Top

Journal of Medical Case Reports

Published in:

Open Access 01-12-2018 | Case report

Carcinoid heart disease revealed by cyanosis with both right and left valvular involvement: a case report

Authors: Khadija Khay, Salim Arous, Tarik Bentaoune, Abdenasser Drighil, Rachida Habbal

Published in: Journal of Medical Case Reports | Issue 1/2018

Abstract

Background

Carcinoid heart disease is a frequent complication of carcinoid syndrome. It is related to the release by the carcinoid tumor and/or its metastases of bioactive substances such as serotonin. It is characterized by right-sided valvular involvement and can lead to right-sided heart failure. It is a prognostic factor of carcinoid syndrome.

Case presentation

We report the case of a 53-year-old African woman with an endocrine tumor of her small intestine complicated by carcinoid heart disease, revealed by right-sided heart failure. The diagnosis was confirmed by transthoracic echocardiography, which showed a severe tricuspid regurgitation with a patent foramen ovale, and by increased serum chromogranin A and urinary 5-hydroxyindoleacetic acid.

Initially she was treated with medical therapy (furosemide and injection of somatostatin). Afterwards she was proposed for surgery. The evolution of her treatment was good.

Conclusions

Carcinoid syndrome is complicated in 60% of the cases of a heart disease, and is responsible for an important morbidity and mortality. The prognosis of patients with carcinoid heart disease has improved in recent years through somatostatin analogues and the cardiac surgery.

Yao JC, Hassan M, Phan A, et al. One hundred years after ‘carcinoid’: Epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol. 2008;26:3063–72.CrossRefPubMed

Grozinsky-Glasberg S, Grossman AB, Gross DJ. Carcinoid heart disease: from pathophysiology to treatment – ‘something in the way it moves’. Neuroendocrinology. 2015;101:263–73.CrossRefPubMed

Hassan SA, Banchs J, Iliescu C, Dasari A, Lopez-Mattei J, Yusuf SW. Carcinoid heart disease. Heart. 2017;103:1488–95.CrossRefPubMed

Bhattacharyya S, Toumpanakis C, Chilkunda D, et al. Risk factors for the development and progression of carcinoid heart disease. Am J Cardiol. 2011;107:1221–6.CrossRefPubMed

Bertin N, Favretto S, Pelizzo F, et al. Carcinoid Heart Disease: Starting From Heart Failure. J Investig Med High Impact Case Rep. 2017;5(2). https://doi.org/10.1177/2324709617713511.

Pellikka PA, Tajik AJ, Khandheria BK, et al. Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients. Circulation. 1993;87:1188–96.CrossRefPubMed

Pandya UH, Pellikka PA, Enriquez-Sarano M, et al. Metastatic carcinoid tumor to the heart: Echocardiographic-pathologic study of 11 patients. J Am Coll Cardiol. 2002;40:1328–32.CrossRefPubMed

Dero I, De Pauw M, Borbath I, et al. Carcinoid heart disease—a hidden complication of neuroendocrine tumours. Acta Gastroenterol Belg. 2009;72(1):34–8.PubMed

Palaniswamy C, Frishman WH, Aronow WS. Carcinoid Heart Disease. Cardiol Rev. 2012;20(4):167–76.CrossRefPubMed

10.

Plöckinger U, Gustafsson B, Ivan D, et al. ENETS consensus guidelines for the standards of care in neuroendocrine tumors: Echocardiography. Neuroendocrinology. 2009;90:190–3.CrossRefPubMed

11.

Bhattacharyya S, Toumpanakis C, Caplin ME, et al. Usefulness of N-terminal pro-brain Natriuretic peptide as a Biomarker of the presence of Carcinoid heart disease. Am J Cardiol. 2008;102:938–42.CrossRefPubMed

12.

Bhattacharyya S, Toumpanakis C, Caplin ME, et al. Analysis of 150 patients with carcinoid syndrome seen in a single year at one institution in the first decade of the twenty-first century. Am J Cardiol. 2008;101:378–81.CrossRefPubMed

13.

Luis SA, Pellikka PA. Carcinoid heart disease: diagnosis and management. Best Pract Res Clin Endocrinol Metab. 2016;30:149–58.CrossRefPubMed

14.

Poyet R, et al. Right ventricular failure as the presenting manifestation of a carcinoid syndrome. Rev Med Interne. 2009;30:81–4.CrossRefPubMed

15.

Haugaa KH, Bergestuen DS, Sahakyan LG, et al. Evaluation of right ventricular dysfunction by myocardial strain echocardiography in patients with intestinal carcinoid disease. J Am Soc Echocardiogr. 2011;24:644–50.CrossRefPubMed

16.

Hart EA, Meijs TA, Meijer RCA, et al. Carcinoid heart disease: a guide for screening and timing of surgical intervention. Neth Hear J. 2017;25(9):471–8.CrossRef

17.

Dobson R, Burgess MI, Pritchard DM, et al. The clinical presentation and management of carcinoid heart disease. Int J Cardiol. 2014;173:29–32.CrossRefPubMed

18.

Wolin EM, Jarzab B, Eriksson B, et al. Phase III study of pasireotide long-acting release in patients with metastatic neuroendocrine tumors and carcinoid symptoms refractory to available somatostatin analogues. Drug Des Devel Ther. 2015;9:5075–86.CrossRefPubMedPubMedCentral

19.

Molina-Cerrillo J, Alonso-Gordoa T, Martínez-Sáez O, et al. Inhibition of Peripheral Synthesis of Serotonin as a New Target in Neuroendocrine Tumors. Oncologist. 2016;21(6):701–7.CrossRefPubMedPubMedCentral

20.

Davar J, Connolly HM, Caplin ME, et al. Diagnosing and managing carcinoid heart disease in patients with neuroendocrine tumors: an expert statement. J Am Coll Cardiol. 2017;69(10):1288–304.CrossRefPubMed

21.

Kunz PL. Carcinoid and neuroendocrine tumors: building on success. J Clin Oncol. 2015;33:1855–63.CrossRefPubMed

22.

Edwards NC, Yuan M, Nolan O, et al. Effect of valvular surgery in carcinoid heart disease: An observational cohort study. J Clin Endocrinol Metab. 2016;101:183–90.CrossRefPubMed

23.

Raja SG, Bhattacharyya S, Davar J, et al. Surgery for carcinoid heart disease: current outcomes, concerns and controversies. Future Cardiol. 2010;6:647–55.CrossRefPubMed

24.

Laule M, Pschowski R, Pape UF, et al. Staged catheter-based valve treatment of severe carcinoid heart disease. Neuroendocrinology. 2016;103:259–62.CrossRefPubMed

25.

Møller JE, et al. Prognosis of carcinoid heart disease: analysis of 200 cases over two decades. Circulation. 2005;112(21):3320–7. https://doi.org/10.1161/CIRCULATIONAHA.105.553750.

26.

Mota JM, Sousa LG, Riechelmann RP. Complications from carcinoid syndrome: review of the current evidence. Ecancermedicalscience. 2016;10:1–17.CrossRef

Title: Carcinoid heart disease revealed by cyanosis with both right and left valvular involvement: a case report
Authors: Khadija Khay
Salim Arous
Tarik Bentaoune
Abdenasser Drighil
Rachida Habbal
Publication date: 01-12-2018
Publisher: BioMed Central
Published in: Journal of Medical Case Reports / Issue 1/2018
Electronic ISSN: 1752-1947
DOI: https://doi.org/10.1186/s13256-018-1574-6

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Carcinoid heart disease revealed by cyanosis with both right and left valvular involvement: a case report

Abstract

Background

Case presentation

Conclusions

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Background

Case presentation

Conclusions

Please log in to get access to this content

Other articles of this Issue 1/2018

En bloc corpectomy for late gastrointestinal stromal tumor metastasis: a case report and review of the literature

A rare presentation of pheochromocytoma in pregnancy: a case report

Cytomegalovirus reactivation in a critically ill patient: a case report

Acquired partial lipoatrophy as graft-versus-host disease and treatment with metreleptin: two case reports

Synchronous double primary hepatic cancer consisting of hepatocellular carcinoma and cholangiolocellular carcinoma: a case report

Transient severe distributive shock due to early dumping syndrome: a case report