Supporting the

Neuroendocrine Cancer Community

Carcinoid Heart Disease : The Heart of The Matter

May 2, 2022

Carcinoid Heart Disease (CHD or Hedinger syndrome), is a rare condition that affects about one in five (20%) patients who have a Neuroendocrine Cancer AND Carcinoid Syndrome (CS).

The precise cause is unknown but is thought to be related to abnormally raised levels of peptides and hormones produced by Neuroendocrine Cancer cells – in particular serotonin.

 Meet Craig :

“ I’m a husband and father to 2 beautiful young children. I’ve been living with Neuroendocrine Cancer for more than 8 and a half  years now. My primary is in my rectum with mets to my liver and abdomen.

Unfortunately I have both severe Carcinoid Syndrome and Carcinoid Heart Disease, so am not able to work – I spend my time as a stay at home daddy and enjoying watching my 2 children grow.

Since being diagnosed, I have gone through many treatment options in an attempt to control my cancer with mixed results.

I recently had repeat heart surgery  (a TAVI*) to replace my cancer-related damaged heart valves – my previous surgery 3 years ago – without this procedure* I was told I might not see Christmas (2021)”.

Watch and listen to Craig’s story here.

Neuroendocrine Cancer is not one but a group of cancers that develop in neuroendocrine cells. These cells exist throughout the body – and play a vital role in keeping the body functioning normally.  

Because we have these cells throughout the body, there is not one specific site in which they can develop : the most common sites are the lung, gut and pancreas.

Neuroendocrine cells act like sensors, responding to changes within the body by communicating quickly with each other and other cells (the “Neuro” bit) to release helpful substances to deal with whatever is happening (the “endocrine” bit)

 – for example:

in the gut (digestive tract) they influence how the body responds to, breaks down and uses food – to ensure we get the right nutrients and to maintain stable blood sugar and energy levels.

When these cells become cancerous – this ability to respond and produce hormones and peptides can become corrupted. In approximately 40% of those with Neuroendocrine Cancer – this corruption can lead to raised substance production, causing disruption of normal bodily functions.

One of the most common set of symptoms related to this disruption is Carcinoid Syndrome: primarily associated with primary neuroendocrine cancer of the small bowel, but may also occur in about 10% of those with a primary in the lung or gynaecological structures (eg womb, ovary, etc) – or may develop when the cancer has spread to the liver.

The hormones and peptides most commonly associated with Carcinoid Syndrome include Serotonin, Histamine and Kinnins. These have an important role in keeping the bowel working properly, the immune response and, by acting as messengers, they can carry signals around the body to maintain normal body functions – such as heart rate, blood pressure and breathing. 

Symptoms of Carcinoid Syndrome include 2 or more of the following :  diarrhoea, abdominal (tummy) cramping, ‘dry’ flushing, racing heart (palpitations) and / or wheezing. 

Approximately 40% of those with Carcinoid Syndrome may also be at risk of Carcinoid Heart Disease.

(Image : American Heart Association)

In normal health, each time your heart beats, it first fills with blood and then pumps that blood out. The heart has valves that open and close with every heartbeat, helping the blood to flow smoothly and freely in one direction through the chambers of the heart and to the rest of your body. But if a valve doesn’t open or close properly, problems can occur. The body may not get a sufficient supply of blood (stenosis) or, in some cases, blood can seep back into the heart (regurgitation).

In Carcinoid Syndrome raised serotonin levels can lead to the development of fibres deposited on the surfaces of the valves of the heart. They become thickened, retracted and immobile, resulting in either stenosis (failure of valve to completely open) or, more often, in regurgitation (failure of valve to completely close). 

The damage is irreversible and potentially life-threatening and without proper management or intervention, progressive right heart failure is inevitable. Valvular replacement surgery (generally preferred over repair) has been the only approach that offers improved survival – even in the presence of metastatic disease (where neuroendocrine cancer has spread).

Neuroendocrine Cancer UK was established by patients for patients –  and exists to address the needs voiced by the Neuroendocrine Cancer community.

We’re here to support and inform patients and families from diagnosis, enabling access to the best care and treatment, whilst stimulating Neuroendocrine Cancer research, increasing national awareness and influencing improvements in outcomes. Visit us here : 

“Getting a cancer diagnosis can make you feel lost, afraid for the future and wondering where to turn for support.  Never more so than at the end of 2021 when my condition required me to have heart valve replacement surgery.

NCUK were invaluable to me and my wife. They provide so much support and I really encourage anyone, particularly newly diagnosed, to give them a call. They are available to talk to, to advise, to give support and they genuinely care about you.  

And joining the Natter Patient Support Groups provides reassurance that others understand, are going through similar experiences and takes the loneliness out of the situation. 

Whether it is treatment, medications or just to talk, the right support at the toughest of times is a vital lifeline and I am personally very grateful for everything NCUK has done for me since my diagnosis in 2012”.


References and Further reading:

  • NCUK Carcinoid Heart Disease webpage 
  • UKINETs Carcinoid Heart Disease Bitesize Guidance
  • Genetic Alliance UK article here
  • Davar et al (2017) Diagnosing and Managing Carcinoid Heart Disease in Patients With Neuroendocrine Tumors: An Expert Statement. J Am Coll Cardiol.; 69 (10) 1288–1304
  • Jin et al (2021) Carcinoid Heart Disease: Pathophysiology, Pathology, Clinical Manifestations, and Management.  Cardiology;146:65–73
  • Ram et al (2019) Carcinoid Heart Disease:Review of Current Knowledge. Texas Heart Institute Journal February 2019, Vol. 46, No. 1
  • Steeds et al (2019) Multidisciplinary team management of carcinoid heart disease. Endocrine Connections Review; 8: 12: R184-199