The Two Anomalies of Wilhelm Ebstein

Mazurak, Magdalena; Kusa, Jacek

doi:10.14503/thij-16-6063

Cited by 8 publications

(5 citation statements)

References 15 publications

(11 reference statements)

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“…EA is a rare complex congenital cardiac abnormality first reported by Wilhelm Ebstein in 1866 [ 6 ]. The main pathologic finding of EA is apical displacement of the tricuspid septal leaflet by a minimum of 8 mm/m 2 [ 7 ], and that of our patient was 9 mm/m 2 .…”

Section: Discussionmentioning

confidence: 99%

Ebstein’s Anomaly: An Unanticipated Differential Diagnosis of Egg-on-Stick Appearance and the Diagnostic Dilemma

et al. 2021

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Patient: Female, 42-year-old Final Diagnosis: Ebstein’s anomaly Symptoms: Dyspnoea on exertion • early satiety • fatigue • orthopnea • PND • progressive abdominal swelling • weigh loss Medication: — Clinical Procedure: Paracentesis Specialty: Cardiology Objective: Rare co-existance of disease or pathology Background: Ebstein’s anomaly (EA) is a rare congenital cardiac abnormality with diverse anatomic and spectra of clinical presentations. This heart anomaly occurs in approximately 1 per 200 000 live births and accounts for <1% of congenital cardiac diseases. The main pathologic finding is “atrialization” of the basal region of the right ventricle. EA has been described extensively in the literature; however, to the best of our knowledge, this is the first reported case of EA presenting with ”egg-on-stick” appearance. Case Report: A 42-year-old woman presented on account of 2 years of progressive abdominal swelling, early satiety, and progressive weight loss. There was an associated history of dyspnea on exertion, easy fatigability, paroxysmal nocturnal dyspnea, and orthopnea. General examination revealed egg-on-stick appearance: visible distended abdominal wall veins and massive ascites with no pedal edema. Overall, pertinent cardiovascular examination findings and echocardiographic features are in keeping with EA. Conclusions: We present the likely first case of egg-on-stick appearance as part of the presentation of EA, which posed a diagnostic dilemma. Echocardiography can help in unraveling this dilemma.

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Section: Discussionmentioning

confidence: 99%

Ebstein’s Anomaly: An Unanticipated Differential Diagnosis of Egg-on-Stick Appearance and the Diagnostic Dilemma

et al. 2021

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“…Wilhelm Ebstein's scholarly description of the TV abnormality bearing his name was published in 1866. [ 9 ] Ebstein's anomaly is a rare congenital heart disease. Although the disease has general characteristics, each affected patient has different pathologic and hemodynamic characteristics, resulting in different natural histories.…”

Section: Discussionmentioning

confidence: 99%

Outcomes of Ebstein's Anomaly Patients Treated with Tricuspid Valvuloplasty or Tricuspid Valve Replacement

Sun

Pan

et al. 2018

Chinese Medical Journal

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Background:The incidence of Ebstein's anomaly is extremely low, and except for the Mayo Clinic, no cardiac center has reported on a sufficient number of patients. The aim of our study was to report the outcomes of Ebstein's anomaly patients treated with tricuspid valvuloplasty (TVP) or tricuspid valve replacement (TVR).Methods:TVP or TVR was performed in 245 patients from July 2006 to April 2016. We reviewed patients' records and contacted patients via outpatient service and over the telephone.Results:The mean follow-up time was 43.6 ± 32.6 months, and 224 (91.4%) patients underwent follow-up. The mean operative age was 31.2 ± 15.7 years. TVR was performed in 23 patients, and TVP was performed in 201 patients. The 30-day mortality rate was 1.3%, and the overall survival rate was 97.9% at 5 and 10 years. The early mortality rate of the TVP group was lower than that of the TVR group (0.5% vs. 8.7%, P = 0.028), and the overall mortality rate of the TVP group was lower than that of the TVR group, without statistical significance (1.0% vs. 8.7%). After propensity score matching, the rates of mortality and New York Heart Association class ≥ III were lower in the TVP group than those in the TVR group without statistical significance. Seven patients with Type B Wolff-Parkinson-White (WPW) syndrome underwent one-stage surgery, and arrhythmias disappeared. Six patients suffered from episodes of left ventricular outflow tract obstruction (LVOTO) during surgery. Severe LVOTO could be treated with reoperation of the atrialized right ventricle.Conclusions:Ebstein's anomaly patients treated with TVP or TVR can experience optimal outcomes with midterm follow-up. However, TVP should be the first-choice treatment. Optimal outcomes can be obtained from one-stage operation in patients with Type B WPW syndrome. Severe LVOTO during surgery might be related to improper operation of the atrialized right ventricle.

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“…ble, permitía la comunicación derecha-izquierda con la consiguiente cianosis, un aspecto que pasó desapercibido por el Dr. Ebstein y que se puede considerar como el único punto débil de su análisis (1,4).…”

Section: Antecedentes Históricosunclassified

Ebstein´s anomaly as cause of sudden death. Report of the first case of forensic autopsy in panama

Saldaña,

Lucena-Porcel,

Lucena

2022

Cuadernos De Medicina Forense

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Sudden cardiac death in young adults is often the first manifestation of an underlying cardiac disease not diagnosed previously. We present the case of sudden death in a 33-year-old male which occurred immediately after significant physical exertion. The autopsy revealed the existence of Ebstein´s disease that had not been diagnosed during the patient´s lifetime. Ebstein´s anomaly is a very rare congenital malformation that affects the tricuspid valve and is characterized, among other findings, by a severe dilatation of the right atrium with displacement of the septal and posterior leaflets towards the apex of the right ventricle with atrialization of part of it. As far as we know, this is the first case diagnosed after forensic autopsy in Panama. Due to its low frequency, this anomaly presents diagnostic challenges so it is not usually identified in life. Therefore, it is necessary for pathologists and forensic specialists to be familiar with it and to bear in mind among the differential diagnoses in cases of sudden death, especially in adolescents and young adults. Finally, a historical journey through the interesting life and work of Dr. Wilhelm Ebstein is carried out.

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The Two Anomalies of Wilhelm Ebstein

Cited by 8 publications

References 15 publications

Ebstein’s Anomaly: An Unanticipated Differential Diagnosis of Egg-on-Stick Appearance and the Diagnostic Dilemma

Ebstein’s Anomaly: An Unanticipated Differential Diagnosis of Egg-on-Stick Appearance and the Diagnostic Dilemma

Outcomes of Ebstein's Anomaly Patients Treated with Tricuspid Valvuloplasty or Tricuspid Valve Replacement

Ebstein´s anomaly as cause of sudden death. Report of the first case of forensic autopsy in panama

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