Prominent Trabeculations Research Articles

Extreme variation in the atrial septation of caecilians (Amphibia: Gymnophiona).

Caecilians (order Gymnophiona) are elongate, limbless, snake-like amphibians that are the sister-group (closest relatives) of all other recent amphibians (frogs and salamanders). Little is known of their cardiovascular anatomy and physiology, but one nearly century old study suggests that Hypogeophis (family Indotyphlidae), commonly relied upon as a representative caecilian species, has atrial septation in the frontal plane and more than one septum. In contrast, in other vertebrates there generally is one atrial septum in the sagittal plane. We studied the adult heart of Idiocranium (also Indotyphlidae) using immunohistochemistry and confirm that the interatrial septum is close to the frontal plane. Additionally, a parallel right atrial septum divides three-fourths of the right atrial cavity of this species. Idiocranium embryos in the Hill collection reveal that atrial septation initiates in the sagittal plane as in other tetrapods. Late developmental stages, however, see a left-ward shift of visceral organs and a concordant rotation of the atria that reorients the atrial septa towards the frontal plane. The gross anatomies of species from six other caecilian families reveal that (i) the right atrial septum developed early in caecilian evolution (only absent in Rhinatrematidae) and that (ii) rotation of the atria evolved later and its degree varies between families. In most vertebrates a prominent atrial trabeculation associates with the sinuatrial valve, the so-called septum spurium, and the right atrial septum seems homologous to this trabeculation but much more developed. The right atrial septum does not appear to be a consequence of body elongation because it is absent in some caecilians and in snakes. The interatrial septum of caecilians shares multiple characters with the atrial septum of lungfishes, salamanders and the embryonic septum primum of amniotes. In conclusion, atrial septation in caecilians is based on evolutionarily conserved structures but possibly exhibits greater variation than in any other vertebrate order.

Left Ventricular Noncompaction

Left ventricular noncompaction (LVNC) cardiomyopathy is a heterogeneous condition that is gaining recognition as a distinct clinical entity. It is characterized by numerous, excessively prominent ventricular trabeculations with deep trabecular recesses and the formation of 2 distinct layers of myocardium. It is often unrecognized clinically for decades and poses distinct life-threatening complications. Accurate diagnosis is key to minimizing risks associated with LVNC. This article will introduce clinicians to the defining criteria and diagnostic process for recognition of LVNC. Left ventricular noncompaction is an important clinical entity not easily recognized at first glance but has important clinical ramifications for treatment requiring an accurate diagnosis. Left ventricular noncompaction is present at birth but can remain clinically silent for decades. It is found both in isolation and as a part of other congenital cardiac, extracardiac, neuromuscular, and genetic syndromes. Left ventricular noncompaction is associated with the major complications of heart failure, tachyarrhythmias, and systemic thromboembolism. Transthoracic echocardiography will likely remain the initial screening modality, but modality limitations, questionable findings, and clinical presentations suspicious for noncompaction should be followed up promptly with cardiac magnetic resonance imaging for definitive diagnosis and treatment.

Rare cause of severely impaired left and right ventricular function--case 7/2014

We report on a case of a 31-year-old patient who was admitted to hospital with acute cardiac failure. At physical examination we found peripheral edema and wet rales. Cardiac ultrasound revealed severely impaired left and right ventricular function and prominent trabeculation in the right ventricle, but also in the apex of the left ventricle. This was confirmed by cardiac magnetic resonance imaging. Both pulmonary embolism and coronary heart disease were excluded by thoracic computed tomography and coronary angiography. These findings were in line with the diagnosis of non-compaction cardiomyopathy resulting in cardiac failure. We introduced diuretic therapy which leads to weight loss and release of dyspnoea. Due to prevention of embolic events an anticoagulative therapy was started. In light of rhythmogenic vulnerability, a subcutaneous implantable cardioverter defibrillator (ICD) system was implanted. Afterwards the patient was discharged in good general health. Non-compaction cardiomyopathy (NCCM) is a hereditary non-ischaemic cardiac disease which occurs due to the failure of myocardial compaction during embryogenesis. Common manifestation is the triad of heart failure, rhythmogenic vulnerability and systemic embolic events. First-line imaging technique for diagnosing NCCM is cardiac ultrasound. The prognosis depends on severity of heart failure, ventricular arrhythmia and the incidence of embolic events.

Guiding the management of ventricular arrhythmias in patients with left ventricular noncompaction cardiomyopathy: a knowledge gap.

Left ventricular noncompaction (LVNC) is a cardiomyopathy that occurs due to an arrest of myocardial maturation during embryogenesis. The diagnostic echocardiographic features in individuals with LVNC include a thick, bilayered myocardium, prominent ventricular trabeculations, and deep intertrabecular recesses. Clinical features associated with LVNC vary in asymptomatic and symptomatic patients, and include the potential for heart failure, conduction defects (eg, left bundle branch block), supraventricular and ventricular arrhythmias, thromboembolic events, and sudden cardiac death. The authors report five cases that emphasize asymptomatic and apparently benign symptoms in patients with LVNC; despite normal physical examination and 12-lead electrocardiogram results, all of these cases unveiled potentially serious clinical consequences. These cases highlight the concern that LVNC patients with mild to moderate left ventricular systolic dysfunction, particularly in the presence of ventricular arrhythmias or a family history of sudden cardiac death, may need consideration for an implantable cardioverter defibrillator (ICD). All potential benefits of an ICD need to be balanced by the risk of device infection, lead and device malfunction, and potential for inappropriate shocks.

Can intensive exercise harm the heart? You can get too much of a good thing.

Can intensive exercise harm the heart? You can get too much of a good thing.

Characteristics of Trabeculated Myocardium Burden in Young and Apparently Healthy Adults

Increased myocardial trabeculations define noncompaction cardiomyopathy (NCC). Imaging advancements have led to increasingly common identification of prominent trabeculations with unknown implications. We quantified and determined the impact of trabeculations' burden on cardiac function and stretch in a population of healthy young adults. One hundred adults aged 18 to 35 years (28±4 years, 55% women) without known cardiovascular disease were prospectively studied by cardiovascular magnetic resonance. Left ventricular (LV) volumes, segmental function, and ejection fraction (EF) and left atrial volumes were determined. Thickness and area of trabeculated (T) and dense (D) myocardium were measured for each standardized LV segment. N-terminal pro-brain natriuretic peptide (Nt-pro-BNP) was measured. Eighteen percent of the subjects had ≥1 positive traditional criteria for NCC, and 11% meet new proposed NCC cardiovascular magnetic resonance criteria. Trabeculated over dense myocardium ratio (T/D) ratios were uniformly greater at end-diastole versus end-systole (0.90±0.25 vs 0.42±0.13, p<0.0001), in women versus men (0.85±0.24 vs 0.72±0.19, p=0.006), at anterior versus nonanterior segments (1.41±0.59 vs 0.88±0.35, p<0.0001), and at apical versus nonapical segments (1.31±0.56 vs 0.87±0.38, p<0.0001). The largest T/D ratios were associated with lower LVEF (57.0±5.3 vs 62±5.5, p=0.0001) and greater Nt-pro-BNP (203±98 vs 155±103, p=0.04). Multivariable regression identified greater end-systolic T/D ratios as the strongest independent predictor of lower LVEF, beyond age and gender, left atrial or LV volumes, and Nt-pro-BNP (β=-9.9, 95% CI -15 to 4.9, p<0.001). In conclusion, healthy adults possess variable amounts of trabeculations that regularly meet criteria for NCC. Greater trabeculations are associated with decreased LV function. Apparently healthy young adults with increased trabecular burden possess evidence of mildly impaired cardiac function.

Isolated Non-Compacted Right Ventricular Myocardium with Severe Pulmonary Hypertension

Abstract Background: There have been only at least seven cases of isolated right ventricular non-compaction reported in literature, hence, there are no definite criteria for diagnosis and recommendations for management of these patients. The reported clinical manifestations include heart failure, arrhythmias and cardioembolic events. Left ventricular and biventricular non-compaction are likewise rare but more common than isolated right ventricular non-compaction. Pulmonary hypertension is associated with biventricular non-compaction more commonly but not with isolated right ventricular non-compaction. Setting: University of Santo Tomas Hospital Case report: We report a case of a 25 year old female who presented with progressive dyspnea for one year accompanied by easy fatigability. She denied chest pain, palpitations, pedal edema, paroxysmal nocturnal dyspnea and orthopnea. She had a brother who died at a young age reportedly due to a cardiac illness which was not fully worked-up. Her 2D echocardiogram showed excessive prominent trabeculations and deep inter-trabecular recesses in the right ventricular wall with depressed right ventricular systolic function by tricuspid annular plane systolic excursion and fractional area change. Color Doppler studies also showed severe tricuspid regurgitation, pulmonic regurgitation and severe pulmonary hypertension. Secondary causes of pulmonary hypertension like connective tissue diseases, left heart disease, chronic thrombotic/embolic disease, anomalous cardiac and pulmonary shunts and lung disease were excluded. She was given diltiazem, sildenafil, digoxin, warfarin overlapped with subcutaneous enoxaparin and oxygen supplementation which provided symptomatic relief.

125 Cardiovascular Magnetic Resonance is Valuable in the Investigation of Aborted Sudden Cardiac Death

IntroductionAborted sudden cardiac death (SCD) is an infrequent indication for cardiovascular magnetic resonance (CMR). Patients who survive an arrhythmic cardiac arrest usually receive an implantable cardioverter-defibrillator (ICD). It is important...

B Embryogenesis of Ventricular Myocardial Trabeculae – Novel Insights from Episcopic 3D Imaging and Fractal Analysis of Wild-type and Notch MIB1 Noncompaction Mouse Models

Introduction Left ventricular noncompaction (LVNC) is characterised by prominent ventricular myocardial trabeculations, composed of sheets of cardiomyocytes. They form early during cardiogenesis but their development is complex. Measuring trabecular complexity...

LONG-TERM OUTCOMES IN PATIENTS WITH LEFT VENTRICULAR NONCOMPACTION AND IMPLANTABLE CARDIOVERTER-DEFIBRILLATORS

Left ventricular non-compaction (LVNC) is a rare, genetic cardiomyopathy associated with heart failure, stroke, and fatal arrhythmias. It is characterized by prominent trabeculations in the myocardium. Due to its recent establishment as a diagnosis, there is little consensus regarding best

Clinical and prognosis of the children with noncompaction of ventricular myocardium

Noncompaction of ventricular myocardium (NVM) is reported as a genetic disease characterized by excessively prominent trabeculations and deep intertrabecular recesses.The etiology and pathogenesis are unclear.NVM is a rare disease with poor prognosis.The purpose of this areide is to review NVM manifestations and prognosis,to enhance the awareness of NVM,to improve the diagnosis and treatment,to ensure a better life quality and to increase the lifetime of NVM patients. Key words: Noncompaction of ventricular myocardium; Manifestation; Prognosis; Children

Isolated non-compaction of the left ventricle

Isolated left ventricular non-compaction (LVNC) is a rare congenital disorder caused by failure of normal embryogenesis of the endocardium and myocardium in utero, resulting in prominent ventricular trabeculations with deep intertrabecular recesses. Patients may be asymptomatic or may present with refractory cardiac failure. The best modality of diagnosis is based on echocardiographic diagnostic criteria. Prognosis remains poor for patients with impaired systolic left ventricular function, as treatment options are very limited. We report the case of 2-month-old infant presenting with congestive cardiac failure diagnosed as isolated LVNC on echocardiography.

Subclinical systolic dysfunction in left ventricular non-compaction cardiomyopathy unmasked by contrast echocardiography

An asymptomatic 28-year-old man, first-degree relative of a patient with left ventricular non-compaction (LVNC), underwent an echocardiographic screening. Two-dimensional transthoracic echocardiography demonstrated normal left ventricular (LV) dimensions (end-diastolic diameter of 49mm) with apparently normal systolic function. Characteristic, prominent numerous trabeculations in the LV apex and inferior, posterior, and lateral wall were noticed (Fig. 1 and Movie I). The color Doppler imaging demonstrated communication between intertrabecular spaces and the LV cavity.

Defining Left Ventricular Noncompaction Using Cardiac Computed Tomography

Left ventricular noncompaction (LVNC) is a cardiomyopathy characterized by a distinctive 2-layered appearance of the myocardium because of increased trabeculation and deep intertrabecular recesses. Echocardiography serves as the initial noninvasive diagnostic test. Currently, magnetic resonance imaging (MRI) is increasingly being used to diagnose LVNC because of its improved temporal and spatial resolution. So far, no criteria have been proposed to define pathologic LVNC with the use of computed tomography (CT). We analyzed CT images using an American Heart Association 17-segment model in 8 patients previously diagnosed with LVNC by clinical diagnosis, echocardiography, and/or MRI, as well as in 11 patients with nonischemic dilated cardiomyopathy, 11 patients with hypertrophic cardiomyopathy, 10 patients with severe aortic stenosis, 9 patients with severe aortic regurgitation, 10 patients with left ventricular hypertrophy due to essential hypertension, and, additionally, in a control group of 20 patients who had normal CT scans without a history of cardiovascular disease. The distribution of LVNC was assessed by qualitative analysis of 17 myocardial segments for the presence or absence of any degree of noncompaction. Each segment was analyzed in each of the 3 end-diastolic long-axis views for the presence or absence of noncompaction, and the most prominent trabeculation was chosen for measurement. The left ventricular apex was excluded. Thickness of noncompacted and compacted myocardium was measured perpendicular to the compacted myocardium. The ratio of noncompacted to compacted (NC:C) myocardium was calculated for each segment. Receiver operating characteristics were used to generate cutoff values with sensitivity and specificity to distinguish the LVNC group from other groups. An end-diastolic NC:C ratio >2.3 distinguished pathologic LVNC with 88% sensitivity and 97% specificity; positive and negative predictive values were 78% and 99%, respectively. CT using the standard MRI NC:C ratio cutoff >2.3 accurately characterizes pathologic LVNC.

Dilated cardiomyopathy - three brothers and a BAG3 mutation

We report a case of left ventricular non-compaction (LVNC) associated with hereditary autosomal dominant polycystic kidney disease (ADPKD). A 24 year-old male was referred with a six month history of New York Heart Association (NHYA) Class III exercise intolerance. Past medical history included stage III chronic kidney disease secondary to autosomal dominant polycystic kidney disease (ADPKD). Apart from ADPKD there was no other family history. Echocardiogram showed severe left ventricular dilatation (left ventricular end diastolic volume 240 mL) with ejection fraction of 60%. There was posterior leaf mitral valve prolapse (MVP) and grade 4/4 mitral regurgitation. Cardiac magnetic resonance imaging (cMRI) confirmed non-compacted to compacted myocardium ratio 3.4 (N < 2.3). LVNC is an inherited cardiac cause of ventricular failure. Prominent ventricular muscle trabeculations with corresponding deep recesses communicating with the left ventricular cavity are the hallmark of the condition. Multiple genetic loci have been implicated, including loci that overlap with other types of cardiomyopathies. ADPKD is an hereditary cause of chronic kidney disease secondary to mutations in the PKD1 and PKD2 genes. ADPKD is characterised by the presence of multiple renal cysts. Cardiovascular complications have the greatest impact on morbidity and mortality in patients with ADPKD. Valvular abnormalities especially MVP are common. Boulter et al (2001) showed that homozygote deletion of PKD1 in mice resulted in severe cardiovascular anomalies that included hypertrabeculation, myocardial thinning, and septation defects. Our case suggests the existence of a syndromic association between ADPKD, LVNC, and possibly MVP that needs further elucidation at both clinical and molecular levels.

Left Ventricular Non-Compaction Syndrome Misdiagnosed as Dilated Cardiomyopathy on Several Occasions, Presenting With Recurrent Stroke

A 57-year-old African American female with a history of ischemic cardiomyopathy and a recent stroke with no residual deficits presented with apraxia and confusion. Non-contrast CT scan of the head revealed multiple embolic strokes in both cerebral hemispheres. Transthoracic echocardiography raised the suspicion for increased trabecular meshwork in the left ventricle. Cardiac MRI confirmed the findings of isolated left ventricular non-compaction (LVNC) syndrome. A contrast-enhanced transesophageal echocardiogram demonstrated the characteristic features of this unusual disease with the additional demonstration of contrast filling the trabecular meshwork. Interestingly multiple transthoracic echocardiograms in the past had failed to identify myocardial non-compaction. The patient was started on warfarin for prophylactic anticoagulation and an implantable defibrillator was placed to lower the risk of sudden death. LVNC is a rare type of genetic cardiomyopathy characterized by excessively prominent trabeculations and deep inter-trabecular recesses in the ventricle wall. Non-compaction remains frequently overlooked even by experienced echocardiographers. Failure to diagnosis may lead to insufficient treatment since it is often associated with a risk of thromboembolism, life-threatening arrhythmias and sudden death. Furthermore, because of the familial association described with ventricular non-compaction, screening of first relatives with echocardiography is recommended.

Rare combination of left ventricular noncompaction, bicuspid aortic valve and myocardial bridging. Rare case or common genetic mutations?

Left ventricular noncompaction is a relatively common genetic cardiomyopathy. In most cases, noncompaction is an isolated disease confined to the left ventricular myocardium, characterized by prominent trabeculations with deep intertrabecular recesses. It may be present also in various types of congenital heart diseases [1–20]. We present the case of a 36-year-old man, who came under our observation for stable angina pectoris. He has a history of severe arterial hypertension controlled pharmacologically and a known aortic bicuspid valve with normal flow. Echocardiographic evaluation confirms bicuspid aorta (Fig. 1A) with normal flow, associated with a mild trabecular apical hypertrophy (Fig. 1B). The apexwas also ipokynetic. Coronaric arteriography showed the absence of coronaric lesions (Fig. 1C andD) and the presence of a myocardial bridging (Fig. 2A and B). Ventriculography showed that the contrast mediumwas inserted in small intertrabecular recesses (Fig. 2C), suggesting the existence of a minor form of noncompacted myocardium [21–33]. This findingmade us suspect that different congenital heart diseases could have the same genetical background. In conclusion,we cannot ascertain from this single case the existence of a common genetic background for this association of congenital heart diseases (myocardial noncompaction, bicuspid aortic valve and myocardial bridging). However, the identification of further cases could identify a potentially

Acquired Fontan Physiology Secondary to Acute Mechanical Tricuspid Valve Failure in a Patient With Massive Right Heart Enlargement and Ebstein’s Anomaly

A 25-year-old man with Ebstein’s anomaly and a secundum atrial septal defect had a mechanical tricuspid valve replacement (St. Jude 31-mm valve) and atrial septal defect closure when he was 15 years old. He was under long-term specialist follow-up and had recently been admitted with symptoms and signs of right heart failure secondary to new onset atrial flutter. He was direct-current cardioverted and commenced on ramipril and metoprolol. He then went to have an electrophysiological study and successful atrial flutter ablation. At review 6 months later he reported good exercise tolerance and was now able to play 18 holes of golf, whereas previously he could only manage 9 holes. However, he commented that since the electrophysiological procedure he had been unable to hear his prosthetic valve clicks. On clinical examination he was not cyanosed, the jugular venous pressure was elevated at 4 cm without prominent V waves, there was a soft first heart sound, no audible mechanical valve clicks, and a pansystolic murmur. There were no signs of heart failure or peripheral edema. An electrocardiogram demonstrated sinus rhythm, right axis deviation, first-degree heart block, and right bundle-branch block with QRS duration of 200 ms. Echocardiography and fluoroscopy (Figure 1) showed that the leaflets of the …

Biventricular non-compaction demonstrated on multi-slice computed tomography with echocardiographic correlation

Left ventricular non-compaction (LVNC) is a myocardial disorder characterized by prominent trabeculations and deep intertrabecular recesses within the left ventricular wall. Multi-slice computed tomography (CT) might represent a valid non-invasive imaging technique for the diagnostic work-up of these patients, being able to visualize the compacted and non-compacted layers and to simultaneously rule out the presence of associated coronary artery disease and congenital heart disease. In the present report, the CT features of LVNC are described.

Left ventricular non-compaction: troubles and traps of current imaging techniques

Left ventricular non-compaction (LVNC) is historically considered to be a rare form of cardiomyopathy characterized by excessive and prominent trabeculations associated with deep recesses that communicate with the ventricular cavity.1 Given that prominent trabeculations are a normal feature of the intrauterine developing myocardium, LVNC is postulated to be caused by an arrest of trabecular regression that occurs during normal embryonic development.2 Nevertheless, there has been no proof of an arrest in embryonic endomyocardial morphogenesis. Moreover, both cases of de novo acquired LVNC and cases of non-compaction disappearance have been reported in the literature,3,4 calling into question the developmental hypothesis. The diagnosis of LVNC is based on the identification of a two-layer myocardium, which is thin and compacted adjacent to the epicardium and non-compacted (NC) near the endocardium.1 However, even if frequently overlooked in the past, the inner surface of the ventricles is not smooth and prominent LV trabeculations are present even in normal hearts as shown both at post-mortem inspection5 and by imaging.6 This raises the …