Mitral regurgitation causes

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Varun Kumar, M.B.B.S.; Lakshmi Gopalakrishnan, M.B.B.S.

Complete Differential Diagnosis of the Causes of Mitral Regurgitation

(By organ system)

Cardiovascular	Post-MI pericarditis in the immediate days following acute MI and Dresslers syndrome which develops later; dissecting aortic aneurysm; endocarditis and underlying myocarditis. Following cardiovascular procedures such as: cathether ablation for arrhythmias, coronary artery bypass grafting (CABG) (postpericardiotomy syndrome), pacemaker insertion, percutaneous coronary intervention with either dissection or perforation of the coronary artery, TAVI, thoracic surgery (resulting in chylopericardium), valvuloplasty.
Chemical / poisoning	Silicosis
Dermatologic	Behcet syndrome
Drug Side Effect	Usually associated with small effusions. Common culprits include hydralazine, procainamide, DOH, isoniazid, phenylbutazone, dantrolene, doxorubicin, methylsergide, penicillin.
Ear Nose Throat	Temporal arteritis
Endocrine	Usually in conjunction with clinically severe hypothyroidism. Most early case reports associated with myxedema and patients also had ascites, pleural effusions and uveal edema. Often resolves with thyroid replacement therapy. A pericardial effusion can be seen as part of an Addisonian crisis.
Environmental	No underlying causes
Gastroenterologic	Inflammatory bowel disease, Whipple's
Genetic	Gaucher disease, Jacobs arthropathy-camptodactyly syndrome, Mulibrey nanism syndrome, Recurrent hereditary polyserositis
Hematologic	Leukemia, Lymphoma
Iatrogenic	Chylopericardium (from thoracic duct obstruction secondary to tumor, surgical procedure), Cardiopulmonary resuscitation, Postpericardiotomy syndrome, Radiation therapy, Serum sickness
Infectious Disease	Bacterial: Pneumococcus, Streptococcus and Staphylococcus are most common. Also Borrelia,Brucellosis, E.coli, Francisella, Haemophilus influenza, Klebsiella, Legionella(preodominantly by hematogenous spread and approximately 20% by contiguous spread. Usually these patients are quite ill), Meningococci, Neisseria, Proteus, Psuedomonas, Salmonella, Tularemia. Fungal: Actinomycosis, Amebiasis, Aspergillus, Blastomycosis, Candida, Coccidiomycosis,Echinococcus, Histoplasmosis, Nocardia, Toxoplasmosis. Helminthic: Alveolar hydatid disease Protozoal: Entamoeba histolytica Tuberculous: usually bloody, protein greater than 2.5. Initially mostly polymorphonuclear cells, later lymphocytes, monocytes and plasma cells. Usually develops very slowly with significant fibrous reaction. Initially effusive then becomes constrictive. OtherMycoplasma such as mycoplasma pnuemonia can cause pericarditis is well. Viral: Coxsackie B Virus, Echovirus, Adenovirus (less commonly: CMV-especially in HIV patients, EBV, Hepatitis B, Influenza, Mumps, Varicella). Other: , Lyme disease (usually myopericarditis associated with conduction abnormalities). Rickettsia
Musculoskeletal / Ortho	No underlying causes
Neurologic	No underlying causes
Nutritional / Metabolic	No underlying causes
Obstetric/Gynecologic	No underlying causes
Oncologic	Predominantly lung cancer, breast cancer, leukemia, lymphomas (Hodgkins and non-Hodgkins). Less commonly GI malignancies, ovarian cancer, sarcomas and melanomas, metastic, hematogenous, carcinoma, carcinoid, Sipple syndrome, mesothelioma, fibroma, lipoma . Also Kaposis sarcoma in HIV positive patients.
Opthalmologic	No underlying causes
Overdose / Toxicity	No underlying causes
Psychiatric	No underlying causes
Pulmonary	Sarcoidosis
Renal / Electrolyte	Uremic pericarditis is seen in up to 20% of uremic patients requiring chronic hemodialysis. The mechanism is unknown. Most commonly there is a small effusion associated with pain and a pericardial friction rub, but there can be a large effusion and present with tamponade
Rheum / Immune / Allergy	Systemic Lupus Erythematosus or SLE: Pericarditis usually occurs in the setting of disease flares (systemic symptoms, high erythrocyte sedimentation rate (ESR) , +ANA, +dsDNA, pleural effusions). Occurs in 20-40% of patients with SLE during the course of the disease. Usually the fluid is serous or grossly bloody. Analysis of the fluid usually reveals a high protein and low glucose content. Typically WBC count is less than 10K, and is made up of primarily polymorphonuclear cells (PMNs). Rheumatoid arthritis or RA: Pericarditis can occur without active joint involvement. Also serous or bloody. Usually the protein is > 5 mg/dl, and the glucose is low (<45). The WBC is high at 20-90K. Complement is usually low, and the latex fixation test is usually positive. Other: Amyloidosis, Ankylosing Spondylitis, Behcet syndrome, Familial Mediterranian Fever, Kawasaki disease, Mixed Connective Tissue Disease, Polyarteritis nodosa PAN, Polymyositis,Reiter's Syndrome, acute Rheumatic fever, Sarcoidosis, Scleroderma, Still disease, Systemic sclerosis, Temporal arteritis and , Wegener's.
Sexual	Neisseria gonorrhoeae, Treponema pallidum
Trauma	After blunt or penetrating chest trauma Following cardiovascular procedures such as: cathether ablation for arrhythmias, pacemaker insertion, percutaneous coronary intervention with either dissection or perforation of the coronary artery, TAVI, thoracic surgery (resulting in chylopericardium, valvuloplasty. Following gastrointestinal catastrophes including esophageal rupture, pancreatic-pericardial fistula, esophogeal perforation, gastric perforation.
Urologic	Renal Failure, Uremia
Miscellaneous	Commonly the diagnosis is idiopathic.

Differential Diagnosis of Causes of Acute Pericarditis

In alphabetical order:

• Actinomycosis
• Acute idiopathic pericarditis
• Acute rheumatic fever
• Adenovirus
• Addison's crisis
• Amyloidosis
• Amebiasis
• Ankylosing Spondylitis
• Aortic dissection
• Behcet's Disease
• Borrelia
• Breast cancer
• Cardiopulmonary resuscitation
• Chylopericardium
• Coxsackie A
• Coxsackie B
• Cytomegalovirus
• Dermatomyositis
• Dermatosclerosis
• Dressler's Syndrome
• EBV
• ECHO virus
• Echinococcosis
• Familial Mediterranian Fever
• Francisella
• HIV
• Infectious mononucleosis
• Inflammatory Bowel Disease
• Influenza virus
• Kawasaki disease
• Legionella
• Leukemia
• Lung cancer
• Lymphoma
• Meningococci
• Mixed Connective Tissue Disease
• Mumps virus
• Mycoplasma infection
• Neisseria gonorrhoeae
• Perforated esophagus
• Pneumococci
• Polyarteritis Nodosa
• Polymyositis
• Postpericardiotomy syndrome
• Reiter's Syndrome
• Radiation therapy
• Renal Failure
• Rheumatoid Arthritis
• Rickettsia
• Sarcoidosis
• Scleroderma
• Serum sickness
• Staphylococci
• Streptococci
• Systemic Lupus Erythematosus
• Thorax trauma
• Treponema pallidum
• Toxoplasmosis
• Tuberculosis
• Uremia
• Varicella virus
• Wegener's granulomatosis
• Whipple's Disease

Differential Diagnosis of Causes of Chronic Pericarditis

In alphabetical order:

• Amebiasis
• Bacterial infections
• Cholesterol pericarditis
• Chylopericardium
• Coccidioidomycosis
• Collagen Vascular Disease
• Coxsackie B
• Echinococcosis
• Histoplasmosis
• Neoplastic pericarditis
• Tuberculosis
• Uremic pericarditis

Diseases Causing Mitral Regurgitation

Primary mitral regurgitation is due to any disease process that affects the mitral valve apparatus itself. The causes of primary mitral regurgitation include:

• Mitral valve prolapse now accounts for 45% of cases in the Western world
• Ischemic heart disease / Coronary artery disease
• Rheumatic heart disease In the past, this was the most common cause of MR in the Western world. In developing countries, rheumatic heart disease remains a major cause.
• Infective endocarditis
• Collagen vascular diseases (ie: SLE, Marfan's syndrome)
• Trauma
• Balloon valvuloplasty of the mitral valve
• Certain forms of medication (e.g. fenfluramine)

Secondary mitral regurgitation is due to the dilatation of the left ventricle, causing stretching of the mitral valve annulus and displacement of the papillary muscles. This dilatation of the left ventricle can be due to:

• Any cause of dilated cardiomyopathy including aortic insufficiency,
• Non-ischemic dilated cardiomyopathy,
• Non-compaction Cardiomyopathy and
• As a complication of Takotsubo cardiomyopathy. A recent study revealed mitral valve tethering and systolic anterior motion of mitral valve have independent mechanisms with different pathophysiology in causing acute MR in patients with Takotsubo cardiomyopathy.

Causes of chronic mitral regurgitation include:

• Primary diseases of the valve leaflets such as mitral valve prolapse. MVP is a common cause. However, most patients with MVP do not develop severe mitral regurgitation. Older age, male gender, and auscultatory evidence of severe MR are prognostic clues that identify patients with mitral valve prolapse who are at a relatively high risk of complications.
• Rheumatic heart disease. One out of three cases of chronic mitral regurgitation are caused by rheumatic heart disease, a complication of untreated strep throat that is becoming less common.
• Coronary artery disease and heart attacks.
• cardiomyopathy.
• Endocarditis.
• Heart tumors.
• High blood pressure.
• Marfan syndrome.
• Swelling of the left lower heart chamber.
• Untreated syphilis (rare).
• Congenital (present from birth) mitral regurgitation is most often part of a more complex heart defect or syndrome.

Chronic Mitral Regurgitation

• Developed World: Mitral valve prolapse (MVP).
• Developing World: Rheumatic heart disease
• Ischemic mitral regurgitation: Ischemic heart disease causes mitral regurgitation by the combination of ischemic dysfunction of the papillary muscles, the abnormal motion of the underlying wall, and the dilatation of the left ventricle that is present in ischemic heart disease, with the subsequent displacement of the papillary muscles and the dilatation of the mitral valveannulus.
• Secondary mitral regurgitation due to the dilatation of the left ventricle, caused by stretching of the mitral valve annulus and displacement of the papillary muscles. This dilatation of the left ventricle can be due to any cause of dilated cardiomyopathy, including aortic insufficiency, nonischemic dilated cardiomyopathy and Noncompaction Cardiomyopathy. It is also called functional mitral regurgitation, because the papillary muscles, chordae, and valve leaflets are usually normal.
• Marfan's syndrome

Acute Mitral Regurgitation

• Acute bacterial endocarditis. The predominant organism is S. aureus, but varies depending upon the patient.
• Papillary muscle rupture or dysfunction, associated with ST elevation myocardial infarction