A new approach to congenital heart disease


How a pilot trial at RB&HH Specialist Care led to significant improvements in the quality of care for congenital heart disease patients at a leading NHS trust

Congenital heart disease, a term that covers a range of birth defects that disrupt the normal function of the heart, affects up to 1% of new-borns worldwide. In most cases, there is no obvious cause, but some factors are known to increase the risk. These include genetic disorders such as Down’s syndrome, or the mother smoking or drinking alcohol during pregnancy, or having poorly controlled type 1 or type 2 diabetes.

There are many different types of congenital heart disease and they can sometimes occur in combination. Some of the more common defects include a hole between chambers of the heart (a ‘hole in the heart’), the main blood vessels of the heart being narrower than normal, and ventricular septal defect, where a hole appears between the lower chambers of the heart, causing blood to be pumped into the lungs.

Symptoms in babies and children can include a rapid heartbeat and/or breathing, extreme fatigue and a blue tinge to the skin caused by low blood oxygen levels. However, depending on the heart defect, symptoms might not appear until later in life.

Congenital heart disease is usually diagnosed with routine scans during pregnancy, but, depending on the type, it may sometimes be diagnosed only after birth, or later in childhood. Treatment depends on the type of defect and can include surgery to restore the heart’s normal function. People with congenital heart disease often require treatment and monitoring throughout their life as they can experience further problems with their heart rhythm or valves over time.

Thankfully, with advances in medicine, survival into adulthood is expected for the vast majority of patients in developed countries. For this reason, specialist hospitals throughout the UK offer treatment and monitoring clinics for adults with congenital heart disease.

A personalised health service
Adult congenital heart disease clinics need to cover a broad spectrum of heart defects, so care pathways can be complicated for patients to navigate.

Patients require a range of regular tests, including MRI and CT scans of their hearts, as well blood tests, and ECGs to check their heart rhythm. However, due to the way NHS trusts are organised, these testing services are usually managed independently of each another, so can potentially be arranged months apart at inconvenient times of the day. As well as inconveniencing the patients, this can cause problems with the effectiveness of multi-disciplinary consultant meetings, where consultants from different specialities come together to review patient cases. The result can be delays in care, as well as greatly reduced quality of life for patients.

The team behind the RB&HH congenital heart disease pilot

Professor Michael Gatzoulis, consultant cardiologist and clinical and academic lead for adult congenital heart disease services at Royal Brompton and Harefield hospitals, felt there was a better way of providing care for these patients. In May 2020, in the midst of the COVID-19 pandemic, he led a trial to see if a same-day adult congenital heart disease service could be delivered within the NHS.

“Our patients are already burdened with a heart condition from birth and the way we currently deliver care within the NHS just adds to their burden. It shouldn’t be this way. Patients deserve to have a personalised health service designed around them, that delivers quick results and requires as few visits to a hospital as possible,” says Professor Gatzoulis.   

The pilot trial, which ran over three months and involved 51 patient volunteers, was delivered in partnership with RB&HH Specialist Care, the trust’s private patient services unit at 77 Wimpole Street, where the framework for a one-stop service already existed.

The new service combined all the routine diagnostic investigations needed (cardiac MRI, echocardiogram, ECG, chest x-ray and a comprehensive suite of blood tests), reporting and evaluation with a specialist cardiology consultant, and a session with a clinical nurse specialist on personalised education and lifestyle advice – all delivered in a single day at a single location.

A new framework of care
The pilot was a success with all patients receiving their monitoring tests and follow-up with a consultant and specialist nurse on the same day. One patient from the trial commented: “We live in Sussex and to have separate appointments for every investigation was much more inconvenient, as well as being spread over months which causes more stress while you wait for the final consultation to tell you the results and diagnosis. This day assessment addressed every single one of those issues in one visit for us.”

Professor Gatzoulis added: “We are very pleased to say that following the success of the pilot, the service is now being implemented for NHS adult congenital heart disease patients at our hospitals and we have invested in a new imaging centre to accommodate and facilitate this vision. We hope that more NHS trusts will follow our new framework of care with time. The partnership with our private patient services was the first of its kind for our team and enabled us to continue researching ways to improve patient care even during the challenging conditions of the pandemic.”