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Left-to-Right Shunt Lesions

I. Atrial Septal Defect (Ostium Secundum ASD) A. Prevalence Thirty to 40 percent of all CHDs. Female preponderance (male-to-female ratio of 1:2). B. Pathology and Pathophysiology 1. Three types of ASDs occur in the atrial septum ( Fig. 7.1 ). a. Secundum ASD is in the central portion of the septum and is the most common type (50% to 70% of ASDs). b. Primum ASD (or…

Invasive Procedures

There are two kinds of invasive procedures that are used in the practice of pediatric cardiology: diagnostic cardiac catheterization (including angiocardiography) and catheter intervention procedures (therapeutic cardiac catheterization). I. Cardiac Catheterization and Angiocardiography Cardiac catheterization and angiocardiography are the definitive diagnostic tests for most cardiac patients. They are carried out under sedation or anesthesia. A. Indications Accurate diagnosis of most CHDs does not require diagnostic catheterization.…

Other Noninvasive Tools

Besides noninvasive imaging tools, there are other noninvasive investigational tools that are frequently used in the evaluation of cardiac patients. They include exercise stress testing, long-term ECG monitoring, and ambulatory blood pressure (BP) monitoring. I. Exercise Stress Testing Exercise stress testing plays an important role in the evaluation of cardiac symptoms by quantifying the severity of the cardiac abnormality and assessing the effectiveness of management. Although…

Noninvasive Imaging Tools

I. Echocardiography Echocardiography (echo) is an extremely useful noninvasive test used in the diagnosis and management of heart disease. An echo study currently begins with real-time two-dimensional echo (2D echo), which produces high-resolution tomographic images of cardiac structures and their movement, and vascular structures leaving and entering the heart. With the support of Doppler and color flow mapping (as described later), echo studies provide reliable anatomic…

Chest Roentgenography

Chest radiography was an essential part of cardiac evaluation before the echocardiographic (echo) studies became widely available to cardiologists. This simple test remains very useful to physicians who do not have access to the echocardiograph. In addition, cardiovascular abnormalities may be incidentally suspected by chest radiographic films. Information to be gained from chest radiographs includes (1) heart size and silhouette, (2) enlargement of specific cardiac chambers,…

Electrocardiography

One normal cardiac cycle is represented by successive waveforms on an ECG tracing: the P wave, the QRS complex, and the T wave ( Fig. 2.1A ). These waves produce two important intervals, PR and QT, and two segments, PQ and ST. Open full size image Fig. 2.1 (A) Definition of ECG configuration and (B) diagrammatic representation of the conduction system of the heart. From Park,…

History and Physical Examination

I History Taking Prenatal, natal, perinatal, postnatal, past, and family histories should be obtained. A Gestational and Perinatal History 1. Maternal infection: Rubella during the first trimester of pregnancy commonly results in PDA and PA stenosis (rubella syndrome, see Table 1.1 ). Other viral infections early in pregnancy may be teratogenic. Viral infections (including human immunodeficiency virus) in late pregnancy may cause myocarditis. Table 1.1 Major…

Drugs for Pulmonary Hypertension

Drug Class Overview and Guidelines Pulmonary arterial hypertension (PAH) is a rare, heterogenous family of disease states marked by pathologic remodeling of the pulmonary vascular system, leading to increased pulmonary artery pressure and ultimately right ventricular failure, hypoxia, and death. Unlike the majority of pulmonary hypertension in the United States, which is secondary to either chronic lung disease or chronic left ventricular dysfunction, PAH is thought…

Vascular Medicine Drugs

Overview Drugs for Patients With Peripheral Artery Disease Peripheral artery disease (PAD) is broadly defined as arterial occlusive disease outside of the coronary circulation and generally refers to atherosclerotic occlusive disease. While nonatherosclerotic occlusive disease including that related to fibromuscular dysplasia and vasculitis does exist, it is relatively uncommon relative to atherosclerotic disease, and the focus of the current chapter will be on the latter. The…

Antiarrhythmic Drugs

Overview of New Developments Antiarrhythmic drugs have not changed substantially over the last several decades. Even when newer agents have been developed, the potential for proarrhythmia and increased mortality continue to plague their broader implementation. Recently, novel ion channel targets have been proposed and newer agents are being researched, but even some experimental agents have halted trials in phase II because of unexpected toxicity. Furthermore, as…

Antithrombotic Drugs

Introduction Antithrombotic therapy represents a time-tested foundation for the prevention and treatment of cardiovascular disease that is firmly rooted in an understanding of the pathophysiology of common clinical phenotypes, including acute coronary syndrome, acute ischemic stroke, venous thromboembolic disease, stent thrombosis, heart chamber thrombosis, and mechanical device thrombosis. In each case, one or more well-delineated abnormalities present within the vasculature, circulating blood cells, plasma and its…

Lipid-Modifying Drugs

Serum cholesterol level is known to be related to incident atherosclerotic cardiovascular disease (ASCVD), with low-density lipoprotein cholesterol (LDL-C) found to be a dominant contributor to atherosclerosis. Multiple large landmark randomized controlled trials of lipid-lowering therapy have consistently shown that LDL-C lowering reduces risk of incident ASCVD. The widespread availability, convincing clinical evidence, and relative safety of the statins have established pharmacologic control of blood lipids…

Drugs for Diabetes

Metabolic Syndrome and Prediabetes Obesity has become a common problem in Western society, and it is a strong predictor of type 2 diabetes mellitus (T2DM). In the United States it is estimated that almost one-third of the population will develop T2DM in their lifetimes. T2DM, in turn, predisposes to cardiovascular abnormalities. An increased waistline is one of the five criteria of the metabolic syndrome (MetSyn)—a prediabetic…

Heart Failure

Introduction Drug therapies for heart failure (HF) differ depending on the phenotype of presentation. Distinctive phenotypes of presentation have different management targets, ranging from acute decompensated HF (ADHF) to chronic HF with reduced ejection fraction (HFrEF) or preserved ejection fraction (HFpEF). For the purposes of this discussion, we shall focus on drug therapy for specific phenotypic syndromes including ADHF, HFrEF, and HFpEF. Preventive care, self-management, cardiac…

Antihypertensive Therapies

Introduction The formal classification and blood pressure (BP) threshold to define an individual as having hypertension continues to vary based on available evidence. The appraisal of such evidence by professional associations and societies has led to different BP thresholds adopted in guidelines worldwide. Given that the thresholds by which we define hypertension can and will continue to evolve, it is less helpful to think of hypertension…

Drugs for Ischemic Heart Disease

Introduction The contemporary management of patients with ischemic heart disease demands a sound understanding of the pathophysiologic precipitants of both angina pectoris and myocardial ischemia from which the principles of pharmacotherapy can be applied and tailored to the specific causes underlying these perturbations of myocardial oxygen supply and demand. This chapter details several broad classes of drug therapies directed at both symptom relief and ameliorating the…