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Neuroendocrine Cancer – don’t break my heart!

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NEW – 2017 guidance issued.  Diagnosing and Managing Carcinoid Heart Disease in Patients With Neuroendocrine Tumors – An Expert Statement published in the Journal of the American College of Cardiology.

The following are key points to remember from this Expert Statement about the diagnosis and management of carcinoid heart disease in patients with neuroendocrine tumors:

  1. Carcinoid heart disease is a frequent occurrence in patients with carcinoid syndrome and is accountable for substantial morbidity and mortality.
  2. The pathophysiology of carcinoid heart disease is not well understood; however, chronic exposure to excessive circulating serotonin is considered one of the most important contributing factors.
  3. N-terminal pro–B-type natriuretic peptide (NT-proBNP) appears to be the best biomarker to date for screening carcinoid syndrome patients for evidence of clinically significant carcinoid heart disease (Evidence Level 2-3, Grade B).
  4. Measurement of either 24-hour urine 5-hydroxyindoleacetic acid (5-HIAA) or plasma 5-HIAA is mandatory for diagnosis and follow-up of carcinoid syndrome. Furthermore, a 24-hour urinary 5-HIAA level >300 μmol/24 hour is a useful marker for identifying those at risk of developing carcinoid heart disease (Evidence Level 2, Grade B).
  5. Transthoracic echocardiography remains the gold standard for diagnosis and follow-up of carcinoid heart disease. It should be performed in all patients with carcinoid syndrome and high suspicion of carcinoid heart disease, such as clinical features or raised NT-proBNP and/or 5-HIAA levels. For established carcinoid heart disease, echocardiography should be performed if dictated by a change in clinical status; otherwise/thereafter every 3-6 months, depending on the severity of established carcinoid heart disease and clinical status (Evidence Level 2, Grade B).
  6. Cardiac magnetic resonance can be used to evaluate the pulmonary valve, identify cardiac metastases, and assess right ventricular size and function (Evidence Level 2, Grade B).
  7. Long-acting formulations of somatostatin analogs are the standard treatment used to alleviate symptoms related to the carcinoid syndrome, and prevent the development and/or progression of carcinoid heart disease (Evidence Level 2, Grade B).
  8. In cases of carcinoid syndrome that are refractory to somatostatin analogs, options include escalation of the somatostatin analog dose to above labeled doses, addition of IFN-alfa, or peptide receptor radionuclide therapy (PRRT). The oral serotonin synthesis inhibitor, telotristat, represents a promising agent to improve symptoms of the carcinoid syndrome; however, it is not yet approved, and is currently only available for compassionate use in the United States. Given the limited data, everolimus cannot currently be recommended for the treatment of carcinoid syndrome (Evidence Level 2-4, Grade B/C).
  9. The patient with carcinoid heart disease should be managed by a specialized multidisciplinary team, within a setting of a specialized neuroendocrine tumor (NET) center (Evidence Level 5, Grade D).
  10. An experienced medical (cardiologists and NET specialists with involvement of other specialists as necessary), surgical, and anesthetic team approach to the patient with carcinoid heart disease is critical to provide state-of-the-art management for these patients (Evidence Level 5, Grade D).
  11. The choice of valve prosthesis should be individually tailored on the basis of the patient’s bleeding risk, and possible future therapeutic interventions. Biological valve prostheses are the preferred option (Evidence Level 4, Grade D).
  12. To prevent a carcinoid crisis during surgery, the patient should be started on an IV octreotide infusion at a rate of 50-100 mcg/h at least 12 hours preoperatively; this should be continued throughout the procedure and until stable. Patients should be monitored for occurrence of bradycardia if high doses of octreotide are used (Evidence Level 4, Grade C).
  13. Patients with confirmed carcinoid heart disease should be referred to a NET center with cardiology and cardiac surgery departments having expertise in dealing with this complex pathology (Evidence Level 5, Grade D).

A useful abstract of Carcinoid Heart Disease information written by a patient for patients is below.

Neuroendocrine Cancer has certain unique features whereby tumours can produce one or more symptoms which are known collectively as a syndrome.  The most common type of Neuroendocrine Tumour (so called Carcinoid), can be accompanied by Carcinoid Syndrome which if not diagnosed and treated early enough, can lead to an additional complication known as Carcinoid Heart Disease (CHD) or Hedlinger Syndrome. However, very late Carcinoid 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’.  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 secondaries 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, 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 carcinoid 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 lay persons 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 which 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. Combine 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 did blog about a new treatment for Carcinoid Syndrome called  XERMELO (Telotristat Ethyl) read here.  At ENETS 2016, a report claimed that it appeared to ‘halt Carcinoid Heart Disease’ or certainly reduce the risk.  Reducing the risk sounds feasible as Telotristat Ethyl reduces the ability to manufacture serotonin to levels which appear subthreshold to that which stimulates fibrosis associated with CHD. This drug might prevent the need for valve surgery in many cases, and enable the use of bioprosthetic valves in others, without recurrent fibrosis.  You can read the ENETS poster here.

Although I’m fairly stable, I still try to get an Echocardiogram on an annual basis and am very happy to have this one in my ‘test golfbag’. The procedure is painless and takes around 20-30 minutes.  My results have always been OK.  Information on the guidelines for CHD have been a bit sparse but a new paper published has proposed an ‘Algorithm for the Screening and Investigation of CHD.

Proposed Algorithm for the Screening and Investigation of CHD This graphic provides an algorithm for how patients with metastatic (serotonin-producing) neuroendocrine tumors (NETs) should be screened and assessed for carcinoid heart disease (CHD), including, importantly, when to refer to cardiology. f/u = follow-up; NT-proBNP = N-terminal pro–B-type natriuretic peptide.

If you have time please check out this excellent video presentation on Carcinoid and Your Heart with cardiologist Dr. Jerome Zacks from Mount Sinai Hospital and the Carcinoid Heart Center, both in New York City.

thanks for reading

Ronny

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23 Comments

  1. Kelly says:

    Great info. Thanks for the test tips!

    Liked by 1 person

  2. Lily says:

    Thank you very much for your information will know what to look for

    Liked by 1 person

  3. Donna Head says:

    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

    Liked by 1 person

  4. Claudia says:

    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.

    Liked by 1 person

  5. Irene says:

    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.

    Liked by 1 person

  6. Irene says:

    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.!!!

    Liked by 1 person

  7. Irene says:

    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.

    Liked by 1 person

  8. Irene says:

    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

    Liked by 1 person

  9. Ed says:

    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. 🙂

    Liked by 2 people

  10. Saudade says:

    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

    Liked by 2 people

  11. Han Na says:

    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!

    Liked by 2 people

  12. cy says:

    Reblogged this on Cy Ball — Music and commented:
    This has been a big problem which is getting better because of detection and treatment improvements.

    Liked by 1 person

  13. cy says:

    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

    Liked by 2 people

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