European Heart Journal Supplements (2016) 18 (Supplement E), E15–E18 The Heart of the Matter doi:10.1093/eurheartj/suw011

The care for adults with congenital heart disease: organization and function of a grown-up congenital heart disease unit `1, Alessandro Giamberti1*, Alessandro Varrica1, Giuseppe Pome Angelo Micheletti2, Diana Negura2, Marco Ranucci3, Mario Carminati2, Alessandro Frigiola1, and Massimo Chessa2 1

Congenital Cardiac Surgery Unit, IRCCS Policlinico San Donato, Via Morandi 30, San Donato M.se (Mi) 20097, Italy Pediatric and Adult Congenital Heart Center, IRCCS Policlinico San Donato, San Donato M.se (Mi), Italy 3 Department of Cardio-Anesthesia and Intensive Care Unit, IRCCS Policlinico San Donato, San Donato M.se (Mi), Italy 2

KEYWORDS Congenital heart disease; Adult with congenital heart disease; Cardiac surgery; Interventional cardiology;

Thanks to the improvement of surgical and interventional cardiac procedures, the majority of children with congenital heart defects (CHD) can now be expected to reach adulthood. The number of adults with congenital heart disease (ACHD) will inevitably increase, and recent data affirm that in Europe, we are actually faced with an estimated patient population of 2.3 million. These patients, particularly the adults with moderate and highly complex CHD, can be very difficult to manage, and should be treated in few experienced and specialized ‘grown-up congenital heart disease’ (GUCH) units, concentrating resources, patients, funding, and professional experiences. On 2000, we created a GUCH unit in our hospital that rapidly became a referral centre in Italy for number and complexity of patients treated. The present study is a presentation of how is organized our GUCH unit and a brief overview of our more recent experience with ACHD patients.

Introduction Thanks to the improvement of surgical and interventional cardiac procedures in the last 30 years, over 85% of the children with a congenital heart defects (CHD) can now be expected to reach adulthood. The number of adults with congenital heart disease (ACHD) will inevitably increase in the near future,1 and recent data suggest that the number of ACHD, whether repaired or not, approaches the number of children with the disorder.2 Data published3 affirm that in Europe, we are actually faced with an estimated ACHD patient population of 2.3 million, which significantly outnumbers the paediatric CHD population of 1.9 million patients. There is a large consensus that ACHD patients, particularly those with moderate and highly complex CHD can be

* Corresponding author. Tel: +39 02 52774519, Fax: +39 02 55602262, Email: [email protected]

very difficult to manage and that these patients have very special needs. The majority of these patients require long-term surveillance and often reoperations or cardiac catheterizations for residual defects, complications, or degeneration of prosthetic materials. Physicians dedicated to complex ACHD need specific expertise and training and they should be concentrated in centres of excellence that will provide care of the highest level. This is a timely topic of increasing clinical interest, as shown by recent publications,4,5 suggesting that complex ACHD patients should be treated and managed in few experienced and specialized ‘grown-up congenital heart disease’ (GUCH) units, where these patients are frequently treated, concentrating resources, patients, funding, and professional experiences. The European Society of Cardiology (ESC) through the Working Group on Grown-up Congenital Heart Disease has

Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2016. For permissions please email: [email protected]

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recently published practice guidelines for the management of GUCH patients6 and recommendations for organization of care for ACHD in Europe.7 On 2000, conscious that the management and treatment of ACHD was a growing and emerging problem, we created a GUCH unit in our hospital that rapidly became a referral centre in Italy for number and complexity of patients treated. The present study is a presentation of how is organized our GUCH unit and a brief overview of our experience with ACHD patients.

Unit structure Our GUCH unit, created on 2000, became rapidly a supra-regional centre of excellence providing care of the highest level, providing education and training, and promoting research and innovation. The GUCH unit is located inside the Cardio-Vascular Department of IRCCS Policlinico San Donato where adult and paediatric cardiology, and acquired and congenital heart surgery services are available since 1988. The entire spectrum of adult medicine is available including: electrophysiology, coronary artery and valvular interventions (acquired cardiac surgery), ECMO team, and internal medicine (nephrology, neurology, hepatology). Weekly outpatients clinics are working specifically dedicated to pulmonary hypertension patients and chronic heart failure patients. All invasive procedures (surgery, catheter interventions, electrophysiology ablations) can be performed under direct supervision of GUCH experts. All GUCH patients are discussed in regular multi-disciplinary conferences. We created strong links with two Transplant Centres and with services and/or experts not available in our hospital like: (i) obstetric and gynaecology department for pregnancy in woman with CHD; (ii) sport medicine expert; (iii) microbiologists; and (iv) genetists. Since 2006 a psychocardiology service, including Peer Counselling, is active and dedicated to adults with congenital heart disease. A 24 h emergency service for GUCH patients with on-call GUCH specialist experts is available.

Staff As suggested in the ‘recommendations’7 from the ESC Working Group on Grown-up Congenital Heart Disease, all the physicians involved in the GUCH unit have extensive experience and expertise in GUCH patients. Three cardiologists fully trained are available together with three cardiac surgeons able to perform all the cardiac surgery procedures in GUCH patients. At the same time are available: one more cardiac surgeon expert on acquired heart surgery frequently involved in GUCH patients, two specialists in imaging, three anaesthesiologists with CHD experience, and one interventional electrophysiologist with GUCH experience. Psychosocial service is also available with three full-time psychologists and one Peer Counsellor.

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Nurses specifically dedicated to GUCH patients are available in intensive care unit, in the wards, and in the rehabilitation department.

Equipment All diagnostic facilities are available: ECG, Holter monitoring, cardiopulmonary exercise test, echocardiography, MRI, and CT scan. Electrophysiology lab (electrophysiology studies, ablation therapy, PM, and ICD implantation), catheterization lab (diagnostic and interventional procedures), theatres, and intensive care unit are fully equipped including ECMO support.

Scientific activity A Research Unit specifically dedicated to GUCH patients has been created on 2012 strictly linked with the GUCH unit providing education and promoting research and innovation. The Research Unit coordinates and supports all the papers published on GUCH patients in our hospital.8–24 Since 2000, 70 papers on GUCH topics have been published in peer-reviewed journals. We organize every 2 years the ‘Italian Meeting on Adults with Congenital Heart’ arrived at the edition number 6 (next will on March 2016) that is the most important Italian meeting dedicated to GUCH problems. Dr Massimo Chessa and Dr Alessandro Giamberti (GUCH Unit Directors) are Editors of a Series of 10 books on ‘Congenital Heart Disease in Adolescents and Adults’ published by Springer International with the endorsement of the ESC Working Group on Grown-up Congenital Heart Disease. The first two books have been already published.25,26 Education to the local team is provided by regular (every 4 months) internal meetings with experts, monthly ‘Journal Club’ on GUCH topics, and with ‘Teaching Courses’ for nurses on GUCH patients management.

Clinical activity Adults with CHD represent an important part of our clinical activity in terms of number of patients submitted surgical and cath lab procedures. During the last 5 years (January 2010–December 2014), 2189 patients with CHD were operated on in our institution. Of these, 362 (17%) were ACHD, defined as patients with CHD aged older than 18 years. The procedures most frequently performed have been: pulmonary valve implantation or conduit replacement in 116 patients (32%), atrial septal closure in 94 (26%), aortic valve surgery in 53 (15%), tetralogy of Fallot repair in 20 (6%), Fontan conversion or revision in 20 (6%), tricuspid valve surgery in 15 (4%), mitral valve surgery in 13 (4%), and other in 30 (8%) (Figure 1). Similarly, in the same period in catheterization lab, on 2867 cardiac catheterizations performed, 1159 (40%) were in GUCH patients including: 701 (60%) diagnostic procedures, and 458 (40%) interventional procedures. In addition, 463 adult patients have been submitted PFO closure (Figure 2).

The care for adults with congenital heart disease

Figure 1

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Surgical procedures performed in adults with congenital heart defects in the period 2010–14.

The number of adults with CHD continues to increase by 5% per year with more than 1 million individuals in the USA and more than 2 million in Europe.3 Residual haemodynamic and electrophysiological abnormalities are present very often, especially in patients with moderate and highly complex CHD. Residual defects, shunts, complications, degeneration of prosthetic materials, ventricular dysfunction, and arrhythmias contribute to the increasing number of patients at risk for heart failure. As ACHD patients are getting older, we are observing more and more patients with comorbidities like systemic hypertension, diabetes, chronic renal failure, and coronary artery disease. These patients can be very difficult to treat and as suggested by ESC,6,7 they must be managed in few experienced specialized centres concentrating resources, patients, funding, and professional experiences. In this contest, we created on 2000 a GUCH unit in our institution that became rapidly a referral centre in Italy. We reported here how is organized our GUCH unit and some data about our recent clinical activity. Figure 2 Activity of cath lab dedicated to adults with congenital heart defects in the period 2010–14.

Outpatient clinic, working 5 days a week, is following more than 1000 patients. More than 100 patients with chronic heart failure are regularly controlled and 21 of them in the last 7 years have been sent for heart transplantation. Fifty-three patients with pulmonary hypertension are enrolled in different protocols of medical treatment in our GUCH unit.

Conclusion The advances of surgical and interventional cardiac procedures have allowed to the majority of patients born with CHD to reach adulthood.

Conflict of interest: none declared.

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The care for adults with congenital heart disease: organization and function of a grown-up congenital heart disease unit.

Thanks to the improvement of surgical and interventional cardiac procedures, the majority of children with congenital heart defects (CHD) can now be e...
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