scholarly journals Progression of Carcinoid Heart Disease in the Modern Management Era

2021 ◽  
Author(s):  
Emilie Baron ◽  
Catherine Szymanski ◽  
Hélène Hergault ◽  
Céline Lepère ◽  
Olivier Dubourg ◽  
...  
Keyword(s):  
Heart Disease ◽  
Cardiac Involvement ◽  
Independent Predictor ◽  
Endocrine Tumor ◽  
Carcinoid Heart Disease ◽  
Maximum Value ◽  
Echocardiographic Examination ◽  
Hydroxyindoleacetic Acid ◽  
History Of

Background The development of carcinoid heart disease (CaHD) is still relatively unclear. It is difficult to define an optimal follow‐up for patients without any cardiac involvement at baseline. The aim of this study was to assess the prevalence and natural history of CaHD by annual echocardiographic examinations. Methods and Results We studied 137 consecutive patients (61±12 years, 53% men) with proven digestive endocrine tumor and carcinoid syndrome between 1997 and 2017. All patients underwent serial conventional transthoracic echocardiographic studies. Right‐sided and left‐sided CaHD were systematically assessed. We used a previous validated echocardiographic scoring system of severity for the assessment of CaHD. An increase of 25% of the score was considered to be significant. Mean follow‐up was 54±45 months. Prevalence of CaHD was 27% at baseline and 32% at 5‐year follow‐up. Disease progression was reported in 28% of patients with initial CaHD followed up for >2 years (n=25). In patients without any cardiac involvement at baseline, occurrence of disease was 21%. CaHD occurred >5 years from the initial echocardiographic examination in 42% of our cases, especially in patients presenting with new recurrence of a digestive endocrine tumor. An increase of urinary 5‐hydroxyindoleacetic acid by 25% during follow‐up was identified as an independent predictor of CaHD occurrence during follow‐up (hazard ratio [HR], 5.81; 95% CI, 1.19–28.38; P =0.03), as well as a maximum value of urinary 5‐hydroxyindoleacetic acid >205 mg/24 h during follow‐up (HR, 8.41; 95% CI, 1.64–43.07; P =0.01). Conclusions Our study demonstrates that in patients without initial CaHD, cardiac involvement may occur late and is related to serotonin. Our data emphasize the need for cardiologic follow‐up in patients with recurrence of the tumor process.

P4653A natural history of carcinoid heart disease in the modern management era

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
E Baron ◽  
C Szymanski ◽  
C Lepere ◽  
H Mustafic ◽  
O Dubourg ◽  
...  
Keyword(s):  
Heart Disease ◽  
Natural History ◽  
Neuroendocrine Tumor ◽  
Cardiac Involvement ◽  
Initial Assessment ◽  
Carcinoid Heart Disease ◽  
Echocardiographic Examination ◽  
History Of ◽  
One Year

Abstract Background The development of carcinoid heart disease (CaHD) is still relatively unknown at present. It is difficult to define an optimal follow-up for patients initially free from cardiac involvement. The aim of this study was to assess the prevalence and the evolution of CaHD using annual echocardiographic follow-up. Methods We reviewed from our database 137 patients (61±12 years, 53% men) with histologically proven neuroendocrine tumor between 1997 and 2017. All patients underwent serial conventional transthoracic echocardiographic studies. Right-sided and left-sided CaHD were systematically assessed. We used a previous validated echocardiographic scoring system of severity for the assessment of CHD. An increase of 25% of the score was considered as significant. Results Mean follow-up was 2.6±3.5 years [0; 16]. Prevalence of CaHD was 27% (37 pts) at baseline and 36% (49 pts) at the end of follow-up. Among patients with initial CaHD followed for more than one year, disease progression was observed in 28% of cases. Among the patients free from initial cardiac involvement, an onset of the disease was observed during follow-up in 21% of cases. The onset of CHD could be very late, more than 5 years from the initial echocardiographic examination in 42% of our cases (Figure). This late occurrence of CaHD was only observed in patients presenting with new resumption of neuroendocrine tumor (symptoms, increased of 5-HIAA, occurrence of new metastasis). Conclusion Our study demonstrated that in patients without initial CaHD, cardiac involvement may occur tardily after a normal initial assessment. Our data suggest the need for prolonged echocardiographic follow-up in patients presenting with a resumption of tumor process.

Burden of carcinoid heart disease in patients initiating treatment for carcinoid syndrome.

2019 ◽  
Vol 37 (4_suppl) ◽  
pp. 262-262
Author(s):  
Vijay N Joish ◽  
Raul Perez-Olle ◽  
Pablo Lapuerta ◽  
Sam Dharba ◽  
Jerome Zacks
Keyword(s):  
Heart Disease ◽  
Carcinoid Syndrome ◽  
Cardiac Involvement ◽  
Somatostatin Analogs ◽  
Delayed Diagnosis ◽  
Carcinoid Heart Disease ◽  
Medical Claim ◽  
Before And After ◽  
And Control

262 Background: Carcinoid heart disease (CaHD) develops as a complication of uncontrolled carcinoid syndrome (CS) due to overproduction of serotonin. Delayed diagnosis or control of CS may result in a greater prevalence of cardiac involvement. The objective of this study was to determine the burden of CaHD in patients initiating somatostatin analogs (SSA) for CS. Methods: This retrospective study investigated claims for commercially insured adults receiving SSA for CS between 2010 and 2016. Claims were evaluated for markers of CaHD such as tricuspid or pulmonary valve-related disease. Eligible patients had ≥ 1 medical claim for CS, had initiated SSA treatment, were continuously enrolled in their health plan for 30 days prior to SSA initiation and for ≥ 1 year, had no sign of acromegaly, and did not participate in a clinical trial during the study period. Patients were followed as long as they were observed in the dataset. Descriptive statistics examined demographic and treatment characteristics and costs for patients with (cases) and without (controls) a marker for CaHD during the study period. Results: A total of 654 patients with CS met the study criteria, of which 38% (n = 248) already had ≥ 1 marker for CaHD prior to initiating SSA treatment. At least 1 CaHD marker was identified in 46% (n = 185) of patients (cases) during the follow-up period. Except for age, all demographic characteristics were similar between cases and controls (n = 221). Cases were significantly older than controls (mean 67 vs. 64 years, p = 0.004). Average follow-up was 26 months (SD 22.0) for cases and 35 months (SD 24.8) for controls. Average time to observation of first CaHD marker for cases was 16 months (SD 16.7) after SSA initiation; most (84%) had ≥ 2 CaHD markers. Markers of right heart involvement were present in 20% of cases, and aortic or mitral valve involvement in 15% and 24%, respectively. Annual per patient expenditures were significantly higher for cases versus controls (mean [SD]: $51,825 [$70,423] vs $29,068 [$56,454], respectively). Conclusions: This study revealed that CaHD is common among patients with CS both before and after initiating SSA treatment. Early diagnosis and control of CS is necessary to reduce the burden of CaHD.

scholarly journals Coronary risk of patients with valvular heart disease: prospective validation of CT-Valve Score

Open Heart ◽  
2020 ◽  
Vol 7 (2) ◽  
pp. e001380
Author(s):  
Rasmus Bo Hasselbalch ◽  
Mia Marie Pries-Heje ◽  
Sarah Louise Kjølhede Holle ◽  
Thomas Engstrøm ◽  
Merete Heitmann ◽  
...  
Keyword(s):  
Heart Disease ◽  
Valvular Heart Disease ◽  
Primary Outcome ◽  
Multislice Ct ◽  
Cost Effective ◽  
University Hospital ◽  
Before And After ◽  
History Of ◽  
Artery Disease

ObjectiveTo prospectively validate the CT-Valve score, a new risk score designed to identify patients with valvular heart disease at a low risk of coronary artery disease (CAD) who could benefit from multislice CT (MSCT) first instead of coronary angiography (CAG).MethodsThis was a prospective cohort study of patients referred for valve surgery in the Capital Region of Denmark and Odense University Hospital from the 1 February 2015 to the 1 February 2017. MSCT was implemented for patients with a CT-Valve score ≤7 at the referring physician’s discretion. Patients with a history of CAD or chronic kidney disease were excluded. The primary outcome was the proportion of patients needing reevaluation with CAG after MSCT and risk of CAD among the patients determined to be low to intermediate risk.ResultsIn total, 1149 patients were included. The median score was 9 (IQR 3) and 339 (30%) had a score ≤7. MSCT was used for 117 patients. Of these 29 (25%) were reevaluated and 9 (7.7%) had CAD. Of the 222 patients with a score ≤7 that did not receive an MSCT, 14 (6%) had significant CAD. The estimated total cost of evaluation among patients with a score ≤7 before implementation was €132 093 compared with €79 073 after, a 40% reduction. Similarly, estimated total radiation before and after was 608 mSv and 362 mSv, a 41% reduction. Follow-up at a median of 32 months (18–48) showed no ischaemic events for patients receiving only MSCT.ConclusionThe CT-Valve score is a valid method for determining risk of CAD among patients with valvular heart disease. Using a score ≤7 as a cut-off for the use of MSCT is safe and cost-effective.

scholarly journals Double Inlet Left Ventricle with Eisenmenger Syndrome in an Adult – A Case Report

2017 ◽  
Vol 5 (1) ◽  
pp. 53-56
Author(s):  
Rahul Regi Abraham ◽  
Rahul Regi Abraham
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Left Ventricle ◽  
Congenital Heart ◽  
Pulmonary Stenosis ◽  
Eisenmenger Syndrome ◽  
Tertiary Center ◽  
History Of ◽  
Double Inlet Left Ventricle

Background: Patient diagnosed with double inlet left ventricle (prevalent in 5 – 10 in 100,000 newborns) complicated with Eisenmenger syndrome had a median survival age of 14 years without corrective surgery. Congenital heart disease such as this is usually treated by multiple surgeries during early childhood. A surgically uncorrected case in adults is not of common occurrence. Further, generalized itching after coming in contact with water (aquagenic pruritis) presented an interesting conundrum to treat. Case: A 29-year-old patient in India presented at a primary health care center with a history of difficulty breathing and discoloration of extremities since birth. He also gave a history of itching which commonly occurred after taking bath, hemoptysis and history of turning blue in color after birth. Patient had received no treatment besides regular phlebotomies. On examination, there was grade IV clubbing and conjunctival congestion. Cardiovascular examination revealed an enlarged heart, heaving apex beat and a pan-systolic murmur. A provisional diagnosis of a congenital cyanotic heart disease was made. Investigations revealed hemoglobin of 16.8g/dl. X–ray and electrocardiogram showed hypertrophy of the ventricles. An echocardiogram showed double inlet left ventricle with L-malposed vessels but without pulmonary stenosis. A final diagnosis of congenital heart disease; double inlet left ventricle, L-malposed vessels without pulmonary stenosis, Eisenmenger Syndrome and absolute erythrocytosis was made. Patient was advised for further management with a cardiologist in a tertiary center but the patient did not follow up. Conclusion: Unlike in high-income countries where most congenital heart diseases are detected and dealt with at birth whereas low-and middle-income nations often have to deal with cases that present much later and should often be included in the differential diagnosis. Inability to follow up cases, centers that are poorly equipped and lack of facilities for investigations, patient’s lack of medical awareness, and financial restrictions are major barriers to providing optimal treatment.

Indolent Neuroendocrine Tumor Involving the Bone Marrow

2003 ◽  
Vol 127 (4) ◽  
pp. 488-491
Author(s):  
Ellen Schlette ◽  
L. Jeffrey Medeiros ◽  
Miloslav Beran ◽  
Carlos E. Bueso-Ramos
Keyword(s):  
Bone Marrow ◽  
Neuroendocrine Tumor ◽  
Bone Marrow Biopsy ◽  
Clinical Symptoms ◽  
Eosinophilic Cytoplasm ◽  
Hydroxyindoleacetic Acid ◽  
History Of ◽  
Cardiac Valve Disease ◽  
Immunohistochemical Studies

Abstract We report a unique case of a patient with a neuroendocrine tumor localized to the bone marrow. The patient had a history of hairy cell leukemia, and the neuroendocrine tumor was detected in a bone marrow biopsy specimen obtained to assess response to 2-chlorodeoxyadenosine therapy. The neuroendocrine tumor was present as nodules that replaced approximately 15% of the bone marrow medullary space and was composed of round cells with fine chromatin, indistinct nucleoli, and relatively abundant, granular, eosinophilic cytoplasm. Histochemical stains showed cytoplasmic reactivity with Grimelius and Fontana-Masson stains, and immunohistochemical studies showed positivity for keratin and chromogranin. The histologic, cytochemical, and immunohistochemical features resembled a carcinoid tumor, and metastasis to the bone marrow was considered initially. The patient was asymptomatic without diarrhea, flushing, or cardiac valve disease. Serotonin production, assessed by the measurement of serum 5-hydroxyindoleacetic acid and substance P levels, was normal. Extensive clinical and radiologic work-up and endoscopy of the gastrointestinal tract to detect a primary site other than the bone marrow were negative. Follow-up bone marrow biopsy 7 years after the initial diagnosis was positive for persistent neuroendocrine tumor. The patient has not received any therapy specific for the neuroendocrine tumor and has had no clinical symptoms or evidence of progression after 9 years of clinical follow-up. We suggest that this neuroendocrine tumor may have arisen in the bone marrow.

Cardiac involvement in genetic disease

2010 ◽  
pp. 2834-2841
Author(s):  
Thomas A. Traill
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Family History ◽  
Genetic Disease ◽  
Congenital Heart ◽  
Cardiac Involvement ◽  
Atrioventricular Canal ◽  
Marfan's Syndrome ◽  
Atrioventricular Canal Defect ◽  
History Of

Many clinicians find themselves faced, from time to time, with a patient who has a family history of a known disorder, such as Marfan’s syndrome, or who has noncardiac features that suggest a syndrome. Down’s syndrome—25 to 50% have congenital heart disease, most characteristically atrioventricular canal defect....

scholarly journals Validation of a simplified score for predicting latent rheumatic heart disease progression using a prospective cohort of Brazilian schoolchildren

BMJ Open ◽  
2020 ◽  
Vol 10 (5) ◽  
pp. e036827 ◽  
Author(s):  
Bárbara Martins Bechtlufft ◽  
Bruno Ramos Nascimento ◽  
Craig Sable ◽  
Clara Leal Fraga ◽  
Márcia Melo Barbosa ◽  
...  
Keyword(s):  
Heart Disease ◽  
Public Schools ◽  
Rheumatic Heart Disease ◽  
Heart Diseases ◽  
Diagnostic Category ◽  
Secondary Outcome ◽  
Unfavourable Outcome ◽  
History Of ◽  
Rheumatic Heart

ObjectivesEchocardiographic (echo) screening is an important tool to estimate rheumatic heart disease (RHD) prevalence, but the natural history of screen-detected RHD remains unclear. The PROVAR+ (Programa de RastreamentO da VAlvopatia Reumática) study, which uses non-experts, telemedicine and portable echo, pioneered RHD screening in Brazil. We aimed to assess the mid-term evolution of Brazilian schoolchildren (5–18 years) with echocardiography-detected subclinical RHD and to assess the performance of a simplified score consisting of five components of the World Heart Federation criteria, as a predictor of unfavourable echo outcomes.SettingPublic schools of underserved areas and private schools in Minas Gerais, southeast Brazil.ParticipantsA total of 197 patients (170 borderline and 27 definite RHD) with follow-up of 29±9 months were included. Median age was 14 (12–16) years, and 130 (66%) were woman. Only four patients in the definite group were regularly receiving penicillin.Primary and secondary outcome measuresUnfavourable outcome was based on the 2-year follow-up echo, defined as worsening diagnostic category, remaining with mild definite RHD or development/worsening of valve regurgitation/stenosis.ResultsAmong patients with borderline RHD, 29 (17.1%) progressed to definite, 49 (28.8%) remained stable, 86 (50.6%) regressed to normal and 6 (3.5%) were reclassified as other heart diseases. Among those with definite RHD, 13 (48.1%) remained in the category, while 5 (18.5%) regressed to borderline, 5 (18.5%) regressed to normal and 4 (14.8%) were reclassified as other heart diseases. The simplified echo score was a significant predictor of RHD unfavourable outcome (HR 1.197, 95% CI 1.098 to 1.305, p<0.001).ConclusionThe simple risk score provided an accurate prediction of RHD status at 2-year follow-up, showing a good performance in Brazilian schoolchildren, with a potential value for risk stratification and monitoring of echocardiography-detected RHD.

scholarly journals A natural history of carcinoid heart disease in the modern management era

2020 ◽  
Vol 12 (1) ◽  
pp. 99
Author(s):  
E. Baron ◽  
C. Szymanski ◽  
C. Lepère ◽  
H. Mustafic ◽  
O. Dubourg ◽  
...  
Keyword(s):  
Heart Disease ◽  
Natural History ◽  
Carcinoid Heart Disease ◽  
Modern Management ◽  
History Of
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