scholarly journals Carcinoid Heart Disease: Pathophysiology, Pathology, Clinical Manifestations, and Management

Cardiology ◽  
2020 ◽  
pp. 1-9
Author(s):  
Chengyue Jin ◽  
Ajay Nair Sharma ◽  
Balasingam Thevakumar ◽  
Muhammad Majid ◽  
Shahad Al Chalaby ◽  
...  
Keyword(s):  
Heart Disease ◽  
Heart Valves ◽  
Fibrous Tissue ◽  
Clinical Manifestations ◽  
Carcinoid Heart Disease ◽  
Right Heart ◽  
Limited Mobility ◽  
Vasoactive Substances ◽  
Pulmonic Regurgitation ◽  
Surgical Valve Replacement

Carcinoid heart disease (CHD) is a rare and potentially lethal manifestation of an advanced carcinoid (neuroendocrine) tumor. The pathophysiology of CHD is related to vasoactive substances secreted by the tumor, of which serotonin is most prominent in the pathophysiology of CHD. Serotonin stimulates fibroblast growth and fibrogenesis, which can lead to cardiac valvular fibrosis. CHD primarily affects right heart valves, causing tricuspid and pulmonic regurgitation and less frequently stenosis of these valves. Left heart valves are usually spared because vasoactive substances such as serotonin are enzymatically inactivated in the lung vasculature. The pathology of CHD is characterized by plaque-like deposition of fibrous tissue on valvular cusps, leaflets, papillary muscles, chordae, and ventricular walls. Symptomatic CHD usually presents between 50 and 70 years of age, initially as dyspnea and fatigue. Echocardiography is the mainstay of imaging and demonstrates thickened right heart valves with limited mobility and regurgitation. Treatment focuses on control of the underlying carcinoid syndrome, targeting subsequent valvular heart disease and managing consequent heart failure. Surgical valve replacement and catheter-directed valve procedures may be effective for selected patients with CHD.

scholarly journals Post Radiotherapy Lesions of the Heart Valves. Principles of Diagnosis and Results of Treatment

Kardiologiia ◽  
2019 ◽  
Vol 59 (3) ◽  
pp. 36-42
Author(s):  
R. M. Muratov ◽  
S. I. Babenko ◽  
A. S. Sachkov ◽  
N. N. Soboleva ◽  
E. A. Andrianova
Keyword(s):  
Radiation Therapy ◽  
Heart Disease ◽  
Aortic Valve ◽  
Coronary Arteries ◽  
Heart Valves ◽  
Severe Heart Failure ◽  
Clinical Manifestations ◽  
Cardiac Complications ◽  
Valve Prosthesis ◽  
Thoracic Irradiation

Background. The use of radiation therapy for the treatment of tumors of the chest сan lead to the development of cardiac pathology, including that of the valves and coronary arteries. Study aim: to analyze the specifics of post-radiation lesions of the valvular apparatus and coronary arteries, and to assess the immediate results and risks of surgical correction of detected defects. Materials and  methods. In the Emergency department of surgery of acquired heart disease of A. N. Bakulev National Medical Research Center of Cardiovascular Surgery in the period from 2004 to 2017 were examined and operated 46 patients aged 35–81 years (mean age 56±12.4 years, 80 % women). The period from primary irradiation of the chest to surgical treatment of valvular pathology ranged from 4 to 40 years. Indications for thoracic radiotherapy were Hodgkin’s lymphoma in 23 patients (50 %), breast cancer – in 20 (43 %). Results. Stenosis of the aortic valve was the leading defect in 42 patients (91 %). According to coronary angiography, coronary artery disease was diagnosed in 31 patients (67 %). Isolated aortic valve prosthesis was performed in 14 (30 %) patients, other operations were combined. Hospital mortality was 11 % (5 patients). There were no deaths among patients who underwent radiation therapy after mastectomy. Main nonlethal complications were: pericardial effusion in 6 patients (13 %), hydrothorax requiring repeated pleural punctures in 5 patients (11 %). Conclusion. The variety of clinical manifestations of radiation heart disease and its progressive nature emphasize the need for long-term dynamic observation of patients after thoracic irradiation in order to timely identify the pathology and eliminate the risk of sudden cardiac complications and the development of severe heart failure decompensation.

scholarly journals P221 Carcinoid heart disease

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A Tyminska ◽  
D Kaczmarska-Turek ◽  
A Kaplon-Cieslicka ◽  
J Kochanowski ◽  
P Scislo ◽  
...  
Keyword(s):  
Heart Disease ◽  
Tricuspid Regurgitation ◽  
Carcinoid Syndrome ◽  
Pulmonary Valve ◽  
Pulmonary Stenosis ◽  
Pulmonary Regurgitation ◽  
Good Control ◽  
Carcinoid Heart Disease ◽  
Long Acting ◽  
Surgical Valve Replacement

Abstract Carcinoid heart disease is a rare disease, which develops in 20-50% of patients with carcinoid syndrome and is a main predictor of clinical outcome in those patients. Typical cardiac involvement of this disease presents as primary diseases of the tricuspid or pulmonary valves with a rare affection of left sided heart valves. The characteristic pathological findings are endocardial plaques of fibrous which may involve not only the valve leaflets, but also the subvalvar apparatus.Typical management of patients with this condition consist of the treatment of right heart failure (HF), pharmacotherapy to reduce the secretion of tumour products, and surgical valve replacement. Here we report a 56-year old male admitted to the Department of Endocrinology because of flushing with abdominal pain and diarrhea 2-3 times a week for 2 years. During hospitalization carcinoid syndrome with metastases to the liver and abdominal lymph nodes was diagnosed. Treatment with a long-acting somatostatin analog was initiated, resulting in a good control of the symptoms of the carcinoid syndrome. Trans-thoracic echocardiography revealed right atrial and right ventricular enlargement with degenerative lesions of tricuspid valve leaflets and its subvalvular apparatus, with leaflet stiffening, retraction and malcoaptation, resulting in severe tricuspid regurgitation (vena contracta [VC] width - 7 mm). Pulmonic valve was also involved with thickening of pulmonary valve cusps leading to mild pulmonary stenosis (peak gradient [PG] - 27 mmHg, mean gradient [MG] - 14 mmHg) and mild pulmonary regurgitation. Moreover, signs of pulmonary hypertension (with tricuspid regurgitation pressure gradient [TRPG] of 50 mmHg and estimated systolic pulmonary artery pressure [SPAP] of 50-55 mmHg) was observed. There were no signs of hemodynamically significant left-sided valve disease, nor of any abnormalities in segmental or global left ventricular function. After 12 months of treatment with a long-acting somatostatin analog, the patient was reassessed. Despite a good control of carcinoid syndrome symptoms and reduction in carcinoid syndrome marker (5-hydroxyindoloacetic acid), exacerbates the tricuspid regurgitation and worsens right HF was observed. Control echocardiographic examination showed significant progression of the pulmonary valve disease with severe pulmonary regurgitation (VC width - 10 mm) and mild pulmonary stenosis (PG 18 mmHg, MG 8 mmHg), a deterioration of tricuspid regurgitation (VC width - 11 mm, effective regurgitant orifice area - 0.94 cm2, regurgitant volume - 64 ml) with further enlargement of the right ventricle and right atrium, and with a consequent decrease in TRPG value (25 mmHg), despite well preserved right ventricular systolic function (TAPSE - 24 mm). Due to disease progression, treatment of HF and peptide receptor radionuclide therapy were initiated. The patient was presented for surgical valve replacement, however he did not agree to surgical treatment. Abstract P221 Figure

scholarly journals Carcinoid heart disease: relationship of circulating vasoactive substances to ultrasound-detectable cardiac abnormalities.

Circulation ◽  
1988 ◽  
Vol 77 (2) ◽  
pp. 264-269 ◽  
Author(s):  
L Lundin ◽  
I Norheim ◽  
J Landelius ◽  
K Oberg ◽  
E Theodorsson-Norheim
Keyword(s):  
Heart Disease ◽  
Carcinoid Heart Disease ◽  
Cardiac Abnormalities ◽  
Vasoactive Substances ◽  
Relationship Of

scholarly journals Transcatheter Pulmonary Valve Replacement in a Carcinoid Heart

2016 ◽  
Vol 43 (4) ◽  
pp. 341-344 ◽  
Author(s):  
Pranav Loyalka ◽  
Michael Schechter ◽  
Angelo Nascimbene ◽  
Ajay Sundara Raman ◽  
Cezar A. Ilieascu ◽  
...  
Keyword(s):  
Heart Disease ◽  
Valve Replacement ◽  
Pulmonary Valve ◽  
Open Heart Surgery ◽  
Pulmonary Valve Replacement ◽  
Carcinoid Heart Disease ◽  
Pulmonary Valve Stenosis ◽  
Percutaneous Valve Replacement ◽  
Surgical Valve Replacement ◽  
Percutaneous Valve

Carcinoid heart disease presents as right-sided heart failure attributable to the dysfunction of the tricuspid and pulmonary valves. Although surgical valve replacement is the mainstay of treatment when patients become symptomatic, it is associated with substantial perioperative mortality rates. We present a case of severe pulmonary valve stenosis secondary to carcinoid heart disease, treated successfully with percutaneous valve replacement. A 67-year-old man with severe pulmonary valve stenosis was referred to our center for pulmonary valve replacement. The patient had a history of metastatic neuroendocrine tumor of the small bowel with carcinoid syndrome, carcinoid heart disease, and tricuspid valve regurgitation previously treated with surgical valve replacement. Because of the patient's severe chronic obstructive pulmonary disease and hostile chest anatomy seen on a computed tomographic scan dating from previous cardiothoracic surgery, we considered off-label percutaneous valve replacement a viable alternative to open-heart surgery. A 29-mm Edwards Sapien XT valve was successfully deployed over the native pulmonary valve. There were no adverse sequelae after the procedure, and the patient was discharged from the hospital the next day. This case report shows that percutaneous valve replacement can be a valid option in carcinoid heart disease patients who are not amenable to surgical valve replacement.

scholarly journals Multimodality Imaging in Approach to A Patient with Carcinoid Heart Disease Involving Four Heart Valves

2021 ◽  
Author(s):  
Shirin Habibi khorasani ◽  
Mozhgan Parsaee ◽  
Niloufar Samiei ◽  
Mahshid Hesami ◽  
Feidoun Noohi ◽  
...  
Keyword(s):  
Heart Disease ◽  
Heart Valves ◽  
Carcinoid Syndrome ◽  
Multimodality Imaging ◽  
Rare Condition ◽  
Carcinoid Heart Disease ◽  
Complicated Form

Carcinoid heart disease is a rare condition that occurs in half of patients with carcinoid syndrome. The disease mostly affects right-sided heart valves; however, in 5-10%, it can also involve left-sided valves. This case illustrates the most complicated form of the disease with involvement of four heart valves.

scholarly journals P839 The lucky unlucky patient: a case report

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
S Habjan ◽  
D Cantisani ◽  
I S Scarfo` ◽  
V Avitabile ◽  
G Semeraro ◽  
...  
Keyword(s):  
Heart Disease ◽  
Neuroendocrine Tumor ◽  
Heart Valve ◽  
Heart Valves ◽  
Pulmonary Valve ◽  
Carcinoid Heart Disease ◽  
Left Heart ◽  
Surgical Closure ◽  
Hepatic Lesions ◽  
Valve Lesions

Abstract Introduction Carcinoid tumors are rare neuroendocrine malignancies arising from the neural crest amine precursor uptake decarboxylation cells and are most commonly (90%) located in the gastrointestinal system, the most malignant arising from the ileum. Approximately 50% of patients with carcinoid syndrome will develop carcinoid heart disease, which is commonly characterized by the thickening and retraction of the tricuspid ± the pulmonary valves resulting in significant regurgitation ± stenosis. Hepatic metastasis must be present for the carcinoid heart disease to develop. In order to develop left-sided heart valve lesions, communication between the left and right heart chambers must be present. Case presentation A 50-year-old female patient with a known neuroendocrine tumor of the ileum with hepatic and peritoneal metastasis presented at our facility for evaluation of the heart valves. She was diagnosed with the neuroendocrine tumor in 2017, a resection of the intestinal ileocecal segment and peritoneal nodules was done in the same year (stadium pT3N1M1b ki 67 2%), the tumor was angio and neuroinvasive. The disease was treated with somatostatin analogues with minimal regression of the disease. She started peptide receptor radionuclide therapy and completed 3 cycles. In October 2018 an increment of one of the hepatic lesions was documented, with other hepatic lesions remaining of stable dimensions. Due to the recent appearance of dyspnea in mild to moderate physical activities she was sent to our facility for echocardiography. The transthoracic and transesophageal echocardiography revealed severe tricuspid regurgitation with mild stenosis (tricuspid anatomic area 3.5 cm², max gradient 7 mmHg, mean gradient 4 mmHg) caused by thickened and retracted cusps with a significant coaptation deficit. Severe stenosis and insufficiency of the pulmonary valve was also noted (maximum gradient 22 mmHg, mean gradient 15 mmHg, pulmonary valve area 0.5 cm²), with a rapid deceleration regurgitation jet limited to protodiastole, caused by thickened and retracted pulmonary valve leaflets. The interatrial septum exhibited an aneurysm with a patent foramen ovale (PFO) and mild left-to-right shunt. Despite the shunt left-sided heart valves were not affected, therefore we recommended a replacement of the tricuspid and pulmonary valves with an intraoperative closure of PFO and intraoperative verification of the efficacy of the surgical closure. Conclusion Carcinoid heart disease is an important cardiac complication of the neuroendocrine neoplasms. Communication between the right and left heart chambers (for example a PFO) must be present for the development of left heart valve lesions. Our patient had a PFO with a mild left to right shunt, but fortunately no left sided lesions were found. Abstract P839 Figure. Carcinoid heart disease

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

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.

scholarly journals Heidinger syndrome - Carcinoid syndrome

2021 ◽  
Vol 12 (1) ◽  
pp. 041-046
Author(s):  
Ivaneta Dimitrova Yonceva ◽  
Denislav Emilov Biserov ◽  
Vasil Dimitrov Velchev ◽  
Vasil Atanasov Geguskov ◽  
Mariya Negrinova Negreva
Keyword(s):  
Heart Disease ◽  
Carcinoid Syndrome ◽  
Single Photon ◽  
Photon Emission ◽  
Fibrous Tissue ◽  
Clinical Results ◽  
Systemic Circulation ◽  
Tumor Formation ◽  
Emission Computed Tomography ◽  
Carcinoid Heart Disease

Carcinoid heart disease was first described in 1954. It develops in over 70% of carcinoid syndrome patients. Up to 20% of patients with carcinoid syndrome have pronounced carcinoid heart disease at diagnosis. Cardiac involvement is usually well tolerated by patients. Symptoms are associated with secretion of serotonin, tachykinin and other vasoactive substances in the systemic circulation. Deposition of fibrous tissue along the tricuspid annulus leads to ring constriction and is the basis of tricuspid stenosis. Primary insular carcinoid tumor of the ovary is a very rare tumor that accounts for less than 1% of all carcinoid cases. We present a case of a 62-year-old woman, demonstrating shortness of breath and fatigue, diarrhea, redness and progressive swelling on both lower legs. After single-photon emission computed tomography we found pathologically increased somatostatin expression in a tumor formation in the pelvis. After removal of the primary focus, the patient received a prosthetic tricuspid valve with good clinical results.

scholarly journals A clinical and echocardiographic case report of carcinoid-related valvular heart disease

2020 ◽  
Author(s):  
Ali J Ebrahimi ◽  
Peter Marogil ◽  
Gregory Von Mering ◽  
Mustafa Ahmed
Keyword(s):  
Heart Disease ◽  
Carcinoid Syndrome ◽  
Three Dimensional ◽  
Systemic Circulation ◽  
Neuroendocrine Neoplasms ◽  
Carcinoid Heart Disease ◽  
Malignant Carcinoid ◽  
Vasoactive Substances ◽  
Malignant Carcinoid Syndrome ◽  
Echocardiographic Images

Abstract Background Carcinoid syndrome is a rare disease caused by malignant neuroendocrine neoplasms. When vasoactive substances enter the systemic circulation, the triad of cutaneous flushing, bronchospasm, and diarrhoea often characterize carcinoid syndrome. Rarely, carcinoid syndrome can progress to involve the cardiac system, a condition known as carcinoid heart disease, often affecting right-sided valvular structures. Case summary Here, we present a case of malignant carcinoid syndrome with associated carcinoid heart disease in a 63-year-old female. The diagnosis of her dual regurgitant and stenotic valvular disease is detailed, with accompanying two- and three-dimensional echocardiographic images demonstrating the patient’s complex tricuspid dysfunction. Discussion Carcinoid heart disease encompasses a rare but important subset of valvular dysfunction caused by circulating vasoactive substances. Diagnosis utilizing serum studies, computed tomography scans, and echocardiography can help expedite the diagnosis and treatment of such rare conditions, and assist in the avoidance of complications. Despite its relatively well-recognized clinical presentation, carcinoid syndrome and its associated heart disease still remains a challenging condition to manage and treat, often requiring the input of several subspecialties to treat the condition appropriately.

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