History

Fact Explanation
Cardiovascular symptoms: exercise intolerance, fatigue, sustained heart rate, syncope, dizziness, light headedness, balance problems [1] Cardiovascular neuropathy is a result of damage to vagal and sympathetic nerves. [2] The pathogenesis is complex and involves a cascade of pathways activated by hyperglycaemia resulting in neuronal ischaemia and cellular death [3]
Gastrointestinal symptoms: dysphagia, diarrhea, constipation, loss of bowel control [4] Dysphagia occurs due to reduced contraction amplitudes of the tubular esophagus [2] Diabetic diarrhea is a result of increased or uncoordinated transit time in the small intestine, bacterial overgrowth, or increased intestinal secretions. [2] Constipation occurs due to decreased transit time in the large intestine. Loss of bowel control is due to reduced threshold of conscious rectal sensation,decreased resting anal sphincter pressure.[2]
Genitourinary symptoms: loss of bladder control, urinary tract infection, urinary frequency or dribbling, erectile dysfunction, loss of libido, dyspareunia, vaginal dryness [4] Loss of bladder control occurs by the Inability to sense a full bladder and detrusor muscle hypoactivity.These conditions can progress to overflow incontinence and urinary tract infections.Hyperglycemia alone also can cause increased urine production and incontinence. loss of libido,dyspareunia,vaginal dryness is a result of pelvic plexus neuropathy [2] Erectile dysfunction in diabetes is multifactorial, including neuropathy,hypogonadism, vascular disease, metabolic control and psychogenic factors. Diabetic men have impaired neurogenic and endothelium mediated relaxation of penile smooth muscles [5]
Sudomotor symptoms: pruritus, dry skin, limb hair loss,anhidrosis,heat intolerance,gustatory sweating [4] Dry skin,hair loss- Poor peripheral circulation (neuronal input to the peripheral vasculature is decreased or absent) [2] Gustatory sweating-Loss of autonomic supply to auriculotemporal nerve. [6]
Endocrine symptoms: hypoglycemic unawareness [4] Impaired catecholamine release prevents the warning signs of hypoglcemia. [2]
Eyes: diplopia [2] Loss of autonomic supply to ocular muscles.[2]
Dizziness while standing up from seated position [4] Orthostatic hypotension (systolic blood pressure drop of at least 20 mm Hg or diastolic blood pressure drop of at least 10 mm Hg when standing up.) Vasomotor neuropathy frequently causes orthostatic hypotension by affecting the splanchnic and peripheral vascular beds.[2]
Poor diabetic control , long duration of diabetes, History of hypertension, dyslipidemia [7] Risk factors for the development of diabetic autonomic neuropathy as damage to the nerves will be increased with time due to long exposure to hyperglycemia. The risk is also high in patients who are having other comorbidities which has macrovascular events such as hypertension and dyslipidemia [7]
References
  1. VINIK A. I., MASER R. E., MITCHELL B. D., FREEMAN R.. Diabetic Autonomic Neuropathy. Diabetes Care [online] 2003 May, 26(5):1553-1579 [viewed 13 May 2014] Available from: http://care.diabetesjournals.org/content/26/5/1553.full
  2. VINIK A I., ERBAS T., Recognizing and treating diabetic autonomic neuropathy. Cleveland clinic journal of medicine [online] November 2001, volume 68 number 11 [viewed 13 May 2014] Available from: http://www.ccjm.org/content/68/11/928.full.pdf
  3. DIMITROPOULOS G, TAHRANI AA, STEVENS MJ. Cardiac autonomic neuropathy in patients with diabetes mellitus. World J Diabetes [online] 2014 Feb 15, 5(1):17-39 [viewed 09 September 2014] Available from: doi:10.4239/wjd.v5.i1.17
  4. ARING A M., JONES D E., FALKO J M., Evaluation and Prevention of Diabetic Neuropathy. American Family Physician [online] June 2005, 71(11):2123-2128 [viewed 13 May 2014]. Available from: http://www.aafp.org/afp/2005/0601/p2123.html#afp20050601p2123-t1
  5. PENSON D. F., WESSELLS H.. Erectile Dysfunction in Diabetic Patients. Diabetes Spectrum [online] 2004 October, 17(4):225-230 [viewed 10 September 2014] Available from: doi:10.2337/diaspect.17.4.225
  6. BRONSHVAG M M., Spectrum of gustatory sweating with especial reference to its presence in diabetics with autonomic neuropathy. The American Journal of Clinical Nutrition [online] February 1978, page 307-309 [viewed 13 May 2014] Available from: http://ajcn.nutrition.org/content/31/2/307.full.pdf
  7. BOULTON A. J.M., VINIK A. I., AREZZO J. C., BRIL V., FELDMAN E. L., FREEMAN R., MALIK R. A., MASER R. E., SOSENKO J. M., ZIEGLER D.. Diabetic Neuropathies: A statement by the American Diabetes Association. Diabetes Care [online] 2005 April, 28(4):956-962 [viewed 10 September 2014] Available from: doi:10.2337/diacare.28.4.956

Examination

Fact Explanation
Thoracic skeletal abnormalities: the most common are scoliosis, pectus excavatum, straightened thoracic spine and narrowed anteroposterior diameter of the chest [1] Due to connective tissue disorders.Thoracic skeletal abnormalities are commonly associated with mitral valve prolapse (MVP) [2]
Stigmata, such as arachnodactyly, that are more typical of Marfan syndrome [1] Primary MVP due to Marfan syndrome [1]
Heart murmur [1] Midsystolic click, a high-pitched sound of short duration.The midsystolic click is frequently followed by a late systolic murmur, usually medium to high pitched and loudest at the apex [2] Maneuvers that cause the click or murmur to occur earlier in systole include standing from the supine position, performing a sub-maximal isometric handgrip exercise, straining during the Valsalva maneuver and inhaling amyl nitrite. Maneuvers that cause the click and murmur to move toward the second heart sound include squatting from the upright position [1]
Tachycardia [1] May be present sometimes.Paroxysmal supraventricular tachycardia is the most common sustained tachycardia [1]
References
  1. BOUKNIGHT DP, O'ROURKE RA. Current management of mitral valve prolapse. Am Fam Physician [online] 2000 Jun 1, 61(11):3343-50, 3353-4 [viewed 22 June 2014] Available from: http://www.aafp.org/afp/2000/0601/p3343.html
  2. BONOW ROBERTO., CARABELLO BLASE, DE LEON ANTONIOC., EDMUNDS L.HENRY, FEDDERLY BRADLEYJ., FREED MICHAELD., GAASCH WILLIAMH., MCKAY CHARLESR., NISHIMURA RICKA., O’GARA PATRICKT., O’ROURKE ROBERTA., RAHIMTOOLA SHAHBUDINH., RITCHIE JAMESL., CHEITLIN MELVIND., EAGLE KIMA., GARDNER TIMOTHYJ., GARSON ARTHUR, GIBBONS RAYMONDJ., O’ROURKE ROBERTA., RUSSELL RICHARDO., RYAN THOMASJ., SMITH SIDNEYC.. ACC/AHA guidelines for the management of patients with valvular heart disease. Journal of the American College of Cardiology [online] 1998 November, 32(5):1486-1582 [viewed 22 June 2014] Available from: doi:10.1016/S0735-1097(98)00454-9

Differential Diagnoses

Fact Explanation
Aortic valve disease [1] Aortic ejection clicks occur earlier in systole than in mitral valve prolapse (MVP), best heard to the right of the upper sternum. Click timing not affected by dynamic maneuvers [1]
Pulmonary valve disease [1] Pulmonic ejection clicks occur earlier in systole than in MVP, best heard to the left of the upper sternum. Click timing not affected by dynamic maneuvers [1]
Atrial myxoma [1] May have symptoms of weight loss, fever, malaise. Exam may reveal a diastolic tumor plop or diastolic murmur similar to mitral stenosis [1]
Ischemia related mitral regurgitation [1] History reveals previous myocardial infarction. Exam features are similar to MVP-related regurgitant murmurs. May be late systolic or holosystolic, which can mimic severe MVP-related MR. Absence of click.Electrocardiogram reveals previous infarction [1]
Rheumatic heart disease related mitral regurgitation [1] History of group A streptococcal pharyngitis followed by migratory large-joint arthritis, fever, rash, and subcutaneous nodules. Rheumatic heart disease typically follows 1 to 2 decades later. Exam features are similar to MVP-related regurgitant murmurs. Usually holosytolic, which can mimic severe MVP-related mitral regurgitation. Absence of click.Vegetations on echocardiogram, typically on the atrial side of the valve [1]
Infective endocarditis affecting mitral valve [1] Fever, chills, sweats, weight loss, Osler nodes, subungual hemorrhages, Janeway lesions, Roth spots, petechiae [1]
Tricuspid valve prolapse [1] While tricuspid prolapse may also produce a midsystolic click sound, it is best heard at the lower left sternal border, or occasionally on the lower right parasternal area. Timing between the S1 and a tricuspid click often increases after inspiration or other maneuvers that increase right ventricular return.Echocardiogram is diagnostic [1]
References
  1. BONOW ROBERTO., CARABELLO BLASE, DE LEON ANTONIOC., EDMUNDS L.HENRY, FEDDERLY BRADLEYJ., FREED MICHAELD., GAASCH WILLIAMH., MCKAY CHARLESR., NISHIMURA RICKA., O’GARA PATRICKT., O’ROURKE ROBERTA., RAHIMTOOLA SHAHBUDINH., RITCHIE JAMESL., CHEITLIN MELVIND., EAGLE KIMA., GARDNER TIMOTHYJ., GARSON ARTHUR, GIBBONS RAYMONDJ., O’ROURKE ROBERTA., RUSSELL RICHARDO., RYAN THOMASJ., SMITH SIDNEYC.. ACC/AHA guidelines for the management of patients with valvular heart disease. Journal of the American College of Cardiology [online] 1998 November, 32(5):1486-1582 [viewed 22 June 2014] Available from: doi:10.1016/S0735-1097(98)00454-9

Investigations - for Diagnosis

Fact Explanation
Electrocardiogram [1] The most common abnormality is the presence of ST-T wave depression or T-wave inversion in the inferior leads (II, III and aVF) [1]
Echocardiography [2] Two-dimensional and Doppler echocardiography is the most useful noninvasive test for diagnosing mitral valve prolapse (MVP). The M-mode echocardiographic definition of MVP includes posterior displacement of one or both leaflets 2 mm or more during late systole or holosystolic posterior displacement greater than 3 mm [2]
Chest X ray [3] Usually show normal cardiopulmonary findings. The skeletal abnormalities,such as pectus excavatum are often seen [3]
References
  1. PEIGHAMBARI MM, ALIZADEHASL A, TOTONCHI Z. Electrocardiographic Changes in Mitral Valve Prolapse Syndrome J Cardiovasc Thorac Res [online] 2014, 6(1):21-23 [viewed 22 June 2014] Available from: doi:10.5681/jcvtr.2014.004
  2. BOUKNIGHT DP, O'ROURKE RA. Current management of mitral valve prolapse. Am Fam Physician [online] 2000 Jun 1, 61(11):3343-50, 3353-4 [viewed 22 June 2014] Available from: http://www.aafp.org/afp/2000/0601/p3343.html
  3. BONOW ROBERTO., CARABELLO BLASE, DE LEON ANTONIOC., EDMUNDS L.HENRY, FEDDERLY BRADLEYJ., FREED MICHAELD., GAASCH WILLIAMH., MCKAY CHARLESR., NISHIMURA RICKA., O’GARA PATRICKT., O’ROURKE ROBERTA., RAHIMTOOLA SHAHBUDINH., RITCHIE JAMESL., CHEITLIN MELVIND., EAGLE KIMA., GARDNER TIMOTHYJ., GARSON ARTHUR, GIBBONS RAYMONDJ., O’ROURKE ROBERTA., RUSSELL RICHARDO., RYAN THOMASJ., SMITH SIDNEYC.. ACC/AHA guidelines for the management of patients with valvular heart disease. Journal of the American College of Cardiology [online] 1998 November, 32(5):1486-1582 [viewed 22 June 2014] Available from: doi:10.1016/S0735-1097(98)00454-9

Investigations - Fitness for Management

Fact Explanation
Full blood count [1] To assess general fitness, fitness for surgery if required [1]
Electrocardiogram [2] To identify coexisting ischemic changes [2]
Fasting blood sugar [1] It is advised to control other co morbid conditions such as diabetes.Also to assess fitness for surgery if required [1]
Lipid profile [1] It is advised to control other co morbid conditions such as dyslipidemia.Also to assess fitness for surgery if required [1]
References
  1. BONOW ROBERTO., CARABELLO BLASE, DE LEON ANTONIOC., EDMUNDS L.HENRY, FEDDERLY BRADLEYJ., FREED MICHAELD., GAASCH WILLIAMH., MCKAY CHARLESR., NISHIMURA RICKA., O’GARA PATRICKT., O’ROURKE ROBERTA., RAHIMTOOLA SHAHBUDINH., RITCHIE JAMESL., CHEITLIN MELVIND., EAGLE KIMA., GARDNER TIMOTHYJ., GARSON ARTHUR, GIBBONS RAYMONDJ., O’ROURKE ROBERTA., RUSSELL RICHARDO., RYAN THOMASJ., SMITH SIDNEYC.. ACC/AHA guidelines for the management of patients with valvular heart disease. Journal of the American College of Cardiology [online] 1998 November, 32(5):1486-1582 [viewed 22 June 2014] Available from: doi:10.1016/S0735-1097(98)00454-9
  2. DüREN DR, BECKER AE, DUNNING AJ. Long-term follow-up of idiopathic mitral valve prolapse in 300 patients: a prospective study. J Am Coll Cardiol [online] 1988 Jan, 11(1):42-7 [viewed 22 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/3335704

Investigations - Followup

Fact Explanation
Echocardiogram [1] To assess the severity with time (eg: left ventricular function-todetect cardiac failure which is a complication of mitral valve prolapse) Signs of mitral regurgitation appears in some patients, but the patients remained clinically asymptomatic. Also to detect features of infective endocarditis (vegetations), which is a complication of mitral valve prolapse (MVP) [2]
Elactrocardiogram [1] To identify arrhythmia which is a complication of mitral valve prolapse.Ventricular tachycardia may developed.Sudden death, is most likely due to ventricular fibrillation [1]
Clinical evaluation [1] Asymptomatic patients with MVP and no significant mitral regurgitation (MR) can be evaluated clinically every 3 to 5 years.Patients who have high-risk characteristics, including those with moderate to severe MR, should be followed once a year [1]
References
  1. BONOW ROBERTO., CARABELLO BLASE, DE LEON ANTONIOC., EDMUNDS L.HENRY, FEDDERLY BRADLEYJ., FREED MICHAELD., GAASCH WILLIAMH., MCKAY CHARLESR., NISHIMURA RICKA., O’GARA PATRICKT., O’ROURKE ROBERTA., RAHIMTOOLA SHAHBUDINH., RITCHIE JAMESL., CHEITLIN MELVIND., EAGLE KIMA., GARDNER TIMOTHYJ., GARSON ARTHUR, GIBBONS RAYMONDJ., O’ROURKE ROBERTA., RUSSELL RICHARDO., RYAN THOMASJ., SMITH SIDNEYC.. ACC/AHA guidelines for the management of patients with valvular heart disease. Journal of the American College of Cardiology [online] 1998 November, 32(5):1486-1582 [viewed 22 June 2014] Available from: doi:10.1016/S0735-1097(98)00454-9
  2. DüREN DR, BECKER AE, DUNNING AJ. Long-term follow-up of idiopathic mitral valve prolapse in 300 patients: a prospective study. J Am Coll Cardiol [online] 1988 Jan, 11(1):42-7 [viewed 22 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/3335704

Investigations - Screening/Staging

Fact Explanation
Echocardiogram [1] Used to grade mitral valve prolapse (MVP) 1. Diagnosis, assessment of hemodynamic severity of MR, leaflet morphology, and ventricular compensation in patients with physical signs of MVP- class I 2. To exclude MVP in patients who have been given the diagnosis when there is no clinical evidence to support the diagnosis-class I 3. To exclude MVP in patients with first-degree relatives with known myxomatous valve disease- class IIa 4. Risk stratification in patients with physical signs of MVP or known MVP- class IIa 5. To exclude MVP in patients in the absence of physical findings suggestive of MVP or a positive family history- class III 6. Routine repetition of echocardiography in patients with MVP with mild or no regurgitation and no changes in clinical signs or symptoms- class III [1]
Chest X ray [1] To look for the evidence of skeletal abnormalities,such as pectus excavatum which is associated with primary mitral valve prolapse [1]
References
  1. BONOW ROBERTO., CARABELLO BLASE, DE LEON ANTONIOC., EDMUNDS L.HENRY, FEDDERLY BRADLEYJ., FREED MICHAELD., GAASCH WILLIAMH., MCKAY CHARLESR., NISHIMURA RICKA., O’GARA PATRICKT., O’ROURKE ROBERTA., RAHIMTOOLA SHAHBUDINH., RITCHIE JAMESL., CHEITLIN MELVIND., EAGLE KIMA., GARDNER TIMOTHYJ., GARSON ARTHUR, GIBBONS RAYMONDJ., O’ROURKE ROBERTA., RUSSELL RICHARDO., RYAN THOMASJ., SMITH SIDNEYC.. ACC/AHA guidelines for the management of patients with valvular heart disease. Journal of the American College of Cardiology [online] 1998 November, 32(5):1486-1582 [viewed 22 June 2014] Available from: doi:10.1016/S0735-1097(98)00454-9

Management - General Measures

Fact Explanation
Regular exercise [1] A normal lifestyle and regular exercise are encouraged for most patients with mitral valve prolapse (MVP), especially those who are asymptomatic [1]
Restriction from competitive sports [2] Recommended when moderate left ventricular (LV) enlargement, LV dysfunction, uncontrolled tachyarrhythmias, long QT interval, unexplained syncope [2]
Patient education [2] Reassurance- most patients are asymptomatic and not at high risk for serious consequences [3] A familial occurrence of MVP should be explained to the patient and is particularly important in those with associated disease who are at greater risk for complications. There is no contraindication to pregnancy based on the diagnosis of MVP alone [2] Educate about the comlications- Atrial fibrillation and other arrhythmias, Congestive heart failure, Pulmonaryhypertension, Ruptured mitral valve chordae, Infective endocarditis, Central nervous system embolic events [3]
Follwed up with clinical evaluation [2] Asymptomatic patients with MVP and no significant mitral valve prolapse (MR) can be evaluated clinically every 3 to 5 years.Patients who have high-risk characteristics, including those with moderate to severe MR, should be followed once a year [2]
Antibiotic prophylaxis [3] Antibiotic prophylaxis for the prevention of infective endocarditis during procedures that carry a risk for bacteremia is recommended in most patients with a definite diagnosis of MVP [3]
References
  1. DüREN DR, BECKER AE, DUNNING AJ. Long-term follow-up of idiopathic mitral valve prolapse in 300 patients: a prospective study. J Am Coll Cardiol [online] 1988 Jan, 11(1):42-7 [viewed 22 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/3335704
  2. BONOW ROBERTO., CARABELLO BLASE, DE LEON ANTONIOC., EDMUNDS L.HENRY, FEDDERLY BRADLEYJ., FREED MICHAELD., GAASCH WILLIAMH., MCKAY CHARLESR., NISHIMURA RICKA., O’GARA PATRICKT., O’ROURKE ROBERTA., RAHIMTOOLA SHAHBUDINH., RITCHIE JAMESL., CHEITLIN MELVIND., EAGLE KIMA., GARDNER TIMOTHYJ., GARSON ARTHUR, GIBBONS RAYMONDJ., O’ROURKE ROBERTA., RUSSELL RICHARDO., RYAN THOMASJ., SMITH SIDNEYC.. ACC/AHA guidelines for the management of patients with valvular heart disease. Journal of the American College of Cardiology [online] 1998 November, 32(5):1486-1582 [viewed 22 June 2014] Available from: doi:10.1016/S0735-1097(98)00454-9
  3. BOUKNIGHT DP, O'ROURKE RA. Current management of mitral valve prolapse. Am Fam Physician [online] 2000 Jun 1, 61(11):3343-50, 3353-4 [viewed 22 June 2014] Available from: http://www.aafp.org/afp/2000/0601/p3343.html

Management - Specific Treatments

Fact Explanation
Beta blockers [1] Patients with Mitral valve prolapse (MVP) and palpitations associated with mild tachyarrhythmias or increased adrenergic symptoms and those with chest pain, anxiety or fatigue often respond to therapy with beta blockers [1]
Daily aspirin therapy (80 to 325 mg per day) [2] Recommended for patients with MVP who have a history of focal neurologic events and who are in sinus rhythm but have no atrial thrombi. Such patients should also avoid smoking cigarettes and taking oral contraceptives [1]
Long-term anticoagulation therapy with warfarin [1] Recommended for patients with MVP who have had a stroke and those who have recurrent transient ischemic attacks while on aspirin therapy (the International Normalized Ratio [INR] should be maintained between 2 and 3 [1]
Restriction from competitive sports [1] Recommended when moderate left ventricular (LV) enlargement, LV dysfunction, uncontrolled tachyarrhythmias, long QT interval, unexplained syncope [1]
Surgical considerations [3] Management of MVP may require valve surgery, particularly in those patients who develop a flail mitral leaflet due to rupture of chordae tendineae or their marked elongation,Most such valves can be repaired successfully by surgeons experienced in mitral valve repair, especially when the posterior leaflet of the mitral valve is predominantly affected [3] A minimally invasive approach allows repair of almost all degenerative valves with good short-term outcomes in a tertiary referral center, when using proven and efficient surgical techniques [4]
Antibiotic prophylaxis [1] Antibiotic prophylaxis for the prevention of infective endocarditis during procedures that carry a risk for bacteremia is recommended in most patients with a definite diagnosis of MVP [1]
References
  1. BOUKNIGHT DP, O'ROURKE RA. Current management of mitral valve prolapse. Am Fam Physician [online] 2000 Jun 1, 61(11):3343-50, 3353-4 [viewed 22 June 2014] Available from: http://www.aafp.org/afp/2000/0601/p3343.html [1]
  2. DüREN DR, BECKER AE, DUNNING AJ. Long-term follow-up of idiopathic mitral valve prolapse in 300 patients: a prospective study. J Am Coll Cardiol [online] 1988 Jan, 11(1):42-7 [viewed 22 June 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/3335704
  3. BONOW ROBERTO., CARABELLO BLASE, DE LEON ANTONIOC., EDMUNDS L.HENRY, FEDDERLY BRADLEYJ., FREED MICHAELD., GAASCH WILLIAMH., MCKAY CHARLESR., NISHIMURA RICKA., O’GARA PATRICKT., O’ROURKE ROBERTA., RAHIMTOOLA SHAHBUDINH., RITCHIE JAMESL., CHEITLIN MELVIND., EAGLE KIMA., GARDNER TIMOTHYJ., GARSON ARTHUR, GIBBONS RAYMONDJ., O’ROURKE ROBERTA., RUSSELL RICHARDO., RYAN THOMASJ., SMITH SIDNEYC.. ACC/AHA guidelines for the management of patients with valvular heart disease. Journal of the American College of Cardiology [online] 1998 November, 32(5):1486-1582 [viewed 22 June 2014] Available from: doi:10.1016/S0735-1097(98)00454-9
  4. PERIER P, HOHENBERGER W, LAKEW F, BATZ G, DIEGELER A. Rate of repair in minimally invasive mitral valve surgery. Ann Cardiothorac Surg [online] 2013 Nov, 2(6):751-7 [viewed 22 June 2014] Available from: doi:10.3978/j.issn.2225-319X.2013.10.12