scholarly journals THE PLACE OF CARDIAC COMPUTED TOMOGRAPHY IN PREOPERATIVE PLANNING OF EXTENDED SEPTAL MYECTOMY IN PATIENTS WITH OBSTRUCTIVE FORM OF HYPERTROPHIC CARDIOMYOMATHY

2020 ◽  
Vol 25 ◽  
pp. 592-606
Author(s):  
M. Tregubova ◽  
◽  
K. Rudenko ◽  
V. Lazoryshynets ◽  
S. Fedkiv ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Hypertrophic Cardiomyopathy ◽  
Cardiac Ct ◽  
Preoperative Planning ◽  
Interventricular Septum ◽  
Left Ventricular ◽  
Study Group ◽  
Technical Problems ◽  
Septal Myectomy ◽  
Preoperative Ct

Background. Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiomyopathy. Extended septal myectomy ( ESM) is one of the priority methods of treatment of drug-refractory obstructive HCM. In recent years, hospital mortality during surgical correction of obstructive HCM in expert centers does not exceed 1–2 %. However, typical threatening complications of septal myectomy, such as iatrogenic ventricular septal defect (VSD) and rupture of the anterior or posterior walls of the left ventricle (LV), remain a topical issue in surgery of HCM. Objective: to show the role of preoperative CT-planning to predict and reduce possible technical problems associated with ESM, including iatrogenic VSD. Methods and materials. This study includes 217 symptomatic patients with obstructive HCM, who from April 2016 to October 2019 as one of the steps of preoperative planning underwent cardiac CT prior to ESM. Cardiac CT was performed to delineate the left ventricular myocardium, assess the distribution of hypertrophy and the presence of crypts. Special attention was also paid to the anatomy of the mitral valve (MV) and subvalvular apparatus. Coronary artery patency was assessed by CAD-RADS, a standardized method for reporting the results of coronary CT angiography to determine tactics for further management of the patient. Results and discussion. In the study group, the average age of patients was (49 ± 15) years, 48 % – men. All patients had a symptomatic, drug-refractory obstructive form of HCM. The mean maximum wall thickness of the interventricular septum (IVS) was (20 ± 5) mm (range 16–33). The average LV mass was (118 ± 23) g/m2. 195 patients (89.9 %) had systolic anterior motion ( SAM) of the MV. MV and subvalvular apparatus anomalies were detected in 62 patients (28.6 %). A zone of scarring and regression of IVS after alcohol septal ablation (ASA) was detected in 7 patients (0.3 %) with residual LV outflow gradient. Coronary arteries atherosclerosis was detected in 32 patients (14.7 %). Conclusions. Preoperative CT-planning of septal myectomy allows to obtain information on morphology of the LV, IVS, MV and subvalvular apparatus, and gives the surgeon the advantage to form a more accurate plan for the location and volume of septal resection, and avoid complications when correcting obstructive HCM. No iatrogenic VSD was detected in any of the patient in the study group. Key words: hypertrophic cardiomyopathy, computed tomography, preoperative planning, extended septal myectomy.

scholarly journals A new approach to the assessment of outcomes of alcohol septal ablation in patients with hypertrophic obstructive cardiomyopathy

2015 ◽  
Vol 17 (2) ◽  
pp. 46
Author(s):  
A. G. Osiev ◽  
Ye. I. Kretov ◽  
V. P. Kurbatov ◽  
S. P. Mironenko ◽  
R. A. Naydenov ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Interventricular Septum ◽  
Hypertrophic Obstructive Cardiomyopathy ◽  
Left Ventricular ◽  
Alcohol Septal Ablation ◽  
New Approach ◽  
Surgical Interventions ◽  
Septal Myectomy ◽  
Left Ventricular Output ◽  
Cardiac Disorders

Hypertrophic cardiomyopathy is a heterogeneous disease characterized by myocardial hypertrophy, without any other systemic or cardiac disorders and with predominant involvement of the interventricular septum. Approximately 25% of patients have a dynamic obstruction of the left ventricular output tract due its constriction and abnormal systolic anterior motion of the mitral valve. Therapeutic strategy for patients with hypertrophic obstructive cardiomyopathy, who remain symptomatic despite drug therapy, includes surgery (septal myectomy) and non-surgical interventions, such as alcohol septal ablation. In the present study the possibility of cardiac MRI with contrast enhancement in the evaluation of the results of endovascular treatment hypertrophic cardiomyopathy and evidence for the benefits of this method in 25 patients with an obstructive form of hypertrophic cardiomyopathy after alcohol septal ablation are discussed.

scholarly journals P1321 A case of multiple levels of ventricular obstruction

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
D Oikonomidis ◽  
A Klitirinos ◽  
M Koutouzis ◽  
A Kalangos ◽  
E Lazaris ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Mitral Valve ◽  
Aortic Valve ◽  
Interventricular Septum ◽  
Left Ventricular ◽  
Imaging Modalities ◽  
Subaortic Stenosis ◽  
Shortness Of Breath ◽  
Septal Myectomy ◽  
Subaortic Membrane

Abstract Subaortic stenosis (SAS) is a rare entity in adults with unclear etiology and variable presentation. SAS may be presented with symptoms mimicking Hypertrophic Cardiomyopathy (HCM). Often a combination of imaging modalities is needed to distinguish SAS from HCM with obstruction. A 53 years old man, smoker, was referred to our medical center suffering from shortness of breath on exertion. He first presented at another facility with a 2 month history of shortness of breath and chest discomfort during brisk physical activity and the possible diagnosis of HCM was made. On physical examination, a 3/6 systolic murmur was audible along the left sternal border, that became louder with standing and the Valsalva maneuver. The patient had non distended jugular veins, clear lung fields and no ankle edema. The results of laboratory exams did not reveal any pathological sign. The transthoracic echocardiogram revealed significant left ventricular hypertrophy (Interventricular septum 21 mm, Posterior wall 16 mm) with normal left ventricular systolic performance (ejection fraction >70%). The aortic valve was tricuspid and calcified whereas mitral valve was morphologically normal, with systolic anterior motion and mild posterolaterally directed regurgitation. Two systolic gradients, one dynamic, late peaking of 85mmHg and another fixed of 70mmHg were detected in left ventricular outflow track (LVOT). Transesophageal echocardiography was performed for the better evaluation of aortic valve and showed a three level obstruction caused by the systolic motion of the mitral valve towards the hypertrophic septum at LVOT, the presence of a membranous subaortic membrane and the calcified aortic valve respectively. The aortic valve was calcified with a moderate stenosis (0.8cm2 / m2) from 3D planimetry. A Cardiac Magnetic Resonance exam was ordered and confirmed the significance of hypertrophy and the presence of circumferential subaortic membrane. No late enhancement after the administration of Gadolinium was observed. Coronary angiography was performed and demonstrated normal coronary arteries. We hypothesized that the presence of subaortic membrane led to marked myocardial wall thickness and to the destruction of the aortic valve due to turbulent flow in the LVOT. The patient was referred for surgical management Extended septal myectomy combined with complete resection of orbital subaortic membrane were performed. he calcified aortic valve was replaced by bioprosthetic valve No 23mm. The patient tolerated the procedure well with significant symptomatic improvement. TTE performed 1 month postoperatively showed no remarkable LVOT gradient. The results of histopathology and genes investigations are pending. Subaortic membrane is a rare cause of symptoms that can mimic hypertrophic cardiomyopathy. A combination of imaging modalities is needed to distinguish subaortic stenosis from aortic valve stenosis and hypertrophic obstructive cardiomyopathy. Abstract P1321 Figure. Three levels obstruction

scholarly journals Effectiveness of a patient-specific 3-dimensional printed model in Septal Myectomy of hypertrophic cardiomyopathy

2020 ◽  
Vol 36 (7) ◽  
Author(s):  
Yang Wang ◽  
Hongchang Guo ◽  
Shengwei Wang ◽  
Yongqiang Lai
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Interventricular Septum ◽  
Left Ventricular ◽  
Patient Specific ◽  
Class Iii ◽  
3 Dimensional ◽  
Septal Myectomy ◽  
Computed Tomography Images ◽  
Significant Difference ◽  
3D Printed

Objective: Advanced cardiovascular surgery in structural heart disease require accurate pre-operative evaluation. Most of non-invasive imaging technologies remain limited in two-dimensional and show insufficiency of visualization for procedural planning. The aim of this study was to discuss the value of patient-specific 3-dimensional (3D) printing in treatment of hypertrophic cardiomyopathy (HCM). Methods: Patient-specific 3D-printed models were constructed preoperatively in 12 consecutive HOCM patients which come to Beijing Anzhen Hospital for surgical treatment from October 2016 to March 2017. Image files were extracted from multi-slice computed tomography images, 3D models were constructed by the Mimics 19.0 software and generated by Objet350 Connex3 3D printer. The 3D-printed models were made with soft material that can be surgically performed. The modified Morrow myectomy of the model was performed before the operation. Clinical characters and echocardiographic parameters were recorded. Results: There was no significant difference in tissue volume between the models and specimens. Preoperative and postoperative echocardiography showed the septal thickness was reduced from 18.8±4.5 mm to 12.7±3.3 mm (p<0.001), the left ventricular outflow tract obstruction was adequately relieved (83.0±27.73 mm Hg to 8.7±6.5 mm Hg, p<0.001), and the SAM disappeared completely after the operation. Cardiac function was improved in all patients (New York Heart Association functional class III to class I/II). Conclusions: The proposed optimal 3D-modelled septal myectomy allows intraoperative monitoring of the shape and volume of the myocardium resection to achieve the ‘ideal’ interventricular septum. It eliminates obstruction in the LVOT and SAM, resulting in LV remodeling with an increase in LV end-diastolic volume and diameter at early follow-up. doi: https://doi.org/10.12669/pjms.36.7.2620 How to cite this:Wang Y, Guo H, Wang S, Lai Y. Effectiveness of a patient-specific 3-dimensional printed model in Septal Myectomy of hypertrophic cardiomyopathy. Pak J Med Sci. 2020;36(7):---------. doi: https://doi.org/10.12669/pjms.36.7.2620 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Assessment of Diastolic Function in Hypertrophic Cardiomyopathy by Computed Tomography–Derived Analysis of Left Ventricular Filling

2017 ◽  
Vol 41 (3) ◽  
pp. 339-343
Author(s):  
Itai Ghersin ◽  
Eduard Ghersin ◽  
Sobhi Abadi ◽  
Jennifer E. Runco Therrien ◽  
Tanyanan Tanawuttiwat ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Hypertrophic Cardiomyopathy ◽  
Diastolic Function ◽  
Left Ventricular ◽  
Left Ventricular Filling ◽  
Ventricular Filling

scholarly journals Aetiology-discriminative multimodality imaging of hypertrophic cardiomyopathy: deformation patterns relate to synchrotron-based assessment of microstructural tissue remodelling

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
F Loncaric ◽  
A Garcia-Alvarez ◽  
P Garcia-Canadilla ◽  
L Sanchiz ◽  
H Dejea ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Wall Thickness ◽  
Multimodality Imaging ◽  
Global Longitudinal Strain ◽  
Microstructural Analysis ◽  
Left Ventricular ◽  
Doppler Imaging ◽  
Deformation Analysis ◽  
Septal Myectomy ◽  
Regional Deformation

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – EU funding. Main funding source(s): Horizon 2020 European Commission Project H2020-MSCA-ITN-2016 (764738) and the Clinical Research in Cardiology grant from the Spanish Cardiac Society. Background The aetiology of left ventricular hypertrophy (LVH) is a relevant clinical challenge with consequences for patient management. Phenotypes resulting from hypertensive remodelling and sarcomere mutation often overlap. Synchrotron X-ray phase-contrast imaging (X-PCI) is a technique that can provide 3-dimensional detailed information on myocardial micro-structure non-destructively. The aim is to relate macrostructural/functional, non-invasive, imaging phenotypes of hypertrophic cardiomyopathy (HCM) to the underlying myocardial microstructure assessed with X-PCI. Methods Myocardial tissue samples were obtained from three patients (P1-3) with obstructive myocardial hypertrophy undergoing septal myectomy. Medical history and the 5-year HCM risk scores were evaluated. The patients were imaged with magnetic resonance imaging and echocardiography prior to procedure. Myocardial structure was assessed with wall thickness, late gadolinium enhancement (LGE), whereas function with speckle-tracking deformation (STE) and tissue Doppler imaging (TDI). Myectomy tissue was imaged with X-PCI in the TOMCAT beamline, using a multiscale propagation-based protocol combining a low-resolution (LR) and a high-resolution (HR) setup (5.8 and 0.7 um pixel size, respectively). Results The clinical and imaging data are shown in Fig 1. On initial assessment, wall thickness, LGE distribution, global longitudinal strain and septal TDI demonstrated a similar macrostructural and functional phenotype of P1 and P2, whereas P3 stood out with more severe hypertrophy, scarring and dysfunction. Additional regional deformation analysis with STE revealed reduced deformation in the basal and mid septum in P1, paired with a hypertensive pattern of post-systolic shortening (PSS) (yellow arrows). In comparison, in P2 and P3, deformation was more heterogeneous regionally, with regions of almost complete absence of deformation (orange arrows). Upon further exploration with TDI, areas with abnormal deformation were identified on the transition from basal to mid septum in both P2 and P3, whereas deformation was normal, but reduced in P1, and paired with PSS. LR X-PCI defined regions of interest to scan with HR (yellow frame), where HR revealed extensive interstitial fibrosis (orange arrow) with normal myocyte size and organisation in P1, compatible with severe hypertensive remodelling. However, in P2 and P3, patches of fibrosis (yellow arrow) paired with enlarged myocytes organized in visible disarray, considerably more prominent in P3, were both compatible with sarcomere-mutation HCM. Conclusion The results demonstrate multiscale phenotyping of HCM - relating micro- and macrostructural findings to function, and integrating multimodality data. In-depth regional deformation analysis, validated by synchrotron-based microstructural analysis, showed potential to identify distinct imaging phenotypes in HCM, distinguishing between overlapping presentations in different aetiologies. Abstract Figure 1

scholarly journals Clinical Features and Echocardiographic Findings in Children with Hypertrophic Cardiomyopathy

2013 ◽  
Vol 59 (6) ◽  
pp. 285-288
Author(s):  
Blesneac Cristina ◽  
Benedek Theodora ◽  
Togănel Rodica ◽  
Benedek I
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Age Of Onset ◽  
Adult Population ◽  
Systemic Disease ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Septal Myectomy ◽  
Sarcomeric Protein ◽  
Asymptomatic Patients ◽  
Left Ventricular Outflow

Abstract Background: Hypertrophic cardiomyopathy, one of the most common inherited cardiomyopathies, is a heterogeneous disease resulting from sarcomeric protein mutations, with an incidence in the adult population of 1:500. Current information on the epidemiology and outcomes of this disease in children is limited. Methods: Thirty-four children diagnosed with hypertrophic cardiomyopathy in the Pediatric Cardiology Department from Tîrgu Mureș were evaluated concerning familial and personal history, clinical, paraclinical and therapeutic aspects. Hypertrophic cardiomyopathy was defined by the presence of a hypertrophied, non-dilated ventricle, in the absence of a cardiac or systemic disease that could produce ventricular hypertrophy. Results: The youngest diagnosed child was a neonate, a total of 10 patients being diagnosed until 1 year of age. In 6 cases a positive familial history was found. Noonan syndrome was found in 2 cases. Only 21 patients were symptomatic, the predominant symptoms being shortness of breath on exertion with exercise limitations. Left ventricular outflow tract obstruction was present in 21 cases (61.7%). Twenty-four patients were on β-blocking therapy, while 4 patients underwent septal myectomy. Conclusions: Hypertrophic cardiomyopathy is a heterogeneous disorder in terms of evolution, age of onset, type and extent of hypertrophy, and the risk of sudden death. It can affect children of any age. There is a need for a complex evaluation, including familial and personal anamnesis, clinical examination, electrocardiogram and echocardiography of all patients. It is highly important to develop screening strategies, including genetic testing, for an early diagnosis, especially in asymptomatic patients with a positive familial background

scholarly journals Alcohol septal ablation in hypertrophic cardiomyopathy

2018 ◽  
Vol 2018 (3) ◽  
Author(s):  
Juan José Santos Mateo ◽  
Juan R Gimeno
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Interventricular Septum ◽  
Ventricular Outflow Tract ◽  
Clinical Results ◽  
Left Ventricular ◽  
Alcohol Septal Ablation ◽  
Obstructive Hypertrophic Cardiomyopathy ◽  
Left Ventricular Outflow ◽  
Surgical Myectomy

Alcohol septal ablation (ASA) has become an alternative to surgical myectomy in obstructive hypertrophic cardiomyopathy since it was first introduced in 1994 by Sigwart. The procedure alleviates symptoms by producing a limited infarction of the upper interventricular septum, resulting in a decrease in left ventricular outflow tract (LVOT) gradient. The technique has been improved over time and the results are comparable with those of myectomy. Initial concerns about long-term outcomes have been largely resolved. In this review, we discuss indications, technical aspects, clinical results and patient selection to ASA.

3255Characterization of the arrhythmogenic substrate with multimodality imaging in ischemic patients undergoing VT ablation: relationship between cardiac computed tomography and magnetic resonance

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
G Zucchelli ◽  
D Soto Iglesias ◽  
B Jauregui ◽  
C Teres ◽  
D Penela ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Magnetic Resonance ◽  
Cardiac Ct ◽  
Low Voltage ◽  
Multimodality Imaging ◽  
Cardiac Computed Tomography ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Arrhythmogenic Substrate

Abstract Background Cardiac magnetic resonance (CMR)-aided ventricular tachycardia (VT) substrate ablation has shown to improve VT recurrence-free survival, through a better identification of the arrhythmogenic substrate. However, the access to CMR may be limited in certain centers or sometimes Its use can be contraindicated in patients with cardiac implantable electronic device. Cardiac computed tomography (CT) has shown to improve the results of substrate ablation, correlating with low-voltage areas and local abnormal ventricular activity, and identifying ridges of myocardial tissue (CT-channels) that may be appropriate target sites for ablation. Purpose To evaluate the correlation between CT and CMR imaging in identifying anatomical heterogeneous tissue channels (CMR-channels) or CT-channels in ischemic patients undergoing VT substrate ablation. Methods The study included 30 post-myocardial infarction (MI) patients (mean age 69±10; 94% male, left ventricular ejection fraction 35±10%), who underwent both CMR and cardiac CT before VT substrate ablation. Using a dedicated post-processing software, the myocardium was segmented in 10 layers from endocardium to epicardium both for the CMR and CT, characterizing the presence of CMR-channels and CT-channels, respectively, by two blinded operators, assigned either to CMR or CT analysis. CMR-channels were classified as endocardial (CMR-channels in layer <50%), epicardial (CMR-channels in layers ≥50%) or transmural (in both endo and epicardial layers). Presence and location of CT and CMR-channels were compared. Results In 26/30 patients (86.7%) 91 CT-channels (mean 3.0±1.9 per patient) were identified while 30/30 (100%) showed CMR-channels (n=76; mean 2.4±1.2 per patient). We found 190 CT-channel entrances (mean 6.3±4.1 per patient), and 275 CMR-channel entrances (mean 8.9±4.9 per patient) on cardiac CT and CMR, respectively. There were 47/91 (51.6%) true positive CT-channels. On the contrary, 44/91 (48.4%) CT-channels were considered false positives [19/91 (20.9%) identified out of CMR scar], and 29/76 (38.2%) CMR-channels could not be identified on CT. Thirty-six out of 76 (47.4%) CMR-channels were considered as non-endocardial (epi- or transmural). Twenty-nine out of 36 (80.5%) non-endocardial CMR-channels were coincident with CT-channels. CT and CMR Channels Conclusion CT shows a modest sensitivity in identifying CMR-channels and fails in ascertain their complexity, underestimating the number of entrances; however, channels location at CT fit well with CMR for those classified as transmural or epicardial.

scholarly journals P881 Young patient with left ventricular hypertrophy and accidentally discovered aortic dissection: hypertensive heart or hypertrophic cardiomyopathy?

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
M Gawor ◽  
M Franaszczyk ◽  
E Kowalik ◽  
M Spiewak ◽  
I Michalowska ◽  
...  
Keyword(s):  
Chest Pain ◽  
Hypertrophic Cardiomyopathy ◽  
Aortic Dissection ◽  
Troponin T ◽  
Global Longitudinal Strain ◽  
Interventricular Septum ◽  
Positive Family History ◽  
Left Ventricular ◽  
Jugular Veins ◽  
Rare Differential Diagnosis

Abstract A 36-year-old male with positive family history of sudden cardiac death (his uncle"s son died suddenly at the age of 25), hospitalized a month ago in a local hospital due to acute hypertensive cardiogenic pulmonary edema, was referred to our institution for further evaluation with suspicion of hypertrophic cardiomyopathy. On admission patient was asymptomatic, without fatigue, exertional dyspnoea, chest pain or syncope. On physical examination his BP was significantly elevated (180/100 mmHg). The lungs were clear on auscultation, liver was not enlarged, jugular veins were normal, there was no oedema of lower extremities. Abdominal auscultation revealed vascular murmur in umbilical region. The baseline level of NT-proBNP was 811.4 (range 0–125) pg/mL, and high-sensitivity cardiac troponin T was 20.2 (range 0–14) ng/L. The standard 12-lead electrocardiogram demonstrated sinus rhythm, left atrial enlargement and left ventricular (LV) hypertrophy with nonspecific ST segment and T-wave changes (Fig. 1A). No significant pathology was present on chest X-ray (Fig. 1B). Transthoracic echocardiography revealed significant concentric LV hypertrophy with preserved LV ejection fraction (EF 70%) and moderately decreased global longitudinal strain (GLS-13.7%). There was mild dilatation of left atrium. Ascending aorta diameter was in normal range (Fig. 1C-D). Cardiac magnetic resonance (CMR) scan confirmed concentric LV hypertrophy with the maximal wall thickness of 18 mm at interventricular septum, and increased myocardial mass (LV mass index 124 ml/m2, range 59–92). Moreover, small areas of late gadolinium enhancement were found in LV segments (Fig. 1E-F). Due to presence of vascular murmur in abdomen, ultrasound imaging was performed. The exam revealed abdominal aortic dissection (Fig. 1G-H). Patient was transferred to the computed tomography (CT) unit to confirm the diagnosis. Aortic dissection originated below renal arteries and involving common illiac arteries was detected (Stanford B). The presence of thrombi within the lumen created by the aortic dissection suggested chronic presentation. Patient was managed conservatively with strict blood pressure control and close follow up arranged. We decided to perform genetic analysis. Currently we are awaiting the results in hope that it will help us to establish the diagnosis and differentiate hypertensive heart from hypertrophic cardiomyopathy. In conclusion, aortic dissection typically presents with tearing chest pain and severe hemodynamic compromise. Painless dissection, like in this case, is relatively rare. Differential diagnosis between hypertensive heart and hypertrophic cardiomyopathy is crucial as it has direct therapeutic impact. Abstract P881 Figure 1

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