Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology
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Four inherited disorders known to effect major arteries will be discussed, including Marfan syndrome (MS), Ehlers-Danlos syndrome (EDS), bicuspid aortic valve (BAV) and nonsyndromic familial aortic dissection. Recent advances in understanding their pathophysiology are presented, and how this knowledge impacts on diagnosis, prevention and treatment is discussed.
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Cardiovasc. Pathol. · Jan 2005
Pathology in patients with ventricular assist devices: a study of 21 autopsies, 24 ventricular apical core biopsies and 24 explanted hearts.
Ventricular assist devices (VADs) are used as a bridge to cardiac transplantation or as a permanent or sometimes temporary treatment for end stage heart failure. ⋯ VAD significantly reduced the amount of CN, MC and MW in the left ventricle but may lead to acquired aortic stenosis of native aortic valves or total occlusive thrombosis of aortic prosthetic valves. Proximate cause of death was, most often, VAD related.
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Cardiovasc. Pathol. · Jul 2004
ReviewNaxos disease and Carvajal syndrome: cardiocutaneous disorders that highlight the pathogenesis and broaden the spectrum of arrhythmogenic right ventricular cardiomyopathy.
Naxos disease is a recessive association of arrhythmogenic right ventricular cardiomyopathy (ARVC) with wooly hair and palmoplantar keratoderma or similar skin disorder. The clinical and histopathological spectrum of heart disease, molecular genetics and genotype-phenotype correlation are reviewed in 22 affected families with this cardiocutaneous syndrome reported in the literature from Greece, Italy, India, Ecuador, Israel and Turkey. All patients had the hair and skin phenotype from infancy and developed ARVC by adolescence. ⋯ A lethal autosomal recessive cardiocutaneous syndrome of Poll Hereford calves has been reported in Australia sharing similarities with the human syndrome reviewed here with respect to hair and cardiac phenotype. The cardiomyopathy in Naxos cardiocutaneous syndromes presents with increased arrhythmogenicity and variable left ventricular involovement and is characterized histologically by myocardial loss with fibrofatty or fibrous replacement at subepicardial and mediomural layers. The clinical heterogeneity and tissue characteristics in this cell-adhesion cardiomyopathy might be mutation specific and leads to consideration that the spectrum of ARVC should be broadened.
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Cardiovasc. Pathol. · May 2004
Case ReportsMultiple heart and pericardial lacerations due to blunt trauma from assault.
A case of 80-year-old woman who died of hemorrhagic shock as a consequence of multiple heart and pericardial lacerations as well as myocardial contusion due to blunt trauma from assault is reported. At postmortem examination, fractures of the body of the sternum and bilateral ribs were detected. A laceration of the anterior left-sided pericardium, a 2.8-cm linear tear in the right venous sinus from the origin of the inferior to the origin of the superior vena cava, a 3-cm laceration between the venous sinus and the right auricle, and a 2-cm tear in the anterior wall of the left atrium, behind the aortic trunk, were visible. Multiple heart and pericardial lacerations were thought to be caused by compression of the heart between sternum and vertebrae, together with multiple rib fractures and extensive movements of the sternum toward the spine as a result of kicking.
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Cardiovasc. Pathol. · Jan 2004
Case ReportsCardiac thromboemboli complicating a stab wound to the heart.
A 27-year-old man was noted to have neurologic deficit 4 days following cardiorrhaphy to repair a penetrating cardiac injury. Cerebral computed tomography scan showed multiple embolic infarcts and two-dimensional echocardiography revealed the source as thrombus in the left ventricle. Although this entity has been described following blunt chest trauma and cardiac contusion, it has not been noted in association with penetrating injury. Intracardiac thrombus with systemic emboli should be considered as a possible complication following cardiorrhaphy for penetrating trauma.