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Anomalous Systemic Venous Return

Introduction This chapter deals with abnormalities of position and connection of the major systemic venous channels that drain to the heart. We also discuss abnormal persistence of the valves of the embryonic systemic venous sinus. The anomalies included vary widely, ranging from asymptomatic variants to those that carry serious hemodynamic significance. The anomalies producing abnormal hemodynamic effects modify both the clinical presentation and the subsequent surgical…

Isomerism of the Atrial Appendages

Introduction It has long been recognized that congenital absence of the spleen, or the presence of multiple spleens, is associated with severe congenital malformations of the heart. Some of the most complex forms of congenital cardiac disease are associated with these splenic abnormalities. Prognosis for many patients with these lesions remains poor, even in the modern era of congenital cardiovascular surgery. If progress is to continue…

Cardiovascular Risk Factors in Youth

Autopsy studies have demonstrated that atherosclerosis, the pathologic basis for coronary artery disease and cerebrovascular events, begins in childhood and is associated with the presence of modifiable and nonmodifiable cardiovascular risk factors (CVRFs) such as obesity, dyslipidemia, and hypertension ( Fig. 25.1 ). These risk factors track into adulthood, and their presence in childhood is associated with subclinical adverse vascular changes that are precursors of overt…

Pediatric Cardiovascular Data, Analysis, and Critical Appraisal of the Literature

Introduction In recent years the volume and variety of pediatric cardiovascular data captured across various sources have continued to expand. These datasets are increasingly being integrated and used for a variety of research and quality improvement purposes. Regardless of the data source, there are several important points to consider in analyzing data or utilizing the literature to guide evidence-based care in this population. In this chapter,…

Cardiopulmonary Exercise Testing

Regular exercise is an important part of a healthy lifestyle. Physical activity in some developed countries has diminished for a variety of reasons as society has trended away from outdoor activities toward other types of activities, such as computer games and television, and jobs have become more sedentary. Healthy levels of physical fitness require regular participation in activities that generate energy expenditures significantly above the resting…

Electrophysiology, Pacing, and Defibrillation

Introduction Pediatric electrophysiology has evolved into a fully recognized subspecialty within the field of pediatric cardiology, and recent growth has led to the continued expansion of this subsection. The aging adult population with congenital cardiac disease, growing in size and in complexity, is increasingly burdened with arrhythmias. Arrhythmic sudden death conditions and the evaluation of victims and families at risk for sudden death have pointed to…

Magnetic Resonance Imaging and Computed Tomography: Diagnostic Imaging, Image Management, and Assessment of Physiology and Function

Introduction Noninvasive cardiovascular imaging plays a fundamental role in the clinical history of patients with congenital heart diseases (CHDs) and acquired cardiac conditions, from antenatal diagnosis to long-term follow-up in adult life. Prior to birth, different noninvasive imaging modalities are used to evaluate cardiac anatomy and physiology, to plan surgical and interventional procedures, and to assess the outcomes and complications. With a reported prevalence of 9…

Three-Dimensional Echocardiography

Introduction Appreciation of complex intracardiac anatomy and spatial relationships is inherent to the diagnosis of congenitally malformed hearts. Until recently, the ability of the clinician to image the heart by echocardiography was limited to two dimensions (2DE). The very nature of a 2DE slice, which has no thickness, necessitates the use of multiple orthogonal sweeps. This requires the echocardiographer to mentally reconstruct the anatomy and translate…

Cross-Sectional Echocardiography and Doppler Imaging

Since the late 1970s, perhaps the most important change in the practice of clinical pediatric cardiology has been the introduction of cross-sectional echocardiography and its subsequent technical improvements. Echocardiography currently allows highly accurate diagnosis of nearly all morphologic abnormalities, for the most part making invasive diagnostic techniques obsolete. Cardiac catheterization and angiography are no longer a routine investigation in most patients prior to cardiac surgery, as…

Interventional Techniques

The practice of interventional cardiac catheterization consists of minimally invasive procedures where catheters are used to modify, palliate, or treat congenital or acquired cardiac disease. Evolution of interventional techniques has been a natural progression from open surgical procedures used to treat such lesions. The development of newer interventional techniques has largely been possible by pushing the boundaries of established interventional procedures to achieve results comparable with…

Diagnostic Catheterization, Including Adults With Congenital Cardiac Disease

Introduction An understanding of the anatomy, physiology, and alternative treatment strategies for congenital heart defects is essential to provide the appropriate patient care. This includes an understanding of the guideline recommendations and the natural history of congenital heart defects. This knowledge is required to determine if there is an indication for treatment of these conditions. Pediatric and adult congenital heart patients offer distinct challenges, such as…

Surgical Techniques

Surgical Approaches to the Heart A variety of incisions are used in cardiac surgery. The majority of pediatric cardiac surgeries are performed through two incisions: the median sternotomy and the posterolateral thoracotomy. Other incisions are occasionally used to accommodate atypical anatomy or allow for minimally invasive approaches. Sternotomy The most commonly used incision continues to be the median sternotomy ( Fig. 16.1 ), in which the…

Prematurity and Cardiac Disease

On average, up to 12% of liveborn infants in the developed world are born prior to term. These rates have increased as a consequence of assisted reproductive technology and earlier intervention for either maternal or fetal well-being. The causes of birth prior to term are many ( Table 15.1 ). The care of the premature neonate has improved dramatically over the past 100 years, during which…

Economic Implications of Congenital Heart Disease in Developed Countries

In developed countries, the last few decades have heralded many advances in the diagnosis and management of congenital heart disease (CHD). These include surgery in neonates and infants; the establishment of pediatric cardiac intensive care; nursing and cardiac anesthesia; subspecialization in interventional catheterization, imaging, electrophysiology, heart failure, and transplantation; and the treatment of adults with CHD. Economically advantaged countries have the tools and knowledge available to…

Prevalence of Congenital Cardiac Disease

Introduction Examination of the frequency of congenital cardiac disease, either as a rate or as a proportion, has important implications for the study of congenital cardiac malformations, as well as their clinical management. However, there is much confusion and misuse regarding terminology and methodology, with important implications for the accuracy, validity, and comparability of findings reported in the published literature. Knowledge of how critically to appraise…

Optimizing Prenatal Support of the Mother and Family

Support of the pregnant mother and her partner is a critical element in the diagnosis and management of congenital heart disease (CHD) in the fetus. From the moment a woman/couple are identified as possibly having a fetus with CHD, to entering the fetal cardiology clinic, through the exam, prenatal counseling, subsequent visits, and the peripartum period, care providers have to be sensitive to and actively seek…

The Placenta in Congenital Heart Disease

Introduction The placenta is one of the most important and least understood organs in the human body. It is a complex, highly organized structure that—amazingly—can be grown, discarded, and fully grown again in a repetitive manner. More than just an interface for fetal oxygenation and nutrition, the placenta is now appreciated to exert important influences that affect health and well-being for the lifespan of the individual.…

Percutaneous Intervention in the Fetus, Including Postnatal Management

Introduction As fetal echocardiography has advanced over the past several decades, our understanding of the natural history of congenital heart disease in utero has progressed. For select lesions that evolve in utero and lead to significant morbidity and/or mortality by the time of birth, relatively simple anatomic modifications may alter the natural history and improve prognosis. This understanding, along with improvements in interventional obstetric and catheterization…

Pharmacologic Intervention in the Fetus

Introduction With the arrival of echocardiography and magnetic resonance imaging (MRI), providing the means of noninvasively detecting and monitoring anomalies occurring during pregnancy, the fetus has increasingly become the target of prenatal treatment. Nonetheless, pregnancy is a unique situation, as treatment of the fetus cannot be approached independently from the concomitant treatment of the mother, especially when a medication is used for indications other than those…

Imaging and Quantifying the Fetal Circulation in Congenital Cardiac Disease : Ultrasound

Introduction Malformation of the heart and arterial trunks is the most common form of congenital anomaly found in humans. They occur in approximately 6 of every 1000 live births and in 8 to 10 of every 1000 pregnancies. Fetal echocardiography, or the use of ultrasonic technologies to evaluate the fetal cardiovascular system, enables the diagnosis of structural heart defects and offers a way to observe complex…