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Adults with congenital heart disease: Patched but not cured by Michael Earing, MD Michael Earing, MD, is a cardiologist and program director of Adult Congenital Heart Disease at Children’s Hospital of Wisconsin. He also is an associate professor of Adult Cardiovascular Medicine and Pediatric Cardiology at The Medical College of Wisconsin and a member of Children’s Specialty Group. Introduction Congenital heart defects are the most common group of birth defects, occurring in approximately 9 in 1,000 live births1. Without treatment, the majority of patients would die in infancy or childhood, with only 5-15 percent surviving into adulthood2. The advent of surgical procedures such as ligation of patent ductus arteriosus, resection of coarctation of aorta and the legendary Blalock-Taussig shunt, as well as advances in diagnostic, interventional and critical care skills, have resulted in survival of approximately 90 percent of these children to adulthood. Now, for the first time in history, it is estimated that more adults than children are living with congenital heart disease in the U.S., with a 5 percent increase every year. This growth rate predicts that in the next decade, the number of adults with congenital heart disease living in the U.S. will reach 1 million3-4. Unfortunately, the number of physicians formally trained to care for this complex group of patients is small. In the most recent study, published in 2006, it is estimated that only 31 physicians have received formalized training to care for adults with congenital heart disease in the U.S. in the last 10 years5. As a result, many adults with congenital heart disease do not have access to appropriately trained physicians or required medical and surgical services1, 3-5. Adult c ongenita l hea rt dis eas e Long-term medical considerations About 25 percent of adults with congenital heart disease have a mild form that has allowed them to survive into adulthood without surgical or interventional cardiac catheterization. The most common lesions in this category include mild aortic valve stenosis (usually in setting of bicuspid aortic valve), small restrictive ventricular septal defects, mild pulmonary valve stenosis and mitral valve prolapse. (See Table 1.) These patients need less frequent followup to assess for progression of disease and to identify associated complications. The majority of adults with congenital heart disease now living in the U.S., however, are Table 1 patients who have had previous Congenital heart defects associated with survival into adulthood intervention. (See Table 2.) without surgery or interventional cardiac catheterization. Although the majority of Mild pulmonary valve stenosis children who undergo surgical Bicuspid aortic valve intervention will survive to Small to moderate size atrial septal defect adulthood, with few exceptions, Small ventricular septal defect “total correction” is not the rule. Small patent ductus arteriosus The few exceptions include Mitral valve prolapse patent ductus arteriosus, Partial atrioventricular canal (ostium primum atrial septal defect and ventricular septal defects and cleft mitral valve) atrial septal defects, and only Marfan syndrome if they are closed early before Ebstein’s anomaly the development of irreversible Congenitally corrected transposition (atrio-ventricular and ventriculocardiac or lung damage and no arterial discordance) residual lesions exist. 12 Physician Referral Center (800) 266-0366 Michael Earing, MD, conducts a follow-up appointment with an adult patient at Children’s Hospital of Wisconsin. Dr. Earing is one of the few physicians in the U.S. who has received formalized training to care for adults with congenital heart disease. Table 2 Most common congenital heart defects surviving to adulthood after surgery or interventional catheterization. Aortic valve disease following balloon valvuloplasty or surgical valvotomy Pulmonary valve stenosis following balloon valvuloplasty or surgical valvotomy The Herma Heart Center Adult Congenital Heart Disease Program, established in 2004, is dedicated to advancing Tetralogy of Fallot the care of adults with Ventricular septal defect congenital heart disease. Complete atrioventricular canal It offers all aspects of care Transposition of the great arteries for adults with congenital Coarctation of the aorta heart disease. In addition, Complex single ventricles after the modified Fontan procedure the program is dedicated, through research and chw.org education, to improve the lives of all current and future patients with congenital heart disease. Adult c ongenital hea rt dis ease Because adult patients with congenital heart disease now are surviving longer than ever before, it is becoming increasingly apparent that even the most simple lesions can be associated with long-term complications4, 6. These longterm complications include both cardiac and noncardiac problems. Cardiac complications include arrhythmias and conduction defects, ventricular dysfunction, residual shunts, valvular lesions (regurgitation and stenosis), hypertension and aneurysms. Noncardiac sequelae include developmental abnormalities such as developmental delay, somatic abnormalities such as facial dysmorphism (cleft palate/lip), central nervous abnormalities such as seizure disorders from A collaboration between Children’s Hospital of Wisconsin and Froedtert Hospital, the program now serves more than 1,700 patients annually. 13 previous thromboembolic events or cerebrovascular accidents, disturbances of the senses such as hearing or vision loss and, finally, pulmonary sequelae such as restrictive and obstructive lung disease. In addition, psychosocial problems involving employment, life and health insurance, participation in sports, sexual activity and contraception are common. As a result of these longterm complications, the majority of adults with congenital heart disease need lifelong followup4. References 1. Perloff JK, Warnes CA. Challenges Posed by Adults with Repaired Congenital Heart Disease. Circulation. 2001; 103(21): 2637. 2. Mitchell SC, Korones SB, Berendes HW. Congenital Heart Disease in 56,109 Births: Incidence and Natural History. Circulation. 1971; 43: 323-332. 3. Congenital Heart Disease After Childhood: An Expanding Patient Population: 22nd Bethesda Conference, Maryland, October 18-19, 1990. J Am Coll Cardiol. 1991; 18: 311-342. 4. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA Guidelines for the Management of Adults with Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on Management of Adults with Congenital Heart Disease): Developed in Collaboration with American Society of Echocardiography, Heart Rhythm Society, International Society of Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Intervention, and the Society of Thoracic Surgeons. Circulation. 2008; 118: e714-e833. 5. Gurvitz MZ, Chang RK, Ramos FJ, et al. Variation in Adult Congenital Heart Disease Training in Adult and Pediatric Cardiology Fellowship Programs. J Am Coll Cardiol 2005. Sep; 46(5): 893-8. 6. Earing MG, Connolly HM, Dearani JA, et al. Long-term Follow-up of Patients After Surgical Treatment of Isolated Pulmonary Valve Stenosis. Mayo Clin Proc 2005. Jul; 80(7): 871-6. Adult congenital heart disease CME webcast Practical Pediatrics Grand Rounds CME webcasts provide lectures on recent advances and current knowledge in pediatrics. Each lecture is available on demand, allowing you to earn CME credit when it is convenient for you. “Congenital Heart Disease in Adults” by Michael Earing, MD Objectives • Discuss common medical issues that affect adults with congenital heart disease. • Prevent current psychosocial challenges faced by adults with congenital heart disease. • Discuss current research projects in progress to address medical issues and psychosocial issues. This lecture is available online at chw.org/practicalpediatrics. See Page 19 for CME information. For more information, visit chw.org/hermaheartcenter. For outcomes from our Herma Heart Center, visit chw.org/quality. To refer a patient, visit chw.org/refer or call toll-free (800) 266-0366. Adult c ongenita l hea rt dis eas e Heart Matters Children’s Hospital of Wisconsin’s Herma Heart Center is one of the nation’s top programs for medical and surgical treatment of congenital heart defects and heart disease in children. As one of the largest pediatric cardiac programs in the United States, we have set national benchmarks for surgical outcomes. 14 Physician Referral Center (800) 266-0366