New Guidelines Will Help Diagnose and Treat Carcinoid Heart Disease

February 15, 2018

Neuroendocrine cancer patients and their doctors now have new and important tools for diagnosing and treating carcinoid heart disease (CHD) early. Last year, an international multidisciplinary team comprised of specialists on the leading edge of research and treatment for neuroendocrine cancer published guidelines in the Journal of the American College of Cardiology entitled, Diagnosing and Managing Carcinoid Heart Disease in Patients with Neuroendocrine Tumors: An Expert Statement.” Given that up to 50 percent of neuroendocrine cancer (carcinoid) patients go on to develop CHD, which is often more life-threatening for the patient than the usually slow-growing neuroendocrine tumors, this is a huge step towards the goal of educating patients and doctors about the current gold standard in diagnosis and treatment.

Heidi Connolly, MD
Heidi Connolly, MD

The Problem: CHD occurs in half of patients with neuroendocrine tumors. Symptoms of right heart failure may develop — such as shortness of breath, edema (swelling) of the feet and ankles, and fluid in the stomach and lungs — and may considerably impair quality of life. “Unfortunately,” said Heidi M. Connolly, MD, Director, Carcinoid Heart Disease Clinic, Department of Cardiovascular Diseases, Mayo Clinic, in Rochester, Minnesota, “there is no way at this time to prevent carcinoid heart disease.”

“At the same time, diagnosing, treating and managing CHD can be quite complex, in part because NETs and CHD canpresent in a wide variety of manifestations,” Dr. Connolly said. This has resulted in considerable variations in terms of screening and management of the heart disease. “Until the publication last year of the results of this collaborative effort,” said Patricia A. Pellikka, MD, Chair, Division of Cardiovascular Ultrasound, Mayo Clinic, Rochester, Minnesota, “no generally agreed upon guidelines for diagnosing and managing CHD were available.”

Jerome Zacks, MD, founder and director of the Carcinoid Heart Center in New York City; Associate Clinical Professor, Medicine/Cardiology; and member of the Center for Carcinoid and Neuroendocrine Tumors of the Icahn Medical School at the Mount Sinai Hospital, added, “I still get patients who have edema up to their lungs and are out of breath before they are referred to me.” Because of the very negative impact on a patient’s quality of life and upon the ability of a surgeon to safely operate on neuroendocrine tumors, the sooner CHD is detected, the sooner it can be treated and the sooner the patient’s tumors can be treated.

Patricia Pellikka, MD
Patricia Pellikka, MD

The Takeaways: Once a patient has a diagnosis of neuroendocrine cancer, the guidelines make several things clear. “First, it’s important to be treated at a medical center that has the interdisciplinary teams in place to monitor every aspect of a carcinoid patient’s health,” said Dr. Zacks. Dr. Pellikka agreed: “Multicenter collaboration is a good way to work together because many institutions on their own might not see a lot of these patients.” Team members can facilitate a quick response to a patient’s needs. “Patients can reach me and my colleagues 24/7,” Dr. Zacks said. “If they see me but have a tumor-related issue, I’ll call a team member and get an answer right then.”

Experienced teams mean gold standard treatments. Dr. Pellikka said that the Mayo Clinic has performed more than 200 valve replacement surgeries for patients with carcinoid valve disease and has developed a scoring system to diagnose valvular heart disease and assess its progression.

Second, the patient with carcinoid syndrome should undergo an echocardiogram, not just blood tests. “No one lab value can tell you yes or no that the patient has carcinoid heart disease,” said Dr. Pellikka. “Doing an echocardiogram is more expensive than a blood test but it’s the gold standard.” Even now, though, noted Dr. Zacks, “a cardiologist might not think to have the patient undergo an echocardiogram.” These guidelines should help alleviate this unnecessary delay.

Jerome Zacks, MD
Jerome Zacks, MD

Third, in general physicians treat the heart first, then the cancer. “In the past, the thinking was that you should not do heart surgery first, that you should operate on the tumor first,” said Dr. Connolly. “The heart must be fixed first,” agreed Dr. Zacks. “Otherwise, there’s an increased risk of bleeding during surgery to remove the tumor.” In addition, heart surgery will have the added benefit of improving the patient’s quality of life by eliminating the symptoms of heart disease.

In the works: Drs. Connolly, Pellikka and Zacks are excited about several recent developments and developments on the horizon for CHD patients:

  • The FDA approved the drug telotristat ethyl (Xermelo) in March 2017. “It’s used to control diarrhea but it’s possible that it will also change the progression of carcinoid heart disease,” said Dr. Connolly.
  • The FDA has also approved the use of a Gallium 68 PET/CT scan which is 10 times as sensitive in detecting NETs as the previous scan and might thus facilitate earlier tumor detection and early CHD monitoring.
  • Lutathera for peptide receptor radionuclide therapy (PPRT) was approved by the FDA on January 26, 2018. In the past, many U.S. NET patients traveled to Europe and Canada to have this treatment and now it will be available in their own country.
  • And in a study that can benefit several types of cancer, Dr. Kjell Oberg in Sweden is conducting a study that uses a virus to attack tumors.


The important thing for patients to get from these guidelines, said Dr. Connolly, is that “they should remain optimistic and hopeful. There are good options (for diagnosis and treatment) now.”





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