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The first transplantation of a human heart was performed in South Africa in 1967. By the end of the 1970s, transplantation was established as an effective therapy for end-stage cardiac failure. Over the next 30 years, improvements in donation and the preservation of organs, selection of patients, postoperative management, and treatment of rejection have resulted in markedly improved survival following transplantation in both adults and children.…
Introduction The use of long-term mechanical circulatory support (MCS), predominately in the form of ventricular assist devices (VADs), has undergone a major transformation in the past 15 years. In the early 2000s, device use was uncommon. Support was typically for short duration, in patients with cardiogenic shock, and pediatric-specific device use, especially in the United States, was exceptionally rare. In the initial report on VADs from…
Introduction Chronic heart failure has long been recognized as a cause of considerable mortality and morbidity in adults. The early recognition of heart failure in the 17th and 18th centuries was that of edema, anasarca, and dyspnea, which was appropriately attributed to blood “backing up” behind an impaired pump, the heart. Early descriptions of heart failure in children were usually in the setting of rheumatic fever.…
Background Acute circulatory failure is a clinical syndrome characterized by inadequate effective blood flow and reduced tissue perfusion with decreased delivery of oxygen to the capillaries. The reduction in oxygen delivery leads to impaired oxidative metabolism, lactic acidosis, and cell death. Acute circulatory failure in children may be due to primary myocardial disease such as myocarditis, cardiomyopathy, cardiac allograft failure, or congenital heart disease; or secondary…
This chapter discusses inflammatory diseases of the heart, excluding rheumatic fever (see Chapters 55 and 56 ), Kawasaki disease (see Chapter 54 ), pericarditis (see Chapter 58 ), and infectious endocarditis (see Chapter 57 ). It focuses on those diseases that involve the myocardium either exclusively or predominantly either as a result of a direct effect or secondarily due to toxins or inflammation. Myocarditis Myocarditis is…
Introduction Over the past several decades there have been significant improvements in the care of patients diagnosed with cancer, particularly in the pediatric age range. There are more than 15.5 million survivors of cancer in the United States, and in pediatrics specifically, cancer is diagnosed in 15,700 patients younger than 20 years each year ( Table 62.1 ). With current 5-year survival for all types at…
History of Classification of the Cardiomyopathies Cardiomyopathies are a heterogeneous group of diseases of the myocardium ( Box 61.1 ). They were initially described as “heart muscle disease of unknown cause” by the World Health Organization/International Society and Federation of Cardiology (WHO/ISFC) classification in 1980. This categorized cardiomyopathy into dilated (DCM), hypertrophic (HCM), and restrictive (RCM) types as well as “unclassified cardiomyopathy” based on the morphologic…
Introduction Systemic hypertension remains one of the most common cardiovascular diseases in adults. Over the past decade, several publications have highlighted the increasing prevalence of hypertension in children and young people and its increasing public health importance, mirroring the increasing prevalence of childhood obesity. Moreover, it is increasingly also being recognized that childhood blood pressure levels track into adulthood, with abnormal blood pressure levels during childhood…
Cardiac involvement in systemic disease can broadly be divided into those conditions where the heart is involved in the disease process itself and those where a structural or functional cardiac abnormality is associated with other anomalies, usually in a recognizable syndrome. Many of the conditions in the latter group have received attention in the sections of this book dealing with etiology and genetics. They are not…
Introduction The genetic basis for heart and vascular conditions is heterogeneous and includes both heritable and de novo mutations. More than 100 genes associated with congenital or progressive cardiovascular abnormalities have thus far been identified. In recent years there has been a growing awareness and focus on connective tissue diseases (CTDs) and associated cardiovascular pathology, particularly in children. These diseases are hereditary disorders of the body's…
The pericardium derives its name from the Greek term peri, meaning “around,” and kardia, meaning “heart.” It is composed of two layers. The inner layer (visceral pericardium) consists of a single layer of mesothelial cells, collagen, and elastin fibers separated from epicardium by fat. The outer layer (parietal pericardium) is mostly acellular and consists of collagen and elastin fibers. The pericardial space between these two layers…
Chronic rheumatic heart disease (RHD) is the persistence of valvar dysfunction following an episode of acute rheumatic fever (ARF) or ARF recurrence. Once the inflammatory markers normalize, any persistent valve dysfunction should be termed chronic RHD. Background and Epidemiology ARF, a delayed autoimmune reaction to group A streptococcal infection, is now rarely seen in high-income countries, where RHD is predominantly reported in the aging population as…
Introduction Rheumatic fever (RF) is an acute, diffuse, and nonsuppurative inflammatory disease that occurs as a delayed complication after an untreated or partially treated pharyngotonsillitis, the infection itself sometimes being asymptomatic. It is caused by group A β-hemolytic S treptococcus , specifically, S treptococcus pyogenes . The process is triggered by an inappropriate immunologic response, both humoral and cellular, and results from a complex interaction between…
Kawasaki disease was first described in 1967 by Tomisako Kawasaki, a Japanese pediatrician. He characterized the illness, then termed mucocutaneous lymph node syndrome , as including high fever, nonexudative conjunctivitis, inflammation of the oral mucosa, rash, cervical adenopathy, and findings in the limbs, including swollen hands and feet, red palms and soles, and, later, subungual peeling. Initially thought to be self-limited, the disease was later shown…
Cardiac tumors in infants and children are rare. Previously, their atypical clinical presentation prevented timely diagnosis, and the diagnosis was often postmortem. Currently, the widespread use of echocardiography and other noninvasive diagnostic imaging modalities has resulted in a marked increase in the detection of cardiac tumors during fetal life and childhood, when the patients are often asymptomatic. In turn, early recognition of cardiac tumors has resulted…
Introduction The various abnormal ventriculoarterial connections, such as transposition in its regular or congenitally corrected variants, double-outlet ventricles, and common arterial trunk, are all congenital malformations involving the ventricular outflow tract. They are often described in terms of “conotruncal malformations,” but this term is poorly defined. As currently used, it rarely includes lesions of the arterial valves, which are also components of the ventricular outflow tracts.…
There has been dramatic progress in the field of vascular anomalies in recent years, including improved understanding of the classification of these lesions, their natural history, and optimal management strategies. This section discusses only vascular anomalies that have the potential to pose cardiovascular ramifications, either due to abundant arteriovenous shunting presenting with congestive heart failure (CHF) or pulmonary hypertension or due to right-to-left shunting (at the…
Introduction An abnormally positioned heart is not in itself a malformation of major significance. Such abnormal positioning, for example, can be secondary to a deformity or defect of the chest wall, such as pectus excavatum or carinatum. The exceptions are the extreme examples of exteriorization of the heart, usually described as ectopia cordis, or union of parts of the heart in the setting of conjoined twins.…
Introduction The so-called Rathke diagram, showing six pairs of arteries encircling the tracheoesophageal pedicle, is still frequently used to provide an understanding of the morphogenesis of abnormalities of the brachiocephalic arteries in human patients with congenitally malformed hearts ( Fig. 48.1 ). The existence of six sets of arteries in mammalian species, however, has proved controversial for well over a century. As long ago as 1907,…