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Cardiac Hypertrophy, Heart Failure and Cardiomyopathy
Published in Mary N. Sheppard, Practical Cardiovascular Pathology, 2022
To use the term dilated cardiomyopathy accurately, a pathologist needs knowledge of the clinical picture and the macroscopic and microscopic features as detailed in Table 5.7. Macroscopically, the heart is increased in weight above 550 g in males and 450 g in females. The left ventricular wall is thinned circumferentially (less than 10 mm at midventricular level). There is also dilatation of the left ventricular chamber above 35 mm usually to 40 mm (measured transversely including trabeculae. There can be thrombi particularly at the apex of the left ventricle, with slight thickening of the subendocardium and focal pale areas of thickening scattered throughout the subendocardium (Figs. 5.21 and 5.22). There is often a line of midmyocardial fibrosis noted macroscopically, particularly marked in the interventricular septum (Fig. 5.22), which can also be seen on MRI imaging with gadolinium enhancement which is an important predictor of arrhythmias and sudden death.21 There can also be dilatation of the left atrium with thrombi in the left atrial appendage (seeFig. 5.18) (Fig. 5.23). Annular dilatation of the mitral annulus is also common with functional mitral valve regurgitation and slight thickening of the leaflet edges (seeFig. 5.18).
The cardiovascular system
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
Mary N Sheppard, C. Simon Herrington
X-linked diseases associated with dilated cardiomyopathy include muscular dystrophies (e.g. Becker and Duchenne) and X-linked dilated cardiomyopathy. Dilated cardiomyopathy may also occur in patients with mitochondrial cytopathies and inherited metabolic disorders (e.g. haemochromatosis). Examples of acquired causes of dilated cardiomyopathy include alcohol, nutritional deficiencies, endocrine dysfunction, and the administration of cardiotoxic drugs. It can also occur during pregnancy and in the peripartum period.
Epidermolysis bullosa
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Dominant DEB (DDEB) is the most common subtype of DEB. Clinical features of major subtypes of DEB are summarized in Table 21.5 [5]. Recessive DEB (RDEB) generalized severe presents at birth with widespread blistering, scarring, and deformity. Loss of fluid, blood, and protein leads to malnutrition, hypoalbuminemia, and severe anemia. There is increased risk of glomerulonephritis, renal amyloidosis, and IgA nephropathy in RDEB and rarely in DDEB. Death usually occurs in generalized severe RDEB during infancy and childhood as a result of septicemia, pneumonia, or renal failure. Dilated cardiomyopathy is an uncommon complication seen in 4.5% of patients by the age of 20 years, but it may be fatal if it occurs along with chronic renal failure. Other complications include anal scarring leading to constipation, urethral stenosis, urinary retention, hypertrophy of the bladder, and hydronephrosis [3,5]. RDEB generalized intermediate presents with more esophageal issues with advancing age. Risk of SCC in RDEB is 7.5%, 68%, 80%, and 90% by the age of 20, 35, 45, and 55 years of age. Regular inspection of a nonhealing wound is necessary, and if any suspicion is present, biopsy is mandatory. Children with RDEB generally survive in the neonatal and infantile periods but may develop infections later in childhood or cutaneous carcinomas in adulthood [3].
Myocarditis and autoimmunity
Published in Expert Review of Cardiovascular Therapy, 2023
Myocarditis, inflammation of the myocardium, may be acute or chronic, and persistent inflammation may progress to cardiomyopathy [1–3]. Myocarditis is often difficult to diagnose clinically because it may present with various signs and symptoms and may mimic other common heart diseases. However, early diagnosis is important since the treatment is different depending on the etiology, and an appropriate therapy can improve clinical course and prevent sequelae to dilated cardiomyopathy. Myocarditis is often caused by viral infections, but it is also associated with systemic autoimmune diseases, bacteria and other microorganisms, and drugs and other substances [1–3]. Persistent inflammation following acute myocarditis may lead to the development of dilated cardiomyopathy or cardiac dysfunction. Cytokines and immune cells that contribute to the innate immunity are involved in the inflammation of the acute stage, and the acquired immunity plays a role in the chronic stage [1–3]. Autoantibodies against various epitopes present on the heart were considered to contribute to the development of the disease [3,4].
Diastolic function and cardiovascular risk among patients with severe obesity referred to a lifestyle-program – a pilot study
Published in Scandinavian Cardiovascular Journal, 2023
Line M. Oldervoll, Rolf Gjestad, Christina Hilmarsen C, Anders Ose, Lisbeth Gullikstad, Ulrik Wisløff, Baard Kulseng, Jostein Grimsmo
In contrast to our findings of mostly normal cardiac structure and function, the Northern Manhattan Study in an older population found that changes in cardiac structure and function adjusting for T2D and hypertension may start already among those being overweight as the only factor (BMI > 25) [33]. Another study found that subjects with obesity develop changes in left ventricular structure and function without having known risk factors for CVD [37]. Others have described that severe obesity is associated with different changes in cardiac structure and function, probably because of increased blood volume leading to increased cardiac output and volume overload on the ventricles [4,38]. Cardiac remodelling in obesity is found to be associated with DD, HFpEF or obesity cardiomyopathy, as shown in studies described in the review by Wiling and Jacob [39]. Still, there is a lack of knowledge of the mechanisms leading to cardiac dysfunction in obesity [40]. The term obesity cardiomyopathy has been introduced, which is classified as a subtype of dilated cardiomyopathy, characterised by changes in ventricular structure and function including left ventricular dilatation, eccentric or concentric left ventricular hypertrophy, systolic and DD and right ventricular dysfunction seen in patients with severe obesity [41]. None in our sample fulfilled the criteria for HFpEF, which in the review from Mishra and Kass [42] is characterised as a multisystem disorder, including the adipose tissue described as an organ.
Cardiac involvement in the adult primary vasculitides
Published in Expert Review of Clinical Immunology, 2020
Giulia Pazzola, Nicolò Pipitone, Carlo Salvarani
Overall, aortic regurgitation is the most common type of valve disease in TAK, while pulmonary, mitral, and tricuspid regurgitation is less common and usually mild [3]. Patients with active disease tend to show a higher incidence of aortic regurgitation [4]. Aortic regurgitation in TAK is thought to be due to thickening of the aortic valves and enlargement of the aortic root [1]. On the other hand, myocardial involvement can be due to myocarditis, coronary artery disease, volume overload secondary to aortic insufficiency, while hypertension (secondary to renal artery stenosis) and premature atherosclerosis can be contributing factors [2,5,6]. Dilated cardiomyopathy is rare, but has been reported; histological examination showed evidence of myocardial inflammation [7]. Arrhythmia has been reported in the presence of myocardial disease [2]. With regard to coronary artery disease, the typical lesion of TAK is coronary stenosis [8], which mainly affects the coronary ostia and proximal vessel segments [2,9]. Aneurysms may also occur, but are less frequent [8]. Finally, occlusion of the pulmonary arteries and pulmonary hypertension can cause coronary steal phenomenon [8]. Pericarditis is uncommon, but may be the presenting feature of TAK [10,11].