Noncompaction Of Myocardium Research Articles

An unusual case of myocardial noncompaction involving both ventricles-Case report

Noncompaction of ventricular myocardium (NCVM) is a rare congenital cardiomyopathy characterized by numerous excessively prominent trabeculations and intertrabecular recesses in the myocardium without any other cardiac structural abnormality. Transthoracic echocardiography is the imaging modality of choice for diagnosing NCVM. Although this disease commonly affects the left ventricle, the right ventricle or both ventricles can rarely be affected. We report the case of an 18-year-old male patient with NCVM involving both ventricles.

Trabeculated (Noncompacted) and Compact Myocardium in Adults

Background— A high degree of noncompacted (trabeculated) myocardium in relationship to compact myocardium (trabeculated to compact myocardium [T/M] ratio >2.3) has been associated with a diagnosis of left ventricular noncompaction (LVNC). The purpose of this study was to determine the normal range of the T/M ratio in a large population-based study and to examine the relationship to demographic and clinical parameters. Methods and Results— The thickness of trabeculation and the compact myocardium were measured in 8 left ventricular regions on long axis cardiac MR steady-state free precession cine images in 1000 participants (551 women; 68.1±8.9 years) of the Multi-Ethnic Study of Atherosclerosis cohort. Of 323 participants without cardiac disease or hypertension and with all regions evaluable, 140 (43%) had a T/M ratio >2.3 in at least 1 region; in 20 of 323 (6%), T/M >2.3 was present in >2 regions. A multivariable linear regression model revealed no association of age, sex, ethnicity, height, and weight with maximum T/M ratio in participants without cardiac disease or hypertension ( P >0.05). In the entire cohort (n=1000), left ventricular ejection fraction (β=−0.02/%; P =0.015), left ventricular end-diastolic volume (β=0.01/mL; P <0.0001), and left ventricular end-systolic volume (β=0.01/mL; P <0.001) were associated with maximum T/M ratio in adjusted models, whereas there was no association with hypertension or myocardial infarction ( P >0.05). At the apical level, T/M ratios were significantly lower when obtained on short- compared with long-axis images ( P =0.017). Conclusions— A ratio of T/M of >2.3 is common in a large population-based cohort. These results suggest re-evaluation of the current cardiac MR criteria for left ventricular noncompaction may be necessary.

Tafazzin Knockdown in Mice Leads to a Developmental Cardiomyopathy With Early Diastolic Dysfunction Preceding Myocardial Noncompaction

BackgroundBarth syndrome is a rare, multisystem disorder caused by mutations in tafazzin that lead to cardiolipin deficiency and mitochondrial abnormalities. Patients most commonly develop an early-onset cardiomyopathy in infancy or fetal life.Methods and ResultsKnockdown of tafazzin (TAZKD) in a mouse model was induced from the start of gestation via a doxycycline-inducible shRNA transgenic approach. All liveborn TAZKD mice died within the neonatal period, and in vivo echocardiography revealed prenatal loss of TAZKD embryos at E12.5-14.5. TAZKD E13.5 embryos and newborn mice demonstrated significant tafazzin knockdown, and mass spectrometry analysis of hearts revealed abnormal cardiolipin profiles typical of Barth syndrome. Electron microscopy of TAZKD hearts demonstrated ultrastructural abnormalities in mitochondria at both E13.5 and newborn stages. Newborn TAZKD mice exhibited a significant reduction in total mitochondrial area, smaller size of individual mitochondria, reduced cristae density, and disruption of the normal parallel orientation between mitochondria and sarcomeres. Echocardiography of E13.5 and newborn TAZKD mice showed good systolic function, but early diastolic dysfunction was evident from an abnormal flow pattern in the dorsal aorta. Strikingly, histology of E13.5 and newborn TAZKD hearts showed myocardial thinning, hypertrabeculation and noncompaction, and defective ventricular septation. Altered cellular proliferation occurring within a narrow developmental window accompanied the myocardial hypertrabeculation-noncompaction.ConclusionsIn this murine model, tafazzin deficiency leads to a unique developmental cardiomyopathy characterized by ventricular myocardial hypertrabeculation-noncompaction and early lethality. A central role of cardiolipin and mitochondrial functioning is strongly implicated in cardiomyocyte differentiation and myocardial patterning required for heart development. (J Am Heart Assoc. 2012;1:jah3-e000455 doi: 10.1161/JAHA.111.000455.)

Prenatal diagnosis of fetal left ventricular non‐compaction cardiomyopathy

Isolated left ventricular non-compaction (LVNC) is a rare disorder, classified as a primary genetic cardiomyopathy by the American Heart Association or as an unclassified cardiomyopathy by the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. The key features are the prominent trabeculae and deep intratrabecular recesses resulting in thickened myocardium with the two layers consisting of compacted and non-compacted myocardium. These recesses are in continuity with the left ventricular cavity and are filled with blood without evidence of communication to the epicardial coronary artery system. We present a case of LVNC detected prenatally at 25 + 4 weeks of gestation.

Development of heart failure and stroke in the setting of myocardial noncompaction

We present a 30-year-old male patient who developed new-onset heart failure and stroke in the setting of myocardial noncompaction. He presented with shortness of breath that progressively worsened in the past 15 days. Chest X-ray showed diffuse bilateral infiltration with bilateral hilar fullness. Electrocardiography showed sinus rhythm, signs of left ventricular hypertrophy, and diffuse T-wave negativity. Transthoracic echocardiography demonstrated moderate systolic dysfunction of the left ventricle and trabeculations and intertrabecular recesses in the anterolateral and apical regions of the inferior wall. Dense spontaneous echo contrast was seen in the left ventricular cavity. He also had a bicuspid aortic valve. The patient developed stroke at the eighth hour of hospitalization. Heparin infusion and heart failure treatment were started. Transesophageal echocardiography showed no thrombus. Clinical and radiological findings improved significantly during the follow-up. Oral anticoagulant therapy was initiated and the patient was discharged with control recommendations. Myocardial noncompaction was also demonstrated by cardiac magnetic resonance imaging.

Three-Dimensional Echocardiographic Characterization of Patients with Left Ventricular Noncompaction

Despite several efforts using two-dimensional echocardiography and cardiac magnetic resonance in the diagnosis of left ventricular noncompaction (LVNC), there are no universally accepted diagnostic criteria. The aim of this study was to describe the extent of noncompacted myocardium using a new three-dimensional echocardiographic parameter. Seventeen patients with diagnoses of LVNC on the basis of two-dimensional echocardiographic and clinical criteria, 26 Olympic rowing athletes, and 49 healthy volunteers underwent three-dimensional echocardiography. By offline analysis, left ventricular volumes, mass, ejection fraction, and sphericity index were calculated. Trabeculated left ventricular volume (TLV) was calculated as the difference between left ventricular end-diastolic volume obtained including and excluding the trabeculae in the cavity contour. TLV was also normalized by left ventricular end-diastolic volume (TLV%). TLV and TLV% were significantly higher in patients with LVNC (33.7 ± 10.9 mL and 24 ± 7%) as opposed to controls (7.1 ± 2.2 mL, P < .001, and 6 ± 2%, P < .001, respectively) and athletes (8.0 ± 3.0 mL, P < .001, and 5 ± 2%, P < .001, respectively). In detail, on receiver operating characteristic curve analysis, optimal cutoff values of 15.8 mL for TLV and 12.8% for TLV% were determined for the identification of LVNC (area under the curve, 1.00; P < .001). Mild positive correlations of TLV and TLV% were found with sphericity index (r = 0.294, P = .004, and r = 0.301, P = .004, respectively), and mild negative correlations were found with ejection fraction (r = -0.454, P < .001, and r = -0.217, P = .038, respectively). Because of high spatial resolution and accuracy in volumetric quantification, three-dimensional echocardiography allows accurate measurement of the extent of noncompacted myocardium and identification of patients with LVNC.

Echocardiographic diagnosis of non-compaction of the ventricular myocardium associated with hypertrophic cardiomyopathy in kindred

PurposeNon-compaction of the ventricular myocardium (NVM) is a cardiomyopathy caused by arrest of normal embryogenesis of the endocardium and myocardium with some gene disorders, including hypertrophic cardiomyopathy (HCM). However, familial...

The myocardial architecture of cor triloculare biatrium resembling reptiles

The reptilian heart includes a three-chamber: right and left atria and one partially divided ventricle. This is made up the non-compacted myocardium which includes ventricular trabeculations. Reptiles, which are poikilothermic, lack an advanced coronary tree in contrary to humans and the trabecular …

Rare Visualization of Entrapped Left Ventricular Thrombi in Noncompacted Myocardium

A 64-year-old man with no significant medical history presented to the emergency department with severe acute heart failure. Initial management was based on oxygen therapy, diuretics, and nitroglycerin. Twenty-four hours after admission, the patient developed aphasia and left arm weakness. Brain MRI showed multiple strokes (Figure A) compatible with cardiac emboli. Echocardiography showed dilated cardiomyopathy with severe left ventricular (LV) dysfunction (ejection …

Noncompacted ventricular myocardium: characterization by intracardiac echo

We report a patient with clinical manifestation of arrhythmias and evidence of noncompacted myocardium in both left and right ventricular apex. The diagnosis was made with intracardiac echo performed during the electrophysiologic study. This method has allowed the diagnosis of noncompaction of the ventricular myocardium due to its high resolution. Color Doppler showed trabecular recesses in communication with the ventricular cavity that could not be identified with transthoracic echocardiography.

Regional and global ventricular systolic function in isolated ventricular non-compaction: Pathophysiological insights from magnetic resonance imaging

BackgroundIsolated ventricular non-compaction (IVNC) is frequently, but not invariably, associated with left ventricular (LV) systolic dysfunction. Factors impacting on regional and global LV function are unknown. The aim of the study was to apply magnetic resonance imaging (MRI) to evaluate the impact of extent and severity of ventricular non-compaction on LV systolic function in patients with IVNC. MethodsSixteen adult patients with IVNC as defined by previously validated MRI criteria [ratio between end-diastolic thickness of non-compacted and compacted myocardium (NC/C ratio)>2.3 in ≥1 LV segment] were enrolled. Short-axis cine images were employed for analysis. Applying a 16-segment LV model, regional systolic performance was assessed qualitatively (wall motion score, WMS; 1 = normal, 2 = mild hypokinesia, 3 = moderate-to-severe hypokinesia, and 4 = a/dyskinesia) as well as quantitatively [fractional wall thickening, FWT (%)=100×(end-diastolic wall thickness−end-systolic wall thickness)/end-diastolic wall thickness)]. ResultsMean LV ejection fraction was 43.8±15.4% (range, 17–68%). Regional disease severity, as expressed by the NC/C ratio, revealed a significant correlation with WMS (r=0.26; p=0.018) and FWT (r=−0.30; p=0.006). The total number of non-compacted segments/patient (NoNC) as an index of disease extent was a significant independent correlate of LV ejection fraction by multivariate regression analysis (β=−5.24; p=0.038) and an excellent predictor of global LV dysfunction (ROC analysis, AUC=0.98; p<0.0001). ConclusionsIn patients with IVNC, disease severity correlates with the degree of LV dysfunction at a regional level. The extent of myocardial non-compaction is an independent predictor of global LV dysfunction.

Non-compaction du ventricule droit : à propos de deux cas

BackgroundThe noncompaction of myocardium is a rare myocardiopathy. The isolated right ventricular involvement is exceptional. We report two cases of isolated noncompaction of the right ventricular myocardium. Case 1I., 2years old, investigated for a congenital cyanosis with severe functional repercussion. Her echocardiography revealed a complex congenital heart disease associated with isolate noncompaction of the right ventricle. Case 2A., 5years old, was referred for history of syncope for the 2 last years. The echocardiography showed a severe pulmonary hypertension and an isolate noncompaction of the right ventricle. ConclusionDue to the rarity of the isolate non-compaction of the right ventricle, it diagnosis remains difficult. The treatment and the outcomes of this disease are still unclear. An early diagnosis may help to reduce its complication.

A case report of left ventricular non-compaction in progression to dilated cardiomyopathy: Diagnostic utilization of multiple imaging modalities

Left ventricular non-compaction, which is characterized by left ventricular hypertrabeculation, is a rare congenital cardiomyopathy. The changing and progressing morphology, with different clinical manifestations, made the diagnosis difficult. Here is a case report of a middle-age woman, who had exercise intolerance for many years. Traditional echocardiography showed findings of dilated cardiomyopathy but obscure trabeculations, which was clearly seen by three-dimensional echocardiography. Subsequent cardiac magnetic resonance imaging well demonstrated the myocardial non-compaction and delayed enhancement. LVNC could be found early in a child, and also in a mid-age adult. When a patient with LVNC had progressing heart failure, the hypertrabeculation may become less prominent. The utilization of three-dimensional echocardiography and MRI would help identify such landmark findings.

A Rare Case of Coronary Artery Bypass Grafting in a Patient With Left Ventricular Noncompaction

Ventricular myocardium noncompaction is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. Initially the noncompaction of the left ventricle was described in the pediatrics population with poor prognosis, but recent reports have noticed the presence of this pathology in the adult population. We describe a 54-year-old man with isolated noncompaction of the left ventricle who had ischemic heart disease and was successfully treated with bypass surgery.

Cardiac magnetic resonance imaging characteristics of isolated left ventricular noncompaction in a Chinese adult Han population

To analyze cardiac magnetic resonance imaging (CMR) characteristics in patients with isolated left ventricular noncompaction (IVNC) and assess its value in the diagnosis of IVNC in a Chinese adult Han population. We collected a consecutive series of 30 patients with IVNC from January 1, 2007, to December 31, 2008. During the same period, we prospectively included patients drawn from groups given a potential differential diagnosis for IVNC. All magnetic resonance images were analyzed using 17-segment model. Left ventricular ejection fraction was significantly lower for patients with DCM (16.2±5.2%, P<0.001) and higher in AR (47.6±16.2%, P=0.009), AS (54.6±21.1%, P=0.001) and HHD (62.4±6.8%, P<0.001) compared with IVNC (33.0±14.1%). The two-layered structure was most frequently seen at the apical segments, followed by the mid-cavity and basal segments in patients with INVC. The anterior and lateral walls were more commonly involved in patients with IVNC. The number of noncompacted segments and end-diastolic ratio of non-compacted to compacted myocardium (NC/C ratio) was greater in patients with IVNC than in other five groups. The end-diastolic NC/C ratio of >2.5 had 96.4% sensitivity and 97.4% specificity for identifying patients with IVNC. CMR provides an accurate and reliable evaluation of the localization and extent of noncompacted myocardium at end-diastole. The end-diastolic NC/C ratio of >2.5 had high diagnostic accuracy for IVNC in a Chinese adult Han population.

E0689 Echocardiographic characteristics of left ventricular noncompaction with papillary muscles involvement

ObjectiveTo assess the ultrasonic characteristics of the left ventricular noncompaction with papillary muscles involvement.MethodsWe reviewed the echocardiographic findings in eight patients having left ventricular noncompaction with papillary muscles involvement at...

Approach to Incidentally Diagnosed Isolated Ventricular Noncompaction of Myocardium

Isolated ventricular noncompaction (IVNC) is a congenital cardiomyopathy characterized by a distinctive ("spongy") morphological appearance of the left ventricular (LV) myocardium. It is rare but, with improved imaging modalities and awareness, it is being reported more frequently. Clinical manifestations may range from being asymptomatic to heart failure, arrhythmias, sudden cardiac death, and systemic thromboembolism. With the increasing reporting frequency, the implications of incidentally diagnosed noncompaction need to be addressed. We came across two cases at our medical center which raised important practical questions.

Left Ventricular Noncompaction Cardiomyopathy: What Do We Know?

Noncompaction is a rare and primary genetic cardiomyopathy affecting the left ventricle. The diagnosis is usually established by echocardiography, or, less frequently, by left ventriculography. Cardiac magnetic resonance and electrocardiography-gated multi-detector computed tomography are evolving noninvasive modalities to image cardiac structures, and have the utility to detect noncompacted myocardium. Estimates of the frequency and incidence of left ventricular noncompaction (LVNC) are limited because of the controversy over whether LVNC is a discrete disease entity. There is considerable overlap with dilated cardiomyopathy, apical hypertrophy, and hypertrophic cardiomyopathy. Symptoms, diagnosis, and prognosis are variable because of the heterogeneous nature of these diseases, making treatment often empirical and mimicking the treatment of other cardiomyopathies. However, there are management issues that should be addressed in each patient with LVNC, including genetic testing and family screening, the need for implantable cardioverter defibrillator placement, the role of anticoagulation in prevention of thromboembolic complications, and prescriptions/restrictions for implementation of physical activity.

Sudden Unexpected Death Due to Left Ventricular Noncompaction of Myocardium

Left ventricular noncompaction of the myocardium is a rare genetic cardiac disease characterized by a prominent meshwork and deep intertrabecular recesses of the left ventricle. The condition has frequently been misdiagnosed as other cardiomyopathies. Sudden deaths due to the disease are rarely reported in the literature of forensic sciences. We report 2 cases of sudden death due to undiagnosed left ventricular noncompaction. One was a 30-year-old man who suddenly collapsed while eating breakfast. He was pronounced dead on arrival at the hospital. The other 24-year-old man had a witness collapse at home. Despite immediate resuscitation, he expired 11 hours after hospitalization. Postmortem examination revealed that both patients died of left ventricular noncompaction. The key pathologic findings are described. Additionally, the literature is reviewed, and the incidence, clinical and pathophysiologic presentation, pathogenesis, and diagnostic criteria for the left ventricular noncompaction are discussed.

Transplante cardíaco em criança com miocárdio não compactado

Article received on May 10th, 2010 Article accepted on June 1st, 2010 CLINICAL DATA 1 year and 7 months old male child, 11 kg, from Olimpia (SP). Referred to the Department of Pediatric Cardiology in Sao Jose do Rio Preto for investigation of heart disease. Clinical history of fatigue since the three months, with tachypnea under efforts, bronchospasm and difficulty in gaining weight. With 1 year of age he was admitted to the Intensive Care Unit of Pediatric Cardiology (UTICP) for presentation of bronchospasm and severe tachypnea, showing signs of decompensated congestive heart failure (dCHF). On physical examination, the patient was in regular general state, ruddy, hydrated, dyspneic and afebrile. The heartbeat was regular with two clicks, without murmur. Lung auscultation revealed bilateral vesicular murmur, with breath sounds in the bases. In the abdomen, hepatomegaly called our attention. Blood pressures and pulse were normal in four limbs without edema. After further examination, it became clear the diagnosis of non-compacted myocardium and thrombi in the left ventricle (LV), and we initiated measures for the treatment of dCHF and vigorous anticoagulation. During this hospitalization, the child presented an episode of cardiac arrest, but without sequelae. All medications for the treatment of dCHF were optimized but the child still presented constant admissions due to severe myocardial dysfunction. After four months in UTICP under intravenous vasoactive drugs and numerous additional tests, it was possible to perform cavitary transplant.