Journal ArticleDOI
Loss of base-to-apex circumferential strain gradient: A specific pattern of Fabry cardiomyopathy?
Reads0
Chats0
TLDR
The aims of this study were to describe the pattern of regional strain in patients with Fabry disease and to assess whether this pattern may help differentiate patients withFabry disease from patients with sarcomeric hypertrophic cardiomyopathy (HCM).Abstract:
INTRODUCTION AND OBJECTIVES Cardiac manifestations in Fabry disease are mainly characterized by left ventricular hypertrophy (LVH). The aims of this study were (1) to describe the pattern of regional strain in patients with Fabry disease and (2) to assess whether this pattern may help differentiate patients with Fabry disease from patients with sarcomeric hypertrophic cardiomyopathy (HCM). METHODS Seventy-seven subjects were investigated: patients with Fabry disease (n=37; 57% with LVH), patients with HCM (n=21), and healthy controls (n=19). Global and segmental longitudinal and circumferential strain (CS) analyses were performed by two-dimensional speckle strain imaging. Base-to-apex longitudinal and CS gradient, defined as the peak gradient difference between averaged basal and apical strain, was calculated. RESULTS Longitudinal strain gradient did not differ between controls and Fabry patients without hypertrophy (respectively: -10±3.2 vs -8±4.3, P=.41) or between the HCM group and Fabry patients with hypertrophy (respectively: -7.5±4.5 vs -9±4.5, P=.37). The CS gradient was lower in Fabry patients without hypertrophy compared to the controls (respectively: 1±8 vs 14.2±9.5, P<.01), and lower in Fabry patients with hypertrophy compared to the HCM group (respectively: 0.5±8 vs 6±9, P<.01). Base-to-apex CS gradient was lost in both Fabry groups. CONCLUSION Loss of base-to-apex CS gradient may be a specific left ventricular deformation pattern of Fabry cardiomyopathy in patients with and without LVH.read more
Citations
More filters
Journal ArticleDOI
The Role of Cardiac Imaging in the Diagnosis and Management of Anderson-Fabry Disease.
Rebecca Perry,Ranjit Shah,Madiha Saiedi,Sanjana Patil,Anand N. Ganesan,Aleš Linhart,Joseph B. Selvanayagam +6 more
TL;DR: Echocardiography and cardiovascular magnetic resonance (CMR) offer distinctive and often complementary use to assist in the diagnosis and monitoring pharmacologic therapy in AFD, including detection of the AFD cardiac phenotype, differentiation from other forms of LVH, and patient selection for therapeutic intervention.
Journal ArticleDOI
Normative Data for Left and Right Ventricular Systolic Strain in Healthy Caucasian Italian Children by Two-Dimensional Speckle-Tracking Echocardiography.
Massimiliano Cantinotti,Marco Scalese,Raffaele Giordano,Eliana Franchi,Nadia Assanta,Marco Marotta,Cecilia Viacava,Sabrina Molinaro,Giorgio Iervasi,Giuseppe Santoro,Martin Koestenberger +10 more
TL;DR: The results confirm previous observations, showing only little variations of strain parameters with age and gender, and report pediatric echocardiographic normative data for 2D‐STE for the LV and RV andegr by using vendor‐specific software.
Journal ArticleDOI
Prominent longitudinal strain reduction of left ventricular basal segments in treatment-naïve Anderson-Fabry disease patients.
Roberta Esposito,Maurizio Galderisi,Ciro Santoro,Massimo Imbriaco,Eleonora Riccio,Angela Maria Pellegrino,Regina Sorrentino,Maria Lembo,Rodolfo Citro,Maria Angela Losi,Letizia Spinelli,Bruno Trimarco,Antonio Pisani +12 more
TL;DR: In naïve AFD patients, an early reduction of LV LS is observed, involving mainly LV basal myocardial segments, which demonstrates that apical segments LS, despite still normal, is not spared at diagnosis.
References
More filters
Journal ArticleDOI
Recommendations for chamber quantification: a report from the American Society of Echocardiography's Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiography, a branch of the European Society of Cardiology.
Roberto M. Lang,Michelle Bierig,Richard B. Devereux,Frank A. Flachskampf,Elyse Foster,Patricia A. Pellikka,Michael H. Picard,Mary J. Roman,James B. Seward,Jack S. Shanewise,Scott D. Solomon,Kirk T. Spencer,Martin St. John Sutton,William J. Stewart +13 more
TL;DR: Members of the Chamber Quantification Writing Group are: Roberto M. Lang, MD, Fase, Michelle Bierig, MPH, RDCS, FASE, Richard B. Devereux,MD, Frank A. Flachskampf, MD and Elyse Foster, MD.
Journal ArticleDOI
Recommendations for the evaluation of left ventricular diastolic function by echocardiography
Sherif F. Nagueh,Christopher P. Appleton,Thierry C. Gillebert,Paolo Marino,Jae K. Oh,Otto A. Smiseth,Alan D. Waggoner,Frank A. Flachskampf,Patricia A. Pellikka,Arturo Evangelista +9 more
TL;DR: Recommendations for the evaluation of left ventricular diastolic function by echocardiography are made and further research is needed to determine the best method for this evaluation.
Journal ArticleDOI
Recommendations for the evaluation of left ventricular diastolic function by echocardiography
Sherif F. Nagueh,Christopher P. Appleton,Thierry C. Gillebert,Paolo Marino,Jae K. Oh,Otto A. Smiseth,Alan D. Waggoner,Frank A. Flachskampf,Patricia A. Pellikka,Arturo Evangelisa +9 more
TL;DR: The assessment of left ventricular (LV) diastolic function and filling pressures is of paramount clinical importance to distinguish this syndrome from other diseases such as pulmonary disease resulting in dyspnea, to assess prognosis, and to identify underlying cardiac disease and its best treatment.
Journal ArticleDOI
2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy The Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC)
Perry M. Elliott,Aris Anastasakis,Michael A. Borger,Martin Borggrefe,Franco Cecchi,Philippe Charron,Albert Hagège,Antoine Lafont,Giuseppe Limongelli,Heiko Mahrholdt,William J. McKenna,Jens Mogensen,Petros Nihoyannopoulos,Stefano Nistri,Petronella G. Pieper,Burkert Pieske,Claudio Rapezzi,Frans H. Rutten,Christoph Tillmanns,Hugh Watkins +19 more
TL;DR: The objective of this study was to establish a baseline level of confidence that the once-in-a-lifetime implantation trial—Reduce Inappropriate Therapy protocol can be trusted to provide safe and effective treatment for cardiac arrhythmia and stroke-like episodes.
Journal ArticleDOI
Relative ‘apical sparing’ of longitudinal strain using two-dimensional speckle-tracking echocardiography is both sensitive and specific for the diagnosis of cardiac amyloidosis
Dermot Phelan,Patrick Collier,Paaladinesh Thavendiranathan,Zoran B. Popović,Mazen Hanna,Juan Carlos Plana,Thomas H. Marwick,James D. Thomas +7 more
TL;DR: A relative ‘apical sparing’ pattern of LS is an easily recognisable, accurate and reproducible method of differentiating CA from other causes of LV hypertrophy.