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This chapter deals with the inherited metabolic diseases affecting the heart in which there are morphological changes sufficient to permit a tentative diagnosis to be offered by a pathologist. A large first section deals with glycogen storage disorders and is well illustrated. This is followed by discussion of lysosomal storage disorders, including Niemann–Pick disease, sections on mucopolysaccharidosis and of the commoner disorders of lipid oxidation. Disorders of iron metabolism and amino acidurias close the chapter.
This chapter examines the structure of the normal heart at the gross, microscopic and ultrastructural levels. The pericardium, myocardium, endocardium, valves, arteries, veins, lymphatics, nerves and conduction system are described in detail, together with common variants of normal. References are given to normal values for heart measurements throughout gestation, infancy and childhood.
A separate chapter is devoted to the pericardium, beginning with congenital absence of pericardium and other congenital defects and cysts. Pericardial effusion is treated in some detail together with the related topics of tamponade and haemorrhage. There are sections on inflammation of the pericardium that also include discussion of post-pericardiotomy syndrome and of constrictive pericarditis. The chapter closes with a short discussion of pericardial tumours.
Following a brief introduction listing the salient aspects of heart transplantation as they pertain to children, there is a detailed section on assessment of the explanted heart with special reference to cardiomyopathy and congenital heart disease. This is followed by a section on the implanted heart that deals with the problems of acute cellular rejection, chronic allograft vasculopathy and post-transplant lymphoproliferative disorder. Particular attention is paid to endomyocardial biopsy in these assessments. The chapter closes with sections on re-explant and on post-mortem assessment of the transplanted heart.
This chapter is devoted to the macroscopic and microscopic appearance of myocardial ischaemia and includes discussion of regional myocardial infarction and of papillary muscle rupture. Coronary atherosclerosis can occur, albeit rarely, in the child, and this is discussed particularly in relation to hypercholesterolaemia. Antiphospholipid syndrome and haemolytic-uraemic syndrome are also discussed.
The chapter opens with observations on the normal fetal heart and proceeds to a discussion of fetal hydrops and its associated cardiac causes. There follows a treatment of the syndromes associated with congenital heart disease in the fetus. Common congenital heart defects as they appear in the fetus are illustrated. Fetal cardiomyopathy, myocarditis and arrhythmia all have separate discussions. The chapter closes with a series of discussions on the pathology of twins as it affects the heart: twin–twin transfusion syndrome, conjoined twins and acardiac twins.
A brief account of early embryo formation is followed by an overview of the salient features of heart development. Following this there is a detailed treatment of the processes involved in the construction of the functioning embryonic heart, including a focus on the genes involved. Short sections are devoted to pericardium, coronary arteries and conduction system, the systemic veins and the systemic arteries. Finally, the fetal circulation and its adaptation to post-natal life are described.
Methods of dissection of the heart are described and illustrated. Simulated echocardiographic views are described and illustrated, and advice is given on how best to obtain them and in what circumstances they are most useful. Sequential segmental analysis is described in the context of the normal and malformed heart. Sampling for histology is described for the post-mortem heart and for cardiac specimens submitted to the surgical pathologist. A short guide to photography is given. The tables provide a summary of histological features of the normal heart and their significance and also summarise the application of immunohistochemistry to the heart.
This chapter surveys the entire spectrum of tumours that are described in the fetus, infant and child. The commoner tumours (rhabdomyoma, fibroma, teratoma, myxoma) are discussed in detail, but there is also extensive discussion of rarer tumours such as fatty tumours and vascular tumours and even rarer entities such as juvenile xanthogranuloma or inflammatory myofibroblastic tumour. A brief section is devoted to primary malignant tumours of the heart and to metastatic tumours. Finally, there is discussion of pseudoneoplasms that may be seen in children.
A brief reprise of normal coronary artery structure is followed by a discussion of normal anatomical variants of the coronary arteries. The commoner abnormal variants, including origin of the left coronary artery from the pulmonary artery and intramural course of a coronary artery, are described and illustrated, followed by a discussion of coronary fistula and atresia. A section is devoted to the variations in coronary anatomy associated with the commoner forms of congenital heart disease. Coronary arteritis is discussed, chiefly in the context of Kawasaki disease, but polyarteritis and eosinophilic arteritis are also described. Fibromuscular dysplasia is treated in some detail and idiopathic arterial calcification rounds off the chapter.
A brief introduction on demographics is followed by a section on the aims of investigation of sudden cardiac death. Specific cardiac causes are dealt with in turn: congenital heart disease, coronary artery anomalies, cardiomyopathy, myocarditis and metabolic disease. A section is then devoted to cases where there is no morphological abnormality and death is due to ion channelopathy. There is a separate discussion of sudden infant death syndrome as a possible sudden cardiac death. The chapter closes with a brief discussion of commotio cordis.
This chapter deals with inflammatory disease affecting the heart, both disease confined to the heart and inflammatory disease where the heart is affected as one among many other tissues. Myocarditis and its classification and investigation are detailed. Rarer forms including eosinophilic disease and giant cell myocarditis are also included. Endocarditis, both infective and non-infective, is discussed. Systemic inflammatory disease such as lupus, systemic sclerosis, rheumatic disease and sarcoid are also illustrated. Aortic inflammatory disease including Takayasu disease is described.
The second of the two chapters on congenital heart disease deals with the less common conditions. Conditions covered include double-inlet ventricle, double-outlet ventricle, atrial isomerism and Ebstein's anomaly. The conditions are well illustrated. A section follows on the pathological features of pulmonary arterial hypertension in congenital heart disease. A large section is devoted to common surgical operations for congenital heart disease that may be encountered by the pathologist, and a section is devoted to the pathological assessment of the operated heart with congenital heart disease.
This chapter, the first of two devoted to congenital heart disease, deals with the commoner forms including ventricular septal defect, atrioventricular septal defect, tetralogy of Fallot and hypoplastic left heart. The pathological features are described in detail and profusely illustrated, including images of the histopathology, where relevant.
After a general introduction, the classification systems for cardiomyopathy are discussed. The main clinical types are discussed together with their variants. Hypertrophic, dilated, restrictive, non-compaction, mitochondrial and arrhythmogenic cardiomyopathy are all detailed and illustrated. Tables list the many genes associated with development of these cardiomyopathies. Rarer forms such as histiocytoid cardiomyopathy and mitogenic cardiomyopathy are also illustrated.
In recent years, there have been no books published on paediatric cardiac pathology despite enormous developments in genetics, a marked explosion of paediatric transplant programmes, surges in knowledge of fetal cardiac pathology and understanding of congenital heart disease, and the emergence of a flourishing cardiac imaging discipline. This book will be the first unified and comprehensive source of reference for childhood heart disease, covering the full field of paediatric cardiac pathology, in one volume. Comprising the twenty-five year experience of a single pathologist, the full spectrum of the pathology of heart disease, from the fetus to the adult, is uniquely presented here. Richly illustrated, with over 800 colour photographs, general and paediatric pathologists alike will be able to examine the microscopic features of the conditions described, with a specific focus on metabolic disease for practitioners worldwide.