Ebstein's anomaly and left ventricular noncompaction association

Blasco, Pedro Betrián; Agromayor, Elena Gallardo

doi:10.1016/j.ijcard.2006.07.147

Cited by 18 publications

(7 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…LVNC may occur in isolation or in association with underlying congenital heart disease. In particular, an association with Ebstein's anomaly is recognized [1, 3] with an association with mutations in sarcomere gene MYH7 implicated [5, 6]. …”

Section: Discussionmentioning

confidence: 99%

“…While Ebstein's anomaly may occur in isolation, it may also be associated with other cardiac conditions. While the presence of an interatrial communication is seen in more than 70% of patients, other associations include left ventricular noncompaction (LVNC) [1–3]. LVNC is characterized by failure of the usual myocardial development with the condition notable for deep trabeculations, typically within the left ventricular apex.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Ebstein’s Anomaly, Left Ventricular Noncompaction, and Sudden Cardiac Death

McGee

Warner

Collins

2015

Case Reports in Cardiology

View full text Add to dashboard Cite

Ebstein's anomaly is a congenital disorder characterized by apical displacement of the septal leaflet of the tricuspid valve. Ebstein's anomaly may be seen in association with other cardiac conditions, including patent foramen ovale, atrial septal defect, and left ventricular noncompaction (LVNC). LVNC is characterized by increased trabeculation within the left ventricular apex. Echocardiography is often used to diagnose LVNC; however, magnetic resonance (MR) imaging offers superior characterization of the myocardium. We report a case of sudden cardiac death in a patient with Ebstein's anomaly with unrecognized LVNC noted on post mortem examination with screening documenting the presence of LVNC in one of the patient's twin sons.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Ebstein’s Anomaly, Left Ventricular Noncompaction, and Sudden Cardiac Death

McGee

Warner

Collins

2015

Case Reports in Cardiology

View full text Add to dashboard Cite

show abstract

“…Noncompaction of ventricular myocardium can co-exist with other congenital cardiac disorders such as an atrial septal defect, interventricular septal defect, pulmonic stenosis, anomalous pulmonary venous connection, coronary artery anatomical anomalies and Ebstein's syndrome (6-9). In our study, all patients enrolled were isolated NVM and the majority were male.…”

Section: Discussionmentioning

confidence: 99%

Isolated Noncompaction of Ventricular Myocardium: a Magnetic Resonance Imaging Study of 11 Patients

et al. 2011

View full text Add to dashboard Cite

ObjectiveTo retrospectively summarize the cardiac magnetic resonance imaging (CMRI) findings of isolated noncompaction of ventricular myocardium (INVM).Materials and MethodsEleven patients (M:F = 9:2; mean age, 35 years) were evaluated. Steady-state free precession (SSFP), fast spin echo (SE) sequence, SSFP cine imaging, and delayed enhanced inversion recovery spoiled gradient echo (IR-SPGR) sequence were used for showing abnormal myocardium, measuring ratio of noncompacted/compacted myocardium layers (NC/C ratio), and detecting myocardial viability. The left ventricle was divided into nine segments and a NC/C ratio > 2.3 in diastole was used as cutoff value in diagnosing left INVM. The right ventricle was assessed qualitatively.ResultsCardiac MRI indicated left INVM in seven patients, right INVM in one patient and biventricle INVM in three patients. Characteristic CMRI changes included prominent trabeculations, deep intertrabecular recesses and an increase in the NC/C ratio. The most frequently involved segments was left ventricular apex. Three patients had abnormal high signals within the trabecular structures on SE T2 weighted image. One ventricular aneurysm and one apical thrombus were also observed. Delayed enhancement was seen in six of nine patients with subendocardial and transmural patterns.ConclusionThere are CMRI features that might be characteristic for INVM.

show abstract

“…It is characterized by prominent trabeculation and deep intertrabecular recesses, resulting in thickened myocardium consisting of 2 layers-compacted and noncompacted myocardium 2). LVNC is occasionally combined with other congenital cardiac diseases3-6) and genetic cardiomyopathies 7). Its clinical manifestations include heart failure, thromboembolism, ventricular tachyarrhythmia, and sudden death 8).…”

Section: Introductionmentioning

confidence: 99%

Isolated Left Ventricular Noncompaction with a Congenital Aneurysm Presenting with Recurrent Embolism

Ahn

Koh

Park

et al. 2012

J Cardiovasc Ultrasound

View full text Add to dashboard Cite

Isolated left ventricular noncompaction (LVNC) is a rare disorder caused by embryonic arrest of compaction. LVNC is sometimes associated with other congenital cardiac disorders; however, there have been few reports of its coexistence with a left ventricular aneurysm. A 40-year-old woman was admitted to our hospital for renal infarction. She had a history of embolic cerebral infarction 10 years ago. Transthoracic echocardiography showed prominent trabeculae and deep intertrabecular recesses which are filled with blood from the left ventricular (LV) cavity. A thrombus in the akinetic apical wall was confirmed by contrast echocardiography. Using cardiac computed tomography and magnetic resonance imaging, we rejected a possible diagnosis of suspicion of coronary artery disease. She was diagnosed LVNC with a thrombus in apical aneurysm. Here, we report the first patient in Korea known to have LVNC accompanying LV congenital aneurysm presenting with recurrent embolism.

show abstract

Ebstein's anomaly and left ventricular noncompaction association

Cited by 18 publications

References 6 publications

Ebstein’s Anomaly, Left Ventricular Noncompaction, and Sudden Cardiac Death

Ebstein’s Anomaly, Left Ventricular Noncompaction, and Sudden Cardiac Death

Isolated Noncompaction of Ventricular Myocardium: a Magnetic Resonance Imaging Study of 11 Patients

Isolated Left Ventricular Noncompaction with a Congenital Aneurysm Presenting with Recurrent Embolism

Contact Info

Product

Resources

About