Adult Congenital Heart Disease: A Specialty With Ever-Expanding Challenges.

HomeCirculationVol. 147, No. 12Adult Congenital Heart Disease: A Specialty With Ever-Expanding Challenges Free AccessEditorialPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessEditorialPDF/EPUBAdult Congenital Heart Disease: A Specialty With Ever-Expanding Challenges Eric Rosenthal and Shakeel A. Qureshi Eric RosenthalEric Rosenthal Correspondence to: Eric Rosenthal, MD, Department of Paediatric and Adult Congenital Heart Disease, Evelina London Children’s Hospital, Guy’s and St. Thomas’ NHS Foundation Trust, London, SE1 7EH, UK. Email E-mail Address: [email protected] https://orcid.org/0000-0003-3030-9749 Department of Paediatric and Adult Congenital Heart Disease, Evelina London Children’s Hospital, Guy’s and St. Thomas’ NHS Foundation Trust, London, UK. Search for more papers by this author and Shakeel A. QureshiShakeel A. Qureshi Department of Paediatric and Adult Congenital Heart Disease, Evelina London Children’s Hospital, Guy’s and St. Thomas’ NHS Foundation Trust, London, UK. Search for more papers by this author Originally published21 Mar 2023https://doi.org/10.1161/CIRCULATIONAHA.122.063189Circulation. 2023;147:939–941This article is a commentary on the followingRisk of Heart Failure in Congenital Heart Disease: A Nationwide Register-Based Cohort StudyAdults With Congenital Heart Disease: Trends in Event-Free Survival Past Middle AgeArticles, see p 930 and p 982In the 1960s and 1970s, it became apparent that children with congenital heart disease (CHD) were surviving in increasing numbers but with no natural pathway for their follow-up and management. Both clinical expertise and dedicated facilities to look after these patients were lacking, as was insight into the natural and unnatural history. In Toronto, one of the first adult CHD (ACHD) clinics was set up in the 1960s by Gary Webb, and in the 1970s, a similar unit was set up at the National Heart Hospital in London by Jane Somerville.1 These paved the way for others to follow. The term Grown-up CHD was used to distinguish this new specialty from pediatric CHD and adult cardiology in the United Kingdom and Europe.2 The term grown-up CHD was perceived as a comforting one for many at that time but patronizing by others. It gradually fell out of favor and was replaced with ACHD. The European Society of Cardiology held onto this term for many years, but in 2020, its guidelines referred to ACHD and no longer grown-up CHD for the first time.3 The earlier centers attracted referrals from far beyond their local area, and consideration was given to managing these patients in a restricted number of units to develop and maintain expertise, a view not shared by all.4Globally, the development of ACHD units proceeded in a nonuniform way. In our unit at Guy’s Hospital, the first ACHD clinics were set up in the 1990s: fortnightly outpatient clinics on a Friday afternoon (least attractive time for a clinic) held in the pediatric cardiology department using its infrastructure and staff. Of course, today, it is entirely different, with a dedicated ACHD department, 6 full-time and 3 part-time consultants, and 5 clinical nurse specialists with daily outpatient clinics, outreach clinics, and inpatient medical, interventional, arrhythmia, obstetric, psychological, and surgical facilities. Indeed, in developed countries, virtually all pediatric congenital cardiology services now have an affiliated ACHD service—often colocated—and in stand-alone children’s hospitals, a robust pathway to such a service. The patient volume is increasing year to year, as does the complexity of management. When fetal cardiology is also onsite, patients can have ideal, seamless care from fetus to adult delivered by the same clinical team. Following patients throughout their lifetime and seeing and managing offspring with their parents’ disease deliver continuity of care unlike that in any other specialty.The article by Dellborg et al5 in this issue of Circulation reports data collected by the Swedish National Inpatient, Outpatient and Cause of Death Register on patients with CHD born between 1950 and 1999 who were alive at 18 years of age and compared them with normal population controls identified from the Total Population Register. There were 37 278 cases with ACHD and 412 799 controls enrolled in a national registry from 1968 on for up to 50 years. The survival in those with ACHD was compared with that of at least 10 controls matched for sex and year of birth. Over a mean follow-up of 19 years, 5.2% of those with ACHD died, and the mortality was 3.2 times higher compared with matched controls. The highest mortality was in patients with conotruncal defects, but there was also a higher mortality in those with simpler defects such as atrial and ventricular septal defects. It is important to note that those with ACHD were also at an increased risk of developing atrial fibrillation, heart failure, and nonfatal myocardial infarction compared with the control population. The mortality rate of 2.7 per 1000 patient-years was, however, lower than that seen in specialist tertiary centers.6 It is encouraging that >75% of those with ACHD were alive after 50 years of follow-up, although in those with complex and severe lesions, the survival was down to 50%. It is not surprising that mortality was reduced in those born after the mid-1970s, even for those with complex disease, reflecting the great advances in surgical, interventional, and medical care.7,8 Indeed, congenital heart defects considered to be exclusively surgical are more and more commonly treated by catheter techniques, for example, in sinus venosus atrial septal defects and with percutaneous valve implantation.9,10 One of the key strengths of this article is the use of unselected nationwide data, thus avoiding the bias seen in specialist referral centers, although the accuracy of the diagnoses could not be verified. Furthermore, by focusing only on mortality, the authors provide no information on the types, frequency, and timings of comorbidities.11 The article reinforces the message that treating patients with CHD improves their survival but does not make their survival similar to that of the normal population and therefore they need to be followed up indefinitely because their needs are different from those of the normal population.The accompanying article by Berg et al12 from the same institution in this issue also uses the Swedish National Inpatient, Outpatient and Cause of Death Register and focuses on the risk of heart failure in those with CHD compared with matched normal population controls without CHD. Data were obtained for those with CHD between 1930 and 2017, and cases with CHD were matched by sex and year of birth to 10 controls without CHD. Those with CHD were subdivided into groups with different lesions, similar to the approach of Dellborg et al. A total of 89 532 cases with CHD and 890 469 matched controls without CHD were included. Heart failure, identified with International Classification of Diseases codes, occurred in 7.8% of those with CHD and 1.1% of controls, and the mean age at which heart failure developed was 25 years earlier than in controls. The highest risk of developing heart failure was in the youngest age groups. This study also highlighted the need for improving the management of heart failure in patients with CHD and its occurrence at a younger age. The article is valuable because of its large unselected national population, although the use of International Classification of Diseases codes has drawbacks.13ACHD as a condition with a specialty focus has developed beyond all recognition from its origins. Although many patients with ACHD can be considered to be “cured” and likely have a life expectancy not different from that of the population without ACHD, others require ongoing care, with residual lesions and new issues continuing to arise. Intra-atrial reentry tachycardias after complex surgical repair, liver disease after univentricular repair, pulmonary hypertension, and heart failure requiring medical management and even heart and heart/lung transplantation are increasingly encountered in clinical practice as patient survival improves, and all require specialist ACHD management.14,15 These are in addition to the diseases of aging such as ischemic heart and cerebrovascular disease, hypertension, atrial fibrillation, diabetes, and heart failure. Thus, care of these patients as they enter the fifth and sixth decades is going to require coordination of care with ACHD specialists, adult cardiologists, and elderly specialists, the last two currently having little expertise in dealing with congenital issues.16There are more patients with ACHD than children with CHD alive today, and the numbers will continue to increase. The ACHD specialist of the future can expect to see increased survival but will also need to deal with more morbidity and develop an understanding of and expertise in cardiovascular diseases of aging. New treatments introduced for heart failure in adult cardiology will need to be applied rapidly to the ACHD population to extend the benefits seen with the current treatment of children with CHD. Angiotensin receptor/neprilysin inhibitors have recently emerged as a standard of therapy for adults with heart failure, and early reports from its use in patients with ACHD are promising, although not universally so.17,18 Will heart failure with a systemic right, left, or single ventricle behave differently with current and newer agents? Extended use in ACHD with open-label and randomized trials is urgently needed.19The articles by Dellborg et al and Bergh et al highlight the outcome of the spectacular advances in surgical, interventional, and medical care that have resulted in the survival of >90% of patients with CHD to adulthood,20,21 as well as some of the ongoing morbidity. The population register and the patient register in Sweden are hugely valuable and informative sources of comparative data, and the authors have done an excellent job of collecting and comparing these data. These should be taken as a benchmark for future studies to enable evidence-based management of CHD. At no other time in the history of CHD treatment has there been a greater need for a coordinated multidisciplinary approach to the care of these patients.Article InformationDisclosures None.FootnotesThe opinions expressed in this article are not necessarily those of the editors or of the American Heart Association.For Disclosures, see page 940.Circulation is available at www.ahajournals.org/journal/circCorrespondence to: Eric Rosenthal, MD, Department of Paediatric and Adult Congenital Heart Disease, Evelina London Children’s Hospital, Guy’s and St. Thomas’ NHS Foundation Trust, London, SE1 7EH, UK. Email eric.rosenthal@gstt.nhs.ukReferences1. Perloff JK. Congenital heart disease in adults: a new cardiovascular subspecialty.Circulation. 1991; 84:1881–1890. doi: 10.1161/01.cir.84.5.1881LinkGoogle Scholar2. Warnes CA. Jane Somerville.Clin Cardiol. 2008; 31:183–184. doi: 10.1002/clc.20279CrossrefMedlineGoogle Scholar3. 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Survival in children with congenital heart disease: have we reached a peak at 97%?J Am Heart Assoc. 2020; 9:e017704. doi: 10.1161/JAHA.120.017704LinkGoogle Scholar eLetters(0)eLetters should relate to an article recently published in the journal and are not a forum for providing unpublished data. Comments are reviewed for appropriate use of tone and language. Comments are not peer-reviewed. Acceptable comments are posted to the journal website only. Comments are not published in an issue and are not indexed in PubMed. Comments should be no longer than 500 words and will only be posted online. References are limited to 10. 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