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. Consult with a pediatric cardiologist and a pediatric cardiac surgeon.
Patients with a sinus venosus ASD should be transferred to a center experienced in the repair of such a defect in children or adults. Patients with sinus venosus ASD in heart failure may require short-term continued support until pulmonary edema resolves, myocardial function improves, and until pulmonary vascular resistance, if elevated, normalizes.
Patients with sinus venosus ASD require a brief postoperative admission to a pediatric cardiac intensive care unit. The patient who undergoes uncomplicated surgical repair is usually discharged home within several days.
There are no dietary restrictions, and physical activity should not be limited in patients who undergo early and complete correction.
No long-term medication is required after repair of an uncomplicated atrial septal defect. Some surgeons prescribe aspirin or other anticoagulation regimens for several weeks in patients in whom a prosthetic patch was used to close the defect. This allows for endothelial ingrowth over the thrombogenic surface of the patch. Long-term anticoagulation is not indicated.
Antibiotic prophylaxis is not required in patients 6 months following repair of atrial septal defects.
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.  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.  In addition, concern for injury to the conduction system or sinus node have not been observed to date. 
A very small right upper pulmonary vein that drains into the high SVC could be left intact and not incorporated into the surgical repair if it is deemed high risk for late pulmonary vein obstruction.
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 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.
Postoperative follow-up usually involves an office visit with the pediatric cardiologist (and possibly the cardiac surgeon) 1-3 weeks after hospital discharge.
Echocardiography is used to effectively evaluate the repair for evidence of residual shunting, superior vena cava (SVC) or pulmonary vein obstruction, pericardial effusion, and ventricular function.
The potential for late postoperative narrowing of the SVC and possible superior vena cava syndrome is observed after repair of sinus venosus atrial septal defects.
Sinus node dysfunction screening should be part of outpatient follow-up care, because sinus node dysfunction may become apparent years after repair of a sinus venosus atrial septal defect.
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