Occurrence of left-sided heart valve involvement before right-sided heart valve involvement in carcinoid heart disease

Marupakula, Vidyasagargoud; Vinales, Karyne L.; Najib, Mohammad Q.; Lanza, Louis A.; Lee, Howard R.; Chaliki, Hari P.

doi:10.1093/ejechocard/jeq171

Cited by 8 publications

(5 citation statements)

References 9 publications

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“…The preferential right-sided involvement is due to inactivation of vasoactive substances by the lungs where serotonin is also degraded to 5-HIAA, which substantially reduces the amount of serotonin capable of inducing left heart fibrosis [ 9 ]. However, left-sided valve pathology can be seen in patients with an intracardiac shunt, such as a patent foramen ovale (PFO) or atrial septal defect (ASD), or with primary bronchial carcinoid [ 9 ]. This patient did not have an intracardiac shunt, nor did she have a primary bronchial carcinoid tumor.…”

Section: Discussionmentioning

confidence: 99%

A Case of Carcinoid Syndrome Due to Malignant Metastatic Carcinoid Tumor with Carcinoid Heart Disease Involving Four Cardiac Valves

2018

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Patient: Female, 62Final Diagnosis: Carcinoid heart diseaseSymptoms: Abdominal discomfort • diarrhea • fatigue • weight lossMedication: —Clinical Procedure: —Specialty: CardiologyObjective:Rare diseaseBackground:Carcinoid tumor, benign, low-grade malignant, and high-grade malignant, can be associated with the release of vasoactive substances that cause symptoms including cutaneous flushing, diarrhea, and bronchospasm. In 50–60% of patients with carcinoid syndrome, the vasoactive substances cause fibrosis of the pulmonary and tricuspid heart valves which lead to regurgitation and right-sided heart failure. The right side of the heart is usually affected because monoamine oxidases in the lungs usually inactivate the vasoactive substances.Case Report:A 62-year-old woman presented with a four-month history of diarrhea and abdominal discomfort. The patient had bilateral pelvic masses and elevated serum CA125 and an elevated urinary 5-hydroxyindoleacetic acid (5-HIAA). Total abdominal hysterectomy (TAH) and bilateral salpingo-oophorectomy (BSO) identified a primary appendiceal carcinoid tumor with bilateral ovarian metastases. Post-operatively, a computed tomography (CT) scan showed cardiomegaly. Transthoracic echocardiography showed morphologic changes that affected all four heart valves, consistent with carcinoid heart disease but without a patent foramen ovale (PFO). The patient was evaluated for valve replacement surgery, but surgery was not performed due to the degree of heart failure.Conclusions:This report describes a rare case of carcinoid heart disease with involvement of all four cardiac valves, but without the presence of a PFO, possibly due to a large amount of vasoactive substances in the patient’s circulation, as supported by the patient’s elevated urinary 5-HIAA. This rare presentation highlights the importance of early diagnosis and treatment with tumor resection and, if possible, Cardiac valve replacement to prevent irreversible heart failure.

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Section: Discussionmentioning

confidence: 99%

A Case of Carcinoid Syndrome Due to Malignant Metastatic Carcinoid Tumor with Carcinoid Heart Disease Involving Four Cardiac Valves

2018

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“…Left-sided valve disease occurs in approximately 15% of cases, usually only in the setting of a right-to-left shunt, pulmonary involvement, an ovarian source (whereby systemic venous drainage bypasses hepatic metabolism), or an extraordinary burden of circulating vasoactive peptides [2,7,8]. Occasionally, left-sided cardiac involvement may be seen prior to right-sided involvement in the absence of a pulmonary carcinoid when there is a patent foramen ovale and an elevated right atrial pressure [9]. …”

Section: Discussionmentioning

confidence: 99%

Carcinoid Heart Disease without Severe Tricuspid Valve Involvement

et al. 2015

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Carcinoid syndrome causes a rare form of acquired valvular heart disease which typically occurs in the setting of liver metastases. In carcinoid-induced valvular heart disease, the tricuspid valve is almost universally affected; left-sided valve disease occurs infrequently in affected patients. Herein, we report 2 cases of carcinoid-induced valvular heart disease; one case had no evidence of tricuspid valve involvement despite severe involvement of all other valves, while the other case was without severe tricuspid valve involvement.

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“…There is a single case report of left-sided CHD prior to right-sided disease [24]. Conversely degenerative disease of the aortic and/or mitral valves occurs in approximately 2-7% of the population aged over 65 years [25].…”

Section: Discussionmentioning

confidence: 99%

Determination of the Optimal Echocardiographic Scoring System to Quantify Carcinoid Heart Disease

Dobson¹,

Cuthbertson²,

Jones³

et al. 2014

Neuroendocrinology

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Background: Carcinoid heart disease (CHD) is an important complication of metastatic neuroendocrine disease, requiring regular monitoring to enable intervention prior to right heart failure. We aimed to identify the most appropriate echocardiographic scoring systems for the quantitative assessment of CHD. Methods: In this prospective study conducted between April and October 2012 in two European Neuroendocrine Tumor Society (ENETS) Centres of Excellence, patients with neuroendocrine tumours with liver metastases and/or carcinoid syndrome underwent transthoracic echocardiography and blood sampling for serum N-terminal pro-brain natriuretic peptide (NT-proBNP) and plasma 5-hydroxyindoleacetic acid (5-HIAA). Each patient was assessed according to six echocardiographic scoring systems. The individual scoring systems' feasibility, observer variability, sensitivity, specificity and correlation with the concentration biomarkers were determined. Results: 100 patients were included; 21% had echocardiographic evidence of CHD. All scores discriminated highly between those with/without CHD, with no single score performing significantly better than another. The severity, determined using all of the scoring systems, correlated with the concentration of both biomarkers, but the strongest correlations were seen between the Bhattacharyya score and serum NT-proBNP. Conclusion: All scoring systems are comparable in terms of sensitivity and specificity for the detection of CHD. There is a variation in the feasibility of the scoring systems due to varying complexity of the score components. All scores correlate with NT-proBNP and plasma 5-HIAA. The Westberg score appears to be the most optimal scoring system for use in screening of CHD whereas the more complex scoring systems are more suited to the patient with established disease who may require surgical intervention.

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Occurrence of left-sided heart valve involvement before right-sided heart valve involvement in carcinoid heart disease

Cited by 8 publications

References 9 publications

A Case of Carcinoid Syndrome Due to Malignant Metastatic Carcinoid Tumor with Carcinoid Heart Disease Involving Four Cardiac Valves

A Case of Carcinoid Syndrome Due to Malignant Metastatic Carcinoid Tumor with Carcinoid Heart Disease Involving Four Cardiac Valves

Carcinoid Heart Disease without Severe Tricuspid Valve Involvement

Determination of the Optimal Echocardiographic Scoring System to Quantify Carcinoid Heart Disease

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