Two-Layered Appearance of Noncompaction of the Ventricular Myocardium on Magnetic Resonance Imaging.

Daimon, Yumiko; Watanabe, Shigeru; Takeda, Shin’ichi; Hijikata, Yasuyoshi; Komuro, Issei

doi:10.1253/circj.66.619

Cited by 67 publications

(45 citation statements)

References 13 publications

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Section: A B Cmentioning

confidence: 98%

“…10 A >2.3:1 ratio of noncompacted-to-compacted myocardium yields the highest diagnostic sensitivity (86%) and specificity (99%). 10,11 Late gadolinium hyperenhancement is related to myocardial fibrosis and scarring in the hypertrabeculated myocardium. 12 Differential diagnoses include prominent normal myocardial trabeculations, false tendons, localized LV hypertrophy, LV thrombus, intramyocardial hematoma, arrhythmogenic RV dysplasia, endocardial fibroelastosis, cardiac metastases, and intramyocardial abscesses.…”

Section: A B Cmentioning

confidence: 98%

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Left Ventricular Noncompaction Cardiomyopathy Presenting with Heart Failure in a 35-Year-Old Man

Papadopoulos¹,

Petrou²,

Michaelides³

2017

Texas Heart Institute Journal

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I solated ventricular noncompaction (IVNC) is a rare genetic cardiomyopathy characterized by excessively prominent ventricular trabeculations and deep intertrabecular recesses. It is thought to arise in utero from arrested compaction of a loose myocardial meshwork. The major clinical presentations of left ventricular noncompaction (LVNC) are heart failure, arrhythmias, and thromboembolism. The diagnosis is established with use of 2-dimensional echocardiography or cardiac magnetic resonance (CMR).We report the case of a patient with dilated cardiomyopathy and LVNC, and we discuss the effects of standard heart failure therapy on his condition. Case ReportIn March 2011, a 35-year-old man presented at a cardiology outpatient clinic with a several-week history of palpitations. His medical history yielded nothing relevant, and routine laboratory test results were normal. His chest radiograph showed mild cardiomegaly and normal lung fields. Physical examination revealed normal heart sounds and a mild systolic murmur heard best at the apex. A 12-lead electrocardiogram (ECG) showed sinus rhythm with poor R-wave progression and T-wave inversion in the precordial leads. A transthoracic echocardiogram (TTE) revealed a dilated left ventricle (LV), a depressed LV ejection fraction (LVEF) of 0.25, and moderate mitral regurgitation. A coronary angiogram revealed normal results. A 24-hour ECG showed frequent premature ventricular complexes and runs of nonsustained ventricular tachycardia.Results of a CMR study included a markedly dilated LV with an LVEF of 0.19, an end-diastolic volume of 395 mL, and an end-systolic volume of 319 mL (Fig. 1). Prominent trabeculations were seen in the apical segments and lateral wall of the LV (Fig. 2). The ratio of noncompacted-to-compacted myocardium was 3.35:1. The indexed LV mass was 76 g/m 2 with no evidence of hypertrophy. Right ventricular (RV) size, function, and wall structure were normal. Delayed myocardial gadolinium enhancement produced subtle mid-wall stripes along the anterior and lateral LV wall and the inferior half of the interventricular septum. There was also marked myocardial wall-thinning, particularly of the apical segments. The left atrial diameter was 36 mm. The diagnosis was LVNC with heart failure. The patient was started on perindopril and carvedilol, with gradual upward titration.Because of the patient's ventricular tachycardia and low LVEF, we referred him for implantable cardioverter-defibrillator placement. However, according to the im- Case ReportsKyriacos Papadopoulos, MD Petros M. Petrou, MBChB, MRCP (UK) Demos Michaelides, FRCR, FRCP

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Section: A B Cmentioning

confidence: 98%

Section: A B Cmentioning

confidence: 98%

Left Ventricular Noncompaction Cardiomyopathy Presenting with Heart Failure in a 35-Year-Old Man

Papadopoulos¹,

Petrou²,

Michaelides³

2017

Texas Heart Institute Journal

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“…Their recommendations include a minimum magnet strength of 1.5 Tesla in order to provide visualization of both long and short axis views in approximately 17 segments (22,23). To diagnose LVNC on CMR it is recommended to take three diastolic long axis views and choose the myocardial segment with the most prominent myocardial trabeculations to measure the NC/C ratio (22,24) (Figure 3). In contrast to echocardiography, the NC/C ratio on CMR is higher, NC/C >2.3, and the measurements are taken at the end of diastole ( Table 2) (25).…”

Section: Cardiac Mri Criteriamentioning

confidence: 99%

Left ventricular non-compaction cardiomyopathy.

Kok¹,

Ma²,

Ma³

2012

Journal of the Belgian Society of Radiology

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“…With respect to the findings of electron microscopy, there is not a specific histological pattern, although some studies have described the presence of necrosis and fibrosis in the endomyocardial biopsy [11][12][13][14] . Some cardiac abnormalities may be associated with NCC: • Presence of Ebstein's anomaly, bicuspid aortic valve and transposition of great vessels 16,17 .…”

Section: Pathogenesis and Genetics Of The Noncompactionmentioning

confidence: 99%

“…Thus, cardiac resonance has become the method of choice to confirm or rule out the diagnosis of NCC, because it provides a more detailed description of the cardiac morphology in any image plane (Figure 2). A ratio of compacted myocardium to noncompacted myocardium > 2.3 produces the highest sensitivity (86%) and specificity (99%) in the diagnosis 34,35 . The effectiveness of the diagnosis made by resonance was evaluated by a study with seven patients with NCC, who were compared with 170 healthy patients, athletes, patients with hypertrophic cardiomyopathy, hypertensive heart disease and aortic stenosis 35 , where the following was observed:…”

Section: Cardiac Magnetic Resonance Imagingmentioning

confidence: 99%

Miocardiopatia não compactada: uma visão atual

Rosa¹,

Salemi²,

Alexandre³

et al. 2011

Arq. Bras. Cardiol.

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Isolated non-compaction cardiomyopathy is a rare disease that is likely to develop in the embryonic period. It is caused by the intrauterine arrest of the myocardial compaction process in the beginning of the fetal development. It is characterized by prominent myocardial trabeculations and deep intertrabecular recesses, as well as the thickening of the myocardium into two distinct layers (compacted and not compacted). Even though this disease is said to be prevalent in the pediatric population or together with congenital heart disease, one can understand that this disease occurs in isolation, because the diagnosis is becoming more common in adult patients that have no other heart disease. The clinical manifestations vary greatly, because they range from absence of symptoms to congestive heart failure, arrhythmias and systemic thromboembolism. Echocardiography is the most widely used diagnostic procedure, but the little knowledge about this disease, its similarity to other myocardial diseases and the limitation of the echocardiographic technique used delay the diagnosis. The purpose of this review is to show that that other imaging techniques, such as MRI, CT and left ventriculography have emerged as diagnostic alternatives.

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Two-Layered Appearance of Noncompaction of the Ventricular Myocardium on Magnetic Resonance Imaging.

Cited by 67 publications

References 13 publications

Left Ventricular Noncompaction Cardiomyopathy Presenting with Heart Failure in a 35-Year-Old Man

Left Ventricular Noncompaction Cardiomyopathy Presenting with Heart Failure in a 35-Year-Old Man

Left ventricular non-compaction cardiomyopathy.

Miocardiopatia não compactada: uma visão atual

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