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Innovative Therapies and New Perspectives on Hypertrophic Cardiomyopathy
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Innovative Therapies and New Perspectives on Hypertrophic Cardiomyopathy

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Cardiology".

Deadline for manuscript submissions: closed (27 February 2021) | Viewed by 5633

Special Issue Editors

Dr. Yuchi Han

E-Mail Website
Guest Editor
Cardiovascular Division, Hospital of the University of Pennsylvania, Philadelphia, PA, USA
Interests: application of novel cardiac MRI; echocardiography techniques; ventricular mechanics/remodeling; cardiac MRI imaging of fibrosis
Dr. Gianluca Di Bella

E-Mail Website
Guest Editor
Clinical and Experimental Department of Medicine, University of Messina, Messina, Italy
Interests: cardiomyopathies; cardiac imaging; cardiac magnetic resonance; heart failure; rare heart diseases
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Sarcomeric hypertrophic cardiomyopathy (HCM) is the most common cardiovascular disease with genetic transmission that is characterized by left ventricular (LV) hypertrophy unexplained by secondary causes. The most common mutations involved in HCM are β-myosin heavy chain (MYH7) and myosin-binding protein C (MYBPC3). The histopathological mechanisms involved in HCM are disarray of myocytes, extracellular matrix modification, microvascular dysfunction, and myocardial fibrosis.
The typical LV morphology is characterized of severe hypertrophy, non-dilated ventricle associated with preserved or increased ejection fraction, and diastolic dysfunction.
Severe hypertrophy and nondilated ventricle may also occur in patients with so-called phenocopy conditions, which may mimic sarcomeric HCM. These include cardiac amyloidosis, Fabry disease, glycogen storage diseases, lysosomal storage diseases, mitochondrial diseases, and others.
Cardiac imaging represents the first diagnostic step in diagnosis of both sarcomeric HCM and HCM phenocopies. Additionally, cardiac imaging is mandatory to follow HCM patients.
The routine applications of genetic testing represent an important advance in the diagnosis and managements of HCM. The development of gene therapy represents a promising opportunity for many patients with HCM and photocopies.

Dr. Yuchi Han
Prof. Dr. Gianluca Di Bella
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • cardiomyopathy
  • hypertrophic cardiomyopathy
  • hypertrophic cardiomyopathy phenocopies
  • heart failure

Published Papers (2 papers)

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Research

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13 pages, 3471 KiB  
Article
The Value of Cardiac Magnetic Resonance Imaging in Identification of Rare Diseases Mimicking Hypertrophic Cardiomyopathy
by Tingting Fang, Jie Wang, Yu Kang, Fuyao Yang, Yuanwei Xu, Ke Wan, Jiayu Sun, Yuchi Han and Yucheng Chen
J. Clin. Med. 2021, 10(15), 3339; https://doi.org/10.3390/jcm10153339 - 28 Jul 2021
Cited by 3 | Viewed by 2201
Abstract
Background: The cardiac Magnetic Resonance Imaging (MRI) characteristics of rare diseases with the hypertrophic cardiomyopathy (HCM) phenotype are not well defined. Methods: Seventy-three sequential patients and 34 of their relatives, who have the HCM phenotype, were included. All subjects underwent cardiac MRI and [...] Read more.
Background: The cardiac Magnetic Resonance Imaging (MRI) characteristics of rare diseases with the hypertrophic cardiomyopathy (HCM) phenotype are not well defined. Methods: Seventy-three sequential patients and 34 of their relatives, who have the HCM phenotype, were included. All subjects underwent cardiac MRI and genetic testing. Results: Of these 107 patients with phenotypic HCM, seven rare diseases were identified: four cases with LAMP2, one case with PRKAG2, one case with TTR mutation, and one case with senile systemic amyloidosis. Subjects with rare diseases had diffuse LGE, and the percentage of those with LGE was significantly higher than that of other HCM (median: 18.9%, interquartile range (IQR): 14.05 to 28.2% versus 7.8%, IQR: 4.41 to 14.56%; p = 0.003). Additionally, global T1 and ECV were significantly higher in subjects with rare diseases (global T1: 1423.1 ± 93.3 ms versus 1296.2 ± 66.6 ms; global ECV: 44.3 ± 11.5% versus 29.9 ± 4.5%; all p < 0.001). Conclusions: Cardiac MRI suggests the existence of distinct imaging characteristics, including via LGE and T1 mapping, among rare diseases that mimic HCM and HCM itself. Full article
(This article belongs to the Special Issue Innovative Therapies and New Perspectives on Hypertrophic Cardiomyopathy)
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Review

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12 pages, 1408 KiB  
Review
Alcohol Septal Ablation: An Option on the Rise in Hypertrophic Obstructive Cardiomyopathy
by Victor Arévalos, Juan José Rodríguez-Arias, Salvatore Brugaletta, Antonio Micari, Francesco Costa, Xavier Freixa, Mónica Masotti, Manel Sabaté and Ander Regueiro
J. Clin. Med. 2021, 10(11), 2276; https://doi.org/10.3390/jcm10112276 - 24 May 2021
Cited by 14 | Viewed by 2906
Abstract
Hypertrophic cardiomyopathy (HCM) can cause symptoms due to the obstruction of the left ventricle outflow tract (LVOT). Although pharmacological therapy is the first step for treating this condition, many patients do not fully respond to the treatment, and an invasive approach is required [...] Read more.
Hypertrophic cardiomyopathy (HCM) can cause symptoms due to the obstruction of the left ventricle outflow tract (LVOT). Although pharmacological therapy is the first step for treating this condition, many patients do not fully respond to the treatment, and an invasive approach is required to manage symptoms. Septal reduction therapies include septal myectomy (SM) and alcohol septal ablation (ASA). ASA consists of a selective infusion of high-grade alcohol into a septal branch supplying the basal interventricular septum to create an iatrogenic infarction with the aim of reducing LVOT obstruction. Currently, SM and ASA have the same level of indication; however, ASA is normally reserved for patients of advanced age, with comorbidities or when the surgical approach is not feasible. Recent data suggests that there are no differences in short- and long-term all-cause mortality, cardiovascular mortality and sudden cardiac death between ASA and SM. Despite the greater experience and refinement of the technique gained in recent years, the most common complication continues to be complete atrio-ventricular block, requiring a permanent pacemaker. Septal reduction therapies should be performed in experienced centres with comprehensive programs. Full article
(This article belongs to the Special Issue Innovative Therapies and New Perspectives on Hypertrophic Cardiomyopathy)
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