Clinical and pathologic findings of aortic dissection at autopsy: Review of 336 cases over nearly 6 decades.

BACKGROUND: We aimed to characterize the clinical and pathologic findings of aortic dissection (AD) over a nearly 60-year period. METHODS: The Jesse E. Edwards Registry of Cardiovascular Disease database was queried for cardiac specimens from autopsies with AD as a diagnosis and compared 2 cohorts: early (1956-1992) and current (1993-2015). RESULTS: From 1956 to 2015, 338 cases (166 early, 170 current) with AD were included (mean age: 60; 62% male). The AD was 86% type A and 14% type B. Sixty-two percent of cases were under medical care at time of death (61% early, 62% current, P = not significant). Of those under medical care, 63% were not diagnosed prior to death (64% early, 62% current, P = not significant). Risks for dissection did not differ between time intervals and include left ventricular hypertrophy, suggestive of hypertension (84%), prior cardiovascular surgery (38%), bicuspid valve (14%), and connective tissue disease (9%). An intimal tear was identified in the ascending aorta in the majority (68%), followed by descending (14%), root (9.5%), and arch (7%). Aortic rupture occurred in 58%, most frequently in the ascending aorta (41%). CONCLUSIONS: In a large cardiovascular registry, >60% of cases of AD were not detected clinically and first identified at autopsy. Although diagnostic techniques have significantly improved over the time interval, the percentage of AD discovered at autopsy did not differ from the early to the current era. The most prevalent risk factors for dissection including hypertension and prior cardiovascular surgery remain similar in both time periods. AD death is related to rupture of the aorta in the majority of cases.

Uhl's anomaly: perspective of fetal echocardiography and histopathological correlation.

We report a case of Uhl's anomaly imaged at 19 weeks of gestation by fetal echocardiography with pathological confirmation by anatomical gross heart specimen and tissue histology. Uhl's anomaly of the right ventricle is a rare cardiac disorder with isolated right ventricular enlargement with almost complete absence of the right ventricular myocardium.

Patho-Histological Findings of Annular Rupture Related to Left Ventricular Outflow Tract (LVOT) Calcification Following Transcatheter Aortic Valve Replacement (TAVR).

Peri-aortic hematoma has been recently described as a potentially life-threatening complication following transcatheter aortic valve replacement (TAVR). Patient- and procedure-related factors exist that predispose to peri-aortic hematoma formation, which can progress to myocardial rupture at the aortic root-myocardial junction. While conservative therapy with blood pressure control is the expectant management following peri-aortic hematoma formation, myocardial rupture can occur at the site of the aortic annulus. Hence, interventionists and echocardiologists must be prepared for emergent intervention to salvage the patient once the complication is recognized. The present report highlights the patho-histological findings related to left ventricular outflow tract calcification following TAVR.

Expression of cathepsin K and tartrate-resistant acid phosphatase is not confined to osteoclasts but is a general feature of multinucleated giant cells: systematic analysis.

OBJECTIVE: Cathepsin K and tartrate-resistant acid phosphatase (TRAP) are two proteins expressed in osteoclastic giant cells. Recently we showed that lesional multinucleated giant cells (MNGs) in pulmonary granulomatosis with polyangiitis expressed these proteins. We aimed to clarify whether the expression of these two proteins has any specificity or is a general feature of MNGs associated with multiple types of granulomatous inflammation. METHODS: In total, 7 Crohn's disease (CD), 5 GCA, 5 giant cell myocarditis (GCM), 11 sarcoidosis and 6 tuberculosis cases were examined for expression of cathepsin K and TRAP using immunohistochemistry (IHC). Protein expression was semi-quantitatively classified as none, weak, moderate or strong. In addition, tissue TRAP activity was examined using an enzymatic reaction. RESULTS: The expression of cathepsin K was robust in >95% of MNGs of all examined disease groups, whereas TRAP expression varied; CD, GCA and tuberculosis showed strong TRAP expression. TRAP expression in sarcoidosis and GCM was weaker (CD vs GCM, P = 0.04; CD vs sarcoidosis, P = 0.06). Compared with IHC, TRAP detection using an enzymatic colour reaction had limited sensitivity. CONCLUSION: Expression of TRAP and cathepsin K is a general feature of MNGs and their expression might be related to histopathological pattern.

Sudden death due to spontaneous acute dissection of the left subclavian artery with rupture during postpartum period: a case report.

Subclavian artery dissection is usually associated with coexisting aortic disease. Isolated and spontaneous acute subclavian artery dissection is uncommon and rarely reported. In addition, no case of left subclavian artery dissection during pregnancy and early puerperium has been described. We report the autopsy case of a 24-year-old female who died suddenly 3 days after delivery due to a spontaneous left subclavian artery dissection with rupture.

Pathology of the aortic arch in hypoplastic left heart syndrome: surgical implications.

Aortic arch reconstruction plays an important role in the success of the Norwood procedure (NP) for hypoplastic left heart syndrome (HLHS). This study investigated the cardiac specimens to determine the etiology of distal aortic arch obstruction after the NP for HLHS and to locate coarctation of the aorta in HLHS untreated by surgery. This study examined 17 cardiac specimens: 9 that had NP and 8 not treated by surgery. The findings after NP showed frequent failure to resect the coarctation segment completely and failure to extend the augmentation patch into the descending aorta. Five (62.5%) of the eight hearts not treated by surgery had significant periductal coarctation of the aorta. After NP for nine patients, three (33%) had residual coarctation of the aorta. To minimize the risk of recurrent or persistent aortic arch obstruction after NP and to improve the long- and short-term outcome, the ductal tissue and the coarctation segment encircling the aortic lumen should be resected. The distal wall incision should be extended at least 5 mm beyond the distal aspect of the ductal tissue. These steps could avoid major aortic arch obstruction, promote growth of the native aortic tissue, and avoid ventricular dysfunction.

Sudden death with circumferential subepicardial fibrofatty replacement: left-sided arrhythmogenic ventricular cardiomyopathy.

We report 5 cases of sudden cardiac death, with similar cardiac findings. All 5 cases had circumferential left ventricular subepicardial fibrofatty replacement of the myocardium, similar to the histologic features of arrhythmogenic right ventricular cardiomyopathy (ARVC). In these cases, the findings were predominantly in the left ventricle with minimal or no involvement of the right ventricle. Four of the 5 cases had siblings with either sudden death or cardiac symptoms. This report highlights 5 cases of sudden death in the young with histologic findings similar to ARVC, with predominant left ventricular involvement and questions whether the cases represent a larger spectrum of the cardiomyopathy known as ARVC, which perhaps should be more correctly termed as "arrhythmogenic cardiomyopathy" or represent a separate, potentially inheritable cardiomyopathic entity. We report these cases to familiarize forensic pathologists with this uncommon and potentially inheritable condition.

Histologic Examination of the Heart in the Forensic Autopsy.

Histologic examination of the myocardium, valves, and cardiac blood vessels is often as important as the gross examination. The diagnostic features and categories of heart disease are many and varied, possibly more than any other organ. We present a review of the histologic features of forensically important heart disease.