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Marantic Endocarditis.pptx
- 1. Dr Mohammad Alhalabi M.D. Marantic Endocarditis Department of Cardiology, Faculty of Medicine, Damascus University
- 2. Outline Overview Definition, Classification, Pathophysiology, Etiology 01 Presentation Signs & Symptoms, Evaluation & Workup 02 Diagnosis Diagnostic Strategy, Differential Diagnoses 03 Treatment Medical, Surgical, Causal, Prognostic 04
- 3. Endocardium • Inner layer of the cardiac wall and covers the inner surface of the cardiac chambers • The superficial surface of the cardiac valves
- 4. Endocardium Histology • Endothelium endothelial cells (squamous epithelial cells) • Subendothelial connective tissue layer collagen fibrils and fibroblasts more prominent in the atria, in particular, the left atrium • Elastic tissue layer elastic fibers, collagen fibers and smooth muscle cells more prominent in the left atrium and the left ventricular septum • Subendocardial tissue layer connects endocardium to the myocardium collagen and elastic fibers, small blood vessels and Purkinje fibers
- 5. Endocarditi s Inflammation of the endocardial surfaces of the heart
- 6. Endocarditis ▹ Primarily of 1 or more heart valves ▹ The mural endocardium ▹ A septal defect Inflammation of the endocardial surfaces of the heart
- 7. Endocarditis Classification NBTE does not cause an inflammation response from the body Infectious Etiology Caused by bacteria, fungi, and germs that enter through the bloodstream Non-Infectious Etiology Thought to be caused by a combination of different mechanisms 01 Acute or subacute or Chronic Native Valve or Prosthetic Valve 02 Can be divided into culture-positive or culture- negative Bacterial or non- bacterial 01 SLE / Antiphospholipid Associated Verruceous (Libman- Sacks) 02 Malignancy / Wasting Associated Nonbacterial thrombotic endocarditis (NBTE) “Marantic”
- 8. • Commonly affects undamaged valves (in contrast with infective endocarditis) • A separate entity to culture-negative endocarditis which is due to infectious etiologies that are not readily identified • Pathogenesis is unknown, but thought to be endothelial injury in the setting of a hypercoagulable state Deposition of sterile platelet thrombi on heart valves (mostly aortic and mitral) Marantic Endocarditis Pathogenesis A form of noninfectious endocarditis. a spectrum of noninfectious lesions of the heart valves that is most commonly seen in advanced malignancy Nonbacterial thrombotic endocarditis (NBTE)
- 9. Marantic Endocarditis Pathogenesis Presentation Cancer is spread widely throughout the body Carcinomatosis Wasting, disseminated intravascular coagulation, Hypercoagulable State But can occur in patients with valvular pathology Valve usually undamaged Valve Dysfunction is rare Systemic Embolism
- 10. Marantic Endocarditis Characteristics • A rare condition most often found postmortem with rates from 0.9 to 1.6 percent • Up to 2.7% to 10% in adenocarcinomas and mucin-secreting carcinomas, most commonly affects patients between the fourth and eighth decades of life • No gender predilection • Libman-Sacks endocarditis could span up to 43% under TEE in patients with SLE
- 12. Pathogenesis
- 16. How can we differentiate NBTE from IE? • No pathognomonic signs/symptoms or echo features that are specific to NBTE • Diagnosis can only be differentiated with demonstration of platelet thrombi on histologic exam • High clinical suspicion is required for diagnosis!
- 17. Marantic vs Libman-Sacks Endocarditis? Guidelines for the Use of Echocardiography in the Evaluation of a Cardiac Source of Embolism. Journal of the American Society of Echocardiography 2016 29, 1-42
- 18. Marantic vs Libman-Sacks Endocarditis? Guidelines for the Use of Echocardiography in the Evaluation of a Cardiac Source of Embolism. Journal of the American Society of Echocardiography 2016 29, 1-42
- 19. Marantic vs Libman-Sacks Endocarditis?
- 20. Maranctic Endocarditis Etiology Marantic Endocarditis Malignancy (80%) Chronic wasting diseases Chronic Infections DIC • Tuberculosis • Pneumonia • Osteomyelitis Mucin-producing metastatic carcinomas / Trousseau syndrome • Pancreas • Lung • Colon • Ovary • Biliary • Prostate • AML-M3 • Pregnancy complications Acute Malnutrition • Tuberculosis • Chronic diarrhea • AIDS • Superior mesenteric artery syndrome
- 22. Presentation Imaging Signs and symptoms Echocardiography 1 2 3 4 • Vegetations are frequently left-sided with • two-thirds of cases involving the mitral valve, a quarter the aortic TEE is more sensitive than TTE • Three sets of blood cultures and serological testing to rule out Infective Endocarditis • Workup for disseminated intravascular coagulation (DIC) should be performed in every case of suspected NBTE No laboratory tests that confirm the diagnosis of NBTE • CT of the brain or MRI may be performed in those with suspected cerebral embolization Presents with strokes not radiologically specific to NBTE • Major clinical manifestations of NBTE result from systemic emboli in up to 50% • Cardiac murmurs infrequently in less than half of patients • Fever is uncommon, may be an underlying manifestation of malignancy Often asymptomatic Laboratory
- 23. Diagnosis Gold Standard • Made pathologically by demonstration of thrombi on autopsy or specimens • Routine acquisition of valvular tissue is not practical Clinical Diagnosis • A constellation of clinical, echocardiographic, and absence of microbiologic findings • Demonstration of valvular vegetations on echocardiography in the absence of systemic infection in patients who are at high risk
- 24. Diagnostic Challenges Diagnosis of NBTE is difficult to establish antemortem • Paucity of symptoms • Late presentation Fever due to the underlying malignancy can lower the clinical suspicion for NBTE Small remnants on affected valves (≤3 mm) following embolization not readily identified by echocardiogr aphy False negatives
- 25. The Mitral valve revealing thickened leaflets 3D TEE PLAX Transoesophageal echocardiography short axis showing the thickening in motion 3D TEE PSAX Mitral
- 26. Transoesophageal echocardiography showing large oscillating structures in long axis 3D TEE PLAX The aortic valve revealing severely thickened cusps 3D TEE PSAX Aortic
- 27. Treatment Outline Anticoagulation Mainstay Treatment Surgery Indications in Select Cases Underlying Cause Effective Systemic Therapy Followup & Prognosis Monitoring & Complications
- 28. Anticoagulation NBTE patients are routinely anticoagulated • Rather than warfarin or a direct thrombin or factor Xa inhibitor • Anticoagulation should be continued indefinitely Therapeutic dose subcutaneous LMWH or intravenous UFH should be used • Provided there is no contraindication (e.g., active central nervous system bleeding) • Due to high rates of recurrent and extensive embolization in this population With or without evidence of systemic emboli 01 02
- 29. Surgery May be considered in select cases • Heart failure • Acute valve rupture • Prevention of recurrent embolization Indications for surgery same as IE • Benefits should be weighed against the risks in the context of the life expectancy • Vegetation excision or valve replacement Where the risk benefit is favorable 01 02
- 30. Treatment for underlying disease Disease treatment often ineffective in the absence of effective systemic therapy • Antibiotics do not need to be continued unless infective endocarditis is diagnosed Many patients may be placed on empiric antibiotics during investigation • It is unknown whether NBTE improves with cancer therapy • Anticoagulation should continue regardless of the response Metastatic nature of most malignancies at presentation 01 02
- 31. Followup & Prognosis Optimal followup has not been defined and prognosis of NBTE has not been formally evaluated — should be individualized Due to the strong association between NBTE and advanced malignancy Prognosis is grim despite anticoagulation NBTE complications • Infective endocarditis • Embolization despite anticoagulation Complications of therapy • Bleeding • Thrombocytopenia Patients should be followed clinically for complications 01 02