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Surgical Interventions for Congenital Heart Disease

Our understanding of the complexities of congenital heart disease, which is a deviation from normal cardiac anatomic development that affects 8 in 1000 births, has progressed immensely since the establishment of the Board of Pediatric Cardiology in 1961. Improvements in diagnostic imaging (including echocardiography, cardiac angiography, and MRI) and innovations in surgical repair techniques have resulted in greatly improved outcomes for children with congenital heart disease.…

Catheter-Based Therapies for Adult Congenital Heart Disease

Sometime at the turn of the millennium, due to improved treatment and outcomes, the prevalence of congenital heart disease in adults surpassed that of children. With longer term survival, this disproportionate share of adult patients will continue to grow. Many of these patients will have stable mild disease with a favorable natural history. However, a substantial number of patients will require close surveillance and specialized treatment…

Clinical Presentation of Adults With Congenital Heart Disease

Etiology and Pathogenesis Congenital heart disease refers to any heart defect present at birth. The etiology of such defects is multifactorial, with environmental and genetic factors playing important roles. In utero development can be affected by metabolic stress from toxins, hypoxemia, and medications. Chromosomal abnormalities, such as trisomy 21, or inherited single gene disorders can also play important roles. Congenital heart disease therefore can present along…

Surgical Treatment of Valvular Heart Disease

Competency of the atrioventricular valves allows blood to enter the ventricles, where pressure is generated. When adequate systolic blood pressure is generated, the aortic and pulmonary valves open, allowing blood to enter the arterial system. The atrioventricular valves close, preventing the flow of blood into the atria. During diastole, the aortic and pulmonary valves close, the atrioventricular valves open, the ventricles fill, and ultimately begin the…

Tricuspid and Pulmonic Valve Disease

Acquired disease of the right-sided cardiac valves is much less common than disease of the left-sided valves, possibly because of the relatively lower pressures and hemodynamic stress to which the right-sided valves are subjected. Indeed, right-sided valvular dysfunction most commonly occurs when morphologically normal valves are subjected to abnormal hemodynamic stresses (e.g., pulmonary hypertension) but can also be seen with congenitally malformed valves (tricuspid and pulmonic…

Cardiovascular Manifestations of Rheumatic Fever

Etiology and Pathogenesis Acute rheumatic fever (ARF), also known as scarlet fever, is caused by an autoimmune reaction to group A β-hemolytic streptococcal pharyngitis, which is caused by the common childhood infection of bacterial streptococcal pharyngitis, also known as “strep throat.” It typically affects children between the ages of 5 and 15 years. Group A streptococcus is a bacteria that lives in oral flora and is…

Mitral Valve Disease

The origin of the word mitral comes from the Latin mitre , which means “bishop or Pope's hat” due to the physical resemblance between them. The mitral valve is a bicuspid valve, that is, it consists of two leaflets that separate and coordinate the flow of blood between the left atrium (LA) and the left ventricle (LV). During diastole, LA pressure surpasses LV pressure, which allows…

Aortic Valve Disease

The aortic valve is a semilunar valve that includes three pocket-like cusps of approximately equal size. The normal aortic valve opens completely during systole, allowing ejection of blood from the left ventricle (LV), whereas closure during diastole prevents retrograde blood flow from the aorta into the LV. Dysfunction of the valve can lead to either impairment of LV outflow or valvular incompetence with regurgitation, either of…

Cardiac Pacemakers and Defibrillators

Technological advances have improved the versatility and function of implantable devices used to treat bradyarrhythmias and tachyarrhythmias. Surgical placement of pacemakers and implantable cardioverter-defibrillators (ICDs) can be performed on an outpatient basis, with low risk and minimal morbidity, which allows most patients to return to full functional capacity quickly. Indications for Implantation of Cardiac Rhythm Devices Pacemakers Pacemakers are indicated primarily for patients with symptomatic bradycardia…

Syncope

Syncope is a transient, self-limited loss of consciousness and voluntary muscle tone. It is typically followed quickly by spontaneous recovery of consciousness. The incidence rate of syncope varies between 0.80 and 0.93 per 1000 person-years. Syncope accounts for 3% to 5% of emergency department visits and 1% to 3% of hospital admissions. There is an increased association with acute illness and noxious stimuli. There is also…

Sudden Cardiac Death

Sudden cardiac death (SCD) is defined as any death from a cardiac cause occurring within an hour of symptom onset. The term sudden cardiac arrest (SCA) refers to an event from which an individual is resuscitated or spontaneously recovers. Sudden unexpected death has many potential etiologies ( Box 42.1 ). Patients with coronary artery disease (CAD) and previous myocardial infarction (MI) have an annual incidence of…

Ventricular Arrhythmias

Ventricular arrhythmias originate from the distal conduction system (distal to the His bundle) or ventricular myocardium, and with few exceptions, present with a wide QRS morphology on ECG. These may occur as isolated premature ventricular contractions (PVCs), couplets (2 consecutive PVCs), or as ventricular tachycardia (VT) (≥3 consecutive beats with a rate of >100 beats/min). Nonsustained VT refers to ≥3 self-terminating episodes that last between 3…

Invasive Management of Atrial Fibrillation and Atrial Tachycardias

Although they were once considered investigational techniques, catheter and surgical ablation have evolved to become commonly performed procedures in major hospitals worldwide. Catheter ablation is the preferred therapy for typical atrial flutter, and it should be considered as a first-line therapy in select patients with atrial fibrillation (AF) when a rhythm control strategy is being pursued. A decision to pursue invasive management of atrial arrhythmias depends…

Atrial Fibrillation: Stroke Prevention

Etiology and Pathogenesis Approximately 800,000 strokes occur annually in the United States, and it is estimated that atrial fibrillation is the primary cause in approximately 15% of these events. The prevention of stroke remains a critical focus in the initial treatment of atrial fibrillation and should be carefully addressed at the time of presentation after symptoms are controlled, and the patient is able to engage in…

Atrial Fibrillation: Rate Versus Rhythm

Atrial fibrillation (AF) is the most common sustained cardiac rhythm abnormality. The prevalence of AF is increasing because of a combination of factors, including the aging of the population, a rising prevalence of chronic heart disease, and more frequent diagnosis by enhanced monitoring devices. Because it is more common with advanced age, rates of AF in those older than 80 years have been reported to be…

Supraventricular Tachycardia

Supraventricular tachycardia (SVT) is an abnormally rapid heart rhythm originating proximal to the bifurcation of the bundle of His. SVTs are relatively common clinical arrhythmias that present in people of all age groups. They are frequently symptomatic and often result in the patient seeking medical attention on an emergent or nonemergent basis. Vagal maneuvers may be useful to terminate SVTs that are atrioventricular (AV) node–dependent. In…

Bradyarrhythmias

In adults, bradycardia refers to a ventricular rate that is <60 beats This figure is somewhat arbitrary and does not necessarily connote disease. For instance, it is common to find healthy athletes with resting heart rates of approximately 40 beats In general, bradycardia becomes a clinical issue if it correlates with symptoms—syncope, dizziness, exercise intolerance, breathlessness, angina, fatigue, or mental confusion. These correlations can be difficult…

Stress-Induced Cardiomyopathy

Stress-induced cardiomyopathy, also called Takotsubo cardiomyopathy, represents a syndrome of transient left ventricular (LV) dysfunction from a variety of psychological or physiological stressors. Patients in the critical care setting are particularly vulnerable, but ambulatory patients subject to severe emotional distress may also develop stress-induced cardiomyopathy. In the intensive care setting, sepsis, respiratory failure, intracranial hemorrhage, and pancreatitis are a few of the described precipitators. Despite its…

Cardiac Transplantation and Mechanical Circulatory Support Devices

Cardiac transplantation developed in conjunction with research into myocardial protection and heart preservation, which facilitated safe open heart surgery. In 1961, Shumway and Lower published their seminal article describing the technique of orthotopic cardiac transplantation in a canine model, with successful functioning of the transplanted heart for several days. While Shumway was preparing to begin a human clinical trial of cardiac transplantation, Christiaan Barnard, a South…

Myocarditis

Myocarditis is an inflammatory process that can involve one or more components of the myocardium, including cardiomyocytes, the interstitium, and the coronary vasculature. This inflammatory process may result from infectious processes, responses to pharmacological or toxic agents, hypersensitivity reactions, or physical damage. Myocarditis may also be a cardiac manifestation of a systemic disease. The clinical course of myocarditis is as diverse as its etiologies. Most patients…