Specialized Delivery Room Planning for Fetuses With Critical Congenital Heart Disease

Donofrio, Mary T.; Levy, Richard J.; Schuette, Jennifer; Skurow-Todd, Kami; Sten, May Britt; Stallings, Caroline; Pike, Jodi I.; Krishnan, Anita; Ratnayaka, Kanishka; Sinha, Pranava; duPlessis, Adré J.; Downing, David S.; Fries, Melissa H.; Berger, John

doi:10.1016/j.amjcard.2012.11.029

Cited by 125 publications

(105 citation statements)

References 26 publications

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“…175,176,481,547,572,584,585,[588][589][590][591][592][593][594] Fetuses with HLHS identified to have a severely restrictive or intact atrial septum are at increased risk for compromise in the delivery room if fetal pulmonary vein flow shows significant reversed flow suggesting severe left atrial hypertension in the third trimester. 175,176,481,549,572 In 2 studies, 176,481 the ratio of pulmonary vein forward to reversed velocity-time integral was used to determine potential need for intervention. These studies suggest that a ratio <3 is predictive of an increased likelihood of needing emergent opening of the atrial septum by catheterization or surgery and therefore should prompt delivery room management to include immediate access to a cardiac team for the procedure if it is indicated.…”

Section: Foramen Ovale-dependent Lesionsmentioning

confidence: 99%

“…Of note, a recent study of high-risk fetuses with CHD found that using the criteria of a tethered or bowing septum primum in d-TGA fetuses did not by guest on http://circ.ahajournals.org/ Downloaded from predict postnatal compromise and need for emergent intervention with an acceptable sensitivity or specificity. 572 In this study, if there were any concerning foramen ovale findings with a ductus arteriosus that was small or had abnormal flow, risk of postnatal compromise and need for urgent balloon atrial septostomy and possible treatment of pulmonary hypertension were high. Given the difficulty in predicting which fetuses with d-TGA will develop foramen ovale restriction and compromise at birth, all fetuses with d-TGA with a concerning septum primum should be delivered in a hospital that can manage the hypoxia and hemodynamic compromise that occur with foramen ovale closure and possible associated pulmonary hypertension.…”

Section: Foramen Ovale-dependent Lesionsmentioning

confidence: 99%

“…Models of risk assessment that include stratification of patients and specific postnatal care recommendations have been reported. 571,572 In practice, anticipated postnatal level of care should be assigned by the fetal diagnostic team, with concomitant delivery room and neonatal care recommendations made before delivery. Table 19 summarizes risk-stratified level of care assignment and coordinating action plans based on reported algorithms.…”

Section: Delivery Room and Neonatal Care Planningmentioning

confidence: 99%

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Diagnosis and Treatment of Fetal Cardiac Disease

Donofrio¹,

Moon-Grady²,

Hornberger³

et al. 2014

Circulation

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979

543

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Background— The goal of this statement is to review available literature and to put forth a scientific statement on the current practice of fetal cardiac medicine, including the diagnosis and management of fetal cardiovascular disease. Methods and Results— A writing group appointed by the American Heart Association reviewed the available literature pertaining to topics relevant to fetal cardiac medicine, including the diagnosis of congenital heart disease and arrhythmias, assessment of cardiac function and the cardiovascular system, and available treatment options. The American College of Cardiology/American Heart Association classification of recommendations and level of evidence for practice guidelines were applied to the current practice of fetal cardiac medicine. Recommendations relating to the specifics of fetal diagnosis, including the timing of referral for study, indications for referral, and experience suggested for performance and interpretation of studies, are presented. The components of a fetal echocardiogram are described in detail, including descriptions of the assessment of cardiac anatomy, cardiac function, and rhythm. Complementary modalities for fetal cardiac assessment are reviewed, including the use of advanced ultrasound techniques, fetal magnetic resonance imaging, and fetal magnetocardiography and electrocardiography for rhythm assessment. Models for parental counseling and a discussion of parental stress and depression assessments are reviewed. Available fetal therapies, including medical management for arrhythmias or heart failure and closed or open intervention for diseases affecting the cardiovascular system such as twin–twin transfusion syndrome, lung masses, and vascular tumors, are highlighted. Catheter-based intervention strategies to prevent the progression of disease in utero are also discussed. Recommendations for delivery planning strategies for fetuses with congenital heart disease including models based on classification of disease severity and delivery room treatment will be highlighted. Outcome assessment is reviewed to show the benefit of prenatal diagnosis and management as they affect outcome for babies with congenital heart disease. Conclusions— Fetal cardiac medicine has evolved considerably over the past 2 decades, predominantly in response to advances in imaging technology and innovations in therapies. The diagnosis of cardiac disease in the fetus is mostly made with ultrasound; however, new technologies, including 3- and 4-dimensional echocardiography, magnetic resonance imaging, and fetal electrocardiography and magnetocardiography, are available. Medical and interventional treatments for select diseases and strategies for delivery room care enable stabilization of high-risk fetuses and contribute to improved outcomes. This statement highlights what is currently known and recommended on the basis of evidence and experience in the rapidly advancing and highly specialized field of fetal cardiac care.

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Section: Foramen Ovale-dependent Lesionsmentioning

confidence: 99%

Section: Foramen Ovale-dependent Lesionsmentioning

confidence: 99%

Section: Delivery Room and Neonatal Care Planningmentioning

confidence: 99%

See 1 more Smart Citation

Diagnosis and Treatment of Fetal Cardiac Disease

Donofrio¹,

Moon-Grady²,

Hornberger³

et al. 2014

Circulation

Self Cite

979

543

View full text Add to dashboard Cite

show abstract

“…Place of delivery of the fetus with critical CHD should be decided based on individual diagnosis and assessment of risk associated with the cardiac diagnosis and its pathophysiology, as well as the level of care available locally 5. Decision making should be tailored to the individual case, taking all factors into account—but ensuring that place of elective delivery is optimal and sufficiently placed to look after the neonate, with the awareness that a significant number of babies will be born prematurely.…”

Section: Discussionmentioning

confidence: 99%

Antenatal diagnosis of critical congenital heart disease. Optimal place of delivery is where appropriate care can be delivered

Carvalho

2016

Arch Dis Child

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“…Therefore, to have a better prenatal assessment, magnetic resonance imaging (MRI) is applied in addition to ultrasonography. As suggested by Donofrio 30 , ectopia cordis currently belongs to those fetal cardiac abnormalities for which a specialized delivery room should be an option with a multi-specialist perinatal team (consisting of an obstetrician, neonatologist, prenatal cardiologist, anaesthestologist, pediatric surgeon, special midwife and nurses).…”

Section: Discussionmentioning

confidence: 99%

What is the Survival Rate in Prenatally Detected Cantrell’s Pentalogy?

Pośpiech-Gąsior

Słodki

Respondek-Liberska

2016

Prenatal Cardiology

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Cantrell's pentalogy is a congenital defect characterized by uncompleted fusion of the anterior chest wall, resulting in an extrathoracic location of the heart. Ultrasound diagnosis during the first trimester of prenatal life is possible, and termination of pregnancy is usually chosen by pregnant women. We analysed 57 fetuses: 56 from literature and one additional recent case from our institute (from 2016) to evaluate what was the survival rate reported after prenatal diagnosis, including the possibility to terminate the pregnancy, intrauterine deaths and neonatal deaths. We found 10 survivors -18% since 1984.Despite dismal prognosis of fetal ectopia cordis, there is a chance for postnatal survivorship probably due to evolving anatomical structures, not only in the first trimester of pregnancy but also during the following weeks of prenatal life. Key words: ectopia cordis, Cantrell syndrome, diaphragmatic herniaCorresponding author: majkares@uni.lodz.pl Submitted: 2015Submitted: -12-11, accepted: 2016 INTRODUCTIONCantrell's pentalogy is an anomaly resulting from a defect in embryologic development and consists of the following: a deficiency of the anterior diaphragm, a midline supra-umbilical abdominal wall defect, a defect in the diaphragmatic pericardium, congenital intracardiac abnormalities and a defect of the lower sternum. MATERIAL AND METHODSWe analyzed the published reports of prenatal Cantrell's pentalogy from the literature, since 1984, which were detected in 1 st or second trimester of pregnancy. (Table 1), and focused on the gestational age at the time of diagnoses, number of termination of pregnancies, intrauterine demises and neonatal follow-up. The last case from 2016 from our institution is presented below:A 28-year-old woman (G1, P0) had no history of intake of any teratogens or exposure to unusual environment during the antenatal period. The family history was negative for congenital anomalies or genetic abnormalities, and there was no-cosanguinity. A screening ultrasound at 12 weeks of pregnancy revealed a nuchal translucency of 3,8 mm, nasal bone present and omphalocele (containing liver, stomach and cardiac apex)(Foto 1, 2-cine, 3). Biochemical tests of maternal blood showed PAPPA-A of 0,3 MoM and free bHCG of 0,733 MoM. Chorionic villus sampling was nondiagnostic and amniocentesis showed a karyotype of 46, XX. The pregnant women was offered termination of pregnancy, however, she decided to continue her pregnancy and to deliver at a tertiary obstetrical and cardiac center. Prenatal echocardiography at 18, 20, 27 and 30 th week of gestational age revealed a fourchamber heart with normal flow in the great arteries. Ultrasound biometry was adequate. At 27 th week due to visualization of intestines in fetal chest cavity suggesting diaphragmatic hernia (DH), MRI examination was offered and right anterior DH was confirmed. In the second half of pregnancy, the position of the fetal heart and size of omphalocele changed: there was an improvement in 'niche' in the fetal chest and a decrea...

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Specialized Delivery Room Planning for Fetuses With Critical Congenital Heart Disease

Cited by 125 publications

References 26 publications

Diagnosis and Treatment of Fetal Cardiac Disease

Diagnosis and Treatment of Fetal Cardiac Disease

Antenatal diagnosis of critical congenital heart disease. Optimal place of delivery is where appropriate care can be delivered

What is the Survival Rate in Prenatally Detected Cantrell’s Pentalogy?

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