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![Carcinoid Heart Disease
• a systemic disorder mediated by
elevated circulating levels of
vasoactive substances, including
serotonin (5-hydroxytryptamine
[5-HT]), 5-hydroxytryptophan,
histamine, bradykinin,
tachykinins, and prostaglandins
produced by a rare metastatic
neuroendocrine malignancy,
carcinoid
•
• Carcinoid syndrome is
characterized by a triad of
symptoms—flushing, diarrhea,
and bronchospasm—that occur
in association with hepatic
metastases](https://image.slidesharecdn.com/cardiomyopathy-170512071237/85/Cardiomyopathy-52-320.jpg)
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Cardiomyopathy
The document discusses various forms of cardiomyopathy, their definitions, classifications, symptoms, and complications. It encompasses types such as dilated, hypertrophic, restrictive, and arrhythmogenic right ventricular dysplasia, detailing pathophysiology, risk factors, diagnostics, and treatment options. Additionally, it covers associated conditions like carcinoid heart disease and long QT syndrome, highlighting the importance of genetic factors and the clinical implications of these heart muscle diseases.
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Cardiomyopathy
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- 2. WHO,1980 Heart muscle diseasesof unknown cause But not from cardiac dysfunction due to known cardiovascular entities Hypertension Ischemic heart disease Valvular disease
- 3. WHO/International Society andFederation of Cardiology (ISFC) Task Force,1995 1. Dilated • Enlarged • Systolic dysfunction 2. Hypertrophic • Thickened • Diastolic dysfunction 3. Restrictive • Diastolic dysfunction 4. Arrhythmogenic RV dysplasia • Fibrofatty replacement 5. Unclassified • Fibroelastosis • LV noncompaction Anatomy & physiology
- 4. European Working Groupdefinition,2008
- 5. American Heart Association •Featured by • Mechanical dysfunction • Electrical dysfunction • Ventricular hypertrophy • Ventricular dilation • Mostly genetic • Primary (In situ) • Secondary (A part of systemic disease) EXCLUDED • Ischemic cardiomyopathy • Valvular disease • Congenital heart disease • Hypertension heart disease
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- 7. CMP but normalwith looking heart
- 8. Pathophysiology Mutation:Pathological and polymorphicExogenous insult:Viral,toxins,alcohol Contraction and relaxation disorder Ineffective energy utilization Altered Ca ions handling Activation of compensatory neurohumoral mechanisms Apoptosis Fibrosis Hypertrophy Heart failure:Systolic ,diastolic,both Arrhythmia, sudden death Thromboembolic complication
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- 15. Risk of SuddenDeath (LVEF >35%) Maggioni AP. GISSI-2 Trial Circulation. 1993;87:312-322. EF<35% (LVEF < 35%) NO PVC SOME PVC MANY VPCo PVBs 1-10 PVBs/h > 10 PVBs/h 0.86 A 0.88 0.90 0.92 0.94 0.96 0.98 1.00 0 30 60 90 120 150 180 Days Survival p log-rank 0.002 0.88 0.90 0.92 0.94 0.96 0.98 1.00 0 30 60 90 120 150 180 Days Survival B p log-rank 0.0001 0.86
- 16. Complications • Heart failure •Blood clots • Valve problems • Cardiac arrest /Sudden death:VT/VF
- 17. Symptoms • SYSTOLIC HEARTFAILURE • DIASTOLIC HEART FAILURE • OUTFLOW TRACT OBSTRUCTION • ARRYTHMIA • SYSTEMIC FEAURES • SYNDROMIC ASSOCIATION
- 18. Tests • Chest X-ray •Echocardiogram • Electrocardiogram (ECG) • Treadmill stress test • Cardiac catheterization • Cardiac magnetic resonance imaging (MRI) • Cardiac computerized tomography (CT) scan • Blood tests. • ELECTROPHYSIOLOGY • Myocardial or endocardial biopsy • Genetic testing or screening
- 19. TREATMENT • Exogenous insultprevention • Sudden death prevention • Primary=ICD • Secondary:ICD • Heart failure therapy • Symptomatic • Life prolonging=MYECTOMY AND HEART TRANSPLANTATION,CRT • Thromboembolic complications • Gene therapy:GAUCHER’S AND FABRY’S DISEASE
- 20. SYMPTOMATIC :MM=Frank-Starling curve •B=diuretic or venodilator • B”=excess diuretic or venodilator • C=inotrope • D=arterial dilator • E=Inotrope and vasodilator
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- 24. THE DILATED CARDIOMYOPATHIES •Dilated left ventricle • systolic dysfunction • that is not caused by ischemic or valvular heart disease • The most common cardiomyopathy
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- 26. Hypertrophic cardiomyopathy 1. Mostcommon of the genetic cardiovascular diseases 2. Compatible with normal longevity in many patients 3. Most common cause of sudden death in the young 4. 1 in 500 people
- 27. Wall thickness of13-15 mm in males and 11-12 mm in females
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- 36. RESTRICTIVE CARDIOMYOPATHY • INVRTEDPEAR SIGN
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- 38. Cardiac Amyloidosis Basedon Amyloid
- 39. SARCOID CARDIOMYOPATHY • Hematoxylin-eosinstaining: Noncaseating granuloma typical of sarcoid. The arrow points to an “asteroid body” in the cytoplasm of the giant cell
- 40. Peripartum cardiomyopathy • LVsystolic dysfunction • Towards the end of pregnancy(last month) or in the months(puriperium) following delivery • When no other cause of heart failure is found • The LV may not be dilated but the ejection fraction is nearly always below 45%
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- 44. Arrythmogenic right ventriculardysplasia
- 48. Revised Task ForceCriteria for the Diagnosis of ARVD
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- 52. Carcinoid Heart Disease •a systemic disorder mediated by elevated circulating levels of vasoactive substances, including serotonin (5-hydroxytryptamine [5-HT]), 5-hydroxytryptophan, histamine, bradykinin, tachykinins, and prostaglandins produced by a rare metastatic neuroendocrine malignancy, carcinoid • • Carcinoid syndrome is characterized by a triad of symptoms—flushing, diarrhea, and bronchospasm—that occur in association with hepatic metastases
- 53. • The characteristicpathologic features of carcinoid heart disease are right-sided valve thickening and retraction resulting from myofibroblast proliferation along with deposition of collagen, smooth muscle cells, and elastic tissue. Tricuspid annular and subvalvar involvement and pulmonary root constriction also occur, thereby adding to the valvular dysfunction. Very rarely the heart is involved directly by carcinoid metastases
- 54. • Untreated, patientswith carcinoid syndrome have a median survival of 3 to 4 years, and the presence of carcinoid heart disease shortens this to less than 1 year. Therapy is not generally curative and includes debulking the hepatic metastases by embolization or partial hepatic resection and by the use of octreotide, a somatostatin analogue that binds to somatostatin receptors on the surface of carcinoid tumor cells and inhibits the secretion of vasoactive substances
- 55. Endomyocardial Disease • CARCINOIDHD • LOEFFLLER’S CMP • ENDOCARDIAL FIBROSIS
- 56. • Elevation ofurinary 5-HIAA levels is highly specific and moderately sensitive for the diagnosis of carcinoid syndrome, and the echocardiographic and cardiac MRI features of thickened immobile tricuspid and pulmonary valves with combined stenosis and regurgitant lesions are highly suggestive of carcinoid heart disease
- 57. Löffler (Eosinophilic) Endocarditis •Hypereosinophilia has been defined as either a chronic absolute eosinophil count higher than 1500 cells/mL for at least 1 month, although hypereosinophilia persisting for 6 months or longer is common, or pathologic evidence of hypereosinophilic tissue invasion • Three stages: • acute: Myocarditis • Intermediate: Valvular and thrombus formation • Fibrotic :RCM
- 58. Endomyocardial Fibrosis • Fibrosisof the LV and RV apical endocardium • Presents as RCM • The south Asian subcontinent • More males affected than females (23% versus 17%) • Family clustering • IDIOPATHIC /hypereosinophilic syndrome • A bimodal peak in age :First decade and second to fourth decades
- 59. • Heart failuresymptoms from left or right restrictive physiology • Dyspnea on exertion • Paroxysmal nocturnal dyspnea • Edema/ Ascites • Imaging • apical fibrosis / atrial enlargement and MV and TV SVF • TREAMENT • surgical resection of the endocardial fibrosis with valve repair or replacement can have a dramatic effect on symptoms and survival • significant risk for morbidity and mortality.
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- 61. LONG QT • Delayedrepolarization of the myocardium • QT prolongation :QTc >480 msec • syncope, seizures, and SCD • Structurally normal heart
- 62. BRUGADA SYNDROME BrS isan heritable arrhythmia syndrome characterized by an ECG pattern consisting of coved-type ST-segment elevation (≥2 mm) followed by a negative T wave in the right precordial leads V 1 through V 3 (often referred to as a type 1 Brugada ECG pattern) and increased risk for sudden death resulting from episodes of polymorphic ventricular tachyarrhythmias
- 63. LOSS OF SCN5ACHANNEL FUNCTION
- 64. Catecholaminergic Polymorphic Ventricular Tachycardia •Gain-of-function mutations in RyR2lead to leaky calcium release channels, which results in excessive release of calcium, particularly during sympathetic stimulation, that can precipitate calcium overload, delayed depolarizations, and ventricular arrhythmias
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