Abstract
Congenital heart disease (CHD) occurs in approximately 8 of 1,000 births, with approximately one-third requiring invasive treatment or resulting in death within the first year of life. Parents of children with complex CHD need timely and accurate information regarding the diagnosis, treatments, surgical outcomes, and protocols of follow-up to provide the best care for their children. Decision-making at the time of diagnosis is especially critical; hospital choice is a major predictor of survival, yet most parents do not have access to data about surgical outcomes upon which to choose the most appropriate facility for their child. The information received from physicians, the Internet, and other sources is highly variable, unverifiable, and often inaccurate, further complicating the process of decision-making. Despite the limitations and concerns of publicly reporting data about surgical outcomes, we argue that this information must be accessible to parents at diagnosis to ensure true informed consent and to achieve best outcomes. Improved communication throughout the lifetime of the patient is also needed to ensure awareness and treatment of potential co-morbidities associated with CHD. The Internet provides an unprecedented opportunity for improved education of patients and their parents as well as collection of data regarding issues related to complications and quality of life that are not yet well understood in this population. These methods of communication and collection of data are critical in achieving the best long-term outcomes for patients with complex congenital heart disease.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Karamlou T, Diggs BS, Ungerleider RM, Welke KF. Evolution of treatment options and outcomes for hypoplastic left heart syndrome over an 18-year period. J Thorac Cardiovasc Surg. 2010;139(1):119–26. doi:10.1016/j.jtcvs.2009.04.061; discussion 126–7. Epub 2009 Nov 11.
Chang RK, Klitzner TS. Can regionalization decrease the number of deaths for children who undergo cardiac surgery? A theoretical analysis. Pediatrics. 2002;109(2):173–81.
Checchia PA, McCollegan J, Daher N, Kolovos N, Levy F, Markovitz B. The effect of surgical case volume on outcome after the Norwood procedure. J Thorac Cardiovasc Surg. 2005;129:754–9.
Hirsch JC, Gurney JG, Donohue JE, Gebremariam A, Bove EL, Ohye RG. Hospital mortality for Norwood and arterial switch operations as a function of institutional volume. Pediatr Cardiol. 2008;29(4):713–7. Epub 2007 Dec 14.
McHugh KE, Hillman DG, Gurka MJ, Gutgesell HP. Three-stage palliation of hypoplastic left heart syndrome in the University HealthSystem Consortium. Congenit Heart Dis. 2010;5(1):8–15.
European Association for Cardio-Thoracic Surgery (EACTS) Congenital Database. http://www.eactscongenitaldb.org. Accessed 25 May 2013.
Congenital Heart Disease Website; National Institute for Cardiovascular Outcomes Research (NICOR). https://nicor4.nicor.org.uk/CHD/an_paeds.nsf/vwContent/home?Opendocument. Accessed 25 May 2013.
Nicholls M. UK congenital heart centres under scrutiny. Circulation. 2007;116:F73–8. doi: 10.1161/CIRCULATION AHA.107.186287. http://circ.ahajournals.org/content/116/13/F73.full.pdf. Accessed 25 May 2013
The National Archives; The inquiry into the management of care of children receiving complex heart surgery at the Bristol Royal Infirmary. In: Learning from Bristol: the report of the public inquiry into children’s heart surgery at the Bristol Royal Infirmary 1984–1995. Bristol Royal Infirmary Inquiry. 2001; http://webarchive.nationalarchives.gov.uk/20090811143745/http://www.bristol-inquiry.org.uk/index.htm. Accessed 25 May 2013.
University College London; National Institute for Cardiovascular Outcomes Research (NICOR) Background. http://www.ucl.ac.uk/nicor/audits/congenitalheartdisease/background. Accessed 25 May 2013.
New York State Department of Health. State Health Department Releases reports on cardiac surgery: New York first in nation to release heart valve and pediatric cardiac surgery data. 6 May 2004. http://www.health.ny.gov/press/releases/2004/cardiac_release_05-06-2004.htm. Accessed 25 May 2013.
Arya B, Glickstein S, Levasseur SM, Williams IA. Parents of children with congenital heart disease prefer more information than cardiologists provide. Congenit Heart Dis. 2012. doi:10.1111/j.1747-0803.2012.00706.x [Epub ahead of print].
Feinstein JA, Benson DW, Dubin AM, Cohen MS, Maxey DM, Mahle WT, Pahl E, Villafañe J, Bhatt AB, Peng LF, Johnson BA, Marsden AL, Daniels CJ, Rudd NA, Caldarone CA, Mussatto KA, Morales DL, Ivy DD, Gaynor JW, Tweddell JS, Deal BJ, Furck AK, Rosenthal GL, Ohye RG, Ghanayem NS, Cheatham JP, Tworetzky W, Martin GR. Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol. 2012;59(1 Suppl):S1–42.
Hilton-Kamm D, Chang RK, Sklansky M. Prenatal diagnosis of hypoplastic left heart syndrome: impact of counseling patterns on parental perceptions and decisions regarding termination of pregnancy. Pediatr Cardiol. 2012;33(8):1402–10. doi:10.1007/s00246-012-0366-9.
Bove EL. Current status of staged reconstruction for hypoplastic left heart syndrome. Pediatr Cardiol. 1998;19:308–15.
Gaynor JW, Bridges ND, Cohen MI, Mahle WT, Decampli WM, Steven JM, Nicolson SC, Spray TL. Predictors of outcome after the Fontan operation: is hypoplastic left heart syndrome still a risk factor? J Thorac Cardiovasc Surg. 2002;123(2):237–45.
Mahle WT, Spray TL, Wernovsky G, Gaynor JW, Clark BJ. Survival after reconstructive surgery for hypoplastic left heart syndrome: a 15-year experience from a single institution. Circulation. 2000;102(19 Suppl 3):III136–41.
Mitchell ME, Ittenbach RF, Gaynor JW, Wernovsky G, Nicolson S, Spray TL. Intermediate outcomes after the Fontan procedure in the current era. J Thorac Cardiovasc Surg. 2006;131(1):172–80.
Tweddell JS, Hoffman GM, Mussatto KA, Fedderly RT, Berger S, Jaquiss RD, Ghanayem NS, Frisbee SJ, Litwin SB. Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: lessons learned from 115 consecutive patients. Circulation. 2002;106:I82–9.
Bailey LL. Transplantation is the best treatment for hypoplastic left heart syndrome. Cardiol Young. 2004;14 Suppl 1:109–11.
Galantowicz M, Cheatham JP. Lessons learned from the development of a new hybrid strategy for the management of hypoplastic left heart syndrome. Pediatr Cardiol. 2005;26(3):190–9. PMID: 16179977.
McElhinney DB, Tworetzky W, Lock JE. Current status of fetal cardiac intervention. Circulation. 2010;121(10):1256–63.
Kon AA, Ackerson L, Lo B. How pediatricians counsel parents when no “best-choice” management exists. Arch Pediatr Adolesc Med. 2004;158:436–41.
Kon AA. Healthcare providers must offer palliative treatment to parents of neonates with hypoplastic left heart syndrome. Arch Pediatr Adolesc Med. 2008;162(9):844–8.
Wernovsky G. The paradigm shift toward surgical intervention for neonates with hypoplastic left heart syndrome. Arch Pediatr Adolesc Med. 2008;162(9):849–54.
Jacobs JP, O'Brien SM, Pasquali SK, Jacobs ML, Lacour-Gayet FG, Tchervenkov CI, Austin 3rd EH, Pizarro C, Pourmoghadam KK, Scholl FG, Welke KF, Mavroudis C. Variation in outcomes for benchmark operations: an analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database. Ann Thorac Surg. 2011;92(6):2184–91. doi:10.1016/j.athoracsur.2011.06.008; discussion 2191–2.
Hilton-Kamm D, Sklansky M, Chang RK. How not to tell parents about their child’s new diagnosis of congenital heart disease: an Internet survey of 841 parents. Pediatr Cardiol. 2014;35(2):239–52. doi:10.1007/s00246-013-0765-6.
Prsa M, Holly CD, Carnevale F, Justino H, Rohlicek C. Attitudes and practices of cardiologists and surgeons who manage HLHS. Pediatrics. 2010;125(3):e625–30. Epub 2010 Feb 15.
Marino BS, Tomlinson RS, Drotar D, Claybon ES, Aguirre A, Ittenbach R, Welkom JS, Helfaer MA, Wernovsky G, Shea JA. Quality-of-life concerns differ among patients, parents, and medical providers in children and adolescents with congenital and acquired heart disease. Pediatrics. 2009;123(4):708–15.
Ikemba CM, Kozinetz CA, Feltes TF, Fraser CD, McKenzie ED, Shah N, Mott A. Internet use in families with children requiring cardiac surgery for congenital heart disease. Pediatrics. 2002;109(3):419–22. doi:10.1542/peds.109.3.419.
HLHS Information Page. www.HLHSinfo.org. Accessed 25 May 2013.
CHD Resources. www.CHDResources.org. Accessed 25 May 2013.
Optum Health. https://www.myoptumhealthcomplexmedical.com/gateway/public/patients/evaluationAndCostSavings.jsp. Accessed 25 May 2013.
Aetna Institutes of Excellence™ Pediatric Congenital Heart Surgery Facilities. http://www.aetna.com/docfind/cms/html/institutes_of_excellence_pediatric.html. Accessed 25 May 2013.
Parents Magazine; 10 best children’s hospitals for heart care 2013. http://www.parents.com/health/doctors/best-heart-care-hospitals/. Accessed 25 May 2013.
U.S. News & World Report Methodology; Best children’s hospitals 2012–13. http://www.usnews.com/pubfiles/7-17PedsReport2012_June25.pdf. Accessed 25 May 2013.
U.S. News and World Report: Top-ranked pediatric hospitals for cardiology & heart surgery. http://health.usnews.com/best-hospitals/pediatric-rankings/cardiology-and-heart-surgery. Accessed 25 May 2013.
Boston Children’s Hospital; #1 ranked pediatric hospital by U.S. news and world report. 2013. http://www.childrenshospital.org/microsites/Site3412/mainpageS3412P0.html. Accessed 25 May 2013.
Children’s Hospital of Philadelphia; More no. 1 rankings than any other hospital in nation: CHOP leads U.S. News & World Report’s best children’s hospital list. 2013. http://www.chop.edu/about/best-in-the-nation/. Accessed 25 May 2013.
Mott Children’s Hospital, University of Michigan; Congenital heart conditions and treatments 2013. http://mottchildren.org/medical-services/ped-heart. Accessed 25 May 2013.
Texas Children’s Hospital; Heart Center. 2013. http://www.texaschildrens.org/heart/. Accessed 25 May 2013.
Hilton-Kamm, D. Hypoplastic left heart syndrome and the myths of informed consent. (Invited commentary) American Academy of Pediatrics Bioethics News Letter (Fall 2012): p. 14–17.
Consumer Reports Health and The Society of Thoracic Surgeons. Heart Surgery Ratings Background and Methodology, Consumers Union, August 2011. http://www.consumerreports.org/health/resources/pdf/society-of-thoracic-surgeons/Heart-Surgery-Ratings-Background-and-Methodology.pdf. Accessed 25 May 2013.
Jacobs JP, Cerfolio RJ, Sade RM. The ethics of transparency: publication of cardiothoracic surgical outcomes in the lay press. Ann Thorac Surg. 2009;87(3):679–86. doi:10.1016/j.athoracsur.2008.12.043.
The Society of Thoracic Surgeons; STS public reporting online: Heart Surgery Outcomes – public access. STS Heart Surgery Outcomes – star ratings avail by hospital or surgical group. http://www.sts.org/quality-research-patient-safety/sts-public-reporting-online. Accessed 25 May 2013.
Consumer Reports; Heart bypass surgery ratings. http://www.consumerreports.org/health/doctors-hospitals/surgeon-ratings/ratings-of-bypass-surgeons.htm. Accessed 25 May 2013.
State of California; Office of Statewide Planning and Development (OSHPD) Coronary Artery Bypass Graft (CABG) Surgery in California. http://www.oshpd.ca.gov/hid/Products/Clinical_Data/CABG/index.html. Accessed 25 May 2013.
Department of Health Care Policy – Harvard Medical School. Reports on risk-standardized mortality rates for hospitals performing coronary artery bypass graft surgery and percutaneous coronary interventions in the Commonwealth of Massachusetts. MASS-DAC Data Analysis Center. http://www.massdac.org/index.php/reports. Accessed 25 May 2013.
State of New Jersey Department of Health; Cardiac Surgery. http://www.state.nj.us/health/healthcarequality/cardiacsurgery.shtml. Accessed 25 May 2013.
New York State Department of Health: Cardiovascular disease data and statistics. http://www.health.ny.gov/statistics/diseases/cardiovascular/index.htm. Accessed 25 May 2013.
Pennsylvania Health Care Cost Containment Council (PHC4) Cardiac Surgery in Pennsylvania 2008–2009. http://www.phc4.org/reports/cabg/09/. Accessed 25 May 2013.
Truven Health Analytics; 50 top cardiovascular hospitals. http://www.100tophospitals.com/top-cardio-hospitals/. Accessed 25 May 2013.
Scientific Registry of Transplant Recipients (SRTR); US hospitals with heart transplant centers. http://www.srtr.org/csr/current/Centers/TransplantCenters.aspx?organcode=HR. Accessed 25 May 2013.
Finlayson SRG, Birkmeyer JD, Tosteson ANA, Nease RF. Patient preferences for local care: implications for regionalization. Med Care. 1999;37:204–9.
Vinocur JM, Menk JS, Connett J, Moller JH, Kochilas LK. Surgical volume and center effects on early mortality after pediatric cardiac surgery: 25-year North American Experience From a Multi-institutional Registry. Pediatr Cardiol. 2013;34(5):1226–36.
Hannan E, Kilburn H, Racz M, Shields E, Chassin MR. Improving the outcomes of coronary artery bypass surgery in New York State. JAMA. 1994;271(10):761–6. doi:10.1001/jama.1994.03510340051033.
Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote L. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation. 2007;115:163–72.
Wernovsky G, Rome JJ, Tabbutt S, Rychik J, Cohen MS, Paridon SM, Webb G, Dodds KM, Gallagher MA, Fleck DA, Spray TL, Vetter VL, Gleason MM. Guidelines for the outpatient management of complex congenital heart disease. Congenit Heart Dis. 2006;1:10–26. doi:10.1111/j.1747-0803.2006.00002.x.
Marino BS, Lipkin PH, Newburger JW, Peacock G, Gerdes M, Gaynor JW, Mussatto KA, Uzark K, Goldberg CS, Johnson Jr WH, Li J, Smith SE, Bellinger DC, Mahle WT. American Heart Association Congenital Heart Defects Committee, Council on Cardiovascular Disease in the Young, Council on Cardiovascular Nursing, and Stroke Council. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation. 2012;126(9):1143–72. Epub 2012 Jul 30.
Barreira JL, Baptista MJ, Moreira J, Azevedo A, Areias JC. Understanding of endocarditis risk improves compliance with prophylaxis. Rev Port Cardiol. 2002;21(9):939–51.
Bulat DC, Kantoch MJ. How much do parents know about their children’s heart condition and prophylaxis against endocarditis? Can J Cardiol. 2003;19(5):501–6.
Cheuk DK, Wong SM, Choi YP, Chau AK, Cheung YF. Parents’ understanding of their child's congenital heart disease. Heart. 2004;90(4):435–9.
da Silva DB, Souza IP, Cunha MC. Knowledge, attitudes and status of oral health in children at risk for infective endocarditis. Int J Paediatr Dent. 2002;12(2):124–31.
Fernandes S, Verstappen A, Ackerman K, Adams E, Barton C, Breitinger P, Crumb S, Dummer K, Harada K, Khairy P, Landzberg M, Linstead-Goldsmith R, Meadows AK, Nieves JA, Saidi A, Takahashi M, Zhou J, Ziniel S, Williams R, the Adult Congenital Cardiac Care Associate Research Network. Parental knowledge regarding lifelong congenital cardiac care. Pediatrics. 2011;128:e1489. doi:10.1542/peds.2010-3068.
Frogel MP, Stewart DL, Hoopes M, Fernandes AW, Mahadevia PJ. A systematic review of compliance with palivizumab administration for RSV immunoprophylaxis. J Manag Care Pharm. 2010;16(1):46–58.
Lobel A, Geyer S, Grosser U, Wessel A. Knowledge of congenital heart disease of mothers: presentation of a standardized questionnaire and first results. Congenit Heart Dis. 2012;7:31–40.
Williams IA, Shaw R, Kleinman C, Gersony W, Prakash A, Levasseur SM, Glickstein JS. Parental understanding of neonatal congenital heart disease. Pediatr Cardiol. 2008;29:1059–65. doi:10.1007/s00246-008-9254-8.
Yang HL, Chen YC, Wang JK, Gau BS, Moons P. An evaluation of disease knowledge in dyads of parents and their adolescent children with congenital heart disease. J Cardiovasc Nurs. 2013;28(6):541–9.
Lesch W, Specht K, Lux A, Frey M, Utens E, Bauer U. Disease-specific knowledge and information preferences of young patients with congenital heart disease. Cardiol Young. 2013. doi:10.1017/S1047951113000413. Published online: 29 April 2013.
Rosenfeld JS. Section 504 and IDEA: basic similarities and differences. Wrights Law. http://www.wrightslaw.com/advoc/articles/504_IDEA_Rosenfeld.html. Accessed 25 May 2013.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2015 Springer-Verlag London
About this chapter
Cite this chapter
Hilton-Kamm, D., Haskell, H. (2015). Communication Chaos: How Incomplete and Conflicting Information Prevents Improved Outcomes for Patients with Pediatric and Congenital Cardiac Disease (and What We Can Do About It). In: Barach, P., Jacobs, J., Lipshultz, S., Laussen, P. (eds) Pediatric and Congenital Cardiac Care. Springer, London. https://doi.org/10.1007/978-1-4471-6587-3_37
Download citation
DOI: https://doi.org/10.1007/978-1-4471-6587-3_37
Published:
Publisher Name: Springer, London
Print ISBN: 978-1-4471-6586-6
Online ISBN: 978-1-4471-6587-3
eBook Packages: MedicineMedicine (R0)