Carcinoid heart disease: Current understanding and future directions

Patel, Chirdeep; Mathur, Moses; Escárcega, Ricardo O.; Bové, Alfred A.

doi:10.1016/j.ahj.2014.03.018

Cited by 57 publications

(45 citation statements)

References 58 publications

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“…1 First described in the 1950s, 2 CHD occurs in 50% to 60% of patients with carcinoid syndrome, thereby accounting for substantial illness and death. 3 The time period between the onset of symptoms and the diagnosis of CHD is about 2 years. 4 The cause of valve injury in CHD is not completely understood, but the high level of circulating serotonin is most likely the major contributor.…”

Section: Discussionmentioning

confidence: 99%

“…4 The cause of valve injury in CHD is not completely understood, but the high level of circulating serotonin is most likely the major contributor. 3,4 Typically, fibrous plaque-like endocardial thickening causes retraction and fixation of the rightsided heart valves, thereby leading to right-sided heart failure.…”

Section: Discussionmentioning

confidence: 99%

“…[3][4][5] Valve replacement should be considered in patients who have severely dysfunctional valves or are refractory to medical therapy. In our case of severe PV stenosis not amenable to surgical replacement because of the patient's high perioperative risk, we showed that percutaneous valve replacement could be a valid option.…”

Section: Discussionmentioning

confidence: 99%

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Transcatheter Pulmonary Valve Replacement in a Carcinoid Heart

Loyalka¹,

Schechter²,

Nascimbene³

et al. 2016

Texas Heart Institute Journal

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Carcinoid heart disease presents as right-sided heart failure attributable to the dysfunction of the tricuspid and pulmonary valves. Although C arcinoid heart disease (CHD) presents as right-sided heart failure attributable to dysfunction of the tricuspid valve and pulmonary valve (PV). Although surgical valve replacement is the mainstay of treatment when patients become symptomatic, it is associated with substantial perioperative mortality rates. In the transcatheter valve-replacement era, the percutaneous option has become more appealing for application to poor surgical candidates. We present a case of severe PV stenosis secondary to CHD that was treated successfully with percutaneous valve replacement. Case ReportA 67-year-old man with severe PV stenosis was referred to our center for PV evaluation and replacement. The patient had a medical history of metastatic neuroendocrine tumor of the small bowel with carcinoid syndrome, CHD, and tricuspid valve regurgitation treated by means of surgical valve replacement with a 31-mm Mosaic ® valve (Medtronic, Inc.; Minneapolis, Minn) 4 years prior. He reported increased fatigue and shortness of breath over the past several months. The patient was already oxygen dependent because of severe chronic obstructive pulmonary disease (FEV1, 35% by pulmonary function test).Echocardiographic examination revealed severe PV stenosis (peak gradient, 55 mmHg; and peak velocity, 3.71 m/s), moderate pulmonary regurgitation, mild-tomoderate tricuspid regurgitation, and increased right ventricular (RV) systolic pressure with preserved left ventricular ejection fraction.Poor lung function and hostile chest anatomy, revealed by a computed tomogram (CT) from prior tricuspid valve replacement, allowed the patient to undergo implantation of an Edwards Sapien XT transcatheter heart-tissue valve (Edwards Lifesciences LLC; Irvine, Calif ) on an off-label compassionate indication. Lone pulmonary valvuloplasty was considered the second-best treatment option because of the baseline presence of moderate pulmonary regurgitation.We chose off-label use of the Sapien XT valve deployed over a Palmaz ® XL stent (Palmaz Scientific; Fremont, Calif ), as previously described in the medical literature.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Transcatheter Pulmonary Valve Replacement in a Carcinoid Heart

Loyalka¹,

Schechter²,

Nascimbene³

et al. 2016

Texas Heart Institute Journal

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“…Carcinoid syndrome has been reported to be present in 8%-35% of patients with NETs and is characterized by a wide range of symptoms, such as flushing episodes, diarrhea, abdominal pain, intermittent bronchial wheezing, telangiectasia, heart valve disease, mesenteric fibrosis, and pellagra [5,[9][10][11]. Carcinoid crisis is considered an immediate life-threatening complication of carcinoid syndrome due to a massive release of vasoactive peptides from the tumor.…”

Section: Carcinoid Syndromementioning

confidence: 99%

Inhibition of Peripheral Synthesis of Serotonin as a New Target in Neuroendocrine Tumors

et al. 2016

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The carcinoid syndrome represents a set of signs and symptoms associated with neuroendocrine tumors (NETs) that occur primarily when metastases are developed in the liver, resulting in the worsening of quality of life. Serotonin plays a central role in the physiology of carcinoid syndrome by promoting intestinal motility. Somatostatin analogs (SSAs) have widely demonstrated their efficacy as symptomatic relievers of carcinoid syndrome, but this control is ephemeral, being reduced by approximately 50% within the first year. The exact mechanisms of resistance to SSAs are not fully understood, but it is believed that serotonin might be involved. Patients with carcinoid syndrome present with a significant increase in serotonin plasma levels and, consequently, in the soluble urinary metabolite 5‐hydroxyindole acetic acid. Telotristat etiprate is a potent inhibitor of tryptophan hydroxylase, a rate‐limiting enzyme in the synthesis of serotonin, that has demonstrated in the phase III TELESTAR clinical trial a significant improvement in the control of bowel movements in patients with NETs who have carcinoid syndrome and who have progressed to an SSA. Based on these results, telotristat etiprate has emerged as a potential new option in the treatment algorithm of symptomatic control of functioning NETs. However, some issues need to be clarified, such as the safety profile of the drug outside clinical trials, the benefit in quality of life, and the possible impact on tumor growth, as well as its role within sequencing or combination treatment strategies with pre‐existing drugs effective in NET treatment. Implications for Practice: This article reviews the literature about carcinoid syndrome, which affects patients diagnosed with neuroendocrine tumors. Few articles have been published about this syndrome and its pathophysiology. Somatostatin analogs provide symptomatic relief; however, patients may become refractory to this strategy, usually within the first year of treatment. In this context, as an agent with an innovative mechanism of action, telotristat etiprate has demonstrated activity in a phase III trial, and findings may offer a path to an improve quality of life and prolonged survival for certain patients.

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“…Carcinoid heart disease (CHD) or Hedinger syndrome is a unique clinical entity which develops in approximately 20-25% of patients with a carcinoid tumor and CS, and is characterized by fibrous, plaque-like deposits on the endocardial surfaces of valve leaflets and the subvalvular apparatus [7]. CHD is the first manifestation of CS in up to 20% of patients and primarily afflicts right-sided heart valves, causing, in order of frequency, tricuspid valve (TV) regurgitation, pulmonary valve (PV) regurgitation and PV stenosis, and eventually leads to right heart failure (RHF) [8].…”

Section: Introductionmentioning

confidence: 99%

Cardiac Surgery for Carcinoid Heart Disease: A Weapon Not to Be Misused

Bonou

Kapelios²,

Kaltsas³

et al. 2016

Cardiology

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Carcinoid heart disease (CHD) complicates approximately 25% of patients with a carcinoid tumor and carcinoid syndrome and leads to heart valve degeneration with mixed-stenotic and regurgitation pathology and consequent heart failure (HF) leading to significant morbidity and mortality. Cardiac surgery in symptomatic, severe CHD leads to significantly better functional capacity and prolonged survival when compared to medical treatment alone. Recent studies have shown improvement in postoperative outcomes of patients undergoing surgery for CHD over the last decades. The trend for early diagnosis and application of surgery prior to the manifestation of HF symptoms, which tended to develop during the previous years, does not seem justifiable based on the findings of recent studies. Therefore, the optimal timing of intervention in CHD and the type of valve that should preferably be used remain issues of controversy. This review comprehensively examines the existing literature on the treatment options for patients with CHD, with a special focus on short- and long-term survival after cardiac surgery, and discusses the selection of the exact patient profile and intervention timing that are more likely to optimize the benefit-to-risk ratio for surgical intervention.

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Carcinoid heart disease: Current understanding and future directions

Cited by 57 publications

References 58 publications

Transcatheter Pulmonary Valve Replacement in a Carcinoid Heart

Transcatheter Pulmonary Valve Replacement in a Carcinoid Heart

Inhibition of Peripheral Synthesis of Serotonin as a New Target in Neuroendocrine Tumors

Cardiac Surgery for Carcinoid Heart Disease: A Weapon Not to Be Misused

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