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…
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…
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…
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…
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…
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…
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…
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…
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…
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…