Carcinoid Tumours and Carcinoid Syndrome What we know What ...
Carcinoid - American Society of Echocardiography · Carcinoid heart disease is NOT a cardiac...
Transcript of Carcinoid - American Society of Echocardiography · Carcinoid heart disease is NOT a cardiac...
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2/11/2016NYU Leon H. Charney Division of Cardiology
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2016 ASE State of the Art Echocardiography Course | Tucson, AZ
CarcinoidSunday, February 14, 2016 | 7:00 – 7:15 PM | 15 min
M U H A M E D S A R I Ć , M D , P H D
D i r e c t o r o f E c h o c a r d i o g r a p h y L a b
D i r e c t o r o f O p e r a t i o n s , N o n i n v a s i v e C a r d i o l o g y
A s s o c i a t e P r o f e s s o r o f M e d i c i n e
N e w Y o r k U n i v e r s i t y L a n g o n e M e d i c a l C e n t e r
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Speakers Bureau
Philips, Medtronic
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Disclosures
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Siegfried Oberndorfer (1876 – 1944)
German pathologist
In 1907, Siegfried Oberndorfer coined the term Karzinoide(based on καρκινώδης , later anglicized as carcinoid)
Carcinoid = ‘carcinoma like’
Histologically, a malignant tumor…
… but clinically often behaves like a benign nonmetastatic tumor
CARCINOID TUMOR
Karzinoide Tumoren des Dünndarmes. [Carcinoid tumors of the small intestine]
Frankfurter Zeitschrift für Pathologie 1907;1:426-429.
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CARCINOID TUMOR VS. CARCINOID HEART DISEASE
What’s the difference?
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Carcinoid of the small intestine
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CARCINOID TUMOR
• Malignant tumor of neuroendocrine cells in the gut or bronchial tree
• Slow growing and occasionally metastatic
• Secretes vasoactive substances & hormones including serotonin (5-HT) resulting in carcinoid syndrome:• Facial flushing• Diarrhea• Carcinoid heart disease
What portion of the heart gets involved depends on the primary tumor’s location:
• Intestinal carcinoid >> Preferential RIGHT heart involvement• Bronchial carcinoid >> Preferential LEFT heart involvement
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CARCINOID HEART DISEASE
Paraneoplastic manifestation of a carcinoid tumor• Mural plaques• Valvulitis >> Stenosis + Regurgitation
Carcinoid heart disease is NOT a cardiac malignancy.
Carcinoid heart disease is a paraneoplastic syndromecaused by tumor-derived vasoactive substances.
Because intestinal carcinoid are more common than bronchial ones, carcinoid disease involves primarily the
right heart.
Carcinoid heart disease is often associated with liver metastases of an intestinal carcinoid.
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CARCINOID HEART DISEASE
Case #1
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CLINICAL PRESENTATION
56-year-old previously healthy man• Presented with right upper quadrant pain• Referred for abdominal CT
ABDOMINAL CT
Multiple liver metastases of an unknown primary tumor
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TRANSTHORACIC ECHOCARDIOGRAM | APICAL 4-CHAMBER VIEW
Dilated RV
Dilated RA
Noncoapting Tricuspid Valve
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TRANSTHORACIC ECHOCARDIOGRAM | PARASTERNAL SHORT-AXIS VIEW
Dilated RV
Dilated RA
AV
LA
Noncoapting Tricuspid Valve
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TRANSTHORACIC ECHOCARDIOGRAM | PARASTERNAL SHORT-AXIS VIEW
Dilated RV
Dilated RA
AV
LA
Severe Tricuspid Regurgitation
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TRANSTHORACIC ECHOCARDIOGRAM | CW DOPPLER OF TRICUSPID VALVE
TR JETLow velocity
Triangular shape
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TRANSTHORACIC ECHOCARDIOGRAM | PULMONIC VALVE
RV
AV
Severe Pulmonic Regurgitation
PA
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Systole
Diastole
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TRANSTHORACIC ECHOCARDIOGRAM | PULMONIC VALVE
PULMONIC REGURGITATION JETRapidly decelerating
Triangular shape
RV
AVPA
PULMONIC VALVEFrozen in semi-
open, semi-closed position
To-and-Fro Flow
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CARCINOID HEART DISEASE
Case #2
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CLINICAL PRESENTATION
71-year-old female with 8-year history of metastatic carcinoid
OCTREOTIDESomatostatin analog used to
counteract effects of carcinoid-derived hormones
• Severe abdominal pain & increased diarrhea x 2 weeks
• On somatostatin analog for many years
• For the last 3 years, she has been taking tincture of opium for her diarrhea with relatively good control; however, she stopped 4 days ago because she developed severe right upper quadrant pain when she took it.
• Since then, she has been taking at least 12 Lomotilantidiarrheal pills with poor diarrhea control.
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TRANSTHORACIC ECHOCARDIOGRAM | RV INFLOW VIEW
Dilated RV
Dilated RA
TRICUSPID VALVEFrozen in semi-open, semi-closed position
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TRANSTHORACIC ECHOCARDIOGRAM | RV INFLOW VIEW
Dilated RV
Dilated RA
SEVERETRICUSPID
REGURGITATION
--------------------------Laminar flow
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TRANSTHORACIC ECHOCARDIOGRAM | CW DOPPLER OF TRICUSPID VALVE
TRICUSPID REGURGITATION JETLow velocity
Triangular shape
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TRANSTHORACIC ECHOCARDIOGRAM | CW DOPPLER OF PULMONIC VALVE
PULMONIC REGURGITATION JETRapidly decelerating
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CARCINOID HEART DISEASE
Case #3
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CLINICAL PRESENTATION
57-year-old female with shortness of breath over the past 6 weeks.
• Previously unlimited exercise tolerance
• Progressive bilateral leg edema, increasing abdominal girth and loose bowel movement.
• Recently noticed intermittent flushing and shivering without fever
OCTREOTIDE ABDOMINAL CT SCANMultiple foci of uptake within the liver and in a calcified
mesenteric mass compatible with octreotide avid tumors such as carcinoid.
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24-HOUR URINE TEST
Increased urinary excretion of serotonin metabolites,consistent with carcinoid syndrome.
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TRANSTHORACIC ECHOCARDIOGRAM
Heart
LIVERCarcinoid Metastases
TTE showed severe tricuspid and pulmonic regurgitation… and something else in subcostal view
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LIVER BIOPSY
CARCINOID TUMORMetastatic well differentiated
neuroendocrine tumor
CARCINOID TUMORPositive staining for chromagranin
consistent with carcinoid
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CARCINOID HEART DISEASE
Case #4
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CLINICAL PRESENTATION
64-year-old men with recent weight loss and bilateral lower extremity edema
• A year ago, he noticed some flushing and rosy appearance to his cheeks• Six months ago he started having diarrhea • Abdominal MRI
• Mesenteric soft tissue mass • Multiple hepatic metastases
• Liver biopsy • Carcinoid tumor
MRI | MESENTERIC CARCINOID MRI | INNUMERABLE LIVER METASTASES
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3D TRANSTHORACIC ECHOCARDIOGRAM | BIPLANE VIEW
Dilated RV
Dilated RA
Frozen Tricuspid Valve
Dilated RA
Dilated RV
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TRANSTHORACIC ECHOCARDIOGRAM | APICAL 4-CHAMBER VIEW
Dilated RV
Dilated RA
Severe Tricuspid
Regurgitation
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TRANSTHORACIC ECHOCARDIOGRAM | SUBCOSTAL VIEW
Dilated RA
Hepatic Vein
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TRANSTHORACIC ECHOCARDIOGRAM | HEPATIC VEIN
SYSTOLIC FLOW REVERSAL(sign of severe TR)
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3D TRANSTHORACIC ECHOCARDIOGRAM | LV PERSPECTIVE
Frozen Tricuspid Valve
Normal Mitral Valve
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SURGERY FOR CARCINOID VALVE DISEASE
Underwent surgical replacement of both tricuspid & pulmonic valve
33-mm Edwards Magna Pericardial
TVR
21-mm Sorin Pericardial PVR
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TRANSTHORACIC ECHOCARDIOGRAM | TRICUSPID BIOPROSTHESIS
RV
RA
TV Bioprosthesis
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TRANSTHORACIC ECHOCARDIOGRAM | TRICUSPID BIOPROSTHESIS
Subcostal View
TV Bioprosthesis
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TRANSTHORACIC ECHOCARDIOGRAM | PULMONIC BIOPROSTHESIS
RV
LA
Pulmonic Bioprosthesis
AV
PA
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TRANSTHORACIC ECHOCARDIOGRAM | PULMONIC BIOPROSTHESIS
RV
LA
Pulmonic Bioprosthesis
AV
PA
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CASE #4 EPILOGUE
At present, 4 years after TVR & PVR, patient is doing well on octreotide.
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CARCINOID HEART DISEASE
Case #5
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J. Am. Coll. Cardiol. 2010;55;1996.
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Thank you
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New York University Medical Center