scholarly journals Tricuspid and pulmonary valve involvement in carcinoid heart disease

2009 ◽  
Vol 102 (6-7) ◽  
pp. 591-592
Author(s):  
Stéphane Ederhy ◽  
Franck Engel ◽  
Ariel Cohen
Author(s):  
Michele Flagiello ◽  
Matteo Pozzi ◽  
Laurent Francois ◽  
Ahmed Al Harthy ◽  
Julien Forestier ◽  
...  

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
J Bergsten ◽  
A Albage ◽  
F Flachskampf

Abstract Background Valvular surgery for carcinoid heart disease (CaHD) has been found to improve long-term prognosis. Experience is limited and questions about single versus double-valve surgery as well as selection of prosthetic valves remain under debate. Purpose We reviewed the surgical experience after valvular surgery for CaHD in our institution, which is a national referral center. Methods We reviewed clinical records and echocardiograms. Perioperative death was defined as mortality within 30 days of operation. Results Sixty consecutive patients (32 men, 62±9 years) underwent valve surgery between 1986 and 2019, of whom 59/60 had an intestinal carcinoid disease with hepatic metastases. Mean time from diagnosis to heart surgery was 3.8±3.7 years. Preoperatively, 6 patients were in NYHA class I/II, the others were in class III or IV. All 60 surgical procedures involved tricuspid valve replacement (TVR). In 47 cases, the pulmonary valve was treated surgically: Early in the series, five patients underwent pulmonary valvotomy or commissurotomy, and more recently, 42 patients received pulmonary valve replacement (PVR). All valves were replaced with bioprostheses, except for one pulmonary homograft. Concomitant significant aortic and mitral regurgitation occurred in 2 patients who received quadruple valve replacement. Two re-operations were performed due to degeneration of bioprostheses. Mean hospital stay was 13±9 days. Overall 30-day mortality was 11.6% (n=7) but was reduced to 8.3% in the last decade. The overall median survival was 2.2 years. Maximal survival free of reoperation was 18 years, and maximal survival for a single patient was 21 years. Median survival for combined TVR and PVR was significantly better than for single TVR or TVR combined with pulmonary valvotomy (3.0 years vs. 0.94 years, respectively, p=0.02; see Figure). Preoperatively, left ventricular ejection fraction (EF) was normal in 83%. Severe tricuspid regurgitation was always present. The right ventricle (RV) was dilated (RVD1 49 mm ± 5 mm) as well as the right atrium (60±16 ml/m2). The TAPSE was in normal range (17 to 29 mm). Pulmonary regurgitation was mild, moderate, severe, or indeterminate in 6%, 26%, 51%, and 17% respectively. In 17% the transpulmonary velocity was >2.5 m/s, indicating stenosis. On last available postoperative echo (mean time 2.1 years) EF was found unchanged. RV diameter was nearly normalized (RVD1 41 mm ± 5 mm). TAPSE was reduced (12 mm ± 4 mm). No significant regurgitation was detected in any TVR. There was significant stenosis (mean gradient >5 mmHg) in 23% of the TVR. No regurgitation of significance was detected in any PVR. A mean gradient >20 mmHg was found in 11% of the PVR. Conclusions While perioperative risk of valve replacement in CaHD remains substantial, this study shows relatively favourable outcomes of surgical valve replacement with bioprostheses. PVR together with TVR had better outcomes than those undergoing TVR only. Figure. Survival by Surgical Treatment. Funding Acknowledgement Type of funding source: Public hospital(s). Main funding source(s): Department of Medical Sciences, Uppsala University. Department of Surgical Sciences, Uppsala University


2018 ◽  
Vol 27 ◽  
pp. S524-S525
Author(s):  
Oliver Pumphrey ◽  
David McCormack ◽  
Damian Gimpel ◽  
Adam El-Gamel

2020 ◽  
Vol 13 (9) ◽  
pp. e235190
Author(s):  
Yash Paul Sharma ◽  
Prashant Kumar Panda ◽  
Lipi Uppal ◽  
Uma Debi

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.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A L Gamarra Lobato ◽  
A M Rojas ◽  
A Cecconi ◽  
L Dominguez ◽  
A Benedicto ◽  
...  

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


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A Tyminska ◽  
D Kaczmarska-Turek ◽  
A Kaplon-Cieslicka ◽  
J Kochanowski ◽  
P Scislo ◽  
...  

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


2016 ◽  
Vol 43 (4) ◽  
pp. 341-344 ◽  
Author(s):  
Pranav Loyalka ◽  
Michael Schechter ◽  
Angelo Nascimbene ◽  
Ajay Sundara Raman ◽  
Cezar A. Ilieascu ◽  
...  

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.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
S Habjan ◽  
D Cantisani ◽  
I S Scarfo` ◽  
V Avitabile ◽  
G Semeraro ◽  
...  

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


Sign in / Sign up

Export Citation Format

Share Document