Congenital Heart Disease.

Abstract

1. Kriti Puri, MD* 2. Hugh D. Allen, MD* 3. Athar M. Qureshi, MD*,† 1. *Department of Pediatrics, 2. †CE Mullins Cardiac Catheterization Laboratories, The Lillie Frank Abercombie Section of Cardiology, Texas Children’s Hospital, Baylor College of Medicine, Houston, TX * Abbreviations: ASD: : atrial septal defect AVSD: : atrioventricular septal defect CHD: : congenital heart disease CHF: : congestive heart failure CoA: : coarctation of the aorta EKG: : electrocardiogram HLHS: : hypoplastic left heart syndrome IAA: : interrupted aortic arch IVC: : inferior vena cava LV: : left ventricle mBTT: : modified Blalock-Taussig-Thomas PAPVD: : partial anomalous pulmonary venous drainage PDA: : patent ductus arteriosus RV: : right ventricle SBE: : subacute bacterial endocarditis TAPVD: : total anomalous pulmonary venous drainage VSD: : ventricular septal defect Congenital heart disease (CHD) is present in about 9 of every 1,000 live-born children. (1)(2)(3)(4)(5) Children with CHD are surviving longer, and better understanding of the long-term complications of CHD is continuously emerging. Hence, it is important to be comfortable with the primary care requirements for these children, including physical manifestations prior to surgery and interventional cardiac catheterizations, as well as those concerning for potential need for reintervention, the latest recommendations for endocarditis prophylaxis, respiratory precautions and immunization considerations, and close monitoring of development and behavior. In this article, we will discuss the common types of cyanotic (“blue”) and acyanotic (“pink”) CHD and the role of the primary care physician in the health care of these children before and after surgery and interventional cardiac catheterizations. After completing this article, readers should be able to: 1. Describe newborn pulse oximetry screening for congenital heart disease (CHD) and clinical features of CHD during the newborn period. 2. Describe the clinical presentations and briefly outline management strategies of infants and children with different types of CHD. 3. Discuss single-ventricle palliation by using hypoplastic left heart syndrome as the model lesion. 4. Describe the evaluation and primary care management factors (including endocarditis prophylaxis, immunizations, and exercise restriction) in children with different types of CHD. Newborns with critical congenital heart disease (CHD) may present with symptoms of cyanosis, congestive heart failure (CHF), poor pedal pulses, or a failed newborn CHD pulse oximetry screen prior to discharge. CHD lesions that are dependent on a patent ductus arteriosus (PDA) to support flow either to the systemic circulation or to the pulmonary circulation …