severe mitral regurgitation, and fetuses with HLHS have been included in this series.
Clouchoux C, du Plessis AJ, Bouyssi-Kobar M, et al. Delayed cortical development in fetuses with complex congenital heart disease. Cereb Cortex. 2013;23(12):2932–43.
McElhinney DB, Benson CB, Brown DW, et al. Cerebral blood flow characteristics and biometry in fetuses undergoing prenatal intervention for aortic stenosis with evolving hypoplastic left heart syndrome. Ultrasound Med Biol. 2010;36(1):29–37.
• Tulzer G1, Arzt W. Fetal cardiac interventions: rationale, risk and benefit. Semin Fetal Neonatal Med. 2013;18(5):298–301. In this review the authors focus on the rationale for intervention describing different kinds of pathophysiology that may develop in fetuses with aortic stenosis, to be considered in patient selection. They also highlight the importance of limiting fetal cardiac interventional programmes to few specialized centres.
• Marantz P, Aiello H, Grinenco S, et al. Foetal aortic valvuloplasty: experience of five cases. Cardiol Young. 2013;23(5):675–81. The initial experience of five fetal aortic valvuloplasties is reported in this study, describing the patients´ fetal and postnatal echocardiographic findings and their postnatal outcome. In this series there was no fetal mortality or maternal morbidity.
• Kalish BT1, Tworetzky W, Benson CB, et al. Technical challenges of atrial septal stent placement in fetuses with hypoplastic left heart syndrome and intact atrial septum. Catheter Cardiovasc Interv. 2014;84(1):77–85. This is the first report of fetal atrial septal stent placement attempted in fetuses with HLHS and intact atrial septum. It highlights the difficulties and challenges of this novel technique.
Vogel M, McElhinney DB, Wilkins-Haug LE, et al. Aortic stenosis and severe mitral regurgitation in the fetus resulting in giant left atrium and hydrops: pathophysiology, outcomes, and preliminary experience with pre-natal cardiac intervention. J Am Coll Cardiol. 2011;57(3):348–55.
Huhta J, Quintero RA, Suh E, et al. Advances in fetal cardiac intervention. Curr Opin Cardiol. 2004;29(2):140–4.
Wohlmuth C, Tulzer G, Arzt W, et al. Maternal aspects of fetal cardiac intervention. Ultrasound Obstet Gynecol. 2014;44(5):532–7.
• Edwards A, Menahem S, Veldman A, et al. Fetal cardiac catheterization using a percutaneous transhepatic access technique: preliminary experience in a lamb model. Ultrasound Obstet Gynecol. 2013;42(1):58–63. In this study percutaneous transhepatic cardiac catheterization performed in a fetal sheep model is reported. It constitutes a valuable contribution although further animal model studies are needed to improve safety and feasibility of the technique.
• Nugent AW, Kowal RC, Juraszek AL, et al. Model of magnetically guided fetal cardiac intervention: potential to avoid direct cardiac puncture. Fetal Neonatal Med. 2013;26(18):1778–81. Magnetic navigation was successfully used to maneuver a guidewire and balloon across a fetal aortic valve from abdominal venous access in a fetal heart model. Feasibility and safety remains to be tested in animal studies.
• Friedman KG, Schidlow D, Freud L, et al. Left ventricular diastolic function and characteristics in fetal aortic stenosis. Am J Cardiol. 2014;114(1):122–7. This study shows diastolic function impairment in fetuses with midgestation aortic stenosis. The incorporation of diastolic function parameters to actual patient selection criteria could help identifying better candidates for fetal cardiac intervention.
• Ishii T, McElhinney DB, Harrild DM, et al. Ventricular strain in fetuses with aortic stenosis and evolving hypoplastic left heart syndrome before and after prenatal aortic valvuloplasty. Diagn Ther. 2014;35(1):18–26. Ventricular function, assessed with speckle tracking technique, is reported to improve after fetal aortic valvuloplasty in a subset of patients, possibly contributing to postnatal outcome in those evolving with a biventricular circulation. These findings could help assess patients´ prognosis after fetal aortic valvuloplasty.
• Kovacevic A, Roughton M, Mellander M, et al. Fetal aortic valvuloplasty: investigating institutional bias in surgical decision making. Ultrasound Obstet Gynecol. 2014;44(5):538–44. This study describes the use of a blinded multidisciplinary team to simulate decision making, and the comparison with local teams´ choices in funnel plots as a tool to assist in interpretation of data on multicentre studies, and helpful to reveal confounding factors.
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Marantz, P., Grinenco, S. & Huhta, J. Fetal Valvuloplasty for Critical Aortic Stenosis. Curr Pediatr Rep 3, 255–258 (2015). https://doi.org/10.1007/s40124-015-0090-z
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DOI: https://doi.org/10.1007/s40124-015-0090-z