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Ronny Allan
Neuroendocrine Cancer has certain unique features whereby tumours can produce one or more symptoms which are known collectively as a syndrome. Neuroendocrine Tumours secreting excess amounts of serotonin, can be accompanied by Carcinoid Syndrome which if not diagnosed and treated early enough, can lead to an additional complication known as Hedinger Syndrome (often known as Carcinoid Heart Disease (CHD)). However, very late diagnoses can present with CHD already in place.
Excess serotonin, a hormone released by NETs into the bloodstream seems to be the prime and lead suspect for causing thick ‘plaques’ or fibrosis tissue within the heart muscle and damage to (mainly) the tricuspid and pulmonary valves on the right side of the heart which can become ‘tightly narrowed’ or ‘leaky’. It’s very similar to the reasons for mesenteric and peritoneal fibrosis which commonly occurs in midgut and other types of NET, in varying clinical degrees of severity. With Hedinger Syndrome, other substances associated with Carcinoid Syndrome may also be involved (e.g. tackykinins). The presence of liver metastases may allow large quantities of these substances to reach the right side of the heart without being filtered out by the liver but the primary and other secondary tumours can still contribute to the problem. It’s important to note that the damage is nearly always caused by excess secretions of substances from malignant neuroendocrine cells rather than any direct metastatic involvement of the heart.
Patients with carcinoid heart disease normally present with symptoms such as breathlessness (dyspnea), fatigue, ascites, and swollen ankles (edema). However, some patients can be asymptomatic. The left side of the heart is relatively protected, with the pulmonary circulation filtering out the majority of the serotonin and other substances produced by the tumours. However, involvement of the left-sided valves can sometimes be seen in patients with very active metastatic disease, bronchial NET or those with an existing heart condition known as Patent Foramen Ovale (hole in the heart).
When I was diagnosed in 2010, I was displaying symptoms of carcinoid syndrome and had to undergo a plethora of tests including something called an Echocardiogram – a sonogram (ultrasound) of the heart. Note – it is NOT abbreviated as ECG, which laypersons often use as an abbreviation for an Electrocardiogram – a totally different test. Carcinoid heart disease is a relatively late manifestation of neuroendocrine tumours; however, it can have an impact on the prognosis of these patients. Thus, early testing is vital for each patient presenting with carcinoid syndrome so that treatment can be considered. Whilst there are certain biomarkers that might indicate the potential for Carcinoid Heart Disease to be present, Echocardiography is the gold standard for detection. Depending on the results of the Echocardiogram, two further investigatory tests may be ordered up – transoesophageal echocardiogram and cardiac catheterisation. Patients without symptoms can undertake a blood test called NT-proBNP which can function as a screening test.
If you ‘google’ Carcinoid Heart Disease, be careful where you look as there are some statistics to be found in terms of incidence and prognosis. I suspect they may be out of date and have yet to catch up with improvements in the latest diagnostic and treatment techniques. Either that or they fail to mention the disease might only be clinically significant in much smaller percentages.
On a positive note, I sense major strides in worldwide awareness campaigns which should lead to earlier diagnosis and therefore earlier treatment for Neuroendocrine Cancer. Combining that with new and innovative treatments in debulking/removing/shrinking tumours and controlling syndromes, particularly the use of somatostatin analogues with the latter, should mean that fewer people will succumb to this additional complication. I don’t see a lot of Carcinoid Heart Disease posts on the various forums which hopefully is a good sign.
I’m fairly stable now so very low risk. I had an Echocardiogram every year from 2010 -2015 and I had one in 2018 as a precaution. The procedure is painless and takes around 20-30 minutes. My results have always been OK. Information on the guidelines for CHD has been a bit sparse but a new paper published has proposed an ‘Algorithm for the Screening and Investigation of CHD’ – you can see this below.
Who is susceptible to Carcinoid heart disease?
Given the name we are clearly talking about serotonin secreting NETs and the statistics would appear to confirm that. I’ve read various figures, but many seem to agree the risk is greater in those with small intestine NET and carcinoid syndrome with high levels of 5HIAA (>300 μmol/24 hour – see guidance below).
I read some texts which put carcinoid heart disease as high as 50-60% of patients with both NETs and carcinoid syndrome. To put that into even more context, only around one-third of the types of NETs with the potential to oversecrete serotonin will be functional i.e. have an active carcinoid syndrome. Some recent texts put that figure at 20% citing the long-term use of somatostatin analogues as a driver for that reduction – that figure looks credible when you do the math I gave above.
The vast majority of patients succumbing to carcinoid heart disease will be those with liver metastases. When hepatic spread from a primary gastrointestinal NET results in hormonally active tumour products exceeding the hepatic capacity for degradation, it is at this point that the classical carcinoid syndrome ensues. There are rare exceptions to that when carcinoid syndrome may appear without pre-existing liver metastases in patients with extensive retroperitoneal lymph node metastases and drainage bypassing the liver via the thoracic duct and retroperitoneal venous collaterals; or when the tumour products drain directly into the systemic circulation such as in the rare case of ovarian NETs.
I found two studies that gave some statistical breakdown of where the primary site was. It appears carcinoid heart disease occurs most frequently in patients with NETs originating in the:
– small bowel (72%)
– unknown primary (18%)
– NETs of the lung, large bowel, pancreas, appendix, or ovarian origin (10%).
Based on these ratios, spreading the unknown primaries around would potentially put the small bowel figures as closer to 85% with a slight male preponderance (∼60%) and a mean age at diagnosis of 56-63 years.
In another study from Germany, the figure for Small Intestine NETs and unknown primary was as high as 94% combined leaving only 6% for the other locations. This study assessed the clinical characteristics and prognosis of patients with carcinoid syndrome and carcinoid heart disease in 276 patients. Carcinoid syndrome patients had a mean age of 57 years (range 21–84) and a normal BMI of 24.9 (SD 4.5; range 13.8–39.6). Most primaries were of small bowel or unknown primaries with distant metastasis in 94.6%. Flushing was the most frequent symptom in 74.3% of patients, followed by diarrhea in 68.8%, and wheezing in 40.9%. Pain was described by 45.3%, weakness by 23.5%, and weight loss of >10% in 6 months by 30.1% of patients. Carcinoid heart disease was diagnosed in 37.3% of patients (n = 104) by echocardiography and involved predominantly in the tricuspid valve. Combinations with other valve defects were common. Somatostatin analogs were taken by 80.4% of patients and 17% needed additional loperamide/opium tincture. Surgery and peptide receptor radiotherapy were the most frequent treatments.
NEW – 2022 ENETS guidance issued
The algorithm used in the above Reference is excellent.
Please also note that other types of fibrosis due to excess serotonin (and other substances) can also induce fibrosis in the mesentery, retroperitoneum, pleural and pulmonary cavity and the skin. This is fully covered in my article Neuroendocrine Cancer: Fibrosis – an unsolved mystery?
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That’s really helpful Ronnie, Ray went into carcinoid crisis last week and we only knew about CC due to your FB page and the NETS Foundation, with this information we went to A &E confident enough to advise the Doctors in what they needed to do.
sorry to hear about Ray, do hope he’s OK now?
Great info. Thanks for the test tips!
Thank you very much for your information will know what to look for
Hello from New York😁. I just discovered this blog, thank you for great information. My 60 year old husband, Mark, was diagnosed in January 2015 with a pancreatic neuroendocrine tumor, inoperable so far. It is 7.5 x 7.8 cm., in the head of the pancreas, with extensive vascular involvement. He had no symptoms, and it was found by accident on a CT scan after he had several pulmonary embolisms out of the blue in December.
They have done 6 months of CAPTEM (Capcitabine and Temodar) chemo, which is a phase 2 trial, and he gets scanned in 10 days to see if the tumor has been reduced in size at all. If it shrinks enough and in the right places they may be able to do the Whipple surgery. I am very nervous about everything-up until recently he has felt almost normal and even able to golf 2-3 times a week all while on the chemo. Recently he has started to have symptoms like flushing and some digestive issues. His attitude is wonderful-no fear at all, but there doesn’t seem to be many options if the CAPTEM doesn’t work…
But thank you for being a light to all of those going through this!
Donna
Good luck with the treatment Donna, hoping the tumour shrinks to make it operable – best wishes to Mark. Thanks for finding my blog, I hope my words help.
Ronny
I have carcinoid heart disease. My mitral Valve doesnt Funktion properly. In fact this is How my cancer was discovered 8 years ago. I had a heart and lang Infektion. 8 years later i am stable. I take beta blockers daily and my heart Valve has improved due to the sandostatin shots. My liver mets are also stable.
Thanks for the comment Claudia, useful for those with CHD issues and interests to read. I hope you are doing OK?
Hi Ronny, my husband got a letter wed to say that he was being admitted to Christie last fri am for the second part of his liver embolisation. This came out of the blue as he was told he neede an ultrasound first. We went fri as arranged but unfortunately he collapsed in the admissions unit and the crash team had to intervene. There was concern that he had a heart problem as he has a damaged tricuspid and thckened heart wall both caused by secretions from his liver metastases. It turned out not to be so. Still dont know what happened. Couldn’t go ahead with the planned embolisation but kept in for a few hours then allowed home. It is being rescheduled we hope. He is seen 6 monthly by the cardiac dept at Wythenshawe. It is important as you say to make sure that you get referred to a cardiac consultant to keep an eye on things.
Hi Ronny, one of the Christie doc rang today. The radiology dept have not yet accepted my husband for the second part of his embolization. When he had the first one he reacted very badly and was an in patient for 20 days. At one point they were considering moving him to critical care. He was fine in the end and has kept wellthe past few months. His consultant still thinks he needs embolization so we are waiting to see what happens. He is to have an ultrasound scan to see whats happening in the liver cos he has new symptoms. If they dont do the embolization, interferon has been mentioned. Do you or anyone else knows weather this will help and how it might work? At the moment it seems to be one step forward and two steps back.!!!
Irene – sorry to hear that. Some people react differently to treatments, that is clear from looking on forums. What was the nature of the reaction by the way?
I don’t know too much about Interferon except that it’s been around a while. I did find a couple of things mentioned on the NET Patient Forum:. http://www.netpatientfoundation.org/forum/6_1066_1.html
and
http://www.netpatientfoundation.org/forum/5_728_1.html
It’s mentioned in the NPF handbook here http://www.netpatientfoundation.org/wp-content/uploads/NPF-GUIDE-TO-NETS.pdf
And Tara mentions it in her nutrition book too here on page 18 http://www.netpatientfoundation.org/wp-content/uploads/037-NPF-NUTRITION-v3.pdf
Christies is a NET Centre so I’m sure he is getting good advice and they will know to put him on an Octreotide drip before any interventional procedure (did they do this for this first embolisation?)
Yes he has numerous tumors. I ues FB so will check it out. We are waiting for a call from Christie sometime this we with info about an embolization date.
Good luck with embo – allow a working week off work!
Ronny
Hi Ronny,
I have just found your blog. My husband has been attending the Christie Hospital since Jan. He has carcenoid syndrome initially in the small intestine but now has liver metastases. These have now caused damage to his tricuspid valve . He has had one half of his liver embolised and was quite poorly afterwards. The tumors in the other half of his liver have got bigger so he is now waiting for another embolization. I am trying to keep positive but it is not always easy. As you said he doesnt look ill and can do most of the thinngs he has always done. He is now also under a cardiac surgeon as well. He currently has monthly injections of sandostatin LAR which help to keep his symptoms in check. It helps me to know there are other people who understand the problems of this condition. Thanks for the blog. Xx
Hi Irene, when you say carcinoid syndrome, you mean Carcinoid tumours? There are some really good treatments nowadays and it sounds like he is in good hands.
I’m glad you’re enjoying my blog and I post even more on my associated Facebook page here: https://www.facebook.com/NETCancerBlog Keep in touch and let me know how he gets on?
Thanks for this Ronny. I am wondering if my fatigue is related to this or my chemo. I will follow up on this. Thanks so very much. 🙂
Have you been diagnosed with CHD then?
Sorry, I don’t know what CHD is. Chronic Heart Disease?
I just got an EKG and they said I need to follow up. Nobody would tell me anything further…..of course. Thanks for the fear, docs.
I have no breath, fatigue…..exhaustion…….not sure if it’s chemo or what you are writing about.
Carcinoid Heart Disease, the subject of the blog. You need an Echocardiogram to check
Thanks again Ronny. 🙂
My beloved twin sister had breathlessness (dyspnea), fatigue, ascites, swollen ankles (edema)… more and more I wish we could’ve had proper treatment because no one told us about the echocardiogram.
Thank you so much for your always informative posts. You are truly amazing, and you are always in my thoughts!!
Big hug from Portugal
Reblogged this on Patrícia e Susana.
Hi That is interesting. I was not offered one and only after I had one done privately abroad and it shoved a floppy pulmonary valve my doctor said: Of I better refer you to a cardiologist!
Reblogged this on Cy Ball — Music and commented:
This has been a big problem which is getting better because of detection and treatment improvements.
Hi Ronnie,
I also get an annual echocardiogram to check for carcinoid heart disease. This test shows no sign of the problem for me. It is heartening that statistics are showing fewer deaths in the carcinoid community due to heart disease, probably because detection and treatment is improving as you said above.
For me, I have the problem of a pulse that can drop to a very low (35 bpm) rate unexpectedly. This may also be a rare condition caused by carcinoid. I just had a pacemaker implanted to combat that problem.
Carry on!
Cy