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Clinical Evaluation and Diagnosis of Acute Rheumatic Fever

Introduction The diagnosis of acute rheumatic fever (ARF) relies on a combination of clinical evaluation and laboratory studies. Diagnosis is usually made using the clinical criteria first formulated by T. Duckett Jones in 1944, which have subsequently undergone multiple modifications, most recently in 2015. Acute fever and arthritis characterize the usual presentation of the disease, although in many patients it may not be acute, rheumatic, or…

Pathogenesis of Acute Rheumatic Fever

Acknowledgments Research grants to MWC: (a) HL35280 and HL56267 from the National Heart Lung and Blood Institute and National Institute of Mental Health Bench to Bedside; (b) grants from the Oklahoma Center for the Advancement of Science and Technology and the American Heart Association. Declaration of financial interest: MWC is a chief scientific officer and consultant at Moleculera Labs, a company offering diagnostic testing for antineuronal autoantibodies…

Epidemiology, Risk Factors, Burden and Cost of Acute Rheumatic Fever and Rheumatic Heart Disease

Introduction Acute rheumatic fever (ARF) is an immune-mediated, nonsuppurative consequence of group A β-hemolytic streptococcus (GAS) infection. Although episodes of ARF can result in significant disability, the major impact of ARF at the population level is that it causes long-term, irreversible damage to heart valves—termed rheumatic heart disease (RHD)—often as a result of recurrences. For this reason, we discuss the epidemiology of two conditions together emphasizing…

Quality Improvement: Surgical Performance

Background According to the Centers for Disease Control and Prevention (CDC), congenital heart disease (CHD) is the most common type of birth defect, affecting 1% of the population (40,000 births/year). CHD is the leading cause of illness and death associated with birth defects. Fifteen percent of all patients with CHD have associated genetic conditions, and 20% to 30% have physical, developmental, or cognitive problems. In 2004,…

Quality Improvement for the Treatment of Patients with Pediatric and Congenital Cardiac Disease: The Role of the Clinical Database

Although significant progress has been made in the care of patients with pediatric and congenital cardiac disease, complications and death still occur. As a result, optimization of outcomes remains a constant goal. Substantial effort has been devoted to advancing the science of assessing the outcomes and improving the quality of care associated with the treatment of patients with pediatric and congenital cardiac disease. The importance of…

Arrhythmia and Pacemaker Surgery in Congenital Heart Disease

The progress in the management of cardiac arrhythmias related to congenital heart disease (CHD) over the past 40 years mirrors the improvement in outcomes associated with heart disease in children. During the earliest era in the management of CHD, postoperative arrhythmias and heart block were a substantial cause of perioperative mortality. With the advent of direct myocardial pacing and cardiac defibrillation more than 50 years ago,…

Adult Congenital Cardiac Surgery

Patients with congenital heart disease require lifelong surveillance. The number of adults living with congenital heart disease continues to grow, in part because of advances in the diagnosis and treatment of congenital heart lesions. There are more than 1 million adults with congenital heart disease in the United States; this population grows by approximately 5% each year. The challenges of caring for adults with congenital heart…

Ebstein Malformation

Historical Aspects In an 1866 report titled “Concerning a very rare case of insufficiency of the tricuspid valve caused by a congenital malformation,” Dr. Wilhelm Ebstein (1836-1912), a German physician, described the unusual cardiac findings of a 19-year-old laborer who died of cyanotic heart disease. He accurately described the characteristic anatomic and hemodynamic abnormalities of Ebstein malformation ( Fig. 130-1 ). By 1950, only three case…

Management of Single Ventricle and Cavopulmonary Connections

Single-ventricle circulation is a generic term that covers a wide range of structural cardiac abnormalities. It is widely accepted that the definitive surgical palliation for these hearts is the Fontan circulation, whereby the pulmonary and systemic blood flows are in series with the single ventricle connected to the systemic circulation. To optimize clinical outcomes with the Fontan, many patients need prior interventions to adjust the pulmonary…

Hypoplastic Left Heart Syndrome

Hypoplastic left heart syndrome (HLHS) is characterized by a generalized underdevelopment of the left ventricle and its dependent structures: the mitral valve, the aortic valve, and the preductal and ductal aorta. Because the anatomic severity of HLHS constitutes a spectrum of disease, the value of a consistent, defined nomenclature has been recognized. Such a nomenclature has been proposed by the Congenital Heart Surgery Nomenclature and Database…

Congenital Anomalies of the Mitral Valve

This chapter treats only the congenital anomalies of the mitral valve to the exclusion of the mitral valve in atrioventricular (AV) discordance, the mitral valve in univentricular hearts, and the mitral valve of the hypoplastic left heart syndrome. It also excludes all acquired mitral valve disease, including the mitral valve insufficiency secondary to myocardial infarction or stunning as seen in anomalous origin of the left coronary…

Surgery for Congenitally Corrected Transposition of the Great Arteries

Congenitally corrected transposition of the great arteries (ccTGA) is a rare defect, seen in approximately 0.5% of patients with congenital heart defects. It entails discordance of the atrioventricular (AV) connections and discordance of the ventricular arterial connections, so there is double discordance. The morphology of the heart is distinctly abnormal, but the circulatory system is physiologically normal. Commonly, ccTGA is associated with ventricular septal defects (VSDs),…

Transposition of the Great Arteries: Simple and Complex Forms

History Transposition of the great arteries (TGA) was first recognized and described more than 2 centuries ago. Although reference was made to malposition of the aorta and pulmonary artery by Steno in 1672 and Morgagni in 1761, the anatomic description of TGA is credited to Baillie in 1797. It was Farre in 1814 who introduced the term transposition of aorta and pulmonary artery, meaning that the…

Surgery for Congenital Anomalies of the Coronary Arteries

This chapter discusses the management of coronary artery anomalies in patients without other congenital heart defects. Most coronary artery anomalies in number, origin, and distribution are of intellectual interest only. However, a few are clinically significant because they may result in myocardial ischemia, left ventricular dysfunction, and sudden death. Anomalies discussed in this chapter include anomalous origin of a coronary artery from the pulmonary artery, anomalous…

Surgery for Congenital Anomalies of the Aortic Valve and Aortic Root

Congenital aortic valve disease is one of the more commonly encountered congenital cardiac defects, occurring in 3% to 6% of children born with congenital heart disease. Congenital aortic valve disease primarily manifests as aortic stenosis and tends to be a progressive disease with morbidity and mortality resulting from the hemodynamic burden imposed on the left ventricle. Aortic stenosis is generally defined as a narrowing at the…

Interrupted Aortic Arch

Interrupted aortic arch (IAA) is a rare genetic disorder of the cardiovascular system, present in approximately two cases per 100,000 live births and comprising 1.5% of all cases of congenital heart disease. Its hallmark feature is a lack of luminal continuity between the ascending aorta and the descending aorta. In the absence of surgical repair, mortality approaches 75% in the first month of life and 90%…

Truncus Arteriosus and Aortopulmonary Window

Truncus Arteriosus Truncus arteriosus is a relatively rare congenital heart defect with a single vascular trunk arising from the heart, giving origin to the true pulmonary arteries, aorta, coronary arteries, and brachiocephalic vessels. The lesion accounts for approximately 0.4% to 4.0% of all congenital heart lesions. Truncus arteriosus was first described by Wilson in 1798. In 1942, Lev and Saphir defined the anatomy currently associated with…

Pulmonary Atresia with Ventricular Septal Defect and Right Ventricle–to–Pulmonary Artery Conduits

Pulmonary Atresia with Ventricular Septal Defect Pulmonary atresia with ventricular septal defect (PA/VSD) is a congenital cardiac malformation characterized by discontinuity of blood flow from the right ventricle to the pulmonary arteries, a ventricular septal defect (VSD) resulting from anterior deviation of the infundibular (conal) septum, and an overriding aorta. Because it shares many attributes of tetralogy of Fallot, it is also referred to as tetralogy…

Tetralogy of Fallot with Pulmonary Stenosis

History Stensen, in 1672, described for the first time the anatomic features of what is now termed tetralogy of Fallot (TOF). In 1888, Etienne-Louis Arthur Fallot published his findings describing the four features of the congenital cardiac anomaly that bears his name : infundibular pulmonic stenosis, ventricular septal defect (VSD), dextroposition of the aorta, and right ventricular (RV) hypertrophy. More than 50 years later, the first…

Pulmonary Atresia with Intact Ventricular Septum

Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare congenital heart defect occurring at a rate of 4 to 10 per 100,000 live births. This malformation is characterized by a variably sized right ventricle that has no exit, failing to provide pulmonary blood flow and unable to decompress itself through the interventricular septum. Blood flow through the ductus arteriosus permits survival at birth, but within…