Ebstein's anomaly associated with severe valvar pulmonary stenosis: Successful palliation with balloon pulmonary valvuloplasty in an adult

We report a 52-year-old adult with Ebstein's anomaly associated with severe valvar pulmonary stenosis, secundum atrial septal defect, polycythemia, hypoxemia, and severe exertional limitations. Balloon pulmonary valvuloplasty relieved the valvar obstruction, reduced the degree of tricuspid regurgitation and polycythemia, and improved systemic oxygenation and exercise abilities. Use of this technique in patients with this combination of lesions can postpone the need for surgical intervention. Cathet. Cardiovasc. Intervent. 46:441–444, 1999. © 1999 Wiley-Liss, Inc.     

Moyamoya Disease Complicating Ebstein's Anomaly

Ebstein''s anomaly is an uncommon congenital disorder affecting the tricuspid valve. We herein report a 38-year-old woman who experienced consciousness and sensory disturbance during treatment for heart failure caused by Ebstein''s anomaly. Urgent magnetic resonance imaging and cerebral angiography demonstrated acute cerebral infarction and internal carotid artery obstruction with the development of collateral arteries. We diagnosed her with multiple cerebral infarctions due to moyamoya disease. Ebstein''s anomaly concomitant with moyamoya disease is extremely rare. However, we should consider the possibility of this rare but important concurrence when treating patients with heart failure due to Ebstein''s anomaly to avoid excessive diuresis and vasodilation and irreversible brain injury.     

The shape and function of the left ventricle in Ebstein's anomaly

Background

Left ventricular (LV) failure is common in Ebstein's anomaly, though remains poorly understood. We investigated whether shape deformity impacts LV function.

Methods

Three-dimensional models of the right ventricle (RV) and LV from 29 adult Ebstein's patients and nine normal subjects were generated from cardiac magnetic resonance image tracings. LV end diastolic (ED) shape, systolic function, septal motion and ventricular interaction were analyzed.

Results

LV ED volume index was normal in Ebstein's (75 ± 19 vs. 78 ± 11 ml/m² in normals, p = 0.50) but the LV was basally narrowed and modestly dilated apically. LV function was reduced globally (ejection fraction (EF) 41 ± 7 vs. 57 ± 5% in normals, p < 0.0001) and regionally (decreased mean segment displacement at end systole (ES) in 12/16 segments, basal Z-scores − 2.1 to − 1.0). Septal dyskinesis was suggested by outward mean segment displacement in at least one basal septal segment in 25 patients (86%) but refuted by septal thickening in 14 (48%), normal septal curvature at ED and ES, and by visually evident basal LV anterior translation in 27 patients (93%). LV EF correlated better with normalized tricuspid annular plane systolic excursion (r = 0.70) than with RV EF (r = 0.42) or RVEDVI (r = 0.18).

Conclusions

Although the Ebstein's LV has preserved volume, it exhibits basal narrowing, modest apical dilation and global hypokinesis. The apparent basal septal dyskinesis observed in most patients is likely attributable to anterior cardiac translation rather than true paradoxical motion. LV EF is unaffected by RV volume, correlating well instead with RV longitudinal shortening.     

Transcatheter closure of atrial septal communication during pregnancy in women with Ebstein's anomaly of the tricuspid valve and cyanosis

Transcatheter atrial septal defect occlusion is described in three pregnant patients with Ebstein's anomaly and exercise‐induced cyanosis. Procedures took place in the second and third trimester. Careful hemodynamic testing was performed prior to permanent atrial septal occlusion to confirm the capacity of the right ventricle to accept the entirety of systemic venous return in the volume‐expanded state of pregnancy. Outcomes for mother and fetus were favorable in all patients with significant reduction in maternal symptoms and resolution of cyanosis. © 2014 Wiley Periodicals, Inc.     

Value of Electro‐Vectorcardiogram in Hypertrophic Cardiomyopathy

The electrocardiogram is an important tool for the initial diagnostic suspicion of hypertrophic cardiomyopathy in any of its forms, both in symptomatic and in asymptomatic patients because it is altered in more than 90 percent of the cases. Electrocardiographic anomalies are more common in patients carriers of manifest hypertrophic cardiomyopathy and the electrocardiogram alterations are earlier and more sensitive than the increase in left ventricular wall thickness detected by the echocardiogram. Nevertheless, despite being the leading cause of sudden death among young competitive athletes there is no consensus over the need to include the method in the pre‐participation screening. In apical hypertrophic cardiomyopathy the electrocardiographic hallmarks are the giant negative T waves in anterior precordial leads. In the vectorcardiogram, the QRS loop is located predominantly in the left anterior quadrant and T loop in the opposite right posterior quadrant, which justifies the deeply negative T waves recorded. The method allows estimating the left ventricular mass because it relates to the maximal spatial vector voltage of the left ventricle in the QRS loop. The recording on electrocardiogram or Holter monitoring of nonsustained monomorphic ventricular tachycardia in patients with syncope, recurrent syncope in young patient, hypotension induced by strain, bradyarrhythmia, or concealed conduction are markers of poor prognosis. The presence of rare sustained ventricular tachycardia is observed in mid‐septal obstructive HCM with apical aneurysm. The presence of complete right bundle branch block pattern is frequent after the percutaneous treatment and complete left bundle branch block is the rule after myectomy.     

Ebstein's Anomaly with Subpulmonary Obstruction– A Rare Association

Ebstein's anomaly comprises a wide spectrum of congenital cardiac abnormalities involving the tricuspid valve (TV) apparatus and the architecture of the morphological right ventricle (RV). We report a case of Ebstein's anomaly in a 3‐year‐old boy having a unique association with severe subpulmonic obstruction caused by tethered and dysplastic anterior tricuspid leaflet (ATL), and a large ventricular septal defect. In view of poorly functioning RV, the baby was planned for a bidirectional Glenn shunt with subsequent Fontan completion.     

Paradoxical embolism associated with Ebstein's anomaly in an adult: Case report

Ebstein's anomaly (EA) is a rare congenital malformation of the tricuspid valve, often associated with other cardiac malformations, especially atrial septal defect, which is present in 80–90% of patients and predisposes to paradoxical embolization. We describe the case of a 47-year-old male, a drug abuser, with a known but not investigated cardiac murmur. He presented to the emergency department with worsening exertional dyspnea and fatigue associated with recent recurrent transient ischemic attacks. On brain computed tomography there were multiple non-recent ischemic infarctions. Transthoracic echocardiography showed EA with severely dilated right cardiac chambers, right systolic dysfunction and severe tricuspid regurgitation. Contrast and transesophageal echocardiography revealed a patent foramen ovale with right-to-left shunt. After exclusion of other potential causes of the neurologic events, they were assumed to be the consequence of paradoxical embolism.     

Clinical presentation and echocardiographic characteristics of Uhl's anomaly

Uhl's anomaly is a rare cardiac malformation that results in partial or complete absence of the right ventricular myocardium. It most commonly presents in prenatal or newborn infants; however, it may also be found in some adults as advanced right‐sided heart failure. Differential diagnoses include arrhythmogenic right ventricular dysplasia and Ebstein's anomaly. Herein, we describe the clinical presentation of Uhl's anomaly in a previously undiagnosed middle‐aged adult, and review the echocardiographic criteria used to diagnose and differentiate this rare, albeit important, myocardial disorder.     

Use of nesiritide in Ebstein's anomaly

A rare case of a 77-year-old man with congestive heart failure due to a previously diagnosed condition of Ebstein's anomaly is presented. Unique to the present case is the treatment of New York Heart Association class IV congestive heart failure associated with Ebstein's anomaly with nesiritide.     

Ebstein心脏畸形24例的外科矫治

目的总结24例Ebstein畸形患者的外科手术治疗经验。方法回顾性分析2002年9月至2009年5月24例Ebstein畸形患者手术治疗的临床资料,采用Danielson水平折叠法矫治畸形17例,其中同时行瓣环成形12例,瓣交界成形5例,瓣叶修补4例;采用Carpentier纵行折叠5例,均同时行瓣环成形;1例采用解剖矫治;1例行三尖瓣置换术。合并畸形同期处理。结果全组无死亡,无严重心功能不全和顽固性低心排出量综合征发生,无因三尖瓣病变再次手术者。随访2～81个月,心功能Ⅰ级15例,Ⅱ级7例,2例恢复稍差,心功能Ⅱ～Ⅲ级。结论 Ebstein畸形应尽早外科矫治,外科治疗应根据每例患者瓣膜和心室的具体条件选择恰当的术式,恰当的瓣膜成形或瓣膜置换可以取得较好的中、远期效果。     

40例Ebstein畸形患儿的心电图分析

目的通过分析40例小儿三尖瓣下移畸形的心电图，探讨小儿三尖瓣下移畸形的心电图特点。方法分析40例三尖瓣下移畸形的心电图变化；并与其它50例房间隔缺损、50例各种紫绀型先天性心脏病心电图比较；并对三尖瓣下移畸形组中不同心功能级别的患儿（Ⅰ～Ⅱ级、Ⅱ级以上）的心电图进行分析。结果右房大、右束支阻滞、一度房室阻滞、V1导联R’波电压低、右胸导联出现q波伴T倒置、B型预激综合征、阵发性心动过速史与对照组有显著性差异（p〈0．01）。结论三尖瓣下移畸形的心电图特点对诊断和鉴别诊断有重要价值。     

Pearls for Ablation in Congenital Heart Disease

Ablation in Congenital Heart Disease. Tachyarrhythmias occur in patients having congenital heart disease most commonly related to surgically created or naturally occurring conduction obstacles, and to postoperative hemodynamic effects on cardiac muscle. Less frequently, the underlying disease is associated with tachycardia substrates. Thorough knowledge of the patient's congenital anatomy and surgical procedures is required before considering catheter ablation. In many instances, procedural considerations should include meticulous hemodynamic surveillance, analogous to patients having structurally normal heart but cardiomyopathy. This includes careful selection of sedating and anesthetic agents. Congenital heart defects that have a higher than expected incidence of naturally occurring tachyarrhythmia substrates include Ebstein anomaly of the tricuspid valve, congenitally corrected transposition, and some of the heterotaxies. Intraatrial reentry tachycardia and atrial flutter are especially prevalent following the Mustard or Senning operations for d‐transposition of the great arteries and the earlier Fontan type operations. Although these tachyarrhythmias are not as frequent following atrial septal defect repair, the high incidence of this defect also makes these patients germaine to this discussion. Focal atrial tachyacrdia and atrioventricular nodal reentry tachycardia also occur in these patient groups. Macroreentry ventricular tachycardia occurs most frequently following right ventricular outflow tract surgery, especially following repair of tetralogy of Fallot. This article focuses on the technical aspects of catheter ablation of these arrhythmias, due to the challenges presented by the underlying anatomy compared with patients having normal hearts. (J Cardiovasc Electrophysiol, Vol. 21, pp. 223‐230, February 2010)     

Ebstein's Malformation of the Tricuspid Valve: Short‐term Outcomes of the “Cone Procedure” versus Conventional Surgery

Objectives. We report our analysis of conventional surgery and the cone procedure for Ebstein's malformation (EM) of the tricuspid valve at a single institution. Previous conventional surgery for EM, including use of bioprosthetic valves, has inherent problems especially in pediatrics. The newer cone procedure aims to construct a funnel‐like valve out of native leaflets, obviating problems with artificial valves. Methods. This is a retrospective cohort study to examine short‐term outcomes of both surgeries for EM. Results. Nineteen patients (our initial cohort) had the cone procedure, and 13 had conventional tricuspid valve repair or replacement. No early deaths occurred in either group. Three cone and one conventional repair patients required reoperation. Two of 19 patients in the cone and one of 13 in the conventional group died suddenly >30 days after operation, assumed secondary to dysrhythmias. At discharge, by two‐dimensional echocardiography, the cone group had 85% reduction in tricuspid valve regurgitation (TVR), and the conventional group had 56% reduction, P= .004. This decrease of TVR persisted to a greater extent in the cone group. Discussion. Short‐term results for the cone procedure are similar to conventional surgery. The cone procedure uses autologous tissue; hypothetically, early favorable improvement in reduction of TVR should persist.