Clinical Features, Management, and Outcome of Children With Fetal and Postnatal Diagnoses of Isomerism Syndromes

Lim, Jessie Mei; McCrindle, Brian W.; Smallhorn, Jeffrey F.; Golding, Fraser; Caldarone, Christopher A.; Taketazu, Mio; Jaeggi, Edgar

doi:10.1161/circulationaha.105.552364

Cited by 100 publications

(85 citation statements)

References 43 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…539 This has not been a consistent observation; several other studies have failed to demonstrate a survival advantage among infants with a prenatal diagnosis for lesions such as d-TGA, congenitally corrected TGA, PA/IVS, TOF with pulmonary atresia, HLHS, heterotaxy syndrome, or double-inlet left ventricle. 176,[539][540][541][542]545,[554][555][556][557] In Utero Management Prenatal diagnosis of CHD may improve fetal and perinatal outcome associated with intrauterine heart failure or sudden intrauterine demise by guiding the initiation of intrauterine medical therapy and optimization of perinatal management strategies, including early delivery when necessary. As discussed in the Fetal Therapy for Cardiovascular Conditions Before Birth section, fetuses with tachyarrhythmias, particularly when incessant, occurring early in pregnancy, or in association with CHD, will benefit from the initiation of transplacental medical therapy.…”

Section: Impact On Survivalmentioning

confidence: 99%

Diagnosis and Treatment of Fetal Cardiac Disease

Donofrio¹,

Moon-Grady²,

Hornberger³

et al. 2014

Circulation

925

543

View full text Add to dashboard Cite

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.

show abstract

Section: Impact On Survivalmentioning

confidence: 99%

Diagnosis and Treatment of Fetal Cardiac Disease

Donofrio¹,

Moon-Grady²,

Hornberger³

et al. 2014

Circulation

925

543

View full text Add to dashboard Cite

show abstract

“…The success of heart transplantation as a therapy for hypoplastic left heart syndrome led pediatric transplantation centers to apply heart transplantation as primary therapy in other conditions for which there was a poor natural history with standard surgical therapy. These conditions have included pulmonary atresia with intact septum and right ventricledependent coronary circulation 54,55 and complex heterotaxy syndromes 56,57 in which a functional single ventricle can be associated with anomalous pulmonary venous return and severe atrioventricular or semilunar valve disease.…”

Section: Congenital Heart Diseasementioning

confidence: 99%

Indications for Heart Transplantation in Pediatric Heart Disease

Canter¹,

Shaddy²,

Bernstein³

et al. 2007

Circulation

252

View full text Add to dashboard Cite

Background— Since the initial utilization of heart transplantation as therapy for end-stage pediatric heart disease, improvements have occurred in outcomes with heart transplantation and surgical therapies for congenital heart disease along with the application of medical therapies to pediatric heart failure that have improved outcomes in adults. These events justify a reevaluation of the indications for heart transplantation in congenital heart disease and other causes of pediatric heart failure. Methods and Results— A working group was commissioned to review accumulated experience with pediatric heart transplantation and its use in patients with unrepaired and/or previously repaired or palliated congenital heart disease (children and adults), in patients with pediatric cardiomyopathies, and in pediatric patients with prior heart transplantation. Evidence-based guidelines for the indications for heart transplantation or retransplantation for these conditions were developed. Conclusions— This evaluation has led to the development and refinement of indications for heart transplantation for patients with congenital heart disease and pediatric cardiomyopathies in addition to indications for pediatric heart retransplantation.

show abstract

“…Historically visceral heterotaxy has been associated with significant morbidity and mortality [2,[4][5][6][7]. Right atrial isomerism has been described by some as one of the worst forms of contemporary heart disease [8] with overall 5-year survival ranging from 30% to 74% [9,10]. The results are better in left atrial isomerism with 5-year survival rates ranging between 65% and 84%, which is still considerably lower than survival for most other forms of congenital heart disease [2,10].…”

Section: Introductionmentioning

confidence: 99%

Improved current era outcomes in patients with heterotaxy syndromes☆

Anagnostopoulos

Pearl

Octave

et al. 2009

European Journal of Cardio-Thoracic Surgery

View full text Add to dashboard Cite

Surgical outcomes in heterotaxy patients are improving in the current era. The risk for operative mortality and attrition is highest between the first and second stage palliation procedures. Significant atrioventricular valve regurgitation and obstructed TAPVR remain risk factors for RAI mortality. Survivors are doing well with no activity restrictions, although LAI patients maintain a higher proclivity of sinus node dysfunction.

show abstract

Clinical Features, Management, and Outcome of Children With Fetal and Postnatal Diagnoses of Isomerism Syndromes

Abstract: Background-Isomerism is associated with a complex spectrum of anomalies. There is paucity of data on prenatally detected cases. Methods and Results-Between

Cited by 100 publications

References 43 publications

Diagnosis and Treatment of Fetal Cardiac Disease

Diagnosis and Treatment of Fetal Cardiac Disease

Indications for Heart Transplantation in Pediatric Heart Disease

Improved current era outcomes in patients with heterotaxy syndromes☆

Contact Info

Product

Resources

About