Blanche Cupido | Consultant Adult Cardiologist, Groote Schuur Hospital, Cape Town, South Africa; University of Cape Town (UCT) Private Academic Hospital, South Africa
Citation: EMJ Cardiol. 2023; DOI/10.33590/emjcardiol/10309169. https://doi.org/10.33590/emjcardiol/10309169.
As a senior clinical cardiologist, can you tell us about what inspired you to specialise in adult congenital heart disease?
I have worked in the public healthcare sector in South Africa since 2001, when I obtained an undergraduate degree from the University of Cape Town (UCT). South Africa is a diverse country and is, regrettably, also known as the most unequal country in terms of income distribution globally (Gini score of inequality 63 in 2021). Although we provide excellent care to those who can afford it, many of our patients lack access to even basic primary level healthcare. During my time as a junior doctor, working in rural Northern KwaZulu-Natal, one of the poorest provinces in South Africa, I witnessed firsthand the effects of the fragmented health service, and subsequently made a commitment to improving healthcare to those with meagre financial ability.
After completing my subspecialisation in cardiology in 2013, I encountered several patients with congenital heart disease (CHD), presenting either with complicated pregnancies or clinical emergencies (heart failure and arrhythmias). I recall a 24-year-old female patient, presenting in pregnancy at 26 weeks gestation, with heart failure and profound cyanosis. The patient was known with an atrio-ventricular septal defect, banded in infancy. Their family moved to a rural area, and they were subsequently lost to follow-up, thus never receiving definitive corrective surgery. The patient presented after a care gap of over 22 years with severe pulmonary stenosis, due to the pulmonary artery band (gradient 120 mmHg)!
Another patient presented at 36 weeks gestation, with a dilated aortic root of 6 cm, and severe aortic incompetence due to a bicuspid aortic valve (de novo diagnosis). Upon attempting to seek clinical help in the management of these complex patients, it became clear that there was a distinct deficiency in expertise in complex adult CHD (ACHD) in South Africa, sparking my desire to improve my own knowledge pertaining to these disorders. I had the opportunity to train as an ACHD Fellow with Kate English at Leeds General Infirmary, UK. Not only did I gain experience in the management of patients with ACHD, but learnt a great deal around systems and service delivery in this field. My relationship and collaboration with this amazing clinical unit has continued.
In 2017, you established a dedicated ACHD-specialist driven ACHD unit in Cape Town, the first of its kind in Sub-Saharan Africa. Could you please elaborate on the significance of establishing this unit? What were the challenges you faced and had to overcome in the process?
There are several other clinics where adult patients with CHD are being seen, and where important and much needed work is being done diligently. My aim, however, was to establish an ACHD-specialist-led referral centre, where complex adult patients with CHD could be assessed and offered a complete range of clinical services, on par with similar centres of excellence abroad. Starting a service from scratch posed many challenges. There was little awareness or recognition for the need of such a service, both from our local institution and the regional and national healthcare sector. In 2013, prior to my training, we noted that within the existing cardiac clinic services, we had around 40–50 patients with CHD, despite children’s surgery having started in the 1960s, and continuing at a rate of 350–400 surgeries per annum at the children’s hospital. These patients, once overaged for the children’s hospital, were often lost to follow-up, as no adequate CHD services existed in the adult cardiology framework. Many patients remained under the care of their paediatrician, though these visits were often erratic. When admissions for emergencies were required, overage patients could only be admitted to adult hospitals, where none of the clinicians knew them or the complexity of their disease, or were not necessarily trained adequately to deal with it.
Initially, I utilised existing clinic space and time to create this specialised clinic; no additional hospital resources were required. It started as a fortnightly service, alternating with the general cardiac clinic. Most referrals came from the overaged population at the local children’s hospital. The first task was to build a service on the adult cardiology side, in parallel with a new established transition clinic at the children’s hospital. Over a period of 3 years, most overaged patients transitioned successfully to the adult congenital clinic. Currently, we transfer patients around age 15.
The service has allowed for continuity of care and the effective reduction of care gaps. We collaborate extensively with our paediatric colleagues, and have established a multidisciplinary team, including advanced imaging (MRI and CT), cardiopulmonary exercise testing, pulmonology, obstetrics, cardiothoracic surgery, and advanced heart failure and transplantation services. For further expansion of the service, buy-in from institutional and governmental structures will be required. This is an ongoing challenge, given the competing health priorities in developing countries, where the burden of communicable and other non-communicable diseases, maternal and perinatal health, and injury-related disease is overwhelming, thus making ACHD a low priority to funders. Further challenges relate to training and increasing the number of clinically trained ACHD specialists.
As the Regional Ambassador for Africa within the International Society of Adult Congenital Heart Disease (ISACHD), what initiatives do you have in place to promote awareness and improve care for patients with adult congenital heart disease?
My role as the Secretary, and Regional Ambassador for Africa, within the ISACHD is relatively new. As an organisation, we promote awareness of ACHD, and are involved with global educational programmes. The website provides links to useful information, as well as a forum where complex cases are discussed.1 The aim is to encourage collaboration and discussion around topics from diverse clinical settings. I am actively promoting the incorporation of more teaching time in the adult cardiology curriculum, including topics pertaining to ACHD in congress and Fellows Courses, and to upskill general cardiologists in the management of ACHD emergencies, whilst establishing robust referral pathways. The interest remains largely from paediatric cardiology at this point, but there is a growing recognition of the complexities of patients with ACHD in general adult cardiology.
You are the Chair of the Education Committee for the SA Heart®. How do they enhance public awareness, guidelines, fellowship, research, training, and teaching in cardiovascular healthcare delivery?
The Education Committee of SA Heart® co-ordinates various educational activities aimed at cardiovascular practitioners in South Africa, with a local reach to Sub-Saharan Africa as well. The Committee comprises a member from each of the special interest groups: interventional cardiology, electrophysiology, basic sciences, lipidology, allied professionals (e.g., nurses, cardiac physiologists, radiographers), paediatric cardiology, and cardiac imaging. The special interest groups are responsible for the development and promotion of educational webinars, in-person academic meetings, and conferences, that are then endorsed by the Education Committee. An annual Cardiology Fellows Examination Preparation course is aimed at preparing cardiology trainees for their final cardiology certification examination. This involves a 3-day programme, summarising basic concepts and the latest up-to-date information, presented by local thought-leaders. The last day of the course entails a mock-examination, similar to the final objective structured clinical and oral examination that they will do as part of their certification. The committee is also responsible for the endorsements of academic meetings, allocation of travel and research grants, and contributes to the academic programme development of the Scientific Committee of the SA Heart® Congress.
The current engagement with the public is through bi-weekly educational posts distributed through our various social media platforms (Facebook, Instagram, LinkedIn, and X (formerly Twitter); search ‘South African Heart Association’).
A novel role, which I instituted during my tenure as SA Heart® President, was a platform for academic training unit heads of department to meet 2–3 times annually, to discuss issues pertaining to training posts, the examination processes, and other issues related to the development of the discipline, and the training of Fellows in South Africa. All training in cardiovascular care occurs at the eight academic training centres, so this this allows for uniformity to be created, at least in principle for now, thus hopefully strengthening the voice of clinicians and training institutions when dealing with training bodies and government.
You have recently published a paper focusing on the management of rheumatic heart disease in pregnancy. What are the major take-home messages from that article?
Rheumatic heart disease remains a leading cause of mortality and morbidity in female patients who are pregnant in low- and middle-income countries. There is also a rise in these pathologies in high-income countries due to immigration. The article2 provides a step-wise, evidence-based approach to the clinical and diagnostic assessment of rheumatic heart disease, looking at four major steps: maintaining a high index of suspicion; preconception counselling and risk stratification; the lesion-specific management and intervention, especially pertaining to mitral stenosis, the most dominant and severe of these lesions; and the peripartum and post-partum period.
During the European Society of Cardiology (ESC) Congress in 2022, you presented on the topic of how to overcome diagnostic challenges in endocarditis. Could you share the take-home message from this presentation?
Although infective endocarditis in pregnancy is rare, it carries a very high mortality (+/- 11%) and morbidity. There are many clinical diagnostic challenges; the symptoms may be relatively non-specific; and there is also an overlap with some symptoms of pregnancy. Cardiac imaging, too, may be more difficult than usual. In terms of antibiotic therapies, these are largely similar, but be cognisant of the teratogenic profile of the drugs. Always maintain a multidisciplinary approach with these rare but difficult cases.
Were there any outstanding sessions from the ESC Congress 2022 that come to mind?
The standout for me was the INVICTUS trial, which compared the direct oral anticoagulant rivaroxaban with the vitamin K antagonist (VKA) warfarin, in patients with echocardiographic proven rheumatic heart disease and atrial fibrillation. Twenty-four countries in Africa and South America participated. A total of 4,565 patients were randomised to either rivaroxaban 20 mg daily, or adjusted dose VKA. Median follow-up was 3.1 years. The primary outcome was a composite of stroke, systemic embolism, myocardial infarction, or death from vascular or unknown causes. This outcome occurred at 8.26% per year in the rivaroxaban group, compared with 6.46% in the VKA group (statistically more events in the rivaroxaban arm), concluding that in patients with rheumatic heart disease and atrial fibrillation, warfarin reduced cardiovascular events and mortality without increasing major bleeding when compared with rivaroxaban, and should thus be the standard of care for this patient group.
Are there any exciting innovations on the horizon for the management of adult congenital heart disease, valvular heart disease?
In our clinical context, we are increasingly using percutaneous pulmonary valve implants for patients with Tetralogy of Fallot with late residual pulmonary incompetence. We are also only now introducing MitraClip to South Africa for degenerative or secondary mitral regurgitation in patients who are too high-risk for surgical options. This is an exciting development for us. The two national centres that will be involved in the first implants are Groote Schuur Hospital, which is where I work, and Tygerberg Hospital, both in Cape Town, South Africa.
As a specialist in a relatively rare and complex field, how do you approach patient care, considering the unique challenges faced by patients with ACHD, and the importance of individualised treatment plans?
The biggest challenge I face is the sheer volume of patients. For those patients who do get to our clinic, each patient is clinically evaluated, the residual lesions assessed by cardiac imaging (at least echocardiography; more advanced imaging is reserved for specific clinical indications), and a functional status assessed. We have intermittent support in terms of genetics and psychiatry input, though this is an area we need to expand on. Furthermore, we are struggling to establish a cohort of specialist nurses for this area, which, as you well know, is an essential component to a service.
What are some points of emphasis you incorporate into practice to be the best cardiologist you can be, particularly in the field of ACHD?
Give your best for each patient. Try to, within your ability and influence, provide the best and most holistic clinical care. Be intentional about educating your patients about their disease, allowing for realistic expectations, and forming a partnership in their management strategy.
