Cardiovascular Journal of Africa: Vol 29 No 4 (July/August 2018)

67 / 76

CARDIOVASCULAR JOURNAL OF AFRICA • Volume 29, No 4, July/August 2018 AFRICA e1
Cases in a series of carcinoid syndrome and carcinoid
heart disease
Mamotabo R Matshela
Abstract
Although carcinoid syndrome is regarded as a rare entity,
carcinoid patients with evidence of cardiac involvement show
a markedly reduced survival time. Patients with advanced
signs of right-sided heart failure represent a subgroup at
particularly high risk. Echocardiography remains the gold
standard to diagnose or confirm structural cardiac involve-
ment in patients with underlying carcinoid disease. This is
the notion that propelled us to report on cases of carcinoid
syndrome with cardiac involvement. We also review carcinoid
syndrome and carcinoid heart disease, and challenges regard-
ing the diagnosis and management of carcinoid heart disease.
Keywords: carcinoid syndrome, carcinoid heart disease, 5-hydroxy-
indoleacetic acid
Submitted 20/9/17, accepted 24/6/18
Cardiovasc J Afr 2018; 29: e1–e7
DOI: 10.5830/CVJA-2018-040
Carcinoid heart disease (CHD) has previously been reported
as a rare form of valvular heart disease, mostly associated with
metastatic carcinoid tumour. Most systemic manifestations
of carcinoid tumours are related to the release of vasoactive
substances from the tumour, including serotonin and other
circulating humoral substances. Although CHD is presumed
to be rare, we use this opportunity to report on a collection of
interesting patients with CHD, who presented differently and
posed challenges in their short- and long-term management.
Case report
Seven patients with carcinoid and cardiac involvement are
summarised in Table 1. However, two of these patients with
classical echocardiographic images were extensively reviewed
and are reported on below.
Patient 1: The first patient was a 78-year-old African male
who first presented to his local hospital with constitutional
symptoms and abdominal distension, which progressed over
a four-month period. He was later referred to us for further
management. This was his first-ever consultation and admission
to any medical facility. There was no past surgical or medical
history of note and no history of illicit drug use. However he
was an occasional drinker and a smoker with a three-pack year
history. He had no family history of note.
Physical examination revealed skin hyperpigmentation, lower
abdominal mass and features of severe tricuspid regurgitation
with right heart failure. The rest of his clinical examination was
unremarkable. Carcinoid syndrome with CHD was suspected
during routine transthoracic echocardiographic assessment, and
his images are shown in Fig. 1.
His biochemical laboratory results revealed a markedly
elevated serum 5-hydroxyindoleacetic acid (HIAA), which was
more than 10 times the upper limit of normal. An octreotide
scan was positive for the primary lesion localised around the peri-
prostatic area. Additional blood results revealed normal renal
and hepatic function. His full blood count revealed features in
University of KwaZulu-Natal, Durban, South Africa;
Mediclinic Heart Hospital, Pretoria, South Africa; London
School of Economics and Political Science, London, UK
Mamotabo R Matshela, MB ChB, PhD, FESC, matshela.mamota-
,
Case Report
Table 1. Summary of patients with confirmed carcinoid syndrome and carcinoid heart disease
Parameters Patient 1 Patient 2 Patient 3 Patients 4 Patient 5 Patient 6 Patient 7
Age, years 78 32 81 55 63 71 68
Gender Male Male Female Female Male Male female
Urinary 5-HIAA, µmol/24 h 406–548 465–269 < 300 < 200 < 200 < 200 < 200
Echo features Restrictive TV
leaflet motion,
torrential TR
Restrictive TV
and PV leaflets
motion
Moderately
restrictive TV
leaflet motion
Mildly restrictive
TV leaflet motion
Moderately
restrictive TV
leaflet motion
Mildly restrictive
TV leaflet motion
Mildly restrictive
TV leaflet motion
Octreotide scan + + + + + + +
Hepatic lesions on CT Multiple Multiple One None Multiple None None
Management Declined surgery TVR Medical
management
Medical
management
Medical
management
Medical
management
Medical
management
Follow up Died Improved Died Improved Improved Improved Improved
5-HIAA, 5-hydroxyindole acetic acid; TV, tricuspid valve; TR: tricuspid regurgitation, TVR, tricuspid valve replacement; PV, pulmonary valve.