Sudden coronary death due to IgG4-related disease

A 54-year-old male entered the emergency room in cardiorespiratory arrest after syncope at home. Resuscitation was attempted, but the patient died a few hours later. At necropsy, aneurysms were found at the right and left anterior descending coronary arteries. At microscopic examination, there was no significant coronary atherosclerosis, and a dense inflammatory infiltrate was detected, with a high number of igG4-positive cells (94.0 positive cells/hpf). The case illustrates that IgG4-related disease can cause coronary disease and sudden cardiac death.     

Sudden death from coronary artery dissection.

Spontaneous dissection of the coronary arteries is an uncommon condition that may lead to sudden, fatal coronary artery occlusion. Three cases of sudden death attributable to coronary artery occlusion are presented. Dissection was associated with Marfan's syndrome in the first case, and occurred three weeks postpartum in the second case. In case 1, dissection occurred within the intima, and was not associated with an inflammatory cell infiltrate. In cases 2 and 3, dissection occurred between the tunica media and the external elastic lamina, and was associated with a mixed inflammatory infiltrate, rich in eosinophils, T lymphocytes, and histiocytes. The spatial limitation of the inflammatory infiltrate to the adventitial compartment, together with the absence of inflammation in case 1, suggests a reactive origin rather than a causative role for the inflammatory cells. Detailed examination of serial blocks of any coronary artery occlusion is essential in young patients.     

Sudden cardiac death due to fibromuscular dysplasia of the coronary arteries

A man to whom sudden cardiac death occurred after he had lived to the age of 25 years in perfect condition is reported in this paper. Fibromuscular dysplasia of the coronary arteries was established as the cause of death by postmortem examination.     

Sudden death of an infant with coronary involvement due to Takayasu arteritis

We present an unusual case of sudden death of an 8-month-old female infant with coronary involvement due to Takayasu arteritis. She had been thought to be healthy, but died after presenting to a hospital with complains of vomiting. At autopsy, the aorta and its main branches were thickened and stenotic, with the abdominal aorta below the level of the orifice of renal arteries most severely affected. The ascending aorta was thickened and showed ostial stenosis in the coronary arteries bilaterally. The proximal segment of the left and right coronary arteries showed approximately 60% and over 90% occlusion, respectively. Microscopically, the intima was thickened due to an increase of intimal cells and fibers, and the adventitia showed thickening with fibrosis. In addition, remarkable infiltration of inflammatory cells, including multinuclear giant cells phagocytosing fragmented elastic fibers, and destruction of elastic fibers were observed in the media. We diagnosed the cause of death as coronary insufficiency induced by coronary involvement due to Takayasu arteritis. Takayasu arteritis was not considered as a cause of sudden infant death, although this disease can affect the coronary artery. This report suggests that Takayasu arteritis can be a life-threatening condition even in infants.     

Sudden coronary death in children

Coronary disease is an uncommon cause of severe morbidity and death in the young. The great majority of lesions are congenital, and anomalous origins and courses of the coronary arteries are the most common groups of diseases in the age group. Intimal proliferation and coronary arteritis are even less common causes of death in this age population. For this review, we retrospectively studied cases of coronary cardiac death in children and adolescents, from 2004 to 2007, from a statewide medical examiner's office. There were 197 natural deaths in children ages ≤16 years and 58 deaths from sudden infant death syndrome. Of these, the largest group of deaths showed no morphologic abnormalities and was attributed to arrhythmias. The next largest group of deaths comprised complex congenital heart disease (n=13; 20%), followed by myocarditis (n=11; 17%), coronary disease (n=9; 14%), cardiomyopathy (n=8; 12%), valve disease (n=4; 6%), and cardiac fibroma (n=1). Nine coronary deaths occurred in seven boys and two girls, ranging from 14 days to 16 years of age (mean, 8 years). An anomalous course of the coronaries was seen in seven of the nine coronary deaths, and the other two casualties were examples of intimal proliferations involving the coronaries. The rare examples of coronary lesions in the young are discussed in this article.     

Sudden infant death due to congenital adrenal hypoplasia

Adrenocortical hypoplasia was found to be the cause of sudden death in a 9-month-old infant. Most sudden infant deaths in the first year of life are related to the sudden infant death syndrome, in which no specific pathologic changes are found at autopsy. Although specific causes for sudden death in infancy are well documented, adrenocortical hypoplasia is not a well-recognized cause of sudden infant death.     

Sudden cardiac death not related to coronary atherosclerosis

Sudden cardiac death (SCD) accounts for approximately 300,000 cardiac events in the United States each year, representing an overall incidence of 0.1-0.2% per year. Although the vast majority of these may be attributed to coronary atherosclerosis, a wide variety of nonatherosclerotic-related cardiac diseases have been associated with SCD. This review highlights three general categories of cardiac disease not related to atherosclerosis: the cardiomyopathies, inflammatory myocardial diseases, and ion channel disorders. The important role played by genetics in some of these cardiovascular diseases is presented as well as toxic and drug-related etiologies.     

Fatal hemopericardium due to ruptured solitary myocardial abscess unassociated with infective endocarditis

A case is presented of hemopericardium and sudden death due to rupture of a solitary myocardial abscess in an elderly woman with cirrhosis and mild urosepsis. If suspected clinically, such myocardial abscesses may be detectable by noninvasive tomographic cardiac imaging.     

Cardiac function in open-chest dogs after left to right ventricular shunting and right coronary artery occlusion

Patients with acquired ventricular septal defect (VSD) after myocardial infarction have a particularly bad prognosis if right ventricular function is severely impaired. The significance of an ischaemic right ventricular free wall on cardiac function during interventricular shunting was examined in open-chest dogs. An external interventricular shunt could be opened and closed at will, and by occlusion of the right coronary artery (RCA), a part of the right ventricular free wall was rendered ischaemic. Aortic flow decreased by 8 +/- 2% when the shunt was opened in the presence of a normal right ventricle, and by 16 +/- 2% (difference: P less than 0.05) in the presence of right ventricular ischaemia. Aortic flow fell by 19 +/- 3% when the RCA was occluded. Right ventricular dyskinesia was demonstrated after occlusion of RCA, by recording segment lengths in the right ventricular free wall. The dyskinesia was aggravated when the shunt was opened. The left ventricle exerted a 'negative' work on the ischaemic right ventricular free wall. Retention of blood in the right ventricle, with a subsequent decline in left ventricular filling and an almost unchanged interventricular shunting of blood, explain why aortic flow fell more when the shunt was opened in the presence of right ventricular ischaemia.     

Favorable outcome of infective endocarditis due to Streptococcus agalactiae after conservative treatment.

Infective endocarditis due to Streptococcus agalactiae is uncommon and carries an ominous prognosis, leading some authors to advocate early surgery. This report describes an 83-year-old woman with community-acquired infective endocarditis due to S. agalactiae. The patient, who had a history of surgery for colon cancer, presented with fever, agitation and general malaise. She achieved a favorable outcome with antibiotic treatment only. For infective endocarditis due to S. agalactiae, appropriate antimicrobial agents should be started as soon as possible, with surgery reserved for those cases of particular indication.     

Sudden cardiac death caused by coronary vasculitis

Coronary vasculitis is a rare and diagnostically challenging cause of sudden cardiac death (SCD). There are currently no large-scale series on this rare entity. A retrospective non-case–control observational study of SCD with coronary vasculitis referred to a tertiary cardiac pathology referral centre at the National Heart and Lung Institute at the Royal Brompton Hospital between 1996 and 2010 was completed. Ten cases of SCD with coronary artery vasculitis were retrieved from a database of 1,980 SCD cases (0.5%) with a 1:1 male/female ratio; median age was 39 years and range 15–71 years. Six deaths occurred in hospital following symptoms or cardiac arrest in the community; the remaining died at rest at home (n = 4). Appearances ranged from aneurysms of the coronary artery to occlusive lesions mimicking atheroma or masses imitating tumour. Types of vasculitis detected were: eosinophilic (n = 5), two associated with Churg–Strauss syndrome; lymphoplasmacytic vasculitis (n = 4); and idiopathic giant cell arteritis (n = 1). This study shows coronary vasculitis as a rare cause of SCD with a variable macroscopic and microscopic presentation that pathologists need to be aware of.