Carcinoid Heart Disease: Early and Late Outcomes of Surgical Treatment in 195 Consecutive Patients

Educational Objectives

Evaluate timing of valve operations in symptomatic patients with carcinoid heart disease.

Summary

Take-home Messages:

• Symptoms and survival of patients with carcinoid syndrome have improved, but development of carcinoid heart disease (CaHD) continues to decrease survival.
• Despite the complexity of this disease, symptomatic patients with CaHD who are referred for valve surgery have an acceptable perioperative risk when treated at an experienced center by a multidisciplinary team.
• Valve operation before the onset of advanced symptoms probably carries a survival benefit for this population compared with surgery performed for more advanced symptoms.

Carcinoid tumors are rare (2.5-5.0 cases per 100,000 of the population per year) neuroendocrine malignancies that tend to grow slowly and follow a prolonged course of 20 years or more. However, there is a significant potential to produce hepatic metastases that release excessive amounts of vasoactive amines into the systemic circulation. Thus, up to 15% of these patients may develop carcinoid syndrome with possible cardiac involvement.

Carcinoid heart disease is the result of endocardial deposition of pearly fibrotic plaques (notable for absence of elastic fibers) that generally extend to the right-sided valves, leading to multiple patterns of severe valve dysfunction. Right-sided heart failure worsens patient quality of life and leads to excess mortality. Without valve surgery, only 10% of patients survive 2 years after the onset of New York Heart Association (NYHA) functional class III or IV symptoms.

Investigators sought to analyze patient outcomes after valve surgery for CaHD during a 27-year period at one center (Mayo Clinic, Rochester, Minnesota) to evaluate outcomes and determine whether there are opportunities to improve patient care.¹They retrospectively studied the short-term and long-term outcomes of 195 consecutive patients with CaHD who underwent valve replacement between 1985 and 2012.

In an accompanying commentary,² Richard Warner, MD, and Javier Castillo, MD (both from The Mount Sinai Hospital, New York City), referred to this “pioneering series with long-term survival analysis” and emphasized 3 points that they think will potentially become tenets of decision making:

1. The surgical management of right-sided CaHD should routinely consist of valve replacement and subsequent enlargement of the right ventricular outflow tract.
2. There is a clear trend toward significantly improved perioperative outcomes and survival, which will probably impact management and referral patterns in the very near future.
3. A comprehensive multidisciplinary assessment of tumor burden and cardiac status (introduction of new imaging tools and more accurate biomarkers) is critical in guiding optimal timing of surgery in patients with carcinoid syndrome and CaHD.

Specifically, for example, with increased experience and a multidisciplinary approach, operative mortality at the Mayo Clinic decreased from 20% before 1990 to 6% after 2000. Survival rates at 1, 5, and 10 years were 69%, 35%, and 24% respectively. That 10-year survival rate is markedly higher than medically managed historical control subjects with CaHD and NYHA functional symptoms greater than class II: only 10% of those patients survived 2.5 years.

The Mayo experience shows that overall mortality is associated with older age, cytotoxic chemotherapy, and tobacco use; 75% of survivors had symptomatic improvement at follow-up. Increasingly, data suggest survival benefit with presymptomatic surgical intervention for left-sided valve disorders. However, in these patients with CaHD, surgery done before symptom onset was not associated with late survival benefit after multivariate analysis. The authors emphasized that this most likely reflects the comorbid malignancy that independently decreases life expectancy.

In other words, as Warner and Castillo wrote in their editorial comment, while this survival benefit was not observed in multivariate analysis, it was strongly evident before adjusting for factors such as age, tobacco use, and preoperative chemotherapy. Importantly, the authors noted that valve surgery before the onset of advanced symptoms did indeed show a survival benefit for this population compared with operations performed for more advanced symptoms.

Warner and Castillo concluded, “We concur with the authors that this might be a reflection of tumor burden on life expectancy”; therefore, earlier is probably still better.

Readings

1. Connolly HM, Schaff HV, Abel MD, et al. Early and Late Outcomes of Surgical Treatment in Carcinoid Heart Disease. J Am Coll Cardiol 2015;66:2189-96. http://content.onlinejacc.org/article.aspx?articleID=2468950

2. Warner RP, Castillo JG. Carcinoid Heart Disease: The Challenge of the Unknown Known. J Am Coll Cardiol 2015;66:2197-200. http://content.onlinejacc.org/article.aspx?articleID=2468937

Disclosures

Valentin Fuster, MD, PhD, MACC
This author has nothing to disclose.

The planning committee reported nothing to disclose.

A = Advisory panel B = Speakers’ bureau C = Consultant fees/honoraria D = Data and Safety Monitoring Board E = Equity interests/stock options F = Fellowship support G = Grant support L = Licensing Agreement O = Other relationship R = Royalties S = Salary W = Expert witness

Acknowledgements

CME/CE INFO

Accreditation:

The Audio- Digest Foundation is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.

The Audio- Digest Foundation designates this enduring material for a maximum of 0 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Audio Digest Foundation is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center's (ANCC's) Commission on Accreditation. Audio Digest Foundation designates this activity for 0 CE contact hours.

Lecture ID:

AC490112

Expiration:

This CME course qualifies for AMA PRA Category 1 Credits™ for 3 years from the date of publication.

Instructions:

To earn CME/CE credit for this course, you must complete all the following components in the order recommended: (1) Review introductory course content, including Educational Objectives and Faculty/Planner Disclosures; (2) Listen to the audio program and review accompanying learning materials; (3) Complete posttest (only after completing Step 2) and earn a passing score of at least 80%. Taking the course Pretest and completing the Evaluation Survey are strongly recommended (but not mandatory) components of completing this CME/CE course.

Estimated time to complete this CME/CE course:

Approximately 2x the length of the recorded lecture to account for time spent studying accompanying learning materials and completing tests.

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