eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology
Ventricular Septal Defect, Perimembranous: Treatment & Medication
Updated: Nov 25, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
- Small perimembranous ventricular septal defects (VSDs) have a spontaneous closure rate as high as 50% within the first 2 years of life and often do not require medical or surgical management.
- Larger defects may not close but often become smaller with time. Medical therapy may be required with large membranous VSDs due to excessive left-to-right shunting and congestive heart failure (CHF). Therapy is directed at alleviating the symptoms of pulmonary overcirculation. Therapy typically includes increased calorie feedings, diuretics, and, sometimes, an ACE inhibitor.
- Diuretic therapy with furosemide is used to lessen volume overload. Significant potassium wasting may warrant the addition of spironolactone or potassium supplementation.
- The use of afterload reduction to improve systemic-pulmonary flow ratios may be beneficial in selected cases. ACE inhibitors also inhibit the tissue-based renin-angiotensin system, preventing deleterious remodeling. Be aware that ACE inhibitors have a potassium-sparing effect. When these are used, spironolactone or supplemental potassium should be avoided or judiciously used.
Surgical Care
- Failure of medical management to alleviate symptoms in the first 6 months of life requires intervention.
- Growth failure despite optimal medical therapy and maximized caloric intake is the most important evidence of failure of medical therapy.
- Elevated pulmonary arteriolar resistance more than 12 Wood units, which does not decrease with oxygen or selective pulmonary vasodilator therapy, may be regarded as inoperable.
- Very large left-to-right shunts are usually electively repaired within the first year of life.
- Intervention is either by surgery or cardiac catheterization.
- Surgery
- Surgical repair is the most common intervention currently performed.
- Surgical repair of an isolated large VSD involves closure of the defect with a Gore-Tex patch.
- Surgical intervention in younger infants, especially those younger than 1 month, is associated with an increased risk of mortality (historically as high as 10%, although currently much lower).
- Surgical mortality is now very low (approximately 1%) in patients older than 6 months with isolated perimembranous VSDs.
- New surgical approaches using smaller incisions have proven effective in VSD closure.
- Surgery is indicated in patients with progressive aortic insufficiency or greater than trivial insufficiency at the time of initial presentation.
- Cardiac catheterization or hybrid procedures
- Devices are now available for closure of perimembranous VSDs.4,5 .
- VSD closure devices typically have 2 asymmetrical opposing discs (one for the right ventricular side and one for the left ventricular side), which are released during catheterization under fluoroscopic and transesophageal echocardiographic guidance to occlude the defect. These devices can be placed percutaneously in the cardiac catheterization laboratory or in the operating room during a "hybrid procedure." These procedures are slightly more complicated than closure of muscular VSDs because of the asymmetry of the device, the proximity to the aortic valve, and the presence of conduction tissue very near the defect.
- Hybrid procedures may involve inserting the device through a very small incision in the free wall of the right ventricle.
- Ongoing investigational trials are currently being performed to assess indications and outcomes in VSD closure with these devices.
- One report noted effective closure in children using the Amplatzer asymmetric perimembranous occluder in 35 patients with a median age 4.5 years.6 The defects were 3-8 mm in size, and the size of the occluder varied from 4-12 mm. After 2.5 years, the rate of complete closure was 91%. Complications included residual shunting that required surgical closure of the defect subsequent to the insertion of the device and persistent regurgitation across the tricuspid or aortic valve related to the occluder. Conduction abnormalities related to the procedure occurred in 20% of the patients. The abnormalities were permanent in all but one of these patients.
- Surgery
Consultations
- Pediatric cardiologist
- Pediatric cardiothoracic surgeon if surgery is needed
Diet
- Patients with significant CHF may require caloric supplementation with fortified formula or breast milk.
Activity
- Patients with small perimembranous VSDs have no activity restrictions.
- Patients with moderate-to-large perimembranous defects and significant symptomatology limit their own exercise activity levels until the defect is repaired.
- Patients with repaired VSDs and no residual cardiac sequelae have no activity restrictions.
Medication
Diuretics are now the mainstay of medical therapy for infants and children with large ventricular septal defects (VSDs), large left-to-right shunts, and evidence of congestive heart failure (CHF). Current debate is ongoing concerning the use of digoxin. In certain situations, the addition of afterload reduction may also be beneficial. Hemoglobin levels should be normal.
Diuretics
These agents relieve ventricular volume load and peripheral and pulmonary congestion.
Furosemide (Lasix)
Increases excretion of water by interfering with chloride-binding cotransport system, which, in turn, inhibits sodium and chloride reabsorption in ascending loop of Henle and distal renal tubule.
Adult
20-250 mg/d PO/IV/IM qd or divided bid/tid
Pediatric
0.5-2 mg/kg PO qd or divided bid/tid; alternatively, 0.5-1 mg/kg IV qd or divided bid/tid
Metformin decreases furosemide concentrations; furosemide interferes with hypoglycemic effect of antidiabetic agents and antagonizes muscle relaxing effect of tubocurarine; auditory toxicity appears to be increased with coadministration of aminoglycosides and furosemide; hearing loss of varying degrees may occur; anticoagulant activity of warfarin may be enhanced when taken concurrently with this medication; increased plasma lithium levels and toxicity are possible when taken concurrently
Documented hypersensitivity, hepatic coma, anuria, and state of severe electrolyte depletion
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Perform frequent serum electrolyte (eg, potassium), carbon dioxide, glucose, creatinine, uric acid, calcium, and BUN determinations during first few mo of therapy and periodically thereafter
Spironolactone (Aldactone)
For management of edema resulting from excessive aldosterone excretion. Competes with aldosterone for receptor sites in distal renal tubules, increasing water excretion while retaining potassium and hydrogen ions.
Adult
25-200 mg/d PO in 1-2 divided doses
Pediatric
Maintenance: 1 mg/kg/dose PO up to qid
May decrease effect of anticoagulants; potassium and potassium sparing diuretics may increase toxicity of spironolactone
Documented hypersensitivity; anuria, renal failure or hyperkalemia
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Caution in renal and hepatic impairment
Afterload reducers
These drugs decrease systemic afterload and may decrease left-to-right shunting through large VSD. They are used to improve preoperative or postoperative cardiac output. They reduce systemic vascular resistance and increase systemic blood flow resulting from myocardial dysfunction.
Enalapril (Vasotec)
Competitive inhibitor of angiotensin converting enzyme. Reduces angiotensin II levels, decreasing aldosterone secretion.
Adult
5 mg PO qd initial; not to exceed 40 mg/d
Pediatric
Neonates: 0.1 mg/kg/d PO
Infants and children: 0.1 mg/kg/d PO divided bid; may gradually increase, not to exceed 0.5 mg/kg/d
Adolescents: 2.5 mg PO qd initial; not to exceed 5-10 mg/d
NSAIDs may reduce hypotensive effects of enalapril; ACE inhibitors may increase digoxin, lithium, and allopurinol levels; rifampin decreases enalapril levels; probenecid may increase enalapril levels; the hypotensive effects of ACE inhibitors may be enhanced when given concurrently with diuretics
Documented hypersensitivity; children <16 y with severe renal impairment (ie, GFR <30 mL/min)
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Category D in second and third trimesters of pregnancy; caution in renal impairment, use in children with severe renal impairment is limited; caution with valvular stenosis or severe congestive heart failure
Captopril (Capoten)
Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion.
Adult
6.25-12.5 mg PO tid; not to exceed 150 mg tid
Pediatric
Newborns and premature infants: 0.01 mg/kg/dose PO q8-12h; titrate gradually
Neonates: 0.05-0.1 mg/kg/dose PO initially; may gradually titrate to daily dose of 2.5-6 mg/kg/d
Children: 0.3-0.5 mg/kg/dose PO; may gradually increase, not to exceed 6 mg/kg/d divided in 2-4 doses
Older children: 6.25-12.5 mg PO q12-24h; may gradually increase, not to exceed 6 mg/kg/d divided in 2-4 doses
Adolescents: Administer as in adults
NSAIDs may reduce hypotensive effects of captopril; ACE inhibitors may increase digoxin, lithium, and allopurinol levels; rifampin decreases captopril levels; probenecid may increase captopril levels; the hypotensive effects of ACE inhibitors may be enhanced when given concurrently with diuretics
Documented hypersensitivity; renal impairment
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Category D in second and third trimesters of pregnancy; caution in renal impairment, valvular stenosis, or severe congestive heart failure
Inotropic agents
These agents augment ventricular contractility. Positive inotropic agents increase the force of contraction of the myocardium and are used to treat acute and chronic CHF. Some may also increase or decrease the heart rate (ie, positive or negative chronotropic agents), provide vasodilatation, or improve myocardial relaxation. These additional properties influence the choice of drug for specific circumstances. Cardiac glycosides are used predominantly for their inotropic effects.
Digoxin (Lanoxin)
Cardiac glycoside with direct inotropic effects and indirect effects on the cardiovascular system. Inhibits NaK-ATPase, which causes intracellular calcium in the sarcoplasmic reticulum of cardiac cells to increase.
Adult
0.125-0.375 mg PO qd
Pediatric
Digitalization: 25-40 mcg/kg IV; 50% of dose initially, then 25% q8h for remaining 2 doses
Maintenance: 8-10 mcg/kg/d PO divided bid; or 6-9 mcg/kg/d IV divided bid
Medications that may increase digoxin levels include alprazolam, benzodiazepines, bepridil, captopril, cyclosporine, propafenone, propantheline, quinidine, diltiazem, aminoglycosides, PO amiodarone, anticholinergics, diphenoxylate, erythromycin, felodipine, flecainide, hydroxychloroquine, itraconazole, nifedipine, omeprazole, quinine, ibuprofen, indomethacin, esmolol, tetracycline, tolbutamide, and verapamil
Medications that may decrease serum digoxin levels include aminoglutethimide, antihistamines, cholestyramine, neomycin, penicillamine, aminoglycosides, PO colestipol, hydantoins, hypoglycemic agents, antineoplastic treatment combinations (eg, carmustine, bleomycin, methotrexate, cytarabine, doxorubicin, cyclophosphamide, vincristine, procarbazine), aluminum or magnesium antacids, rifampin, sucralfate, sulfasalazine, barbiturates, kaolin/pectin, and aminosalicylic acid
Documented hypersensitivity, beriberi heart disease, idiopathic hypertrophic subaortic stenosis, constrictive pericarditis, and carotid sinus syndrome
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Hypokalemia may reduce positive inotropic effect of digitalis; IV calcium may produce arrhythmias in digitalized patients; hypercalcemia predisposes patient to digitalis toxicity, and hypocalcemia can make digoxin ineffective until serum calcium levels are normal; magnesium replacement therapy must be instituted in patients with hypomagnesemia to prevent digitalis toxicity; patients with incomplete AV block may progress to complete block when treated with digoxin; exercise caution in hypothyroidism, hypoxia, and acute myocarditis
More on Ventricular Septal Defect, Perimembranous |
| Overview: Ventricular Septal Defect, Perimembranous |
| Differential Diagnoses & Workup: Ventricular Septal Defect, Perimembranous |
 Treatment & Medication: Ventricular Septal Defect, Perimembranous |
| Follow-up: Ventricular Septal Defect, Perimembranous |
| References |
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References
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Further Reading
Keywords
ventricular septal defect, VSD, perimembranous, membranous ventricular septal defect, ventricular septum, right ventricular outflow obstruction, congestive heart failure, CHF, cardiac lesion, atrial septal defect, ASD, patent ductus arteriosus, prematurity, pulmonary valve stenosis, pulmonary venous obstruction, persistent elevation of pulmonary vascular resistance, mitral stenosis, Eisenmenger syndrome, cardiomegaly
