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Ventricular Septal Defect and Double-Outlet Right Ventricle

Ventricular Septal Defect A ventricular septal defect (VSD) is a hole between the left and right ventricles. A VSD may occur as an isolated anomaly or with a wide variety of intracardiac anomalies, such as tetralogy of Fallot or transposition of the great arteries. This chapter discusses isolated VSD. Banding of the pulmonary artery as a palliative maneuver was first described in 1952. This decreased left-to-right…

Atrioventricular Canal Defects

Atrioventricular (AV) canal defects include a spectrum of lesions in which the common etiology appears to be abnormal development of the endocardial cushions, resulting in a defect in the AV septum and AV valves. This group of lesions forms approximately 3% of all major congenital cardiac defects, and approximately half of the patients have Down syndrome. In children with Down syndrome, AV canal defects are seen…

Surgical Considerations in Pulmonary Vein Anomalies

Etiology Normal embryologic development of the pulmonary venous system involves creation of a connection between the left atrium and the pulmonary venous plexus, and subsequent regression of systemic-to-pulmonary venous connections. Inappropriate connection of the pulmonary venous system to the systemic venous system is termed anomalous pulmonary venous drainage . As part of normal embryologic development, the lungs are derived from buds arising from the primitive foregut.…

Atrial Septal Defect and Cor Triatriatum

Historical Considerations Ingenious and risky surgical corrections of atrial septal defect (ASD) predated cardiopulmonary bypass. The earliest closed approaches included a technique in which a straight needle with suture, guided by palpation, was passed blindly through the defect and both atria, and the free walls of the left and right atria were drawn together to obstruct the defect. Bailey and colleagues described the “atrio-septo-pexy” consisting of…

Patent Ductus Arteriosus, Coarctation of the Aorta, and Vascular Rings

Included under the rubric of congenital heart defects are abnormalities involving the distal transverse arch and proximal descending aorta. Clinical manifestations range widely, from severe congestive heart failure or debilitating stridor to mere incidental finding on routine evaluation. The treatment of these conditions represents the first procedures performed for congenital cardiovascular malformations. The first surgical closure of a patent ductus arteriosus (PDA), performed by Dr. Robert…

Congenital Tracheal Disease

History of Pediatric Tracheal Surgery In 2000, the Congenital Heart Surgery Nomenclature and Database Project classified congenital tracheal stenosis as congenital–complete tracheal rings, postintubation, traumatic, or congenital web. Localized stenosis was defined as less than 50% of the tracheal length, and long-segment stenosis as greater than 50% of the tracheal length. The initial classification of tracheal stenosis had been proposed by Drs. Cantrell and Guild from…

Neuromonitoring and Neurodevelopmental Outcomes in Congenital Heart Surgery

History of Intraoperative Neuromonitoring Brain injury is the most common and potentially disabling complication following congenital heart surgery. With improved survival, the focus has shifted to optimizing functional outcomes. An important goal of therapy for every congenital heart surgical patient is to reduce the risk of brain injury as much as possible. Along with updated perfusion, anesthetic, and surgical strategies, techniques for neuromonitoring have been refined…

Pediatric Anesthesia and Critical Care

The management of congenital heart disease (CHD) has progressed significantly over the past three decades. Most congenital heart lesions are now amenable to anatomic or physiologic repair early in infancy. Advances in diagnostic and interventional cardiology, the evolution of surgical techniques and conduct of cardiopulmonary bypass, and refinements in postoperative management have all contributed to a substantial decrease in morbidity and mortality associated with CHD. The…

Surgical Approaches, Cardiopulmonary Bypass, and Mechanical Circulatory Support in Children

For infants and children, mechanical support of the circulation has important roles in providing short-term circulatory support for reversible myocardial failure, in providing cardiopulmonary support before and after cardiac surgery, and in providing longer-term support as a potential bridge to cardiac transplantation. Mechanical circulatory support (MCS) modalities commonly available include extracorporeal membrane oxygenation (ECMO), intra-aortic balloon pump (IABP) counterpulsation, and ventricular assist devices (VADs). Although a…

Surgical Approaches and Cardiopulmonary Bypass in Pediatric Cardiac Surgery

Thoracic incisions traditionally used in adult cardiac and thoracic surgery have been used in children with varying success with respect to exposure, pain, and cosmetic result. Special considerations relevant to pediatric surgery are related to the lack of development and growth of soft tissue structures such as breast tissue and bony structures such as ribs and vertebra. Thus, incisions that fix growing bony structures, such as…

Cardiac Catheterization and Fetal Intervention

Introduction and Overview Catheterization in the field of congenital cardiology continues to improve understanding of disease and expand options for therapy. Diagnostic information previously obtained exclusively through catheterization is now routinely acquired through use of a host of diverse and highly sophisticated, noninvasive diagnostic imaging modalities. Invasive hemodynamic measurement and angiographic assessment, however, remain a mainstay in the comprehensive evaluation of the patient with complex heart…

Diagnostic Imaging: Echocardiography and Magnetic Resonance Imaging

Before the advent of cardiopulmonary bypass in the mid-1950s, little attention was given to the diagnosis of congenital heart disease (CHD) because no effective treatment was available. Physical examination, auscultation, electrocardiography, and radiography were the main diagnostic tools. Progress in open-heart techniques for repair of CHD required accurate and comprehensive delineation of cardiovascular anatomy and function. During the 1960s and 1970s, cardiac catheterization and angiography were…

Segmental Anatomy

The heart is a complex organ. It is difficult to understand the anatomy of the heart, particularly when abnormal, and it is even more difficult to describe it clearly and succinctly. Yet this is the goal of the diagnostician cardiologist: to understand and describe the anatomy of a defective heart in a way that colleagues will comprehend sufficiently to be able to contribute meaningfully to the…

Cardiac Embryology and Genetics

Development of the functioning heart is a complex process that is essential for survival of the embryo. The mechanisms responsible for cardiovascular morphogenesis have been studied for centuries by classical embryologists and more recently by molecular developmental biologists. Major progress in the understanding of cardiac specification and morphogenesis has been made from the study of cardiovascular development in a number of invertebrate and vertebrate model systems,…

Tumors of the Heart

Neoplastic involvement of the heart can be divided into primary cardiac tumors arising in the heart and secondary cardiac tumors that have metastasized to the heart. Primary cardiac tumors can be further stratified into benign and malignant tumors. Secondary involvement of the heart is relatively common, with 10% to 20% of patients dying of disseminated cancer having metastatic involvement of the heart or pericardium. Surgical resection…

Surgery for Pulmonary Embolism

Pulmonary thromboembolism is a significant cause of morbidity and mortality in the United States and worldwide. The estimated incidence of acute pulmonary embolism is approximately 63 per 100,000 patients in the United States, based on clinical and radiographic data. Acute pulmonary embolism is the cause of approximately 235,000 deaths per year based on autopsy data. Acute pulmonary embolism occurs half as often as acute myocardial infarction…

Regenerative Cell-Based Therapy for the Treatment of Cardiac Disease

Heart failure (HF) is a burgeoning disease. Recently published American Heart Association projections indicate that its prevalence will increase by 46% from 2012 to 2030 resulting in approximately 8 million people with HF in the United States alone. Ischemic heart disease is the major contributor to HF and despite best practices and management strategies, many patients are left with significant disability. Events such as myocardial infarction…

Left Ventricular Restoration : Surgical Treatment of the Failing Heart

In memory of Professor Marisa Di Donato. In response to the increasing health, economic, and social impacts of heart failure, clinicians are investigating new surgical therapies that do not involve heart transplantation. Heart failure affects about 5 million patients in the United States, and more than 250,000 die annually. Nearly 70% of patients with heart failure have coronary artery disease, and nearly all have had a…

Heart–Lung Transplantation

The clinical realization of heart–lung transplantation evolved through much earlier experimental developments in the laboratory. Alexis Carrel ( Fig. 99-1 ) and later Demikhov and Marcus and associates performed experiments that explored the possibility of using heart–lung transplantation to replace diseased organs. When heart transplantation was first performed in 1968, Cooley, Lillehei, and Barnard all performed human heart–lung transplants, but patient survival time was short. Progress…

Heart Transplantation

In the span of 5 decades, cardiac transplantation has progressed from an experimental dream to a treatment reality for patients with heart failure. According to the 2013 Registry of the International Society for Heart and Lung Transplantation, 4096 transplants were performed in 2011. The registry captures an estimated 66% of all transplants. Short- and long-term results improved until 2006, after which they remained stable. Consequently, heart…