Is pulmonary hypertension a feature of carcinoid heart disease?

Introduction

Carcinoid symptoms typically occur between the fifth and seventh decades of life with a mean age of 55–60 years.1-4 Carcinoid heart disease is reported to be a rare but important cause of right sided valvular heart diseases and its occurrence is associated with significant morbidity and mortality due to right heart failure. Cardiac involvement occurs in at least 50% of patients with florid carcinoid symptoms. In patients with carcinoid heart disease, right atrial and right ventricular enlargement is present in up to 90% of cases and ventricular septal wall motion abnormalities are seen in almost half of the cases.5 The tricuspid valve leaflets and subvalvar structures are often thickened, shortened and retracted, leading to incomplete coaptation and usually moderate or severe tricuspid regurgitation (Figures 1 and 2). The continuous wave Doppler profile of tricuspid regurgitation shows a characteristic dagger shaped spectrum with an early peak pressure and rapid decline.6, 7 The pulmonary valve may also be thickened and retracted, leading to pulmonary insufficiency and less commonly, pulmonary stenosis. However, the occurrence of pulmonary hypertension (PH) in carcinoid heart diseases is quite rare, unless the patient presents with left heart valve involvement or intracardiac shunt.

Case presentation

The clinical case is a 78 year old African male who presented with constitutional symptoms, including associated lower limbs and abdominal swelling for a period of three to four months. Additionally, he reported a history of lower abdominal mass and skin hyperpigmentation, which had progressively gotten worse over the same period of time. At time of presentation, he was a pensioner and previously worked as a domestic worker. He had no significant smoking history, nor prior surgical history. His clinical examination revealed features of pulmonary hypertension (PH), severe tricuspid regurgitation and right heart failure.

Differential diagnosis

Based on his initial presentation, he was evaluated for constrictive pericarditis, which was subsequently ruled out. Cor-pulmonale was fully entertained, however carcinoid syndrome and carcinoid heart disease were suspected during echocardiographic assessment. His echocardiographic pictures are shown in Figures 1 and 2.

 

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Figure 1: An apical four chamber view showing tricuspid leaflets which are thickened and retracted as shown in the above diagram. The right ventricle and atrium are dilated.

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Figure 2: Parasternal short axis view at the level of the aortic valve. This show colour Doppler with free flow through the tricuspid valve during systole. The right ventricle and atrium are dilated

Further Investigations

His 5-hydroxyindoleacetic acid was elevated more than 10 times the upper limit of normal (ULN) and octreotide scan of his liver and intestines was positive. In addition to his echocardiographic images below, his pulmonary arterial systolic pressure (PAS) was 65 mmHg with no significant left sided valvular heart disease. The transoesophageal echocardiography revealed no intracardiac shunt or significant left sided valve disease. His chest radiography revealed mild hyperinflation with clear ling fields. The CT scan of his abdomen revealed multiple lesions in the liver and ileum.

Patients’ management and outcome

Unfortunately the patient refused further management including surgery, and he demised a year later.

Discussion

 Even though this patient was diagnosed with having carcinoid syndrome with cardiac involvement, his initial presentation was quite intriguing. The diagnosis of carcinoid syndrome was mainly suspected during work-ups, including transthoracic echocardiography. The echocardiographic features are classical of carcinoid heart diseases; however, the presence of severe pulmonary hypertension could not be easily explained as his clinical and echocardiographic finding ruled out significant left sided heart disease. Additionally, there was no intracardiac shunting noted during neither transthoracic nor transoesophageal echocardiographic evaluations. Even though the occurrence of PH in carcinoid syndrome is thought to be rare, one small study reported four of sixteen patients who presented with mild PH8,9, who were also diagnosed with metastatic gastrointestinal (GIT) carcinoid disease without left sided heart disease or documented intracardiac shunt. The postulate to this is that serotonin is normally taken up by endothetial cells and platelets, and if this process is impaired or overwhelmed then PH can develop.10

 

 

References

 1. Westberg G, Wangberg B, Ahlman H, Bergh CH, Beckman-Suurkula M, Caidahl K. Prediction of prognosis by echocardiography in patients with midgut carcinoid syndrome. Br J Surg. 2001;88:865-872

2. Moller JE, Connolly HM, Rubin J, Seward JB, Modesto K, Pellikka PA. Factors associated with progression of carcinoid heart disease. N Engl J Med. 2003;348:1005-1015

3. Connolly HM, Schaff HV, Mullany CJ, Rubin J, Abel MD, Pellikka PA. Surgical management of left-sided carcinoid heart disease. Circulation. 2001;104:36-40

4. Zuetenhorst JM, Bonfrer JM, Korse CM, Bakker R, van Tinteren H, Taal BG. Carcinoid heart disease: The role of urinary 5-hydroxyindoleacetic acid excretion and plasma levels of atrial natriuretic peptide, transforming growth factor-beta and fibroblast growth factor. Cancer. 2003;97:1609-1615

5. Pellikka PA, Tajik AJ, Khandheria BK, Seward JB, Callahan JA, Pitot HC, Kvols LK. Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients. Circulation. 1993;87:1188-1196

6. Bhattacharyya S, Davar J, Dreyfus G, Caplin ME. Carcinoid heart disease. Circulation. 2007;116:2860-2865

7. Fox DJ, Khattar RS. Carcinoid heart disease: Presentation, diagnosis, and management. Heart. 2004;90:1224- 1228

8. Jacobsen MB, Nitter-Hauge S, Bryde PE, Hanssen LE. Cardiac manifestations in mid-gut carcinoid disease. Eur Heart J. 1995;16:263-268

9. Tornebrandt K, Eskilsson J, Nobin A. Heart involvement in metastatic carcinoid disease. Clin Cardiol. 1986;9:13-19

10. Herve P, Launay JM, Scrobohaci ML, Brenot F, Simonneau G, Petitpretz P, Poubeau P, Cerrina J, Duroux P, Drouet L. Increased plasma serotonin in primary pulmonary hypertension. Am J Med. 1995;99:249-254

Topics

Endothelin and Endothelium & Epithelium and Epithelial Transport
Pulmonary Hypertension

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PVRI Chronicle Vol 1: Issue 2 cover image

December 2014

PVRI Chronicle Vol 1: Issue 2

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