Clinical aspects of myocardial fibrosis in adults with Ebstein’s anomaly

Ciepłucha, Aleksandra; Trojnarska, Olga; Kociemba, Anna; Łanocha, Magdalena; Barczyński, M; Rozmiarek, Szymon; Kramer, Lisa A.; Pyda, Małgorzata

doi:10.1007/s00380-018-1141-5

Cited by 23 publications

(20 citation statements)

References 42 publications

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“…A study on 37 patients with ebstein anomaly in adulthood aged 43.0 ± 14.4 years revealed that fibrosis was the initial cause of arrhythmias and heart failure assessed by LGE on cardiac MRI. 20% of patients reaching adulthood died from arrhythmias, whereas 50% died from heart failure [9]. Sudden cardiac death may occur in asymptomatic patients with mild disease and normal cardiothoracic index on chest-X-ray.…”

Section: Discussionmentioning

confidence: 99%

Ebstein Anomaly in a 60 Years Patient: A Lucky Finding

Tabbah¹,

Karaminissian²

2018

CCR

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Ebstein anomaly is a rare congenital disease of the tricuspid valve (<1%) diagnosed at all ages. A single case of an 85 y old patient was reported in 1979 as the longest survival with ebstein anomaly who had no cardiac symptoms until 79 years old. The aim of this case report is to highlight the need for an early echocardiographic diagnosis of this disease to prevent sudden death from arrhythmias or other complications because as we see patients with ebstein anomaly can live a healthy long life asymptomatic. The patient described in this case is a 60 years old male diabetic and heavy smoker, who presented to the cardiology department with fatigue and atypical angina with dyspnea on moderate effort. Cardiac ultrasound was in favor of an isolated Ebstein anomaly type A, with partial atrialization of the right ventricle (RV) with an adequate volume of the right ventricle (17cm 2) and no specific other associated anomalies. Symptoms described by the patient were purely pulmonary due to a mild obstructive disease. Patient was diagnosed with chronic obstructive lung disease due to his smoking habits. Reaching this age and being asymptomatic with conserved RV and LV function is a sign of good outcome. This case was an interesting lucky finding. It was astonishing to see a patient surviving this anomaly at 60 years old asymptomatically.

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Section: Discussionmentioning

confidence: 99%

Ebstein Anomaly in a 60 Years Patient: A Lucky Finding

Tabbah¹,

Karaminissian²

2018

CCR

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“…Late gadolinium enhancement (LGE) in various CHD revealed important prognostic associations between solid myocardial fibrosis and adverse clinical outcomes (49)(50)(51). In adults with EA, LGE highlighting myocardial scar tissue was a frequent finding within the right atrium and right ventricle (52). However, LGE is less reliable for B A detecting diffuse fibrosis, which is common in CHD (53).…”

Section: Cardiac Magnetic Resonance Imagingmentioning

confidence: 99%

“…However, even when tricuspid valve repair is carried out early RV performance cannot be restored to a satisfactory level in some cases. Structural RV myocardial changes have been described in EA which may be accountable for reduced RV performance (12,14,52). In this context, the optimal timing for surgery remains unclear.…”

Section: Open Questions and Future Perspectivesmentioning

confidence: 99%

Narrative review of Ebstein’s anomaly beyond childhood: Imaging, surgery, and future perspectives

Neumann¹,

Rüffer²,

Sachweh³

et al. 2021

Cardiovasc Diagn Ther

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Ebstein's anomaly is a rare congenital heart disease with malformation of the tricuspid valve and myopathy of the right ventricle. The septal and inferior leaflets adhere to the endocardium due to failure of delamination. This leads to apical displacement of their hinge points with a shift of the functional tricuspid valve annulus towards the right ventricular outflow tract with a possibly restrictive orifice. Frequently, a coaptation gap yields tricuspid valve regurgitation and over time the "atrialized" portion of the right ventricle may dilate. The highly variable anatomy determines the clinical presentation ranging from asymptomatic to very severe with need for early operation. Echocardiography and magnetic resonance imaging are the most important diagnostic modalities to assess the tricuspid valve as well as ventricular morphology and function.While medical management of asymptomatic patients can be effective for many years, surgical intervention is indicated before development of significant right ventricular dilatation or dysfunction. Onset of symptoms and arrhythmias are further indications for surgery. Modified cone reconstruction of the tricuspid valve is the state-of-the-art approach yielding the best results for most patients. Alternative procedures for select cases include tricuspid valve replacement and bidirectional cavopulmonary shunt depending on patient age and other individual characteristics. Long-term survival after surgery is favorable but rehospitalization and reoperation remain significant issues. Further studies are warranted to identify the optimal surgical strategy and timing before adverse right ventricular remodeling occurs. It is this article's objective to provide a comprehensive review of current literature and an overview on the management of Ebstein's Anomaly. It focuses on imaging, cardiac surgery, and outcome. Additionally, a brief insight into arrhythmias and their management is given. The "future perspectives" summarize open questions and fields of future research.

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“…Further evaluation can be done with cardiac magnetic resonance imaging (MRI), which can provide better quantification of RV size and function [ 12 ]. Cardiac MRI is also useful in locating fibrosis (late gadolinium enhancement) which can be focal or diffuse in the right atrium (RA) and RV of patients with Ebstein’s anomaly as well as quantifying it (with pre- and post-contrast T1 mapping), which is related with disease severity and prognosis [ 13 , 14 ].…”

Section: Introductionmentioning

confidence: 99%

Ebstein’s Anomaly in Disguise: Follow the Cues and the Diagnosis Can Be Made

Sharma¹,

Habash²,

Mounsey³

et al. 2020

Cureus

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Ebstein’s anomaly is a congenital defect, which is rarely present in adults with arrhythmias and right heart failure with tricuspid regurgitation. The diagnosis is made by non-invasive cardiac imaging with transthoracic echocardiography or cardiac magnetic resonance imaging. However, mild and atypical anatomical variants require a more specific investigation to make the diagnosis and differentiate it from other pathologies which have a similar presentation, including Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC). We present the case of a 66-year-old gentleman with a history of recurrent admissions due to decompensated heart failure exacerbations, now presenting with gradually worsening dyspnea. An echocardiogram was obtained, showing a thin-walled, dilated, and dysfunctional right ventricle (RV) with severe tricuspid regurgitation due to poor coaptation of the tricuspid leaflets. Although a very distinctive epsilon wave was seen on his electrocardiogram, highly suggestive of arrhythmogenic RV cardiomyopathy (ARVC), which would be enough to explain his presentation and initial echocardiogram; an off-axis plane of the tricuspid valve without any RV aneurysm or dilation of the RV outflow tract was incongruent with this diagnosis. Additional echocardiographic images were determinant to demonstrate both apical displacement and tethering of the septal tricuspid leaflet with an abnormally long anterior tricuspid leaflet, suggestive of Ebstein’s anomaly. This diagnosis was confirmed with cardiac magnetic resonance imaging. Mild variants of Ebstein’s anomaly, especially in the presence of confounding findings require focused imaging to ascertain the diagnosis. We review these non-traditional findings in trying to differentiate Ebstein’s from ARVC.

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Clinical aspects of myocardial fibrosis in adults with Ebstein’s anomaly

Cited by 23 publications

References 42 publications

Ebstein Anomaly in a 60 Years Patient: A Lucky Finding

Ebstein Anomaly in a 60 Years Patient: A Lucky Finding

Narrative review of Ebstein’s anomaly beyond childhood: Imaging, surgery, and future perspectives

Ebstein’s Anomaly in Disguise: Follow the Cues and the Diagnosis Can Be Made

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