AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Background

Double-orifice mitral valve (DOMV) is an uncommon anomaly of surgical importance characterized by a mitral valve with a single fibrous annulus with 2 orifices opening into the left ventricle (LV). Subvalvular structures, especially the tensor apparatus, invariably show various degrees of abnormality. Although DOMV may allow normal hemodynamic flow between the left atrium and LV, it can substantially obstruct mitral-valve inflow or cause mitral-valve incompetence.

Surgical intervention is necessary when stenosis or incompetence is severe or if repair of an associated cardiac lesion is needed. Selected cases of stenotic DOMV are amenable to nonsurgical balloon valvuloplasty. Recognition of DOMV and awareness of the anatomic variations are important to achieve good therapeutic results.

Pathophysiology

The normal mitral valve consists of a large, central orifice located between a large sail-like anterior leaflet and a small, C-shaped posterior leaflet, whereas in DOMV, abnormal tissue divides the orifice is divided into 2 parts.

The following 3 major types of DOMV are recognized:

• Eccentric or hole type: This is the most common variety of DOMV (accounting for about 85%), is characterized by a small accessory orifice situated at either the anterolateral or posteromedial commissure. Other anomalies of the valve apparatus, such as cleft leaflets, accessory papillary muscles, fused papillary muscles, and crossing chordae tendineae, are commonly present. When the accessory orifice is located at the posteromedial commissure, a common atrioventricular canal is usually present. By comparison, the atrioventricular canal is normally divided if the accessory orifice is located at the anterolateral commissure.
• Central or bridge type: In about 15% of patients with DOMV, a central bridge of fibrous or abnormal leaflet tissue connects the 2 leaflets of the mitral valve, dividing the orifice into medial and lateral parts. These 2 openings may be equal or unequal, and the papillary muscles are usually normal, with chordae surrounding each orifice inserting into one papillary muscle. In this type of DOMV, dilatation of the posteromedial orifice is feasible by means of balloon valvuloplasty.
• Duplicate mitral valve: This condition involves 2 mitral-valve annuli and valves, each with its own set of leaflets, commissures, chordae, and papillary muscles.

The combined area of DOMV in the presence of an atrioventricular canal defect is 85-90% of the normal expected area. With a near-normal total valve, the area the flow remains adequate both at rest and during exercise. However, in the absence of an associated atrioventricular septal defect (AVSD), the area may be substantially less than normal. Abnormal structure, including large bridging tissue, bulky abnormal leaflets, fused chordae, or abnormal papillary muscles, reduce the effective area of the valve. Abnormalities in the leaflets include thickening, fusion, perforations, restricted movements, and ruptured chordae with flail cusps. Such valves can result in clinically significant degrees of mitral incompetence.

Associated congenital heart defects are common, though DOMV can occur as an isolated anomaly. The most common associated lesion is atrioventricular septal defect (endocardial cushion defect). Other lesions include VSD, coarctation of aorta, interrupted aortic arch, subaortic stenosis, patent ductus arteriosus, and primum atrial septal defect. On occasion, DOMV is observed with secundum atrial septal defect, tetralogy of Fallot, hypoplastic left heart syndrome, left superior vena cava, unroofed coronary sinus, Ebstein anomaly of the tricuspid valve, dysplastic tricuspid valve, double-orifice tricuspid valve, parachute mitral valve, flail mitral-valve leaflet, truncus arteriosus, pulmonary stenosis, bicuspid aortic valve, or left ventricular noncompaction. DOMV has also been reported in patients presenting with atrial tachycardia or congenital complete heart block.

The mitral valve can function reasonably well in about 50% of patients with DOMV. In the other 50%, it can cause clinically significant mitral stenosis or mitral regurgitation.

Mitral stenosis

A reduction in the total area of the mitral valve obstructs its inflow. When mitral stenosis is clinically significant, a diastolic pressure difference builds up between the left atrium and the LV. The rise in left atrial and pulmonary venous pressure leads to exudation of fluid into the interstitium of the lung and increased pulmonary stiffness (the main cause of breathlessness). In severe cases, frank pulmonary edema develops.

Persistent pulmonary venous hypertension leads to pulmonary arterial hypertension, a rise in pulmonary vascular resistance, and, eventually, failure of the right ventricle (RV) with tricuspid regurgitation.

Mitral regurgitation

About 25% of patients with DOMV present with mitral regurgitation as the dominant hemodynamic abnormality, especially when it is associated with an atrioventricular canal defect. Similar to mitral stenosis, mitral regurgitation causes left atrial and pulmonary venous hypertension. Clinical effects depend on the severity and duration of mitral regurgitation.

LV outflow obstruction from associated subaortic stenosis or coarctation can aggravate mitral regurgitation. With increasing mitral regurgitation, cardiac output is maintained by increasing LV diastolic volume and hypertrophy. In chronic mitral regurgitation, LV function deteriorates, leading to further worsening of pulmonary venous congestion, pulmonary hypertension, and right heart failure. Ventricular dysfunction secondary to chronic dilatation is often irreversible even after the primary mitral lesion is corrected. The left atrium tends to markedly enlarge in mitral regurgitation and may cause pressure effects on the left bronchus.

Frequency

International

To the author's knowledge, no data are available on the incidence of DOMV. Approximately 50% of all cases of DOMV are detected during an investigation of other congenital heart diseases (CHDs). More than 100 cases of DOMV are reported in the English literature.

CLINICAL

Section 3 of 11  

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• Clinical
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• Workup
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• Medication
• Follow-up
• Miscellaneous
• Multimedia
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History

• DOMV is detected in one of 3 ways:
• • DOMV is most commonly diagnosed as an associated lesion with other congenital heart defects, especially in the presence of AVSD or left-sided obstructive lesions. The abnormal mitral valve aggravates the pulmonary congestion and heart failure that occurs in common atrioventricular canal or VSD. In the converse, conditions such as tetralogy of Fallot and large left-to-right atrial shunts reduce mitral-valve flow and mask the presence of DOMV. In patients with such conditions, DOMV is not detected unless the clinician specifically looks for it.
  • DOMV may be detected as the anatomic cause when children or young adults are evaluated for symptomatic mitral-valve disease. However, symptoms due to a flail leaflet in DOMV have been reported even in an elderly patients.
  • On occasion, DOMV is an incidental finding in asymptomatic patients who undergo echocardiography for any reason.
• Symptoms of DOMV: The severity of symptoms depends on the degree of left atrial hypertension. Common symptoms include one or more of the following:
• • Dyspnea, nocturnal cough, and tachypnea occur from pulmonary venous congestion and increased lung stiffness.
  • Frequent respiratory infections and wheezing occur from 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 DOMV presents with acute pulmonary edema or generalized edema.
  • Hemoptysis and syncope can occur in older patients with DOMV.

Physical

• Physical signs in DOMV with mitral stenosis
• • Children with severe mitral stenosis often are ill, with respiratory distress, tachypnea, and subcostal recession.
  • Reduced pulse volume and peripheral cyanosis indicate diminished cardiac output and poor tissue perfusion.
  • Central cyanosis can develop in the presence of 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 child with severe mitral regurgitation can 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 hypertrophy.
  • The first and second heart sounds are usually normal in intensity, though 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.
  • Pulmonary hypertension and tricuspid incompetence can occur in mitral regurgitation, though not as commonly or as severely as in mitral stenosis.

DIFFERENTIALS

Section 4 of 11  

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• Clinical
• Differentials
• Workup
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• Medication
• Follow-up
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• Multimedia
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Other Problems to be Considered

Mitral stenosis, valvar
Pulmonary hypertension, CHD

WORKUP

Section 5 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Lab Studies

• Investigate to quantify the hemodynamic abnormality and to define the anatomy of the valve before undertaking surgical treatment.
• No specific laboratory blood tests are required for diagnosis.

Imaging Studies

• Chest radiography
• • Diagnose left atrial enlargement (the most common abnormality in DOMV) if straightening of the left upper cardiac border (mitralization), widening of the tracheal carina, and elevation of the left bronchus are present. In older children, the enlarged left atrium can be observed as a double opacity near the right atrial border that tends to enlarge in a posterior direction. Barium-swallow study of the esophagus in lateral projection shows a rounded indentation of the anterior wall. Mitral incompetence, if severe, causes LV enlargement.
  • Prominent upper-lobe veins, increased interstitial markings, and Kerley lines indicate pulmonary venous hypertension. In severe cases, alveolar edema produces a hazy appearance in the hilar regions of both lung fields. The pulmonary trunk and its branches become dilated with the rise in pulmonary arterial pressure.
• Echocardiography
  
  
  • Two-dimensional echocardiography with Doppler imaging is the most important tool for the diagnosis and detailed assessment of DOMV.
  • Systematically examine the mitral valve by using multiple views for imaging and Doppler interrogation. Pay particular attention to evaluate all components of the mitral-valve apparatus.
  • The 2 orifices in DOMV are best observed on a cross-sectional view of the LV in its short axis by scanning the ventricle from the apex to the base. Apical and subcostal 4-chamber views are also useful to visualize the subvalvular apparatus.
  • Identify the relative positions of the 2 openings and measure the area. Carefully study the nature and size of the bridging tissue and leaflets, as well as the movements of the valve cusps. Any associated congenital heart defect may also be identified and quantified.
  • Look for and measure the enlargement of the left atrium, LV, RV, and pulmonary artery on 2-dimensional echocardiography.
  • M-mode echocardiography of the pulmonary valve often shows signs of pulmonary hypertension such as an abbreviated a wave, midsystolic closure, and systolic flutter of the pulmonary leaflets.
  
  
  • Real-time 3-dimensional echocardiography also allows comprehensive noninvasive assessment of the anatomical details in patients with DOMV and provides incremental information to that obtained by 2-dimensional echocardiography.
• Doppler echocardiography
• • Doppler study and color flow mapping are useful to show the pattern of flow through the mitral valve and to identify the exact site of regurgitation.
  • Assess the severity of mitral regurgitation in a semiquantitative manner from the area of the regurgitant jet in the left atrium.
  • The severity of mitral obstruction can be quantified by measuring the mean velocity of diastolic flow through the mitral valve. The mean diastolic velocity and the pressure half-time (time for the peak diastolic velocity to decrease to half its initial value) are well correlated with the severity of mitral stenosis.
  • Obtain a good estimate of the systolic pressure in the pulmonary artery by measuring the peak velocity of the tricuspid regurgitant jet in the right atrium.
• Transesophageal echocardiography
• • In children, transesophageal echocardiography is generally not necessary to assess DOMV because adequate information can be obtained by using the transthoracic and subcostal windows.
  • In adults, transesophageal study can enable clear visualization of all valve components. A deep transgastric view with Doppler interrogation is often useful to show the 2 orifices and the precise origin of the regurgitant jet.
  • Thrombi in the left atrium can be detected.
  • In patients of all ages, intraoperative transesophageal echocardiography is extremely useful to assess the adequacy of mitral-valve repair in the operating room.

Other Tests

• Electrocardiography
• • A sinus rhythm is commonly present. On occasion, atrial flutter or atrial fibrillation can develop in patients with DOMV and chronic left atrial dilatation.
  • Left atrial enlargement occurs in most patients with DOMV and is diagnosed on the basis of wide bifid P waves (P mitrale pattern) in the limb leads and/or an increased P terminal force in lead V1.
  • Tall, peaked P waves in the inferior leads indicate right atrial hypertrophy because of pulmonary hypertension and tricuspid incompetence.
  • The mean frontal-plane QRS axis may be normal or shifted to the right.
  • RV hypertrophy is common in stenotic DOMV, whereas mitral incompetence is associated with LV hypertrophy.
  • Associated congenital heart defects can modify electrocardiographic findings.

Procedures

• Cardiac catheterization
• • Cardiac catheterization is often necessary to quantify the hemodynamic effects of abnormal mitral-valve function.
  • DOMV with obstruction is characterized by elevation of left atrial pressure. Pressure tracings obtained from the pulmonary-artery wedge position reflect left atrial pressure and avoid the need to enter the left atrium by puncturing the interatrial septum. Simultaneous pressure recording shows that pulmonary-artery wedge pressure is substantially higher than LV pressure throughout diastole.
  • In patients with mitral regurgitation, the left atrial and pulmonary-artery wedge pressures are elevated, but no gradient can be demonstrated across the mitral valve at end of diastole; LV diastolic pressure is often increased.
  • Cardiac output and vascular resistance may be measured. Associated shunts and obstructive lesions, if any, may also be identified and measured during cardiac catheterization.
• Cardiac angiography
• • With the availability of high-quality echocardiography, cardiac angiography has a limited role in the assessment of DOMV. Echocardiography is superior to angiography in defining anatomic and functional abnormalities of the valve.
  • LV angiography may be performed to confirm the severity of mitral regurgitation and to assess LV function.
  • Cardiac angiography is also important to assess other associated defects.

TREATMENT

Section 6 of 11  

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Medical Care

General principles of management

Patients with DOMV can be evaluated as outpatients. Admit patients to the hospital for cardiac catheterization, for the treatment of severe heart failure or pulmonary edema, and for surgery.

Management depends on the type and severity of mitral-valve dysfunction. Isolated DOMV causing neither obstruction nor regurgitation needs no active intervention. This principle is important because the hemodynamics of a DOMV at rest and during exercise do not differ from those of a normal mitral valve with a similar valvular area. However, long-term follow-up and care are necessary to detect the onset of hemodynamic problems or complications.

All patients with clinically significant mitral stenosis or regurgitation require medical therapy and possibly transcatheter balloon or surgical treatment.

Roles of medical therapy

• To initially stabilize the patient and to relieve pulmonary edema
• To control congestive heart failure (CHF) while detailed assessment and surgical repair is awaited
• To serve as an adjunct to surgical repair in the postoperative period
• To treat small infants, in whom medical therapy may be the only option (Control of CHF may defer surgery until the child grows to an appropriate age and size.)

Adjunctive therapy

• Administer potassium supplements to all patients receiving frusemide or thiazide diuretics.
• Restrict physical activity of symptomatic patients.
• Treat patients with severe pulmonary venous congestion in a sitting or propped-up position.
• Administer parenteral morphine in patients with pulmonary edema to help relieve anxiety and reduce pulmonary congestion.
• Administer oxygen by a nasal catheter or mask to improve oxygenation in patients with acute pulmonary edema.
• Vigorously treat concurrent infections or other aggravating factors.
• Correcting anemia, if present, is important. Increasing the oxygen-carrying capacity by a packed-cell transfusion in patients with severe symptoms or heart failure may provide considerable relief.
• All patients with DOMV need antibiotic prophylaxis against infective endocarditis when they undergo any dental or surgical procedure.

Surgical Care

• The aim of surgical treatment of DOMV is to correct abnormal mitral-valve function and to repair all associated defects. A functionally normal DOMV may be left intact even when the accompanying lesions are repaired. In fact, surgical creation of a DOMV has been described as an effective additional procedure for repair of atypical cases of atrioventricular septal defect.
• • Perform surgical repair in all symptomatic patients with regurgitant DOMV and in patients with stenotic DOMV if balloon dilation is not feasible or fails to relieve obstruction. Emergency surgical intervention is occasionally needed to manage severe mitral regurgitation resulting from balloon valvuloplasty.
  • Perform early operation for DOMV in the presence of intractable heart failure or pulmonary edema, pulmonary arterial hypertension, worsening LV function, or a progressive increase in heart size due to ventricular dilatation.
  • Adjust the type of operation depending on the anatomic abnormality in the mitral-valve apparatus. Make every effort to define the anatomy in detail before undertaking surgery. In many patients, the valve may be amenable to repair and reconstruction procedures, including resection of tissue, repair of defects and clefts, shortening of chordae, placement of artificial chordae, and annuloplasty. Preserve the delicate relationships among the various components of the valve and the subvalvar apparatus. However, if the valvular mechanism is markedly abnormal, replacement of the mitral valve is necessary.
• Selected cases of DOMV with mitral stenosis are amenable to percutaneous transcatheter balloon dilatation.
• • This option offers a nonsurgical method of relieving obstruction.
  • Successful dilatation has been reported in patients with the bridge type of DOMV, especially if the bridge is incomplete.
  • Stepwise dilatations by using an Inoue balloon are applied to the posteromedial orifice of the DOMV.
  • The results are best if the DOMV is an isolated defect and if no major deformity of the subvalvular tensor apparatus is present.
  • Surgical standby is recommended to manage incomplete relief of obstruction or clinically significant disruption of the valve apparatus that causes mitral regurgitation.

Consultations

• Cardiologist
• Cardiothoracic surgeon

Diet

• Asymptomatic patients with DOMV require no special diet.
• Counsel patients with heart failure to avoid excess intake of salt or to reduce their salt intake. Prescribe salt restriction cautiously in infants.
• Restrict fluid intake to approximately 60-80 mL/kg/d in infants with CHF.

Activity

Patients with pulmonary venous congestion or CHF should avoid strenuous exertion.

MEDICATION

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Medical therapy for DOMV consists of the use of drugs to control of CHF. The 3 groups of drugs used are diuretics (to promote excretion of excess water), positive inotropic drugs (to improve myocardial contraction), and vasodilators (to reduce arterial resistance).

Antibiotics for endocarditis prophylaxis are administered to patients before procedures that may cause bacteremia are performed. For more information, see Antibiotic Prophylactic Regimens for Endocarditis.

Drug Category: Diuretics

These drugs promote the excretion of water and electrolytes by the kidneys and are used to treat heart failure or hepatic, renal, or pulmonary disease when sodium and water retention results in edema or ascites. They are useful to remove excess water that accumulates in patients with CHF. They also relieve symptoms associated with pulmonary congestion and reduce peripheral edema.

Drug Name	Chlorothiazide (Diuril)
Description	Increases water excretion by inhibiting reabsorption of sodium chloride in distal renal tubule. Less potent than furosemide, thiazide diuretics useful in maintenance therapy of CHF; in severe heart failure or refractory edema, act synergistically with furosemide to promote diuresis.
Adult Dose	125-500 mg/d PO qd or divided bid
Pediatric Dose	20 mg/kg/d PO divided bid
Contraindications	Documented hypersensitivity; anuria or renal decompensation
Interactions	May decrease effects of anticoagulants, antigout agents, and sulfonylureas; may increase toxicity of allopurinol, anesthetics, antineoplastics, calcium salts, loop diuretics, lithium, diazoxide, digitalis, amphotericin B, and nondepolarizing muscle relaxants
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Periodically monitor serum electrolyte levels; observe for hypokalemia, hyponatremia, hypochloremic alkalosis, hyperuricemia, hypomagnesemia, hypercalcemia, hyperglycemia, rise in serum low-density lipoprotein cholesterol (LDL-C) and triglyceride levels, and pancreatitis

Drug Name	Hydrochlorothiazide (Esidrix, HydroDIURIL)
Description	Increases water excretion by inhibiting reabsorption of sodium chloride in distal renal tubule. Less potent than furosemide. Useful in maintenance therapy of CHF; in severe heart failure or refractory edema, act synergistically with furosemide to promote diuresis.
Adult Dose	12.5-50 mg/d PO divided bid; not to exceed 200 mg/kg/d
Pediatric Dose	2 mg/kg/d PO divided bid
Contraindications	Documented hypersensitivity; anuria or renal decompensation
Interactions	May decrease effects of anticoagulants, antigout agents, and sulfonylureas; may increase toxicity of allopurinol, anesthetics, antineoplastics, calcium salts, loop diuretics, lithium, diazoxide, digitalis, amphotericin B, and nondepolarizing muscle relaxants
Pregnancy	B - Usually safe but benefits must outweigh the risks.
Precautions	Periodically monitor serum electrolyte levels; observe for hypokalemia, hyponatremia, hypochloremic alkalosis, hyperuricemia, hypomagnesemia, hypercalcemia, hyperglycemia, rise in serum LDL-C and triglyceride levels, and pancreatitis

Drug Name	Spironolactone (Aldactone)
Description	Counteracts secondary hyperaldosteronism in cardiac failure. Inhibits sodium absorption in collecting duct. Potassium-sparing diuretic effect. Used alone, produces relatively mild diuresis. Can be used with furosemide for synergistic action in severe CHF.
Adult Dose	25-100 mg/d PO divided bid
Pediatric Dose	2-4 mg/kg/d PO divided bid
Contraindications	Documented hypersensitivity; anuria, renal failure, or hyperkalemia
Interactions	May decrease effect of anticoagulants; potassium and potassium-sparing diuretics may increase toxicity
Pregnancy	D - Unsafe in pregnancy
Precautions	Renal and hepatic impairment; monitor serum potassium level; special care in combination with captopril; observe for hyperkalemia, metabolic acidosis, rash, and gynecomastia

Drug Category: Inotropic agents

Positive inotropic agents increase the force of myocardial contraction and are used to treat acute and chronic CHF. Some may also provide vasodilatation, improve myocardial relaxation, or increase or decrease the heart rate (positive or negative chronotropic agents, respectively). These additional properties influence the choice of drug for specific circumstances.

Drug Category: Vasodilators

Drugs that produce vasodilation include angiotensin-converting enzyme (ACE) inhibitors, nitrates, and direct vasodilators (eg, hydralazine). Of these drug classes, ACE inhibitors are frequently used because their adverse effects are the most tolerated. They reduce afterload on the LV by decreasing systemic arterial resistance. ACE inhibitors are particularly helpful in patients with mitral regurgitation, in whom decreased afterload reduces the severity of regurgitation and improves ventricular function. The DOC is captopril. Newer drugs from this category, such as enalapril and lisinopril, are also used for the treatment of CHF, but experience with their use in children is limited.

Drug Name	Enalapril (Vasotec)
Description	Competitive ACE inhibitor. Reduces angiotensin II level, potent vasoconstrictor. Lowers vascular resistance and aldosterone secretion.
Adult Dose	5-30 mg/d PO divided bid
Pediatric Dose	0.2–0.5 mg/kg/d PO divided bid
Contraindications	Documented hypersensitivity; renal impairment
Interactions	NSAIDs may reduce hypotensive effects; ACE inhibitors may increase digoxin, lithium, and allopurinol levels; rifampin decreases levels; probenecid may increase levels; concurrent diuretics may enhance hypotensive effects of ACE inhibitors
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Pregnancy category D in second or third trimester; caution in renal impairment, valvular stenosis, or severe CHF; start at low doses and gradually increase according to blood pressure, patient tolerance, and clinical effects; observe for hypotension, rash, cough, dizziness, fatigue, hyperkalemia, hypoglycemia, uremia, proteinuria, and neutropenia

Drug Name	Lisinopril (Zestril, Prinivil)
Description	Prevents conversion of angiotensin I to angiotensin II, potent vasoconstrictor. Lowers vascular resistance and aldosterone secretion.
Adult Dose	10-30 mg/d PO divided bid
Pediatric Dose	Not established, limited data suggests: 0.2–1 mg/kg/d PO divided bid
Contraindications	Documented hypersensitivity; renal impairment
Interactions	NSAIDs may reduce hypotensive effects; ACE inhibitors may increase digoxin, lithium, and allopurinol levels; rifampin decreases levels; probenecid may increase levels; concurrent diuretics may enhance hypotensive effects of ACE inhibitors
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Pregnancy category D in second or third trimester; caution in renal impairment, valvular stenosis, or severe CHF; start at low doses and gradually increase according to blood pressure, patient tolerance, and clinical effects; observe for hypotension, rash, cough, dizziness, fatigue, hyperkalemia, hypoglycemia, uremia, proteinuria, and neutropenia

FOLLOW-UP

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Further Inpatient Care

• Hemodynamic study by cardiac catheterization and angiography
• Surgical treatment

Further Outpatient Care

• Monitor medication for compliance, dose requirements, and adverse effects.
• Periodically check for electrolyte disturbances.
• Provide follow-up care for prompt detection and treatment of intercurrent infections, arrhythmia, and other complications.

In/Out Patient Meds

• Medications for DOMV include diuretics, digoxin, potassium chloride, and vasodilators.
• Antibiotics are indicated for intercurrent bacterial infections.
• Counsel patient concerning antibiotic prophylaxis against infective endocarditis during dental and surgical procedures.

Transfer

• Transfer to a tertiary cardiac facility may be required for further diagnostic evaluation and surgical treatment.

Deterrence/Prevention

• Symptomatic patients with DOMV should avoid sports and other strenuous activity that could aggravate their pulmonary congestion and CHF.

Complications

• Possible complications are mitral regurgitation, mitral stenosis, pulmonary edema, recurrent respiratory infection, atrial fibrillation and other atrial arrhythmias, infective endocarditis, left atrial thrombus, and pulmonary arterial hypertension.

Prognosis

• Isolated DOMV is a rare but correctable defect, if the abnormality is recognized and appropriately treated.
• When DOMV is associated with other congenital heart defects the long-term outcome depends on the major cardiac abnormality.

Patient Education

• Impose activity restrictions on symptomatic patients.
• Advise the patient concerning the need for periodic medical review.
• Inform the patient regarding prophylaxis for infective endocarditis and prompt attention to all infections.
• Counsel the patient about the need for regular anticoagulant therapy with warfarin (Coumadin) in those who undergo valve replacement.

MISCELLANEOUS

Section 9 of 11  

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• Medication
• Follow-up
• Miscellaneous
• Multimedia
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Medical/Legal Pitfalls

• Failure to identify DOMV as the cause of mitral valve obstruction or regurgitation
• Failure to identify DOMV as an associated defect in patients with atrioventricular-canal defect and other CHDs.
• Failure to recognize associated abnormalities of the mitral valve apparatus in patients with DOMV

Special Concerns

• DOMV may not be detected easily in children with CHD; therefore, awareness of the problem and careful echocardiographic screening are important in all children with mitral valve disease.

MULTIMEDIA

Section 10 of 11  

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• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  Two-dimensional echocardiogram, parasternal short-axis view, shows 2 mitral-valve orifices.
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Media type:  Photo

Media file 2:  Two-dimensional echocardiogram of the mitral valve, apical view with color flow mapping, shows diastolic flow through 2 separate orifices.
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Media type:  Photo

REFERENCES

Section 11 of 11

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• Agricola E, Maisano F, Oppizzi M, et al. Mitral valve reserve in double-orifice technique: an exercise echocardiographic study. J Heart Valve Dis. Sep 2002;11(5):637-43. [Medline].
• Amorgianos D, Kranidis A, Koulouris S, Triantafyllou K, Manolis AS. Well-functioning double-orifice mitral valve in a young woman with Marfan-like habitus and atrial tachycardia. Cardiology. 2005;104(4):169-70. [Medline].
• Bano-Rodrigo A, Van Praagh S, Trowitzsch E, et al. Double-orifice mitral valve: a study of 27 postmortem cases with developmental, diagnostic and surgical considerations. Am J Cardiol. Jan 1 1988;61(1):152-60. [Medline].
• Baylen BG, Waldhausen JA. Heart disease in infants, children and adolescents. In: Adams FH, Emmanouilides GC, Riemenschneider TA, eds. Diseases of the Mitral Valve. 4^th ed. Baltimore, Md: Lippincott, Williams and Wilkins; 1989:650.
• Bedi HS, Bazaz S, Kler TS, et al. Double orifice mitral valve with gross regurgitation in both orifices. Indian Heart J. Mar-Apr 1998;50(2):213-4. [Medline].
• Das BB, Pauliks LB, Knudson OA, et al. Double-orifice mitral valve with intact atrioventricular septum: an echocardiographic study with anatomic and functional considerations. J Am Soc Echocardiogr. Mar 2005;18(3):231-6. [Medline].
• De Castro S, Caselli S, Papetti F, et al. Feasibility and clinical impact of live three-dimensional echocardiography in the management of congenital heart disease. Echocardiography. Aug 2006;23(7):553-61. [Medline].
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Article Last Updated: Jul 17, 2007