Carcinoid Heart Disease
(CHD, Hedinger syndrome).
Carcinoid Heart Disease (CHD, Hedinger syndrome), is a rare condition that affects about one in five (20%) patients who have a Neuroendocrine Cancer AND Carcinoid Syndrome (CS), particularly those whose primary is in the small bowel followed by lung, large bowel, pancreas or ovary.
The precise cause is unknown but is thought to be related to raised levels of peptides and hormones produced by Neuroendocrine Cancer cells – in particular serotonin.
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 may lead to the development of plaques on the surfaces of the valves of the heart. Valve leaflets become thickened, retracted and immobile, resulting in either stenosis (failure of valve to completely open) or, most often, in regurgitation (failure of valve to completely close) that causes right ventricular dilatation and ultimately, right heart failure.
The right sided heart valves : Tricuspid and / or Aortic : are the most likely to be affected, whilst left sided valves – Pulmonary and/or Mitral valves are less frequently involved.
Left-sided valve disease is unusual (less than 5%), and may be limited to those with a Patent Foramen Ovale (PFO), those with lung secondaries (metastases), and those with high tumour burden who may secrete particularly high levels of serotonin.
A PFO is a hole in the heart that didn’t close the way it should after birth, allowing blood to avoid passage through the lungs, by leaking through from the right side of the heart to the left.
Many patients with Carcinoid Heart Disease may have no obviously notable heart-related symptoms, however, early signs may include:
- A gradual reduction in exercise capacity for example walking and / or exercise
- Dyspnoea (shortness of breath) – mainly on exertion
- Increasing fatigue.
Some of which may be thought to be due to other potential causes, such as the cancer itself, general health issues and / or increasing age.
In advanced Carcinoid Heart Disease however, symptoms may include worsening shortness of breath, peripheral oedema (for example swollen ankles), ascites (fluid collection of the abdomen), unintentional weight loss, reduced muscle mass and more obvious signs of right heart failure.
UKINETs have provided Bitesize Guidance for Healthcare Professionals on CHD here.