Pipeline for Multi-Scale Three-Dimensional Anatomic Study of the Human Heart.

Detailed study of non-failing human hearts rejected for transplantation provides a unique opportunity to perform structural analyses across microscopic and macroscopic scales. These techniques include tissue clearing (modified immunolabeling-enabled three-dimensional (3D) imaging of solvent-cleared organs) and immunohistochemical staining. Mesoscopic examination procedures include stereoscopic dissection and micro-computed tomographic (CT) scanning. Macroscopic examination procedures include gross dissection, photography (including anaglyphs and photogrammetry), CT, and 3D printing of the physically or virtually dissected or whole heart. Before macroscopic examination, pressure-perfusion fixation may be performed to maintain the 3D architecture and physiologically relevant morphology of the heart. The application of these techniques in combination to study the human heart is unique and crucial in understanding the relationship between distinct anatomic features such as coronary vasculature and myocardial innervation in the context of the 3D architecture of the heart. This protocol describes the methodologies in detail and includes representative results to illustrate progress in the research of human cardiac anatomy.

Right parasternal echocardiographic reference values in the healthy male Iranian Shall sheep.

BACKGROUND: Among the large animals, the heart of sheep is functionally and structurally very similar to the human heart. In research, sheep are used as an animal model to study the process of cardiac disease pathogenesis and treatment. Therefore, determining the normal values of the heart structures of sheep with echocardiography is of particular emphasis. The purpose of the present research is to define the normal echocardiography values of heart in Iranian Shall breed sheep. MATERIALS AND METHODS: In 20 healthy Iranian Shall male sheep weighing 30-35 kg and aged 4-6 months, standing echocardiography was done from the right parasternal approach concentrated on the 3rd to 5th intercostal spaces by 2.5-5 MHz phased array transducer in the longitudinal and transverse views by B-mode, M-mode and Doppler systems. In M-mode echocardiographic, the parameters of interventricular septal, left ventricular internal diameter, left ventricular free wall, right ventricular free wall and right ventricular internal diameter in diastole and systole as well as end point septal separation, ejection fraction (EF) slope, aortic root diameter, left atrial appendage, left atrial diameter/aortic valve diameter, left ventricular ejection time, fractional shortening, end-diastolic volume, end-systolic volume, EF, stroke volume and cardiac output and in pulsed-wave spectral Doppler echocardiographic, the parameters of mitral valve maximum velocity (Vmax), mitral valve mean velocity (Vmean), mitral valve maximum pressure gradient (PGmax), mitral valve mean pressure gradient (PGmean), mitral valve velocity time integral (VTI), mitral valve E-wave (MV-E), MV-E pressure gradient, mitral valve A-wave (MV-A), MV-A pressure gradient, aortic Vmax, aortic valve Vmean, aortic valve PGmax, aortic valve PGmean, aortic valve VTI, left ventricular outflow tract (LVOT)-Vmax, LVOT-Vmean were measured. RESULTS: All the sheep in this study were healthy and had no signs of heart disease. In this study, the parameters of M-mode and spectral Doppler echocardiographic were assessed and recognized in Iranian Shall sheep. The results demonstrated the parameters of echocardiographic could be dependably determined in Shall sheep which, established normal reference values for these parameters and left ventricular function indices in healthy Shall sheep. These results can be beneficial in appropriate imagination, recognition and measuring cardiac structures. CONCLUSION: This study can be exerted as a reference for the assessment and diagnosis of heart diseases in sheep medicine and human cardiovascular research in sheep experimental models.

Normal Cardiac Anatomy and Clinical Evaluation.

The heart is positioned in the middle, superior, and posterior regions of the mediastinum. Although it is a midline structure, the apex of the heart is typically situated to the left of the midline (Fig. 4.1).

Anatomy of blood microcirculation in the pig epicardial ganglionated nerve plexus.

Embolization of coronary arteries and their terminal arterioles causes ischemia of all tissues distributed within a cardiac wall including the intrinsic cardiac ganglionated nerve plexus (ICGP). The disturbed blood supply to the ICGP causes chronic sympathetic activation with succeeding atrial and ventricular arrhythmias. This study analyses the anatomy of microcirculation of epicardial nerves and ganglia using the hearts of 11 domestic pigs. Our findings demonstrate that thicker epicardial nerves are normally supplied with blood via 12 epineural arterioles penetrating the endoneurium regularly along a nerve, and forming an endoneurial capillary network, which drains the blood into the myocardial blood flow. The mean diameter of intraneural capillaries was 7.2 ± 0.2 µm, while the diameters of arterioles were 25.8 ± 0.7 µm and involved 45 endothelial cells accompanied by circular smooth muscle cells. Usually, two or three arterioles with a mean diameter of 28.9 ± 1.7 µm supplied blood to any epicardial ganglion, in which arterioles proceeded into a network of capillaries with a mean diameter of 6.9 ± 0.3 µm. Both the epicardial nerves and the ganglia distributed near the porta venarum of the heart had tiny arterioles that anastomosed blood vessels from the right and the left coronary arteries. The density of blood vessels in the epicardial nerves was significantly lesser compared with the ganglia. Our electron microscopic observations provided evidence that blood vessels of the pig epicardial nerves and ganglia may be considered as either arterioles or capillaries that have quantitative and qualitative differences comparing to the corresponding blood vessels in humans and, therefore, a pig should not be considered as an animal model of the first choice for further heart functional studies seeking to improve the treatment of cardiac arrhythmias via trans-coronary cardiac neuroablation. STRUCTURED ABSTRACT: This study details the anatomy of microcirculation of epicardial nerves and ganglia, from which intracardiac nerves and bundles of nerve fibers extend into all layers of the atrial and ventricular walls in the most popular animal model of experimental cardiology and cardiac surgery - the domestic pig. Our findings provided evidence that blood vessels of the pig epicardial nerves and ganglia may be considered as either arterioles or capillaries that have quantitative and qualitative differences comparing to the corresponding blood vessels in humans and, therefore, a pig should not be considered as an animal model of the first choice for further heart functional studies seeking to improve the treatment of cardiac arrhythmias via trans-coronary cardiac neuroablation.

Normal cardiac dimensions by magnetic resonance imaging and topographic anatomy of the adult arabian one-humped camel (Camelus dromedarius).

BACKGROUND: Dromedaries' normal heart architecture and size have not been adequately examined utilizing magnetic resonance imaging (MRI) and topographic anatomy. RESULT: we aimed to investigate the regular appearance of the heart and its dimensions, using MRI and cross-sectional anatomy, in mature Arabian one-humped camels (Camelus dromedarius). We also analyzed hematological and cardiac biochemical markers. MRI scans were conducted on twelve camel heart cadavers using a closed 1.5-Tesla magnet with fast spin echo (FSE) weighted sequences. Subsequently, the hearts were cross-sectionally sliced. Additionally, hematobiochemical studies were conducted on ten mature live camels. The study analyzed standard cardiac dimensions including HL, BW, RA, LA, RV, LV, IVS, LAD, RAD, RVD, AoD, TCVD, and MVD. The results showed a strong positive correlation between the cardiac dimensions obtained from both gross analysis and MR images, with no significant difference between them. On both gross and MRI images, the usual structures of the heart were identified and labeled. Along with the cardiac markers (creatine kinase and troponin), the average hematological values and standard biochemical parameters were also described. CONCLUSION: According to what we know, this investigation demonstrates, for the first time the typical heart structures and dimensions of the heart in dromedaries, and it could serve as a basis for diagnosing cardiac disorders in these animals.

Exploring heart dissection techniques for enhancing anatomical education: a pilot study to replicate transthoracic echocardiography.

PURPOSE: For novice learners, converting two-dimensional (2D) images of echocardiography to three-dimensional (3D) cardiac structures is deemed challenging. This study aimed to develop an accurate dissection method of the heart to reproduce the transthoracic echocardiographic views on cadavers and elucidate new educational methods in human anatomy dissection courses. METHODS: A total of 18 hearts were used in this study. After reflecting the anterior thoracic wall inferiorly, the hearts were excised from embalmed cadavers. Thereafter, three landmarks were set on the heart for each plane of the incision, and the hearts were incised to observe the three different echocardiographic views, which include the apical four-chamber view (A4C), parasternal long axis (PLAX) view, and parasternal short axis (PSAX) view at the papillary muscle level. If all structures for observation during routine echocardiography are clearly observed in each view, a successful incision is considered. All procedures and incisions were performed by the medical students. After a successful incision, hearts were returned to the original position in the pericardial sac for further observation. RESULTS: The success rates of incision for each view were 83.3% (5/6 success cases), 83.3% (5/6 success cases), and 66.7% (4/6 success cases) in the A4C view, PLAX view, and PSAX view at the papillary muscle level, respectively. CONCLUSION: This dissection method could probably be employed to reproduce transthoracic echocardiographic views on cadaveric hearts, which is beneficial for novice learners for a deeper understanding of the anatomy.

Transthoracic echocardiography is a simple tool for size matching in cardiac xenotransplantation.

BACKGROUND: Preoperative size matching is essential for both allogeneic and xenogeneic heart transplantation. In preclinical pig-to-baboon xenotransplantation experiments, porcine donor organs are usually matched to recipients by using indirect parameters, such as age and total body weight. For clinical use of xenotransplantation, a more precise method of size measurement would be desirable to guarantee a "perfect match." Here, we investigated the use of transthoracic echocardiography (TTE) and described a new method to estimate organ size prior to xenotransplantation. METHODS: Hearts from n = 17 genetically modified piglets were analyzed by TTE and total heart weight (THW) was measured prior to xenotransplantation into baboons between March 2018 and April 2022. Left ventricular (LV) mass was calculated according to the previously published method by Devereux et al. and a newly adapted formula. Hearts from n = 5 sibling piglets served as controls for the determination of relative LV and right ventricular (RV) mass. After explantation, THW and LV and RV mass were measured. RESULTS: THW correlated significantly with donor age and total body weight. The strongest correlation was found between THW and LV mass calculated by TTE. Compared to necropsy data of the control piglets, the Devereux formula underestimated both absolute and relative LV mass, whereas the adapted formula yielded better results. Combining the adapted formula and the relative LV mass data, THW can be predicted with TTE. CONCLUSIONS: We demonstrate reliable LV mass estimation by TTE for size matching prior to xenotransplantation. An adapted formula provides more accurate results of LV mass estimation than the generally used Devereux formula in the xenotransplantation setting. TTE measurement of LV mass is superior for the prediction of porcine heart sizes compared to conventional parameters such as age and total body weight.

Intrinsically explainable deep learning architecture for semantic segmentation of histological structures in heart tissue.

BACKGROUND: Analysis of structures contained in tissue samples and the relevant contextual information is of utmost importance to histopathologists during diagnosis. Cardiac biopsies require in-depth analysis of the relationships between biological structures. Statistical measures are insufficient for determining a model's viability and applicability in the diagnostic process. A deeper understanding of predictions is necessary in order to support histopathologists. METHODS: We propose a method for providing supporting information in the form of segmentation of histological structures to histopathologists based on these principles. The proposed method utilizes nuclei type and density information in addition to standard image input provided at two different zoom levels for the semantic segmentation of blood vessels, inflammation, and endocardium in heart tissue. RESULTS: The proposed method was able to reach state-of-the-art segmentation results. The overall quality and viability of the predictions was qualitatively evaluated by two pathologists and a histotechnologist. CONCLUSIONS: The decision process of the proposed deep learning model utilizes the provided information sources correctly and simulates the decision process of histopathologists via the usage of a custom-designed attention gate that provides a combination of spatial and encoder attention mechanisms. The implementation is available at https://github.com/mathali/IEDL-segmentation-of-heart-tissue.

Can thoraco-abdominal organ boundaries be accurately determined from X-ray and anthropometric surface scans? Implications for body armour system coverage and design.

To optimise soldier protection within body armour systems, knowledge of the boundaries of essential thoraco-abdominal organs is necessary to inform coverage requirements. However, existing methods of organ boundary identification are costly and time consuming, limiting widespread adoption for use on soldier populations. The aim of this study was to evaluate a novel method of using 3D organ models to identify essential organ boundaries from low dose planar X-rays and 3D external surface scans of the human torso. The results revealed that, while possible to reconstruct 3D organs using template 3D organ models placed over X-ray images, the boundary data (relating to the size and position of each organ) obtained from the reconstructed organs differed significantly from MRI organ data. The magnitude of difference varied between organs. The most accurate anatomical boundaries were the left, right, and inferior boundaries of the heart, and lateral boundaries for the liver and spleen. Visual inspection of the data demonstrated that 11 of 18 organ models were successfully integrated within the 3D space of the participant's surface scan. These results suggest that, if this method is further refined and evaluated, it has potential to be used as a tool for estimating body armour coverage requirements.

Characterization, number, and spatial organization of nerve fibers in the human cervical vagus nerve and its superior cardiac branch.

BACKGROUND: Electrical stimulation of the vagus nerve (VN) is a therapy for epilepsy, obesity, depression, and heart diseases. However, whole nerve stimulation leads to side effects. We examined the neuroanatomy of the mid-cervical segment of the human VN and its superior cardiac branch to gain insight into the side effects of VN stimulation and aid in developing targeted stimulation strategies. METHODS: Nerve specimens were harvested from eight human body donors, then subjected to immunofluorescence and semiautomated quantification to determine the signature, quantity, and spatial distribution of different axonal categories. RESULTS: The right and left cervical VN (cVN) contained a total of 25,489 ± 2781 and 23,286 ± 3164 fibers, respectively. Two-thirds of the fibers were unmyelinated and one-third were myelinated. About three-quarters of the fibers in the right and left cVN were sensory (73.9 ± 7.5 % versus 72.4 ± 5.6 %), while 13.2 ± 1.8 % versus 13.3 ± 3.0 % were special visceromotor and parasympathetic, and 13 ± 5.9 % versus 14.3 ± 4.0 % were sympathetic. Special visceromotor and parasympathetic fibers formed clusters. The superior cardiac branches comprised parasympathetic, vagal sensory, and sympathetic fibers with the left cardiac branch containing more sympathetic fibers than the right (62.7 ± 5.4 % versus 19.8 ± 13.3 %), and 50 % of the left branch contained sensory and sympathetic fibers only. CONCLUSION: The study indicates that selective stimulation of vagal sensory and motor fibers is possible. However, it also highlights the potential risk of activating sympathetic fibers in the superior cardiac branch, especially on the left side.

Genetic diversity, growth and heart function of Auckland Island pigs, a potential source for organ xenotransplantation.

One of the prerequisites for successful organ xenotransplantation is a reasonable size match between the porcine organ and the recipient's organ to be replaced. Therefore, the selection of a suitable genetic background of source pigs is important. In this study, we investigated body and organ growth, cardiac function, and genetic diversity of a colony of Auckland Island pigs established at the Center for Innovative Medical Models (CiMM), LMU Munich. Male and female Auckland Island pig kidney cells (selected to be free of porcine endogenous retrovirus C) were imported from New Zealand, and founder animals were established by somatic cell nuclear transfer (SCNT). Morphologically, Auckland Island pigs have smaller body stature compared to many domestic pig breeds, rendering their organ dimensions well-suited for human transplantation. Furthermore, echocardiography assessments of Auckland Island pig hearts indicated normal structure and functioning across various age groups throughout the study. Single nucleotide polymorphism (SNP) analysis revealed higher runs of homozygosity (ROH) in Auckland Island pigs compared to other domestic pig breeds and demonstrated that the entire locus coding the swine leukocyte antigens (SLAs) was homozygous. Based on these findings, Auckland Island pigs represent a promising genetic background for organ xenotransplantation.

The athlete's heart: allometric considerations on published papers and relation to cardiovascular variables.

To evaluate the morphology of the "athlete's heart", left ventricular (LV) wall thickness (WT) and end-diastolic internal diameter (LVIDd) at rest were addressed in publications on skiers, rowers, swimmers, cyclists, runners, weightlifters (n = 927), and untrained controls (n = 173) and related to the acute and maximal cardiovascular response to their respective disciplines. Dimensions of the heart at rest and functional variables established during the various sport disciplines were scaled to body weight for comparison among athletes independent of body mass. The two measures of LV were related (r = 0.8; P = 0.04) across athletic disciplines. With allometric scaling to body weight, LVIDd was similar between weightlifters and controls but 7%-15% larger in the other athletic groups, while WT was 9%-24% enlarged in all athletes. The LVIDd was related to stroke volume, oxygen pulse, maximal oxygen uptake, cardiac output, and blood volume (r = ~ 0.9, P < 0.05), while there was no relationship between WT and these variables (P > 0.05). In conclusion, while cardiac enlargement is, in part, essential for the generation of the cardiac output and thus stroke volume needed for competitive endurance exercise, an enlarged WT seems important for the development of the wall tension required for establishing normal arterial pressure in the enlarged LVIDd.

Spatially organized cellular communities form the developing human heart.

The heart, which is the first organ to develop, is highly dependent on its form to function1,2. However, how diverse cardiac cell types spatially coordinate to create the complex morphological structures that are crucial for heart function remains unclear. Here we integrated single-cell RNA-sequencing with high-resolution multiplexed error-robust fluorescence in situ hybridization to resolve the identity of the cardiac cell types that develop the human heart. This approach also provided a spatial mapping of individual cells that enables illumination of their organization into cellular communities that form distinct cardiac structures. We discovered that many of these cardiac cell types further specified into subpopulations exclusive to specific communities, which support their specialization according to the cellular ecosystem and anatomical region. In particular, ventricular cardiomyocyte subpopulations displayed an unexpected complex laminar organization across the ventricular wall and formed, with other cell subpopulations, several cellular communities. Interrogating cell-cell interactions within these communities using in vivo conditional genetic mouse models and in vitro human pluripotent stem cell systems revealed multicellular signalling pathways that orchestrate the spatial organization of cardiac cell subpopulations during ventricular wall morphogenesis. These detailed findings into the cellular social interactions and specialization of cardiac cell types constructing and remodelling the human heart offer new insights into structural heart diseases and the engineering of complex multicellular tissues for human heart repair.

Vertebral heart size and vertebral left atrial size reference ranges in healthy Miniature Schnauzers.

OBJECTIVE: The inclusion of vertebral heart score (VHS) and, more recently, the inclusion of the vertebral left atrial size (VLAS) in radiographic evaluation have become important screening tools for identifying dogs with occult cardiac disease. Several recent papers have shown there are interbreed variations in the VHS reference range. Our hypothesis is that the Miniature Schnauzer would also have a higher reference range for its VHS. ANIMALS: The electronic medical records of IDEXX Telemedicine Consultants were searched for Miniature Schnauzers undergoing thoracic radiographs between March 1, 2022, and February 28, 2023. METHODS: Dogs were included if they had 3 view thoracic radiographs performed and no evidence of cardiopulmonary disease was detected. Dogs with incomplete radiographic studies or cardiac or extracardiac disease were excluded. The VHS and VLAS measurements were performed by 2 board-certified cardiologists independent of one another. RESULTS: A total of 1,000 radiographs were obtained of which 272 were included for the study. The overall range for the VHS in this cohort was 9.68 to 12.07 with a median of 10.9. For VLAS measurements, a range of 1.71 to 2.4 was documented with a median of 2.0. CLINICAL RELEVANCE: The VHS for Miniature Schnauzers without cardiac disease was confirmed to be higher than the canine reference range.

Structure-aware independently trained multi-scale registration network for cardiac images.

Image registration is a primary task in various medical image analysis applications. However, cardiac image registration is difficult due to the large non-rigid deformation of the heart and the complex anatomical structure. This paper proposes a structure-aware independently trained multi-scale registration network (SIMReg) to address this challenge. Using image pairs of different resolutions, independently train each registration network to extract image features of large deformation image pairs at different resolutions. In the testing stage, the large deformation registration is decomposed into a multi-scale registration process, and the deformation fields of different resolutions are fused by a step-by-step deformation method, thus solving the difficulty of directly processing large deformation. Meanwhile, the targeted introduction of MIND (modality independent neighborhood descriptor) structural features to guide network training enhances the registration of cardiac structural contours and improves the registration effect of local details. Experiments were carried out on the open cardiac dataset ACDC (automated cardiac diagnosis challenge), and the average Dice value of the experimental results of the proposed method was 0.833. Comparative experiments showed that the proposed SIMReg could better solve the problem of heart image registration and achieve a better registration effect on cardiac images.

Morphological Assessment and Possible Clinical Implications of the Left Ventricle Papillary Muscles. Study in a Colombian Population Sample

SUMMARY: Papillary muscles in the left ventricle present multiple anatomic expressions that are relevant for medical fields focusing on the understanding of clinical events involving these structures. Here, the aim was to perform a morphological characterization of the left ventricle papillary muscles in a sample of Colombian population. In the study were included eighty-two hearts from male individuals who underwent autopsy at the Institute of Legal Medicine and Forensic Sciences in Bucaramanga, Colombia. In each heart was carefully performed a longitudinal incision on the obtuse margin to visualize the papillary muscles. Data set was registered, and analysis of the continuous and categorical variables was carried out. Single anterior papillary muscle was observed in 74 samples (90.2 %) whereas this represented only 48 specimens (58.5 %) for the posterior papillary muscle (p = 0.3). Mean length and breadth of the anterior muscle were 29.9 ± 4.94 and 11.74 ± 2.75 mm, and those for the posterior muscle were 27.42 ± 7.08 and 10.83 ± 4.08 mm. Truncated apical shape was the most frequent type observed on the papillary muscles, anterior 41 (50 %) and posterior 37 (45.1 %), followed by flat-topped in the anterior 25 (30.5 %) and bifurcated in posterior muscle 14 (17.1 %). A mean of 9.04 ± 2.75 chordae raised from the anterior and 7.50 ± 3.3 from posterior papillary muscle. In our study we observed a higher incidence of single papillary muscles and slightly larger dimensions than information reported in the literature. The anatomic diversity of the papillary muscles should be considered for the correct image interpretation, valve implantation and performance evaluation on myocardial ischemic events.

Los músculos papilares del ventrículo izquierdo presentan múltiples expresiones anatómicas que son relevantes para las áreas médicas que se centran en la comprensión de los eventos clínicos que involucran estas estructuras. El objetivo fue realizar una caracterización morfológica de los músculos papilares del ventrículo izquierdo en una muestra de población colombiana. En el estudio se incluyeron ochenta y dos corazones de individuos masculinos a los que se les realizó autopsia en el Instituto de Medicina Legal y Ciencias Forenses de Bucaramanga, Colombia. En cada corazón se realizó cuidadosamente una incisión longitudinal en el margen obtuso para visualizar los músculos papilares. Se registró el conjunto de datos y se realizó el análisis de las variables continuas y categóricas. Se observó un solo músculo papilar anterior en 74 muestras (90,2 %), mientras que este rasgo se presentó en 48 muestras (58,5 %) para el músculo papilar posterior (p = 0,3). La longitud y anchura media del músculo anterior fueron 29,9 ± 4,94 y 11,74 ± 2,75 mm, y las del músculo posterior fueron 27,42 ± 7,08 y 10,83 ± 4,08 mm. La forma apical truncada fue el tipo más frecuente observado en los músculos papilares, anterior 41 (50 %) y posterior 37 (45,1 %), seguido de la forma plana en los 25 anteriores (30,5 %) y bifurcada en el músculo posterior 14 (17,1 %). Una media de 9,04 ± 2,75 cuerdas elevadas desde el músculo papilar anterior y 7,50 ± 3,3 desde posterior. En nuestro estudio observamos una mayor incidencia de músculos papilares únicos y dimensiones ligeramente mayores que la información reportada en la literatura. La diversidad anatómica de los músculos papilares debe ser considerada para la correcta interpretación de imágenes, implantación valvular y evaluación del desempeño en eventos isquémicos miocárdicos.

Trabeculations of the porcine and human cardiac ventricles are different in number but similar in total volume.

An intricate meshwork of trabeculations lines the luminal side of cardiac ventricles. Compaction, a developmental process, is thought to reduce trabeculations by adding them to the neighboring compact wall which is then enlarged. When pig, a plausible cardiac donor for xenotransplantation, is compared to human, the ventricular walls appear to have fewer trabeculations. We hypothesized the trabecular volume is proportionally smaller in pig than in human. Macroscopically, we observed in 16 pig hearts that the ventricular walls harbor few but large trabeculations. Close inspection revealed a high number of tiny trabeculations, a few hundred, within the recesses of the large trabeculations. While tiny, these were still larger than embryonic trabeculations and even when considering their number, the total tally of trabeculations in pig was much fewer than in human. Volumetrics based on high-resolution MRI of additional six pig hearts compared to six human hearts, revealed the left ventricles were not significantly differently trabeculated (21.5 versus 22.8%, respectively), and the porcine right ventricles were only slightly less trabeculated (42.1 vs 49.3%, respectively). We then analyzed volumetrically 10 pig embryonic hearts from gestational day 14-35. The trabecular and compact layer always grew, as did the intertrabecular recesses, in contrast to what compaction predicts. The proportions of the trabecular and compact layers changed substantially, nonetheless, due to differences in their growth rate rather than compaction. In conclusion, processes that affect the trabecular morphology do not necessarily affect the proportion of trabecular-to-compact myocardium and they are then distinct from compaction.

Miocardiopatía Hipertrófica en ausencia de criterios de hipertrofia. Serie de casos / Hypertrophic cardiomyopathy in the absence of hypertrophy criteria. Case series

La miocardiopatía hipertrófica (MCH) es la miocardiopatía hereditaria más frecuente, su principal expresión fenotípica consiste en hipertrofia ventricular izquierda (HVI) en ausencia de condiciones de carga que la justifiquen. Cuando existe una variante genética patogénica se denomina MCH sarcomérica. Los criterios diagnósticos más aceptados son HVI ≥ 15 mm en cualquier segmento o ≥ 13 en ciertas condiciones, criterios que tienen tres inconvenientes: 1) La HCM es una patología donde la HVI es evolutiva, existiendo otros elementos más precoces, pero menos precisos, como criptas, bandas musculares y alteraciones de la válvula mitral y músculos papilares; 2) Pacientes de baja estatura pueden no alcanzar estos umbrales; 3) La MCH apical no queda siempre bien representada usando estos grosores, requiriendo indexar por tamaño del paciente y/o considerar la HVI relativa (relación grosor apical / basal que no debe superar 1). Presentamos una serie de casos con genotipo confirmado para MCH que no cumplen los criterios de HVI aceptados para MCH y donde se debe individualizar el diagnóstico considerando los tres elementos señalados.

Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac condition; its phenotypic expression consists of ventricular hypertrophy (LVH) unrelated to loading conditions. In patients with a genetic pathogenic variant, the condition is termed sarcomeric HCM. Current diagnostic criteria are based on absolute left ventricular thickness, requiring ≥15 mm in any segment or ≥13 mm in particular conditions. These criteria have three pitfalls: 1) HCM is an evolving disease where LVH occurs gradually, with other early -but less precisephenotypic expressions such as myocardial crypts, muscular bands, or mitral and papillary muscle alterations; 2) Patients with short stature tend to have less LVH and do not reach the proposed thickness threshold. 3) Apical HCM is not correctly addressed in this cut-off as the heart tapers from base to apex, warranting indexing wall thickness to body size and using relative LVH in the apex (ratio from apex/base, abnormal,>1). This small case series includes three patients with a pathogenic genetic variant for HCM that doesn't satisfy the current criteria of LVH. For its precise assessment, the aforementioned points must be considered.