Skip to main content
    • Aa
    • Aa
  • Get access
    Check if you have access via personal or institutional login
  • Cited by 16
  • Cited by
    This article has been cited by the following publications. This list is generated based on data provided by CrossRef.

    Singh, Swaran P. and Tuomainen, Helena 2015. Transition from child to adult mental health services: needs, barriers, experiences and new models of care. World Psychiatry, Vol. 14, Issue. 3, p. 358.

    Abu-Sultaneh, Samer Hehir, David A. Murkowski, Kathleen Ghanayem, Nancy S. Liedel, Jennifer Hoffmann, Raymond G. Cao, Yumei Mitchell, Michael E. Jeromin, Andreas Tweddell, James S. and Hoffman, George M. 2014. Changes in Cerebral Oxygen Saturation Correlate With S100B in Infants Undergoing Cardiac Surgery With Cardiopulmonary Bypass. Pediatric Critical Care Medicine, Vol. 15, Issue. 3, p. 219.

    May, Lindsay J. Longhurst, Christopher A. Pageler, Natalie M. Wood, Matthew S. Sharek, Paul J. and Zebrack, C. Michelle 2014. Optimizing Care of Adults With Congenital Heart Disease in a Pediatric Cardiovascular ICU Using Electronic Clinical Decision Support*. Pediatric Critical Care Medicine, Vol. 15, Issue. 5, p. 428.

    Hinze, Alicia Kutty, Shelby Sayles, Harlan Sandene, Erin K. Meza, Jane and Kugler, John D. 2013. Reproductive and Contraceptive Counseling Received by Adult Women with Congenital Heart Disease: A Risk-based Analysis. Congenital Heart Disease, Vol. 8, Issue. 1, p. 20.

    Lawler, Billie A. 2013. The Need for Nursing Education in the Care of Adults With Congenital Heart Defects. The Journal of Continuing Education in Nursing, Vol. 44, Issue. 2, p. 81.

    Favilli, Silvia Santoro, Gennaro Ballo, Piercarlo Arcangeli, Chiara Bovenzi, Francesco M. Chiappa, Enrico Conti, Umberto Monopoli, Anna Murzi, Bruno Rosini, Costanza and Zuppiroli, Alfredo 2012. Prevalence and clinical characteristics of adult patients with congenital heart disease in Tuscany. Journal of Cardiovascular Medicine, Vol. 13, Issue. 12, p. 805.

    Rouine-Rapp, Kathryn Russell, Isobel A. and Foster, Elyse 2012. Congenital Heart Disease in the Adult. International Anesthesiology Clinics, Vol. 50, Issue. 2, p. 16.

    Said, Sameh M. Dearani, Joseph A. Silversides, Candice K. Martinez, Richard M. and Drajpuch, David B. 2011. Longer-term issues for young adults with hypoplastic left heart syndrome: contraception, pregnancy, transition, transfer, counselling, and re-operation. Cardiology in the Young, Vol. 21, Issue. S2, p. 93.

    Wauthy, Pierre Massaut, Jacques Sanoussi, Ahmed Demanet, Hélène Morissens, Marielle Damry, Nasroolla Dessy, Hughes Malekzadeh-Milani, Sophie G. and Deuvaert, Frank E. 2011. Ten-year experience with surgical treatment of adults with congenital cardiac disease. Cardiology in the Young, Vol. 21, Issue. 01, p. 39.

    Brown, Morgan L Dearani, Joseph A and Burkhart, Harold M 2009. The adult with congenital heart disease: medical and surgical considerations for management. Current Opinion in Pediatrics, Vol. 21, Issue. 5, p. 561.

    Dauphin, Claire Labbé, Guillaume and Lusson, Jean-René 2009. Centre de compétence HTAP adulte-enfant : expérience pratique. La Presse Médicale, Vol. 38, p. 1S28.

    Hartyánszky, István Székely, Andrea Király, László Prodán, Zsolt Mihályi, Sándor Bodor, Gábor Tamás, Csaba Kassai, Imre Fazakas, Levente Temesvári, András and Szatmári, András 2009. Surgical management of congenital heart defects in adolescent and adult patients, between years 2001–2008. Orvosi Hetilap, Vol. 150, Issue. 37, p. 1739.

    Medrano López, Constancio Guía Torrent, José Manuel Rueda Núñez, Fernando and Moruno Tirado, Antonio 2009. Actualización en cardiología pediátrica y cardiopatías congénitas. Revista Española de Cardiología, Vol. 62, p. 39.

    Medrano López, Constancio Guía Torrent, José Manuel Rueda Núñez, Fernando and Moruno Tirado, Antonio 2009. Actualización en cardiología pediátrica y cardiopatías congénitas. Revista Española de Cardiología Suplementos, Vol. 9, Issue. 1, p. 39.

    Dimopoulos, Konstantinos Giannakoulas, Georgios Wort, Stephen John and Gatzoulis, Michael A 2008. Pulmonary arterial hypertension in adults with congenital heart disease: distinct differences from other causes of pulmonary arterial hypertension and management implications. Current Opinion in Cardiology, Vol. 23, Issue. 6, p. 545.

    Cohen, Meryl S. Jacobs, Jeffrey P. Quintessenza, James A. Chai, Paul J. Lindberg, Harald L. Dickey, Jamie and Ungerleider, Ross M. 2007. Mentorship, learning curves, and balance. Cardiology in the Young, Vol. 17, Issue. S2,


Caring for adults with congenital cardiac disease: successes and challenges for 2007 and beyond

  • Joseph A. Dearani (a1), Heidi M. Connolly (a2), Richard Martinez (a3), Hector Fontanet (a3) and Gary D. Webb (a4)
  • DOI:
  • Published online: 01 September 2007

Patients with congenital cardiac disease require lifelong medical care. Current challenges that face practitioners who care for adults with congenital heart disease include identifying the best location for procedures, which could be a children’s hospital, an adult hospital, or a tertiary care facility; providing appropriate antenatal management of pregnant women with congenitally malformed hearts, and continuing this care in the peripartum period; and securing the infrastructure and expertise of the non-cardiac subspecialties, such as nephrology, hepatology, pulmonary medicine, and haematology. The objectives of this review are to outline the common problems that confront this population of patients and the medical community, to identify challenges encountered in establishing a programme for care of adults with congenitally malformed hearts, and to review the spectrum of disease and operations that have been identified in a high volume tertiary care centre for adult patients with congenital cardiac disease. Three chosen examples of the fundamental problems facing the practitioner and patient in the United States of America in 2007 are the neglected patient with congenital cardiac disease, weak infrastructure for adults with congenital cardiac disease, and family planning and management of pregnancy for patients with congenital cardiac disease.

Patients with adult congenital cardiac disease often do not receive appropriate surveillance. Three fundamental reasons for this problem are, first, that most adults with congenitally malformed hearts have been lost to follow-up by specialists, and are either receiving community care or no care at all. Second, patients and their families have not been educated about their malformed hearts, what to expect, and how to protect their interests most effectively. Third, adult physicians have not been educated about the complexity of the adult with a congenitally malformed heart. This combination can be fatal for adults with complications related to their congenitally malformed heart, or its prior treatment. Two solutions would improve surveillance and care for the next generation of patients coming out of the care of paediatric cardiologists. The first would be to educate patients and their families during childhood and adolescence. They would learn the names of the diagnoses and treatments, the problems they need to anticipate and avoid, the importance of expert surveillance, career and family planning information, and appropriate self-management. The second solution would be to encourage an orderly transfer of patients from paediatric to adult practice, usually at about 18 years of age, and at the time of graduation from high school.

Clinics for adults with congenital cardiac disease depend upon multidisciplinary collaboration with specialties in areas such as congenital cardiac imaging, diagnostic and interventional catheterization, congenital cardiac surgery and anaesthesia, heart failure, transplantation, electrophysiology, reproductive and high risk pregnancy services, genetics, pulmonary hypertension, hepatology, nephrology, haematology, and others. None of these services are easily available “off the rack”, although with time, experience, and determination, these services can develop very well. Facilities with experienced personnel to provide competent care for adults with congenital cardiac disease are becoming increasingly available. Parents and patients should learn that these facilities exist, and be directed to one by their paediatric caregivers when the time comes for transition to adult care.

With the steady increase in the number of adults with congenital heart disease, an ever increasing number of women with such disease are becoming pregnant. Services are not widely available to assess competently and plan a pregnancy for those with more complex disease. It is essential to have a close interplay between the obstetrician, the adult congenital cardiologist, the fetal medicine perinatologist, and neonatologist.

In both a community based programme and a tertiary care centre, the nuances and complexities of congenital cardiac anatomy, coupled with the high probability of previous operation during childhood, makes the trained congenital cardiothoracic surgeon best suited to deal with the surgical needs of this growing population. It is clear that the majority of adults with congenital heart disease are not “cured”, but require lifelong comprehensive care from specialists who have expertise in this complex arena. There is a growing cadre of healthcare professionals dedicated to improving the care of these patients. More information has become available about their care, and will be improved upon in the next decade. With the support of the general paediatric and paediatric cardiologic communities, and of the Adult Congenital Heart Association, and with the persistence of the providers of care for adults with congenital cardiac disease currently staffing clinics, the care of these patients should become more secure in the next decade as we mature our capabilities.

Corresponding author
Correspondence to: Joseph A. Dearani, MD, Mayo Clinic, 200 First Street, SW, Rochester, MN 55905, United States of America. Tel: +507 255 2034; Fax: +507 255 7378; E-mail:
Linked references
Hide All

This list contains references from the content that can be linked to their source. For a full set of references and notes please see the PDF or HTML where available.

2.GD Webb , RG Williams . 32nd Bethesda Conference: Care of the adult with congenital heart disease: 32nd Bethesda Conference, Maryland, October 2–3, 2000. J Am Coll Cardiol 2001; 37: 11621198.

5.CA Warnes , R Liberthson , GK Danielson , . Task force 1: the changing profile of congenital heart disease in adult life. J Am Coll Cardiol 2001; 37: 11701175.

6.A Knauth , A Verstappen , J Reiss , GD Webb . Transition and transfer from pediatric to adult care of the young adult with complex congenital heart disease. Cardiol Clin 2006; 24: 619629.

7.A Verstappen , D Pearson , AH Kovacs . Adult congenital heart disease: the patient’s perspective. Cardiol Clin 2006; 24: 515529.

8.JS Child , RL Collins-Nakai , JS Alpert , . Task force 3: workforce description and educational requirements for the care of adults with congenital heart disease. J Am Coll Cardiol 2001; 37: 11831187.

9.MJ Landzberg , DJ Murphy Jr., WR Davidson , . Task force 4: organization of delivery systems for adults with congenital heart disease. J Am Coll Cardiol 2001; 37: 11871193.

11.S Thorne , C Nelson-Piercy , A MacGregor , . Pregnancy and contraception in heart disease and pulmonary arterial hypertension. J Fam Plann Reprod Health Care 2006; 32: 7581.

12.C Oakley , CA Warnes . Heart disease in pregnancy, 2nd edn.Blackwell Pub/BMJ Books, Malden MA, Oxford, 2007.

14.RG Williams , JS Child , KS Kuehl , M Myerson , DJ Sahn , CL Webb . Report of the national heart, lung and blood institute working group on research in adult congenital heart disease. J Am Coll Cardiol 2006; 47: 701707.

16.JA Quintessenza , JP Jacobs , VO Morell , JM Giroud , RJ Boucek . The initial experience with a bicuspid polytetrafluoroethylene pulmonary valve in 41 children and adults: a new option for right ventricular outflow tract reconstruction. Ann Thor Surg 2005; 79: 924931.

17.J Deanfield , E Thaulow , C Warnes , . The taskforce on the management of grown up congenital heart disease of the European society of cardiology. Eur Heart J 2003; 24: 10351084.

18.C Warnes . The adult with congenital heart disease: born to be bad? J Am Coll Cardiol 2005; 46: 18.

Recommend this journal

Email your librarian or administrator to recommend adding this journal to your organisation's collection.

Cardiology in the Young
  • ISSN: 1047-9511
  • EISSN: 1467-1107
  • URL: /core/journals/cardiology-in-the-young
Please enter your name
Please enter a valid email address
Who would you like to send this to? *