Carcinoid Heart Disease without Severe Tricuspid Valve Involvement

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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Valvular heart disease in autoimmune diseases in Mexico

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jeaa356.071 ◽

2021 ◽

Vol 22 (Supplement_1) ◽

Author(s):

J Aceituno Melgar ◽

JF Fritche-Salazar ◽

ME Soto-Lopez

Keyword(s):

Heart Disease ◽

Mitral Valve ◽

Aortic Valve ◽

Aortic Stenosis ◽

Autoimmune Diseases ◽

Tricuspid Valve ◽

Tricuspid Regurgitation ◽

Valvular Heart Disease ◽

Valve Disease ◽

Calcium Deposit

Abstract Funding Acknowledgements Type of funding sources: None. Background The autoimmune diseases (AD) have high morbidity and mortality due to their affection to the heart. Purpose Our objective was to describe the valvular heart disease (VHD) in patients with AD. Methods Patients with systemic lupus erythematous (SLE), rheumatoid arthritis (RA), and systemic sclerosis (SS) diagnosis were included, from January 1st 2008 to December 31th 2018. Prevalence rates of valve involvement were calculated. Results A total of 163 patients (57.6% with SLE, 23.3% with RA, 19.0% with SS) were included. The global prevalence of VHD was 5.4% in SLS, 23.6% en RA, and 15.9% in SS. The more affected valve in SLS was the tricuspid valve in 24% (12% with severe tricuspid regurgitation (STR), p = 0.028), in RA was the aortic valve in 26% (13% with severe aortic stenosis (SAS), p = 0.02), and with SS was the tricuspid valve in 48% (29% with moderate tricuspid regurgitation (MTR)). The calcium deposit was present in 66% in RA (37% in aortic valve, p < 0.001). The valve thickening (>5 mm) was higher in RA (50%, p < 0.001), with predominance in mitral valve (26%). Conclusions We found significant higher rates of STR in SLE, SAS in RA, and MTR in SS compared with the literature. Moreover, calcification and valve thickening were found more often in RA. Early diagnosis of subclinical VHD is mandatory to improve the long-term prognosis of these patients. Valvular heart disease. Autoimmune Disease (n = 163) P value* SLE (n = 94) RA (n = 38) ES (n = 31) Demographic characteristics Age, years. Gender, Male / Female, n Body Mass Index (kg/m2) Arterial hypertension, n (%) Diabetes Mellitus, n (%) 38.8 (12.6) 9/85 26.2 (5.9) 21(22.3%) 6 (6.3%) 62.45 (12.3) 7/31 26.6 (7.1) 14(36.8%) 4 (10.5%) 53.8 (13.3) 2/29 25.4 (4.7) 12 (38.7) 5 (16.1%) <0.001 NS NS NS NS Echocardiographic findings. Valve thickening Aortic Mitral 8 (9%) 1 (1%) 7 (7%) 19 (50%) 9 (24%) 10 (26%) 1 (3%) 0 1 (3%) <0.001 Calcium Deposit Aortic Mitral 4 (4%) 2 (2%) 2 (2%) 25 (66%) 14 (37%) 11 (29%) 8 (26%) 4 (12.8%) 4 (12.9%) <0.001 Aortic valve disease 4 (4%) 10 (26%) 0 Aortic stenosis Moderate Severe 0 0 0 7 (18%) 2 (5%) 5 (13%) 0 0 0 0,02 Moderate Aortic Regurgitation 4 (4%) 3 (8%) 0 NS Mitral valve disease 8 (9%) 2 (5%) 2 (6%) Mitral stenosis Moderate Severe 4 (4%) 2 (2%) 2 (2%) 1 (3%) 0 1 (3%) 1 (3%) 0 1 (3%) NS Mitral Regurgitation Moderate Severe 4 (4%) 2 (2%) 2 (2%) 1 (3%) 0 1 (3%) 1 (3%) 0 1 (3%) NS Tricuspid Regurgitation Moderate Severe 22 (24%) 11 (12%) 11 (12%) 8 (21%) 7 (18%) 1 (3%) 15 (48%) 9 (29%) 6 (19%) 0,028 Pulmonic valve disease Moderate Pulmonic Stenosis Moderate Pulmonic Regurgitation 6 (6%) 1 (1%) 5 (5%) 1 (3%) 0 1 (3%) 0 0 0 NS * Not Significant.

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Case of calcific tricuspid and pulmonary valve stenosis

BMJ Case Reports ◽

10.1136/bcr-2020-235190 ◽

2020 ◽

Vol 13 (9) ◽

pp. e235190

Author(s):

Yash Paul Sharma ◽

Prashant Kumar Panda ◽

Lipi Uppal ◽

Uma Debi

Keyword(s):

Heart Disease ◽

Tricuspid Valve ◽

Valvular Heart Disease ◽

Pulmonary Valve ◽

Carcinoid Heart Disease ◽

Pulmonary Valve Stenosis ◽

Almost All ◽

Unusual Finding ◽

Severe Calcification ◽

Tricuspid Valve Stenosis

Isolated right-sided valvular disease is a much less recognised entity when compared with left-sided valvular heart disease. Almost all the cases of combined pulmonary valve with tricuspid valve involvement are a consequence of underlying carcinoid heart disease. Moreover, severe calcification of tricuspid valve is an extremely unusual finding. We report a case of a severe calcific tricuspid valve stenosis along with severe pulmonary valve stenosis where the exact aetiology could not be established. On reviewing the literature, we did not find any reports describing such a morphology.

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1640 Pulmonary valve in carcinoid disease: be suspicious of functional assessment

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.1030 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

A L Gamarra Lobato ◽

A M Rojas ◽

A Cecconi ◽

L Dominguez ◽

A Benedicto ◽

...

Keyword(s):

Heart Disease ◽

Right Ventricle ◽

Tricuspid Valve ◽

Functional Assessment ◽

Carcinoid Syndrome ◽

Pulmonary Valve ◽

Previous History ◽

Pulmonary Regurgitation ◽

Lower Limbs ◽

Carcinoid Heart Disease

Abstract Carcinoid heart disease occurs in 50% of patients with carcinoid syndrome, especially on serotonin-secreting neuroendocrine tumors (NET) [1, 2]. Carcinoid heart disease"s typical findings are carcinoid plaques, composed of smooth muscle, myofibroblasts and endothelium. These plaques, most frequently localized on the right side valvular cusps and leaflets, produce fixation and retraction, causing regurgitation and/or stenosis [3, 4, 5]. Left-sided heart disease is rare (<10%), as serotonin is purified in the pulmonary circulation [3, 6, 7]. We present the case of a 67 year old female patient with previous history of right ovarian NET. She presented with dyspnea (II-III NYHA functional class), and slight edema in lower limbs. She denied symptoms of carcinoid syndrome before or after the ovarian surgery. Examination showed an increased jugular venous pulse. Transthoracic echocardiography (TTE) showed severely dilated right cavities and a rigid, immobile tricuspid valve, with massive tricuspid regurgitation (Panel A, 1). Anatomy of pulmonary valve was not properly visualized but jet area of pulmonary regurgitation was small (Panel A, 2). Left cavities and valves were intact. As TTE was not anatomically conclusive, a cardiac magnetic resonance (CMR) and a cardiac computed tomography (CCT) were performed to assess the carcinoid involvement of pulmonary valve. CMR showed severely dilated right ventricle with mild impairment of systolic function (Panel A, 3). Based on phase contrast imaging, pulmonary regurgitation fraction was 14%, suggestive of a mild grade. However, CCT showed a diffuse thickening of the pulmonary valve, with complete opening during diastole (Panel A, 4, arrowhead). Finally, the patient underwent replacement of tricuspid valve and pulmonary valve for biological prosthesis without complications. Our case is remarkable because it highlights the limitations of the functional assessment of pulmonary regurgitation in the presence of a concomitant massive tricuspid valve regurgitation, since the rapid equalization of pressure between pulmonary artery and right ventricle reduces the expression of pulmonary regurgitation. For these reason, the anatomic assessment of the pulmonary valve is mandatory to stage the involvement of pulmonary valve in carcinoid disease. Abstract 1640 Figure. Panel A

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P225 Carcinoid heart disease: report of a case in a patients with trombocytopenia absent radius

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.091 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

M Quezada ◽

R Ayala ◽

M Ramos ◽

Z Villa Benayas ◽

M Calderon-Dominguez ◽

...

Keyword(s):

Heart Disease ◽

Tricuspid Valve ◽

Tricuspid Regurgitation ◽

Carcinoid Syndrome ◽

Clinical Manifestations ◽

Somatostatin Analogues ◽

Carcinoid Heart Disease ◽

Delay In Diagnosis ◽

Bilateral Absence ◽

Absent Radius

Abstract Background The carcinoid syndrome is characterized by extensive and several clinical manifestations. The diarrhea, the cutaneous flushing are the most frequents symptoms while cardiac manifestations (carcinoid heart disease) (CHD) occurs in a mean of 40%. Nowadays, the number of cases of CHD is lower than 20%, as a consequence of the widespread use of somatostatin analogues. At present, there is a mean delay in diagnosis of CHD of 1.5 years from the time of carcinoid syndrome detection. Hence, CHD is associated with a poor prognosis for clinical management. Case report We present a case of 45-years-old active woman, with Thrombocytopenia absent radius (TAR). This is characterized by a bilateral absence of the radio with the presence of both thumbs and thrombocytopenia. Our patient was attended for dyspnea of medium efforts, history of diarrhea, cutaneous flushing with tachycardia and elevated urinary 5-hydroxyindoleacetic acid (5-HIAA) (89,6 mg/24 (2,0-9,0)). The Transthoracic echocardiography showed morphologic changes that affected the tricuspid valve: diminished curvature of the leaflets, altered dynamic motion of the leaflets during diastole, fused and shortened chordae retraction and reduced excursion of the valve. A moderate to severe tricuspid regurgitation and tricuspid stenosis with gradient media de 5 mmHg was observed. In addition, the right ventricle was dilated, a severe pulmonary hypertension, a right pleural effusion and a minor pericardial effusion circumference were detected. All these findings were consistent with CHD. Conclusions This report describes an unusual case of CHD in TAR patient. In fact, the interest of this case is the role played by the echocardiogram in the differential diagnosis for tricuspid valve diseases. Tricuspid stenosis is an infrequent condition and it is usually related with rheumatic disease associated with mitral valve disease. Although the carcinoid syndrome is infrequent, any changes in the anatomical structure of the tricuspid valve (thickening, fibrosis and rigidity associated with stenosis and tricuspid regurgitation) should alert us to the suspicion of CHD Abstract P225 Figure.

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Chest Radiography in the Carcinoid Syndrome

Acta Radiologica ◽

10.1177/028418518702800109 ◽

1987 ◽

Vol 28 (1) ◽

pp. 49-51 ◽

Cited By ~ 1

Author(s):

B. Thorvinger ◽

C. Lundstedt ◽

H. Mårtensson

Keyword(s):

Heart Disease ◽

Liver Metastases ◽

Chest Radiography ◽

Carcinoid Syndrome ◽

Cardiac Involvement ◽

Carcinoid Tumors ◽

Carcinoid Heart Disease

The chest films of 44 patients with liver metastases from intestinal carcinoid tumors were reviewed in order to evaluate the frequency of significant carcinoid heart disease. Only two patients had obvious signs of cardiac involvement, which is contradictory to most other studies.

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Choice of approaches for surgical correction of tricuspid pathology in patients with rheumatic multi-valvular heart disease

Heart Vessels and Transplantation ◽

10.24969/hvt.2017.9 ◽

2017 ◽

Vol 1 (Issue 1) ◽

Author(s):

Hamidullah Abdumadzhidov ◽

Hayrullah Buranov ◽

Ilkhom Huzhakulov ◽

Ikrom Mirhodzhaev ◽

Sh. Artikov

Keyword(s):

Heart Disease ◽

Tricuspid Valve ◽

Reconstructive Surgery ◽

Tricuspid Regurgitation ◽

Valvular Heart Disease ◽

Heart Defects ◽

Open Heart Surgery ◽

Surgical Correction ◽

Valve Disease ◽

Moderate Degree

Objective:- To analyze the results of surgical correction of patients with tricuspid pathology in rheumatic multi-valvular heart disease. Methods: We retrospectively analyzed outcomes of surgical correction of tricuspid valve disease in 292 patients with rheumatic multi-valvular heart defects, who underwent surgery in our clinic. Results: The age of our patients ranged from 12 to 74 years (mean age 36.7 (9.4) years), among them 197 (67.4%) women and 95 (32.6%) - men. According to the degree of circulatory disorders, 21 (7.2%) patients were in NYHA class III- and 271 (92.8%) patients - class IV. Of them 235 (80.5%) patients were operated by the method of De Vega using plastic fibrous ring.- After tricuspid valve (TV) and fibrous ring repair- in 26.9% - tricuspid regurgitation disappeared, in 62.8% - regurgitation decreased to the 1st degree, and the remaining 10.3% of patients had- 2nd- (moderate) degree tricuspid regurgitation. In 7 (2.38%) cases of infective endocarditis, the "open heart surgery" correction - replacement of TV with biological prosthesis was made. Creation of the bicuspid tricuspid valve techniques was used in 13.4% of cases. -------- Conclusion: Our study demonstrated that correction of tricuspid valve disease in our cohort of patients, including valve repair and replacement and reconstructive surgery of fibrous ring alone or in combination with mitral or aortic valve replacement/ repair is accompanied by reduction of tricuspid regurgitation- and- reduction of cardiac chamber size and right ventricular pressure. No complications intrinsic to operative technique of tricuspid valve reconstructive surgery as advanced atrioventricular block or myocardial ischemia and infarction were recorded.

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Clinical outcomes of Japanese atrial fibrillation patients with combined valvular heart disease: the Fushimi AF Registry

European Heart Journal ◽

10.1093/ehjci/ehaa946.0528 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

K Doi ◽

K Ishigami ◽

Y Aono ◽

S Ikeda ◽

Y Hamatani ◽

...

Keyword(s):

Atrial Fibrillation ◽

Heart Disease ◽

Incidence Rate ◽

Clinical Outcomes ◽

Valvular Heart Disease ◽

Cardiac Death ◽

Clinical Characteristics ◽

Valve Disease ◽

The Impact

Abstract Background We previously reported that valvular heart disease (VHD) was not at the significant risk of stroke/systemic embolism (SE), but was associated with an increased risk of hospitalization for heart failure (HF) in Japanese atrial fibrillation patients. However, the impact of combined VHD on clinical outcomes has been little known. Purpose The aim of this study is to investigate the prevalence of combined VHD and its clinical characteristics and impact on outcomes such as stroke/SE, all-cause death, cardiac death and hospitalization for HF. Method The Fushimi AF Registry is a community-based prospective survey of AF patients in one of the wards of our city which is a typical urban district of Japan. We started to enroll patients from March 2011, and follow-up data were available for 4,466 patients by the end of November 2019. In the entire cohort, echocardiography data were available for 3,574 patients. 68 AF patients with prosthetic heart valves were excluded and we compared clinical characteristics and outcomes between 488 single VHD (103 Aortic valve disease (AVD), 315 mitral valve disease (MVD), 70 tricuspid valve disease (TVD)) and 158 combined VHD (46 AVD and MVD, 11 AVD and TVD, 66 MVD and TVD, 35 AVD and MVD and TVD). Result Compared with single VHD, patients with combined VHD were older (combined vs. single VHD: 78.5 vs. 76.0 years, respectively; p<0.01), more likely to have persistent/permanent type AF (73.4% vs. 63.9%, p=0.02) and prescription of warfarin (63.1% vs. 53.8%, p=0.04). Combined VHD was less likely to have diabetes mellitus (13.9% vs. 23.6%, p=0.01) and dyslipidemia (26.6% vs. 40.4%, p<0.01). Sex, body weight, hypertension, pre-existing HF were comparable between the two groups. During the median follow-up of 1,474 days, the incidence rate of stroke/SE was not significantly different between the two groups (1.58 vs. 1.89 per 100 person-years, respectively, log rank p=0.10). The incidence rate of all-cause death (7.35 vs. 5.33, p=0.65), cardiac death (1.20 vs. 0.99, p=0.91) and hospitalization for HF (5.55 vs. 4.43, p=0.53) were also not significantly different. We previously reported AVD had significant impacts on cardiac adverse outcomes in AF patients, and we further analyzed event rates between combined VHD including AVD (AVD and MVD/TVD) and without AVD (MVD and TVD). Combined VHD with AVD group had higher incidence rate of all-cause death (10.7 vs. 5.79, p=0.03) than that without AVD group. However, the incidence rate of stroke/SE (1.98 vs. 1.56, p=0.59), cardiac death (0.98 vs. 1.14, p=0.68), hospitalization for HF (8.03 vs. 5.38, p=0.17) were not significantly different between the two groups. Conclusion As compared with single VHD, the risk of stroke/SE, all-cause death, cardiac death and hospitalization for HF in combined VHD was not significantly different. Among patients with combined VHD, those having AVD had higher incidence rate of all-cause death than those without AVD. Figure 1 Funding Acknowledgement Type of funding source: None

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