Objective: The aim of this study was to report on sudden cardiac death (SCD) during or immediatel... more Objective: The aim of this study was to report on sudden cardiac death (SCD) during or immediately after a stressful event in a predominately young cohort. Methods: This study used retrospective non-case-controlled analysis. A total of 110 cases of SCD in relation to a stressful event such as altercation (45%), physical restraint (31%) in police custody (10%), exams/school/job stress (7.27%), receiving bad news (4%), or a car accident without injuries (2.73%) were retrospectively investigated. The majority of the subjects experiencing SCD were male (80.91%). The mean age was 36 AE 16 years (range 5–82 years). Twenty-three cases (20.91%) were psychiatric patients on antipsychotic medication. Results: Fifty-three per cent of cases died with a negative autopsy and a morphologically normal heart, indicating sudden adult death which is linked to cardiac channelopathies predisposing to stress-induced SCD. Cardiomyopathy was found in 16 (14.5%) patients and coronary artery pathology in 19 (17%) patients, with atherosclerosis predominating in older patients. Conclusions: This study highlights SCD during psychological stress, mostly in young males where the sudden death occurred in the absence of structural heart disease. This may reflect the proarrhythmic potential of high catecholamines on the structurally normal heart in those genetically predisposed because of cardiac channelopathy. Structural cardio-myopathies and coronary artery disease also feature prominently. Cases of SCD associated with altercation and restraint receive mass media attention especially when police/other governmental bodies are involved. This study highlights the rare but important risk of SCD associated with psychological stress and restraint in morphologically normal hearts and the importance of an expert cardiac opinion where prolonged criminal investigations and medico-legal issues often ensue.
A retained surgical swab (gossypiboma) is a rare but important complication of intrathoracic surg... more A retained surgical swab (gossypiboma) is a rare but important complication of intrathoracic surgery. The radiographic and computed tomography (CT) appearances are variable and depend on the chronicity and site of the swab within the chest. Two cases of retained swabs within the chest are reported. In both cases, the swab had become surrounded by lung. The swab within the pleural space acted as a nidus and resulted in infolding of the lung, superficially resembling an intrapulmonary abscess on CT.
Congenital coronary artery anomalies are of major significance in clinical cardiology and cardiac... more Congenital coronary artery anomalies are of major significance in clinical cardiology and cardiac surgery due to their association with myocardial ischaemia and sudden death. Such anomalies are detectable by imaging modalities and, according to various definitions, their prevalence ranges from 0.21 to 5.79%. This consensus document from the Development, Anatomy and Pathology Working Group of the European Society of Cardiology aims to provide: (i) a definition of normality that refers to essential anatomical and embryological features of coronary vessels, based on the integrated analysis of studies of normal and abnormal coronary embryogenesis and pathophysiology; (ii) an animal model-based systematic survey of the molecular and cellular mechanisms that regulate coronary blood vessel development; (iii) an organization of the wide spectrum of coronary artery anomalies, according to a comprehensive anatomical and embryological classification scheme; (iv) current knowledge of the pathophysiological mechanisms underlying symptoms and signs of coronary artery anomalies, with diagnostic and therapeutic implications. This document identifies the mosaic-like embryonic development of the coronary vascular system, as coronary cell types differentiate from multiple cell sources through an intricate network of molecular signals and haemodynamic cues, as the necessary framework for understanding the complex spectrum of coronary artery anomalies observed in human patients.
... To my family, Rachel, Nicky and Madeline, to whom I owe ... Special thanks go to Bob Kutys an... more ... To my family, Rachel, Nicky and Madeline, to whom I owe ... Special thanks go to Bob Kutys and Nathaniel Cresswell, who have helped us and taught us so much in cardiovascular pathology, through their patience, insight, curiosity, and insistence on diagnostic accuracy and ...
Takotsubo syndrome is an acute reversible heart failure syndrome that is increasingly recognized ... more Takotsubo syndrome is an acute reversible heart failure syndrome that is increasingly recognized in modern cardiology practice. This Position Statement from the European Society of Cardiology Heart Failure Association provides a comprehensive review of the various clinical and pathophysiological facets of Takotsubo syndrome, including nomenclature, definition, and diagnosis, primary and secondary clinical subtypes, anatomical variants, triggers, epidemiology, pathophysiology, clinical presentation, complications, prognosis, clinical investigations, and treatment approaches. Novel structured approaches to diagnosis, risk stratification, and management are presented, with new algorithms to aid decision-making by practising clinicians. These also cover more complex areas (e.g. uncertain diagnosis and delayed presentation) and the management of complex cases with ongoing symptoms after recovery, recurrent episodes, or spontaneous presentation. The unmet needs and future directions for r...
Infection of the mural endocardium within a left ventricular aneurysm without valvular involvemen... more Infection of the mural endocardium within a left ventricular aneurysm without valvular involvement is exceedingly rare. The presenting clinical features can be non-specific, and a high index of suspicion is required for its diagnosis. Delay in diagnosis invariably leads to a fatal outcome. Although no controlled studies are available to guide therapy and management of these patients, appropriate antibiotic therapy and early surgical resection of the infected ventricular aneurysm remain the cornerstone of therapy.
To determine whether the new classification system for thymic carcinoid tumors/neuroendocrine car... more To determine whether the new classification system for thymic carcinoid tumors/neuroendocrine carcinomas provides prognostic data, and to study the presentation, diagnosis, treatment, and prognostic factors of these rare tumors. Retrospective analysis. Royal Brompton Hospital, London, UK. Eight men and four women with a median age of 58 years. Complete excision was possible in nine patients. Postoperative staging revealed two stage I tumors, two stage II tumors, three stage III tumors, one stage IVA tumor, and four stage IVB tumors. All tumors demonstrated the histopathologic features of neuroendocrine tumors, which were confirmed by positive immunohistochemical staining for chromogranin A in 11 of 12 tumors and for CD56 in 12 of 12 tumors, and the presence of dense core granules on ultrastructural analysis in 9 of 9 tumors. All 12 tumors did not stain positively for somatostatin receptors. Three tumors were grade 1, six cases were grade 2, and three cases were grade 3. Follow-up was available in all patients. One patient died 1 month postoperatively. Distant metastasis developed in nine patients (82%). Local recurrence was evident in six patients, of whom five had not received postoperative radiotherapy. Seven patients died of distant metastasis (22 to 83 months after surgery). Two are alive and disease-free (at 67 and 81 months), and two are alive with disease (at 60 and 86 months) Neither grading as neuroendocrine carcinomas nor any individual histologic parameter showed a significant association with prognosis. Initial aggressive treatment, including complete surgical excision and adjuvant radiotherapy, appears to offer the best hope for prolonged survival. Adjuvant chemotherapy also should be considered, since the incidence of distant relapse is high.
c Abstract We present a case of rapid onset acute cardiac failure developing as part of Neurolept... more c Abstract We present a case of rapid onset acute cardiac failure developing as part of Neuroleptic malignant syndrome in a 35-year-old woman following treatment with Thioridazine and Lithium. Post mortem histology of cardiac and skeletal muscle showed similar changes of focal cellular necrosis and vacuolation suggesting a common disease process. 2003 European Society of Cardiology. Published by Elsevier Science
We report a case of primary cardiac lymphoma presenting as myopericarditis and rapidly deteriorat... more We report a case of primary cardiac lymphoma presenting as myopericarditis and rapidly deteriorating into biventricular heart failure and ventricular arrhythmias. Computed tomography and cardiac magnetic resonance (CMR) imaging showed extensive myocardial infiltration with typical patterns on tissue characterization CMR images, raising clinical suspicion. Diagnosis was confirmed by myocardial histologic examination. Marked regression of tumor burden was apparent after 6 cycles of anthracycline-based chemotherapy. This case illustrates that a high degree of suspicion for this rare entity is mandated to institute timely treatment. Rapid tumor lysis may induce life-threatening acute cardiac decompensation that requires intensive monitoring and support therapy.
Objective: The aim of this study was to report on sudden cardiac death (SCD) during or immediatel... more Objective: The aim of this study was to report on sudden cardiac death (SCD) during or immediately after a stressful event in a predominately young cohort. Methods: This study used retrospective non-case-controlled analysis. A total of 110 cases of SCD in relation to a stressful event such as altercation (45%), physical restraint (31%) in police custody (10%), exams/school/job stress (7.27%), receiving bad news (4%), or a car accident without injuries (2.73%) were retrospectively investigated. The majority of the subjects experiencing SCD were male (80.91%). The mean age was 36 AE 16 years (range 5–82 years). Twenty-three cases (20.91%) were psychiatric patients on antipsychotic medication. Results: Fifty-three per cent of cases died with a negative autopsy and a morphologically normal heart, indicating sudden adult death which is linked to cardiac channelopathies predisposing to stress-induced SCD. Cardiomyopathy was found in 16 (14.5%) patients and coronary artery pathology in 19 (17%) patients, with atherosclerosis predominating in older patients. Conclusions: This study highlights SCD during psychological stress, mostly in young males where the sudden death occurred in the absence of structural heart disease. This may reflect the proarrhythmic potential of high catecholamines on the structurally normal heart in those genetically predisposed because of cardiac channelopathy. Structural cardio-myopathies and coronary artery disease also feature prominently. Cases of SCD associated with altercation and restraint receive mass media attention especially when police/other governmental bodies are involved. This study highlights the rare but important risk of SCD associated with psychological stress and restraint in morphologically normal hearts and the importance of an expert cardiac opinion where prolonged criminal investigations and medico-legal issues often ensue.
A retained surgical swab (gossypiboma) is a rare but important complication of intrathoracic surg... more A retained surgical swab (gossypiboma) is a rare but important complication of intrathoracic surgery. The radiographic and computed tomography (CT) appearances are variable and depend on the chronicity and site of the swab within the chest. Two cases of retained swabs within the chest are reported. In both cases, the swab had become surrounded by lung. The swab within the pleural space acted as a nidus and resulted in infolding of the lung, superficially resembling an intrapulmonary abscess on CT.
Congenital coronary artery anomalies are of major significance in clinical cardiology and cardiac... more Congenital coronary artery anomalies are of major significance in clinical cardiology and cardiac surgery due to their association with myocardial ischaemia and sudden death. Such anomalies are detectable by imaging modalities and, according to various definitions, their prevalence ranges from 0.21 to 5.79%. This consensus document from the Development, Anatomy and Pathology Working Group of the European Society of Cardiology aims to provide: (i) a definition of normality that refers to essential anatomical and embryological features of coronary vessels, based on the integrated analysis of studies of normal and abnormal coronary embryogenesis and pathophysiology; (ii) an animal model-based systematic survey of the molecular and cellular mechanisms that regulate coronary blood vessel development; (iii) an organization of the wide spectrum of coronary artery anomalies, according to a comprehensive anatomical and embryological classification scheme; (iv) current knowledge of the pathophysiological mechanisms underlying symptoms and signs of coronary artery anomalies, with diagnostic and therapeutic implications. This document identifies the mosaic-like embryonic development of the coronary vascular system, as coronary cell types differentiate from multiple cell sources through an intricate network of molecular signals and haemodynamic cues, as the necessary framework for understanding the complex spectrum of coronary artery anomalies observed in human patients.
... To my family, Rachel, Nicky and Madeline, to whom I owe ... Special thanks go to Bob Kutys an... more ... To my family, Rachel, Nicky and Madeline, to whom I owe ... Special thanks go to Bob Kutys and Nathaniel Cresswell, who have helped us and taught us so much in cardiovascular pathology, through their patience, insight, curiosity, and insistence on diagnostic accuracy and ...
Takotsubo syndrome is an acute reversible heart failure syndrome that is increasingly recognized ... more Takotsubo syndrome is an acute reversible heart failure syndrome that is increasingly recognized in modern cardiology practice. This Position Statement from the European Society of Cardiology Heart Failure Association provides a comprehensive review of the various clinical and pathophysiological facets of Takotsubo syndrome, including nomenclature, definition, and diagnosis, primary and secondary clinical subtypes, anatomical variants, triggers, epidemiology, pathophysiology, clinical presentation, complications, prognosis, clinical investigations, and treatment approaches. Novel structured approaches to diagnosis, risk stratification, and management are presented, with new algorithms to aid decision-making by practising clinicians. These also cover more complex areas (e.g. uncertain diagnosis and delayed presentation) and the management of complex cases with ongoing symptoms after recovery, recurrent episodes, or spontaneous presentation. The unmet needs and future directions for r...
Infection of the mural endocardium within a left ventricular aneurysm without valvular involvemen... more Infection of the mural endocardium within a left ventricular aneurysm without valvular involvement is exceedingly rare. The presenting clinical features can be non-specific, and a high index of suspicion is required for its diagnosis. Delay in diagnosis invariably leads to a fatal outcome. Although no controlled studies are available to guide therapy and management of these patients, appropriate antibiotic therapy and early surgical resection of the infected ventricular aneurysm remain the cornerstone of therapy.
To determine whether the new classification system for thymic carcinoid tumors/neuroendocrine car... more To determine whether the new classification system for thymic carcinoid tumors/neuroendocrine carcinomas provides prognostic data, and to study the presentation, diagnosis, treatment, and prognostic factors of these rare tumors. Retrospective analysis. Royal Brompton Hospital, London, UK. Eight men and four women with a median age of 58 years. Complete excision was possible in nine patients. Postoperative staging revealed two stage I tumors, two stage II tumors, three stage III tumors, one stage IVA tumor, and four stage IVB tumors. All tumors demonstrated the histopathologic features of neuroendocrine tumors, which were confirmed by positive immunohistochemical staining for chromogranin A in 11 of 12 tumors and for CD56 in 12 of 12 tumors, and the presence of dense core granules on ultrastructural analysis in 9 of 9 tumors. All 12 tumors did not stain positively for somatostatin receptors. Three tumors were grade 1, six cases were grade 2, and three cases were grade 3. Follow-up was available in all patients. One patient died 1 month postoperatively. Distant metastasis developed in nine patients (82%). Local recurrence was evident in six patients, of whom five had not received postoperative radiotherapy. Seven patients died of distant metastasis (22 to 83 months after surgery). Two are alive and disease-free (at 67 and 81 months), and two are alive with disease (at 60 and 86 months) Neither grading as neuroendocrine carcinomas nor any individual histologic parameter showed a significant association with prognosis. Initial aggressive treatment, including complete surgical excision and adjuvant radiotherapy, appears to offer the best hope for prolonged survival. Adjuvant chemotherapy also should be considered, since the incidence of distant relapse is high.
c Abstract We present a case of rapid onset acute cardiac failure developing as part of Neurolept... more c Abstract We present a case of rapid onset acute cardiac failure developing as part of Neuroleptic malignant syndrome in a 35-year-old woman following treatment with Thioridazine and Lithium. Post mortem histology of cardiac and skeletal muscle showed similar changes of focal cellular necrosis and vacuolation suggesting a common disease process. 2003 European Society of Cardiology. Published by Elsevier Science
We report a case of primary cardiac lymphoma presenting as myopericarditis and rapidly deteriorat... more We report a case of primary cardiac lymphoma presenting as myopericarditis and rapidly deteriorating into biventricular heart failure and ventricular arrhythmias. Computed tomography and cardiac magnetic resonance (CMR) imaging showed extensive myocardial infiltration with typical patterns on tissue characterization CMR images, raising clinical suspicion. Diagnosis was confirmed by myocardial histologic examination. Marked regression of tumor burden was apparent after 6 cycles of anthracycline-based chemotherapy. This case illustrates that a high degree of suspicion for this rare entity is mandated to institute timely treatment. Rapid tumor lysis may induce life-threatening acute cardiac decompensation that requires intensive monitoring and support therapy.
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Papers by Mary Sheppard