Physical Address

304 North Cardinal St.
Dorchester Center, MA 02124

Hereditary Cardiomyopathies

The World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of Cardiomyopathies define cardiomyopathies as a group of diseases of the myocardium that result in cardiac dysfunction. Although cardiomyopathy may be secondary to myocardial damage (e.g., myocardial infarction or hypertension), this chapter discusses the genetic basis of intrinsic cardiomyopathies without other identifiable causes. Cardiomyopathies are classically divided into several categories…

Restrictive Cardiomyopathy

Etiology and Pathophysiology The variety of disease states that may result in a restrictive cardiomyopathic process are summarized in Box 31.1 . Myocardial fibrosis, myocardial infiltration by specific proteins, endomyocardial scarring, and cardiac muscle hypertrophy all may contribute to diastolic dysfunction. Recent genetic studies have found a substantial number of patients with “idiopathic” restrictive cardiomyopathy (CM) caused by mutations in genes that have traditionally been associated…

Hypertrophic Cardiomyopathy

Etiology and Pathogenesis Approximately 700,000 individuals in the United States have hypertrophic cardiomyopathy (HCM), a monogenetic disorder that results in hypertrophy of the left ventricle (LVH) without another cardiac or systemic cause. HCM results from 1 of >1500 different mutations of genes that encode sarcomeric proteins. The mutation is transmitted in an autosomal dominant Mendelian pattern, such that 50% of direct descendants are gene-positive. Phenotypic expression…

Management of Chronic Heart Failure

Heart failure (HF) is a complex clinical syndrome that can result from any structural or functional cardiac disorder that impairs the ability of the ventricle to fill with (HF with preserved ejection fraction; HFpEF) or eject (HF with reduced ejection fraction; HFrEF) blood. Most commonly, HF results from myocardial muscle dysfunction with accompanying dilation and/or hypertrophy of the left ventricle (LV), remodeling, and neurohormonal activation. However,…

Management of Acute Heart Failure

Definitions and Epidemiology The term acute decompensated heart failure (ADHF) encompasses an array of disease processes related to inefficient cardiac function with compromised hemodynamics and/or volume status. Although ADHF has historically been treated on an inpatient basis, there are increasing trends to intervene on an outpatient basis. Accordingly, admissions for HF have decreased over time. One study that examined epidemiological trends of HF found an approximate…

Epidemiology of Heart Failure: Heart Failure With Preserved Ejection Fraction and Heart Failure With Reduced Ejection Fraction

Over the last few decades, heart failure (HF) has emerged as a true epidemic, with an estimated global prevalence of 38 million patients. HF affects 6.5 million American adults, with nearly 1 million new cases annually. HF can be due to multiple different etiologies, and there are numerous identifiable risk factors. HF is the most common cause of hospitalization for patients 65 years and older in…

Coronary Hemodynamics and Fractional Flow Reserve

Basic Principles of Coronary Blood Flow Myocardial cell contraction and relaxation are aerobic processes that require oxygen. Determinants of myocardial oxygen demands include preload, afterload, heart rate, contractility, and basal metabolic rate. Other than basal metabolic rate, these are factors that influence stroke volume. Systolic wall tension uses approximately 30% of myocardial oxygen demand. Wall tension itself is affected by intraventricular pressure, afterload, end-diastolic volume, and…

Congenital Coronary Anomalies

Unfortunately, presentation with cardiac arrest or sudden cardiac death is a common manifestation of congenital coronary anomalies. This clinical relevance underpins the necessity of understanding the anatomy and presentation of congenital coronary anomalies and their treatment options. The two primary congenital coronary anomalies, anomalous connection of the left coronary artery from the pulmonary artery (ALCAPA) and anomalous connection of a main coronary artery to the aorta,…

Cardiogenic Shock After Myocardial Infarction

Cardiogenic shock (CS) is characterized by hypotension and end-organ hypoperfusion as a result of low cardiac output. CS remains the most common cause of death after presentation with a myocardial infarction (MI). This clinical state occurs in up to 10% of patients hospitalized with acute MI and remains the leading cause of death during hospitalization. The incidence of CS has decreased only slightly over time, and…

Coronary Artery Bypass Surgery

Cardiovascular disease is the leading cause of death of both sexes in the United States and all industrialized nations, and is increasingly becoming an important cause of death in developing countries. According to the Centers for Disease Control and Prevention, approximately 600,000 people die annually in the United States as a result of cardiac disease; 150,000 of them are aged younger than 65 years. In 2017,…

Percutaneous Coronary Intervention

Percutaneous coronary intervention (PCI) has undergone a dramatic transformation since its introduction in the 1970s. In the early 1990s, coronary stenting revolutionized PCI, improving procedural results and dramatically reducing the need for emergency coronary artery bypass graft surgery (CABG). The introduction of drug-eluting stents (DESs) in the 2000s greatly reduced the frequency of late repeat revascularization compared with bare metal stents (BMSs), and second-generation DESs have…

ST-Elevation Myocardial Infarction

The diagnosis of acute coronary syndrome (ACS) is based on findings ranging from clinical presentation on ECG and/or biochemical findings to pathological characteristics. Patients with ACS include those whose clinical presentations cover the following range of diagnoses: unstable angina, non–ST-elevation myocardial infarction (NSTEMI), and ST-elevation MI (STEMI). An estimated 220,000 STEMI events per year occur in the United States. Etiology and Pathogenesis The initial event in…

Non–ST-Elevation Acute Coronary Syndrome

Definition and Epidemiology Acute coronary syndrome (ACS) is a term used to describe a group of clinical syndromes included with acute myocardial ischemia. Unstable angina (UA), non–ST-segment elevation myocardial infarction (NSTEMI), and STEMI are included in this group of syndromes. Because the pathophysiology and management of patients with UA and NSTEMI are similar, these two groups are further subclassified as non–ST-segment elevation ACS (NSTE-ACS). It is…

Stable Coronary Artery Disease

Advances in pharmacotherapy and revascularization strategies have dramatically improved the short-term and long-term outcomes for patients with atherosclerotic coronary artery disease (CAD). At the same time, the worldwide incidence of atherosclerosis and CAD has also increased, largely driven by significant increases in obesity and type 2 diabetes mellitus. As a result, atherosclerotic CAD will remain a major public health issue for the foreseeable future. Patients with…

Cardiovascular Effects of Air Pollutants

Cardiovascular disease risk factors—including hypertension, lipid abnormalities, diabetes mellitus, obesity, physical inactivity, and tobacco use—provide targets for the prevention or progression of heart disease. Yet, these risk factors account for only approximately 50% to 75% of cases of ischemic heart disease and cardiac events. Air pollution is an environmental factor that contributes independently and modifies the known cardiovascular risk factors. The World Health Organization estimates that…

Diabetes and Cardiovascular Events

Etiology and Pathogenesis Diabetes mellitus is the resultant state of insulin deficiency that causes elevated blood glucose. The etiologies of this insulin deficiency can broadly be classified as either type 1 or type 2 diabetes. Type 1 diabetes is characterized by an autoimmune process that results in β-cell dysfunction and inadequate insulin production. This form of diabetes frequently presents during childhood or adolescence, and requires insulin…

Management of Lipid Abnormalities

The management of lipid disorders in reducing the risk of coronary heart disease (CHD) has evolved in the past few years. There are a number of factors that account for these changes—the introduction of the 2013 American Heart Association/American College of Cardiology (AHA/ACC) guideline report on cholesterol management and a series of clinical trials on nonstatin therapies (notably, several trials involved the cholesteryl ester transfer protein…

Hypertension

Hypertension, or high blood pressure (BP), is a major risk factor for atherosclerotic cardiovascular disease ( Box 15.1 ). Despite advances in the understanding of the pathophysiology, epidemiology, and natural history of hypertension, as well as improvements in therapy, many patients with hypertension are undiagnosed or inadequately treated. Hypertension remains an important contributor to coronary events, heart failure, stroke, and end-stage kidney disease. Box 15.1 Hypertension…

Angiogenesis and Atherosclerosis

Revascularization via coronary artery bypass surgery and percutaneous coronary intervention (PCI) remains the definitive therapy for patients with refractory ischemic heart disease, particularly when accompanied by left ventricular (LV) dysfunction. In particular, bypass surgery reduces mortality in patients with multivessel coronary artery disease and LV dysfunction. However, the surgery itself is invasive and is associated with significant mortality and morbidity. In fact, 37% of patients who…

Left and Right Heart Catheterization

Right and left heart catheterization is the introduction of a catheter into the right heart and left heart chambers, respectively. Right and left heart catheterization provide key hemodynamic data that can be used to diagnose various cardiac disorders. Left heart catheterization also allows for the performance of left ventriculography to assess left ventricular (LV) systolic function and valvular function. This chapter focuses on the procedural techniques,…