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Critical Care

Increasingly, more patients with congenital heart disease (CHD), including those with more complex disease, survive into adulthood. They present to critical care physicians by virtue of their underlying cardiac disease, following surgical or cardiologic intervention to replace failing valves and conduits or with unrelated reasons such as pregnancy or surgery for noncardiac conditions. Moreover, CHD is associated with a range of other noncardiac pathologies ( Table…

Noncardiac Surgery in Adult Congenital Heart Disease

Every year, 4% of the population undergoes major surgery, defined as an intervention occurring in a hospital operating theater and usually requiring anesthesia for pain control. Worldwide, the mortality rate in noncardiac surgery is 0.8% to 1.5%, and complications occur in 7% to 11% of all inpatients. More than 40% of all complications are cardiac related. Comprehensive guidelines exist to direct the cardiovascular assessment and management…

Heart and Lung Transplantation in Adult Congenital Heart Disease

Many patients who survive into adulthood with congenital heart disease (CHD) will develop progressive cardiopulmonary dysfunction. Some will develop ventricular failure, some will have pulmonary hypertension, and many will experience progressive cyanosis. Among these patients, some will require cardiac replacement, others will need heart-lung transplantation, and a few may be suitable for lung transplantation with repair of the congenital defect. In many, the risks of transplantation…

Late Complications Following the Fontan Operation

The univentricular heart encompasses a spectrum of rare and complex congenital cardiac malformations whereby both atria predominantly egress into one functionally single ventricular chamber, precluding biventricular repair. Population studies indicate an overall prevalence of approximately 2 per 10,000 live births. Subtypes include hypoplastic right or left ventricles, absence or atretic atrioventricular (AV) valves, common AV valves with only one well-developed ventricle, and heterotaxy syndromes (or isomerism),…

Venous Shunts and the Fontan Circulation in Adult Congenital Heart Disease

Venous shunts are surgical reconstructions involving an anastomosis between one or both venae cavae to one or both pulmonary arteries (PAs), and were developed to palliate infants born without two ventricular chambers. Staging venous shunts are typically performed during infancy and childhood, and include the older Glenn shunt, which anastomosed the superior vena cava to the left PA, and the more recent bidirectional cavopulmonary shunt, which…

Late Repair and Reoperations in Adult Congenital Heart Disease

The number of adult patients with congenital heart disease (CHD) is increasing due to improving outcomes after neonatal and infant surgery. Some 85% of neonates with CHD survive into adult life. The majority of adults with CHD require lifelong cardiologic surveillance in tertiary centers: many patients require further surgical management related both to the underlying diagnosis and the techniques used in the initial reparative surgery. The…

Cardiac Catheterization in Adult Congenital Heart Disease

Acknowledgment Dr. Horlick is supported by the Peter Munk Chair in Structural Heart Disease. Cardiac catheterization remains a fundamental modality for the diagnosis and interventional treatment of adult congenital heart disease (ACHD); however, there has been a noticeable change in case mix and clinical demands for the ACHD interventionalist over the past several decades. Advances in imaging such as three-dimensional (3D)-echocardiography and the exquisite resolution of…

Cardiac Computed Tomography

Over the past few decades, advances in pediatric cardiology and cardiac surgery have revolutionized the prospect for patients with adult congenital heart disease (ACHD). Although cardiovascular magnetic resonance imaging (CMR) and transthoracic echocardiography (TTE) remain the techniques of choice for their routine assessment and follow-up, advances in cardiac computed tomography (CCT) have led to its emergence as both a complementary technique and an alternative to CMR…

Cardiovascular Magnetic Resonance

Cardiovascular magnetic resonance (CMR) gives unrestricted access to the heart and great vessels noninvasively and without ionizing radiation. It can provide biventricular functional assessment, flow measurement, myocardial viability assessment, angiography, and more, and is therefore recommended for long-term follow-up in adult congenital heart disease (ACHD). Transthoracic echocardiography remains the first-line approach to imaging the hearts of patients, and provides a relatively rapid and comprehensive evaluation of…

Heart Failure, Exercise Intolerance, and Physical Training

Adults with congenital heart disease (ACHD) are an expanding population who pose a significant challenge to the medical professionals who are caring for them. Although early surgery has transformed the outcome of these patients, it has not been curative. Exercise intolerance is a major problem for ACHD patients and significantly affects their quality of life. Physical limitation is common, even in patients with simple lesions, and…

Echocardiography

Most patients with congenital heart lesions are diagnosed in infancy or childhood and then undergo palliative and/or reparative surgery. Despite ongoing advances in cardiac surgery and intervention, residual anatomic and hemodynamic abnormalities remain common among such individuals. A large proportion of patients with congenital heart disease (CHD) need lifelong follow-up in specialized centers. Echocardiography plays an important role not only in the initial diagnosis but also…

Clinical Assessment

† Deceased. Congenital malformations of the heart, by definition, originate in the embryo, then evolve during gestation, and change considerably during the course of extrauterine life. Before World War II, these malformations were regarded as hopeless futilities. Abbott was advised by William Osler to devote herself to the anatomic specimens in the collection at McGill University, and Helen Taussig was advised to occupy herself with the…

Adults With Congenital Heart Disease: A Genetic Perspective

As a result of the genetic revolution, the impact of genetics must be considered in the diagnosis, management, and treatment of the patient populations of most specialty clinics. It is likely that genetic information will eventually transform the definitions and taxonomy of congenital heart disease (CHD) used in daily practice. As we learn to apply genetics to risk assessment and develop a better understanding of pathogenesis…

Cardiac Morphology and Nomenclature

The care of adults with congenital heart malformations has evolved into a specialty in its own right. The malformations are conceived by the general cardiologist as extremely complex, requiring a sound knowledge of embryologic development for their appreciation. The defects are so varied, and can occur in so many different combinations, that to base their descriptions on embryologic origins is at best speculative and at worst…

Adults With Congenital Heart Disease: A Growing Population

Congenital heart disease (CHD) lesions occur during embryonic development and consist of abnormal formations of the heart walls, valves, or blood vessels. The dramatic improvement in CHD diagnosis and continued progress of CHD interventions since the 1960s have resulted in a growing population of adults who require cardiac and noncardiac services. As a result of the confluence of success in pediatrics, medicine, and surgery, adult CHD…

The Quality Chasm: Diabetes Mellitus

Over the past several decades there has been much interest in refining the definition of quality of health care, moving the definition beyond an individual’s perception of quality. A pivotal article by Avedis Donabedian in 1988 introduced the concept that quality of care can and should be measurable and that there will be multiple dimensions to identifying high-quality care depending on the perspective from which one…

Disparities in Diabetes Risk, Cardiovascular Consequences, and Care: Women, Ethnic Minorities, and the Elderly

Disparities in Diabetes Risk Type 2 diabetes is a growing problem that closely parallels the obesity epidemic and places a severe burden on health care resources in the United States (see also Chapter 1 ). Diabetes currently affects 25.8 million Americans, 8.3% of the United States population, approximately 95% of whom have type 2 diabetes. , The lifetime risk of developing type 2 diabetes for individuals…

Cardiovascular Autonomic Neuropathy

Cardiovascular autonomic neuropathy (CAN) is defined as the impairment of autonomic control of the cardiovascular system in the setting of diabetes after exclusion of other causes. CAN is usually detected at a subclinical stage by means of several cardiovascular autonomic reflex tests and may affect patients with type 1 or type 2 diabetes mellitus (T1DM or T2DM). Poor glycemic control is a major determinant of this…

Cerebrovascular Disease in Patients with Diabetes

Stroke—in particular the ischemic subtype—is one of the major vascular manifestations of diabetes, together with coronary heart disease (CHD), peripheral arterial occlusive disease, and diabetic retinopathy. The relationship between hyperglycemia and stroke is bidirectional: on the one hand, diabetic patients exhibit more than a twofold risk of ischemic stroke compared with patients without diabetes (see the later discussion of epidemiology of stroke in diabetes), even after…