Cardiac imaging in Ebstein anomaly

Ebstein anomaly is a congenital disorder of right ventricular myocardial development, which affects the tricuspid valve in addition to the right ventricular myocardium. Cardiac imaging by transthoracic echocardiography and cardiac magnetic resonance imaging are the key modalities used to assess timing and type of surgery. In this article, we review the current standards of echocardiographic and magnetic resonance imaging in Ebstein anomaly.     

Ebstein cardiac anomaly,functional pulmonary atresia and isovaleric acidemia: A case report

In this report, we present a rare association between Ebstein anomaly (EA) and isovaleric acidemia (IVA) in a newborn who was admitted to our cardiac center. He underwent for PDA stenting to maintain adequate pulmonary blood flow, later he developed recurrent metabolic acidosis, prominent sweaty feet odor, neutropenia and thrombocytopenia. His organic acids profile in the urine confirmed the diagnosis of IVA.To the best of our knowledge, there is no association between these two rare diseases. We are presenting this case report to highlight this rare association.     

超声心动图对三尖瓣下移畸形术前诊断及术后疗效评价的作用

目的探讨超声心动图诊断三尖瓣下移畸形(Ebstein anomaly)的准确性和对术后近期疗效的评价。方法8例行Ebstein anomaly手术的患者,术前均经超声心动图明确诊断,同时评价三尖瓣下移的程度、瓣叶发育情况及活动度、房化右心室的大小、三尖瓣反流程度及右心室功能等指标。术后随访和比较超声心动图变化及评价手术疗效。结果8例患者均有隔叶下移,三尖瓣环扩张和前叶发育异常并活动受限,伴后叶下移5例,三尖瓣中度以上反流6例,右心室功能减低1例,房化右心室大小为37mm×18mm~54mm×34mm。术中所见与术前检查基本一致。术后3~6个月进行超声心动图随访。结果显示:5例房化右心室基本消失,三尖瓣瓣叶基本回复到正常位置,3例房化右心室较前明显缩小,6例三尖瓣反流消失,所有患者三尖瓣环均明显缩小,右心室功能均正常。结论超声心动图对Ebstein anomaly术前诊断及术后疗效的评价均具有重要的作用。     

Arrhythmia after cone repair for Ebstein anomaly: The Mayo Clinic experience in 143 young patients

Background

The increased incidence of preoperative and postoperative arrhythmia in Ebstein anomaly (EA) prompted some clinicians to perform an electrophysiology study (EPS) in all patients prior to surgery for EA. The cone repair (CR) is the current surgical option of choice for most young patients with EA but the effect of the CR on arrhythmia is not well established.

Objectives

To assess the burden of arrhythmia in young patients after CR and to assess the utility of selective preoperative EPS.

Materials and Methods

A retrospective review of all patients <21 years of age with EA who had a CR at Mayo Clinic from June 2007 to December 2015 was performed. Surveys were mailed and telephone calls were made to all individuals to assess antiarrhythmic medication use and EP/device procedures performed after CR.

Results

There were 143 patients; median age, 10 years (0.1–20.9 years). Thirty‐five (24%) patients had a preoperative EPS of which 26 (18%) had a preoperative ablation. Indications for EPS were Wolff–Parkinson–White (WPW), documented arrhythmia, or suspected arrhythmia. Posthospital discharge data were available for 140 (98%) patients. Mean follow‐up was 2.9 years (0.1–9.2 years). At follow‐up, 7 (5%) patients were receiving antiarrhythmic medications. After CR, only 3 (2%) patients who did not have a preoperative EPS have required an ablation.

Conclusions

The risk of arrhythmia after CR for EA in young patients is very low when a preoperative EPS is limited to those with WPW, known arrhythmia, or suspected arrhythmia. In smaller patients, it may be reasonable to defer the EPS.     

Outcomes in adults with congenital heart disease and heterotaxy syndrome: A single‐center experience

Background: Heterotaxy syndrome (HS) is a condition in which the thoracoabdominal organs demonstrate an abnormal lateral arrangement and is often associated with con‐ genital heart disease (CHD). Little is known about the adult HS population with CHD.
Objective: To describe the outcomes and sociodemographics of the adult CHD popu‐ lation with HS.
Methods: Records of patients 18 years of age or older with diagnoses of both CHD and HS at Texas Children's Hospital from 1964 to 2018 were reviewed.
Results: Sixty‐two patients metinclusion criteria.Median agewas 22.7 [IQR19.6‐30.0] years; 26 (42%) were female; and 13 (21%) of patients had a gap in care of >3 years. Median follow‐up time in adulthood was 2.9 [IQR 1.3‐8.2] years. Forty‐three (69%) of patients had single ventricle heart disease, 31 (71%) of whom completed Fontan circulation. A total of 36 interventions occurred in 24 patients which included 16 cardiac catherization interventions, 13 electrophysiology‐related procedures, and 18 surgical procedures including 2 orthotopic heart transplants. The median age for death or heart transplant was 45.3 (95%CI 34.3‐56.1) years. Heart failure‐free sur‐ vival was 80.8 ± 5.2%, 58.7 ± 11.0%, and 31.1 ± 15.7% at 20, 30, and 40 years old, respectively. Cerebrovascular accident‐free survival was 84.3 ± 5.1%, 54.2 ± 11.3%, and 40.6 ± 14.5% at 20, 30, and 40 years old, respectively. Tachyarrhythmia‐free survival was 54.0 ± 7.1%, 29.2 ± 8.3%, and 19.5 ± 9.7% at 20, 30, and 40 years old and bradyarrhythmia‐free survival was 66.0 ± 6.3%, 41.7 ± 9.4%, and 33.4 ± 10.6% at ages 20, 30, and 40 years, respectively.
Conclusions: At a tertiary referral center, adult patients with CHD and HS have high rates of comorbidities and early death or heart transplant. Longitudinal surveillance and further exploration into factors associated with improved survival in this popula‐ tion are warranted.     

Percutaneous closure of paravalvular leak after Cone repair for Ebstein's anomaly

We report the use of an Amplatzer Vascular plug for percutaneous closure of a paravalvular leak after Cone repair in a 5‐year‐old boy with Ebstein's anomaly. A paravalvular leak of the tricuspid valve developed gradually after Cone repair‐surgery. The combination of fluoroscopy, transesophageal and transthoracic imaging during general anesthesia was necessary for correct and safe positioning of the device by percutaneous approach. The results were promising.     

Percutaneous closure of an atrial septal defect in a patient with Ebstein anomaly and right-to-left shunt

Ebstein anomaly is a congenital disease frequently associated with atrial septal defects, which can generate a right-to-left shunt, leading to systemic desaturation and right ventricular failure.We describe the case of a 68-year-old man with central cyanosis due to Ebstein anomaly and a patent foramen ovale. An atrial septal occluder was initially implanted after having performed prolonged test occlusion of the interatrial communication. In this case, device embolization occurred due to high right pressure.Percutaneous closure of atrial septal defects in the presence of a right-to-left shunt can offer a significant clinical improvement in selected cases. In patients with Ebstein anomaly, the implantation of atrial septal defect closure devices may be desirable, due to the larger size of the waist, which may provide better stability in the event of an increase in right pressure.