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Pharmacologic Intervention in the Fetus

Introduction With the arrival of echocardiography and magnetic resonance imaging (MRI), providing the means of noninvasively detecting and monitoring anomalies occurring during pregnancy, the fetus has increasingly become the target of prenatal treatment. Nonetheless, pregnancy is a unique situation, as treatment of the fetus cannot be approached independently from the concomitant treatment of the mother, especially when a medication is used for indications other than those…

Imaging and Quantifying the Fetal Circulation in Congenital Cardiac Disease : Ultrasound

Introduction Malformation of the heart and arterial trunks is the most common form of congenital anomaly found in humans. They occur in approximately 6 of every 1000 live births and in 8 to 10 of every 1000 pregnancies. Fetal echocardiography, or the use of ultrasonic technologies to evaluate the fetal cardiovascular system, enables the diagnosis of structural heart defects and offers a way to observe complex…

Magnetic Resonance Assessment of the Fetal Circulation in Congenital Heart Disease

Introduction The ability to accurately measure blood flow and oxygen saturations across the human late-gestation fetal circulation offered by advances in magnetic resonance imaging (MRI) has provided new insights into the impact of congenital heart disease (CHD) on the developing fetus. MRI has demonstrated redistribution of blood flow and interruption of the normal streaming of blood resulting from the obstructions to flow and abnormal connections that…

Physiology of the Developing Heart

Introduction The heart is the first organ to become fully functional in the developing embryo, providing the circulatory system necessary for embryogenesis and subsequent fetal development when growth cannot be sustained by diffusion of nutrients. Rapid advances in genetics and molecular biology have revolutionized our knowledge of the developing embryonic heart. Furthermore, technical improvements in imaging and noninvasive physiologic recording of the early human fetus have…

Myocardium and Development

A functional myocardium is necessary for viability during embryonic and fetal development. As such, the heart is the first functional organ because its ability to distribute essential nutrients to the developing embryo is essential for viability and normal progression of development. In the human embryo, a beating heart is apparent by 22 to 25 days postcoitum, and continued development and viability depend on the heart's ability…

Etiology of Congenital Cardiac Disease

Perhaps one of the most vexing aspects of congenital cardiac disease is the current inability to explain its origin. Environmental causes have been invoked, and until recently only scant evidence had pointed toward a genetic component. Recent experimental data, combined with advances in human genetics, have now provided a clearer understanding of how some malformations may occur, and certainly have illuminated general concepts that are certain…

Embryology of the Heart

Introduction From the functional point of view, the heart is simply a specialized part of the vascular system. Nonetheless, development of the heart as a specialized pump is of great significance. We have learned a great deal over recent decades regarding the origin of the muscular parts of this pumping organ. Until recently, it was believed that the initial linear tube, which gives rise to the…

Anatomy

Introduction It is axiomatic that to understand abnormal anatomy, and to describe it adequately, it is necessary first to understand the normal arrangement, including the relationships of the conduction tissues and coronary arteries to the various components of the heart. We review these features in this chapter and emphasize the significance of describing the various cardiac components as they are seen with the heart normally positioned…

Terminology

Introduction It might reasonably be thought that those who diagnose and treat patients with congenitally malformed hearts would, by now, have reached consensus concerning the most appropriate way of describing the malformations with which they are confronted. It is certainly the case that nomenclature is far less contentious now than in the previous millennium. It would be a brave person, nonetheless, who stated that the field…

Complications of Rheumatic Heart Disease and Acute Emergencies

Introduction The clinical course of both acute rheumatic fever (ARF) and rheumatic heart disease (RHD) may be complicated by acute life-threatening conditions. This chapter will focus on: (1) the clinical evaluation, diagnosis, and management of acute heart failure (AHF); (2) infective endocarditis (IE); and (3) the management of overanticoagulation and bleeding in patients receiving oral anticoagulation. The etiology and pathophysiology of AHF in rheumatic carditis is…

Awareness, Education, and Advocacy

Introduction Health-seeking behavior is a critical determinant of success for rheumatic heart disease (RHD) control programs. If people at risk of RHD do not seek care for group A streptococcal (GAS) infections, primary prevention is impossible. If they do not present with symptoms of acute rheumatic fever (ARF), the window of opportunity for successful secondary prevention closes. Similarly, people with RHD will not benefit from advanced…

Group A Streptococcus Vaccines

Introduction Although a reinvigorated global vaccine development effort gives legitimate reason for cautious optimism, it is a sad reality that in 2019 the best introduction to a discussion on vaccines for group A Streptococcus (GAS) comes from 1967, by Professor Gene Stollerman: … one might have expected that the vast amount of biochemical and immunologic information about this organism would have led to the successful induction…

Echocardiographic Screening for Rheumatic Heart Disease

Introduction Over the past decade, echocardiographic screening for rheumatic heart disease (RHD) has emerged as a potentially transformative strategy for global RHD control. School-aged children from nearly every continent have been screened under the premise of epidemiology or research. New Caledonia has established a national echocardiographic screening program and several other countries (including Tonga and Samoa) have embraced population-based screening. Echocardiographic screening has revealed a large…

Rheumatic Heart Disease Control Programs, Registers, and Access to Care

Rheumatic Heart Disease Control Programs Disease control is the “reduction in the incidence, prevalence, morbidity, or mortality of an infectious disease to a locally acceptable level.” Efforts to control acute rheumatic fever (ARF) and rheumatic heart disease (RHD) have been underway for nearly a century and are entering a new era with passing of the resolution on rheumatic fever and RHD at the 71st World Health…

Secondary Prevention of Acute Rheumatic Fever and Rheumatic Heart Disease

Introduction Definition of Secondary Prevention Individuals within a vulnerable age group who have had an episode of acute rheumatic fever (ARF), or already have established rheumatic heart disease (RHD), are at high risk of recurrence of ARF if further episodes of group A streptococcal (GAS) infection occur. Secondary prevention of ARF and RHD is therefore warranted ( Box 11.1 ). A holistic view of secondary prevention comprises…

Primary Prevention of Acute Rheumatic Fever and Rheumatic Heart Disease

Primordial Prevention Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) are predominantly diseases of social, environmental, and economic poverty. Primordial prevention, defined as improving socioeconomic and living conditions, and having well-organized, effective health systems, is arguably the most important and effective population-based strategy for prevention of both ARF and RHD. Even before the introduction of penicillin in the 1940s and 1950s, the incidence of ARF…

Rheumatic Heart Disease in Pregnancy

Introduction Heart disease is the highest indirect (nonobstetric) cause of mortality for pregnant women in both high-income countries (HICs) and low- and middle-income countries (LMICs). Valvular heart disease is an important contributor to the burden of maternal disease, accounting for 15% of pregnancy-related complications in HICs, although this figure is significantly higher in LMICs owing to the much higher prevalence of rheumatic heart disease (RHD). In…

Surgical Management of Rheumatic Valvular Heart Disease

Introduction New cases of acute rheumatic fever (ARF) have almost disappeared from most high-income countries (HICs), where rheumatic heart disease (RHD) is now a remnant of the past and usually only seen in the older population where it does not represent a problem significantly different from that of other etiologies of valve disease. By contrast, RHD remains an important health burden in low- and middle-income countries…

Catheter-Based Evaluation and Treatment of Rheumatic Heart Disease

Introduction Rheumatic heart disease (RHD) remains the most common cause of multivalvular heart disease worldwide, and a major cause of cardiac morbidity and mortality in low- and middle-income countries. The definitive management of patients with advanced rheumatic valvular disease includes replacement, repair, or mechanical opening of stenotic valves by either percutaneous (catheter-based) or surgical means. Appropriate management of sequelae such as atrial fibrillation and heart failure…

Medical Management of Rheumatic Heart Disease

Introduction Chronic ambulatory heart failure (HF) is usually the most common clinical manifestation of advanced rheumatic heart disease (RHD) . HF in RHD patients typically develops after a chronic, often asymptomatic, period of progressive valvular heart disease, typically manifesting clinically only when the valve disease is severe (or less than severe in mixed or multivalve disease). The pathophysiology of rheumatic valvular lesions and how this relates…