Left Ventricular Non-compaction Cardiomyopathy: A Report of a Rare Case From Saudi Arabia.

Left ventricular non-compaction cardiomyopathy (LVNC) is an unusual congenital heart disease that predominantly affects the heart's left ventricle. This disease is characterized by deep intertrabecular recesses and hypertrabeculations of the myocardial wall that link with the ventricle cavity. During embryogenesis, the fetal myocardium has to undergo a compaction process, wherein the trabeculated and spongy myocardial tissue compacts into a dense, solid form. An incomplete compaction process results in persistent non-compacted spongy myocardial tissue and trabeculations prominent in the left ventricle. This disease could be marked alone or be present in coexistence with other congenital heart abnormalities. We present a rare case of a 57-year-old Saudi male who presented to the ER with chest pain and dyspnea. Due to severe chest pain, he was admitted to the coronary care unit. On further investigation, an echocardiogram revealed heavy trabeculations in the dilated left ventricle and a reduced ejection fraction. The case was diagnosed as LVNC and possible heart failure. The patient was discharged after he was kept under guideline-directed medical therapy (GDMT) along with certain medications and will be evaluated after six months of GDMT to decide on implantable cardiac resynchronization therapy. Although LVNC is rare, it can lead to severe heart conditions like arrhythmias, thromboembolism, and heart failure.

Left Ventricular Trabeculation: Arrhythmogenic and Clinical Significance in Elite Athletes.

INTRODUCTION: Left ventricular (LV) trabeculations (LVTs) are common findings in athletes. Limited information exists regarding clinical significance, management, and outcome. OBJECTIVES: The purpose of this study is to examine the prevalence and morphologic characteristics of LVTs in elite athletes, with a focus on clinical correlates and prognostic significance. METHODS: We enrolled 1,492 Olympic elite athletes of different sports disciplines with electrocardiogram, echocardiogram, and exercise stress test. Individuals with a definite diagnosis of LV noncompaction (LVNC) were excluded; we focused on athletes with LVTs not meeting the criteria for LVNC. RESULTS: Four hundred thirty-five (29.1%) athletes presented with LVTs, which were more frequent in male athletes (62.1% vs 53.5%, P = .002) and Black athletes compared with Caucasian (7.1% vs 2.4%, P < .0001) and endurance athletes (P = .0005). No differences were found with relation to either the site or extent of trabeculations. Endurance athletes showed a higher proportion of LVTs and larger LV volumes (end-diastolic and end-systolic, respectively, 91.5 ± 19.8 mL vs 79.3 ± 29.9 mL, P = .002; and 33.1 ± 10 mL vs 28.6 ± 11.7 mL, P = .007) and diastolic pattern with higher E wave (P = .01) and e' septal velocities (P = .02). Ventricular arrhythmias were found in 14% of LVTs versus 11.6% of athletes without LVTs (P = .22). Neither the location nor the LVTs' extension were correlated to ventricular arrhythmias. At 52 ± 32 months of follow-up, no differences in arrhythmic burden were observed (11.1% in LVT athletes vs 10.2%, P = .51). CONCLUSIONS: Left ventricular trabeculations are quite common in athletes, mostly male, Black, and endurance, likely as the expression of adaptive remodeling. In the absence of associated clinical abnormalities, such as LV systolic and diastolic impairment, electrocardiogram repolarization abnormalities, or family evidence of cardiomyopathy, athletes with LVTs have benign clinical significance and should not require further investigation.

Overlapping Phenotypes of Alcoholic Cardiomyopathy and Left Ventricular Non-compaction: A Case Report and Discussion of Converging Cardiomyopathies.

Left ventricular non-compaction cardiomyopathy, often known as LVNC, is a form of congenital cardiomyopathy that is extremely uncommon. It is a condition that may be identified by an elevated number of endomyocardial trabeculations as well as an increase in their prominence. Alcoholic cardiomyopathy, also known as ACM, is a non-ischemic form of dilated cardiomyopathy that is characterized by contractile failure and an enlargement of the heart ventricles. It is not entirely known whether or not there is a clinically significant overlap in phenotypic characteristics between the two illnesses. We report a patient who had previously been diagnosed with ACM and who had cardiac MRI results that fit the criteria for both LVNC and ACM.

Left Ventricular Non-compaction Cardiomyopathy: The Key to Its Diagnosis and Implications for Management.

Left ventricular non-compaction (LVNC) cardiomyopathy is a condition with increasing prevalence as cardiac imaging technology improves, although there is currently no diagnostic gold standard. Characterized by the presence of a bilayered myocardium with prominent trabeculations, LVNC cardiomyopathy has a wide range of presentations, from asymptomatic to severe heart failure, thromboembolism, and sudden cardiac death. We present the case of a 62-year-old male who was admitted for a heart failure exacerbation with a worsening ejection fraction and signs of increased trabeculations of the left ventricle on an echocardiogram. We highlight the rarity of this condition, especially when diagnosed via echocardiogram, and the importance of considering anticoagulation as part of the treatment plan.

Left Ventricular Non-compaction Cardiomyopathy: A Case Report and Literature Review.

Left Ventricular Non-Compaction Cardiomyopathy (LVNC) is a rare myocardial disorder characterized by abnormal myocardial tissue formation in which the left ventricular wall appears to be trabecular with prominent intertrabecular recesses. The diagnosis of LVNC is predominantly reliant on cardiac imaging, namely thoracic echocardiography, however, cardiac MRI is indicated in conditions in which echocardiography is inconclusive. Diagnostic criteria for both echocardiography and cardiac MRI differ, however, the general principle of diagnosis is a comparison of the thickness of non-compacted to compacted myocardial tissue. The management of LVNC is nearly identical to that of Heart Failure with reduced Ejection Fraction (HFrEF), however, anticoagulation is an additional measure of management to the thrombogenic nature of non-compacted myocardial tissue. Here, we discuss a case of LVNC and the current data on its management.

Left Ventricular Noncompaction Cardiomyopathy in an Elderly Patient: A Case Report and Literature Review.

Isolated left ventricular noncompaction cardiomyopathy (LVNC), also known as spongy myocardium, is an extremely rare congenital disorder belonging to unclassified cardiomyopathies by the World Health Organization and classified as a genetic cardiomyopathy by the American Heart Association. Adult prevalence is 0.017-0.26% in observational echocardiographic studies. The disease occurs due to the intrauterine arrest of normal myocardial compaction, leading to left ventricular dysfunction. Reported mortality is high, ranging from 35 to 47% over a 42- to 72-month follow-up period. Knowledge regarding proper diagnosis, morbidity, and prognosis is limited; thus, this disease is subdiagnosed. Our aim is to highlight a diagnostic approach to LVNC in an elderly patient and to stress specific diagnostic signs that make the disease more recognizable. We are reporting a case of noncompaction cardiomyopathy in a 62-year-old male without any significant past medical history who was referred to our clinic for arrhythmia evaluation. The patient had several brief episodes of palpitations over the past two months. On physical examination, he presented a blowing systolic murmur at the apex and an irregularly irregular rhythm. The 12-lead electrocardiogram (ECG) demonstrated atrial fibrillation and ST-T segment depression in the V4-V6 leads. A transthoracic echocardiogram (TTE) showed signs of dilated cardiomyopathy, severe eccentric left ventricular hypertrophy, decreased contractility with an ejection fraction (EF) <30%, moderate mitral and tricuspid regurgitations, and moderate pulmonary hypertension. Multiple prominent trabeculations were noticed in the middle and apical segments of the left ventricle. The noncompacted to compacted myocardium ratio was >2.5:1. Cardiac catheterization excluded ischemic heart disease. Cardiac magnetic resonance (CMR) imaging confirmed the diagnosis of LVNC. The patient started treatment with carvedilol, ramipril, verospiron, torasemide, and rivaroxaban. An implantable cardioverter-defibrillator (ICD) was recommended. In conclusion, the diagnosis of LVNC in the adult population is often delayed because of similarities with more frequently diagnosed diseases. TTE is the initial diagnostic test of choice. Additional imaging modalities (contrast echocardiography, CMR) can help confirm the diagnosis. Early diagnosis is crucial because of the high incidence of life-threatening complications related to heart failure, thromboembolic events, and ventricular arrhythmias. Additional prospective studies are needed to improve the management and outcomes of this rare cardiomyopathy.

Isolated Left Ventricular Noncompaction Presenting With Heart Failure With Reduced Ejection Fraction and Intrahospital Cardiac Arrest: A Case Report and Literature Review.

Left ventricular noncompaction (LVNC) is characterized by a bilayered appearance of the myocardium with excessive trabeculations and deep intertrabecular recesses. Manifestations of this condition are widely variable, ranging from incidental findings in asymptomatic individuals to symptomatic heart failure, conduction abnormalities, tachyarrhythmia, and sudden cardiac death. Heart failure, ventricular arrhythmias, and systemic embolisms are the most frequent cardiovascular complications. We describe a case of a 53-year-old woman who presented to the emergency department with acute presentation of previously unknown heart failure with reduced ejection fraction and was diagnosed with LVNC. During hospitalization, the patient presented a defibrillated cardiac arrest rhythm, which was resuscitated after six minutes, and then treated with the placement of an implantable cardioverter defibrillator. After two years of follow-up with optimized medical therapy, the patient currently is asymptomatic and with a preserved ejection fraction.

Ventricular Noncompaction With Left Ventricular Thrombus: A Case Report.

Noncompaction cardiomyopathy (NCC) is congenital cardiomyopathy characterized by trabeculations of the left ventricle found on echocardiogram and/or cardiac magnetic resonance imaging (CMRI). This rare disease is associated with thromboembolism and an increased risk of ventricular thrombus formation. We present the case of a 73-year-old female who was admitted for a suspected cerebrovascular accident (CVA), later found on echocardiogram and CMRI to have NCC with left ventricular thrombus. She was started on warfarin indefinitely. We highlight the rarity of this phenomenon as well as the unique questions regarding initiation, length, and choice of therapeutic anticoagulation in the absence of atrial fibrillation in these patients. Consideration of this diagnosis should be made in the absence of other cardioembolic etiologies with prompt management based on available guidelines.

Anatomy for right ventricular lead implantation.

To understand the position of a pacing lead in the right ventricle and to correctly interpret fluoroscopy and intracardiac signals, good anatomical knowledge is required. The right ventricle can be separated into an inlet, an outlet, and an apical compartment. The inlet and outlet are separated by the septomarginal trabeculae, while the apex is situated below the moderator band. A lead position in the right ventricular apex is less desirable, last but not least due to the thin myocardial wall. Many leads supposed to be implanted in the apex are in fact fixed rather within the trabeculae in the inlet, which are sometimes difficult to pass. In the right ventricular outflow tract (RVOT), the free wall is easier to reach than the septal due to the fact that the RVOT wraps around the septum. A mid-septal position close to the moderator band is relatively simple to achieve and due to the vicinity of the right bundle branch may produce a narrower paced QRS complex. Special and detailed knowledge is necessary for His bundle and left bundle branch pacing.

Informe de caso de miocardiopatía no compactada en una mujer de 34 años / Case report of left ventricular noncompaction cardiomyopathy in a 34-year-old woman

Introducción: la miocardiopatía no compactada (MCNC) es una miocardiopatía no clasificada por la Organización Mundial de la Salud. Se describe como una enfermedad congénita muy rara, donde se observan trabeculaciones endomiocárdicas que aumentan en número y prominencia. Esta miocardiopatía conlleva un alto riesgo de arritmias malignas, fenómeno tromboembólico y disfunción ventricular izquierda. Objetivo: reportar el caso de una mujer de 34 años, diagnosticada previamente con obesidad mórbida, que acudió a consulta externa para una evaluación cardiovascular prequirúrgica. El electrocardiograma mostró el ventrículo izquierdo (VI) ligeramente dilatado y llamativa trabeculación del mismo. Resultados: se confirma el diagnóstico de MCNC a través de una imagen de resonancia magnética. Se autoriza su cirugía y se recomiendan cambios en su estilo de vida y cambio de medicación para riesgos de fallo cardíaco. En el último ecocardiograma, los diámetros del VI muestran mejoría con respecto al primero. Conclusiones: la presencia de trabeculaciones en el VI debe considerarse un dato clínico de sospecha de MCNC. Se deben realizar más investigaciones sobre las causas de esta miocardiopatía no clasificada para desarrollar mejores formas terapéuticas, sin embargo, ha sido probada la eficacia de los bloqueadores de los receptores de la angiotensina II en el manejo farmacoterapéutico de esta condición

Introduction: Left ventricular non-compaction cardiomyopathy (LVNC) is a cardiomyopathy not classified by the World Health Organization. It is described as a very rare congenital disease where endomyocardial trabeculations that increase in number and prominence are observed. This cardiomyopathy carries a high risk of malignant arrhythmias, thromboembolic events and left ventricular dysfunction. Objective: To report the case of a 34-year-old woman, previously diagnosed with morbid obesity, who came to the outpatient clinic for a preoperative cardiovascular evaluation. The electrocardiogram showed a slightly dilated left ventricle (LV) and striking trabeculation. Results: The diagnosis of LVNC was confirmed by magnetic resonance imaging. Surgery was authorized and lifestyle changes and change of medication for heart failure risks were recommended. On the last echocardiogram, LV diameters show improvement from the first. Conclusions: The presence of trabeculations in the LV should be considered as clinical data of suspected LVNC. Further investigations on the causes of this unclassified cardiomyopathy should be performed to develop better therapeutic ways, however, the efficacy of angiotensin II receptor blockers in the pharmacotherapeutic management of this condition has been proven

Left Ventricular Noncompaction: New Insights into a Poorly Understood Disease.

Left ventricular noncompaction (LVNC) is a congenital pathology that directly affects the lining walls of myocardial tissue, causing trabeculations with blood filling in the inner wall of the heart, concomitantly with the development of a mesocardial thinning. Although LVNC was described for the first time as long ago as 1984, our understanding of the disease with regard to its genetic pattern, diagnosis, clinical presentation and treatment is still scanty. LVNC can present as an isolated condition or associated with congenital heart disease, genetic syndromes or neuromuscular disease. This suggests that LVNC is not a distinct form of cardiomyopathy, but rather a morphological expression of different diseases. Recognition of the disease is of fundamental importance because its clinical manifestations are variable, ranging from the absence of any symptom to congestive heart failure, lethal arrhythmias and thromboembolic events. The study of this disease has emphasized its genetic aspects, as it may be of sporadic origin or hereditary, in which case it most commonly has an autosomal dominant inheritance or one linked to the X chromosome. Echocardiography is the gold standard for diagnosis, and magnetic resonance imaging may refine the identification of the disease, especially in those patients with non-conclusive echocardiography. This article sets out to review the main characteristics of LVNC and present updates, especially in the genetic pattern, diagnosis and treatment of the disease.

Prevalence and significance of isolated left ventricular non-compaction phenotype in normal black Africans using echocardiography.

BACKGROUND: Several large, prospective screening studies of predominantly Caucasian patients have suggested that hypertrabeculation may not necessarily be pathologic unless there is concomitant left ventricular (LV) dysfunction, LV dilatation, history of arrhythmia, family history, or characteristic gene mutations. This conundrum may be magnified in blacks, in whom hypertrabeculation and LV hypertrophy is more common. We therefore investigated the frequency of hypertrabeculation/isolated LV noncompaction (ILVNC) phenotype in normal black Africans and evaluated LV function using sensitive measures of deformation and twist. METHODS: Two hundred and fifty-three volunteers were recruited and evaluated according to strict inclusion and exclusion criteria. Their mean age was 36.3 ± 12.2 years. RESULTS: Trabeculations were found in 12 (4.74%) participants. Three (1.2%) subjects had ≥ 4 LV trabeculations. The LV apex was the most common anatomical site for the location of trabeculations. Subjects with trabeculations were more likely to be males of a younger age, and had greater LV end-diastolic and end-systolic parameters and lateral e'. However, 0.8% of the population fulfilled the Stollberger criteria, and none fulfilled the Jenni, Milwaukee, or Baragwanath criteria. All subjects in this study had normal rotation patterns with no differences in rotational parameters or net twist. CONCLUSIONS: Trabeculations may be found as a normal variant in black Africans. Assessing trabeculations alone may infer ILVNC; however, utilizing the more comprehensive ILVNC criteria enables differentiation of a possible LVNC phenotype. Normal individuals with hypertrabeculation have normal LV function and normal rotation patterns, with no differences in rotational parameters or net twist.

Left ventricular non-compaction in a child with bicuspid aortic valve and aortic coarctation.

We present the case of a 3-year-old boy with bicuspid aortic valve, aortic coarctation, and left ventricular non-compaction. The diagnosis was made post-natally with ultrasonography and was verified by cardiac MRI. Aortic coarctation was initially repaired surgically. At age 3 months, recoarctation and heart failure developed. Balloon angioplasty was performed with immediate improvement. At age 3 years, the patient remains asymptomatic and normotensive.

Increased fractal dimension of left ventricular trabeculations is associated with subclinical diastolic dysfunction in patients with type-2 diabetes mellitus.

The aim of this study was to investigate the relationship among left ventricular (LV) concentric hypertrophy, endocardial remodeling, and myocardial deformation in type-2 diabetes mellitus (T2DM). Fifty-three T2DM patients with normotension and 36 healthy controls underwent cardiovascular magnetic resonance imaging to assess for LV concentric hypertrophy (LV myocardial mass index, LVMMi; LVMMi-to-LV end-diastolic volume index ratio, MVR), endocardial remodeling (fractal dimension of trabeculations, FD), and myocardial deformation (global longitudinal, radial and circumferential strain, systolic and diastolic strain rate). When compared with healthy controls, T2DM was associated with LV concentric hypertrophy (LVMMi: T2DM, 52.7 ± 8.9 g/m2; controls, 48.7 ± 8.4 g/m2, p = 0.032; MVR: T2DM, 0.88 ± 0.19 g/mL; controls, 0.77 ± 0.16 g/mL, p = 0.007), endocardial remodeling (max. apical FD: T2DM, 1.265 ± 0.056; controls, 1.233 ± 0.055, p = 0.008; mean apical FD: T2DM, 1.198 ± 0.043; controls, 1.176 ± 0.043, p = 0.020), and subtle diastolic dysfunction (peak longitudinal diastolic strain rate, PDSRL: T2DM, 1.1 ± 0.2/s; controls, 1.2 ± 0.3/s, p = 0.031). In the stepwise multivariable regression model, the MVR was an independent determinant of the maximum apical FD (standardized ß, sß = 0.525, p < 0.001) and mean apical FD (sß = 0.568, p < 0.001). The mean apical FD was an independent determinant of the PDSRL (p = 0.004). LV concentric hypertrophy is an independent determinant of endocardial remodeling, a process that may contribute to subtle LV diastolic dysfunction in T2DM patients.

Streamlining risk stratification in infants and young children with spinal dysraphism: Vesicoureteral reflux and/or bladder trabeculations outperforms other urodynamic findings for predicting adverse outcomes.

BACKGROUND: Baseline and interval dimercaptosuccinic acid (DMSA) scans and urodynamic (UD) studies are often obtained in infants and young children with spinal dysraphism (SD). OBJECTIVE: To identify practical UD parameters which accurately stratify urologic risk young children with SD. STUDY DESIGN: 130 expectantly managed infants/young children with SD and initial DMSA and UD before age 2 were reviewed. End fill pressure (EFP), bladder trabeculations, vesicoureteral reflux (VUR), initial volume (IV) drained at UD catheter placement, and detrusor pressure at initial volume (DPIV) were evaluated for association with subsequent febrile urinary tract infection (UTI), DMSA abnormalities, and early clean intermittent catheterization (CIC). A combination of factors to accurately stratify risk was sought. Groups were compared by log-rank test. The association of CIC and febrile UTI incidence was evaluated. RESULTS: 31/130 patients developed DMSA abnormalities, 52/130 started early CIC, and 61/130 developed a febrile UTI with median follow-up of 3.8 years. Trabeculations, VUR, EFP ≥40 cm H2O, IV ≥50% estimated bladder capacity (EBC), and DPIV >10 cm H2O were associated with subsequent abnormal DMSA scan (p < 0.001). The best predictor was combination of trabeculation and/or VUR (p < 0.001) (Figure). Among patients who maintained a non-trabeculated bladder without VUR during follow-up, 0/51 developed DMSA abnormalities compared with 31/79 who developed one or both (p < 0.001). Patients with trabeculations and/or VUR were more likely to start early CIC (8/51 vs. 44/79; p < 0.001) and have febrile UTI (11/51 vs. 50/79; p < 0.001). In those with trabeculations, CIC was associated with decreased incidence of febrile UTI (incidence rate ratio (IRR) 0.5, 95% CI 0.3-0.9); in those without trabeculations, CIC was associated with increased incidence of febrile UTI (IRR 1.8, 95% CI 1.1-3.1). CONCLUSIONS: VUR, bladder trabeculations, EFP ≥40 cm H20, IV ≥50% of EBC, and DPIV >10 cm H2O were associated with subsequent DMSA abnormalities in young children with SD managed expectantly. Many of these parameters were associated with febrile UTI and early CIC. The combination of trabeculations and/or VUR outperformed other UD parameters in identifying those high and low-risk for adverse urologic outcomes. Routine DMSA scan may have limited utility in patients with a non-trabeculated bladder without VUR, as none developed an abnormal DMSA. Most (71%) abnormal DMSAs were in patients with trabeculations and/or VUR following a febrile UTI. Given these findings and that incidence of febrile UTI may be lower in those with trabeculations while on CIC, patients with trabeculations and/or VUR should be managed aggressively to protect kidneys.

Quantification of the detailed cardiac left ventricular trabecular morphogenesis in the mouse embryo.

During embryogenesis, a mammalian heart develops from a simple tubular shape into a complex 4-chamber organ, going through four distinct phases: early primitive tubular heart, emergence of trabeculations, trabecular remodeling and development of the compact myocardium. In this paper we propose a framework for standardized and subject-independent 3D regional myocardial complexity analysis, applied to analysis of the development of the mouse left ventricle. We propose a standardized subdivision of the myocardium into 3D overlapping regions (in our case 361) and a novel visualization of myocardial complexity, whereupon we: 1) extend the fractal dimension, commonly applied to image slices, to 3D and 2) use volume occupied by the trabeculations in each region together with their surface area, in order to quantify myocardial complexity. The latter provides an intuitive characterization of the complexity, given that compact myocardium will tend to occupy a larger volume with little surface area while high surface area with low volume will correspond to highly trabeculated areas. Using 50 mouse embryo images at 5 different gestational ages (10 subjects per gestational age), we demonstrate how the proposed representation and complexity measures describe the development of LV myocardial complexity. The mouse embryo data was acquired using high resolution episcopic microscopy. The complexity analysis per region was carried out using: 3D fractal dimension, myocardial volume, myocardial surface area and ratio between the two. The analysis of gestational ages was performed on embryos of 14.5, 15.5, 16.5, 17.5 and 18.5 embryonic days, and demonstrated that the regional complexity of the trabeculations increases longitudinally from the base to the apex, with a maximum around the middle. The overall complexity decreases with gestational age, being most complex at 14.5. Circumferentially, at ages 14.5, 15.5 and 16.5, the trabeculations show similar complexity everywhere except for the anteroseptal and inferolateral area of the wall, where it is smaller. At 17.5 days, the regions of high complexity become more localized towards the inferoseptal and anterolateral parts of the wall. At 18.5 days, the high complexity area exhibits further localization at the inferoseptal and anterior part of the wall.

Relationship between the left ventricular size and the amount of trabeculations.

Contemporary imaging modalities offer noninvasive quantification of myocardial deformation; however, they make gross assumptions about internal structure of the cardiac walls. Our aim is to study the possible impact of the trabeculations on the stroke volume, strain, and capacity of differently sized ventricles. The cardiac left ventricle is represented by an ellipsoid and the trabeculations by a tissue occupying a fixed volume. The ventricular contraction is modeled by scaling the ellipsoid whereupon the measurements of longitudinal strain, end-diastolic, end-systolic, and stroke volumes are derived and compared. When the trabeculated and nontrabeculated ventricles, having the same geometry and deformation pattern, contain the same amount of blood and contract with the same strain, we observed an increased stroke volume in our model of the trabeculated ventricle. When these ventricles contain and eject the same amount of blood, we observed a reduced strain in the trabeculated case. We identified that a trade-off between the strain and the amount of trabeculations could be reached with a 0.35- to 0.41-cm dense trabeculated layer, without blood filled recesses (for a ventricle with end-diastolic volume of about 150 mL). A trabeculated ventricle can work at lower strains compared to a nontrabeculated ventricle to produce the same stroke volume, which could be a possible explanation why athletes and pregnant women develop reversible signs of left ventricular noncompaction, since the trabeculations could help generating extra cardiac output. This knowledge might help to assess heart failure patients with dilated cardiomyopathies who often show signs of noncompaction.

Case report: Left ventricular noncompaction cardiomyopathy and RASopathies.

The following is a case report of 6 patients with Noonan syndrome (NS) and/or a related RASsopathy that also have evidence of left ventricular noncompaction cardiomyopathy (LVNC). Noonan syndrome,a type of RASopathy, is an autosomal dominant disorder that is typically associated with congenital heart defects and hypertrophic cardiomyopathy. There have been minimal reports of Noonan syndrome or other RASopathy and the association of LVNC. This report promulgates 6 nonrelated cases of Noonan syndrome or unspecified RASopathy and LVNC.

Patient independent representation of the detailed cardiac ventricular anatomy.

Reparameterization of surfaces is a widely used tool in computer graphics known mostly from the remeshing algorithms. Recently, the surface reparameterization techniques started to gain popularity in the field of medical imaging, but mostly for convenient 2D visualization of the information initially represented on 3D surfaces (e.g. continuous bulls-eye plot). However, by consistently mapping the 3D information to the same 2D domain, surface reparameterization techniques allow us to put into correspondence anatomical shapes of inherently different geometry. In this paper, we propose a method for anatomical parameterization of cardiac ventricular anatomies that include myocardium, trabeculations, tendons and papillary muscles. The proposed method utilizes a quasi-conformal flattening of the myocardial surfaces of the left and right cardiac ventricles and extending it to cover the interior of the cavities using the local coordinates given by the solution of the Laplace's equation. Subsequently, we define a geometry independent representation for the detailed cardiac left and right ventricular anatomies that can be used for convenient visualization and statistical analysis of the trabeculations in a population. Lastly we show how it can be used for mapping the detailed cardiac anatomy between different hearts, which is of considerable interest for detailed cardiac computational models or shape atlases.