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Global Burden of Pediatric Heart Disease and Pediatric Cardiac Care in Low- and Middle-Income Countries

An estimated 15 million children die or become disabled annually by treatable or preventable heart disease in low- and middle-income countries (LMICs). Sadly, for 90% of children with heart disease, treatment is either unavailable, unaffordable, or of suboptimal quality. Global efforts to reduce mortality in children younger than 5 years have focused on reducing death from communicable diseases in LMICs, with little to no attention focusing…

Quality Improvement in Congenital Cardiac Disease

Introduction The field of congenital heart disease is expansive in the breadth of patient complexity and exciting in the continuous improvement in patient outcomes. Several tools have been used to achieve improvement in patient outcomes over the past several decades. These tools include pioneering work by congenital heart surgeons, advances in anesthesiology, critical care and nursing, improved diagnostic tools, and innovations in nonsurgical interventional techniques. The…

Growth and Nutrition

Malnutrition Nutrition is fundamental for growth and neurologic development during childhood. However, malnutrition and growth impairment are common worldwide in infants and children with congenital heart disease (CHD). Growth failure is often multifactorial and may be attributable to inadequate energy intake, disordered oral feeding, increased metabolic demands, and disturbances in gastrointestinal (GI) function contributing to malabsorption. Associated genetic syndromes and chromosomal abnormalities may also lead to…

Discharge Planning, Communications With the Team Providing Care in the Ambulatory Setting and the “Medical Home”

Communication between the cardiologist and providers of primary care is an essential component of the medical care of the patient and is of increasing importance in the current era, which is marked by increased complexity of medical testing and polypharmacy. Breakdowns in this vital area of communication can result in delayed or missed diagnoses, repeated or unnecessary testing, increased morbidity and possible mortality, and increased risk…

Clinical Care Pathways Supporting the Care of Children and Young People With Congenital Heart Disease

Introduction Nursing professionals use data and information about their patients’ care journeys to support decision making and the development of evidence-based practice. Care pathways provide a structured care approach using an evidence-based framework. In her work during the Crimean War (1854), Florence Nightingale (1820–1910) demonstrated a commitment to robust data collection to improve patients’ care experiences. Data and evidence-based practice are key drivers toward the improved…

Ethical Issues in Pediatric Cardiology and Congenital Heart Disease

Introduction Although descriptions of congenitally malformed hearts date back more than 200 years, the field of pediatric cardiology began to rapidly change in the 1950s, when surgical interventions to treat congenital heart disease (CHD) were created. The development of cardiopulmonary bypass was revolutionary and allowed treatment of previously untreatable diseases. These were innovative, exciting, and challenging times. However, many if not all innovative contributions such as…

Psychological and Social Aspects of Pediatric Cardiac Disease

Introduction What outcomes really matter to patients, families, and societies living with the implications of congenital and acquired pediatric cardiac disease? Survival and physiologic function are certainly paramount. Dramatically improved survival and reduced morbidity for virtually all congenital and acquired pediatric heart lesions have now shifted focus to the question of quality versus quantity of life. The patients whom we follow are not “hearts”; they are…

Preparing the Young Adult With Complex Congenital Cardiac Disease to Transfer From Pediatric to Adult Care

More than 90% of children born with congenitally malformed hearts now survive to adulthood, as a result of the spectacular achievements of pediatric cardiology and surgery over the past half-century. This has created a new and growing population of adults who often have complex congenital cardiac malformations that are unfamiliar to conventionally trained adult cardiologists. The need for an integrated strategy of care for such patients…

Pregnancy and Congenital Heart Disease

The successes of pediatric cardiology and cardiac surgery have enabled a new cohort of women, born with congenitally malformed hearts, to reach adulthood. Many of these women are now considering pregnancy. Most women in this new cohort can anticipate safe and successful pregnancies. Pregnancy, however, imparts an additional hemodynamic load, is prothrombotic, and increases the propensity to arrhythmias, all of which increase the risk of adverse…

Pharmacogenetics in the Treatment of Congenital and Acquired Cardiac Disease During Childhood

Introduction Pediatric cardiology providers commonly observe variable responses to cardiovascular pharmacotherapy and, as a result, inconsistent or counterintuitive outcomes in congenital and acquired heart disease. The contribution of development and/or genetic variation to the observed variability in cardiovascular drug disposition and response in childhood requires further investigation and clarity. In the absence of data that are applicable to the individual child, providers making clinical decisions must…

Acute and Chronic Renal Consequences of Cardiac Disease in Children

Introduction The complex interplay between the cardiovascular and renal systems has received significant clinical, translational, and basic research attention over the past 2 decades. In both the acute and chronic care settings, dysfunction of either the heart or the kidneys can lead to dysfunction of the other organ. While this interaction has been obvious and well known in severe cases (e.g., advanced kidney failure leading to…

Congenital Cardiac Disease in the Setting of Genetic Syndromes

The majority of congenital heart defects (CHDs) occur as isolated malformations, whereas approximately 25% to 30% of them are associated with extracardiac anomalies, in the setting of large or submicroscopic chromosomal anomalies, monogenic mendelian disorders, and genetic associations. Furthermore, recent studies reported a higher frequency (approximately 40%) of detecting an underlying disorder when the cohort studies were done on patients with cardiac defects requiring invasive or…

Neurodevelopmental and Psychosocial Outcomes in Children and Young Adults With Complex Congenital Cardiac Disease

Introduction Prior to the early 1980s, it was uncommon for children with complex congenital heart disease (cCHD) to survive into later childhood. The nearly simultaneous advances in congenital heart surgery, echocardiography, and intensive care medicine were coupled with the availability of prostaglandins and the developing discipline of interventional cardiology. Together, these factors resulted in a dramatic fall in surgical mortality, with complex repairs taking place at…

Pulmonary Hypertension

Pulmonary Hypertension Pulmonary hypertension describes a number of different diseases involving the pulmonary vasculature, which have common hemodynamic, histologic, and therapeutic features. The hallmark of these diseases is an abnormally high pulmonary vascular resistance. The histologic changes such as medial hypertrophy and intimal fibrosis do not vary significantly between different etiologies, whether the cause is idiopathic, hemodynamic, inflammatory, or toxin mediated. The hemodynamic and clinical consequences…

Systemic Circulation

Introduction The systemic circulation carries blood from the systemic ventricle through a network of arteries and arterioles to the tissue capillaries and drains it via the systemic venous system to the systemic venous atrium. The systemic arterial system serves two important functions. First, it acts as a low-resistance conduit through which blood is distributed to different parts of body. Second, the arterial tree buffers the pulsatile…

Longer-Term Outcomes and Management for Patients With a Functionally Univentricular Heart

Evolution of the Fontan Procedure The Fontan operation is the treatment of choice for patients with a single anatomic or functional ventricular chamber. From the late 1940s, survivors with a functionally univentricular heart could be palliated with a systemic-to-pulmonary artery or Glenn shunt, but by the early 1970s, only 50% of those with tricuspid atresia—the most favorable form of functionally univentricular heart—survived 15 years ( Fig.…

Interstage Management

Introduction Several fundamentals of perioperative care for shunt-dependent dual-distribution circulation (see Chapter 70 ) extend to transitional care from the intensive care unit and throughout the interstage period in an effort to preserve organ function and promote somatic growth. Conventional outpatient surveillance limited to vital signs and routine growth assessment has historically been associated with high interstage mortality rates after discharge from Norwood or stage I…

Fontan Pathway From Birth Through Early Childhood

Good judgement comes from experience. Experience comes from bad judgement. ANONYMOUS Half of what I'm teaching you is wrong. The problem is that I don't know what half it is. ANONYMOUS Introduction Some of the most challenging forms of congenital heart disease (CHD) fall into the category of those with functionally one pumping chamber, colloquially known as “single ventricle” defects, although we prefer the term functionally…

Physiologic Principles to Maximize Outcome in Patients With a Functionally Univentricular Heart

Introduction The advances made in the management of newborns and infants with all forms of critical congenital heart disease (cCHD) over the past 3 decades have truly been among the triumphs of cardiac care. In all forms of cCHD, nearly simultaneous, cumulative, and synergistic advancements have been made in surgical strategies, anesthesia and bypass techniques, critical care, bedside nursing care, imaging, and catherization; importantly also, an…

Anatomic Considerations in the Functionally Univentricular Heart

Introduction For the anatomist and pathologist, the terms “univentricular” and “single” have proven to be among the most controversial words used to describe a congenitally malformed heart. Since the mid-1990s, light has begun to emerge at the end of the tunnel for those seeking a logical framework for the use of these words. In no small way, this reflects the recognition by clinicians that it is…