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      Fetal cardiac interventions: an update of therapeutic options Translated title: Intervenções cardíacas fetais: atualização de opções terapêuticas

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          Abstract

          Objective

          This article aims to present updated therapeutic options for fetal congenital heart diseases.

          Methods

          Data source for the present study was based on comprehensive literature retrieval on fetal cardiac interventions in terms of indications, technical approaches and clinical outcomes.

          Results

          About 5% of fetal congenital heart diseases are critical and timely intrauterine intervention may alleviate heart function. Candidates for fetal cardiac interventions are limited. These candidates may include critical aortic valve stenosis with evolving hypoplastic left heart syndrome, pulmonary atresia with an intact ventricular septum and evolving hypoplastic right heart syndrome, and hypoplastic left heart syndrome with an intact or highly restrictive atrial septum as well as fetal heart block. The advocated option are prenatal aortic valvuloplasty, pulmonary valvuloplasty, creation of atrial communication and fetal cardiac pacing.

          Conclusion

          Fetal cardiac interventions are feasible at midgestation with gradually improved technical success and fetal/postnatal survival due mainly to a well-trained multidisciplinary team, sophisticated equipment and better postnatal care.

          Translated abstract

          Objetivo

          Este artigo tem como objetivo apresentar opções terapêuticas atualizadas para cardiopatias congênitas fetais.

          Métodos

          Os dados do presente estudo foram baseados em uma revisão abrangente da literatura a respeito de intervenções cardíacas fetais em termos de indicações, abordagens técnicas e resultados clínicos.

          Resultados

          Cerca de 5% das cardiopatias congênitas fetais são críticas e a intervenção intrauterina oportuna pode aliviar a função cardíaca. Os candidatos para intervenções cardíacas fetais são limitados e podem incluir aqueles que são portadores de estenose valvar aórtica crítica com evolução para síndrome de hipoplasia do coração esquerdo, atresia pulmonar com septo ventricular íntegro e com evolução da síndrome de hipoplasia do coração direito e síndrome de hipoplasia do coração esquerdo com septo atrial intacto ou altamente restritivo, bem como bloqueio cardíaco fetal. As opções preconizadas são a valvoplastia aórtica fetal, valvoplastia pulmonar, criação de comunicação interatrial e implante de marca-passos em fetos.

          Conclusão

          Intervenções cardíacas fetais são viáveis na fase intermediária da gestação com sucesso técnico e sobrevivência fetal/pós-natal, sendo aprimorados gradualmente, devido, sobretudo, a uma equipe multidisciplinar bem treinada, equipamentos sofisticados e melhores cuidados no pós-natal.

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          Most cited references193

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          Fetal aortic valve stenosis and the evolution of hypoplastic left heart syndrome: patient selection for fetal intervention.

          Fetal aortic valvuloplasty may prevent progression of aortic stenosis (AS) to hypoplastic left heart syndrome (HLHS). Predicting which fetuses with AS will develop HLHS is essential to optimize patient selection for fetal intervention. The aim of this study was to define echocardiographic features associated with progression of midgestation fetal AS to HLHS. Fetal echocardiograms were reviewed from 43 fetuses diagnosed with AS and normal left ventricular (LV) length at < or =30 weeks' gestation. Of 23 live-born patients with available follow-up data, 17 had HLHS and 6 had a biventricular circulation. At the time of diagnosis, LV length, mitral valve, aortic valve, and ascending aortic diameter Z-scores did not differ between fetuses that ultimately developed HLHS and those that maintained a biventricular circulation postnatally. However, all of the fetuses that progressed to HLHS had retrograde flow in the transverse aortic arch (TAA), 88% had left-to-right flow across the foramen ovale, 91% had monophasic mitral inflow, and 94% had significant LV dysfunction. In contrast, all 6 fetuses with a biventricular circulation postnatally had antegrade flow in the TAA, biphasic mitral inflow, and normal LV function. With advancing gestation, growth arrest of left heart structures became evident in fetuses developing HLHS. In midgestation fetuses with AS and normal LV length, reversed flow in the TAA and foramen ovale, monophasic mitral inflow, and LV dysfunction are predictive of progression to HLHS. These physiological features may help refine patient selection for fetal intervention to prevent the progression of AS to HLHS.
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            Predictors of technical success and postnatal biventricular outcome after in utero aortic valvuloplasty for aortic stenosis with evolving hypoplastic left heart syndrome.

            Aortic stenosis in the midgestation fetus with a normal-sized or dilated left ventricle predictably progresses to hypoplastic left heart syndrome when associated with certain physiological findings. Prenatal balloon aortic valvuloplasty may improve left heart growth and function, possibly preventing evolution to hypoplastic left heart syndrome. Between March 2000 and October 2008, 70 fetuses underwent attempted aortic valvuloplasty for critical aortic stenosis with evolving hypoplastic left heart syndrome. We analyzed this experience to determine factors associated with procedural and postnatal outcome. The median gestational age at intervention was 23 weeks. The procedure was technically successful in 52 fetuses (74%). Relative to 21 untreated comparison fetuses, subsequent prenatal growth of the aortic and mitral valves, but not the left ventricle, was improved after intervention. Nine pregnancies (13%) did not reach a viable term or preterm birth. Seventeen patients had a biventricular circulation postnatally, 15 from birth. Larger left heart structures and higher left ventricular pressure at the time of intervention were associated with biventricular outcome. A multivariable threshold scoring system was able to discriminate fetuses with a biventricular outcome with 100% sensitivity and modest positive predictive value. Technically successful aortic valvuloplasty alters left heart valvar growth in fetuses with aortic stenosis and evolving hypoplastic left heart syndrome and, in a subset of cases, appeared to contribute to a biventricular outcome after birth. Fetal aortic valvuloplasty carries a risk of fetal demise. Fetuses undergoing in utero aortic valvuloplasty with an unfavorable multivariable threshold score at the time of intervention are very unlikely to achieve a biventricular circulation postnatally.
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              Fetal cardiac function: technical considerations and potential research and clinical applications.

              Fetal echocardiography was initially used to detect structural anomalies but has more recently also been proposed to assess fetal cardiac function. This review summarizes technical issues and limitations in fetal cardiac function evaluation, as well as its potential research and clinical applications. Functional echocardiography has been demonstrated to select high-risk populations and to be associated with outcome in several fetal conditions including intrauterine growth restriction, twin-to-twin transfusion syndrome, maternal diabetes, and congenital diaphragmatic hernia. Fetal heart evaluation is challenging due to the smallness and high heart rate of the fetus and restricted access to the fetus far from the transducer. Due to these limitations and differences in cardiac function which are related to fetal maturation, cardiovascular parameters should be validated in the fetus and used with caution. Despite these precautions, in expert hands and with appropriate ultrasound equipment, evaluation of cardiac function is feasible in most fetuses. Functional fetal echocardiography is a promising tool that may soon be incorporated into clinical practice. Research is warranted to further refine the contribution of fetal cardiac assessment to the diagnosis, monitoring, or prediction of outcomes in various fetal conditions.
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                Author and article information

                Journal
                Rev Bras Cir Cardiovasc
                Rev Bras Cir Cardiovasc
                Revista Brasileira de Cirurgia Cardiovascular : órgão oficial da Sociedade Brasileira de Cirurgia Cardiovascular
                Sociedade Brasileira de Cirurgia Cardiovascular
                0102-7638
                1678-9741
                Jul-Sep 2014
                Jul-Sep 2014
                : 29
                : 3
                : 388-395
                Affiliations
                [01]The First Hospital of Putian, Teaching Hospital, Fujian Medical University, Putian, China
                Author notes
                Correspondence address: Shi-Min Yuan, Longdejing Street, 389 - Chengxian District, Putian, Fujian Province, People's Republic of China. E-mail: shi_min_yuan@ 123456yahoo.com
                Article
                10.5935/1678-9741.20140099
                4412330
                25372914
                50ab929e-5f39-4dba-aa5b-0d8d25e29213

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 30 April 2014
                : 24 July 2014
                Categories
                Review Articles

                fetal heart,heart defects, congenital,prognosis
                fetal heart, heart defects, congenital, prognosis

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