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Background: The association between Ebstein anomaly and myocardial fibrosis, particularly in the left ventricle, has been controversial. We aimed to assess the prevalence of replacement fibrosis with a focus on the left ventricle (LV) using cardiac magnetic resonance (CMR), make a histopathological association between LV fibrosis and CMR findings, and explore whether LV fibrosis is an independent risk factor for cardiovascular disease mortality using a derived risk score. Methods: We performed a 12-year (2009–2021) retrospective cohort of adult patients with Ebstein anomaly who underwent CMR. The CMR evaluation included a comprehensive assessment of myocardial fibrosis by late gadolinium enhancement (LGE). Four postmortem samples were obtained from our cohort and stained using Masson trichrome to characterize LV fibrosis. We used Cox-regression analysis to identify and derive a prediction score that associated LV fibrosis with cardiovascular disease mortality. Results: We included 57 adults with Ebstein anomaly (52% men; median age, 29.52 [interquartile range, 21.24–39.17] years), of whom 12 died during follow-up. LGE prevalence by CMR was observed in 52.6% in any chamber; LV-LGE in 29.8%. Histopathological findings revealed a mid-wall pattern with predominantly interstitial fibrosis and minimal replacement fibrosis. LV-LGE was associated with increased risk of cardiovascular disease mortality (hazard ratio, 6.02 [95% CI, 1.22–19.91]) attributable to lateral and mid-wall LV segment involvement. Our mortality score achieved an overall good prediction capacity (R 2 , 0.435; C statistic, 0.93; D xy , 0.86). Conclusions: There is a high prevalence of LV fibrosis replacement in adults with Ebstein anomaly, characterized by specific CMR and histological patterns. Furthermore, LV-LGE fibrosis is an independent predictor of cardiovascular disease mortality, which could be integrated into risk assessment in clinical management.

Background Congenital aortic diseases (CAoD) encompass a wide variety of disorders that range from asymptomatic findings to life-threatening conditions. Multiple imaging techniques are available for the assessment of CAoD. Case summary We present seven case reports of congenital aortic diseases, including obstructions in the aortic arch (coarctation, hypoplasia, and interruption) and vascular rings, in which the clinical manifestations throughout the cases are discussed, highlighting the heterogeneity of the symptoms. Discussion Multi-imaging techniques are indispensable for the assessment of CAoD, where cardiac computed tomography angiography is the main modality for rapid acquisition of three-dimensional volume-rendered images for optimal surgical planning.

Introduction: Congenitally corrected transposition of the great arteries (CCTGA) is a rare heart disease that encompasses an atrioventricular and ventriculoarterial discordance and accounts for less than 1 percent of congenital heart diseases. Objective: To present an atypical case of a man with complex congenital heart disease and conduction anomalies. Case Presentation: This is a case of a 34-year-old patient who came to the hospital with 1 week of dyspnea on exertion and episodes of lipothymia. The patient was referred to our hospital after an electrocardiogram from his primary care with evidence of blocked atrial fibrillation. During the initial evaluation blocked atrial fibrillation was confirmed with a rescue ventricular rate of 38 bpm. A magnetic resonance confirmed the presence of the atrioventricular and ventriculoarterial discordance, the aorta had a left anterior position, perimembranous ventricular septal defect with a right to left shunt, biventricular systolic dysfunction, moderate tricuspid, and mitral regurgitation, interventricular septal intramyocardial and biatrial fibrosis, left atrial dilation, and dilation of the pulmonary artery. After pacemaker placement, the patient has an improvement in his clinical symptoms and quality of life. Conclusions: Cardiac arrhythmias are CCTGA’s leading cause of death, mostly ventricular tachycardia, and atrial fibrillation. Right bundle branch block is a previously unreported and potentially very rare presentation of this disease. This, added to the fact that our patient was diagnosed at an advanced age, but without symptoms of heart failure, makes him an atypical case of CCTGA, with new potential treatment options.

Anomalous origin of the pulmonary artery branches is a rare phenomenon. We describe a case of an adult with anomalous origin of the right pulmonary artery (hemitruncus arteriosus) associated with patent ductus arteriosus. Non-invasive imaging studies played an important role in the diagnosis and follow-up. Angiography allowed to determine the severity of pulmonary hypertension. He underwent surgical closure of patent ductus arteriosus, redirection of right pulmonary artery and atrioseptostomy with decrease of the pulmonary pressure in the follow-up. A high index of clinical suspicion of this entity is required in adults with heart failure, recurrent hemoptysis and pulmonary hypertension, because it could go unnoticed.

Female 23-year-old patient with heart murmur diagnosed in the first year of life. She attended to our institute with progressive dyspnea and palpitations of 7-month evolution. Physical examination showed perioral and distal cyanosis with digital clubbing, oxygensaturation of 79%, jugular plethora, arrhythmic heartsounds of upper limbs, fixed second heart sound, systolic tricuspid murmur, and edema. Electrocardiogramand 24 hour Holter monitoring showed atrial fibrillationand right bundle branch block.

Background Saw-tooth cardiomyopathy (STC) is an unusual type of left ventricular dysplasia. To our knowledge, six cases have been reported in the literature. Two new cases are presented with a review of all the case reports that have been published. Case summary Two patients with STC were examined. The first one was a 69-year-old woman with shortness of breath on mild exertion and chest pain, and the second was a 49-year-old man with a history of myocardial infarction who required stent implantation and is now asymptomatic. Both patients revealed findings of STC in the cardiac computed tomography (CT). Discussion When analysing the cases and comparing them to the ones reported in the literature; STC is a generally benign heart disease, although the clinical spectrum can range from asymptomatic to heart failure. Imaging studies such as cardiac magnetic resonance and cardiac CT are essential for the diagnosis.

Introduccion Los defectos cardiacos congenitos constituyen el 30% de todas las anomalias congenitas. La prevalencia es de 8/1,000 recien nacidos vivos, sin predominio de genero. Para una planificacion quirurgica optima es esencial una evaluacion precisa de la anatomia en los defectos cardiacos congenitos. Las modalidades de imagen como el ecocardiograma, la angiografia por cateterismo cardiaco, la tomografia computarizada (TC) o la resonancia magnetica (RM) se utilizan de forma regular para el diagnostico de las cardiopatias congenitas. Estos metodos pueden proporcionar reconstrucciones virtuales en reconstruccion volumetrica o 3D, pero no replicas tactiles reales de la anatomia cardiaca. Objetivo Realizar modelos de corazon impresos en 3D con la finalidad de proporcionar replicas tactiles 3D reales de la anatomia cardiaca para visualizar de forma detallada todas las perspectivas posibles de las estructuras extracardiacas o intracardiacas. Metodos Los datos de la imagen se obtuvieron en formato DICOM, se editaron en el paquete de software "3D slicer 4.3" y se exportaron para la impresion en formato de archivo (.stl). Resultados y conclusiones Con la impresion 3D se puede evaluar de forma detallada la anatomia intracardiaca y extracardiaca con modelos cardiacos en tiempo real. Esta tecnica es de gran utilidad, sobre todo en los defectos cardiacos congenitos complejos, ya que permite hacer una planificacion precisa del procedimiento quirurgico. Introduction Congenital heart disease makes up for 30% of all congenital anomalies. The prevalence is 8/1,000 live newborns, without predominance of gender. Imaging methods such as echocardiography, angiography, computed tomography or magnetic resonance imaging must be routinely used in congenital heart disease. The mentioned methods can provide virtual reconstructions in volumetric reconstruction or in three dimensional (3D), but only 3D-printed heart models can provide real 3D tactile replicas of cardiac anatomy. Objective To make 3D printed heart models in order to provide real 3D tactile replicas of the cardiac anatomy that allow a detailed visualization from all possible perspectives, either of extracardiac or intracardiac structures. Methods This information is useful for surgical decision making, especially in patients with complex cardiac defects. DICOM, edited in a software package “3D slicer 4.3” and exported for printing in file format (.stl). Results and conclusions With 3D printing, the intracardiac and extracardiac anatomy can be evaluated in detail with real-scale cardiac models of the patient, avoiding unexpected findings. This technique is very useful especially in complex congenital heart defects, since it allows precise planning of the surgical procedure.

Female 23-year-old patient with heart murmur diagnosed in the first year of life. She attended to our institute with progressive dyspnea and palpitations of 7-month evolution. Physical examination showed perioral and distal cyanosis with digital clubbing, oxygensaturation of 79%, jugular plethora, arrhythmic heartsounds of upper limbs, fixed second heart sound, systolic tricuspid murmur, and edema. Electrocardiogramand 24 hour Holter monitoring showed atrial fibrillationand right bundle branch block.

Background: Apical hypertrophic cardiomyopathy (AHCM) is a variant of hypertrophic cardiomyopathy affecting 25% of the Japanese population. Echocardiography is an essential method for the diagnosis and follow-up of this disease. Aim: The main objective of this study is to describe the prevalence and characteristics of AHCM in the Mexican population, who attended the National Institute of Cardiology Ignacio Chávez Material and methods: From 1994 to 2017, a total of 13 patients with AHCM diagnosis were included. All patients had complete medical history, electrocardiogram (ECG) and echocardiogram. In some patients, myocardial perfusion, nuclear magnetic resonance imaging (NMRI), pharmacological stress echocardiography, coronary angiotomography and cardiac catheterization were analyzed. Patients with other types of hypertrophic cardiomyopathy. Results: The mean age was 67.5±9.5 years, 62% were men. The clinical diagnosis for AHCM was on first instance in only 23% of the cases, the remaining 77% had an initial diagnosis of ischemic heart disease. Negative T wave was detected in 100% of the patients and 46.1% presented giant T-wave inversion. All of the patients were diagnosed by echocardiography, four patients presented a mixed form of AHMC and the rest of them pure AHMC. Conclusions: The prevalence of AHCM in our institution is of 0.4% in general population and in relation to hypertrophic cardiomyopathy (HM) of 3%. The clinical and electrocardiographic characteristics of AHCM suggested data compatible with ischemic heart disease. In all patients echocardiography should be the first-choice non-invasive study. In cases of diagnostic doubt or presence of concomitant diseases, other non-invasive imaging studies, such as CMRI or nuclear medicine are recommended.

A 58-year-old male with a history of a soft tissue sarcoma in remission presented with a 2 weeks history of progressive dyspnea. Transthoracic echocardiography showed right ventricular dilation; right ventricular systolic pressure (RVSP) of 110 mm Hg, and a lobulated mass in the right ventricular outflow tract (RVOT) causing obstruction. Microbubble contrast was administered showing perfusion within the mass, which suggested malignancy. A CT pulmonary angiogram (CTPA) confirmed the presence of the mass in the RVOT without evidence of pulmonary embolism. This case demonstrates the importance of the multimodality imaging approach for the differential diagnosis of masses in the RVOT.

Nowadays, there is a wide array of imaging studies available for the evaluation of coronary artery disease, each with its particular indications and strengths. Cardiac single photon emission tomography is mostly used to evaluate myocardial perfusion, having experienced recent marked improvements in image acquisition. Cardiac PET has its main utility in perfusion imaging, atherosclerosis and endothelial function evaluation, and viability assessment. Cardiovascular computed tomography has long been used as a reference test for non-invasive evaluation of coronary lesions and anatomic characterization. Cardiovascular magnetic resonance is currently the reference standard for non-invasive ventricular function evaluation and myocardial scarring delineation. These specific strengths have been enhanced with the advent of hybrid equipment, offering a true integration of different imaging modalities into a single, simultaneous and comprehensive study.