Lifelong Specialized Care

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Why specialized lifelong care is so important


​​​​​The message that children and adults living with CHD should receive specialized care throughout their lifetime is clear—all patients with CHD require lifelong congenital cardiology evaluation. (1) It is estimated that 40,000 babies are born with a congenital heart defect (CHD) each year which equals about 1 in 110 births. There are between 2 -3 million adults and children in the United States living with repaired and unrepaired congenital heart defects today. Due to improved pediatric medical care and the success of surgical repairs, 90% of people born with CHD are living to adulthood. (2) The number of adults with CHDs is increasing steadily at about 5% each year. (3) In fact, there are more adults than children living with CHDs in the United States today. The great advances in congenital cardiac care have truly made congenital heart disease not only a “lifelong” condition but frequently a “long life” condition. ​

The oft-used words in CHD care of, “fixed, repaired, and live a normal life” lead to the false assumption that surgery is a cure. Although CHD surgery can help people feel better and live longer, more productive lives, it is not a cure. Many people with CHDs will require more than one operation or catheter-based intervention during their lifetime and others will have residual effects from the procedure or of their underlying condition that occur later in life. Also, as patients get older, the impact of the interaction between the CHD and typical conditions of adulthood, such as diabetes and hypertension, must be considered. 

Clinical guidelines and recommendations for care are available for the management of adult patients with CHD, as well as for the transition of pediatric CHD patients to adult care. However, even though these are available, many patients do not receive recommended care.(1) It is incumbent upon primary care providers and general cardiologists to provide necessary co-management to assure that patients of all ages remain in care throughout their life. A jointly developed care plan should include roles for the different providers in the care team based on the medical needs and goals of the patient. These should be established in partnership with the patient. Explicitly stated goals with therapeutic (including the next scheduled visit to congenital cardiologist), educational/vocational, and family interventions should be used in order to maximize outcomes for children and youth with CHD and to drive successful transitions to adult systems of care.(4)

While many patients do remain in the care of a congenital heart physician through childhood and into adulthood to address later effects of the CHD and issues of adulthood, it is estimated that 50% of children with congenital heart defects drop out of care or have a gap in care prior to 15 years of age.(5) Additional studies show that among adults presenting to a specialty congenital heart clinic, 42% have experienced a gap in care greater than 3 years; the typical gap occurs around 20 years of age.(6) Moreover, those with gaps in care have a higher rate of presenting with problems or the need for an urgent intervention.(7) When asked about reasons for gaps in care, many patients cite that they “felt well” or “didn’t know I needed follow up”.(6) The educational barrier seems to occur in childhood but the consequences occur into adulthood. 

In 2011, the American Heart Association (AHA) published a Scientific Statement, “Best Practices in Managing Transition to Adulthood for Adolescents with Congenital Heart Disease: The Transition Process and Medical and Psychosocial Issues.”(8) It recommends a formal transition program that is “patient-centered, age and developmentally appropriate, flexible and comprehensive”. The ultimate goal of this type of program is to provide the best quality of life for young adults with CHD, making sure they live life to the fullest potential and live it well into their adult years. Additional information about transition can be found through the National Alliance to Advance Adolescent Health at

The 2008 American College of Cardiology (ACC) and American Heart Association (AHA) expert guidelines for the management of the adult with CHD recommend that all adults with CHD receive care from specially trained adult congenital heart disease (ACHD) cardiologists—the frequency of which is dependent on the severity of their CHD.(1) Individuals living with a CHD who have experienced a gap in care should be encouraged to make an appointment with a congenital cardiologist. Family members, educators and health care clinicians should play a part in encouraging patients to stay in specialty cardiac care and, if necessary, return to care. 

Shared care among primary and specialty and subspecialty providers can positively affect the care and improve the outcomes for both children and adults living with CHD. It allows the patient to maintain a relationship with his/her pediatrician, primary care doctor and/or general cardiologist while at the same time receiving consultation through available subspecialty referral. This is especially important for patients who may face insurance, geographic, transportation or other system barriers in receiving care from an adult congenital heart specialist. 

Co-management between CHD specialists and primary care providers in the medical home is important to address nutritional needs, exercise intolerance or restrictions, developmental, behavioral or cognitive issues and many cardiac specific risk factors (eg, obesity, smoking). As more people with congenital heart disease live to adulthood and become parents, there is the question of the potential of recurrence of CHD in the fetus as well as questions regarding not only medical risks to women with CHD during a pregnancy. It is essential that education about this aspect of their care be an integral part of the ongoing follow-up of teen and adult patients with CHD. 

Individuals with​ CHD may also have other diagnoses that affect and or interact with the CHD, such as acquired heart disease, obesity, diabetes, epilepsy or kidney or liver disease. In addition, children with CHD are at increased risk of developmental disorders or disabilities or developmental delays. Primary care providers need to work with patients and families to monitor each child’s development throughout childhood to enhance his/her success at school, relationships with friends and families and independence in daily living activities. This interaction and communication must also continue through adolescence and adulthood as a means to ensure appropriate therapeutic interventions and successful and smooth transitions. ​

  1. Wa​rnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, del Nido P, Fasules JW, Graham TP, Jr., Hijazi ZM, Hunt SA, King ME, Landzberg MJ, Miner PD, Radford MJ, Walsh EP, Webb GD, Smith SC, Jr., Jacobs AK, Adams CD, Anderson JL, Antman EM, Buller CE, Creager MA, Ettinger SM, Halperin JL, Krumholz HM, Kushner FG, Lytle BW, Nishimura RA, Page RL, Riegel B, Tarkington LG, Yancy CW. 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). Developed in collaboration with the american society of echocardiography, heart rhythm society, international society for adult congenital heart disease, society for cardiovascular angiography and interventions, and society of thoracic surgeons. J Am Coll Cardiol. 2008;52:e143-263
  2. Warnes CA, Liberthson R, Danielson GK, Dore A, Harris L, Hoffman JI, Somerville J, Williams RG, Webb GD. Task force 1: The changing profile of congenital heart disease in adult life. J Am Coll Cardiol. 2001;37:1170-1175
  3. Niwa K, Perloff JK, Webb GD, Murphy D, Liberthson R, Warnes CA, Gatzoulis MA. Survey of specialized tertiary care facilities for adults with congenital heart disease. Int J Cardiol. 2004;96:211-216
  4. Patient- and family-centered care coordination: A framework for integrating care for children and youth across multiple systems. Pediatrics. 2014;133:e1451-1460
  5. Mackie AS, Ionescu-Ittu R, Therrien J, Pilote L, Abrahamowicz M, Marelli AJ. Children and adults with congenital heart disease lost to follow-up: Who and when? Circulation. 2009;120:302-309
  6. Gurvitz M, Valente AM, Broberg C, Cook S, Stout K, Kay J, Ting J, Kuehl K, Earing M, Webb G, Houser L, Opotowsky A, Harmon A, Graham D, Khairy P, Gianola A, Verstappen A, Landzberg M. Prevalence and predictors of gaps in care among adult congenital heart disease patients: Heart-achd (the health, education, and access research trial). J Am Coll Cardiol. 2013;61:2180-2184
  7. Yeung E, Kay J, Roosevelt GE, Brandon M, Yetman AT. Lapse of care as a predictor for morbidity in adults with congenital heart disease. Int J Cardiol. 2008;125:62-65
  8. Sable C, Foster E, Uzark K, Bjornsen K, Canobbio MM, Connolly HM, Graham TP, Gurvitz MZ, Kovacs A, Meadows AK, Reid GJ, Reiss JG, Rosenbaum KN, Sagerman PJ, Saidi A, Schonberg R, Shah S, Tong E, Williams RG. Best practices in managing transition to adulthood for adolescents with congenital heart disease: The transition process and medical and psychosocial issues: A scientific statement from the american heart association. Circulation. 2011;123:1454-1485
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Katherine Buchanan, 19

College sophomore, blogger - "Recovery of a Zipper Sister"

College student Katherine Buchanan knew something was wrong when she had to stop for rest every 100 feet or so during a hike with friends last summer. Buchanan, a French major also considering a double major in religion, was born with a cleft mitral valve and atrial septal defect in her heart. The conditions were corrected with surgery when she was 18 months old. "I always assumed I was cured for life," she said.

After visits with a cardiology specialist, the normally active, healthy college sophomore learned that rather than a one-time defect, she had congenital heart disease. She underwent open heart surgery to receive a valve replacement. Buchanan and her family were surprised to learn of her condition after she went nearly 20 years without any heart problems. Buchanan recognizes now that she'll always have heart disease and will likely have to have surgery about every 15 years. But for now, Buchanan's heart is in tiptop shape. She's looking forward to a big hike during a trip to Greece and Italy this summer.

Asha Krishnaswamy, 53

Health Scientist (Informatics) at CDC/NCBDDD/OD

Here is my incredible journey as a congenital heart patient. I was born with four defects in the heart called tetralogy of Fallot, commonly known as "blue baby". Uncorrected, individuals with this condition rarely survive into adulthood. I had my first surgery at age 4, which helped me attend school. Trying my best to be "normal" despite a long "do-not-do" list I completed high school. Armed with a strong spirit and the support of my family and doctors, I opted to become a software engineer. I successfully completed my master's degree, took up a career in information technology, married and became a mother.

I required additional surgery in my teenage years and again in my twenties and forties. I am one of the first groups of adults living with this congenital heart defect. Stepping into the sixth decade of life, I realize that my heart will never be totally corrected. My advice to others like me is to contact specialized clinics and face the unknown together with the doctors. ​​​


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