Four decades of experience with carcinoid heart disease: An analysis of 84 patients

Levy, Sonja; Korse, Catherina E.; Groot, Andre C. A. de; Meijer, Rudy; Tesselaar, Margot E. T.; Valk, Gerlof D.

doi:10.1111/jne.13199

Cited by 5 publications

(8 citation statements)

References 41 publications

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“…24 NT-proBNP levels have also been found to be associated with CHD progression and mortality. 19 Hence, NT-proBNP is also recommended as a routine biomarker for diagnostic and prognostic evaluation of CHD in patients with NETs (Table 1). 4,[24][25][26]…”

Section: N-terminal Pro-b-type Natriuretic Peptide (Nt-probnp)mentioning

confidence: 99%

“…17,19,48,55 Subsequently, the proportion of patients with CHD recurrence on bioprostheses requiring reintervention has gradually increased, accounting for 5%-10% of all patients in recent studies. 19,48,67 The main pejorative prognostic factors of patients with CHDin addition to surgical replacementare older age, advanced New York Heart Association (NYHA) class, high NT-proBNP and 5-HIAA levels and right ventricular dilatation (Table 4). These features underline the role of clinical, biological and echocardiographic follow-up in these patients.…”

Section: Perioperative Managementmentioning

confidence: 99%

“…CHD generally occurs 2-5 years after the initial diagnosis of metastatic NETs, and despite the presence of valvular involvement, cardiac symptoms are often absent for a long time. 7,[17][18][19] Indeed, approximately 60% of patients with moderate to severe tricuspid regurgitation have mild symptoms or are even asymptomatic. When symptoms such as asthenia and/or dyspnoea are present, they are mainly related to right-sided heart involvement.…”

Section: Clinical Presentationmentioning

confidence: 99%

“…A cutoff of 260 pg/mL had a sensitivity and specificity of 92% and 91%, respectively, for the diagnosis of CHD 24 . NT‐proBNP levels have also been found to be associated with CHD progression and mortality 19 . Hence, NT‐proBNP is also recommended as a routine biomarker for diagnostic and prognostic evaluation of CHD in patients with NETs (Table 1).…”

Section: Circulating Biomarkersmentioning

confidence: 99%

“…Since then, the prognosis of patients with CHD has gradually improved, with an increasing in median survival from 1–2 years in 2000 to 3–4 years in 2020 (Table 4). 7,17,19,55 Overall, recent studies have reported 1‐, 5‐ and 10‐year survival rates of approximately 70%, 35% and 20%, respectively, in patients operated on for CHD 6,17,48,51–55 …”

Section: Prognosismentioning

confidence: 99%

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Carcinoid heart disease in patients with midgut neuroendocrine tumours

Delhomme

Walter

Arangalage

et al. 2023

J Neuroendocrinology

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Carcinoid heart disease (CHD) is the main complication of carcinoid syndrome (CS) associated with metastatic small intestine neuroendocrine tumours (NETs).The pathophysiology of CHD is partly understood but vasoactive hormones secreted by NETs, especially serotonin, play a major role, leading to the formation of fibrous plaques. These plaque-like deposits involve the right side of the heart in >90% of cases, particularly the tricuspid and pulmonary valves, which become thickened, retracted and immobile, resulting in regurgitation or stenosis. CHD represents a major diagnostic and therapeutic challenge for patients with NET and CS and is associated with increased risk of morbidity and mortality. CHD often occurs 2-5 years after the diagnosis of metastatic NET, but diagnosis of CHD can be delayed as patients are often asymptomatic for a long time despite severe heart valve involvement. Circulating biomarkers (5HIAA, NT-proBNP) are relevant tools but transthoracic echocardiography is the key examination for diagnosis and follow-up of CHD. However, there is no consensus on the optimal indications and frequency of TTE and biomarker dosing regarding screening and diagnosis. Treatment of CHD is complex and requires a multidisciplinary approach. It relies on antitumour treatment, control of CS and surgical valve replacement in cases of severe CHD. However, cardiac surgery is associated with a high risk of mortality, notably due to perioperative carcinoid crisis and right ventricular dysfunction. Timing of surgery is the most crucial point of CHD management and relies on the case-by-case determination of the optimal compromise between tumour progression, cardiac symptoms and CS control.

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Section: N-terminal Pro-b-type Natriuretic Peptide (Nt-probnp)mentioning

confidence: 99%

Section: Perioperative Managementmentioning

confidence: 99%

Section: Clinical Presentationmentioning

confidence: 99%

Section: Circulating Biomarkersmentioning

confidence: 99%

Section: Prognosismentioning

confidence: 99%

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Carcinoid heart disease in patients with midgut neuroendocrine tumours

Delhomme

Walter

Arangalage

et al. 2023

J Neuroendocrinology

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Biomarkers to Inform Prognosis and Treatment for Unresectable or Metastatic GEP-NENs

Loree,

Chan,

Lim

et al. 2024

JAMA Oncol

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ImportanceEvidence-based treatment decisions for advanced gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) require individualized patient-centered decision-making that accounts for patient and cancer characteristics.ObjectiveTo create an accessible guidance document to educate clinicians and patients on biomarkers informing prognosis and treatment in unresectable or metastatic GEP-NENs.MethodsA multidisciplinary panel in-person workshop was convened to define methods. English language articles published from January 2016 to January 2023 in PubMed (MEDLINE) and relevant conference abstracts were reviewed to investigate prognostic and treatment-informing features in unresectable or metastatic GEP-NENs. Data from included studies were used to form evidence-based recommendations. Quality of evidence and strength of recommendations were determined using the Grading of Recommendations, Assessment, Development and Evaluations framework. Consensus was reached via electronic survey following a modified Delphi method.FindingsA total of 131 publications were identified, including 8 systematic reviews and meta-analyses, 6 randomized clinical trials, 29 prospective studies, and 88 retrospective cohort studies. After 2 rounds of surveys, 24 recommendations and 5 good clinical practice statements were developed, with full consensus among panelists. Recommendations focused on tumor and functional imaging characteristics, blood-based biomarkers, and carcinoid heart disease. A single strong recommendation was made for symptomatic carcinoid syndrome informing treatment in midgut neuroendocrine tumors. Conditional recommendations were made to use grade, morphology, primary site, and urinary 5-hydroxyindoleacetic levels to inform treatment. The guidance document was endorsed by the Commonwealth Neuroendocrine Tumour Collaboration and the North American Neuroendocrine Tumor Society.Conclusions and RelevanceThe study results suggest that select factors have sufficient evidence to inform care in GEP-NENs, but the evidence for most biomarkers is weak. This article may help guide management and identify gaps for future research to advance personalized medicine and improve outcomes for patients with GEP-NENs.

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Le cœur carcinoïde : physiopathologie, diagnostic, pronostic et prise en charge

François

2023

Archives des Maladies du Coeur et des Vaisseaux - Pratique

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Four decades of experience with carcinoid heart disease: An analysis of 84 patients

Cited by 5 publications

References 41 publications

Carcinoid heart disease in patients with midgut neuroendocrine tumours

Carcinoid heart disease in patients with midgut neuroendocrine tumours

Biomarkers to Inform Prognosis and Treatment for Unresectable or Metastatic GEP-NENs

Le cœur carcinoïde : physiopathologie, diagnostic, pronostic et prise en charge

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