british heart valve society carcinoid heart disease dr c hayward mb bchir mrcp, dr s bhattacharyya...
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British Heart Valve SocietyCarcinoid Heart Disease
Dr C Hayward MB BChir MRCP , Dr S Bhattacharyya MD MRCP, Dr J Davar MD PhD
Royal Free Hospital, London, UK
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.
Case Presentation
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.
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.
Management
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
1.Bhattacharyya S, Davar J, Dreyfus G, Caplin ME. Carcinoid Heart Disease. Circulation 2007; 116:2860-2865. 2.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. 3.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 2010:3:103-111.4.Korse CM, Taal BG, de Groot CA, Bakker RH, Bonfrer JM. Chromogranin-A 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. 5.Bhattacharyya S, Toumpanakis C, Caplin M, Davar J. Usefulness of N-Terminal Brain Natriuretic Peptide As A Biomarker Of The Presence Of Carcinoid Heart Disease. American Journal of Cardiology 2008;102:938-942.6.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.
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