Cardiac Inflammatory Myofibroblastic Tumours in Three Young Adult Cats.

Inflammatory myofibroblastic tumours (IMTs) of the heart are described in three young adult cats (case 1, 3.5 years old; case 2, 2.7 years old; case 3, 1.8 years old) that died due to pleural and pericardial effusions resulting from congestive heart failure. At necropsy, cardiac masses, measuring 4 × 3 × 2.5 cm (case 1), 3.5 × 2.5 × 2 cm (case 2) and 3 × 2.5 × 2 cm (case 3), were located mainly in the right auricle (case 1) and atrial septum (cases 2 and 3) with variable extension into the surrounding tissue. Histologically, the atrial masses in all three cats comprised proliferation of spindloid mesenchymal cells and an inflammatory infiltrate of plasma cells and lymphocytes in variable amounts of myxoid background. The spindloid cells were uniform and characterized by pale eosinophilic cytoplasm and a vesicular nucleus containing prominent nucleoli, with no remarkable cytological atypia or mitotic activity. Immunohistochemically, these cells were positive for vimentin and α-smooth muscle actin in all cases, supporting a myofibroblastic origin. The histopathological and immunohistochemical features were consistent with those of IMTs in humans and animals. There have been no previous reports of such tumours occurring in the heart of the cat.

Histological Features of the Atrioventricular Conduction System in Cats with High-Grade Atrioventricular Block.

The histopathological features of the atrioventricular (AV) conduction system of nine cats with high-grade atrioventricular block (H-AVB) were compared with those of 22 cases of third-degree atrioventricular block (3-AVB). All nine H-AVB cats had experienced syncopal attacks of variable severity and had been diagnosed electrocardiographically. The AVB, which was permanent in all cases, had been observed for 15-1,981 days (average 663 days) before death. Histological examination of the AV conduction system revealed moderate (25-50%) or severe (>50%) loss of conduction fibres associated with fibrous replacement in the regions of the branching portion of the AV bundle and the upper portion of the left bundle branch in almost all of the H-AVB cases and in all the 3-AVB cases. Comparison of lesion severity in each region in the H-AVB and 3-AVB cases revealed that the branching portion of the AV bundle was less severely affected in the H-AVB cases. This finding might explain why the H-AVB cases had intermittent block of AV conduction while the 3-AVB cats had permanent block.

Case Report: Occurrence of Severe Thoracic Aortic Aneurysms (Involving the Ascending, Arch, and Descending Segments) as a Result of Fibulin-4 Deficiency: A Rare Pathology With Successful Management.

Aortic diseases requiring surgery in childhood are distinctive and rare. Very few reports in the literature account for the occurrence of multiple thoracic aortic aneurysms in the same pediatric patient because of a genetic cause. We report a rare occurrence of severe thoracic aortic aneurysms (involving the ascending, arch and descending aortic segments) with severe aortic insufficiency in a 7-year-old female child secondary to the extremely rare and often lethal genetic disorder, cutis laxa. She was eventually identified as a carrier of a homozygous EFEMP2 (alias FBLN4) mutation. This gene encodes the extracellular matrix protein fibulin-4, and its mutation is associated with autosomal recessive cutis laxa type 1B that leads to severe aortopathy with aneurysm formation and vascular tortuosity. Parents of the child were not known to be consanguineous. Significant symptomatic improvement in the patient could be discerned after timely intervention with the valve-sparing aortic root replacement (David V procedure) and a concomitant aortic arch replacement. This is a unique report with a successful outcome that highlights the occurrence of a rare hereditary aortopathy associated with a high morbidity and mortality, and the importance of an early diagnosis and timely management. It also offers insight to physicians in having a very broad differential and multimodal approach in handling rare pediatric cardio-pathologies with a genetic predisposition.

The Anatomical Substrate for Sick Sinus Syndrome in Dogs.

The hearts of 28 dogs, clinically diagnosed as having symptomatic sick sinus syndrome (SSS), were examined post mortem, with a particular focus on the sinus node (SN) region. The affected dogs were divided into two groups according to the findings of ambulatory electrocardiography: 16 dogs with severe sinoatrial (SA) block and/or sinus arrest (group A) and 12 dogs with long sinus or atrial pauses due to SA block and/or sinus arrest accompanied by atrial tachyarrhythmias (group B). The most significant histopathological changes found in both SSS groups were extensive destruction of the SN characterized by depletion of nodal cells with fatty or fibrofatty replacement and interruption of contiguity between the SN and the surrounding atrial myocardium. Furthermore, in group B, the SN lesions were combined with fibrosis of the atrial myocardium. The results of this investigation improve our understanding of the close relationship between the electrocardiogram findings and pathological alterations in each group. Because most human cases of SSS are due to degenerative fibrosis of the SN, the loss and disappearance of nodal cells with a corresponding increase in fatty or fibrofatty tissue, may be specific to canine cases of SSS.

Non-18F-FDG/18F-NaF Radiotracers Proposed for the Diagnosis and Management of Diseases of the Heart and Vasculature.

18F-fluorodeoxyglucose (18F-FDG) and 18F-sodium fluoride (18F-NaF) are front-runners in PET. However, these tracers have limitations in the imaging of diseases in the heart. A multitude of other radiotracers have been identified as potentially useful PET agents in the identification of cardiovascular disease. This critical review examines recent studies with the use of non-18F-FDG/18F-NaF radiotracers in the identification and surveillance of cardiovascular diseases. We highlight the need for further investigation into alternative PET radiotracers to demonstrate their clinical value in the management of these pathologies.

Idiopathic multifocal myocardial atrophy with fibrosis and fatty infiltration involving Purkinje fibres in a 13-year-old Arabian broodmare: Histopathological features.

Myocardial atrophy with fibrosis and fatty infiltration involving the cardiac conduction system is relatively unusual in horses. We herein report of such a case in a 13-year-old Arabian broodmare that had spontaneously died on a paddock. An autopsy revealed multifocal myocardial atrophy with concomitant fibrosis and fatty infiltration in both the ventricles and interventricular septum. The Purkinje fibres in the ventricles and interventricular septum were surrounded by thick fibrous or adipose tissues adjacent to atrophic myocardial cells. Myocardial fibrosis and fatty infiltration were likely secondary to myocardial atrophy, occurring as a pathological response triggered by the repair of muscular wall injury. However, there were no major vascular pathologies (e.g. atherosclerosis and arteriosclerosis); hence, the pathogenesis of myocardial atrophy was unclear. There was no evidence of myocardial atrophy ̵ induced pathologies such as infarct, ischaemic lesions, myocardial degeneration, myocarditis and endocarditis. However, such an unusual histopathological pattern may be associated with rapid clinical deterioration and death.

Caracterización de pacientes con infarto agudo del miocardio con elevación del segmento ST, Baracoa, Guantánamo 2017-2019 / Characterization of the patients with ST-segment elevation myocardial infarction, Baracoa, Guantanamo, in the period 2017-2019

RESUMEN Introducción: En el Hospital General Docente "Dr. Octavio de la Concepción y de la Pedraja" de Baracoa, Guantánamo, hasta la fecha, no se ha caracterizado la morbilidad y mortalidad por infarto agudo del miocardio. Objetivo: Caracterizar la morbilidad y mortalidad por infarto agudo del miocardio en el citado hospital durante el trienio 2017-2019. Método: Se hizo un estudio descriptivo, retrospectivo y de corte transversaldel total de pacientes infartados en el trienio 2017-2019 (n=75). Se estudió la edad, sexo, características del infarto (semiología del dolor, localización, clasificación pronóstica-clínica, complicaciones, estado al egreso y causas de muerte). Resultados: El 72,0 % de los pacientes fueron hombres y el 37,3 % tenía 50 y 59 años de edad. La letalidad representó el 14,7 %. Fue más común el infarto anterior del ventrículo izquierdo (53,4 %). El 28,0 % presentó una clase IV, según criterios de Killip-Kimball y de Forrester. El 49,3 % mostró alto riesgo según la escala GRACE. El 88,0 % tuvo complicaciones, la más común del tipo mecánica (60,0 %). La encefalopatía isquémica-hipóxica posparada cardiorrespiratoria secundaria a fibrilación ventricular (54,5 %) fue la causa directa de muerte más frecuente. Conclusiones: Se elabora un referente que describe el infarto agudo del miocardio en el contexto territorial.

ABSTRACT Introduction: Morbidity and mortality by myocardial infarction has not been characterized so far in the General Teaching Hospital "Dr. Octavio de la Concepcion y la Pedraja" in Baracoa, Guantanamo. Objective: Tocharacterize the morbidity and mortality by myocardial infarction on the institution in the triennium 2017-2019. Method: A descriptive, retrospective and cross-sectional study was carried out in the patients diagnosed with infarction in the triennium 2017-2019 (n=75). Were taken into account the following variables: age, gender, clinical characteristics of infarction (painful symptoms, location, prognostic and clinical classification, complications, status of the patient at the time of discharge and cause of death). Results: 72.0 % of the patients were male, and the 37.3 % had an age ranging from 50 to 59 years old. Lethality represented a 14.7 %. The anterior left ventricle wall infarction was the most common (53.4 %). 28.0 % presented a class IV type, according to the Killip-Kimball and the Forrester classifications. 49.3 % presented high risks according to the GRACE score. 88.0 % had complications, the most common of them being the mechanical type (60.0 %). The most common cause of death was the hypoxic-ischemic encephalopathy caused by secondary atrial fibrillation (54.5 %). Conclusions: A reference to describe the myocardial infarction in the province was elaborated.

Sudden unexpected death in a case of necrotizing eosinophilic myocarditis.

Acute myocarditis is related to a significant number of sudden deaths among young adults and its diagnosis is often demanded to post-mortem investigations performed on a forensic setting. Eosinophilic myocarditis (EM) is a rare form of myocarditis that is pathologically characterized by myocardial inflammation with eosinophils, often in association with elevated levels of circulating blood eosinophils. The sudden death of a 19-year-old boy with no past medical history is reported. Diagnosis of fatal acute EM was performed after a comprehensive investigation including an in-depth analysis of anamnestic and circumstantial data, and complete autopsy followed by toxicologic and cardio-pathological investigations. Discussion focuses on the forensic issues related to diagnosis and therapy of this rare form of acute myocarditis. As acute EM may be patchy, extensive myocardial sampling is mandatory in order to recognize the extent and the phase of the disease. An early diagnosis is the basis for a timely therapy, which is the key-point for prevent extensive myocardial damage, allowing a better outcome, especially when EM is acute and necrotizing. However, as demonstrated from the case herein reported, the course of EM is sometimes fulminant and does not allow any therapy nor even clinical diagnosis. Finally, this paper serves as reminder to consider this infrequent disease in differential diagnosis when facing with a sudden death, even in a young subject and in the absence of any prodrome.

Post-mortem thermal angiography: a pilot study on swine coronary circulation.

Thermal imaging (TI) allows the detection of thermal patterns emitted from objects as a function of their temperature in the long-infrared spectrum and produces visible images displaying temperature differences. The aim of this pilot study was to test TI to visualize the coronary circulation of swine hearts. Thirty swine hearts were prepared for ex situ coronarography, and thermal images were acquired through a FlirOne thermal camera (FLIR Systems®) paired with a Google Android Smartphone. Coronary arteries were cannulated, namely the anterior interventricular artery, the circumflex branch of the left coronary artery, and the right coronary artery. The heart was cooled, and contrast medium (CM) consisting of distilled water heated to 40 °C was injected in a coronary vessel, while thermal images were captured. These steps were repeated for each coronary vessel and under experimentally simulated coronary heart disease. Thermal imaging coronarography (TIC) allowed a clear representation of the morphology and course of the coronary vessels and of experimentally simulated coronary heart disease, moreover, demonstrated to be easy to perform during or after autopsies on ex situ hearts, non-destructive, reproducible, and cheap. On the basis of these preliminary results, TIC might allow a subsequent more focused and comprehensive cardiopathological examination of the heart, which remains mandatory for the definitive diagnosis of coronary heart disease. Although these preliminary results seem encouraging, further systematic studies on human hearts, both normal and pathological, are necessary for estimating the sensitivity and specificity of the proposed method and to draw any definitive conclusion.

The First 5-Year-Long Survey on Intrauterine Unexplained Sudden Deaths from the Northeast Italy.

PURPOSE: Sudden intrauterine unexplained death syndrome (SIUDS) represents one of the main open issues in the scientific and social setting of the modern medicine, and our efforts have aimed to understand its possible causes and risk factors. METHODS: A 43-case series of consecutive unexplained fetal deaths coming from Northeast Italy, collected in a 5-year period (2011-2015), has been submitted to an in-depth investigation, based on neuropathological and cardiopathological examinations, immunohistochemistry for neuronal nuclear antigen (NeuN), genetic characterization for the serotonin transporter (5-HTT) gene polymorphisms, and toxicological environmental analyses. RESULTS: The overall survey from the neuropathological findings highlights one or more congenital morphological abnormalities of the autonomic nervous system in 77% of cases of sudden fetal deaths. CONCLUSIONS: From our results emerges the need to perform a complete autopsy of all SIUDS victims with an in-depth examination of the neuronal centers of the brainstem, which modulate the vital functions.