Medical Care

Most patients with isolated coronary sinus atrial septal defects (ASDs) are asymptomatic in childhood, and no specific medical treatment is necessary. Admit patients with coronary sinus ASDs for the management of arrhythmias in selected cases and for surgical intervention. Transfer may be required for further diagnostic evaluation or surgical intervention.

In the rare pediatric patient with symptoms of heart failure, anticongestive therapy with diuretics and possibly digoxin may be beneficial. Because of the rarity of heart failure, such patients deserve thorough evaluation for complicating factors such as clinically significant anemia.

Bacterial endocarditis is rare in the absence of associated abnormalities. Antibiotic prophylaxis is not routinely recommended. Antiarrhythmic therapy may become necessary in selected cases but usually not until adulthood.

Transcatheter device occlusion has not been considered a feasible option for coronary sinus ASDs because of their proximity to the tricuspid valve and cardiac conduction system and because of a lack of adequate tissue rims for device seating.^[7]However, Klijima et al reported success with two patients who had a coronary sinus atrial septal defect without persistent left superior caval vein. The orifice of the coronary sinus was closed using the Amplatzer Septal Occluder without any complications.^[8] Another paper reported the successful closure of small coronary sinus ASD.^[9]

St. Jude Medical’s Amplatzer atrial septal occluder may cause tissue erosion and lead to problems, such as cardiac tamponade, that require immediate surgery, according to the US Food and Drug Administration (FDA). The agency estimated the rate of emergencies associated with the device to be 1-3 implanted patients per 1000.^{[10, 11, 12]} Although the FDA did not recommend that the device be removed from patients unless a physician, following patient evaluation, deems it necessary, the agency directed St. Jude Medical to perform a retrospective, case-controlled study to determine risk factors for Amplatzer-associated erosion and the effects of erosion on the atrial septal occluder’s performance. This discussion largely pertains to ostium secondum ASDs.

Consultations

Consultations with the following specialists are important in the management of patient's with coronary sinus ASDs:

Pediatric cardiologist
Pediatric cardiothoracic surgeon

Diet and activity

No special dietary restrictions are necessary.

Note the following activity considerations:

Patients with unrepaired defects without pulmonary hypertension can participate in all competitive sports.
Patients with clinically significant pulmonary hypertension can participate only in low-intensity sports, such as bowling or golf.
Competitive sports may need to be restricted in patients with associated significant atrial or ventricular arrhythmias.
Patients can participate in all sports 6 months after successful closure of an uncomplicated ASD.

Outpatient care

Discuss activity, dietary, and pregnancy restrictions (see the section on Prevention, below) when appropriate.

Monitor patients for the development of symptoms associated with left-to-right shunt or arrhythmia. Also monitor for the efficacy of medications and for any adverse effects of these agents.

Indications

Surgical closure in childhood is the recommended therapy for secundum ASDs with clinically significant left-to-right shunts associated with cardiomegaly, symptoms, or both. The same recommendations hold true for coronary sinus ASDs. The indications generally used for ostium secundum ASDs are right ventricular volume overloading on echocardiogram or a pulmonary to systemic flow ratio (Qp:Qs) > 1.5:1,^[13] irrespective of symptomatology. Similar indications were also used for coronary sinus ASDs.^[13]

Indications for surgery in patients with small ASDs are controversial. Because symptoms are minimal and because morbidity and mortality are nonexistent in childhood, the risk of cardiopulmonary bypass may not be justified. Despite the smallness of such defects, a risk of paradoxical embolism and cryptogenic stroke remains.

Severe pulmonary hypertension (pulmonary vascular resistance >15 Wood units) is associated with unacceptably high postoperative morbidity and mortality rates. Therefore, surgical ASD repair is not recommended in this setting.

Echocardiography is generally adequate for diagnosis and preoperative planning. Preoperative cardiac catheterization is necessary in selected complicated cases or patients with evidence of elevated pulmonary artery resistance.

Surgical technique

This section addresses the surgical approach to coronary sinus ASDs in the absence of complex associated cardiac lesions. In patients with associated complex congenital heart disease, the other lesions are usually most important surgically.

The surgical treatment of isolated coronary sinus ASD is complicated by its proximity to the atrioventricular node. To avoid atrioventricular block, sutures must be placed close to the superior rim of the defect; therefore, patch repair is recommended.

The presence of a persistent left superior vena cava (SVC) affects cannulation for cardiopulmonary bypass and must be addressed during repair. If an adequate bridging vein is present and the left SVC can be occluded without substantial elevation in jugular venous pressure, the left SVC can be ligated. If not, the atrial septum is partially excised and patched by using a pericardial baffle to redirect blood from the left SVC to the right atrium and by closing the interatrial communication.

Surgical results

In uncomplicated secundum ASDs, surgical results are excellent, with published mortality rates of less than 1% and near 0% in some centers. Residual septal defects are rare. Long-term results are excellent, with mortality rates similar to those of the general population if defects are closed in patients younger than 25 years with normal or near-normal pulmonary artery pressures. Although data are limited, the risk of death from repair of a coronary sinus ASD, either alone or in conjunction with a left SVC, also appears to be low.^{[14, 2]}

After surgery in childhood, atrial fibrillation and flutter remain a long-term risk. The prevalence of these arrhythmias may actually increase over time in adulthood despite of surgical repair. The prevalence of postoperative complete atrioventricular block and the need for a pacemaker, although not established for coronary sinus ASDs, is likely relatively low but higher than the rate for secundum defects (approximately 1-5%).

When coronary sinus defects are associated with other, complex cardiac anatomy, the severity of these other defects largely determines the patient's prognosis and long-term outcome.^[14]

Postoperative care and precautions

After a coronary sinus ASD is surgically closed, patients should receive routine postoperative care similar to that given to patients with secundum ASDs.

Early extubation is expected, usually on the day or evening of surgery.

Blood loss is usually minimal, and blood transfusions are rarely needed.

The need for postoperative inotropic support should also be minimal, with an occasional patient requiring a low-dose infusion of dopamine.

Postoperative complications are unusual, but atrial arrhythmias do occur, and patients should be monitored for signs of SVC syndrome if a left SVC was ligated. The hospital length of stay should be less than 3-4 days.

Precautions against endocarditis are recommended for 6 months after surgery if a synthetic patch was used.

Postoperative complications

Most cases are uncomplicated, although any complication associated with cardiopulmonary bypass is theoretically possible. Examples include stroke, other systemic embolus, organ-system failure, infection, bleeding or coagulopathy, or death.

Postoperative arrhythmias (generally atrial in origin) and SVC syndrome may occur.

For unclear reasons, pericardial effusion and postpericardiotomy syndrome seem to occur relatively frequently after ASD repair.

Prevention

The use of birth control pills is not recommended with unrepaired ASDs because of increased risk of thrombosis and the risk of paradoxical emboli.

The pregnancy -related risk for unrepaired coronary sinus defects is not known but should be similar to that observed with secundum ASDs, given the physiologic similarities. The mortality rate for secundum ASDs in pregnancy is reported to be less than 1%, and the liveborn rate approaches the normal rate.

Pregnancy may cause patients to become more symptomatic in terms of exercise intolerance and congestive heart failure than they were before but is generally well tolerated, uncomplicated, and requires no special management. An exception is the patient with pulmonary vascular disease, as this condition poses a high risk to the expectant mother and often results in miscarriage. Potential complications include secondary pulmonary hypertension (which occur in a subset of patients), paradoxical embolism in the presence of deep vein thrombosis (which common during pregnancy because of stasis), and amniotic fluid embolus.

Medication

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