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Echocardiography in functional midgut neuroendocrine tumors: When and how often

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Abstract

The management of patients with midgut neuroendocrine tumors (MNET) is rapidly evolving. Current preoperative detection rates of primary tumor sites are higher than ever and progression-free survival in patients with already advanced disease is expanding due to the implementation of novel efficacious treatment strategies. This survival benefit may potentially translate into a need for a multidisciplinary approach to an even more heterogenous variety of clinical conditions, among these, carcinoid syndrome (CS) and carcinoid heart disease (CHD). The latter often triggers substantial morbidity and mortality, hence a systematic screening, an accurate diagnosis, as well as effective interventions are critically important. The rarity of the disease has result in a relative lack of statistically powerful evidence, which in turn may have rendered significant variability between practices. In this regard, despite recent guidelines, the optimal follow-up of patients with CHD remain debatable to some authors, perhaps due to the preponderance of certain schools throughout the manuscript. Herein, we present a concise and practical guidance document on clinical screening and echocardiographic surveillance of patients with CHD based on a comprehensive review of the literature, and complemented by our experience at the Center for Carcinoid and Neuroendocrine Tumors at The Mount Sinai Hospital.

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Authors and Affiliations

  1. Department of Cardiovascular Surgery, Mount Sinai Health System, The Mount Sinai Hospital, 1190 Fifth Avenue, GP2 West, New York, NY, 10029-6574, USA

    Javier G. Castillo & David H. Adams

  2. Center for Carcinoid and Neuroendocrine Tumors, Mount Sinai Health System, The Mount Sinai Hospital, New York, NY, USA

    Javier G. Castillo & Jerome S. Zacks

  3. Department of Clinical Cardiology, Mount Sinai Health System, The Mount Sinai Hospital, New York, NY, USA

    Tara Naib & Jerome S. Zacks

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  1. Javier G. Castillo
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Correspondence to Javier G. Castillo.

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Appendix

Appendix

APPROPRIATE USE CRITERIA FOR TRANSTHORACIC ECHOCARDIOGRAPHY (Maximum Score)a

EXTRAPOLATION TO PATIENTS WITH MNETs AND CARCINOID SYDNROME

Symptoms or conditions such as chest pain, dyspnea, palpitations, TIA, stroke, or peripheral embolic event

Unusual fatigue, dyspnea, palpitations and changes in blood pressure (carcinoid crisis and potential dehydration)

Prior testing that is concerning for SHVD such as chest X-ray, ECG, or cardiac biomarkers

Non-specific ST changes, STach, p pulmonale or RBBB on the EKG. Elevation of carcinoid ± cardiac biomarkers

Frequent or exercise-induced ventricular premature complexes, atrial fibrillation, supraventricular or ventricular tachycardia

Restricted HR variability, AFib, SVT and AFib due to heart failure, carcinoid crisis

Clinical symptoms or signs consistent with a cardiac diagnosis known to cause lightheadedness / presyncope / syncope

Carcinoid syndrome and uncontrolled diarrhea may lead to dehydration and lightheadedness / presyncope / syncope

Syncope when there are no other symptoms or signs of cardiovascular disease

Acute changes in blood pressure and arrhythmias (carcinoid crisis) may lead to syncopal episodes

Evaluation (and re-evaluation) of suspected pulmonary hypertension including evaluation of right ventricular function

Evaluation of suspected pulmonary hypertension including evaluation of right ventricular function

Increasing dyspnea, respiratory failure or hypoxemia of uncertain etiology

Increasing fatigue and dyspnea (hypoxemia) due to frequent bronchospasm and/or congestive heart failure

Initial evaluation (and re-evaluation) when there is a reasonable suspicion of valvular or structural heart disease

Initial evaluation after diagnosis of MNET or re-evaluation after positive screening for CHD (see algorithm)

Routine surveillance (≥3y) of mild valvular stenosis or regurgitation without a change in clinical status or cardiac exam

Routine surveillance (Q6M - Q1y) of mild valvular regurgitation w/o a change in symptoms or cardiac exam

Routine surveillance (≥1y) of moderate or severe valvular stenosis or regurgitation without a change in clinical status or cardiac exam

Routine surveillance (Q3M - Q6M) of ≥ moderate valvular regurgitation w/o a change in symptoms or cardiac exam

Suspected cardiac mass, suspected cardiovascular source of embolus, suspected pericardial conditions

Suspected cardiac mass, suspected cardiovascular source of embolus, suspected pericardial conditions

Initial evaluation (and re-evaluation) of known or suspected HF based on symptoms, signs, or abnormal test results

Evaluation (and re-evaluation) of known or suspected HF based on symptoms, signs, or abnormal test results

Routine surveillance (≥1y) of HF when there is no change in clinical status or cardiac exam

Routine surveillance (Q6M) of HF (always after using more advanced imaging techniques to have a baseline assessment)

Initial evaluation (and re-evaluation) of known or suspected cardiomyopathy

Evaluation (and re-evaluation) of known or suspected CMP (always after using more advanced imaging techniques)

Baseline and serial re-evaluations in a patient undergoing therapy with cardiotoxic agents

Baseline and serial re-evaluations in a patient undergoing therapy with cardiotoxic agents

Initial evaluation of known or suspected adult congenital heart disease

Initial evaluation of the atrial septum mandates to perform a “bubble test” during the first TTE assessment

  1. AFib atrial fibrillation, CHD carcinoid heart disease, CMP cardiomyopathy, ECG electrocardiogram, HF heart failure, HR heart rate, m months, MNETs midgut neuroendocrine tumors, Q every, RBBB right bundle branch block, SHVD structural heart valve disease, STach sinus tachycardia, SVT supraventricular tachycardia, TIA transient ischemic attack, TTE transthoracic echocardiography, W/O without, with no, y year
  2. aACCF/ASE/AHA/ASNC/HFSA/HRS/SCAI/SCCM/SCCT/SCMR 2011 Appropriate Use Criteria for Echocardiography

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Castillo, J.G., Naib, T., Zacks, J.S. et al. Echocardiography in functional midgut neuroendocrine tumors: When and how often. Rev Endocr Metab Disord 18, 411–421 (2017). https://doi.org/10.1007/s11154-017-9434-z

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