Prenatal detection of severe right ventricular outflow tract obstruction: pulmonary stenosis and pulmonary atresia.

Hornberger, Lisa K.; Benacerraf, Beryl R.; Bromley, Bryann; Spevak, Philip J.; Sanders, Stephen P.

doi:10.7863/jum.1994.13.10.743

Cited by 41 publications

(24 citation statements)

References 4 publications

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“…164,218 Chamber size discrepancy with a smaller left relative to right side of the heart could be secondary to altered pulmonary venous return 219 or a restrictive foramen ovale. 178 It may also occur as a consequence of right heart pathology that leads to an increased volume load to the right heart, including tricuspid 158,159 or pulmonary insufficiency, 160 severe pulmonary outflow obstruction, 220 or ductus arteriosus constriction. 173 Right heart dilation may also be observed in the presence of arteriovenous malformations such as vein of Galen aneurysm 221 or in agenesis of the ductus venosus where umbilical venous return results in preferential streaming to the right heart.…”

Section: Cardiac Biometrymentioning

confidence: 99%

Diagnosis and Treatment of Fetal Cardiac Disease

Donofrio¹,

Moon-Grady²,

Hornberger³

et al. 2014

Circulation

1,018

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: Cardiac Biometrymentioning

confidence: 99%

Diagnosis and Treatment of Fetal Cardiac Disease

Donofrio¹,

Moon-Grady²,

Hornberger³

et al. 2014

Circulation

1,018

543

View full text Add to dashboard Cite

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“…This may be because this lesion is more readily detectable on a 4-chamber view than other lesions. 13 Increasing experience with fetal cardiac scanning by obstetric ultrasonographers may increase the frequency of antenatal diagnosis. The observation that this disease may evolve from critical pulmonary stenosis, and the potential for late growth failure of the right ventricle in the latter half of pregnancy, suggest that a single scan at 16 to 20 weeks of gestation may not be adequate to identify all cases of this condition.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Atresia With Intact Ventricular Septum

et al. 1998

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Background —Fetal echocardiography is widely established in the United Kingdom for prenatal diagnosis of congenital heart disease. This may result in a substantial reduction in incidence at birth because of selected termination of pregnancy. The objective of this population-based study was to determine the incidence of pulmonary atresia with intact ventricular septum (PAIVS) at birth, the impact of fetal echocardiography on this incidence, and to compare the outcome of cases with and those without prenatal diagnosis. Methods and Results —From 1991 to 1995, all infants born with PAIVS and all fetal diagnoses in the United Kingdom and Eire were studied. There were 183 live births (incidence 4.5/100 000 live births). The incidence was 4.1 cases per 100 000 live births in England and Wales, 4.7 in Scotland, 6.8 in Eire, and 9.6 in Northern Ireland ( P =0.01). There were 86 fetal diagnoses made at a mean of 22.0 weeks of gestation leading to 53 terminations of pregnancy (61%), 4 intrauterine deaths (5%), and 29 live births (34%). The incidence at birth would be 5.6 per 100 000 births in England and Wales, 5.3 in Scotland, and unchanged in Eire and Northern Ireland, if there were no terminations of pregnancy and assuming no further spontaneous fetal deaths ( P =0.28). An initial diagnosis of critical pulmonary stenosis was made in 6 cases, at a mean of 22.3 weeks of gestation with progression to PAIVS by 31.4 weeks. Probability of survival at 1 year was 65% and was the same for live-born infants whether or not a fetal diagnosis had been made. Conclusions —PAIVS is rare, occurring in 1 in 22 000 live births in the United Kingdom and Eire. Termination of pregnancy has resulted in an important reduction in the live-born incidence in mainland Britain.

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“…The whole volume of the right side of the pathway is decreased. As a result, the volume of blood crossing the DA is lower than normal [12]. The DA may thus be narrower, and its connection with the aorta develops an acute inferior angle.…”

Section: Abnormal Diametermentioning

confidence: 93%