Presentation
Acronym CVDX Synonyms Congenital valvular dysplasia Congenital valvular heart disease CVD1 X-linked myxomatous valvular dystrophy XMVD Disclaimer Any medical or genetic information present in this entry is provided for research, educational and informational [uniprot.org]
Other more rare anomalies that present within the right atrium, namely cor triatriatum dextrum and the Chiari network, are also detailed. [mayoclinic.pure.elsevier.com]
A heart murmur is the most common sign detected by a physician indicating that a valve problem may be present. [cincinnatichildrens.org]
Later presenting symptoms typically include dyspnea, chestpain and rarely syncope from acute reduction in cardiac output. Increasing fatigue and decreasing exercise tolerance however is probably the commonest presentation. [bcm.edu]
Stenosis - when the valve doesn't open enough and blocks blood flow Valve problems can be present at birth or caused by infections, heart attacks, or heart disease or damage. [icdlist.com]
Entire Body System
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Fatigue
Increasing fatigue and decreasing exercise tolerance however is probably the commonest presentation. Sudden death is a risk with severe outflow obstruction and competitive exercise is contraindicated. [bcm.edu]
Signs and symptoms of severe defects in newborns include Rapid breathing Cyanosis - a bluish tint to the skin, lips, and fingernails Fatigue Poor blood circulation Many congenital heart defects cause few or no signs and symptoms. [icdlist.com]
When symptoms occur, they often develop gradually, and may include: Cough Fatigue, exhaustion, and lightheadedness Rapid breathing Sensation of feeling the heart beat ( palpitations ) or a rapid heartbeat Shortness of breath that increases with activity [baptistjax.com]
In an older child, severe pulmonary valve stenosis may cause easy fatigue or shortness of breath with physical exertion. Severe pulmonary valve stenosis rarely results in right ventricular failure or sudden death. [cincinnatichildrens.org]
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Congestive Heart Failure
Showing of 9 Percent of people who have these symptoms is not available through HPO Abnormality of metabolism/homeostasis Laboratory abnormality Metabolism abnormality [ more ] 0001939 Aortic regurgitation 0001659 Congestive heart failure Cardiac failure [rarediseases.info.nih.gov]
Heart Failure LeftSided 307 ConjunctivitisCats 311 Contact Dermatitis 317 Coprophagia and Pica 324 Corneal SequestrumCats 330 Cryptococcosis 336 Cutaneous Drug Eruptions 342 Cysticercosis 348 client education handouts are available at www fiveminutevet [books.google.com]
This may lead to congestive heart failure. Mitral regurgitation may begin suddenly. This often occurs after a heart attack. When the regurgitation does not go away, it becomes long-term (chronic). [baptistjax.com]
Long-term results after aortic valve replacement in patients with congestive heart failure. Homografts vs prosthetic valves. Eur Heart J. 2000 Oct. 21(20):1698-707. [Medline]. Harris KM, Malenka DJ, Haney MF, et al. [emedicine.medscape.com]
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Physician
In-Depth Information The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. [rarediseases.info.nih.gov]
A heart murmur is the most common sign detected by a physician indicating that a valve problem may be present. [cincinnatichildrens.org]
Am Fam Physician. 1995 Aug. 52(2):559-68. [Medline]. Levine HJ, Gaasch WH. Vasoactive drugs in chronic regurgitant lesions of the mitral and aortic valves. J Am Coll Cardiol. 1996 Nov 1. 28(5):1083-91. [Medline]. [emedicine.medscape.com]
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Inflammation
Other rare complications of X-linked cardiac valvular dysplasia include inflammation of the inner lining of the heart (endocarditis), abnormal blood clots, or sudden death. [ghr.nlm.nih.gov]
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Weakness
In X-linked cardiac valvular dysplasia, the mitral or aortic valve can also be prolapsed, which means that the valve is weak or floppy. Valve prolapse further prevents the thickened valve from closing properly and can lead to valve regurgitation. [ghr.nlm.nih.gov]
Respiratoric
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Dyspnea
Later presenting symptoms typically include dyspnea, chestpain and rarely syncope from acute reduction in cardiac output. Increasing fatigue and decreasing exercise tolerance however is probably the commonest presentation. [bcm.edu]
They are manifested as chest discomfort, dyspnea, and hypotension. Epidemiology Frequency United States An estimated 5 million Americans have at least one form of heart valve disease. [emedicine.medscape.com]
Gastrointestinal
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Regurgitation
Cardiac failures Heart failure [ more ] 0001635 Mitral regurgitation 0001653 Mitral valve prolapse 0001634 Short chordae tendineae of the mitral valve 0011580 Short chordae tendineae of the tricuspid valve 0006692 Tricuspid regurgitation 0005180 X-linked [rarediseases.info.nih.gov]
Valve prolapse further prevents the thickened valve from closing properly and can lead to valve regurgitation. [ghr.nlm.nih.gov]
Definition A rare X-linked heart disease characterized by mitral and/or aortic valve regurgitation. [uniprot.org]
Ebstein's anomaly, tricuspid valve dysplasia) with severe tricuspid regurgitation and cardiomegaly is associated with poor prognosis. [scholars.northwestern.edu]
There have been very few re-operations for recurrent tricuspid valve regurgitation and nearly 100% survival. [childrenshospital.org]
Cardiovascular
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Heart Disease
Definition A rare X-linked heart disease characterized by mitral and/or aortic valve regurgitation. [uniprot.org]
Title Other Names: CVD1; Valvular heart disease, congenital; Myxomatous valvular dystrophy, X-linked; CVD1; Valvular heart disease, congenital; Myxomatous valvular dystrophy, X-linked; XMVD See More Categories: This table lists symptoms that people with [rarediseases.info.nih.gov]
A characteristic of X-linked inheritance is that fathers cannot pass X-linked traits to their sons. congenital valvular heart disease CVD1 filamin-A-associated myxomatous mitral valve disease filamin-A-related myxomatous mitral valve dystrophy X-linked [ghr.nlm.nih.gov]
[…] defect (Medical Encyclopedia) [ Read More ] Heart Valve Diseases Also called: Valvular heart disease Your heart has four valves. [icdlist.com]
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Heart Failure
Showing of 9 Percent of people who have these symptoms is not available through HPO Abnormality of metabolism/homeostasis Laboratory abnormality Metabolism abnormality [ more ] 0001939 Aortic regurgitation 0001659 Congestive heart failure Cardiac failure [rarediseases.info.nih.gov]
In the adult, surgery, indicated for heart failure resistant to all medical treatment, is as for other organic tricuspid incompetence, annuloplasty or valve replacement, by mechanical or bioprosthesis. [ncbi.nlm.nih.gov]
This may lead to congestive heart failure. Mitral regurgitation may begin suddenly. This often occurs after a heart attack. When the regurgitation does not go away, it becomes long-term (chronic). [baptistjax.com]
Chondrosarcoma Nasal and Paranasal Sinus 268 Cirrhosis and Fibrosis of the Liver 274 Coagulopathy of Liver Disease 281 Coccidiosis 287 Colitis and Proctitis 293 Compulsive DisordersDogs 299 Congenital Spinal and Vertebral Malformations 305 Congestive Heart [books.google.com]
Once heart failure develops, 50% of uncorrected patients die within 2 years. Compare this with an approximate 1-5% mortality rate in patients with surgical correction. Race No racial predilection is reported. [emedicine.medscape.com]
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Cardiomegaly
The early gestational onset of cardiomegaly with bilateral lung compression raised the possibility of severe lung hypoplasia with decreased broncho-alveolar development. [scholars.northwestern.edu]
A radiographic study was conducted as an adjunct to physical examination, by which, generalized cardiomegaly, dorsal displacement of the trachea, lung radiopacity with alveolar pattern is observed, plus an echocardiogram in which ventricular septal defect [ri.uaemex.mx]
congenital Cardiomegaly, congenital (enlarged heart, at birth) Chiari's network Cleft leaflet of mitral valve Cleft leaflet of tricuspid valve Commissural fusion of aortic cusp Commissural fusion of aortic valve Common atrioventricular valve stenosis [icd9data.com]
There is marked cardiomegaly with the left ventricle apex displaced to the left chest wall. The left atrium displaces the esophagus to the right. The pulmonary vascular markings are normal. B. [bcm.edu]
Cyanosis, cardiomegaly and right ventricular failure in the neonatal period raised the differential diagnosis of Ebstein's anomaly and Uhl's disease. The outcome was fatal in several days to weeks. Autopsy confirmed the diagnosis. [ncbi.nlm.nih.gov]
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Heart Murmur
The main sign of heart valve disease is an unusual heartbeat sound called a heart murmur. Your doctor can hear a heart murmur with a stethoscope. But many people have heart murmurs without having a problem. [icdlist.com]
These malformed valves can cause abnormal blood flow and an irregular heart sound during a heartbeat (heart murmur). The signs and symptoms of X-linked cardiac valvular dysplasia vary greatly among affected individuals. [ghr.nlm.nih.gov]
The diagnosis of pulmonary stenosis is usually first suspected because a physician detects a heart murmur. The heart murmur of pulmonary stenosis is a turbulent noise caused by ejection of blood through the obstructed valve. [cincinnatichildrens.org]
murmur Crackles in the lungs (if fluid backs up into the lungs) The physical exam may also reveal: Ankle and leg swelling Enlarged liver Bulging neck veins Other signs of right-sided heart failure The following tests may be done to look at the heart [baptistjax.com]
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Mitral Valve Prolapse
valve prolapse 0001634 Short chordae tendineae of the mitral valve 0011580 Short chordae tendineae of the tricuspid valve 0006692 Tricuspid regurgitation 0005180 X-linked recessive inheritance 0001419 Showing of 9 Last updated: 5/1/2019 Making a diagnosis [rarediseases.info.nih.gov]
For more information: mitral valve prolapse Mitral Stenosis Mitral stenosis is a narrowing or blockage of the mitral valve, which causes blood to back flow into the left atrium instead of flowing down into the left ventricle. [my.clevelandclinic.org]
If they don't, you could have Regurgitation - when blood leaks back through the valve in the wrong direction Mitral valve prolapse - when one of the valves, the mitral valve, has "floppy" flaps and doesn't close tightly. [icdlist.com]
You are at risk for mitral valve regurgitation if you have: Coronary heart disease and high blood pressure Infection of the heart valves Mitral valve prolapse (MVP) Rare conditions, such as untreated syphilis or Marfan syndrome Rheumatic heart disease [baptistjax.com]
Eyes
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Prolapse
In X-linked cardiac valvular dysplasia, the mitral or aortic valve can also be prolapsed, which means that the valve is weak or floppy. Valve prolapse further prevents the thickened valve from closing properly and can lead to valve regurgitation. [ghr.nlm.nih.gov]
[…] of metabolism/homeostasis Laboratory abnormality Metabolism abnormality [ more ] 0001939 Aortic regurgitation 0001659 Congestive heart failure Cardiac failure Cardiac failures Heart failure [ more ] 0001635 Mitral regurgitation 0001653 Mitral valve prolapse [rarediseases.info.nih.gov]
Aortic Valve Prolapse Severe, uncontrolled high blood pressure has been associated with aortic valve prolapse. [my.clevelandclinic.org]
If they don't, you could have Regurgitation - when blood leaks back through the valve in the wrong direction Mitral valve prolapse - when one of the valves, the mitral valve, has "floppy" flaps and doesn't close tightly. [icdlist.com]
In this condition, the annulus is usually enlarged, and the prolapsing A2 scallop can be resected [Figure 3] and the defect edges reapproximated. [annalspc.com]
Psychiatrical
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Suggestibility
Results —Heritability of TVD in this population of dogs was found to be 0.71, a value sufficiently large to suggest a segregating major locus. [doi.org]
Other features suggestive of this type of pulmonary stenosis were slow body growth, abnormal facies, and a positive family history of pulmonary stenosis. [circ.ahajournals.org]
Echocardiography, in our case, showed dilatation of the right ventricle and pulsed Doppler echocardiography suggested tricuspid incompetence. [ncbi.nlm.nih.gov]
Before any surgery is planned, the doctors may suggest medicines to help the heart work better if it is already coping poorly. Surgery if it is necessary is aimed at making the tricuspid valve less leaky and normalising the flows in the heart. [gosh.nhs.uk]
Treatment
It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. Our staff consists of biologists and biochemists that are not trained to give medical advice . [uniprot.org]
You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments. [rarediseases.info.nih.gov]
If treatment is required, a procedure called a balloon valvuloplasty is often the recommended treatment option in children and younger people. [nhs.uk]
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]
TREATMENT: --In the newborn, the rare surgical attempts at repair have all failed. [ncbi.nlm.nih.gov]
Prognosis
Fetal echocardiography can accurately measure right atrial enlargement, which is associated with a poor prognosis in the fetus with tricuspid valve disease. [scholars.northwestern.edu]
You may need surgery to repair or replace the valve if: Heart function is poor The heart becomes enlarged (dilated) Symptoms get worse Outlook (Prognosis) The outcome varies. [baptistjax.com]
Etiology
Pathophysiology Regardless of etiology, aortic valve insufficiency results in volume overload on the LV because the LV is forced to pump the entire diastolic volume received from the left atrium and the regurgitant volume from the aorta through an incompetent [emedicine.medscape.com]
Epidemiology
Epidemiology Frequency United States An estimated 5 million Americans have at least one form of heart valve disease. In 2000, 1 million Americans were affected by congenital heart disease. This is a 3-fold increase from 300,000 in 1980. [emedicine.medscape.com]
Pathophysiology
Tricuspid valve anomalies are part of the pathological spectrum in various congenital heart diseases, including Ebstein anomaly and tricuspid valve dysplasia, with different pathophysiology and clinical implications. [books.google.com]
Pathophysiology A systolic pressure gradient between the pumping chamber and its downstream artery is the primary hemodynamic abnormality of stenosis regardless of whether the obstruction is right- or left-sided. [pedsinreview.aappublications.org]
Pathophysiological mechanisms of atrial fibrillation: a translational appraisal. Physiol Rev 2011 ; 91 : 265 – 325. 5. Apostolakis, E, Shuhaiber, JH. The surgical management of giant left atrium. Eur J Cardiothorac Surg 2008 ; 33 : 182 – 190. 6. [cambridge.org]
Pathophysiology Regardless of etiology, aortic valve insufficiency results in volume overload on the LV because the LV is forced to pump the entire diastolic volume received from the left atrium and the regurgitant volume from the aorta through an incompetent [emedicine.medscape.com]
Prevention
Valve prolapse further prevents the thickened valve from closing properly and can lead to valve regurgitation. [ghr.nlm.nih.gov]
Treat strep infections quickly to prevent rheumatic fever. Always tell your provider and dentist if you have a history of heart valve disease or congenital heart disease before treatment. [baptistjax.com]
Endocarditis Prevention If you have PS, you don't need to take antibiotics to prevent endocarditis unless you've had prior endocarditis or you've had valve replacement. See the section on Endocarditis for more information. [heart.org]
In this condition one or both of the valve leaflets is enlarged or floppy, preventing the valve from closing evenly. When the valve shuts, the leaflets bulge into the left atrium. [my.clevelandclinic.org]
Completely revised and updated with 80 all-new chapters covering the most important information on current diagnostic, treatment, and preventive challenges facing feline practitioners today, Consultations in Feline Internal Medicine is an invaluable addition [books.google.com]