Trabeculated Myocardium in Hypertrophic Cardiomyopathy: Clinical Consequences

Casanova, José D.; González-Carrillo, Josefa; Jiménez, Junior Vega; Muñoz, Javier Cuenca; Esparza, Carmen Muñoz; Alvárez, Marcos Siguero; Escribá, Rubén; Milla, Esther Burillo; Pompa, José Luis de la; Gimeno, Juan R.; Sabater-Molina, María; García, Gregorio Bernabé

doi:10.3390/jcm9103171

Cited by 6 publications

(3 citation statements)

References 28 publications

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“…In a study evaluating 211 HCM patients, an inverse correlation between wall thickness and trabeculation mass, as well as between EF and trabecular mass, was demonstrated [ 30 ].…”

Section: Discussionmentioning

confidence: 99%

Unusual Evolution of Hypertrophic Cardiomyopathy in Non-Compaction Myocardium in a Pompe Disease Patient

Gragnaniello¹,

Rizzardi

Commone³

et al. 2023

JCM

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Classic infantile Pompe disease is characterized by a severe phenotype with cardiomyopathy and hypotonia. Cardiomyopathy is generally hypertrophic and rapidly regresses after enzyme replacement therapy. In this report, for the first time, we describe a patient with infantile Pompe disease and hypertrophic cardiomyopathy that evolved into non-compaction myocardium after treatment. The male newborn had suffered since birth with hypertrophic cardiomyopathy and heart failure. He was treated with standard enzyme replacement therapy (ERT) (alglucosidase alfa) and several immunomodulation cycles due to the development of anti-ERT antibodies, without resolution of the hypertrophic cardiomyopathy. At the age of 2.5 years, he was treated with a new combination of ERT therapy (cipaglucosidase alfa) and a chaperone (miglustat) for compassionate use. After 1 year, the cardiac hypertrophy was resolved, but it evolved into non-compaction myocardium. Non-compaction cardiomyopathy is often considered to be a congenital, primitive cardiomyopathy, due to an arrest of compaction of the myocardium wall during the embryonal development. Several genetic causes have been identified. We first describe cardiac remodeling from hypertrophic cardiomyopathy to a non-compaction form in a patient with infantile Pompe disease treated with a new ERT. This has important implications both for the monitoring of Pompe disease patients and for the understanding of the pathophysiological basis of non-compaction myocardium.

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“…In a study evaluating 211 HCM patients, an inverse correlation between wall thickness and trabeculation mass, as well as between EF and trabecular mass, was demonstrated [ 30 ].…”

Section: Discussionmentioning

confidence: 99%

Unusual Evolution of Hypertrophic Cardiomyopathy in Non-Compaction Myocardium in a Pompe Disease Patient

Gragnaniello¹,

Rizzardi

Commone³

et al. 2023

JCM

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“…Abnormalities in myocardial trabeculation, including hypertrabeculation [33] and multiple myocardial crypts [34], are well-described in hypertrophic cardiomyopathy, although their significance remains unclear. Wang et al investigated the prognostic significance of myocardial trabecular complexity using fractal analysis in 378 individuals with HCM and 100 age-and gender-matched healthy controls [35].…”

Section: Other Cmr Parametersmentioning

confidence: 99%

Novel Magnetic Resonance Imaging Tools for Hypertrophic Cardiomyopathy Risk Stratification

Alajmi,

Kang,

Dundas

et al. 2024

Life

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Hypertrophic cardiomyopathy (HCM) is a common genetic disorder with a well described risk of sudden cardiac death; however, risk stratification has remained a challenge. Recently, novel parameters in cardiac magnetic resonance imaging (CMR) have shown promise in helping to improve upon current risk stratification paradigms. In this manuscript, we have reviewed novel CMR risk markers and their utility in HCM. The results of the review showed that T1, extracellular volume, CMR feature tracking, and other miscellaneous novel CMR variables have the potential to improve sudden death risk stratification and may have additional roles in diagnosis and prognosis. The strengths and weaknesses of these imaging techniques, and their potential utility and implementation in HCM risk stratification are discussed.

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“…To do this, we choose a threshold of VT% over which you are considered to have LVNC. In this light, QLVTHC is a fully automatic tool that uses a threshold of 27.4% for diagnosis [8][9][10].…”

Section: Introductionmentioning

confidence: 99%

Improving a Deep Learning Model to Accurately Diagnose LVNC

Barón,

Bernabé,

González-Férez

et al. 2023

JCM

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Accurate diagnosis of Left Ventricular Noncompaction Cardiomyopathy (LVNC) is critical for proper patient treatment but remains challenging. This work improves LVNC detection by improving left ventricle segmentation in cardiac MR images. Trabeculated left ventricle indicates LVNC, but automatic segmentation is difficult. We present techniques to improve segmentation and evaluate their impact on LVNC diagnosis. Three main methods are introduced: (1) using full 800 × 800 MR images rather than 512 × 512; (2) a clustering algorithm to eliminate neural network hallucinations; (3) advanced network architectures including Attention U-Net, MSA-UNet, and U-Net++.Experiments utilize cardiac MR datasets from three different hospitals. U-Net++ achieves the best segmentation performance using 800 × 800 images, and it improves the mean segmentation Dice score by 0.02 over the baseline U-Net, the clustering algorithm improves the mean Dice score by 0.06 on the images it affected, and the U-Net++ provides an additional 0.02 mean Dice score over the baseline U-Net. For LVNC diagnosis, U-Net++ achieves 0.896 accuracy, 0.907 precision, and 0.912 F1-score outperforming the baseline U-Net. Proposed techniques enhance LVNC detection, but differences between hospitals reveal problems in improving generalization. This work provides validated methods for precise LVNC diagnosis.

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Trabeculated Myocardium in Hypertrophic Cardiomyopathy: Clinical Consequences

Cited by 6 publications

References 28 publications

Unusual Evolution of Hypertrophic Cardiomyopathy in Non-Compaction Myocardium in a Pompe Disease Patient

Unusual Evolution of Hypertrophic Cardiomyopathy in Non-Compaction Myocardium in a Pompe Disease Patient

Novel Magnetic Resonance Imaging Tools for Hypertrophic Cardiomyopathy Risk Stratification

Improving a Deep Learning Model to Accurately Diagnose LVNC

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