We report here the first and a rarest presentation of the AMVT in a 19-year old female patient diagnosed to have double outlet right ventricle, ventricular septal defect (VSD), infundibular stenosis and congenital mitral stenosis (MS). [ncbi.nlm.nih.gov]

• Atrial Septal Defect

  A small atrial septal defect was found at follow up cardiac catheterisation and angiography. Balloon dilatation of the mitral valve is a reasonable alternative to surgical treatment for typical congenital mitral stenosis even in young children. [ncbi.nlm.nih.gov]

  Septal Defects Supplementary ACHD Echo Acquisition Protocol for Atrial Septal Defects The following protocol for echo in adult patients with atrial septal defects (ASDs) is a guide for performing a comprehensive assessment More information Valvular Heart [healthdocbox.com]

  In the patient with Shone's syndrome, nonetheless, palliation was achieved by balloon angioplasty of the aortic coarctation and by creation of an atrial septal defect. [cambridge.org]

  NIH: National Heart, Lung, and Blood Institute Atrial septal defect (Medical Encyclopedia) Bicuspid aortic valve (Medical Encyclopedia) Congenital heart defect corrective surgeries (Medical Encyclopedia) Congenital heart disease (Medical Encyclopedia) [icdlist.com]

• Dysphagia

  Ortner syndrome, dysphagia megalatriensis ) pulmonary embolus (often recurrent) pulmonary infections (e.g. pneumonia, bronchitis ) atrial fibrillation sudden cardiac death from other arrhythmias thrmoboembolic ischemic stroke, including from calcified [radiopaedia.org]

  Thrombus formation in the dilated left atrium (due to stasis of blood) Embolization of left atrial thrombus, stroke Dysphagia from compression of esophagus by the enlarged left atrium Complications of medical treatment include the following: Diuretics [emedicine.medscape.com]

• Diastolic Murmur

  Cardiac auscultation: murmur • low-pitched diastolic rumble that is most prominent at the apex. • Although the intensity of the diastolic murmur does not correlate with the severity of the stenosis, the duration of the murmur is helpful since it reflects [slideshare.net]

  Diastolic murmurs that may coexist with the MS murmur are: Early diastolic murmur of coexisting aortic regurgitation (AR), which may be conducted to the apex Graham Steell murmur (a soft decrescendo diastolic murmur heard best along the left sternal border [merckmanuals.com]

  murmurs, occurring together or individually, heard loudest at the apex; and (3) electrocardiographic and roentgenologic evidence of enlargement of left atrium or left and right atria in combination, and hypertrophy of the right ventricle. [pediatrics.aappublications.org]

  Diastolic Murmurs - Mitral Stenosis You are listening to a typical example of the murmur produced by mitral valve stenosis. As opposed to aortic regurgitation, mitral stenosis has a female preponderance, with the female:male ratio being about 2:1. [med.ucla.edu]

  There is a low frequency mid to late diastolic murmur loudest at the apex, which is accentuated on expiration. The presence of mitral regurgitation should always be sought. [bcm.edu]

• Tachycardia

  Pre medication • To decrease anxiety & any associated likelihood of adverse circulatory responses produced by tachycardia Class Drug Dose (mg/kg) Route BZPs Diazepam 0.1-0.15 PO, IM Lorazepam 0.03-0.06 PO, IM Midazolam 0.03-0.07 IM Opioids Morphine 0.2 [slideshare.net]

  This elevates the left atrial pressure which, in turn, raises pulmonary venous and capillary pressure leading to bouts of DYSPNEA and TACHYCARDIA during physical exertion. RHEUMATIC FEVER is its primary cause. [fpnotebook.com]

  (PSVT)発作性上室性頻拍 paroxysmal ventricular tachycardia (PVT)発作性心室頻拍 patent ductus arteriosus (PDA) 動脈管開存［症］ percardial friction rub心膜摩擦音 percussion 打診［法］、叩打法〈マッサージの一法〉 percutaneous transluminal coronaryangioplasty (PTCA)経皮的冠［状］［動脈］形成［術］ percutaneous transluminal [tokyo-med.ac.jp]

  Mildly symptomatic patients usually respond to diuretics and, if sinus tachycardia or atrial fibrillation is present, to beta-blockers or calcium channel blockers for rate control. [merckmanuals.com]

  Many patients experience symptoms only when the heart rate is elevated, as tachycardia decreases diastolic filling time significantly. [healio.com]

• Cardiomegaly

  CXR: Moderate to severe stenosis is manifested by mild cardiomegaly with pulmonary venous congestion and left atrial enlargement. [bcm.edu]

  · Rheumatic disease causes mitral stenosis in 99.8% of cases · Acute rheumatic fever (ARF) → pancarditis o Mitral valve most commonly involved valve o Followed by mitral and aortic together o Then by aortic alone · Acute phase → cardiomegaly o Mitral [learningradiology.com]

  Eventually, the RV fails, which manifests as hepatomegaly and/or ascites, edema of the extremities, and cardiomegaly on radiography. [emedicine.medscape.com]

  Chest radiograph showed cardiomegaly with prominent pulmonary artery and left atrial shadows. Electrocardiography showed left atrial dilatation with prominent right ventricular forces. Echocardiography showed severe congenital mitral valve stenosis. [jscisociety.com]

• Hypotension

  While overdose cause hypoventilation & hypotension(↑pvr &↓c.o.) exacerbate pulmonary hypertension. [slideshare.net]

  Julian M Stewart, MD, PhD Associate Chairman of Pediatrics, Director, Center for Hypotension, Westchester Medical Center; Professor of Pediatrics and Physiology, New York Medical College Julian M Stewart, MD, PhD is a member of the following medical societies [emedicine.medscape.com]

  症］ hypertensive heart disease高血圧性心疾患 hypertrophic cardiomyopathy (HCM) 肥大型心筋症 hypertrophic nonobstructive cardiomyopathy(HNCM)非閉塞性肥大型心筋症 hypertrophic obstructive cardiomyopathy(HOCM)閉塞性肥大型心筋症 hypertrophy肥大（厚） hypoplastic left heart syndrome 左心低形成症候群 hypotension [tokyo-med.ac.jp]

• Systolic Murmur

  Patients with chronic mitral obstruction develop signs of tricuspid regurgitation and CHF, such as hepatomegaly, engorged neck veins, large expansile CV waves in the jugular venous pulse, and a systolic murmur that accentuates in inspiration at the lower [rjmatthewsmd.com]

  III/IV mid diastolic murmur, early systolic murmur: This heart murmur is caused by an increase blood flow through the tricuspid valve due to ASD; it is heard best in the left lower sternal area or the bottom of the heart (apex). [1] [6] Minor symptoms [en.wikipedia.org]

  ［心］収縮［期］ systolic function収縮機能 systolic murmur (SM)収縮期雑音 t tachycardia 頻拍（脈） tachypnea呼吸頻数、頻呼吸 technetium (Tc)テクネシ（チ）ウム temporal resolution時間分解能 terminal portion終末部 tetralogy of Fallot (T/F, TOF) ファロー四徴［症］ thallium (Tl)タリウム thoracic aorta 胸［部］大動脈 thoractomy [tokyo-med.ac.jp]

• Hepatomegaly

  Eventually, the RV fails, which manifests as hepatomegaly and/or ascites, edema of the extremities, and cardiomegaly on radiography. [emedicine.medscape.com]

  Signs of right ventricular failure including hepatomegaly, ascites and peripheral oedema. Investigations CXR: may show left atrial enlargement and interstitial oedema (Kerley A and B lines) if severe. Mitral valve calcification may be seen. [patient.info]

  Pulmonary Hypertension Backward transmission of the elevated left atrial pressure ↑ Pulmonary artery pressure ↑RAP & development of right sided heart failure RV hypertrophy & enlargement Hepatomegaly & Pulsatile liver Tricuspid regurgitation JVD Parasternal [slideshare.net]

  Palpate the patient’s abdomen for hepatomegaly and auscultate the patient’s lungs for crackles. You may note a normal apical pulse or an irregular rate associated with atrial fibrillation when the heart is auscultated. [medical-dictionary.thefreedictionary.com]

• Renal Insufficiency

  If the reduction in cardiac output is critical, end organ failure with renal and/or hepatic insufficiency, shock, and metabolic acidosis can occur. [emedicine.medscape.com]

• Intracranial Hemorrhage

  Warfarin may cause bleeding, such as intracranial hemorrhage and GI bleeding. [emedicine.medscape.com]

• Irritability

  Fecal transplant is used to treat gut infections and is now being studied as a treatment for obesity, urinary tract infections, irritable bowel syndrome and more. [nytimes.com]

• Ataxia

  […] syndrome Myoclonus-dystonia syndrome Myoclonus epilepsy and ataxia due to potassium channel mutation Myoclonus epilepsy associated with ragged-red fibres Myoclonus epilepsy in non-progressive encephalopathies Myoclonus-nephropathy syndrome Myofibrillar [orpha.net]

Diastolic rumble after opening snap; S 1 is usually load, because the mitral valve remains open by a transient gradient until closed by the systolic force; pulmonary HTN is indicated by a loud P 2, right ventricular lift, ↑ neck veins, ascites, edema Workup [medical-dictionary.thefreedictionary.com]

"Lutembacher Syndrome Workup". ^ a b c d e f g "Atrial Septal Defect (ASD)". Cleveland Clinic. Retrieved May 2, 2014. ^ a b c "Diseases & Conditions". Cleveland Clinic. Retrieved 2 May 2014. ^ a b c d e f g Krasuski, Richard A. (Feb 2007). [en.wikipedia.org]

Assessment of valve anatomy and suitability for PMBV Evaluation of the MV anatomy and pathology is a major aspect of the echocardiographic workup because this identifies the best treatment options. [imaging.onlinejacc.org]

• P Mitrale

  […] the right atrium Splaying of the carina > 90° Signs of pulmonary congestion ; ( see X-ray findings in pulmonary congestion ) Lateral image Dorsal displacement of the esophagus (visible in barium swallow test) Signs of right ventricular hypertrophy ECG P [amboss.com]

  The most common finding is left atrial enlargement (P mitrale), but this finding disappears if the patient enters atrial fibrillation. Right heart strain may produce findings of right axis deviation and right ventricular hypertrophy on ECG. [healio.com]

Clinical trials often give patients access to leading-edge treatments that are not yet widely available. [utswmedicine.org]

Many children with congenital heart defects don't need treatment, but others do. Treatment can include medicines, catheter procedures, surgery, and heart transplants. [icdlist.com]

Balloon dilatation of the mitral valve is a reasonable alternative to surgical treatment for typical congenital mitral stenosis even in young children. [ncbi.nlm.nih.gov]

Daubeney, offers essential guidance on the anatomical issues, clinical presentation, diagnosis, and treatment options available to practitioners today. [books.google.de]

CONCLUSIONS: Patients with supramitral ring constitute a subset of patients with congenital mitral stenosis who have a relatively good prognosis. [ncbi.nlm.nih.gov]

This technology is particularly suited for congenital heart disease in which there is a clear need for more clear and accurate delineation of the congenital heart defects from a 3-dimensional perspective for diagnosis, assessment, and prognosis of these [books.google.de]

Prognosis Untreated newborns with severe MS have a grim prognosis. Surgical intervention is ideally avoided for as long as possible. [emedicine.medscape.com]

The prognosis is much improved in patients who undergo surgical or percutaneous relief of valve obstruction. [ 1 ] However, life expectancy is still shortened compared with that expected for age, largely because of the complications of mitral stenosis [patient.info]

Etiology In addition to being a sequela of rheumatic fever, which is the most common cause world-wide, there are numerous other causes 2-4 : mitral annular calcification with leaflet involvement an age-related cause congenital mitral stenosis infective [radiopaedia.org]

Etiology Most commonly due to rheumatic fever Autoimmune diseases: systemic lupus erythematosus, rheumatoid arthritis Rare inherited metabolic diseases: mucopolysaccharidoses of the Hunter-Hurler phenotype, Fabry disease Congenital Some conditions may [amboss.com]

Other uncommon etiologies include congenital stenosis of the mitral valve or age-related calcification. Overview Stenosis of the mitral valve considerably increases the resistance to blood flow between the left atrium and the left ventricle. [pathwaymedicine.org]

Etiology The etiology of congenital MS remains unknown. However, prevalence of MS in offspring of family members (especially the mother) with left ventricular outflow tract obstruction is increased. [emedicine.medscape.com]

Google Scholar Morton WE, Huhn LA, Litchy JA: Rheumatic heart disease epidemiology: observations in 17,366 Denver School Children. JAMA. 1967, 199: 879-884. 10.1001/jama.1967.03120120067009. [bmccardiovascdisord.biomedcentral.com]

Epidemiology United States data Congenital MS is rare, occurring in 0.5% of patients with congenital heart disease (CHD). Race-, sex-, and age-related demographics No racial or sex predilection is known in congenital MS. [emedicine.medscape.com]

Grasp the full scope of the pathophysiology of CHD with well-organized, expert guidance. Quickly search the contents online, download images in JPG or PPT format, and view 70 echocardiogram videos at expertconsult.com. [books.google.de]

Pathophysiology MS (75-80 have associated lesions) MR MSR 3 Pathophysiology of Congenital Mitral Valve Disease Malformation of the mitral valve apparatus results in mitral stenosis, insufficiency, or both. [powershow.com]

[…] metabolic diseases: mucopolysaccharidoses of the Hunter-Hurler phenotype, Fabry disease Congenital Some conditions may mimic mitral stenosis: bacterial endocarditis of the mitral valve with large vegetation, left atrial myxoma Degenerative aortic stenosis Pathophysiology [amboss.com]

However, increased LAP results in a number of pathophysiological consequences described below. [pathwaymedicine.org]

Source(s): Mitral Stenosis Pathophysiology The normal mitral valve orifice area is 4-6 cm 2. Mitral stenosis occurs when the orifice area is reduced to at least 2.2 cm 2. [wikiecho.org]

Prevention Follow your provider's recommendations for treating conditions that can cause valve disease. Treat strep infections promptly to prevent rheumatic fever. Tell your provider if you have a family history of congenital heart diseases. [mountsinai.org]

Cardiac rehabilitation is a critical component of recovery and can prevent future heart disease. [utswmedicine.org]

Rheumatic fever is very rare in this country due to the use of effective antibiotics to prevent infections. [heart.org]

Prevention Prevention of rheumatic fever. Prevention of endocarditis. British Heart Foundation Carabello BA ; Modern management of mitral stenosis. Circulation. 2005 Jul 19112(3):432-7. [patient.info]

Once the left ventricle is filled it contracts, to pump the blood out to the body — and the mitral valve closes to prevent the blood from going backwards into the left atrium. [verywell.com]