Introduction

The Royal Brompton Hospital (RBH) has a long tradition of innovation and leadership in congenital heart disease. While children’s lives have been transformed by early intervention, many have ongoing needs into adulthood, including further surgery, catheter intervention and/or other procedures. There is both an adult congenital heart disease (ACHD) unit at the RBH and a transplant unit at Harefield hospital, where Professor Sir Magdi Yacoub established heart transplantation in the UK, showed excellent results from the Ross procedure and pioneered the modern arterial switch operation. Many ACHD patients have pulmonary hypertension (PH) and the RBH is the only ACHD centre that is also a designated National Pulmonary Hypertension Centre of Excellence. Through a shared care network of regional partner hospitals, high quality care can be provided closer to home, not just for ACHD patients with PH but all types of PH.

World-leading ACHD research contribution

The role of the RBH ACHD team does not end in patient care as the team is passionate about research as a means by which clinical care can improve, resulting in improved life-span and better quality of life for our ACHD patients. A recent publication showed the world leading contribution of RBH to ACHD research in terms of the importance (impact factor) attributed to research studies accepted for publication after peer review and critical appraisal (Figure 1)

ACHD research collaboration

Our research encompasses all types and aspects of ACHD and embraces collaboration between multiple areas of expertise (Figure 2). Our teamwork approach between adult congenital heart disease (ACHD) cardiologists and surgeons, experts in imaging, electrophysiology, intensive care, palliative care, transplantation, paediatrics and others ensures joined up care for all ACHD patients. This teamwork approach also enables research and extends to working with other units within the UK (e.g. Southampton and Oxford) and across the world (especially Europe, North America and Japan). The team remains enthusiastic to further develop existing collaborations or start new ones to facilitate ACHD research.

Clinical research environment and funding

Our research efforts have over time been supported by funding, including from the British Heart Foundation, the National Institute of Health Research (NIHR) and industry. In recent years, our NIHR Royal Brompton Cardiovascular Biomedical Research Unit (BRU) was created which focuses on clinical research that aims to discover new diagnostic tests and treatments for patients; ACHD is a major theme led by Prof Michael Gatzoulis. The BRU enables access to dedicated state-of-the-art, research-funded facilities for clinical research, genetics and bioinformatics aimed at “bench-to-bedside translation.”

Training the next generation of ACHD clinical researchers

The Royal Brompton ACHD fellowship program, initiated and led by Prof Gatzoulis, is  one of the largest training fellowship programs in the world, educating and enabling over 100 cardiologists to roll out improved practices for ACHD and/or PH in their respective regions. Many of our previous fellows have gone on to establish and lead specialist ACHD clinical and research programs in their home city whether in the UK or abroad. Qualified doctors also complete periods of clinical ACHD research work towards being awarded PhDs from Imperial College London.

Involving patients and the public in our research

As well as participating in research, patients are actively involved in developing our research, ensuring it is relevant to them. Our researchers have opportunities to meet with patients and carers through our patient advisory groups and ACHD patient conferences organised at our centre. Incorporating the patient perspective into developing research ideas keeps our focus on health improvements important to people living with adult congenital heart disease. In addition to patient involvement, public engagement is also important to our research. Our team contributes to public events to raise the profile of ACHD, for example this has recently included researchers’ night at the Natural History Museum, the annual Imperial Science festival and working with charities.

Selection of RBH ACHD research themes

Below some of our major research themes are discussed. Selected specific research interests of the senior ACHD team are summarised in Table 1.

·         Pulmonary Hypertension

Pulmonary hypertension (PH) encompasses conditions characterised by high blood pressure within the blood vessels of the lung. PH is a severe condition as it causes a strain on the right side of the heart and results in shortness of breath and heart failure. PH research at RBH and its academic partner, Imperial College London, covers the entire spectrum of PH categories and embraces both laboratory-based and clinical studies on patients. Our laboratory research focuses on various topics: we aim to understand how inflammation affects the thickening of the blood vessels in the lungs causing PH, by using cells grown from patients’ lungs removed during operations or after transplantation. We are also working on blood vessel cells taken from patients’ blood called “stem cells”: these cells may one day be used regenerate healthy blood vessels in the lungs. Moreover, we are exploring the use of microscopic particles called “nanoparticles” in directing PH drugs to where they work best. Clinical studies in PH focus on understanding PH in ACHD and identifying ways of improving outcome including prolonging life. We are also running trials on new medications and are part of the national network looking at the genetics behind the development of PH.

·         Heart imaging

Both echocardiography and cardiovascular magnetic resonance are suited to clinical research as they can assess the heart harmlessly without the risks of ionising radiation associated with X-ray. Research in the field of ACHD echocardiography applies advanced imaging techniques including 3D and 4D imaging, myocardial contrast perfusion imaging and stress echocardiography to obtain a better understanding of heart structure and function. This way, we can carefully monitor disease progression and guide optimal management. The CMR unit, the first such unit in Europe, opened in 1984 and from its earliest days included clinical research relevant to ACHD patients in its research focus. Current clinical research aims include understanding heart function in ACHD and PH and mechanisms by which it may deteriorate, and finding CMR features that predict timing of treatment, response to treatment, heart rhythm disturbance or survival. The role of heart scarring or fibrosis imaged by CMR is being investigated as well as ways to use 3D imaging to make invasive investigations and treatments easier and more successful.

·         Improving outcomes for ACHD patients

Working in collaboration with paediatric and fetal cardiologists and other experts, our research team has been identifying ways for improving patient outcomes.  Our research benefits from clinicians with expertise in epidemiology (the science that studies the patterns, causes, and effects of health and disease conditions in defined populations) and statistics, enhancing our ability to identify predictors of outcome in ACHD patients overall and within major disease groups such as tetralogy of Fallot, Eisenmenger syndrome, transposition of the great arteries and patients with Fontan circulation.

·         Arrhythmia

Research is thriving in ACHD and electrophysiology, the branch of cardiology aimed at preventing and treating heart rhythm disturbances. Our electrophysiology consultants are able to treat abnormal heart rhythms in patients with even the most complex heart anatomies, using cutting edge advanced technology only available in a handful of centres around the world. They can offer implantation of pacing devices aimed at improving heart function and treating very fast or extremely slow heart rhythms and defibrillator devices to shock the heart if a life-threatening rhythm occurs. Current research is towards developing and applying new technologies that will improve the success of arrhythmia management in ACHD patients.

·         Intervention via catheter or surgery

Several hundreds of children and ACHD patients benefit from surgery or catheter intervention in our centre every year enabling study of the outcomes of ACHD surgical and catheter intervention. Research is also aimed at advancing the development or application of new devices or techniques, such as valves implanted via catheter.

·         Marfan syndrome and diseases of the aorta

Aortopathy refers to diseases that affects the aorta, the major vessel carrying blood from the heart to the body, and is not uncommon in ACHD. It often presents as enlargement of different segments of the aorta, and may require surgery or other intervention. Our unit has ongoing studies in Marfan syndrome and aortopathy from other conditions such as bicuspid aortic valve. This includes the ongoing trial of medication (Irbesartan) to prevent aortic dilatation in Marfan patients. Research into the genetics of aortic disease is also underway involving next generation genetic sequencing to investigate families with other types of aortopathy.

·         Pregnancy and heart disease

The RBH, in partnership with Chelsea and Westminster Hospital, runs a busy pregnancy and heart disease program. Patients are seen for advice on pregnancy and are followed throughout pregnancy and delivery by an expert team of consultants. Our pregnancy and heart disease team are also active in research which looks into improving the outcome of pregnancy, both for the mother and the child.

A word of thanks to our patients

All of the research described would not be possible without the generous support and contribution of the Royal Brompton ACHD patients, to whom our team is very grateful. If you want to find out more about our research and ways you can get involved, please visit our website or email research-findoutmore@rbht.nhs.uk.

 

By Dr Kostas Dimopoulos and Dr Sonya V Babu-Narayan

Royal Brompton Hospital Adult Congenital Heart and Pulmonary Hypertension Centre

Email: achd@rbht.nhs.uk

 

Figure 1 – Systematic review of the scientific contribution to ACHD research by city worldwide (a), in Europe (b) and for the UK (c) in terms of impact factor (importance of publications) for the years 1995-2011.2 The circle above each city represents the relative contribution to ACHD research.  The UK makes a high contribution with regards ACHD research publications and in particular Royal Brompton (red). From “Kempny A, Fernández-Jiménez R, Tutarel O, Dimopoulos K, Uebing A, Shiina Y, Alonso-Gonzalez R, Li W, Swan L, Baumgartner H, Gatzoulis MA, Diller G-P. Meeting the challenge: the evolving global landscape of adult congenital heart disease. Int J Cardiol. 2013;168:5182–5189.”

Figure 2 – Major collaborative ACHD research themes.

 

Table 1 – RBH ACHD team selected major research interests.

Name Major research interests
Prof. Michael Gatzoulis Research and educational lead
Dr Rafael Alonso Pulmonary hypertension, heart failure and transplantation
Dr Babu-Narayan Cardiovascular magnetic resonance, arrhythmia, outcomes, tetralogy of Fallot
Dr Gerhard Diller Pulmonary hypertension, exercise intolerance, health economics
Dr Kostas Dimopoulos Epidemiology, pulmonary hypertension, imaging, exercise testing
Prof. Yen Ho Morphology (anatomy) of congenital heart diseases
Dr Sabine Ernst Arrhythmias
Dr Philip Kilner Cardiovascular magnetic resonance
Dr Wei Li Echocardiography
Dr Laura Price Pulmonary hypertension
Mr Babulal Sethia Congenital heart surgery outcomes, global care
Prof Darryl Shore Congenital heart surgery outcomes, RVOTO reconstruction, Ross operation
Dr Lorna Swan Pregnancy, aortopathy
Dr Anselm Uebing Catheter intervention, pregnancy
Prof Hideki Uemura Congenital heart surgery outcomes, morphology, Ebstein’s anomaly
Dr Tom Wong Arrhythmias and pacing
Dr John Wort Pulmonary hypertension

 

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