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Aortic Regurgitation

Etiology Aortic regurgitation (AR) rarely occurs as an isolated lesion, but is a common finding in patients with concomitant congenital heart lesions. It can result from primary disease of the aortic leaflets or secondary to pathology of the aortic root and surrounding structures ( Table 37.1 ). TABLE 37.1 Etiology of Aortic Regurgitation Aortic Valve Disease Pathologic Process Rheumatic fever Cusps become infiltrated with fibrous tissue…

Subvalvular and Supravalvular Aortic Stenosis

Patients with left ventricular outflow tract (LVOT) obstruction comprise a diverse group of neonates, infants, children, and young adults, accounting for up to 6% of adults with congenital heart disease (CHD). Congenital LVOT obstruction can occur at three levels; in approximately 50% of cases the obstruction is valvar, in around 30% it is subvalvar, and in the remainder it is supravalvar or multilevel. A small proportion…

Valvular Aortic Stenosis

Left ventricular outflow tract obstruction accounts for 5% to 10% of all congenital heart defects and may be due to stenosis at a valvular, subvalvular, or supravalvular level. The obstruction can be isolated or occur at multiple levels and is often associated with other cardiac abnormalities. Valvular aortic stenosis (AS) is by far the most common type of left ventricular outflow tract obstruction and has three…

Partial Anomalous Pulmonary Venous Connections and the Scimitar Syndrome

Acknowledgments I am deeply indebted for the mentorship, comments, and editorial guidance given to me by Drs. Gary Webb and Charles Bullaboy. Furthermore, I am indebted to my wife and children for affording me the time away from them to write this chapter and the support to see it to completion. Lastly, I am grateful to Dr. Harold Litt for providing some of the CT and…

Mitral Valve Prolapse, Mitral Regurgitation

In 1966, Barlow and Bosman described a constellation of clinical findings consisting of nonejection systolic clicks and a late systolic murmur, T-wave abnormalities, and systolic aneurysmal billowing of the posterior mitral leaflet into the left atrium. Since then, in areas without rheumatic heart disease, mitral valve prolapse (MVP) has been portrayed as the most common form of valvular heart disease. It is characterized by pathologic anatomic…

Cor Triatriatum and Congenital Mitral Stenosis

Introduction The main causes of congenital left atrial and ventricular inflow obstruction are pulmonary vein stenosis, cor triatriatum sinister (CTS), and mitral stenosis. Congenital pulmonary vein stenosis is usually a severe disease presenting in infancy with rare adult survival. This chapter focuses on CTS and congenital mitral stenosis. Cor Triatriatum Definition and Epidemiology Cor triatriatum is a rare developmental anomaly in which a membrane divides the…

Atrioventricular Septal Defects

Definition and Morphology Definitions Atrioventricular septal defects (AVSDs) encompass a spectrum of cardiac anomalies. The hallmark feature is a five-leaflet atrioventricular (AV) valve with a common AV annulus that guards a common AV orifice, or separate left and right AV valve orifices ( Fig. 31.1 ). Synonyms for AVSD include atrioventricular canal defect and endocardial cushion defect . Morphology International Pediatric and Congenital Cardiac Code Classification…

Ventricular Septal Defect

Definition and Morphology Ventricular septal defects (VSDs) are openings in the ventricular septum and occur both in isolation and in conjunction with other cardiac defects. The classification of VSDs is based on the location of the defect within the ventricular septum. The ventricular septum can anatomically be considered as having two components, the membranous and the muscular septum ( Fig. 30.1 ). The membranous septum is…

Atrial Septal Defect (Interatrial Communication)

Definition and Morphology An atrial septal defect (ASD) is a direct communication between the cavities of the atrial chambers that permits shunting of blood. In the normal heart the true atrial septum is within the boundaries of the oval fossa; the majority of the remaining tissue separating the atrial chambers is composed of an infolding of the atrial wall. The morphology of the various types of…

Insurability of Adults With Congenital Heart Disease

A 30-year-old woman with tetralogy of Fallot (TOF) repaired at age 5 years was seen in the adult congenital heart disease (ACHD) clinic. Her physician gave encouraging advice: there was no arrhythmia, the pulmonary regurgitation was only moderate, and right ventricular function was close to normal. With this favorable report, the woman asked why an insurer had recently more than doubled her premium for life insurance…

Anesthesia in Adult Congenital Heart Disease, Including Anesthesia for Noncardiac Surgery

Congenital heart disease is one of the most common birth defects, and with 85% of these children now surviving into adulthood, an increasing number of patients require ongoing care. Many, but not all, will have had some corrective or palliative surgery in infancy or childhood and will require further surgery throughout their life. Approximately 40% of patients will have had curative treatment for a simple lesion…

Supportive and Palliative Care for Adults With Congenital Heart Disease

Acknowledgments We wish to thank Professor Adrienne H. Kovacs for the thorough and careful review of the manuscript. A 52-year-old man with severe Ebstein anomaly, atrial septal defect, and chronic cyanosis was followed for almost three decades as an out- and inpatient. He is now admitted with heart failure and shortness of breath with minimal activity. When diagnosed with cardiogenic shock in the emergency room, he…

Psychosocial Issues in Adult Congenital Heart Disease

Introduction Advances in pediatric and interventional cardiology, intensive care medicine, and cardiac surgery have resulted in tremendous improvements in life expectancy of patients born with a heart defect. Due to the decreased mortality in patients with congenital heart disease (CHD), the interest of health care workers and researchers in long-term functioning and quality of life (QOL) of afflicted patients has increased greatly. Indeed, many of these…

Quality of Life in Patients With Pulmonary Hypertension and Nursing in Adults With Congenital Heart Disease

The term pulmonary hypertension (PH) is broad and applicable to a group of conditions. The early 1990s saw major changes in the understanding of the spectrum of disease affecting the pulmonary circulation. We have also seen the central place of issues such as understanding, capturing, and directing treatment pathways towards improving the health-related quality of life (HRQoL) for PH patients. This has led to an increased…

Obstetric Analgesia and Anesthesia

Pregnancy in any woman is a physiological challenge, but in women with congenital heart disease (CHD) it may pose considerable risks to both mother and fetus. Cardiac disease remains the most common cause of indirect maternal death in the United Kingdom, with mortality rates significantly higher in the latest report of the Confidential Enquiries into Maternal Deaths than those in the 1980s (RR: 2.22, 95% CI:…

Pregnancy and Contraception

Heart malformation is the most common form of congenital abnormality, occurring in approximately 0.8% of all babies born. Following improvements in surgery and medicine since the 1960s, most people with congenital heart disease now survive to adulthood, and about half of them are women. Most would like to have children. At one time it was common for women with congenital heart disease to be advised against…

Transition of Care From Pediatrics

For the first time in history, there are now more adults than children living with congenital heart disease (CHD) in North America. Adults with CHD of moderate or great complexity, however, remain at significant risk of heart failure, arrhythmias, additional surgeries and interventional procedures, and premature mortality. This population thus requires specialized CHD care during childhood, adolescence, and adulthood. There are known challenges in retaining these…

Pacemakers and Internal Cardioverter Defibrillators in Adult Congenital Heart Disease

Implantable Cardiac Devices and Congenital Heart Disease Survival of children with congenital heart disease (CHD) continues to improve in lockstep with advances in surgical and medical therapy, and more than 90% now reach adulthood. Despite the impressive anatomic repairs that are now achievable, even adults with fully repaired CHD cannot be regarded as having normal hearts, and many will be prone to arrhythmias. More and more…

Invasive Electrophysiology

Patients who have undergone repair of congenital heart disease are at risk of atrial and ventricular tachyarrhythmias and sudden cardiac death (SCD), both because of their arrhythmia substrate and their altered hemodynamic response to it. While patients with even complex cardiac defects now have a realistic chance to survive into adulthood, cardiac arrhythmias are a very common source of morbidity and mortality in this patient group…

Arrhythmias in Adult Congenital Heart Disease

The great successes of congenital heart surgery have created a new class of cardiology patient: the adult with congenital heart disease (CHD). It is estimated that there are nearly three million patients older than 18 years of age with CHD in Europe and North America, and for the first time ever there are more adults living with congenital heart defects than children with CHD. Cardiac arrhythmias…