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Chagas Cardiomyopathy

Epidemiology of Chagas Disease First described by the Brazilian physician Carlos Chagas in 1909, Chagas cardiomyopathy is the result of a chronic myocarditis caused by infection with the parasite Trypanosoma cruzi. The chief mode of transmission is via the bite of a Triatomine species insect (kissing bug), although other modes of transmission such as congenital, transfusion-related, and oral have become more common. The disease is typically…

Endocrine Disease

Endocrine diseases can result in cardiovascular alterations in response to changes in homeostasis. Diabetes mellitus is the most common endocrine disease and is discussed in detail in another chapter. In this chapter, we cover the other major endocrine diseases along with their effects on the heart. There are no pathognomonic echocardiographic findings related to endocrine diseases such as hypothyroidism, hyperthyroidism, acromegaly, hypercortisolism, and hyperaldosteronism. However, some…

Cardiac Involvement in Hypereosinophilic Syndrome

In 1936, the Swiss physician Wilhelm Loeffler described a patient with progressive cardiac failure, eosinophilia, and inflammatory endocardial thickening. Subsequently, in 1968, Hardy and Anderson coined the term hypereosinophilic syndromes (HESs) encompassing different entities with marked blood eosinophilia in the absence of helminthiasis or allergic disorders. Idiopathic Hypereosinophilic Syndrome In 1975, Chusid and colleagues established diagnostic criteria for idiopathic HES (IHES) ( Box 155.1 ), substantially…

Sarcoidosis

Acknowledgments The authors thank Drs. Amit V. Patel and Gillian Murtagh for their contributions to the previous edition of this chapter. Sarcoidosis is a systemic inflammatory disorder characterized by the formation of noncaseating granulomas in multiple organs. Although its cause remains uncertain, accumulating evidence suggests that it is caused by an immunologic response to an unidentified antigenic trigger in genetically susceptible individuals. The annual incidence of…

Amyloid

The term amyloidosis (from Greek ἄμυλον: amylon , starch) was popularized in the 19th century by the German pathologist Rudolf Virchow because of amyloid’s affinity for staining dyes with starch. It is clearly a misnomer because amyloid deposits are made of protein and not starch. In general, amyloidosis entails typically extracellular infiltration by one of a variety of misfolded proteins, which all share the same β-pleated…

Carcinoid Heart Disease

Carcinoid tumors are rare neuroendocrine tumors that secrete vasoactive compounds, including serotonin. The estimated incidence of carcinoid tumors is reported between 1.0 and 8.4 per 100,000. Carcinoid tumors are classified based on their embryologic site of origin: foregut (bronchus, stomach, proximal duodenum), midgut (distal duodenum, jejunum, ileum, appendix, ascending colon), and hindgut (transverse and descending colon, rectum). Midgut tumors arising from the ileum and appendix are…

Antiphospholipid Syndrome

The pathogenic role of antiphospholipid antibodies (APLAs) in thrombotic events was recognized 30 years ago in a study of 65 patients with systemic lupus erythematous (SLE). The antiphospholipid syndrome (APS) is a clinical entity composed of venous or arterial thrombotic events or pregnancy-associated complications in the presence of APLAs. The frequent subgroups of APLA are lupus anticoagulant, anticardiolipin antibodies, and anti–β-2-glycoprotein I antibodies ( Box 151.1…

Systemic Lupus Erythematosus

In 1924, Emanuel Libman and Benjamin Sacks demonstrated noninfectious, nonrheumatic verrucous endocarditis in an autopsy series of four young patients with multiple symptoms. The seminal description highlighted the constellation of polyarthritis, pericarditis, fever, and cutaneous eruptions common in these patients and that the endocardial lesions extended into the mural endocardium. Since the original description by Libman and Sacks of their eponymous endocarditis, systemic lupus erythematosus (SLE)…

Rheumatic Fever and Rheumatic Heart Disease

Acute rheumatic fever (ARF) is an inflammatory disorder that occurs after a throat infection with group A β-hemolytic streptococcus infection (GAS). Rheumatic heart disease (RHD) is a chronic disorder in which the heart valves are damaged after an episode of ARF, or as is frequently the case, no identifiable history of ARF. The latter scenario has been defined as latent or subclinical RHD. Epidemiology Currently, ARF…

Obesity

Approximately 93.3 million, or 39.8% of adults in the United States, are obese. It is estimated by the World Health Organization that more than 650 million adults worldwide are obese and that this number has tripled since 1975. Because of a number of physiologic and metabolic changes associated with obesity, these individuals are at increased risk for cardiovascular (CV) disease. These changes include insulin resistance and…

End-Stage Renal Disease

Epidemiology Chronic renal disease is a major public health problem. The end-stage renal disease (ESRD) population is increasing in size. More than 26 million people (13%) in the United States have chronic kidney disease (CKD), and most are undiagnosed. Another 20 million are at increased risk of the disease. Cardiovascular disease is the leading cause of death in patients with ESRD. Cardiovascular mortality is 5 to…

Diabetes Mellitus

Pathophysiology Death and disability caused by cardiac dysfunction are among the most common complications of diabetes mellitus (DM). DM can cause pathophysiologic changes in the heart both directly, through its effects on the myocardium (e.g., through deposition of advanced glycosylation products, reactive oxygen species, impaired calcium handling by cells), and secondarily through its effects on the coronary circulation and on the cardiac autonomic nerves, which can…

Hypertension

Systemic arterial hypertension is a major cause of cardiovascular morbidity and mortality and is the number one attributable risk factor for death throughout the world. The adverse effects of hypertension result from structural and functional changes in the heart and arteries and from acceleration of atherosclerosis. Pressure overload–induced concentric left ventricular (LV) hypertrophy, although initially adaptive (by normalizing increased wall stress), is associated with alterations in…

Adult Congenital Heart Disease With Prior Surgical Repair

An extensive variety of operations are used to correct or palliate congenital heart disease (CHD), often cloaked in the language of acronyms and eponyms. This chapter provides simple guidance for clinicians and sonographers in deciphering this occasionally confusing area. The approach to an adult postoperative patient should include some knowledge and history of what has been previously done for the patient. Attempting an echocardiogram without this…

The Adult With Unrepaired Complex Congenital Heart Defects

Most complex congenital heart disease (CHD) present with cyanosis in early childhood and necessitate intervention before adulthood. In contrast, adults with acyanotic complex CHD can escape detection for many decades because they often do not have major associated lesions. The echocardiographic approach should be tailored to the indication: a sequential and segmental approach should be used for an initial diagnostic study. If the study is to…

Obstructive Lesions

Obstructive lesions along the outflow tracts of the right ventricle (RV) and left ventricle (LV) can be found at the level of the semilunar valve, below the valve within the subarterial outflow chamber, or above the valve along the great arteries. Based on a meta-analysis of nearly 40 published studies evaluating the incidence of congenital heart disease (CHD) over many decades, pulmonary stenosis (PS) represents the…

Common Congenital Heart Defects Associated With Left-to-Right Shunts

Congenital heart defects (CHDs) associated with left-to-right shunts are among the most common anomalies. The most common types of left-to-right shunt lesions include atrial septal defect (ASD), ventricular septal defect (VSD), atrioventricular septal defect (AVSD), and patent ductus arteriosus (PDA) ( Table 141.1 ). Their clinical significance is based on volume overload, congestive heart failure, pulmonary hypertension, and endocarditis. Some defects may close spontaneously, such as…

Systematic Approach to Adult Congenital Heart Disease

To efficiently perform an echocardiographic study on an adult with congenital heart disease (CHD), it is extremely important to understand the following: (1) the role of history and natural history, (2) location of the surgical scar(s) and probable surgical repair, (3) knowledge of segmental analysis, (4) special pediatric views and their significance, and (5) the role of transesophageal echocardiography (TEE), particularly with certain CHDs and those…

Congenital Heart Disease: Basic Principles

Over the past few decades, there has been a tremendous advancement in the medical and surgical management of patients with congenital heart disease (CHD), leading to an improved survival into adulthood. It has been estimated in various population-based studies worldwide that the prevalence of patients with adult congenital heart disease (ACHD) ranges between 2.17 and 6.12 per 1000 adults ( Fig. 139.1 ). The most striking…

Aortitis

Aortitis is a general and nonspecific term that refers to a broad group of infectious and noninfectious conditions in which there is abnormal inflammation of the aortic wall. These inflammatory conditions have different clinical and morphologic features and variable prognoses. Some conditions exclusively affect the aorta, whereas others may also extend to its major branches. The clinical manifestations are often vague and nonspecific, including nonspecific pain,…