Carcinoid Valve Disease
- 370 Downloads
Carcinoid is a rare neuroendocrine tumor that typically originates in the gastrointestinal tract and can result in a constellation of symptoms, mediated by vasoactive substances, referred to as carcinoid syndrome. Carcinoid valve and heart disease is characterized by the plaque-like, endocardial fibrous tissue deposits, primarily affecting the right heart endocardium and valves, which result as a consequence of the disease process. Potential mechanisms for the carcinoid valve disease include the complex role of excess serotonin and its interaction with serotonin receptors and transporters. Carcinoid valve and heart disease is a frequent occurrence in patients with carcinoid syndrome and is accountable for substantial morbidity and mortality. Cardiac surgery remains the most effective treatment option for carcinoid valve disease and a multidisciplinary approach at an experienced center is recommended for patients with metastatic carcinoid and carcinoid heart disease.
KeywordsCarcinoid valve disease Carcinoid heart disease Carcinoid tumors Carcinoid Carcinoid syndrome Tricuspid regurgitation Pulmonary regurgitation Pulmonary stenosis Valvular heart disease Serotonin Somatostatin Valves Valve surgery
Compliance with Ethics Guidelines
Conflict of Interest
Dr. J. Wells Askew declares that he has no conflict of interest. Dr. Heidi M. Connolly declares that she has no conflict of interest.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
References and Recommended Reading
Papers of particular interest, published recently, have been highlighted as: • Of importance
- 14.Simula DV, Edwards WD, Tazelaar HD, et al. Surgical pathology of carcinoid heart disease: a study of 139 valves from 75 patients spanning 20 years. Mayo Clinic Proc. 2002;77:139–47.Google Scholar
- 15.•Bhattacharyya S, Schapira AH, Mikhailidis DP, et al. Drug-induced fibrotic valvular heart disease. Lancet. 2009;374:577–85. Review of potential mechanisms in the development of drug-induced valvular heart disease including interaction of serotonin, serotonin transporter, and 5HT2B receptors. Carcinoid valve disease is believed to be caused by a similar mechanism.PubMedCrossRefGoogle Scholar
- 31.Lee KJ, Connolly HM, Pellikka PA. Carcinoid pulmonary valvulopathy evaluated by real-time 3-dimensional transthoracic echocardiography. J Am Soc Echocardiogr. 2008;21:407.e401–2.Google Scholar
- 34.Rinke A, Müller H-H, Schade-Brittinger C, et al. Placebo-controlled, double-blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors: a report from the PROMID study group. J Clin Oncol. 2009;27:4656–63.PubMedCrossRefGoogle Scholar
- 35.Öberg K. Biotherapies for GEP-NETs. Best practice & research clinical. Gastroenterology. 2012;26:833–41.Google Scholar
- 45.Connolly HM, Schaff HV, Mullany CJ, et al. Carcinoid heart disease: impact of pulmonary valve replacement in right ventricular function and remodeling. Circulation. 2002;106:I–51–6.Google Scholar
- 46.Connolly HM, Schaff HV, Mullany CJ, et al. Surgical management of left-sided carcinoid heart disease. Circulation. 2001;104:I–36–40.Google Scholar
- 49.Schoen FJ, Hausner RJ, Howell JF, et al. Porcine heterograft valve replacement in carcinoid heart disease. J Thor Cardiovasc Surg. 1981;81:100–5.Google Scholar
- 52.•Weingarten TN, Abel MD, Connolly HM, et al. Intraoperative management of patients with carcinoid heart disease having valvular surgery: a review of one hundred consecutive cases. Anesth Analg. 2007;105:1192–9. This publication details the anesthetic management of consecutive carcinoid heart disease patients undergoing cardiac surgery at a single center and highlights the importance of a multidiscplinary approach in perioperative management and improvement in perioperative survival over time.PubMedCrossRefGoogle Scholar
- 53.•Møller JE, Pellikka PA, Bernheim AM, et al. Prognosis of carcinoid heart disease: analysis of 200 cases over two decades. Circulation. 2005;112:3320–7. This study demonstrates the improvement in prognosis for metastatic carcinoid disease and carcinoid heart disease over two decades and suggests the important contribution of valvular surgery.PubMedCrossRefGoogle Scholar