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Double Orifice Mitral Valve Clinical Presentation

  • Author: Georgios A Hartas, MD; Chief Editor: Stuart Berger, MD  more...
 
Updated: Mar 19, 2014
 

History

Double orifice mitral valve (DOMV) without mitral regurgitation or mitral stenosis are asymptomatic. Physical findings may only exist if mitral regurgitation or mitral stenosis are present.

The severity of symptoms depends on the degree of left atrial hypertension. Dyspnea, nocturnal cough, and tachypnea occur, related to pulmonary venous congestion and increased lung stiffness. Frequent respiratory infections and wheezing occur, secondary to pulmonary congestion, increased fluid exudation, and airway narrowing. Poor feeding, failure to thrive, fatigue and sweating occur because of heart failure and reduced cardiac output. On occasion, a child with double orifice mitral valve presents with acute pulmonary edema or generalized edema. Hemoptysis and syncope can occur in older patients with double orifice mitral valve.

Double orifice mitral valve is detected in one of 3 ways. As an associated lesion with other congenital heart defects, especially in the presence of atrioventricular (AV) septal defect or left-sided obstructive lesions. The abnormal mitral valve aggravates the pulmonary congestion and heart failure that occurs in complete AV septal defect or ventricular septal defects (VSDs). In the converse, conditions such as large left-to-right atrial shunts and tetralogy of Fallot with reduced mitral valve flow may mask the presence of double orifice mitral valve. In patients with such conditions, double orifice mitral valve is not detected unless the clinician specifically looks for it.

Double orifice mitral valve may be sought as the anatomic explanation for symptomatic mitral valve disease or discovered as an incidental finding in asymptomatic patients who undergo echocardiography for any reason.[18]

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Physical

Physical signs in DOMV with mitral stenosis

Respiratory distress, tachypnea, and subcostal retractions. Reduced pulse volume and peripheral cyanosis indicate diminished cardiac output and poor tissue perfusion. Central cyanosis can develop in the presence of severe pulmonary edema. Jugular venous pressure rises with the onset of right heart failure. Palpation reveals a parasternal heave from the hypertrophied RV and occasionally, a diastolic thrill at the apex.

The first heart sound may be normal or accentuated, while the pulmonary second sound generally is loud because of pulmonary hypertension. Unlike acquired mitral stenosis, an opening snap is not commonly heard.

A low-pitched mid-diastolic murmur of varying intensity is audible at the mitral area. It is often heard best with the patient in the left lateral decubitus position, and it is especially loud when mitral stenosis is associated with a VSD or mitral regurgitation.

In chronic, severe mitral stenosis, signs of tricuspid incompetence, such as systolic expansile pulsation in the jugular vein and liver and a pansystolic murmur at the lower sternal border, appear. This murmur is typically accentuated on inspiration.

Physical signs in DOMV with mitral regurgitation

The patient may present with respiratory distress and pulmonary edema. The pulse is often brisk. The apical impulse is displaced downward and outward and has a hyperdynamic quality because of LV dilatation and hypertrophy. The first and second heart sounds are usually normal in intensity, although the second heart sounds may be widely split. A third heart sound is commonly heard at the apex. A blowing pansystolic murmur is heard at or just inside the apex. It is often conducted toward the sternum rather than toward the axilla.

Severe mitral regurgitation can cause a low-pitched, apical diastolic murmur from large diastolic flow across the mitral valve. Diastolic murmur at apex of the heart (functional MS murmur). Pulmonary hypertension and tricuspid incompetence can occur in MR, though not as commonly or as severely as in MS.

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Contributor Information and Disclosures
Author

Georgios A Hartas, MD Pediatric Interventional Cardiologist, The Children's Heart Institute

Georgios A Hartas, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions, Texas Medical Association, Texas Pediatric Society, Harris County Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

P Syamasundar Rao, MD Professor of Pediatrics and Medicine, Division of Cardiology, Emeritus Chief of Pediatric Cardiology, University of Texas Medical School at Houston and Children's Memorial Hermann Hospital

P Syamasundar Rao, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, American College of Cardiology, American Heart Association, Society for Cardiovascular Angiography and Interventions, Society for Pediatric Research

Disclosure: Nothing to disclose.

Duraisamy Balaguru, MBBS MRCP, FACC, FAAP, FSCAI, Associate Professor of Pediatrics, Division of Pediatric Cardiology, University of Texas Medical School at Houston; Attending Physician, Division of Pediatric Cardiology, Children’s Memorial Hermann Hospital

Duraisamy Balaguru, MBBS is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Society for Cardiovascular Angiography and Interventions, American Stroke Association, Pediatric Cardiac Intensive Care Society, International Society of Invasive Cardiology in Congenital Heart Disease, World Society for Pediatric and Congenital Heart Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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: American Academy of Pediatrics, American Autonomic Society, American Physiological Society

Disclosure: Received grant/research funds from Lundbeck Pharmaceuticals for none.

Chief Editor

Stuart Berger, MD Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

Juan Carlos Alejos, MD Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California, Los Angeles, David Geffen School of Medicine

Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, International Society for Heart and Lung Transplantation

Disclosure: Received honoraria from Actelion for speaking and teaching.

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Two-dimensional echocardiogram (apical view) in a patient with duplicate mitral valve. Two mitral valves can be seen opening into the left ventricle. Each valve has a separate annulus, and a separate set of mitral valve leaflets and subvalvar apparatus.
Two-dimensional echocardiogram (parasternal short-axis view) shows double-orifice mitral valve, the orifice being divided by a bridge of tissue.
Two-dimensional echocardiogram of a double-orifice mitral valve (apical view) with color flow mapping, which shows diastolic flow through 2 separate orifices.
Two-dimensional echocardiogram (parasternal short-axis view) in a patient with duplicate mitral valve. This diastolic frame shows two typical mitral valve orifices opening into the left ventricle. The two orifices are placed apart unlike the more common type of double-orifice mitral valve (see image above) where the orifice is divided by a bridge of tissue.
Real-time two-dimensional echocardiogram (apical view) in a patient with duplicate mitral valve. Two mitral valves can be seen opening into the left ventricle. Each valve has a separate annulus, and a separate set of mitral valve leaflets and subvalvar apparatus.
Real-time two-dimensional echocardiogram (subcostal short-axis view) in a patient with duplicate mitral valve showing two typical mitral valves opening into the left ventricle. The two orifices are placed apart unlike the more common type of double-orifice mitral valve (see image above) where the orifice is divided by a bridge of tissue.
 
 
 
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