British Heart Valve Society
Carcinoid Heart Disease
Dr C Hayward MB BChir MRCP , Dr S Bhattacharyya MD MRCP, Dr J Davar MD PhD
Royal Free Hospital, London, UK
Case Presentation
Clinical History
• 60 year old female.
• 6 month history of flushing, diarrhoea, fatigue and
dyspnoea on exertion. NYHA Class III at presentation.
Investigations
• CT abdomen: multiple liver metastases and a small bowel
mesenteric mass. Liver Biopsy: consistent with low grade
carcinoid tumour.
• 24 hour Urinary 5-HIAA: 800µmol/24 hours.
Cardiac Investigations
• ECG – sinus tachycardia. Normal axis.
• CXR – Cardiothoracic ratio > 50%.
• Echocardiogram:
– Right Ventricle: dilated and mildly impaired (TAPSE
13cm).
– Tricuspid Valve: severe “free flowing” tricuspid
regurgitation.
– Pulmonary Valve: severe pulmonary regurgitation,
moderate pulmonary stenosis.
– NT-proBNP: 700 pg/ml.
Management
Medical
•Reduction of peripheral oedema with diuretics.
Valve Surgery
•Replacement of tricuspid and pulmonary valve:
Pulmonary homograft.
Pericardial tissue valve – tricuspid valve.
Length of hospital stay 5 days. Required permanent
pacemaker for complete heart block.
Outcome 6 months post surgery
•Diuretics weaned off.
•Functional NYHA Class I. Climb > 5 flights of stairs.
Clinical Manifestations
• Carcinoid syndrome consists of a triad: flushing, diarrhoea
and bronchospasm.
• Between 20-50% of all patients with carcinoid syndrome
will develop carcinoid heart disease.
• Vasoactive substances such as 5-hydroxytryptamine
produced by neoplastic cells are able to travel to the right
heart via the hepatic vein/IVC and are thought to be
responsible for deposition of endocardial plaques of fibrous
tissue.
• Classically patients develop signs and symptoms of right
heart failure: fatigue, oedema and ascites.
Pathology – “Carcinoid Plaque”
• Right-sided lesions more
common than left.
• Preferential right-sided
involvement due to inactivation
of vasoactive substances by
lungs.
• 5–10% have left-sided valvular
pathology due to either high
tumour load, bronchial
carcinoid or patent foramen
ovale.
• Plaque - composed of smooth
muscle cells + myofibroblasts
forming white fibrous layer
(arrow) lining endocardial
surface of cardiac valves
superficial to normal valve
Echocardiographic Features
– Tricuspid Valve
• Typically thickened,
retracted, valve leaflets.
Leaflets do not co-apt
(arrow).
• Anatomical features leads
to predominantly tricuspid
regurgitation (TR).
• Classical “Dagger” shaped
Doppler profile of severe
TR (arrow).
Echocardiographic Features – Pulmonary
Valve
• Fixed, thickened cusps
(arrow).
• Non-coaptation of cusps (*).
• Predominantly pulmonary
stenosis with varying degrees
of regurgitation (arrow).
Biochemical Markers
• Elevated urinary 5-hydroxyindolacetic acid is a highly
sensitive but poorly specific maker of carcinoid heart
disease.
• NT-proBNP > 260pg/ml has greater than 90% sensitivity
and negative predictive value for significant carcinoid
heart disease. This may allow its use as a screening
test.
• NT-proBNP also correlated with disease severity and
NYHA Class.
Management
Medical Management
•Poor outcome when
managed medically.
•3 year survival 68% without
cardiac involvement
compared to 31% with
cardiac involvement.
•Diuretics mainstay of
therapy.
Valve Surgery
•High peri-operative risk (10% 20% depending on institution).
•Valve replacement improves
symptom status (functional
NYHA Class).
•Emerging data suggest may
improve prognosis.
Conclusions
• Carcinoid heart disease = common complication of
carcinoid syndrome but is a rare cause of all acquired
valvular heart disease
• 5-HT is produced by metastatic tumour cells in the liver →
deposition of endocardial plaques.
• Right sided valvular dysfunction is common and presents
with characteristic echocardiographic appearances. Left
sided valve lesions in 5-10% of cases of carcinoid heart
disease.
• Medical management alone is associated with poor
survival.
• Valve surgery improves symptoms and may improve
prognosis.
Further Reading
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Bhattacharyya S, Davar J, Dreyfus G, Caplin ME. Carcinoid Heart Disease.
Circulation 2007; 116:2860-2865.
Lundin L, Norheim I, Landelius J, Oberg K, Theodorsson-Norheim E.
Relationship of circulating vasoactive substances to ultrasound detectable
cardiac abnormalities. Circulation 1988;77:264-269.
Bhattacharyya S, Toumpanakis D, Burke M, Taylor AM, Caplin ME, Davar J.
Features of carcinoid heart disease identified by 2- and 3-dimensional
echocardiography and cardiac MRI. Circ Cardiovasc Imaging 2010:3:103111.
Korse CM, Taal BG, de Groot CA, Bakker RH, Bonfrer JM. ChromograninA and N-terminal pro-brain natriuretic peptide: an excellent pair of
biomarkers for diagnostics in patients with neuroendocrine tumor. J Clin
Oncol. 2009;27:4293-4299.
Bhattacharyya S, Toumpanakis C, Caplin M, Davar J. Usefulness of NTerminal Brain Natriuretic Peptide As A Biomarker Of The Presence Of
Carcinoid Heart Disease. American Journal of Cardiology 2008;102:938942.
Moller JE, Pellikka PA, Bernheim AM, Schaff HV, Rubin J, Connolly HM.
Prognosis of carcinoid heart disease: An analysis of 200 cases over two
decades. Circulation 2005;112:3320-3327.