Medical management of cyanotic congenital heart disease in adults
- Heidi M Connolly, MD, FASE
Heidi M Connolly, MD, FASE
- Section Editor — Congenital Heart Disease
- Professor of Medicine
- Mayo Medical School
Centers specialized in the management of adult patients with congenital heart disease provide the optimal combination of clinical expertise, facilities for advanced diagnosis and therapy, structured care, and access to new and evolving therapies. The 2008 American College of Cardiology/American Heart Association (ACC/AHA) adult congenital heart disease guidelines recommended that all adults with congenital heart disease of moderate or great complexity (including those with cyanotic heart disease) should undergo initial evaluation and longitudinal coordination of care by such a specialized center .
Cyanosis is caused by right to left intracardiac or extracardiac shunts. In adults, the most common causes of cyanotic congenital heart disease are Eisenmenger syndrome and palliated complex congenital heart disease (eg, tetralogy of Fallot). (See "Evaluation and prognosis of Eisenmenger syndrome" and "Pathophysiology, clinical features, and diagnosis of tetralogy of Fallot".)
Medical management of adult patients with cyanotic congenital heart disease is discussed here. Management of Eisenmenger syndrome patients includes following recommendations pertinent to all patients to cyanotic heart disease as well as other recommendations specific to Eisenmenger syndrome and pulmonary hypertension in congenital heart disease. These later recommendations are discussed separately. (See "Management of Eisenmenger syndrome" and "Pulmonary hypertension in adults with congenital heart disease".)
MANAGEMENT OF CYANOTIC CONGENITAL HEART DISEASE
Adult patients with cyanotic heart disease should be seen at least annually at a specialty center for adult congenital heart disease patients .
Prevention of endocarditis — Patients should maintain good oral hygiene. Oral and other bacterial infections should be treated promptly. Antimicrobial prophylaxis for bacterial endocarditis is recommended for all patients with unrepaired cyanotic congenital heart disease [1,2]. (See "Antimicrobial prophylaxis for bacterial endocarditis".)
- Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 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 the management of adults with congenital heart disease). Circulation 2008; 118:e714.
- Wilson W, Taubert KA, Gewitz M, et al. Prevention of Infective Endocarditis. Guidelines From the American Heart Association. A Guideline From the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation 2007; 115 published online April 19, 2007. www.circ.ahajournals.org/cgi/reprint/CIRCULATIONAHA.106.183095v1 (Accessed on May 04, 2007).
- Territo MC, Rosove MH. Cyanotic congenital heart disease: hematologic management. J Am Coll Cardiol 1991; 18:320.
- Ammash N, Warnes CA. Cerebrovascular events in adult patients with cyanotic congenital heart disease. J Am Coll Cardiol 1996; 28:768.
- Perloff JK, Marelli AJ, Miner PD. Risk of stroke in adults with cyanotic congenital heart disease. Circulation 1993; 87:1954.
- Diller GP, Gatzoulis MA. Pulmonary vascular disease in adults with congenital heart disease. Circulation 2007; 115:1039.
- Ware JA, Reaves WH, Horak JK, Solis RT. Defective platelet aggregation in patients undergoing surgical repair of cyanotic congenital heart disease. Ann Thorac Surg 1983; 36:289.
- Colon-Otero G, Gilchrist GS, Holcomb GR, et al. Preoperative evaluation of hemostasis in patients with congenital heart disease. Mayo Clin Proc 1987; 62:379.
- Ekert H, Gilchrist GS, Stanton R, Hammond D. Hemostasis in cyanotic congenital heart disease. J Pediatr 1970; 76:221.
- Wedemeyer AL, Edson JR, Krivit W. Coagulation in cyanotic congenital heart disease. Am J Dis Child 1972; 124:656.
- Vongpatanasin W, Brickner ME, Hillis LD, Lange RA. The Eisenmenger syndrome in adults. Ann Intern Med 1998; 128:745.
- Niwa K, Perloff JK, Kaplan S, et al. Eisenmenger syndrome in adults: ventricular septal defect, truncus arteriosus, univentricular heart. J Am Coll Cardiol 1999; 34:223.
- Daliento L, Somerville J, Presbitero P, et al. Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J 1998; 19:1845.
- Territo, MC, Rosove, M, Perloff, JK. Cyanotic congenital heart disease: Hematologic management, renal function, and urate metabolism. In: Congenital Heart Disease in Adults, Perloff, JK, Child, JS (Eds), WB Saunders, Philadelphia 1991. p.93.
- Young D. Hyperuricemia in cyanotic congenital heart disease. Am J Dis Child 1980; 134:902.
- Ross EA, Perloff JK, Danovitch GM, et al. Renal function and urate metabolism in late survivors with cyanotic congenital heart disease. Circulation 1986; 73:396.
- Oya H, Nagaya N, Satoh T, et al. Haemodynamic correlates and prognostic significance of serum uric acid in adult patients with Eisenmenger syndrome. Heart 2000; 84:53.
- Flanagan MF, Hourihan M, Keane JF. Incidence of renal dysfunction in adults with cyanotic congenital heart disease. Am J Cardiol 1991; 68:403.
- Perloff JK, Latta H, Barsotti P. Pathogenesis of the glomerular abnormality in cyanotic congenital heart disease. Am J Cardiol 2000; 86:1198.
- Oechslin EN, Harrison DA, Connelly MS, et al. Mode of death in adults with congenital heart disease. Am J Cardiol 2000; 86:1111.
- Bonow RO, Carabello BA, Chatterjee K, et al. 2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation 2008; 118:e523.
- MANAGEMENT OF CYANOTIC CONGENITAL HEART DISEASE
- Prevention of endocarditis
- Erythrocytosis and anemia
- - Guidelines and recommendations
- - Pulmonary bleeding
- - Menorrhagia
- - Anticoagulation
- Other problems
- - Hyperuricemia
- - Pigment gallstones
- - Orthopedic problems
- - Renal dysfunction
- - Neurological complications
- Other considerations
- Cardiac operation or reoperation
- SUMMARY AND RECOMMENDATIONS