Case Of Noncompaction Research Articles

Kiosk 1Q-TA-08 - An Unusual Case of Myocardial Non-compaction and Mitral Annular Disjunction

Kiosk 1Q-TA-08 - An Unusual Case of Myocardial Non-compaction and Mitral Annular Disjunction

In Utero Detection and Postnatal Follow-up of a Case of LV Non-compaction

In Utero Detection and Postnatal Follow-up of a Case of LV Non-compaction

Necklace Pattern Left Ventricular Noncompaction Cardiomyopathy with Plethora of Paradoxic Septal Premature Ventricular Complexes: A Case Report and Literature Review

We report an extremely rare case of left ventricular noncompaction (LVNC) cardiomyopathy sparing the anterior ventricular wall in an interesting “necklace” pattern in parasternal short axis view in a 76-year-old female with frequent palpitation and shortness of breath for the last 6 months. Interestingly, the patient had a plethora of basal septal premature ventricular complexes (PVCs) both from the anterior and posterior aspects of the paradoxically thinned-out basal septum and they were not from the segment of noncompacted (NC) myocardium. Our case is unique and the first to describe the LVNC in an interesting shape of “necklace” sparing the left ventricular anterior, anteroseptal, and anterolateral wall and paradoxically arising plethora of septal PVCs from the thinned-out basal septum deteriorating the left ventricular function rapidly in an elderly female in her seventh decade of life. Although commonly in LVNC, the PVCs arise from the NC segment, in this unique case plethora of PVCs paradoxically arising from the basal septum were contributing toward rapid deterioration of left ventricular systolic function in an elderly patient in her seventh decade of life without the presence of conventional risk factors.

Abstract 9912: A Case of Left Ventricle Non-Compaction in an Asymptomatic Athlete

Introduction: Left ventricle non-compaction (LVNC) is defined as a primary genetic cardiomyopathy by the American Heart Association and as an unclassified cardiomyopathy by the European Society of Cardiology. Phenotypically, it is characterized by prominent trabeculations and intertrabecular recesses in the ventricular cavity. The clinical manifestations of LVNC range from an asymptomatic state to progressive heart failure and life-threatening arrhythmias. The following case highlights an athletic patient with LVNC. Case Presentation: A 22-year-old male presented to the cardiology clinic for evaluation prior to enrolling in the police academy. At age 15, an echo revealed prominent trabeculations. The patient has been a competitive soccer player since high school. Upon presentation, he was asymptomatic with non-specific T wave changes noted on ECG, stress echocardiography revealed preserved functional capacity, prominent trabeculations, and EF 55%. No arrhythmias were noted. CMR revealed a hyper-trabeculated LV predominantly at the mid-ventricular and apical segments, moderate left and right ventricular enlargement with EF 55%. No LGE enhancement was noted. Discussion: LVNC is estimated to affect 8-12 patients per 1 million annually, but the actual number is likely higher as many individuals are asymptomatic. Although the cause of LVNC is unclear, it has been associated with mutations in sarcomere encoding genes including MYH7 and MYBPC3. It remains controversial whether LVNC is a distinct disorder since it has been reported in other cardiomyopathies and since it has been reported in pregnancy and trained athletes. The current diagnostic criteria for LVNC include a ratio of non-compacted to compacted myocardium >2.1:1 as demonstrated by echo or a ratio of >2.3:1 by CMR. However, it has been proposed that trabeculation formation represents a physiological adaptation to increased loading conditions seen in pregnancy and vigorously trained athletes. Therefore, it remains unclear whether the current diagnostic criteria apply to an athletic population. Furthermore, the prognostic implications of prominent trabeculations meeting accepted diagnostic criteria for LVNC remain uncertain.

Magnetic Resonance Imaging Findings in a Rare Case of Left Ventricular Noncompaction

Left ventricular noncompaction (LVNC) is a relatively rare clinical entity with a grave outcome. Various morphological findings, both on two-dimensional echocardiography and cardiac magnetic resonance imaging (MRI), have been depicted in the literature, helping in establishing the correct diagnosis of this condition. We describe a rare case of LVNC and its MRI features.

Left ventricular noncompaction cardiomyopathy with apical septal ventricular tachycardia

We present a rare case of left ventricular noncompaction (LVNC) in a 23-year-old female with recurrent syncope with electrocardiography documentation of apical septal ventricular tachycardia (VT). Abnormal embryological myocardial maturation presenting as LVNC with electrogenic heterogenity across the noncompacted spongiform segments result in recurrent VT and mandates automated intracardiac defibrillator implantation (AICD). These subsets of patients in spite of AICD implantation require life-long broad-spectrum antiarrythmic in the form of amiodarone and beta-blocker to prevent a crisis of VT storm or sudden cardiac death.

P24-1 - A Case of Left Ventricular Noncompaction Recognized in Scrutiny for a Loss of Consciousness

A 60 years old male is a case. Although patient had consciousness disappearance several years ago and visited general hospital, but scrutiny related to cardiovascular disease was not done. During the work, there was no particular induction, causing a loss of consciousness for several tens of seconds. After recovery of consciousness, Holter ECG was performed at the hospital, and non-sustained ventricular tachycardia was recognized. Therefore, it was introduced to our hospital for the purpose of scrutiny. An elevation of BNP was observed, and cardiac ultrasound examination revealed a decrease in peripheral motion of wall motion. In the electrophysiological examination, ventricular fibrillation was easily induced by early nineteen stimulation from the apex of the right ventricle. After that it returned to normal sinus rhythm by DC defibrillation. The characteristic findings of the cardiac MRI examination were combined and judged as chronic heart failure due to impairment of left ventricular myocardial dysfunction and loss of consciousness due to ventricular arrhythmia. We transplanted an implantable defibrillator and started oral dosing of amiodarone. We will also report on the consideration.

A case of noncompaction of left ventricular myocardium detected by Ventricular fibrillation

A case of noncompaction of left ventricular myocardium detected by Ventricular fibrillation

A case of left ventricular noncompaction in pregnancy and its interdisciplinary management

A case of left ventricular noncompaction in pregnancy and its interdisciplinary management

Acute Heart Failure in a Young Female: A Case of Noncompaction

SESSION TITLE: Cardiovascular Disease Student/Resident Case Report Posters SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM INTRODUCTION: Acute onset congestive heart failure is a rare event in a young patient with minimal cardiac risk factors. We present an uncommon cause of heart failure in an otherwise healthy young female. CASE PRESENTATION: A 27 year-old Asian American female with a history of hypertension presented with generalized edema that began a week prior to admission. She reported worsening dyspnea on exertion, but denied chest pain, palpitations, light-headedness or dizziness. She was adopted and did not know her biological parents' medical history. Physical examination revealed a blood pressure of 155/120 mmHg, jugular venous distention, rhonchi in right middle and lower lung field and evidence of ascites. Her lower extremities were notable for significant edema with serous weeping. Laboratory findings were significant for BNP of 4128, and troponin of 0.24. A transthoracic echocardiogram showed an ejection fraction of less than 20%. There was also evidence of right-sided heart failure, severe tricuspid regurgitation, an apical thrombus in the left ventricle, and anatomy suggestive of non-compaction syndrome. A cardiac MRI was obtained which showed pericardial effusion with increased trabeculae in the lateral wall, which are also consistent with mild non-compaction. She was diuresed and started on anticoagulation. A loculated right pleural effusion, requiring video-assisted thoracotomy and decortication, complicated her hospital course. She had an uncomplicated recovery and was discharged twelve days postoperatively. DISCUSSION: Left ventricular noncompaction is a rare condition with an incidence of less than 0.3%. It can be genetically sporadic or familial. There is an arrest of the normal compaction process in the myocardium during embryogenesis. It is characterized by abnormal two-layered myocardium (compacted and non compacted) and thickened myocardial wall with prominent trabeculae. Although this condition may be asymptomatic, it can present as heart failure, fatal arrhythmias, thromboembolic events, or even sudden cardiac death. Between studies, prognosis is variable with mortality at follow-up from 2% to 47%. CONCLUSIONS: Left ventricular noncompaction should be considered in the differential diagnosis in young patients without cardiac risk factors with decompensated congestive heart failure. Making the correct diagnosis is important for the long-term management of patients, as well as screening of their relatives when necessary. Reference #1: Stanton C, Bruce C, Connolly H MD, Brady P, Syed I, Hodge D, Asirvatham S, Friedman P. Isolated Left Ventricular Noncompaction Syndrome. Am J Cardiol. 2009;104:1135-1138). Reference #2: Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA, Jenni R. Long-Term Follow-up of 34 Adults With Isolated Left Ventricular Noncompaction: A Distinct Cardiomyopathy With Poor Prognosis. JACC. 2000. 36(2):493-500. DISCLOSURE: The following authors have nothing to disclose: Weyman Lam, Gbolahan Ogunbayo, David Lobo No Product/Research Disclosure Information

Tricuspid aortic stenosis with systolic dysfunction in a 14-year-old boy: Critical stenosis, noncompaction or secondary fibroelastosis?

stenosis in the pediatric age group; another cause is annular hypoplasia, with the tricuspid valve being the least commonly involved. In aortic stenosis, causes of systolic dysfunction are critical stenosis, secondary fibroelastosis and noncompaction. Critical stenosis is diagnosed in the usual fashion. Secondary fibroelastosis is characterized by endocardial thickening, systolic dysfunction and distinctive bright echoes originating from the endocardium. Noncompaction is a congenital cardiomyopathy due to embryonic arrest of the normal development of the myocardium, leading to persistence of fetal myocardium in postnatal life. Although described in infants with aortic stenosis or atresia, the presence of noncompaction beyond infancy associated with aortic valve disease is exceedingly rare. The echocardiographic appearance of the ventricular myocardium in noncompaction is the distinctive presence of deep intramyocardial recesses. A case of left ventricular noncompaction in association with a tricuspid aortic valve with severe aortic stenosis with systolic dysfunction in a 14-year-old boy is reported.

A case of Non-compaction of Left Ventricle Coexistent with Juvenile Nephronophthisis. Is this Another Presentation of Ciliopathy?

Nephronophthisis is a chronic tubulo-interstitial nephritis which can progress to end-stage renal disease. Juvenile nephronophthisis is the most common type of nephronophthisis, which accounts for 5-10% of the cases of pediatric end stage renal diseases. Left ventricular non-compaction (LVNC), a rare form of cardiomyopathy, is the result of intrauterine arrest of compaction of the endomyocardial morphogenesis. Clinical manifestations of LVNC range from asymptomatic child to a progressive deterioration in the cardiac function, congestive heart failure, arrhythmias, systemic thromboembolism and sudden cardiac death. This report presents a case of juvenile nephronophthisis with LVNC. A 15-year-old boy was referred to our nephrology outpatient clinic with a 6-month history of non-specific complaints such as lethargy, anorexia, polydipsia, polyuria, and pallor. Abdominal sonography showed a generalized increase in the parenchymal echo of kidneys. Renal biopsy was performed for him which showed nephronophthisis. Echocardiography was done and revealed LVNC. He was discharged with training for careful follow-up. Our reported case had nephronophthisis and LVNC. To the best of our knowledge, there is no report of this combination in the literature. This suggests that LVNC may be another presentation of cilia involvement. The clinical coexistence of LVNC and nephronophthisis could guide us to better localize and discover the underlying genetic mutations and the role of ciliopathies in various human diseases; Therefore, further research with a special focus on potential common derangement of cilia and protein products in these diseases is recommended. Keywords: Juvenile nephronophthisis; Isolated Noncompaction of the Ventricular Myocardium; Ciliary Motility Disorders

A case of noncompaction at all segments of both right and left ventricles.

Background. Noncompaction/hypertrabeculation left ventricle (NCM/HVM) is most commonly reported in one or more segments of left ventricle and sometimes both ventricles. In this case, we present noncompaction of all segments of right and left ventricle, in a young man with mental retardation. Case Presentation. A 19-year-old male was referred to us with sudden dyspnea at rest and chest discomfort. He was a known case of mental retardation. He was born full term with birth weight = 1250 grams. On physical examination. A systolic murmur (II/VI) at left sternal border was heard. ECG showed increased voltage in precordial lead and deep ST segment depression. Chest X-ray (CXR) was within normal limits. Transthoracic echocardiography showed situs solitus, D loop, normal connection of great vessels, noncompaction LV at all segments (noncompaction/compaction = 2.5/0.5) with moderate systolic dysfunction (LVEF = 40%), diastolic dysfunction grade II, normal RV size with mild systolic dysfunction and hypertrabeculation, mild tricuspid regurgitation (TR), and normal pulmonary artery systolic pressure. After injection of agitated saline some bubbles were passed from right to left through patent foramen oval (PFO). Conclusions. Extensive sinusoid formation and trabeculation of RV and nearby all LV segments and its association with mental retardation suggest presence of strong genetic background.

A case of noncompaction of left ventricular myocardium detected by cardiopulmonary arrest

A case of noncompaction of left ventricular myocardium detected by cardiopulmonary arrest

Left ventricular noncompaction cardiomyopathy: updated review

The first case of noncompaction was described in 1932 after an autopsy performed on a newborn infant with aortic atresia/coronary-ventricular fistula. Isolated noncompaction cardiomyopathy was first described in 1984. A review on selected/relevant medical literature was conducted using Pubmed from 1984 to 2013 and the pathogenesis, clinical features, and management are discussed. Left ventricular noncompaction (LVNC) is a relatively rare congenital condition that results from arrest of the normal compaction process of the myocardium during fetal development. LVNC shows variability in its genetic pattern, pathophysiologic findings, and clinical presentations. The genetic heterogeneity, phenotypical overlap, and variety in clinical presentation raised the suspicion that LVNC might just be a morphological variant of other cardiomyopathies, but the American Heart Association classifies LVNC as a primary genetic cardiomyopathy. The familiar type is common and follows a X-linked, autosomal-dominant, or mitochondrial-inheritance pattern (in children). LVNC can occur in isolation or coexist with other cardiac and/or systemic anomalies. The clinical presentations are variable ranging from asymptomatic patients to patients who develop ventricular arrhythmias, thromboembolism, heart failure, and sudden cardiac death. Increased awareness over the last 25 years and improvements in technology have increased the identification of this illness and improved the clinical outcome and prognosis. LVNC is commonly diagnosed by echocardiography. Other useful diagnostic techniques for LVNC include cardiac magnetic resonance imaging, computerized tomography, and left ventriculography. Management is symptom based and patients with symptoms have a poorer prognosis. LVNC is a genetically heterogeneous disorder which can be associated with other anomalies. Making the correct diagnosis is important because of the possible associations and the need for long-term management and screening of living relatives.

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.

A Rare Case of Biventricular Non Compaction with Associated Pentad –An Echocardiographic Diagnosis

Noncompaction of the ventricular myocardium is a cardiomyopathy caused by the arrest of normal embryogenesis of the ventricles. It is classified as isolated noncompaction of the ventricles and ventricular noncompaction associated with other conditions. The left ventricle is the most affected site, but right ventricular involvement has also been reported. As the right ventricle is normally trabeculated, there are controversies regarding noncompaction of right ventricle. We present a case of biventricular noncompaction associated with severe aortic coarctation and arch hypoplasia, patent ductus arteriosus, bicuspid aortic valve, coronary cameral fistula and atrial septal defect.

A Case of Left Ventricular Noncompaction Accompanying Fasciculo-Ventricular Accessory Pathway and Atrial Flutter

Left ventricular hypertrabeculation/noncompaction (LVHT) is an uncommon type of genetic cardiomyopathy characterized by trabeculations and recesses within the ventricular myocardium. LVHT is associated with diastolic or systolic dysfunction, thromboembolic complications, and arrhythmias, including atrial fibrillation, ventricular arrhythmias, atrioventricular block and Wolff-Parkinson-White syndrome. Herein, we describe a patient who presented with heart failure and wide-complex tachycardia. Echocardiography showed LVHT accompanied with severe mitral regurgitation. The electrophysiologic study revealed a fasciculo-ventricular accessory pathway and atrial flutter (AFL). The AFL was successfully treated with catheter ablation.

A Case of Left Ventricular Noncompaction Accompanying Fasciculo-Ventricular Accessory Pathway and Atrial Flutter

Left ventricular hypertrabeculation/noncompaction (LVHT) is an uncommon type of genetic cardiomyopathy characterized by trabeculations and recesses within the ventricular myocardium. Frequently associated arrhythmias included atrial fibrillation, ventricular arrhythmias, atrioventricular block, and Wolff-Parkinson-White syndrome. A 29-year-old male presented with palpitations and shortness of breath. The ECG showed pre-excitation and anteroseptal accessory pathway was suspected. The echocardiogram revealed prominent trabeculations and deep recesses in the left ventricles. The recesses were perfused by intraventricular flow. During the echocardiographic examination, tachycardia developed spontaneously. The ECG showed wide complex tachycardia and the QRS morphology was similar to that of sinus rhythm. An electrophysiologic study was performed. Decremental atrial pacing caused prolongation of both AH and AV intervals in parallel. The HV interval and the degree of preexcitation remained unchanged from both right atrial and coronary sinus pacing with atrial extrastimuli. The His bundle pacing failed to normalize the QRS width. A diagnosis of fasciculo-ventricular accessory pathway was made. Decremental pacing on the proximal coronary sinus area induced typical atrial flutter, which was identical to the clinical tachycardia. A line of block was made across the cavo-tricuspid isthmus (CTI). A rare presentation of LVHT with fasciculo-ventricular accessory pathway and atrial flutter was successfully treated with conventional CTI block.

Noncompaction of the Right Ventricle

Noncompaction of the ventricular myocardium is a disease characterized by an increase of the ventricular trabecular meshwork caused by arrest of the normal endomyocardial morphogenesis (Figs. 1, 2, 3). In accordance with the normal human anatomy, the left ventricular wall is well compacted with a few thin trabeculae; on the contrary, the normal right ventricular wall is furrowed by many trabeculae (the trabecula of the marginal septum as well as other ones). For this reason, the term ‘‘noncompaction’’ usually refers to an exclusive or prevalent disease of the left ventricle [1–16]. Recently Song and Aragona et al. [1–3] reported two cases of isolated right-ventricular noncompaction. According to the data coming from scientific literature and from our own experience, in some patients noncompaction is biventricular [5, 6], and an increase of the right ventricular meshwork is often evident in such cases, even more so than on the left side. The main diagnostic criterion of noncompaction, that is, the only one that is accepted and recognized, is evaluation of the ratio between the spongiosus and the compact thickness of the ventricular wall, which must be [2 [6–16]. This ratio is easy to calculate for the left ventricle; on the contrary, it is more difficult to calculate for the right ventricle. Only one case of right-ventricular noncompaction has been reported in the literature [7, 8]. In our personal clinical experience, we have found that many cases of biventricular noncompaction are reported in the register of Italian Society of Cardiovascular Echocardiography. Recently two patients with an inexplicable dilatation of the right ventricle caught our attention: Both of them showed a more prominent trabecular meshwork on