Ebstein's Anomaly: A Complex Congenital Heart Defect

Cherry, Cecile; DeBord, Starla; Moustapha-Nadler, Noha

doi:10.1016/j.aorn.2009.03.003

Cited by 20 publications

(12 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…37 Tricuspid valve repair with anterior leaflet mobilization was applied by Carpentier in 1988, achieving a late survival of 82% at 20 years. 22 Most patients with Ebstein's anomaly may achieve successful biventricular repair. Fontan operation is not performed routinely because of the difficult access for electrophysiological evaluation and ablation and for pacemaker lead placement.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Ebstein's Anomaly: Genetics, Clinical Manifestations, and Management

Yuan

2017

Pediatrics & Neonatology

View full text Add to dashboard Cite

Ebstein's anomaly is uncommon. Genetic bases of this congenital heart defect may be related to the mutations in myosin heavy chain 7 and NKX2.5, among others. Asymptomatic patients with Ebstein's anomaly can be conservatively treated and kept under close follow-up, whereas surgical operation is indicated for those patients with evidence of right heart dilation and progressively impaired ventricular systolic function. A biventricular repair consisting of the reconstruction of a competent monocuspid tricuspid valve, right ventriculorrhaphy, subtotal atrial septal defect closure, and aggressive reduction atrioplasty is suitable for most patients, and 1.5-ventricular repair (bidirectional Glenn shunt) is indicated for patients with poor right ventricular function; by contrast, heart transplantation is used in patients with severe left ventricular dysfunction.

show abstract

Section: Discussionmentioning

confidence: 99%

“…20,21 Electrocardiogram abnormalities of this anomaly include unusually tall and broad P waves due to right atrial enlargement, right bundle branch block caused by abnormalities of the right bundle branch, tachyarrhythmias related to accessory pathways, and WolffeParkinsoneWhite syndrome. 22…”

Section: Clinical Manifestations and Diagnosismentioning

confidence: 99%

Ebstein's Anomaly: Genetics, Clinical Manifestations, and Management

Yuan

2017

Pediatrics & Neonatology

View full text Add to dashboard Cite

show abstract

“…[ 8 , 9 ] The pathological changes of EA are that septal, posterior and anterior leaflet (in few cases) of tricuspid valve are not adhere to tricuspid annulus which move down to RV spirally with textural anomalies and paramorphia of atrioventricular sulcus, valve, flap structure, and RV. [ 10 , 11 ] Because of interactivity of RV agenesis and dysfunction, together with tricuspid incompetence, RV has an overload of volume and cardiac morphology increased, as a result, anoxia, heart failure, and arrhythmia can be the threat to life, [ 12 ] but complications associated with external cardiac first symptoms are more serious, including cerebrovascular accident, brain abscess, and peripheral arterial embolization, which brings heavy strike to patients and families. Therefore, early surgical correction is the most effective therapeutic method to cure tricuspid regurgitation, abnormal ventricular movement, and other combined malformation.…”

Section: Discussionmentioning

confidence: 99%

Acute arterial embolism of left lower extremity caused by paradoxical embolism in Ebstein's anomaly

Ma²,

Yan³

et al. 2017

Medicine

View full text Add to dashboard Cite

Introduction:Ebstein's anomaly is a benign and stable congenital heart disease for asymptomatic patients. Despite a low incidence of Ebstein's anomaly (EA), patients’ quality of life can be badly affected by EA without positive surgical intervention. Especially EA is associated with other congenital heart disease, such as the atrial septal defect, patent foramen ovale, and arterial embolism exclude other reasons, it is often considered to be the consequence of paradoxical embolism, and surgical intervention must be conducted.Case report:An 11-year-old girl falling off the bed suffered pain from left lower extremity. Echocardiographic evaluation revealed an EA, severe tricuspid regurgitation, and secundum atrial septal defect. Both left leg amputation and cardiac surgery were conducted after recovery. Under the condition of anesthesia cardiopulmonary bypass extracorporeal circulation, atrial septal defect repair and Cone reconstruction of the tricuspid valve were performed. Patient recovered well and left hospital smoothly.Discussion:EA is a rare and complex congenital cardiac malformation. There are about 80% to 90% of EA patients with combined atrial septal defect and patent foramen ovale. Sudden arterial occlusion is very rare especially in childhood. When thoracic roentgenoscopy, arterial blood gas analysis, coagulation test, and echocardiographic of lower extremity deep venous system are all normal, one should consider the possibility of a paradoxical embolism. If patients have the paradoxical embolism or worsening tricuspid regurgitation, the most suitable therapeutic regimen should be chosen according to patients’ condition. With surgical techniques and methods renewed continuously, cone reconstruction of the tricuspid valve has been confirmed in clinical trials, which can use its own tissues to form not only central bloodstream, but also the coaption between leaflet and leaflet.

show abstract

“…The septal, posterior leaflet, and anterior leaflet in few cases do not adhere to tricuspid annulus, which have spiral downward displacement to heart ventricle and are divided into atrialized and functional right ventricle. [10,11] EA was divided into 4 types by Carpentier's classification method according to the morphologies and activities of the tricuspid valve and the right ventricle. [12] Type A: atrial ventricle is contractile; septal leaflet and posterior leaflet have the moderate downward displacement; and tricuspid valve has good activities.…”

Section: Discussionmentioning

confidence: 99%