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Aims: Shaggy aorta is defined as “very extensive atheromatous disease with diffuse ulcers associated with soft, loosely held debris and a paucity of actual thrombus” and often results in visceral or peripheral arterial embolization (shaggy aorta syndrome). Most of the studies are clinico-radiological with hardly any assessment of the pathological features. We present an autopsy analysis of shaggy aorta. Materials and Methods: A retrospective study of autopsied cases of shaggy aorta over 15 years was conducted. The involvement of the various segments of the aorta (ascending, transverse, thoracic, and abdominal) was correlated with the clinical manifestations and cardiac/extra-cardiac findings at autopsy. The mortality was categorized as those related to shaggy aorta (Group I), related to cardiac diseases (Group II), and those unrelated to cardiovascular diseases (Group III). Statistical Analysis: Nil. Results: In a span of 15 years, there were 76 cases of shaggy aorta affecting predominantly males (85.5%) and patients in the sixth decades of life (mean age of 64.5 years). The important associated cardiovascular risk factors included hypertension, tobacco use, and diabetes mellitus. Predominant involvement of the entire aorta and arch + descending aorta was seen in 39.5% and 35.5% of the cases, respectively. Regardless of extreme severity, only half of the patients (37 cases, 48. 7%) had clinical presentation due to shaggy aorta. Conclusions: The occurrence of shaggy aorta may be more common than expected, and it would be important to keep this possibility in mind even in asymptomatic elderly patients with cardiovascular risk factors since aorto-arterial manipulations and anti-coagulant therapy can prove detrimental in such patients.

Background: Non-thrombotic pulmonary embolism, an uncommon entity, is defined as the embolization of tissues, microorganisms, air, or foreign material. One subset in this non-thrombotic category is septic pulmonary embolism (SPE) that refers to embolism of microorganisms with or without a thrombotic mantle into the pulmonary vasculature. This condition is often recognized on the basis of imaging with a clinical correlation. Unfortunately, data regarding the pathological features are meager. This has prompted to review such cases at autopsy. Aims: To study the pathological features of SPE at autopsy. Materials and Methods: Ten-year (2012 to 2021) autopsy records of the hospital were retrospectively reviewed. The diagnosis was based on the identification of acute necrotizing pulmonary arteritis with peri-bronchoarterial consolidation. These cases were analyzed with reference to the demographics, clinical characteristics, and pulmonary/extrapulmonary findings at autopsy. Statistical Analysis: Nil. Results: According to the inclusion criterion, 19 cases demonstrated the presence of SPE. There were 11 men and 8 women with a mean age of 32.1 years. The major source of infection included infection arising from skin and musculo-skeletal system (11 patients, 59.7%). The common clinical presentation included fever, dyspnea, chest pain, hemoptysis, and altered sensorium. The cause of death was mainly due to septicemia and/or confluent lung consolidations. A large number of bacterial colonies were seen in all; Candida species were also identified in two cases. Other lung findings included diffuse alveolar damage, fresh arterial thrombosis, infarction, arterial pseudo-aneurysms, abscess formation, and pyogenic pleuritis. Conclusion: Presence of an extrapulmonary infection with persistent fever, bacteremia, and pulmonary complaints should raise suspicion for this entity, particularly in resource-poor settings, to prevent grave pulmonary complications.

Background: Granulomatous cardiomyopathy (GCM) is relatively uncommon in patients presenting with ventricular tachycardia (VT). Sarcoidosis and tuberculosis are the most common causes of GCM with VT. The aim of study was to evaluate their clinical characteristics and the long-term outcomes. Methods: We retrospectively analyzed patients from March 2004 to January 2020, presenting with VT and subsequently diagnosed to have GCM. Patients were divided into three groups (sarcoid, tuberculosis and indeterminate) based on serologic tests, imaging and histopathology. The response to anti-arrhythmic and disease specific therapy on long-term follow-up were analyzed. Results: There were 52 patients, comprising 27 males and 25 females, age 40 ± 10 years. The follow-up period was 5.9 ± 3.9 years. Sarcoidosis was diagnosed in 20 (38%); tuberculosis (TB) in 15(29%) and 17(33%) patients were indeterminate. Left ventricular ejection fraction (LVEF) of the entire cohort was 0.45 ± 0.14. Erythrocyte Sedimentation Rate(ESR) was found to be significantly higher in TB(43.6 ± 18.4) patients vs sarcoid(18.9 ± 6.7)p

Aims: To study the clinical and pathological manifestations of missed cases of rheumatic heart disease (RHD) and postulate possible reasons behind a missed diagnosis. Materials and Methods: Retrospective 20-year (2000–2019) autopsy data of chronic RHD were reviewed and patients, in whom the valvular deformities had been incidental autopsy findings, were selected. The clinical details of these patients were correlated with the morphology of the affected valves. On this pathological analysis, the patients were assigned to a category of subtle or significant valvular deformity. By clinically correlating, the latter group was subdivided into clinically misdiagnosed, clinically undiagnosed, and sudden cardiac death. Statistical Analysis: Nil. Results: Among the 475 cases of chronic RHD identified at autopsy in the study period, the disease was diagnosed incidentally in 69 patients (14.5%). Significant valvular deformity was noted in 61 cases while the other 8 cases had subtle valvular deformity. The most common cause of death was cardiac failure in 39 out of 69 patients (56%). Eleven (16%) patients had experienced sudden cardiac death. Among the undiagnosed cases, 5 (7%) of them had a diagnosis of non-rheumatic cardiac disease, while the other 14 (20.5%) patients had overwhelming non-cardiac diseases. Conclusions: Our study indicates that mortality and morbidity due to RHD are underdetermined. The patients remain undiagnosed due to either insignificant valvular involvement, clinically silent in the presence of significant valvular deformity, presence of other overwhelming diseases or misdiagnosis partly due to the resemblance with the other pathologies.

Background: Cancer is one of the leading causes of death due to noncommunicable diseases worldwide. Despite increasing public awareness and availability of sophisticated imaging techniques, some cancers evade clinical diagnosis and/or are incidentally encountered at autopsies, often with dissemination. Aims: The present study evaluated the disseminated epithelial cancers at autopsy. Materials and Methods: This is a retrospective observational 5-year autopsy analysis of disseminated epithelial cancers performed at a tertiary-care hospital. The cases were categorized as (1) clinically diagnosed malignancy, known primary; (2) clinically diagnosed malignancy, unknown primary; and (3) clinically undiagnosed malignancy. Statistical Analysis: Nil. Results: Dissemination was identified in 66 (57.9%) of the 114 patients with epithelial malignancies. There were 29 patients (43.9%) in category 1, 26 patients (39.4%) in category 2, and 11 patients (16.7%) in category 3, majority of whom were women (38 patients, 57.6%). When all categories were considered together, lung and colorectal carcinomas were the commonest cancers seen in 13 (19.7%) and 8 (12.1%) patients, respectively, in both men and women. Majority of the patients (43 cases, 65.2%) had symptoms produced by metastases, which were the sole manifestations in 13 patients (19.7%). Lungs and liver were the common metastatic sites. Conclusions: Cancerous dissemination continues to be a major cause of morbidity and mortality even after considerable improvements in the surgical or nonsurgical treatment modalities. An autopsy study can provide important clinical insights in retrospect.

Background: Myasthenia gravis (MG) is a prototypic T-cell-dependent antibody-mediated autoimmune disease that leads to ocular or generalized muscular weakness. The disease is most commonly caused by antibodies to the acetylcholine receptors, often with underlying thymic pathology. Aims: This study is aimed at analyzing the pathological spectrum of the excised thymuses in patients with myasthenia. Materials and Methods: This was a retrospective 10-year study of 68 thymectomy specimens performed as a part of the treatment of patients with MG. Statistical Analysis: Nil. Results: There were 47 males and 21 females (male to female ratio of 2.2:1) with a mean age of 41 years. Only three patients presented with ocular myasthenia. The thymus was normal in 9 patients (13.2%) and atrophic in 17 patients (25%). Follicular hyperplasia and thymomas were seen in 6 and 36 patients, respectively. Conclusion: The thymectomies performed in patients of MG had a fairly variable spectrum on histology; the thymic tumors were predominantly of the cortical phenotype.

‘Black esophagus’or acute esophageal necrosis is a rare condition, frequently affecting the distal esophagus with a sharp transition to normal-appearing mucosa at the gastroesophageal junction. It is characterized by circumferential blackish discoloration and necrosisof the mucosa due to severe hemodynamic compromise.The pathogenesis of black esophagus is unclear, but a “two-hit” hypothesis for a dominant mucosal necrosis has been described - a low-flow vascular setting followed by an altered reparative ability of the mucosal barrier systems. The etiology is usually multifactorial, triggered by an acute event. It is associated with a high mortality rate of 31.8%−50%, most of the deaths caused by underlying illnesses. Mortality specific to acute esophageal necrosis is approximately 6%.We report a case of 72-year-old hypertensive male patient presenting with perforated gastric ulcers and sepsis. Distal gastrectomy revealed two candidial perforations. Patient expired on second post- operative day. Autopsy revealed a diffuse circumferential blackish discoloration of distal one third of the esophageal mucosa abruptly stopping at the gastro esophageal junction. On microscopy, the esophageal mucosa was ulcerated, and covered by necro-inflammatory exudate and blackish pigment extending upto the muscularis propria. Many submucosal vessels showed fresh fibrin thrombi. Special stains for fungus were negative. The diagnosis of black esophagus or acute esophageal necrosis was made. Other findings included fungal peritonitis, ischemic heart disease and chronic pyelonephritis with right-sided nephrolithiasis. The cause of death was related to septicaemia.

Background: India accounts for the highest number of TB cases globally (almost one-fifth of the global burden and almost two-thirds of the cases in South East Asia. Furthermore, the development of drug resistance of varying levels such as multi-drug resistant TB (MDR-TB), extensively-drug resistance TB (XDR-TB) and total-drug resistant TB (TDR-TB) has been on the increase, and now India also features in the 27 high-MDRTB-burden countries. Almost parallel to these developments, in the last few years, we have been encountering less common morphological forms of pulmonary TB (PTB) at autopsies. With these less common manifestations of the disease, we undertook this study to examine the changing trends in the morphological pattern of pulmonary TB over the recent years. Methods: In this 3-year retrospective study, adult autopsy cases of PTB (that significantly contributed to the final cause of death) were studied in detail. HIV-positive cases were excluded from the study. The clinical details, gross appearances of the pulmonary lesions, microscopic pattern and Ziehl-Neelsen (ZN) staining were studied. Extrapulmonary involvement and causes of death were documented. Results: Pulmonary tuberculosis as a cause of death at autopsy was seen in 130 adult patients over 3 years. The age range was between 12 to 70 years. Anti-tuberculous therapy had been administered in 33 of them, but only one patient had taken complete therapy. Dyspnea was the commonest respiratory symptom seen in 51 cases (39.2%). Tuberculous bronchopneumonia was the commonest lesion (45.3%), miliary lesions (including localized miliary) accounted for 26% while fibrocavitary lesions (including the ones not involving apex) were seen in 13% cases. Other morphologies included nodular forms of TB (13%), localized miliary lesions (11.9%), and fibrocavitary lesions, not necessarily involving the apex (11.7% of all fibrocavitary cases), and predominant pleuritis with underlying lung involvement by TB in 1 case. Many cases of TB bronchopneumonia had a bronchocentric pattern of distribution (14.7%). On microscopy, caseating granulomas were seen in 93% cases, only caseation necrosis was seen in 4.6% cases, and necrotizing granulomas with abscess-like reaction in 11.5% cases. ZN staining was positive in 92 cases (70.7%). All the extrapulmonary lesions showed caseating granulomas histologically. The final cause of death was found to be primarily tuberculous in 106 cases (81.5%), whereas in 24 cases (19.5%) pulmonary TB was attributed to the secondary cause of death. Conclusion: The typical apical involvement of secondary TB was not seen in most of our cases. This could indicate a difference in the morphology and the pattern of lung involvement in recent years. The difference in gross morphology does not affect the pattern of involvement of the lung. In our study, we have observed both; a change in morphology, i.e., more cases of TB bronchopneumonia, and a change in the pattern of involvement like nodular forms, localized miliary forms, and fibrocavitary lesions not necessarily involving the apex. We postulate that this less common manifestation of pulmonary TB is closely related to the development of multi-drug and microbial resistance posing serious medical challenges.

Sudden death, a catastrophic event, falls within the purview of the forensic experts. It is often caused by cardiovascular diseases, which may be evident or occult. A vast majority of sudden cardiac deaths (to the extent of 90%) are due to ischemia of the working or conducting myocardial tissues consequent to coronary artery diseases. A heterogeneous group of nonischemic myocardial disorders, most producing structural abnormalities are responsible for the remainder; they predominantly represent various cardiomyopathies. This review, in two parts, covers sudden cardiac death in medicolegal autopsies with an approach to some common and uncommon nonischemic myocardial diseases that have a genetic and/or nongenetic basis.

Sudden death, a catastrophic event, falls within the purview of the forensic experts. It is often caused by cardiovascular diseases, which may be evident or occult. A vast majority of sudden cardiac deaths (to the extent of 90%) are due to ischemia of the working or conducting myocardial tissues consequent to coronary artery diseases. A heterogeneous group of nonischemic myocardial disorders, most producing structural abnormalities are responsible for the remainder; they predominantly represent various cardiomyopathies. This review, in two parts, covers sudden cardiac death in medicolegal autopsies with an approach to some common and uncommon nonischemic myocardial diseases that have a genetic and/or nongenetic basis.

Objective: The objective of this study is to determine the frequency, location, and delineation of the echogenicity of intracardiac echogenic foci (ICEF) and to ponder the possible etiology of ICEF. Materials and Methods: Retrospective study of 2445 referred cases in second and third trimester for fetal cardiac ultrasound scan from 2010 to 2020, targeted for ruling out congenital heart disease were evaluated and analyzed in detail for ICEF. Further postnatal follow-up was done in 64 cases of ICEF till 7 years of age to see the natural course. Histopathology was performed from one case of fetal autopsy at 19 weeks of gestation for further correlation. Results: Among the fetal cardiac ultrasound cases, 323 (13.2%) showed ICEF (95% confidence interval 11.9–14.6). Majority of these ICEF (96%) were in the left ventricular cavity, and then both ventricles (3%) and remaining in right ventricle and all were located within the papillary muscles. On follow-up of 66 postnatal cases, ICEF had resolved in 97% by 5 years of age. Only two cases reported to have congenital heart disease. No chromosomal anomalies was associated in any case. Histopathology of fetal heart specimen in one case revealed myxomatous degeneration at the apices of the papillary muscle. Conclusion: Frequency of single ICEF is much higher in the left ventricle, and it is invariably located within the upper third of the anterior papillary muscle in such cases. As the gestational age advances, ICEF can resolve in utero, and further complete resolution is seen by 1–7 years of postnatal life. Variability in the echogenicity could be part of transformation of myxomatous tissues to fibrosis or microcalcification. In this study, ICEF have no particular association with congenital heart disease or chromosomal association.

The entire world is under a devastating pandemic caused by COVID-19 with a high mortality rate. Knowledge of the viral structure, factors that help in its progression and spread, pathological findings, diagnostic methods and, treatment modalities helps in understanding the viral disease and also in treating the patients in a better way besides preventing the community spread of this deadly infection. The causative agent is a single- stranded RNA virus. The clinical spectrum varies in symptomatic and asymptomatic patients, who later become potential silent carriers, thus unknowingly spreading the virus. The virus constantly undergoes recombination, with reports of cross-species infections. Studies have indicated a strong immunological basis of COVID-19 infection. Not only does it weaken the immune system causing multi-organ involvement but also helps in its progression and spread to others.Multiple organs especially lungs, heart, kidney, gastrointestinal and hepatic system, brain and skin are affected varying in their severity. Similarly, persons with associated co-morbidities are likely to be affected more in terms of the number as well as in the severity. Real- time reverse transcription polymerase chain reaction confirms the presence of COVID-19 infection. Serological diagnosis helps in diagnosing an ongoing outbreak or retrospective infection. Furthermore, it also identifies individuals who have been infected or have recovered from the disease especially the asymptomatic. This helps in the development of an effective vaccine indicating the status of herd immunity in the community. Different treatment modalities are being tried and under trial. This review article thus highlights the global epidemiological status, characteristic of the virus, symptomatology of the patients, role of diagnostic tests available, organs affected including their morphological changes and the latest line of treatment of COVID-19.

Acute aortic syndromes comprise a heterogeneous group of life-threatening disorders, characterized by acute-onset chest or back pain with or without cardiovascular collapse. The major entities include acute aortic dissection, penetrating atherosclerotic ulcer, and intramural hematoma. Spontaneous rupture of the aorta is an additional, rare cause of this syndrome, which frequently leads to sudden cardiac death. This is a report of two cases of this uncommon entity with detailed pathological analysis.

Background: Fistulous tracts within the thorax are best exemplified aorto-esophageal fistulas (AEFs), which are rare exsanguinations of the upper gastrointestinal tract. Most of them are diagnosed at autopsy. Aim: This is an autopsy-based study of fatal cases of AEFs gathered over a period of 20 years. Materials and Methods: Cases of AEFs were retrieved from the autopsy records of the cardiovascular pathology sub-specialty at a tertiary care center and were studied with reference to demographic details, modes of clinical presentation, and pathological features. Results: Ten cases of AEFs (10 males and 8 females, mean age of 46.9 years) were identified in a span of 20 years. A clinical diagnosis of AEF was made in only one patient, while the rest were identified at autopsy. All the patients had a bout of massive and fatal hematemesis. The fistulous connections, involving the middle-third of the esophagus, were caused by a fishbone, esophageal ulceration and aneurismal aortic diseases of varied etiology. Conclusions: AEFs are often missed or misdiagnosed. Early recognition of this rapidly fatal condition is essential as these fistulas require quick and aggressive management.

Atherosclerotic coronary artery disease (CAD) is of great concern in young adults because of its potential to cause great incapacitation. This arena of cardiology has gained importance in South Asian countries, particularly India due to increased prevalence that is related to traditional risk factors, altered life styles and inherent risk factors. In this study, we sought to evaluate, at autopsy, the pathology of atherosclerotic CAD in young patients with ischemic heart disease (IHD). A 10-year retrospective autopsy-based study was carried out in a large tertiary-care centre and patients aged ≤45 years with IHD were selected. Out of 545 autopsied cases of IHD, 95 patients (17.4%) were young. Among these 95 patients, 84 (88.4%) had IHD related to atherosclerotic CAD; the youngest patient was 18 years old. Predictably there was sole involvement of left anterior descending artery and the presence of fibrous plaques. Irrespective of the plaque morphology, the commonest complication was thrombosis produced by plaque erosion seen in 36.9% of patients. Acute coronary insufficiency was noted in 52 patients (61.9%), while healed infarctions were surprisingly noted in 28 patients (33.3%). Screening for IHD in the young population may help to improve prognosis by detecting subclinical disease, although more studies are necessary to establish reference limits for this young population. Additional research must also focus on treatment concerns that are specific to young patients.

Congenital aortic anomalies are uncommon causes of secondary hypertension and are seldom suspected in the adult age group. We present a case of aortic interruption unexpectedly diagnosed on autopsy in a 38-year-old male who presented with cardiovascular collapse. Apart from interruption, a finding unique to our case was aneurysmal dilation of the proximal descending aorta just before the obstruction with thrombosis. We also attempt to review the literature for interrupted aortic arch in adults and clarify the nomenclature of interruption versus coarctation.

Epstein–Barr virus (EBV) promotes the development of undifferentiated carcinomas of the upper aerodigestive tract and different types of lymphomas. This ability of tumorigenesis is heightened in many immunocompromised patients who have an increased incidence of lymphoproliferative disorders. The virus also induces smooth muscle proliferation, and those occurring following transplantation are designated as EBV-associated post-transplant smooth muscle tumors. We report multifocal miliary-sized leiomyomas in the lungs in a renal transplant recipient as an incidental finding.

The mitral valve disease (MVD) in Lutembacher's syndrome has been infrequently analyzed from a pathological standpoint. In this study, we have attempted to elucidate the pathology of MVD in this interesting syndrome in 44 autopsied cases of combined non-primum atrial septal defect (ASD) and MVD collected over 16 years. The patients were divided into 3 groups: Group 1: non-primum ASD with clinically diagnosed mitral stenosis (MS) ± regurgitation, Group 2: non-primum ASD with clinically diagnosed mitral regurgitation (MR) and, Group 3: non-primum ASD with no clinically evident MVD, but with mitral valve pathology diagnosed at autopsy. All 44 patients were symptomatic. There were 26 males (59%). The ages ranged from 13 to 73 years. A history of rheumatic fever was available in 2 patients while 16 patients had undergone surgery or intervention for the disease. Of the 18 patients in Group 1, six patients did not show histological features of rheumatic heart disease, although they shared similar gross morphological features. Furthermore, the mitral regurgitation in 12 of 19 patients in Group 2 was non-rheumatic. Also, only one patient had histological evidence of rheumatic activity among seven cases in Group 3. In spite of a high rheumatic load at our center, more than half (54.5%) of patients had “non-rheumatic” mitral valve pathology. Thus, the mitral valvular lesions commonly labeled ‘rheumatic’ in Lutembacher's syndrome are not always so. The distinction into rheumatic and non-rheumatic MVD in non-primum ASD has to be made on the basis of microscopic criteria.

Thyroid dysfunctions, particularly hypothyroidism, predispose to cardiovascular diseases, such as atherosclerotic coronary artery disease. However, thyroid functions are not always assessed in such patients. In this report, we studied the cases with pathologically evident ischemic heart disease (IHD) at autopsy and correlated with the absence or presence of thyroid disease. In a 5-year retrospective autopsy-based study, cases with IHD in whom the thyroid gland was dissected at autopsy were analyzed. The thyroid gland was available for assessment in 181 of the 255 autopsied cases with IHD. The majority were males with a mean age of 59.9 years. Thyroid pathology was noted in 58 patients (32.0%) in the form of uninodular or multinodular goiter (56.9%), lymphocytic thyroiditis (37.9%), and Hashimoto's thyroiditis (5.2%). Based on our experience, we feel it may be worthwhile to assess the thyroid by simple inspection and palpation followed by ultrasonographic imaging and estimations of hormones, thyroid-stimulating hormone and antithyroidal antibodies, in all patients presenting with IHD. Keywords: Ischemic heart disease, Thyroid, Hypothyroidism, Autopsy

A child presented with an acute febrile illness associated with neurological symptoms. The differential diagnoses of such a presentation with effects of prolonged hospitalization is discussed.

Rheumatic heart disease, particularly rheumatic mitral stenosis and hemoglobinopathies, especially HbS can pose a problem in pregnancy. Though both these conditions have a high incidence and considerable geographic overlap in India, very few studies have reported these diseases occurring concurrently. This is a report of concurrent recently diagnosed rheumatic mitral stenosis and undiagnosed sickling hemoglobinopathy in a post-partum woman.

Background: Balloon mitral valvotomy (BMV) is a well-established therapeutic modality for rheumatic mitral stenosis (RMS). However, there are chances of procedural failure and the more ominous post-procedural severe mitral regurgitation. There are only a few prospective studies, which have evaluated the pathogenic mechanisms for these major complications of BMV, especially in relation to the subvalvular apparatus (SVA) pathology. Methods: All symptomatic patients of RMS suitable for BMV by echocardiographic criteria in a span of 1 year were selected. In addition to the standard echocardiographic assessment of RMS (Wilkins score and score by Padial et al.), a separate grading and scoring system was assigned to evaluate the severity of the SVA pathology. The SVA score was ‘I’, when none of the two SVAs had severe disease, ‘II’ when one of the two SVAs has severe disease, and ‘III’ when both SVAs had severe disease. With these scoring systems, the outcomes of BMV (successful procedure, failure, and post-procedural mitral regurgitation) were analyzed. Emergency valve replacement was performed depending on clinical situation, and in cases of replacement, the pathology of the excised mitral valves were compared with echocardiographic findings. Results: Of the 356 BMVs performed in a year, 43 patients had adverse outcomes in the form of failed procedure (14 patients) and mitral regurgitation (29 patients). Forty-one among these had a SVA score of III. The sensitivity and specificity of the MR score was lesser than the SVA score (sensitivity 0.34 vs. 1.00, specificity 0.92 vs. 0.99, respectively). The mitral valvular morphology in 39 patients who underwent post-procedural valve replacements correlated well with echocardiography findings. Conclusion: It is important to assess the degree of SVA pathology in the conventional echocardiographic assessment for RMS, as BMV would have adverse events when both SVAs were severely diseased.

Sudden cardiac death (SCD) most commonly results from previously undiagnosed congenital, acquired, or hereditary cardiac diseases. Congenital aortic valvular, subvalvular, and supravalvular disease with left ventricular outflow tract obstruction is an important preventable cause of sudden death. This report documents sudden death presumably due to acute myocardial ischemia in a young male with an undiagnosed supravalvular aortic stenosis (SVAS) due to a rare association of isolation of coronary sinuses of Valsalva. Congenital supravalvular pulmonary stenosis and mitral valvular dysplasia were also present.

Takayasu arteritis (TA) is a chronic idiopathic and granulomatous vasculitis, manifesting mainly as a panaortitis. Autoimmune cell-mediated immunity is probably responsible for the disease. The inflammation commences from the adventitia and progresses to the intima and leads to, both in adults and children, segmental stenosis, occlusion, dilatation, and/or aneurysm formation. This review focuses briefly on the etiopathogenesis, and describes the pathological and clinical features in adults and children.

Most of the pulmonary arterial sarcomas arise from multi-potential mesenchymal intimal cells and are designated as intimal sarcomas. These tumors grow in the direction of blood flow into peripheral arteries producing clinical features mimicking pulmonary thromboembolism. Retrograde extension is rare. We report one such case of intimal sarcoma that had a retrograde extension into the right ventricular outflow tract, and review such a presentation in the last ten years.

Context: Hospital-acquired infections are frequently encountered by the physicians for ailments demanding prolonged hospitalization, especially in intensive care units, where patients are often mechanically ventilated. The organisms most often implicated are bacteria; viral etiology is infrequent. Aims: The study aims at reviewing lung pathology at autopsy in mechanically ventilated children admitted in pediatric intensive care unit (PICU) to assess the incidence of viral ventilator-associated pneumonias (VAP). Setting and Design: Retrospective analysis. Materials and Methods: Among the 275 children who had been autopsied, 13 who had been admitted in the PICU satisfied the criteria for VAP. These cases were analyzed on the basis of clinical data and pulmonary pathology. Depending on the overall histology, the cases were classified as being viral or bacterial in etiology. Immunohistochemistry (IHC) for detection of viral antigens was also performed. Results: Of the 13 children, nine (five males and four females) had shown the histomorphologic features, suggesting viral inflammation. The mean age was 33 months. Falling oxygen saturation and increasing respiratory distress had necessitated ventilator support. Acute lymphocytic bronchiolitis, interstitial pneumonitis, diffuse alveolar damage, and necrotizing pneumonia were the histological features. The viruses identified in five patients were adenovirus, respiratory syncytial virus and cytomegalovirus. Conclusion: This communication, though not representing the true incidence, emphasizes that a proportion of nosocomial infections is due to viral infections. This should alert the treating intensivists to actively pursue investigations to confirm viral etiology.

Wilms′ tumor (WT) has a strong propensity to invade the vasculature in the form of tumor-thrombus, into the renal veins, and inferior vena cava and even into the right atrium. This cavo-atrial propagation does not alter the prognosis and pre-operative chemotherapy produces shrinkage to the extent of even disappearance of caval or atrial extensions. We present a case of sudden death due to hemorrhagic expansion of the intra-atrial component of WT, immediately after commencement of chemotherapy, an uncommon incident.

Intravenous leiomyomatosis is an example of unusual growth patterns that is sometimes exhibited by uterine leiomyomas, characterized by smooth muscle proliferations within the uterine or pelvic veins. Extension into the heart via the inferior vena cava is even rarer and is designated as intracardiac leiomyomatosis. This is a report of two such cases that had preoperative diagnoses of a thrombus and right atrial myxoma, respectively.

Context: Thymectomy is an effective therapy for patients with myasthenia gravis, and the lesions usually identified are lymphoid hyperplasia and thymomas. Nodular epithelial proliferations, termed as microscopic thymoma, are rare. Aims: Our aim was to assess the incidence and clinicopathological features of microscopic thymomas. Setting and Design: Retrospective analyses. Materials and Methods: Thymectomies performed in 105 patients with myasthenia gravis were reviewed, and patients with microscopic thymomas were studied with respect to their clinicopathological features. These were compared with those in other reports in literature. Results: Four thymuses showed features of microscopic thymomas, an incidence of 3.8%. There were 2 males and 2 females, all myasthenic; the mean age was 31 years. The resected thymic tissues were seen as soft, capsulated adipose tissue. They showed variable degrees of involution or were atrophic. All had multifocal proliferation of ovoid-to-polygonal cells with tongue-like insinuations into the fat. After thymectomy, there was improvement in the symptoms. Eight patients reported in literature had similar clinicopathological features. Conclusions: Thorough examination of the thymectomies is required to increase the detection of these lesions for assessment of their incidence, causes, pathogenesis and functional significance.