eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology
Atrial Septal Defect, Sinus Venosus: Treatment & Medication
Updated: Jun 12, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- Medical care of sinus venosus atrial septal defect (ASD) is primarily supportive and is not required for asymptomatic patients.
- Patients presenting in heart failure should be stabilized in anticipation of elective repair.
Surgical Care
Surgical correction is the mainstay of therapy.
- Repair of the sinus venosus atrial septal defect is more complex than repair of the average secundum atrial septal defect. A patch (synthetic material or pericardium) is used to redirect blood flow from the right superior pulmonary vein into the left atrium. This effectively closes the interatrial communication while also correcting the anomalous pulmonary venous drainage. Sometimes, to avoid creating superior vena cava (SVC) obstruction, a patch is placed on the anterior surface of the SVC. Care is taken to avoid injuring the nearby sinus node. Ligation of the azygous vein may also be required to eliminate its drainage into the left atrium and to prevent the resulting residual right-to-left shunt.
- When the location of the anomalous venous drainage is in the high SVC and is far from the atrial-caval junction, a different surgical approach can be used to decrease the probability of caval stenosis or pulmonary vein stenosis.
- As described by Warden et al, the repair consists of division of the SVC just above the take off of the anomalous pulmonary vein.4
- The distal caval end is oversewn or patched to assure no pulmonary vein compromise.
- Next, the well-mobilized cava is anastomosed to the right atrial appendage after amputation of the most distal end.
- The atrial septal defect is then closed by sewing a patch to cover the atrial septal defect and divided SVC orifice, thereby baffling the anomalous vein to the left atrium.
- This method is very effective in patients with more complicated pulmonary venous anomalies.
- Although a relatively recent advance in the treatment of high anomalous pulmonary venous drainage, this operation has become the procedure of choice for more difficult cases.
- All reported series have demonstrated excellent results with little or no pulmonary venous or SVC stenosis.5
- In addition, concern for injury to the conduction system or sinus node have not been observed to date.6
- Asymptomatic children generally undergo repair when aged 3-5 years.
- Sinus venosus defects do not close spontaneously.
- Adults with left-to-right shunts greater than 1.5-2:1 benefit from surgical closure.
- Patients with significant pulmonary hypertension and elevated pulmonary vascular resistance unresponsive to pulmonary vasodilator therapy (eg, oxygen, nitric oxide, calcium channel blockers,) may not be good candidates for surgical repair. Such patients may develop acute right ventricular failure if their heart no longer has the ability to shunt right to left at the atrial communication in response to increases in pulmonary vascular resistance.
- Repair is performed most often through a standard median sternotomy. More cosmetic incisions may also be used, such as partial sternotomies, small right anterior thoracotomies, and inframammary incisions. All approaches still require the use of cardiopulmonary bypass for closure of the atrial septal defect.
- Although transcatheter occlusion devices are currently used for closing secundum atrial septal defects, such devices are not indicated (at present) for the closure of sinus venosus atrial septal defects because of the position of the defect and because of the lack of surrounding tissue adequate to seat such an occlusion device. In addition, such a device may obstruct SVC flow and does not achieve redirection of the anomalous right pulmonary venous flow to the left atrium.
Consultations
- Pediatric cardiologist
- Pediatric cardiac surgeon
Diet
- No dietary restrictions
Activity
- Physical activity should not be limited in patients who undergo early and complete correction.
Medication
Medical management is ineffective in the treatment of sinus venosus defects. The rare patient who presents in congestive heart failure can be stabilized medically with diuretics and inotropic support.
Inotropic agents
These agents provide myocardial support in patients with dysfunction secondary to pulmonary overcirculation from left-to-right shunting. Positive inotropic agents increase the force of contraction of the myocardium and are used to treat acute and chronic congestive heart failure (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.
Digoxin (Lanoxin)
Exerts its inotropic effects by increasing amount of intracellular calcium available during excitation-contraction coupling. One of numerous inotropic agents that can be used in infants with congenital cardiac defects. Generally used for long-term administration and is rarely drug of choice for acute management of heart failure in ICU setting.
Adult
Total digitalizing dose (TDD):
0.75-1.5 mg PO divided tid; 0.5-1 mg IV/IM divided tid
Divide TDD as follows: 50% initially; 25% 6-12 h later; 25% and the final 6-12 h later (one half, one quarter, one quarter)
Maintenance dose:
0.125-0.5 mg/d PO; 0.1-0.4 mg/d IV/IM
Pediatric
TDD:
Preterm neonate: 20-30 mcg/kg/d PO; 15-25 mcg/kg/d IV/IM
Term neonate: 25-35 mcg/kg/d PO; 20-30 mcg/kg IV/IM
1 month to 2 years: 35-60 mcg/kg/d PO; 30-50 mcg/kg/d IV/IM
2-5 years: 30-40 mcg/kg/d PO; 25-35 mcg/kg/d IV/IM
5-10 years: 20-35 mcg/kg/d PO; 15-30 mcg/kg/d IV/IM
>10 years: 10-15 mcg/kg/d PO; 8-12 mcg/kg/d IV/IM
Divide TDD as follows: 50% initially, 25% 6-12 h later; and the final 25% 6-12 h later (one half, one quarter, one quarter)
Maintenance dose:
Preterm neonate: 5-7.5 mcg/kg/d PO divided bid; 4-6 mcg/kg/d IV/IM divided bid
Term neonate: 6-10 mcg/kg/d PO divided bid; 5-8 mcg/kg/d IV/IM divided bid
1 month to 2 years: 10-15 mcg/kg/d PO divided bid; 7.5-12 mcg/kg/d IV/IM divided bid
2-5 years: 7.5-10 mcg/kg/d PO divided bid; 6-9 mcg/kg/d IV/IM divided bid
5-10 years: 5-10 mcg/kg/d PO divided bid; 4-8 mcg/kg/d IV/IM divided bid
>10 years: 2.5-5 mcg/kg/d PO; 2-3 mcg/kg/d IV/IM qd
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 (including carmustine, bleomycin, methotrexate, cytarabine, doxorubicin, cyclophosphamide, vincristine, procarbazine), aluminum or magnesium antacids, rifampin, sucralfate, sulfasalazine, barbiturates, kaolin/pectin, and aminosalicylic acid
Documented hypersensitivity; digitalis-induced toxicity, AV block, idiopathic subaortic stenosis, constrictive pericarditis
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 diagnosed with incomplete AV block may progress to complete block when treated with digoxin; exercise caution in hypothyroidism, hypoxia, and acute myocarditis
Dopamine (Intropin)
Adrenergic agonists often are used for inotropic support in critical care setting for their rapid onset of action and rapid time to peak effect, which make them easier to titrate to effect
Adult
1-20 mcg/kg/min continuous IV infusion; not to exceed 50 mcg/kg/min
Pediatric
Neonates: 1-20 mcg/kg/min continuous IV infusion
Infants and children: Administer as in adults
Phenytoin, alpha-adrenergic and beta-adrenergic blockers, general anesthesia, and MAOIs increase and prolong effects of dopamine
Documented hypersensitivity; pheochromocytoma or ventricular fibrillation
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
Treat hypovolemia before infusion; administration through a central vein is recommended; do not use umbilical artery for infusion; if dosages >20 mcg/kg/min are required, consider a different agent (eg, epinephrine, dobutamine)
Loop diuretics
These agents are used for management of right heart failure and pulmonary edema. They promote excretion of water and electrolytes by the kidneys.
Furosemide (Lasix)
Highly effective first-line diuretic in newborns and infants. A sulfonamide derivative, it exerts its effects on the loop of Henle and distal renal tubule, inhibiting reabsorption of sodium and chloride.
Adult
10-200 mg PO/IV initially; titrate dose to effect; not to exceed 600 mg/d
Continuous IV infusions may be more successful; not to exceed 0.4 mg/kg/h
Pediatric
1-2 mg/kg/dose PO/IV bid/qid; titrate dose to effect; not to exceed 6 mg/kg/dose bid/qid
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 with this medication
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
Monitor serum potassium levels closely; may produce intravascular dehydration, severe hypokalemia, and significant hypochloremic metabolic acidosis; inform patients of potential for photosensitivity; may produce hyperuricemia; may produce deafness caused by ototoxicity; most popular strengths of digoxin and furosemide are white tablets of approximately equal size and may be confused by patients; administer oral dose with food or milk to decrease stomach upset
More on Atrial Septal Defect, Sinus Venosus |
| Overview: Atrial Septal Defect, Sinus Venosus |
| Differential Diagnoses & Workup: Atrial Septal Defect, Sinus Venosus |
 Treatment & Medication: Atrial Septal Defect, Sinus Venosus |
| Follow-up: Atrial Septal Defect, Sinus Venosus |
| Multimedia: Atrial Septal Defect, Sinus Venosus |
| References |
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References
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Murphy JG, Gersh BJ, McGoon MD, et al. Long-term outcome after surgical repair of isolated atrial septal defect. Follow-up at 27 to 32 years. N Engl J Med. Dec 13 1990;323(24):1645-50. [Medline].
Radzik D, Davignon A, van Doesburg N, et al. Predictive factors for spontaneous closure of atrial septal defects diagnosed in the first 3 months of life. J Am Coll Cardiol. Sep 1993;22(3):851-3. [Medline].
Sachweh JS, Daebritz SH, Hermanns B, et al. Hypertensive pulmonary vascular disease in adults with secundum or sinus venosus atrial septal defect. Ann Thorac Surg. Jan 2006;81(1):207-13. [Medline].
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Further Reading
Keywords
sinus venosus, atrial septal defect, ASD, superior vena cava type subcaval ASD, SVASD, atrial septum, congenital heart defect, congenital cardiac anomaly, congestive heart failure, murmur, treatment, diagnosis, heart problems, heart disease, heart anomaly
