What are congenital heart defects?
Congenital (inborn) heart defects are structural abnormalities of the heart that are present at birth. They occur in about 1 in 100 newborns. These defects happen while the baby is developing the heart's valves, chambers, and blood vessels.
There are many types of congenital heart defects, ranging from simple to complex, small to large, and producing mild to severe symptoms. Simple defects include holes in the inner walls that separate the chambers of the heart (called septal defects), narrowed heart valves (see heart valve disease), and abnormal blood vessels. Complex defects include combinations of one or more simple defects, abnormally placed blood vessels, and other structural abnormalities.
Although congenital heart defects are present at birth, they may not produce symptoms immediately. Symptoms depend on the type and severity of the defect. Milder forms may not produce symptoms at all or not until adulthood. More severe defects can be life-threatening, causing symptoms such as rapid breathing or a bluish discoloration of the skin.
How are congenital heart defects related to heart failure?
Congenital heart defects can disrupt the heart's ability to pump blood over time, leading to heart failure. The most common types of congenital heart defects are abnormal holes in the heart and narrowing of heart blood vessels or valves. Click here for more detailed information on heart valve disease.
A ventricular septal defect (VSD) is a hole in the heart muscle wall that separates the left lower chamber (the left ventricle) from the right lower chamber (right ventricle), allowing oxygen-rich blood to flow from the left ventricle into the right ventricle instead of out to the rest of the body. A VSD is the most common form of congenital heart defect in newborns.2, 27 About 85% to 95% of small VSDs and 10% to 15% of large VSDs may close on their own.27 A large VSD causes the heart to work too hard to pump blood, which can lead to heart failure.
An atrial septal defect (ASD), is a hole in the part of the muscle wall that separates the upper left chamber (left atrium) of the heart from the upper right chamber (right atrium). This allows oxygen-rich blood from the left (larger) atrium to flow into the right (smaller) atrium rather than into the left ventricle (the heart's main pumping chamber) and the rest of the body. Large ASDs may, over time, stretch and eventually weaken the right side of the heart, setting the stage for heart failure. ASDs occur 2 to 3 times more often in women than in men and account for one third of congenital heart defects in adults.28, 29
Coarctation of the aorta is severe narrowing of the aorta, the main artery that carries oxygen-rich blood from the heart to the rest of the body. This defect causes the heart to pump with more force to get blood through the narrowed aorta. It's 2 to 5 times more common in men and boys than in women and girls.28
How do congenital heart defects affect my risk of developing heart failure?
About 85% of babies born with heart defects will survive to reach adulthood. However, more than half of adults with congenital heart defects are at medium to high risk for complications, repeat surgeries, and premature death.30
The most common complications for adults with congenital heart defects are heart failure and atrial fibrillation.30, 31 When the chambers, valves, and major blood vessels of the heart don't form properly, the heart has to work harder to pump out enough blood. Over time, the extra strain can weaken the heart and lead to heart failure. Two thirds of people aged 40 and older with untreated coarctation of the aorta have symptoms of heart failure; no women-specific data are provided.28 Atrial fibrillation, a risk factor for heart failure, occurs more frequently in adults aged 40 or older who have an ASD. Women are more than twice as likely as men to have ASD.28
Other complications include high blood pressure, premature coronary artery disease, and heart attacks, all of which are major risk factors for heart failure. A Dutch study of about 7500 adults (nearly 50% were women) with congenital heart defects found that women had a 33% higher risk than men of having increased blood pressure in the arteries of the lung, which causes the heart to work harder to pump blood.29 Over time, the increased workload weakens the heart until it becomes an inefficient pump.
What can I do to prevent heart failure if I have a congenital heart defect?
The best way to keep from developing heart failure is to treat the inborn defect. You need to make sure you are maintaining a healthy lifestyle, including eating a healthy diet and not smoking, and following any treatment prescribed by your doctor. Some congenital heart defects are mild and don't need treatment; others need to be treated with medications, surgery, or catheter-based procedures. About 50% to 60% of congenital heart defects are treated surgically in infancy or early childhood.
Surgery can alleviate symptoms and reduce the risk of heart failure by improving the flow of blood, but it does not mean the heart is "normal." About 50% of patients with congenital heart defects in one large US medical center needed two or more surgeries.30
Most adults with congenital heart defects should have lifelong monitoring by a cardiologist (heart specialist). If you have a congenital heart defect and plan to get pregnant, check with your cardiologist to assess your risk. Pregnancy causes the heart to work harder to pump 30% to 50% more blood than normal. The most common heart disease complications in pregnant women with congenital heart defects are heart failure and heart rhythm problems.32
- Schocken DD, Benjamin EJ, Fonarow GC, et al. Prevention of Heart Failure: A Scientific Statement From the American Heart Association Councils on Epidemiology and Prevention, Clinical Cardiology, Cardiovascular Nursing, and High Blood Pressure Research; Quality of Care and Outcomes Research Interdisciplinary Working Group; and Functional Genomics and Translational Biology Interdisciplinary Working Group. Circulation. May 13, 2008;117(19):2544-2565.
- Rosamond W, Flegal K, Furie K, et al. Heart disease and stroke statistics--2008 update: a report from the American Heart Association Statistics Committee and Stroke Statistics Subcommittee. Circulation. Jan 29 2008;117(4):e25-146.
- Ghali JK, Krause-Steinrauf HJ, Adams KF, et al. Gender differences in advanced heart failure: insights from the BEST study. J Am Coll Cardiol. Dec 17 2003;42(12):2128-2134.
- Alla F, Al-Hindi AY, Lee CR, Schwartz TA, Patterson JH, Adams KF. Relation of sex to morbidity and mortality in patients with heart failure and reduced or preserved left ventricular ejection fraction. Am Heart J. June 2007;153(6):1074-1080.
- Ho KK, Anderson KM, Kannel WB, Grossman W, Levy D. Survival after the onset of congestive heart failure in Framingham Heart Study subjects. Circulation. July 1, 1993;88(1):107-115.
- Lund LH, Mancini D. Heart failure in women. Med Clin North Am. Sep 2004;88(5):1321-1345, xii.
- Frazier CG, Alexander KP, Newby LK, et al. Associations of Gender and Etiology With Outcomes in Heart Failure With Systolic Dysfunction: A Pooled Analysis of 5 Randomized Control Trials. J Am Coll Cardiol. April 3, 2007;49(13):1450-1458.
- O'Meara E, Clayton T, McEntegart MB, et al. Sex Differences in Clinical Characteristics and Prognosis in a Broad Spectrum of Patients With Heart Failure: Results of the Candesartan in Heart failure: Assessment of Reduction in Mortality and morbidity (CHARM) Program. Circulation. June 19, 2007;115(24):3111-3120.
- Mezu U, Bott-Silverman C, Hsich E. Heart failure in women is different than in men; should treatment be different? Cleve Clin J Med. Jun 2007;74(6):423-424, 426, 429-435.
- Bibbins-Domingo K, Lin F, Vittinghoff E, et al. Predictors of Heart Failure Among Women With Coronary Disease. Circulation. September 14, 2004;110(11):1424-1430.
- Van den Berg MP, Tuinenburg AE, Crijns HJ, Van Gelder IC, Gosselink AT, Lie KI. Heart failure and atrial fibrillation: current concepts and controversies. Heart. April 1, 1997;77(4):309-313.
- Cha Y-M, Redfield MM, Shen W-K, Gersh BJ. Atrial Fibrillation and Ventricular Dysfunction: A Vicious Electromechanical Cycle. Circulation. June 15, 2004;109(23):2839-2843.
- Stewart S, Hart CL, Hole DJ, McMurray J. A population-based study of the long-term risks associated with atrial fibrillation: 20-year follow-up of the Renfrew/Paisley study. Am J Med. Oct 1, 2002;113(5):359-364.
- Gottdiener JS, Arnold AM, Aurigemma GP, et al. Predictors of congestive heart failure in the elderly: the cardiovascular health study. J Am Coll Cardiol. May 1, 2000;35(6):1628-1637.
- Hunt SA. ACC/AHA 2005 Guideline Update for the Diagnosis and Management of Chronic Heart Failure in the Adult: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Update the 2001 Guidelines for the Evaluation and Management of Heart Failure). J Am Coll Cardiol. September 20, 2005;46(6):e1-82.
- Benjamin EJ, Levy D, Vaziri SM, D'Agostino RB, Belanger AJ, Wolf PA. Independent risk factors for atrial fibrillation in a population-based cohort. The Framingham Heart Study. JAMA. Mar 16 1994;271(11):840-844.
- Wang TJ, Larson MG, Levy D, et al. Temporal Relations of Atrial Fibrillation and Congestive Heart Failure and Their Joint Influence on Mortality: The Framingham Heart Study. Circulation. June 17, 2003;107(23):2920-2925.
- Healey JS, Baranchuk A, Crystal E, et al. Prevention of Atrial Fibrillation With Angiotensin-Converting Enzyme Inhibitors and Angiotensin Receptor Blockers: A Meta-Analysis. J Am Coll Cardiol. June 7, 2005;45(11):1832-1839.
- Vermes E, Tardif J-C, Bourassa MG, et al. Enalapril Decreases the Incidence of Atrial Fibrillation in Patients With Left Ventricular Dysfunction: Insight From the Studies Of Left Ventricular Dysfunction (SOLVD) Trials. Circulation. June 17, 2003;107(23):2926-2931.
- Maggioni AP, Latini R, Carson PE, et al. Valsartan reduces the incidence of atrial fibrillation in patients with heart failure: results from the Valsartan Heart Failure Trial (Val-HeFT). Am Heart J. Mar 2005;149(3):548-557.
- Carroll JD, Carroll EP, Feldman T, et al. Sex-associated differences in left ventricular function in aortic stenosis of the elderly. Circulation. October 1, 1992;86(4):1099-1107.
- He J, Ogden LG, Bazzano LA, Vupputuri S, Loria C, Whelton PK. Risk Factors for Congestive Heart Failure in US Men and Women: NHANES I Epidemiologic Follow-up Study. Arch Intern Med. April 9, 2001;161(7):996-1002.
- Kannel WB. Incidence and epidemiology of heart failure. Heart Fail Rev. Jun 2000;5(2):167-173.
- Dujardin KS, Enriquez-Sarano M, Schaff HV, Bailey KR, Seward JB, Tajik AJ. Mortality and Morbidity of Aortic Regurgitation in Clinical Practice : A Long-Term Follow-Up Study. Circulation. April 13, 1999;99(14):1851-1857.
- Ling LH, Enriquez-Sarano M, Seward JB, et al. Clinical Outcome of Mitral Regurgitation Due to Flail Leaflet. N Engl J Med. November 7, 1996;335(19):1417-1423.
- Ling LH, Enriquez-Sarano M, Seward JB, et al. Early Surgery in Patients With Mitral Regurgitation Due to Flail Leaflets : A Long-term Outcome Study. Circulation. September 16, 1997;96(6):1819-1825.
- Hoffman JIE, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. June 19, 2002;39(12):1890-1900.
- Brickner ME, Hillis LD, Lange RA. Congenital Heart Disease in Adults- First of Two Parts. N Engl J Med. January 27, 2000;342(4):256-263.
- Verheugt CL, Uiterwaal CSPM, van der Velde ET, et al. Gender and Outcome in Adult Congenital Heart Disease. Circulation. July 1, 2008;118(1):26-32.
- Warnes CA, Liberthson R, Danielson GK, Jr., et al. Task Force 1: the changing profile of congenital heart disease in adult life. J Am Coll Cardiol. April 1, 2001;37(5):1170-1175.
- Engelfriet P, Boersma E, Oechslin E, et al. The spectrum of adult congenital heart disease in Europe: morbidity and mortality in a 5 year follow-up period: The Euro Heart Survey on adult congenital heart disease. Eur Heart J. November 1, 2005;26(21):2325-2333.
- Khairy P, Ouyang DW, Fernandes SM, Lee-Parritz A, Economy KE, Landzberg MJ. Pregnancy Outcomes in Women With Congenital Heart Disease. Circulation. January 31, 2006;113(4):517-524.