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Kadir Caliskan - One of the best experts on this subject based on the ideXlab platform.

  • State-of-the art review: Noncompaction Cardiomyopathy in pediatric patients
    Heart Failure Reviews, 2021
    Co-Authors: Sofie Rohde, Danielle Majoor-krakauer, Rahatullah Muslem, Emrah Kaya, Michel Dalinghaus, Jaap I. Waning, Jeffery Towbin, Kadir Caliskan
    Abstract:

    Noncompaction Cardiomyopathy (NCCM) is a disease characterized by hypertrabeculation, commonly hypothesized due to an arrest in compaction during fetal development. In 2006, NCCM was classified as a distinct form of Cardiomyopathy (CMP) by the American Heart Association. NCCM in childhood is more frequently familial than when diagnosed in adulthood and is associated with other congenital heart diseases (CHDs), other genetic CMPs, and neuromuscular diseases (NMDs). It is yet a rare cardiac diseased with an estimated incidence of 0.12 per 100.000 in children up to 10 years of age. Diagnosing NCCM can be challenging due to non-uniform diagnostic criteria, unawareness, presumed other CMPs, and presence of CHD. Therefore, the incidence of NCCM in children might be an underestimation. Nonetheless, NCCM is the third most common Cardiomyopathy in childhood and is associated with heart failure, arrhythmias, and/or thromboembolic events. This state-of-the-art review provides an overview on pediatric NCCM. In addition, we discuss the natural history, epidemiology, genetics, clinical presentation, outcome, and therapeutic options of NCCM in pediatric patients, including fetuses, neonates, infants, and children. Furthermore, we provide a simple classification of different forms of the disease. Finally, the differences between the pediatric population and the adult population are described.

  • FLNC missense variants in familial Noncompaction Cardiomyopathy
    Cardiogenetics, 2019
    Co-Authors: Jaap I. Van Waning, Kadir Caliskan, Yvonne M. Hoedemaekers, Wouter P. Te Rijdt, Arne S. I Jpma, Daphne Heijsman, Elke S. Hoendermis, Tineke P. Willems, Arthur Van Den Wijngaard, Albert J. H. Suurmeijer
    Abstract:

    textabstractThe majority of familial Noncompaction Cardiomyopathy (NCCM) is explained by pathogenic variants in the same sarcomeric genes that are associated with hypertrophic (HCM) and dilated (DCM) Cardiomyopathy. Pathogenic variants in the filamin C gene (FLNC) have been linked to HCM and DCM. We expand the spectrum of FLNC related cardiomyopathies by presenting two families with likely pathogenic FLNC variants showing familial segregation of NCCM and concurrent coarctation of the aorta and/or mitral valve abnormalities.

  • Multimodality Imaging, Diagnostic Challenges and Proposed Diagnostic Algorithm for Noncompaction Cardiomyopathy
    Noncompaction Cardiomyopathy, 2019
    Co-Authors: Osama Ibrahim Ibrahim Soliman, Folkert J. Ten Cate, Jackie Mcghie, Bernard P. Paelinck, Kadir Caliskan
    Abstract:

    Noncompaction Cardiomyopathy (NCCM) is a clinical-patho-anatomic entity, disputably believed to result from abnormal arrest in embryonic endomyocardial morphogenesis. Introduction of echocardiography has led to recognition of NCCM for the first time in 1984. During almost 35 years of technical improvements in imaging alongside a more dedicated research of NCCM, more knowledge has developed alongside uncertainties concerning every aspect of the disease. In this chapter, we present the role of multimodality imaging in NCCM alongside diagnostic uncertainties and practical aspects on how to overcome these challenges. Limitations of the current diagnostic criteria will be reviewed. The inter-technique differences will be contrasted. Finally, future aspirations will be outlined in providing a more thoughtful appraisal towards NCCM diagnosis.

  • Malignant Arrhythmias and Sudden Cardiac Death in Patients with Noncompaction Cardiomyopathy: Prevalence, Prevention, and Use of Implantable Cardiac Defibrillators
    Noncompaction Cardiomyopathy, 2019
    Co-Authors: Emrah Kaya, Tamas Szili-torok, Martijn Otten, Sing-chien Yap, Kadir Caliskan
    Abstract:

    Noncompaction Cardiomyopathy (NCCM) was first described almost a half century ago, it initially began as an unusual autopsy finding. NCCM has a highly variable clinical presentation and is usually diagnosed when the condition becomes symptomatic or when complications occur in patients. These complications are end-stage heart failure, lethal arrhythmias and thromboembolic events [8–10]. Sudden cardiac death (SCD) is its most striking and visible consequence. The mortality in patients with NCCM has been reported in 18% of adults and 0–13% in children. Most SCD within the NCCM population are due to arrhythmias caused by ventricular tachycardia (VTs) and ventricular fibrillation. The implantable cardioverter defibrillator (ICD) is the single most effective therapy to prevent SCD. However, appropriate risk stratification in these patients are not yet established.

  • Cardiac Phenotypes, Genetics, and Risks in Familial Noncompaction Cardiomyopathy
    Journal of the American College of Cardiology, 2019
    Co-Authors: Jaap I. Van Waning, Kadir Caliskan, Michelle Michels, Yvonne M. Hoedemaekers, Arend F.l. Schinkel, Alexander Hirsch, Michiel Dalinghaus, Marja W. Wessels, A. Ijpma, Robert M.w. Hofstra
    Abstract:

    Abstract Background There is overlap in genetic causes and cardiac features in Noncompaction Cardiomyopathy (NCCM), hypertrophic Cardiomyopathy (HCM), and dilated Cardiomyopathy (DCM). Objectives The goal of this study was to predict phenotype and outcome in relatives according to the clinical features and genotype of NCCM index cases. Methods Retrospective DNA and cardiac screening of relatives of 113 families from 143 index patients were used to classify NCCM cases according to the cardiac phenotype. These cases were classified as isolated NCCM, NCCM with left ventricular (LV) dilation (DCM), and NCCM with LV hypertrophy (HCM). Results In 58 (51%) families, screening identified 73 relatives with NCCM and 34 with DCM or HCM without NCCM. The yield of family screening was higher in families with a mutation (p  Conclusions The phenotype of relatives may be predicted according to the NCCM phenotype and the mutation of index patients. NCCM phenotypes were related to outcome. In this way, clinical and genetic features of index patients may help prediction of outcome in relatives.

James Lafferty - One of the best experts on this subject based on the ideXlab platform.

  • Sudden cardiac death in isolated right ventricular hypertrabeculation/Noncompaction Cardiomyopathy.
    Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology Inc, 2017
    Co-Authors: Soad Bekheit, Boutros Karam, Farshid Daneshvar, Julie Zaidan, Rabih Tabet, Jonathan Spagnola, James Lafferty
    Abstract:

    Hypertrabeculation/Noncompaction of the myocardium is a rare disorder that involves most commonly the left ventricle of the heart and it has been recognized as a distinct Cardiomyopathy by the World Health Organization. However, it is extremely rare for this condition to involve exclusively the right ventricle. We report the cases of three patients who presented with ventricular tachyarrhythmia and sudden cardiac death. They were found to have isolated right ventricular hypertrabeculation/Noncompaction on echocardiography. This supports the hypothesis that this condition is highly arrhythmogenic and is associated with high mortality similarly to the left ventricular hypertrabeculation/Noncompaction Cardiomyopathy.

  • Isolated Left Ventricular Noncompaction Cardiomyopathy diagnosed by Transesophageal Echocardiography
    Clinical Medicine Insights. Cardiology, 2011
    Co-Authors: Tariq Bhat, James Lafferty, Sumaya Teli, Georges Abou Rjaili, Yefim Olkovsky, Thomas Costantino
    Abstract:

    Isolated Noncompaction of the ventricular myocardium has often been misdiagnosed as other cardiomyopathies because it is a relatively recently described Cardiomyopathy with literature limited to case reports and case series and little awareness among physicians. We are reporting a case of isolated left ventricular Noncompaction Cardiomyopathy that was misdiagnosed for over two decades.

Marek Joukal - One of the best experts on this subject based on the ideXlab platform.

Attila Nemes - One of the best experts on this subject based on the ideXlab platform.

  • Prevalence and Prevention of Thromboembolic Events in Noncompaction Cardiomyopathy
    Noncompaction Cardiomyopathy, 2019
    Co-Authors: Attila Nemes
    Abstract:

    Noncompaction Cardiomyopathy (NCCM) is a rare Cardiomyopathy characterized by prominent myocardial trabeculations and deep intertrabecular recesses in the left ventricular (LV) cavity. Due to its special phenotype, typical potential complications are heart failure, arrhythmias, and thromboembolic events. Thromboembolic events like stroke is a feared complication, and unfortunately not uncommon in NCCM patients. Cardiac emboli are theorized to result from thrombus formation within the intertrabecular recesses, especially in patients with reduced systolic function. Unfortunately, due to its rarity and relatively young disease entity, evidence-based recommendations for preventing thromboembolic events in NCCM have not been established. According to recent literature, atrial fibrillation (AF) is relatively common in patients with NCCM and therefore, oral anticoagulants, including vitamin K antagonists (VKA) and new oral anticoagulants (NOAC), seem to be reasonable to reduce thromboembolic rate. In case of a history of previous stroke and thromboembolism, VKA or NOAC use is according the current clinical insights beyond any doubt. In NCCM patient with an systolic dysfunction, but sinus rhythm, overall benefit of preventive oral anticoagulation should be yet demonstrated.

  • A kétdimenziós echokardiográfia és háromdimenziós speckle-tracking echokardiográfia összehasonlító vizsgálata a balkamra-funkció megítélésében Noncompaction cardiomyopathiában. Eredmények a MAGYAR-Path Tanulmányból | Comparative evaluation of left ve
    2013
    Co-Authors: Anita Kalapos, Péter Domsik, Tamás Forster, Attila Nemes
    Abstract:

    Bevezetes: A Noncompaction cardiomyopathia a myocardium kompaktalodasanak hianyaban kialakulo korkep, amely a bal kamra diszfunkciojaval jar egyutt. Celkitűzes: Jelen tanulmany celja a balkamra-diszfunkcio osszehasonlito vizsgalata volt ketdimenzios echokardiografia es haromdimenzios speckle-tracking echokardiografia soran Noncompaction cardiomyopathiaban. Modszer: Jelen tanulmanyban het, Noncompaction cardiomyopathiaban szenvedő beteget (62,9±8,5 ev, harom ferfi) es 10, korban es nemben egyeztetett kontrollesetet (60,7±7,7 ev, ket ferfi) vizsgaltak ketdimenzios echokardiografia es haromdimenzios speckle-tracking echokardiografia soran a bal kamra funkciojanak vizsgalata celjabol. Eredmenyek: Emelkedett bal kamrai vegdiasztoles es vegszisztoles terfogatertekek, valamint csokkent bal kamrai ejekcios frakcio volt igazolhato Noncompaction cardiomyopathia fennallasa eseten. Valamennyi haromdimenzios speckle-tracking echokardiografia soran szamitott strainparameter csokkentnek bizonyult Noncompaction cardiomyopathia eseten a kontrollokhoz kepest. A haromdimenzios speckle-tracking echokardiografia soran mert rotacios parameterek a bal kamra apicalis es basalis szegmentumainak egyiranyu mozgasat („rigid body rotation”) igazoltak Noncompaction cardiomyopathiaban. Kovetkeztetesek: A bal kamra funkcioja es szegmentumainak kontraktilitasa lenyegesen beszűkult Noncompaction cardiomyopathiaban. A bal kamrai csavarodas (twist) Noncompaction cardiomyopathia fennallasa eseten hianyzik. Orv. Hetil., 2013, 154, 1352–1359. | Introduction: Noncompaction Cardiomyopathy develops due to the absence of myocardial compaction, and is associated with left ventricular dysfunction. Aim: The aim of the study was to evaluate comparatively left ventricular dysfunction in patients with Noncompaction Cardiomyopathy using two-dimensional echocardiography and three-dimensional speckle-tracking echocardiography. Method: The present study comprised of 7 patients with Noncompaction Cardiomyopathy (62.9±8.5 years, 3 males) and 10 age- and gender-matched healthy controls (60.7±7.7 years, 2 males). All patients were examined by two-dimensional echocardiography and three-dimensional speckle-tracking echocardiography for the evaluation of left ventricular function. Results: Increased left ventricular end-diastolic and end-systolic volumes and reduced left ventricular ejection fraction were detected in patients with Noncompaction Cardiomyopathy as compared to those measured in controls. All three-dimensional speckle-tracking echocardiography-derived strain parameters of patients with Noncompaction Cardiomyopathy were found to be reduced as compared to the values of controls. Three-dimensional speckle-tracking echocardiography-derived rotational parameters showed movements of the apical and basal segments in the same direction suggesting ’rigid body rotation’ in all Noncompaction Cardiomyopathy cases. Conclusions: Left ventricular function and contractility are severely reduced in patients with Noncompaction Cardiomyopathy. Absence of left ventricular twist could be demonstrated in a series of Noncompaction Cardiomyopathy patients. Orv. Hetil., 2013, 154, 1352–1359.

  • No relationship between left ventricular radial wall motion and longitudinal velocity and the extent and severity of Noncompaction Cardiomyopathy
    Cardiovascular ultrasound, 2012
    Co-Authors: Kadir Caliskan, Osama Ibrahim Ibrahim Soliman, Ron T. Van Domburg, Maarten L. Simoons, Attila Nemes, Marcel L. Geleijnse
    Abstract:

    Background Noncompaction Cardiomyopathy (NCCM) is characterized by a prominent trabecular meshwork and deep intertrabecular recesses. Although systolic dysfunction is common, limited information is available on differences in wall motion of the normal compacted and noncompacted segments. The purpose of this study was to assess radial wall motion and longitudinal wall velocity in patients with NCCM, according to the extent and severity of Noncompaction.

  • Alterations in aortic elasticity in Noncompaction Cardiomyopathy
    The International Journal of Cardiovascular Imaging, 2008
    Co-Authors: Attila Nemes, Marcel L. Geleijnse, Kadir Caliskan, Ashraf M. Anwar, Osama I. I. Soliman, Folkert J. Ten Cate
    Abstract:

    Background Noncompaction Cardiomyopathy (NCCM) is a recently recognized disorder frequently associated with systolic and diastolic heart failures. This study was designed to examine aortic stiffness in NCCM patients and to compare these results to age- and gender-matched controls. Methods A total of 20 patients with typical echocardiographic features of NCCM (age 38 ± 16 years, eight males) were investigated. Their results were compared to 20 age- and gender-matched controls. All subjects underwent a complete two-dimensional transthoracic echocardiographic examination. Systolic (SD) and diastolic (DD) ascending aortic diameters were recorded in M-mode at a level of 3 cm above the aortic valve from a parasternal long-axis view. Aortic stiffness index ( β ) was calculated as a characteristic of aortic elasticity, as ln(SBP/DBP)/[(SD - DD)/DD], where SBP and DBP are the systolic and diastolic blood pressures, respectively, and ln is the natural logarithm. Results The number of noncompacted segments in the NCCM patients was 4.6 ± 2.0. NCCM patients had significantly increased left ventricular dimensions and reduced left ventricular ejection fraction. Compared to controls, aortic stiffness index ( β ) was significantly increased in NCCM patients (8.3 ± 5.2 vs. 3.5 ± 1.1, p  

  • Alterations in aortic elasticity in Noncompaction Cardiomyopathy.
    The international journal of cardiovascular imaging, 2007
    Co-Authors: Attila Nemes, Marcel L. Geleijnse, Kadir Caliskan, Osama Ibrahim Ibrahim Soliman, Ashraf M. Anwar, Folkert J. Ten Cate
    Abstract:

    Background Noncompaction Cardiomyopathy (NCCM) is a recently recognized disorder frequently associated with systolic and diastolic heart failures. This study was designed to examine aortic stiffness in NCCM patients and to compare these results to age- and gender-matched controls.

Tamas Szili-torok - One of the best experts on this subject based on the ideXlab platform.

  • Malignant Arrhythmias and Sudden Cardiac Death in Patients with Noncompaction Cardiomyopathy: Prevalence, Prevention, and Use of Implantable Cardiac Defibrillators
    Noncompaction Cardiomyopathy, 2019
    Co-Authors: Emrah Kaya, Tamas Szili-torok, Martijn Otten, Sing-chien Yap, Kadir Caliskan
    Abstract:

    Noncompaction Cardiomyopathy (NCCM) was first described almost a half century ago, it initially began as an unusual autopsy finding. NCCM has a highly variable clinical presentation and is usually diagnosed when the condition becomes symptomatic or when complications occur in patients. These complications are end-stage heart failure, lethal arrhythmias and thromboembolic events [8–10]. Sudden cardiac death (SCD) is its most striking and visible consequence. The mortality in patients with NCCM has been reported in 18% of adults and 0–13% in children. Most SCD within the NCCM population are due to arrhythmias caused by ventricular tachycardia (VTs) and ventricular fibrillation. The implantable cardioverter defibrillator (ICD) is the single most effective therapy to prevent SCD. However, appropriate risk stratification in these patients are not yet established.

  • The Prevalence of Early Repolarization in Patients with Noncompaction Cardiomyopathy Presenting with Malignant Ventricular Arrhythmias
    Journal of cardiovascular electrophysiology, 2012
    Co-Authors: Kadir Caliskan, Ron T. Van Domburg, Maarten L. Simoons, Barbara Ujvari, Tamas Bauernfeind, Dominic A.m.j. Theuns, Ferdi Akca, Luc Jordaens, Tamas Szili-torok
    Abstract:

    Early Repolarization in Noncompaction Cardiomyopathy. Background Early repolarization (ER) is associated with malignant ventricular arrhythmias, including ventricular fibrillation (VF) and sudden cardiac death (SCD). One possible mechanism is increased trabeculation with deep intramyocardial invagination, carrying the Purkinje system deeper into the myocardium resulting in delayed depolarization and inhomogenous repolarization. Noncompaction Cardiomyopathy (NCCM) is a recently classified, primary Cardiomyopathy with excessive trabeculations. In these patients ventricular arrhythmias, including sustained VT and VF, occur frequently. The aim of this study was to determine the prevalence of ER in NCCM patients, especially in those primarily presenting with malignant ventricular arrhythmias or SCD. Methods We analyzed prospective data from our NCCM registry including 84 patients, median age: 40 (3-79) years. Results Fourteen patients (17%) initially presented with sustained VT (n = 5) or VF (n = 9) and 70 (83%) with heart failure or else. After the exclusion of 20 patients with the left bundle branch block, 25 (39%) NCCM patients had ER; 3 (6%) located in inferior leads, 14 (27%) in lateral leads, and 8 (15%) in both. None had ER in leads V1 to V3. In those presenting with VT/VF, 9/12 (75%) had ER (2 in inferior leads, 3 in lateral leads and 4 in both), versus 16/52 (31%) in the other patients (P = 0.02). If the NCCM population was dichotomized according to the presence or absence of ER, the long-term outcome for VT/VF appeared worse in the ER positive patients (P = 0.05). Conclusion There is a high prevalence of ER in NCCM patients, especially in those who present with malignant ventricular arrhythmias. (J Cardiovasc Electrophysiol, Vol. 23, pp. 938-944, September 2012).

  • Indications and Outcome of Implantable Cardioverter-Defibrillators for Primary and Secondary Prophylaxis in Patients with Noncompaction Cardiomyopathy
    Journal of cardiovascular electrophysiology, 2011
    Co-Authors: Kadir Caliskan, Marcel L. Geleijnse, Ron T. Van Domburg, Aggie H.m.m. Balk, Dominic A.m.j. Theuns, Luc Jordaens, Tamas Szili-torok, Attila Kardos, Maarten L. Simoons
    Abstract:

    Prophylactic ICDs for Noncompaction Cardiomyopathy. Background: Noncompaction Cardiomyopathy (NCCM) is a rare, primary Cardiomyopathy, with initial presentation of heart failure, emboli, or arrhythmias, including sudden cardiac death. Implantable cardioverter-defibrillators (ICDs) are frequently used for primary and secondary prevention in different Cardiomyopathy patients, but data about ICD in NCCM are scarce. The aim of this study was, therefore, to investigate ICD indications and outcomes in NCCM patients. Methods and Results: We collected prospective data from our NCCM cohort (n = 77 pts, mean age: 40 ± 14 years). ICD was implanted in 44 (57%) patients with NCCM according to the current ICD guidelines for nonischemic cardiomyopathies: in 12 for secondary prevention (7 × ventricular fibrillation, 5 × sustained ventricular tachycardia [VT]) and in 32 patients for primary prevention (heart failure/severe LV dysfunction). During a mean follow-up of 33 ± 24 months, 8 patients presented with appropriate ICD shocks due to sustained VT after median 6.1 [1–16] months. This included 4 of 32 (13%) patients in the primary prevention group and 4 of 12 (33%) in the secondary prevention group (P = 0.04). 9 patients presented with inappropriate ICD therapy: 6 (19%) in the primary and 3 (25%) in the secondary prevention group, at a median follow-up of 4 (2–23) months. Conclusions: In our cohort of NCCM patients, an ICD was frequently implanted for primary or secondary prevention of sudden cardiac death. At follow-up, frequent appropriate ICD therapy was observed in both groups, supporting the application of current ICD guidelines for primary and secondary prevention of sudden cardiac death in NCCM. (J Cardiovasc Electrophysiol, Vol. 22, pp. 898-904, August 2011)