Left Ventricular Mass Hypertrophy Research Articles

Massive Left Ventricular Hypertrophy With Acromegaly: Hypertrophic Obstructive Cardiomyopathy Phenocopy or Phenotype?

Acromegaly—a rare endocrine disorder—results when a growth hormone–secreting somatotroph pituitary adenoma leads to increased insulin-like growth factor 1 production. Acromegaly is known to cause left ventricular hypertrophy. We present a case of acromegaly with massive left ventricular hypertrophy that was determined to be coexistent with gene-positive hypertrophic obstructive cardiomyopathy. (Level of Difficulty: Intermediate.)

Cardiopulmonary Exercise Testing in Athletes With Hypertrophic Cardiomyopathy

Patients with hypertrophic cardiomyopathy (HCM) have historically been restricted from athletic participation because of the perceived risk of sudden cardiac death. More contemporary research has highlighted the relative safety of competitive athletics with HCM. However, lack of published data on reference values for cardiopulmonary exercise testing (CPET) complicates clinical management and counseling on sports participation in the individual athlete. We conducted a single-center, retrospective cohort study to investigate CPET in athletes with HCM and clinical characteristics associated with objective measures of aerobic capacity. We identified 58 athletes with HCM (74% male, mean age 18±3years, mean left ventricular (LV) wall thickness 20±7mm). LV outflow tract obstruction was present in 22 (38%). A total of 15 (26%) athletes were taking a β blocker (BB), but only 4 (7%) reported exertional symptoms. Overall, exercise capacity was mildly reduced, with a peak myocardial oxygen consumption (peak VO2) of 37.9ml/min/kg (83% of predicted peak VO2). Both LV outflow tract obstruction and BB use were associated with reduced exercise capacity. Limited peak heart rate was more common in athletes taking BB (47% vs 9%, p=0.002). At a mean 5.6years follow-up, 5 patients underwent myectomy (9%), and 8 (14%) received an implantable cardioverter defibrillator (ICD) for primary prevention. One individual with massive LV hypertrophy experienced recurrent ICD shocks for ventricular fibrillation and underwent myectomy 7years after initial evaluation and was no longer participating in sports. There were no deaths over the follow-up period. In conclusion, the prognostic role of CPET remains unclear in athletes with HCM. Mildly reduced exercise capacity was common; however, reduced peak VO2 did not correlate with symptom status or clinical outcomes. A significant proportion went on to require myectomy and/or ICD, thus highlighting the need for close follow-up. These data provide some initial insight into the clinical evaluation of "real world" athletes with HCM; however, further study is warranted to help guide shared decision-making, return-to-play discussions, and the potential long-term safety of competitive athletic participation.

The Squeezed Heart-A Case Report

Pompe’s disease is an autosomal recessive disorder caused by inherited deficiency of α-1,4-glucosidase (acid maltase), a lysosomal enzyme. Patients usually die in the first year of life from cardio-respiratory failure due to massive left ventricular hypertrophy. We report a case of 3-month-old boy presented with fatal infantile onset Pompe’s disease.

Mega Coronary Arteries with Obstructive Coronary Artery Disease in Hypertrophic Obstructive Cardiomyopathy: A Case Report and Literature Review

We report a rare case of hypertrophic obstructive cardiomyopathy (HOCM) presenting with mega coronary arteries with obstructive coronary artery disease. Interestingly, the patient had large septals, large diagonals, large obtuse marginal with a large left atrial branch, and a large conus almost mimicking a duplicate right coronary artery. Because of massive left ventricular hypertrophy, to overcome the supply-demand mismatch, coronary arteries may become large or with more arborization in hypertrophic cardiomyopathy but the same is rarely encountered in routine clinical practice. Our case is a rare illustration of the simultaneous presence of mega coronary arteries and critical obstructive coronary artery disease in HOCM in a young male of 38 years of age.

Sudden cardiac death risk stratification in hypertrophic cardiomyopathy: discrepancies persist among guidelines

Abstract Background Differing criteria have been provided on sudden cardiac death (SCD) risk stratification in hypertrophic cardiomyopathy (HCM) by current guidelines. Recently, AHA/ACC proposed an algorithm with novel clinical markers. It remains to be established the impact of this new approach. Purpose Evaluate the impact of the 2020 AHA/ACC guideline on the 2014 ESC and 2011 ACCF/AHA criteria regarding SCD risk assessment and primary prevention implantable cardioverter-defibrillator (ICD) in HCM. Methods The database of a HCM center non-referred cohort was accessed for SCD risk profile between March 2007 and March 2020. The agreement for primary prevention ICD recommendations among guidelines was assessed with the Cohen's and Fleiss' Kappa coefficient, P<0.05. SCD or appropriate ICD shock were defined as the primary end-point. Results A total of 100 patients, age 60±13 years, 55 (55%) females, were followed by 5±3 years. The maximal left ventricular (LV) wall thickness was 18±4 mm, 38 (38%) patients showed a family history of SCD, 22 (22%) syncope, 6 (6%) ejection fraction ≤50%, 2 (2%) LV apical aneurysm, 1 (1%) massive LV hypertrophy, 26 (26%) non-sustained ventricular tachycardia, and 23 (23%) extensive late gadolinium enhancement. An ICD was placed in 17 (17%) patients. According to the 2020 AHA/ACC guideline, 57 (57%) patients met class IIa recommendation, 27 (27%) class IIb, and 16 (16%) class III. The 2014 ESC model classified 14 (14%) in class IIa, 18 (18%) in class IIb, and 68 (68%) in class III. The 2011 ACCF/AHA considered 66 (66%) in class IIa, 6 (6%) in class IIb, and 28 (28%) in class III. The Cohen's Kappa was 0.200 (95% CI 0.292–0.107), P=0.0005, between the 2020 AHA/ACC and the 2014 ESC, and 0.520 (95% CI 0.651–0.388), P=0.0005, between the North American approaches. The Fleiss' Kappa was 0.219 (95% CI 0.303–0.135) P=0.0005 among the three guidelines, whereas it reached 0.221 (95% CI 0.334–0.108) for class IIa, 0.244 (95% CI 0.357–0.131) for class IIb and 0.202 (95% CI 0.315–0.089) for class III, P=0.0005. Figure 1 shows the patient's reclassification with the new guideline. The primary end-points occurred in 7 (7%) patients in a median follow-up of 6 (17–0.4) years. All of them were classified as IIa with the 2011 ACCF/AHA guideline, but only 4 (4%) met this class under the 2020 AHA/ACC, and none in the 2014 ESC model. Conclusion A low agreement was found among guidelines, especially between the 2020 AHA/ACC and the 2014 ESC criteria. The North American systems differed moderately, but the new approach has reduced the cases in class IIa and III recommendation for primary prevention ICD. In contrast, the recent 2020 guideline has increased the number of patients in class IIa in relation to the European model, but both strategies have not protected the totality of patients with SCD or appropriate shock. Funding Acknowledgement Type of funding sources: Public hospital(s). Main funding source(s): Hospital de Clínicas de Porto Alegre Figure 1

Azygos Vein ICD Lead Implantation Lowers Defibrillation Threshold in a Patient with Hypertrophic Cardiomyopathy

A 14-year-old boy with hypertrophic cardiomyopathy (HCM) diagnosed at the age of 1 year and with massive left ventricular hypertrophy suffered an episode of ventricular fibrillation during mild effort. He underwent a dual-chamber implantable cardioverter defibrillator (ICD) implantation. The defibrillation threshold testing (DFT) was ineffective. Subcutaneous multi-coli arrays tunneled into the left postero-lateral position and connected to the superior vena cava (SVC) port of the dual-chamber ICD were added to increase the myocardial mass involved in the defibrillation shock pathway. A new DFT was unsuccessful. The patient was transferred to our hospital for myectomy. An epicardial defibrillation patch was placed on the left ventricular lateral wall, but again, DFT testing was ineffective using the right ventricular (RV) coil to lateral patch as shock pathway. Another epicardial defibrillation patch was then placed on the inferior wall. In this case, DFT testing was effective with a defibrillation pathway between the two patches and the can. In November 2015, a high shock impedance alarm was recorded through remote monitoring, thus compromising the safety of the ICD shock pathway. The patient underwent the implant of a new trans-venous defibrillation coil lead in the azygos vein. After few months, the patient developed symptomatic severe aortic regurgitation and underwent an aortic valve replacement. During the operation, DFT testing was performed and was successful. Our case illustrates that azygous vein ICD lead implantation is efficacious in HCM with massive hypertrophy and high DFT, and prompts further studies to systematically investigate its efficacy in this particular subgroup of the HCM population.

Evidence of evolution towards left midventricular obstruction in severe Anderson-Fabry cardiomyopathy.

AimsIn Fabry cardiomyopathy, left ventricular outflow tract obstruction mimicking hypertrophic cardiomyopathy is a very rare finding, with few cases reported and successfully treated with cardiac surgery. In our population of patients with Fabry disease and severe left ventricular hypertrophy (LVH) at the time of diagnosis, we observed an evolution towards a midventricular obstructive phenotype.Methods and resultsWe present a case series of three classically affected Fabry male patients with significant diagnostic delay and severe cardiac involvement (maximal wall thickness >20 mm) at first evaluation. All patients developed midventricular obstructive form over time despite prompt initiation and optimal compliance to enzyme replacement therapy. The extension and distribution of the LVH, involving the papillary muscles, was the main mechanism of obstruction, unlike the asymmetric septal basal hypertrophy and the mitral valve abnormalities commonly seen as substrate of left ventricular outflow tract obstruction in hypertrophic cardiomyopathy.ConclusionsFabry cardiomyopathy can evolve over time towards a midventricular obstructive form due to massive LVH in classically affected men with significant diagnostic delay and severe LVH before enzyme replacement therapy initiation. This newly described cardiac phenotype could represent an adverse outcome of the disease.

Prediction and Prevention of Sudden Death in Young Patients (

Highly reliable identification of adults with hypertrophic cardiomyopathy (HC) at risk for sudden death (SD) has been reported. A significant controversy remains, however, regarding the most reliable risk stratification methodology for children and adolescents with HC. The present study assesses the accuracy of SD prediction and prevention with prophylactic implantable cardioverter-defibrillators (ICDs) in young HC patients. The study group is comprised of 146 HC patients <20 years of age evaluated consecutively over 17 years with prospective risk stratification and ICD decision-making. We relied on ≥1 established individual risk markers considered major within each patient's clinical profile, based on an enhanced American College of Cardiology /American Heart Association (ACC/AHA) guidelines algorithm. Of the 60 largely asymptomatic patients implanted with primary prevention ICDs at age 15 ± 4 years, 9 (15%) experienced device therapy terminating potentially lethal ventricular tachyarrhythmias and restoring sinus rhythm at 19 ± 6 years (range 9 to 29), 5.1 ± 6.0 years after implant; 3 patients had multiple appropriate ICD discharges. The individual risk marker algorithm was associated with 100% sensitivity in predicting SD events (95%CI: 69, 100) and 63% specificity for identifying patients without events (95%CI: 54, 71). Of these patients with device therapy, massive left ventricular hypertrophy (absolute wall thickness ≥30 mm) was the most common predictor, present in 70% of patients either alone or in combination with other risk markers. Each of the 146 study patients have survived to date at 22 ± 5 years, including all 86 without ICD recommendations. In conclusion, an enhanced ACC/AHA risk stratification strategy, based on established individual risk markers, was highly reliable in prospectively predicting SD events in children and adolescents with HC, and preventing arrhythmia-based catastrophes in this susceptible high risk population.

P712 When Popeye had shortness of breath

Abstract A 61-year-old man presented to our clinic with progressive shortness-of-breath in the last 3 months. He had a history of hospitalization and endotracheal intubation due to acute pulmonary edema and type – I respiratory failure a week ago and discharged after diagnostic coronary angiography which demonstrating normal coronary arteries. Physical examination revealed bunching of the left biceps when he flexed his left arm which is consistent with rupture of distal biceps tendon (Panel A, Popeye Sign). A 12-lead electrocardiography (ECG) showed low QRS voltage in the limb leads and ST segment depression and asymmetrical T wave inversion in the anterolateral leads (Panel B). Transthoracic echocardiography (TTE) showed concentric left ventricular hypertrophy with left ventricular ejection fraction of 60% (Panel C). Pulsed wave-Doppler obtained from the mitral valve demonstrated restrictive filling pattern with high early diastolic velocity (E-wave) and low late diastolic filling (A-wave) peaks with E/A ratio &amp;gt; 2.0 and reduced lateral mitral annular diastolic velocities with E/e’ ratio &amp;gt; 15 which are compatible with impaired left ventricular diastolic function with elevated left ventricular filling pressures (Panel D-E). Biceps tendon rupture, low QRS voltage and left ventricular hypertrophy with restrictive filling pattern were considered suggestive of cardiac amyloidosis and patient was referred to hematology clinic for diagnostic work-up. Protein electrophoresis demonstrated monoclonal gammopathy in the serum and immunoelectrophoresis showed free lambda light chains. The patient diagnosed as AL amyloidosis with cardiac and musculoskeletal involvement. Biceps tendon rupture is a rare finding of amyloidosis due to musculoskeletal involvement. In the setting of acute pulmonary edema and/or heart failure with preserved ejection fraction with massive left ventricular hypertrophy, restrictive filling pattern, low QRS voltage and spontaneous tendon rupture should raise clinical suspicion of amyloidosis with cardiac and musculoskeletal involvement. Abstract P712 Figure.

Impact of Effective Management Strategies on Patients With the Most Extreme Phenotypic Expression of Hypertrophic Cardiomyopathy

Advances in treatment options for hypertrophic cardiomyopathy (HC) have proven effective in many patients for promoting favorable long-term outcomes. Whether this expectation is similar for patients with the most extreme expression of massive left ventricular (LV) hypertrophy, a particularly aggressive form of the disease is unresolved. Of 1,766 consecutive HC patients presenting to Tufts HC Institute (2004 to 2015), 92 were identified with extreme LV wall thickness (30 to 48 mm), and compared with 1,674 HC patients with less marked hypertrophy (13 to 29 mm). Follow-up assessment was over 5.3 ± 3.4 years. Patients with massive LV hypertrophy (n = 92) had higher sudden death event rates (3.0%/year) than did patients with lesser hypertrophy (0.8%/year; p <0.001). In 16 of the 92 patients (17%), potentially lethal ventricular tachyarrhythmia were successfully aborted by primary prevention implantable cardioverter defibrillator (ICD) therapy at 30 ± 13 years (n = 11), or by resuscitated cardiac arrest with external defibrillation (n = 5) and later by secondary prevention interventions (n = 3); no patient experienced arrhythmic sudden death. Aborted sudden death events (3.0%/year) exceeded HC-related mortality by 7-fold (n = 2; 0.4%/year; p <0.001). European Society of Cardiology risk score would have failed to identify 60% of patients with arrhythmic sudden death events, leaving them exposed to sudden death without ICDs. In addition, 35 patients required surgical myectomy for progressive heart failure due to LV outflow obstruction (improved to NYHA I/II in 30). Eighty-eight (96%) of the 92 patients have survived to age 38 ± 14 years (23% ≥ 50 years). All-cause mortality did not differ from an age and gender-matched general population (p = 0.62). In conclusion, in this referral-based population, patients with the most extreme expression of HC are at increased arrhythmic sudden death risk reliably prevented with prophylactic ICDs. Progressive heart failure secondary to outflow obstruction was reversible with surgical myectomy. Despite extreme phenotypic expression, with contemporary treatment interventions young HC patients have an opportunity to achieve extended survival with good quality of life.

Genetic markers in hypertensive hypertrophic cardiomyopathy

Background: The concept of hypertrophic cardiomyopathy came from observations on patients with systemic hypertension (HTN) where few patients developed inappropriate hypertrophy and even outflow tract obstruction. Over a period of time, research workers found that it is hereditary disorder and has no relationship with systemic HTN. However, many workers have observed that some patients with HTN have massive left ventricular hypertrophy disproportionate to the severity or duration of HTN. Materials and Methods: By using echocardiography, we identified inappropriate left ventricular hypertrophy in long-standing hypertensive patients. Markers of genetic abnormality were tested in those patients to find whether they differ from the usual hypertensive population. Results: We identified 29 hypertensive patients with severe concentric left ventricular hypertrophy (wall thickness, ≥1.6 cm). All patients were considered to have essential HTN. From these patients, we collected blood samples for detailed genetic study. Twenty-nine adult patients with age between 20 and 50 years of either sex with HTN on medications for 5 years or more were selected. Genotyping was done by sequencing. Genetic mutations were detected in 3 (11%) of 27 patients. myosin-binding protein of chromosome were detected in two patients, and mycophenolic acid xanthine hypoxanthine of igg was detected in one patient. Conclusion: In our study, we found that these genes are involved in hypertensive hypertrophic cardiomyopathy also. This suggests that patients with inappropriate left ventricular hypertrophy have a genetic involvement and all the family members should be screened.

Identification of Two Novel LAMP2 Gene Mutations in Danon Disease

BackgroundDanon disease is a rare X-linked inherited disorder characterized by massive left ventricular hypertrophy, skeletal muscle dystrophy, and mental retardation. The disease is caused by mutations in the LAMP2 gene encoding for lysosome-associated membrane protein-2. MethodsTwo young male patients with hypertrophic cardiomyopathy, characterized by marked, concentric left ventricular hypertrophy, elevated levels of creatine kinase, and manifest limb-girdle muscular dystrophy in 1 case, were investigated. Genetic screening included direct sequencing of the whole coding sequence of the LAMP2 gene. ResultsGenetic analysis identified 2 novel LAMP2 gene mutations. In Family A, a G-A transition (c.962G > A) leading to a nonsense mutation at codon 321 (p.Trp321Ter), and in Family B, a one-nucleotide insertion (c.973insC) leading to a full frame-shift (p.Pro324+24X) was detected in exon 8 of the LAMP2 gene. Family screening identified 8 mutation carriers, with 4 nonpenetrant cases and 3 additional, probably affected family members without DNA diagnosis. The cardiac phenotype was hypertrophic cardiomyopathy in all cases, including female mutation carriers. Five disease-related deaths occurred in the families, at an average age of 33 ± 16 years, which was clearly lower in male than in female patients (28 ± 7 vs 42 ± 25 years). A high prevalence of arrhythmias or conduction abnormalities was also observed. ConclusionsThe reported 2 novel LAMP2 gene mutation carrier families, one of them being one of the largest reported to date, highlight the malignant clinical course of Danon disease, characterized by a high rate of disease-related death at an early age and a high prevalence of arrhythmias or conduction abnormalities.

Massive Left Ventricular Hypertrophy

Hypertrophic cardiomyopathy, is unexplained reasons and characterized with left ventricular hypertrophy, is an inherited chronic disease of the heart muscle. Hypertrophic cardiomyopathy is seen rarely but it is very important because of genetic transmission and high risk of sudden cardiac death. Echocardiography is basic application for diagnosis left ventricular hypertrophy. We defined a rare case in literature which has massive left ventricular hypertrophy

Temporary Dual-Chamber Pacing Can Stabilize Hemodynamics During Noncardiac Surgery in a Patient With Left Ventricular Hypertrophy and Outflow Obstruction

HYPERTROPHIC CARDIOMYOPATHY (HCM) often includes asymmetric (or nonphysiologic) massive left ventricular hypertrophy, which can be diagnosed by electrocardiography and echocardiography. In patients with HCM, hypertrophy is more common in the ventricular septum below the aortic valve, leading to left ventricular outflow tract (LVOT) obstruction.1-3 There have been many reports of anesthetic management for noncardiac surgery of patients with ischemic heart disease. However, there have been only a few reports concerning the anesthetic management of patients with HCM undergoing noncardiac surgery.

An elevated cibenzoline level interacted with cyclosporine caused ventricular tachyarrhythmia and high defibrillation threshold in hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy (HCM) includes non-physiological massive left ventricular hypertrophy, which can be diagnosed by electrocardiography and echocardiography. In HCM patients, hypertrophy often leads to left ventricular obstruction and increases the left ventricular pressure gradient (LVPG) resulting in hypertrophic obstructive cardiomyopathy (HOCM). This is often indicated by anterior motion of the septal leaflet of the mitral valve, known as SAM in an echocardiogram.

Rare Forms of Preexcitation

A 16-year-old boy was brought to the hospital when he complained of chest pain while at a basketball game. In the emergency room, he was noted to have a wide complex tachycardia. The ECG at presentation is shown in Figure 1. He was cardioverted after administration of 150 mg of intravenous amiodarone. His ECG after cardioversion is shown in Figure 2. His physical examination and developmental evaluation were significant for obesity, synophrys, mild fifth digit clinodactyly, and subnormal intelligence. Cardiac magnetic resonance imaging with contrast showed massive left ventricular hypertrophy (LVH), with preserved ejection fraction, and several areas of delayed hyperenhancement in the subendocardium. Cardiac catheterization showed no obstructive coronary artery disease or anomalous coronary arteries. What are the diagnostic considerations for this constellation of findings of massive LVH, subendocardial necrosis, and abnormal ECGs? Figure 1. Wide complex tachycardia at presentation. Figure 2. Sinus rhythm ECG showing ventricular preexcitation and normal axis (see text for further details). The ECG in Figure 1 displays a wide complex tachycardia, with a right-bundle branch block morphology with a QRS complex duration >120 ms. A wide complex tachycardia with a right-bundle branch block morphology could be secondary to ventricular tachycardia originating from the left ventricle or a preexcited tachycardia in the presence of a left-sided bypass tract. However, the QS complex in lead V6 rules out a left-sided atrioventricular bypass tract, because such bypass tracts do not have the left ventricle apex as their ventricular insertion site, which is necessary to produce a QS complex in V6. A left-sided nodofascicular bypass tract with insertion in the distal His-Purkinje system can produce QS complexes in lead V6, but these are extraordinarily rare and occur more frequently on the right side. Initial R wave in lead aVR,1 a QS complex in lead …

Characterization of patients with massive hypertrophic cardiomyopathy using contrast-enhanced magnetic resonance imaging: does contrast provide additional information?

Background Hypertrophic cardiomyopathy (HCM) patients with massive left ventricular hypertrophy (LVH) (hereby defined as maximal LV wall thickness >30mm) are known to have lower rates of survival. Recently, contrast-enhanced cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) has emerged as a potential novel marker of increased cardiovascular risk in HCM. Methods Cine CMR and LGE imaging were performed on HCM patients with massive LV hypertrophy and compared with those with a maximal LV wall thickness <30mm. Results Among 902 consecutive HCM patients, 40 (4.4%) had a maximal LV wall thickness of ≥ 30mm (mean thickness 33.7± 4.2mm, range 30 - 50mm, 23% females) with a mean age of 37 ± 18 years old, including 11 patients (28%) ≥ 50 years of age. When compared to those with a maximal wall thickness less than 30mm, these patients were significantly younger (mean age 37 ± 18 vs 47 ± 18, p<0.001) and had a similar proportion of males (77.5% versus 66.8%, p=0.16). Mean left ventricular mass was significantly higher (317 ± 142 versus 163 ± 62 grams, p<0.001). They also had significantly higher LV end diastolic volume (LVEDV) (169.6 ± 58.5 versus 153.0 ± 42.1 mL, p<0.01) and LV end systolic volume

A family with a rare disease

Fabry disease (FD) is a rare disorder resulting from mutations of the alpha-Galactosidase A lysosomal enzyme gene. Accumulation of enzyme substrates leads to multisystemic clinical manifestations and multiorgan progressive damage with high morbidity and mortality. Recombinant enzyme replacement therapy (RERT) now available aims to delay or even avoid the complications of FD. The index case was a 50-year-old man with bone pain since childhood, coarse facies, angiokeratomas, anemia, renal failure, proteinuria, sinus node disease, valvular disease and massive left ventricular hypertrophy and brain ischemic alterations. FD diagnosis was confirmed during hospital admission for bacterial endocarditis leading to death. Family screening revealed an affected brother with acroparesthesia, chronic cough, sinus bradycardia, long QT interval and near-nephrotic proteinuria, now under RERT. Their mother was not screened due to stroke sequelae. This report illustrates the need for early diagnosis, family screening and treatment, aiming to change the natural history of FD.

Management Implications of Massive Left Ventricular Hypertrophy in Hypertrophic Cardiomyopathy Significantly Underestimated by Echocardiography but Identified by Cardiovascular Magnetic Resonance

Cardiovascular magnetic resonance (CMR) is a high spatial resolution, 3-dimensional tomographic imaging technique which may identify regions of massive left ventricular hypertrophy (particularly when confined to the anterolateral free wall) in which the extent of wall thickness is underestimated with traditional 2-dimensional echocardiography in patients with hypertrophic cardiomyopathy (HC). This observation may have potential implications on management strategies as extreme left ventricular hypertrophy is a primary risk factor for sudden death in HC and therefore supports an expanding role for CMR in the evaluation of HC patients.

Left ventricular non-compaction cardiomyopathy mimicking an infiltrative cardiac disease

A 60-year-old male patient, with coronary artery bypass graft due to coronary artery disease 9 years ago, without a history of prior myocardial infarction, was referred to our clinic for further cardiologic evaluation. Routine transthoracic echocardiography showed a slightly reduced left ventricular function with a massive left ventricular hypertrophy located mainly to the anterolateral and inferolateral segments, including basal segments and papillary muscles, mimicking a myocardial infiltrative disease, e.g. cardiac amyloidosis. Echocardiographic criteria for left ventricular non-compaction cardiomyopathy (LVNC) were not fulfilled. For further evaluation of the unusual asymmetric wall thickening, an echocardiographic examination with a left heart contrast agent was conducted. Deep intertrabecular recesses of the anterolateral and inferolateral segments could now be unmasked. Left ventricular hypertrabeculation-noncompaction represents rare abnormalities, diagnosed when many left ventricular trabeculations are visibly apical to the papillary muscles and intertrabecular spaces are perfused from the ventricular cavity. To our knowledge, this is the first report of a patient with LVNC mimicking an infiltrative myocardial disease in echocardiography. This case represents a possible pitfall in the diagnosis of LVNC, which could sometimes be avoided by using LV opacification agents.