Dysplastic Pulmonary Valve Infective Endocarditis With an Atrial Septal Defect-A Black Swan Event: When Rarity Brings Clarity.

ABSTRACT: A case of infective endocarditis of a dysplastic pulmonic valve along with atrial septal defect has been described. Right-sided endocarditis has now increased in frequency because of cardiac invasive procedures and intravenous drug abuse. Although the tricuspid valve usually bears the brunt of right-sided endocarditis, there have now been increasing reports of pulmonary valve infective endocarditis.

An Autopsy Study of Cerebrovascular Atherosclerosis in the Elderly.

Background: Stroke is an important cause of mortality with intracranial atherosclerosis as an important risk factor. Geographical variation in the pattern of atherosclerosis is well documented with various studies in stroke patients showing that African Americans and Japanese people tend to have intracranial vascular occlusion, whereas Caucasians more often have extracranial atherosclerotic lesions. Methods: This is a prospective study of 50 cases of Indian elderly patients over 60 years of age where we have studied the CNS vasculature in detail including intracranial atherosclerosis with regards to its incidence, severity, distribution, plaque composition and associated risk factors. We compared the atherosclerotic pathology in the intracranial vessels and the extracranial vessels including the carotids. Results: Out of 50 cases 31 showed intracranial atherosclerosis. Anterior circulation was more commonly affected. A total of 87 of the intracranial arteries showed atherosclerotic plaques, most common nature of the plaques being fibrofatty. Majority of the intracranial vessels showed mild stenosis and multiple vessels were involved in 15 cases while single vessel involvement was seen in 16 cases with the middle cerebral artery being the most common single vessel involvement. Infarcts were seen in 7 cases and all these cases had history of hypertension. Cerebral atrophy was seen in 28 cases. All cases showing frontoparietal and generalized atrophy had carotid artery stenosis. Of the 100 carotid arteries studied (2 per case) 59 showed atherosclerosis. Majority of the carotid arteries showed mild degree of atherosclerosis and the content was predominantly fatty. Conclusion: This study concludes that the pattern of atherosclerosis is different in the Indian population as compared to the western literature. Intracranial atherosclerosis is more common in Indian population with the anterior portion of circle of Willis being more commonly affected.

Acute respiratory distress syndrome: A study of autopsy findings.

CONTEXT: In this autopsy study, the various morphological patterns of acute respiratory distress syndrome (ARDS) have been analyzed and compared along with their etiopathogenesis. AIMS: We aimed to study the prevalence and clinicopathological correlation of ARDS based on age, gender, hospital stay, symptoms, clinical diagnosis, gross, and microscopy findings. SUBJECTS AND METHODS: Total 130 cases of ARDS were studied over a period of 5 years. Age, gender, hospital stay duration, symptoms, clinical diagnosis, gross and microscopic lung finding, clinicopathological correlation, and cause of death were documented and analyzed. Special stains were done whenever required. STATISTICAL ANALYSIS: This is an observational study, and simple statistics such as mean, median, and standard deviation have been used for continuous variables. RESULTS: The prevalence of ARDS among the adult autopsy was 6.05%. Majority of the cases were in the age group of 18-30 years (36.9%), with a male: female ratio of 1.7:1. Chief complaints were fever (71%), breathlessness (54.6%), and chills (43.8%). The main clinical diagnoses were ARDS (41.6%), sepsis (28.3%), acute febrile illness (17%), and lower respiratory tract infection (12.5%). Most of the patients had a hospital stay of <1 day. Associated conditions mostly included chronic alcoholism (16.1%), pregnancy (16.1%), and chronic smoking (10.7%). Major findings on gross examination were intrapulmonary hemorrhage (38.5%), ARDS (33%), pulmonary edema (13%), and pneumonia (15.3%).On microscopy, major findings were hyaline membrane (84.6%), intrapulmonary hemorrhage (76.1%), pulmonary edema (75.3%), organizing fibrin (55.3%), and bronchopneumonia (36.2%). CONCLUSION: Infections were one of the major predisposing causes of ARDS. Due to the short interval, the underlying cause for ARDS often goes undiagnosed.

Bednar Tumor: An Uncommon Entity.

Bednar tumor is an uncommon variant of dermatofibrosarcoma protuberans. Also known as pigmented dermatofibrosarcoma protuberans, this tumor is of intermediate grade. It is seen in adults and has a predisposition to affect the shoulder region. We report a rare case of Bednar tumor in a 40-year-old female patient. The diagnosis of Bednar tumor must be considered while reporting pigmented subcutaneous spindle cell lesions.

Miliary Adenocarcinoma of the Lung with Lepidic Pattern: A Morphological Conundrum. Infiltrating bilateral pulmonary miliary adenocarcinoma with predominantly lepidic pattern.

Miliary multi-focal bilateral pulmonary adenocarcinoma with lepidic predominant pattern is an unusual morphological presentation and is seen in females who are non-smokers. This gross morphology can be confused with infectious diseases, like tuberculosis, histoplasmosis or with pulmonary metastasis.

Adenoid cystic carcinoma of the trachea.

Primary salivary gland-type tumors of the lung and airways being unusual, they pose a diagnostic challenge on small biopsies and are usually consigned as non-small cell lung carcinomas. Since the clinical behavior of these tumors is different from the conventional lung tumors, it is important to accurately diagnose them. Among this category of tumors, adenoid cystic carcinoma (ACC) of the lungs and airways is exceedingly rare. Few large studies and maximum case reports have been reported in literature on ACC of the lungs and trachea so far. We herein report a rare case of primary ACC of the trachea diagnosed on bronchoscopic biopsy.

Idiopathic mitral valve prolapse with tricuspid, aortic and pulmonary valve involvement: an autopsy case report.

Mitral valve prolapse (MVP) is usually asymptomatic, but can be associated with complications such as infective endocarditis, mitral regurgitation, thromboembolism and sudden cardiac death. It has been very rarely reported to occur in association with other valvular involvement. A 55-year-old male patient was brought dead and at autopsy the mitral valve orifice was stenotic and the leaflets were enlarged, myxoid and bulging suggestive of MVP and chordae tendinae were thickened, stretched and elongated. Similar changes were seen in the tricuspid valve. The pulmonary and aortic valves also showed myxomatous degeneration of their cusps. Myxomatous degeneration is the most common cause of MVP and it can be associated with involvement of the other valves. Concomitant involvement of the aortic valve has been reported, however it is very rare and simultaneous involvement of the pulmonary valve has not been reported in the literature so far. We report a case of MVP associated with myxomatous degeneration of the tricuspid, pulmonary and aortic valves.

Pulmonary tumor thrombotic microangiopathy in an unknown primary cancer.

Pulmonary tumor thrombotic microangiopathy (PTTM) is a highly fatal complication of cancer leading to acute cor pulmonale and pulmonary hypertension. We present a case of 47-year-old male patient who developed acute breathlessness and died suddenly. The pulmonary vessels at autopsy on histopathologic examination showed the presence of fibrocellular intimal proliferation, fibrin thrombi and few tumor emboli consisting of malignant adenocarcinoma cells. There was associated lymphangiosis carcinomatosis. No primary visceral tumor was found despite extensive search. The patient had died following acute cor pulmonale with sudden pulmonary hypertension due to PTTM. This entity (PTTM) must be kept as a differential diagnosis in patients presenting with acute breathlessness especially in cases of cancers.

Contained cardiac rupture: an autopsy case.

Rupture of the myocardium due to myocardial infarction is often fatal but when such patients survive, they present with a pseudoaneurysm where the defect is sealed by the pericardium preventing the complete rupture. This is described as a 'contained myocardial rupture'. We describe here a case of left ventricular contained myocardial rupture following an acute myocardial infarction.

Evolution of antiphospholipid antibody syndrome.

Antiphospholipid antibody syndrome is a very important cause of cerebral infarction, myocardial infarction, and repeated pregnancy losses in women. We present an extremely rare case of a 44-year-old man with antiphospholipid syndrome who collapsed and died suddenly. At autopsy, he was found to have both cerebral and myocardial infarction. In all young patients with cerebral infarction, myocardial infarction, pulmonary embolism, recurrent miscarriages, and unexplained low platelet count, one must consider the strong possibility of antiphospholipid antibody syndrome.

Cardiac findings in leptospirosis.

BACKGROUND/AIM: In leptospirosis, although cardiac involvement in the form of ECG changes and myocarditis is known, it is not considered to be significant. This study analysed cardiac changes in leptospirosis. METHODS: Twenty-four hearts from patients who had died from leptospirosis were studied. Detailed gross and light microscopic examination was carried out. RESULTS: Myocarditis was noted in 96% of cases. Endocardial inflammation was seen in 50% of cases. This endocardial inflammation correlates with vasculitis, which is the principal pathogenetic mechanism of the disease. CONCLUSIONS: There is definite cardiac involvement in leptospirosis, which even though not symptomatically evident, may add to the morbidity or be contributory to the mortality associated with the disease. In addition, a possibility of dilated cardiomyopathy as a delayed consequence of severe myocarditis remains, and may need evaluation.

Cardiac rhabdomyoma.

Primary tumors of the heart are exceedingly rare with a prevalence rate of around 0.01% in autopsy studies. A majority of primary cardiac tumors are benign. Rhabdomyomas are the most common of benign pediatric cardiac tumors. A newborn female infant presented with perioral cyanosis and respiratory distress. She deteriorated progressively. The infant expired, and at autopsy, dissection of the heart revealed a large tumor mass in the interventricular septum. Histologically, the mass showed the classic "spider cells," and a diagnosis of cardiac rhabdomyoma of childhood was established.

Mucinous adenocarcinoma of the renal pelvis with pseudomyxoma peritonei.

Mucinous adenocarcinoma of the renal pelvis is an extremely rare tumor with very few case reports in literature. Pseudomyxoma peritonei is an uncommon condition characterized by the presence of mucinous gelatinous material in the peritoneal cavity. It occurs secondary to primary mucinous neoplasms of particularly the appendix and the ovary. We present a case of a 35-year-old female who had a history of dull aching pain in the right flank since one and a half years. Upon ultrasonography (USG) and computerized tomography (CT) scan, there was a large cystic mass measuring 15x15x12 cm, extending into the right lumbar region. Grossly, the entire kidney was converted into a cystic mass measuring 15x15x12 cm containing gelatinous mucinous material weighing 1 kg. Histologically, the tumor was composed of simple and complex glandular acini together with a superficial resemblance to colonic mucosa with abundant extracellular mucin. Thus, a diagnosis of mucinous adenocarcinoma of the renal pelvis leading to pseudomyxoma peritonei was made.

Papillary fibroelastoma of the heart.

Primary intra-cardiac tumors are rare. Most of them are benign. Of the benign tumors, myxomas are the most common. Others are lipoma, rhabdomyoma, hemangioma, and papillary fibroelastoma (PFE). PFE is a relatively rare benign tumor of the heart. It occurs commonly on cardiac valves and is often an incidental finding. They are most commonly discovered during autopsy, but may present with thromboembolism, which is a dreaded complication. It is important to be aware of this entity because even though it is benign it may present with life-threatening complications, which are well documented in literature. Surgery is the treatment of choice for these tumors. We present the case of a 30-year-old male in whom PFE was an incidental finding.

Infective endocarditis of bicuspid pulmonary valve.

Infective endocarditis of the cardiac valves mainly involves the aortic mitral valves and less frequently the tricuspid valve. Isolated pulmonary valve involvement is extremely rare with few cases reported in literature. We present a case of a 34-year-old female who presented with sudden onset breathlessness and cardiac failure. A complete autopsy showed an uncommon congenital bicuspid pulmonary valve, which was also affected by infective endocarditis.

Cardiac angiosarcoma.

Primary tumors of the heart are extremely rare, with a prevalence rate of around 0.01% in collective autopsy studies. Majority of the primary cardiac tumors are benign. Myxoma is the most common primary cardiac tumor, while angiosarcoma is the commonest primary malignant tumor. We present a case report of a 20-year-old patient with history of breathlessness and hemoptysis. A malignant mass was seen arising in the right atrium, with pericardial effusion and multiple metastases in the lung. A complete autopsy was performed and a diagnosis of cardiac angiosarcoma was confirmed on histology and immunohistochemistry.

Bilateral axillary apocrine nevi--a case report.

Apocrine glands are tubular glands, which function as scent glands. These are found at only a few sites like the axillae and anogenital region. They are present as modified glands in the external ear canal (ceruminous glands), in the eyelid (Moll's glands) and in the breast (mammary glands). Occasionally, apocrine glands are found on the face, in the scalp and on the abdomen where they are usually small and non-functional. Apocrine glands develop their secretory portion and become functional only at puberty. Tumours of these glands involving only apocrine tissue are very rare. We present an uncommon case of benign apocrine nevi of the axilla, which occurred bilaterally. These nevi are uncommon and only occasional reports are available in literature.