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      Three-dimensional echocardiography for the assessment of left ventricular geometry and papillary muscle morphology in hypertrophic cardiomyopathy

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          Abstract

          Background

          Hypertrophic cardiomyopathy (HC) is characterized by left ventricular (LV) hypertrophy and associated with papillary muscle (PM) abnormalities. The aim of this study was to evaluate the utility of three-dimensional echocardiography (3DE) for the geometric assessment of LV hypertrophy and PM morphology.

          Methods

          The study included 24 patients with an established diagnosis of HC and 31 healthy controls. 3DE was performed using an iE33 or EPIQ 7C ultrasound system with an X5-1 transducer. QLAB software was used for the 3D analysis of LV wall thickness (LVWT) and PM morphology and hypertrophy; the number and cross-sectional area (CSA) of anterolateral and posteromedial PMs; and the presence of bifid or accessory PMs.

          Results

          Patients with HC had a larger LVWT compared to controls in all segments ( p < 0.001), and LVWT was largest in the midventricular septal segment (2.12 ± 0.68 cm). The maximum LVWT followed a spiral pattern from the LV base to the apex. The CSA of both anterolateral and posteromedial PMs was larger in patients with HC than in controls (1.92 vs. 1.15 cm 2; p = 0.001 and 1.46 vs. 1.08 cm 2; p = 0.033, respectively). The CSA of the posteromedial PM was larger in patients with LVOT obstruction than in those without (2.64 vs 1.16 cm 2, p = 0.021).

          Conclusions

          3DE allows the assessment of LV geometry and PM abnormalities in patients with HC. 3DE demonstrated that the maximum hypertrophy was variable and generally located in a spiral from the LV base to the apex.

          Sommario

          Premessa

          La cardiomiopatia ipertrofica (HC) è caratterizzata da ipertrofia del ventricolo sinistro (LV) e associata ad anomalie del muscolo papillare (PM).

          Scopo dello studio

          Lo scopo di questo lo studio è stato quello di valutare l’utilità dell’ecocardiografia tridimensionale (3DE) nell’ottenere una valutazione geometrica dell’ipertrofia del VS e della morfologia del PM.

          Metodi

          Lo studio ha incluso 24 pazienti con diagnosi accertata di HC e 31 controlli sani. La 3DE è stata eseguita utilizzando un sistema ecografico iE33 o EPIQ 7C con un trasduttore X5-1. Il software QLAB è stato utilizzato per l’analisi 3D dello spessore della parete del LV (LVWT) e della morfologia e ipertrofia del PM; il numero e l’area della sezione trasversale (CSA) del PM anterolaterale e posteromediale e definire la presenza di PM bifidi o accessori.

          Risultati

          I pazienti con HC avevano una LVWT maggiore rispetto ai controlli in tutti i segmenti ( p < 0,001) e LVWT era maggiore nel segmento del setto medio-ventricolare (2,12 ± 0,68 cm). La LVWT massima seguiva uno schema a spirale dalla base LV all’apice. La CSA di entrambi i PM anterolaterali e posteromediali era maggiore nei pazienti con HC rispetto ai controlli (1,92 vs 1,15 cm 2; p = 0,001 e 1,46 vs 1,08 cm 2; p = 0,033 rispettivamente). La CSA del PM posteromediale era maggiore nei pazienti con ostruzione LVOT rispetto a quelli senza (2,64 vs 1,16 cm 2, p = 0,021).

          Conclusioni

          La 3DE consente di valutare la geometria LV e le anomalie di PM in pazienti con HC. La 3DE ha dimostrato che l’ipertrofia massima era variabile e generalmente conformata a spirale dalla base del LV all’apice.

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          Most cited references19

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          Diversity of structural mitral valve alterations in hypertrophic cardiomyopathy.

          Hypertrophic cardiomyopathy (HCM) is characterized by an asymmetrically hypertrophied left ventricle and is regarded as a disease of cardiac muscle. To assess the possibility that the mitral valve itself may be involved in the disease process, we studied mitral valves from 94 patients with HCM and 45 normal control subjects. The area of the mitral leaflets was increased in patients with HCM compared with control subjects (12.9 +/- 3.7 versus 8.7 +/- 2.0 cm2; p less than 0.001). For the overall group of patients, this increase was largely caused by an increase in anterior leaflet length (2.2 +/- 0.5 cm for HCM versus 1.8 +/- 0.3 cm for control subjects; p less than 0.001), because circumference did not differ between the two groups. Mitral leaflet area was increased (greater than or equal to 12.0 cm2) in 55 (58%) of the 94 valves. In 12 of these 55 valves, both the anterior and posterior leaflets were enlarged; the other 43 valves had asymmetrical or segmental enlargement of either the anterior leaflet (36 patients) or a portion of posterior leaflet (seven patients). In addition, nine patients had a congenital malformation of the mitral apparatus in which one or both papillary muscles inserted directly into anterior mitral leaflet (mitral valve area was normal in seven of the nine). Sixty-two (66%) of 94 mitral valves had a constellation of structural malformations, including increased leaflet area and elongation of the leaflets or anomalous papillary muscle insertion directly into anterior mitral leaflet. These findings expand the morphological definition of HCM by demonstrating that the disease process is not confined to cardiac muscle but rather many patients also have structural abnormalities of the mitral valve that are unlikely to be acquired or secondary to mechanical factors.
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            Hypertrophic cardiomyopathy. The importance of the site and the extent of hypertrophy. A review.

            Hypertrophic cardiomyopathy is a diverse clinical and pathophysiologic entity that involves principally the left ventricle and is caused by asymmetric or concentric hypertrophy of unknown cause. If asymmetric, the hypertrophy is usually greatest in the ventricular septum, but variations occur in which the hypertrophy may be maximal at the apex, at the midventricular level, or, rarely, in the free wall of the left ventricle. Right ventricular involvement is usually less evident. The principal abnormality in systole is the obstruction to left ventricular outflow caused by upper septal hypertrophy narrowing the outflow tract and setting the stage for Venturi forces to cause systolic anterior motion of the anterior or posterior mitral leaflets. The time of onset and duration of mitral leaflet-septal contact determine the magnitude of the pressure gradient. Mitral regurgitation invariably accompanies the obstruction to outflow. Ventriculomyotomy-myectomy surgery, by thinning the septum and widening the outflow tract, abolishes the abnormal mitral leaflet motion and, consequently, the obstruction to outflow and the mitral regurgitation. This form of surgery more dramatically relieves the systolic abnormalities and the accompanying symptoms than any form of medical therapy available today. The extent of hypertrophy is believed to be the principal determinant of the impaired left ventricular relaxation and increased chambers stiffness (decreased compliance) that characterize diastole in hypertrophic cardiomyopathy. Relaxation is impaired by the contraction load (the obstruction), by a decrease in the principal relaxation loads, by a pathologic degree of nonuniformity of contraction and relaxation, and in all likelihood, by impaired inactivation of the biochemical processes responsible for contraction (? due to primary or ischemia-induced calcium overload). Calcium channel-blocking agents may dramatically improve left ventricular relaxation by speeding up the inactivation process, by decreasing the degree of nonuniformity, or by altering the contraction and relaxation loads in a favorable manner. Atrial and ventricular arrhythmias are responsible for a significant proportion of the morbidity and mortality, and their occurrence also appears to depend on the extent of hypertrophy. Thus, the major manifestations of hypertrophic cardiomyopathy in systole and diastole as well as the disturbances of rhythm appear to be related to the site and/or extent of the hypertrophic process.(ABSTRACT TRUNCATED AT 400 WORDS)
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              Extended septal myectomy for hypertrophic obstructive cardiomyopathy with anomalous mitral papillary muscles or chordae.

              Transaortic left ventricular septal myectomy yields excellent results for most severely symptomatic patients with hypertrophic obstructive cardiomyopathy. However, associated anomalies of the mitral subvalvular apparatus may prevent complete relief of obstruction, and mitral valve replacement has been advocated. We reviewed our results of procedures designed to relieve obstruction with preservation of the mitral valve. Among 291 patients undergoing septal myectomy from 1975 to 2002, 56 (ages 2-77 years) had anomalous mitral subvalvular apparatus including anomalous chordae (n = 28) and papillary muscles with direct insertion into mitral leaflets (n = 13) or fusion to septum (n = 31) or free wall (n = 12); 82% of patients were in New York Heart Association class III or IV. Operation included resection of anomalous chordae (28 patients), relief of papillary muscle fusion (36 patients), and extended septal myectomy, wider at the apex than the base. There were no early deaths and no patients required mitral valve replacement. Mean peak pressure gradients decreased from 70 +/- 28 to 4.9 +/- 8.4 mm Hg and mean mitral regurgitation grade decreased from 2.3 to 1.0 (P <.001). Mean follow-up was 2.8 +/- 2.6 years. Freedom from reoperation at 4 years was 95%. There were 3 late noncardiac deaths; 98% of patients were in New York Heart Association class I or II. Hypertrophic obstructive cardiomyopathy associated with anomalous mitral papillary muscles or chordae can be successfully treated without mitral valve replacement by surgical relief of the anomalies and an extended septal myectomy; early mortality is low, obstruction and mitral regurgitation are significantly reduced, and late results are excellent.
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                Author and article information

                Contributors
                +31 10 703 3986 , a.schinkel@erasmusmc.nl
                Journal
                J Ultrasound
                J Ultrasound
                Journal of Ultrasound
                Springer International Publishing (Cham )
                1971-3495
                1876-7931
                6 January 2018
                6 January 2018
                March 2018
                : 21
                : 1
                : 17-24
                Affiliations
                [1 ]ISNI 000000040459992X, GRID grid.5645.2, Department of Cardiology, Thoraxcenter, , Erasmus Medical Center, ; Rotterdam, The Netherlands
                [2 ]ISNI 000000040459992X, GRID grid.5645.2, Department of Cardiology, , Erasmus MC, ; Thoraxcenter Room Ba304, ‘s-Gravendijkwal 230, 3015 Rotterdam, The Netherlands
                Author information
                http://orcid.org/0000-0002-6424-0754
                Article
                277
                10.1007/s40477-017-0277-y
                5845936
                29374400
                3df0cb32-1390-46d3-a022-652b5445f07e
                © The Author(s) 2018

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

                History
                : 3 August 2017
                : 21 November 2017
                Categories
                Original Article
                Custom metadata
                © Società Italiana di Ultrasonologia in Medicina e Biologia (SIUMB) 2018

                hypertrophic cardiomyopathy,3d echocardiography,papillary muscle

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