eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Atrial Septal Defect, Coronary Sinus: Treatment & Medication

Author: Louis I Bezold, MD, Associate Professor, Department of Pediatrics, University of Kentucky College of Medicine; Chief, Division of Pediatric Cardiology, Medical Director, Kentucky Children's Hospital
Contributor Information and Disclosures

Updated: Nov 14, 2008

Treatment

Medical Care

Most patients with isolated coronary sinus atrial septal defects (ASDs) are asymptomatic in childhood, and no specific medical treatment is necessary.

  • 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 be necessary in selected cases but usually not until adulthood.
  • Transcatheter device occlusion is not 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.

Surgical Care

  • 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.
    • 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 such that the left SVC can be occluded without substantial elevation in jugular venous pressure, the left SVC can be successfully 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.5,1
    • 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.5
  • 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 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.

Consultations

  • Pediatric cardiologist
  • Pediatric cardiothoracic surgeon

Diet

  • No special dietary restrictions are necessary.

Activity

  • 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.

Medication

Diuretics and digoxin may be used in the management of congestive heart failure associated with large left-to-right shunts in patients with atrial septal defects (ASDs).

Diuretics, loop

These agents are used to treat pulmonary overcirculation associated with left-to-right atrial level shunt. They promote excretion of water and electrolytes by the kidneys. They are used to treat heart failure or hepatic, renal, or pulmonary disease when sodium and water retention results in edema or ascites.


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. Dose uptitrated to effect; low dose usually adequate.

Adult

20-80 mg/d PO/IV/IM divided q6-12h

Pediatric

1 mg/kg/dose PO/IV/IM bid/tid

Auditory toxicity appears to increase with coadministration of aminoglycosides and furosemide; hearing loss of various degrees may occur; may enhance anticoagulant activity of warfarin when taken concurrently

Documented hypersensitivity; hepatic coma; anuria; 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 fluid and electrolyte status, particularly potassium

Cardiac glycosides, antiarrhythmic agents

These agents are used to treat congestive heart failure and slow the ventricular response in atrial fibrillation or flutter.


Digoxin (Lanoxin, Lanoxicaps)

Positive inotropic effect related to increased cellular influx of calcium ions by inhibiting sodium-potassium exchange. Decreases conduction through sinoatrial and atrioventricular nodes.

Adult

125-250 mcg PO qd

Pediatric

Maintenance dose:
Infants: 6-8 mcg/kg/d PO
2-5 years: 10-15 mcg/kg/d PO
5-10 years: 7-10 mcg/kg/d PO
>10 years: 3-5 mcg/kg/d PO
<10 years: Recommended to divide daily dose bid

Drugs that may increase 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; drugs that may decrease serum levels include aminoglutethimide, antihistamines, cholestyramine, neomycin, penicillamine, aminoglycosides, PO colestipol, hydantoins, hypoglycemic agents, antineoplastic treatment combinations (eg, carmustine, bleomycin, methotrexate, cytarabine, doxorubicin, cyclophosphamide, vincristine, and 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; 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 patients receiving digitalis; hypercalcemia predisposes patient to digitalis toxicity, and hypocalcemia can make digoxin ineffective until serum calcium levels are in reference range; magnesium replacement must be started in patients with hypomagnesemia to prevent digitalis toxicity; patients with incomplete atrioventricular block may progress to complete block with therapy; caution in hypothyroidism, hypoxia, and acute myocarditis

More on Atrial Septal Defect, Coronary Sinus

Overview: Atrial Septal Defect, Coronary Sinus
Differential Diagnoses & Workup: Atrial Septal Defect, Coronary Sinus
Treatment & Medication: Atrial Septal Defect, Coronary Sinus
Follow-up: Atrial Septal Defect, Coronary Sinus
References
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References

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Further Reading

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Keywords

coronary sinus atrial septal defect, coronary sinus ASD, unroofed coronary sinus, interatrial shunting, persistent left superior vena cava draining to the coronary sinus, LSVC, heterotaxy syndrome, abnormalities of atrial situs, anomalies of systemic venous return, venous emboli, paradoxical emboli, communication between the right and left atria, congenital heart disease, CHD, cardiovascular disease, heart disease, tricuspid atresia, pulmonary atresia, right ventricular hypertrophy, mitral stenosis, atrial hypertension, pulmonary vascular disease, mitral valve prolapse, mitral regurgitation, ostium secundum ASD, exercise intolerance, tachycardia, bacterial endocarditis, failure to thrive, reactive airway disease

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Contributor Information and Disclosures

Author

Louis I Bezold, MD, Associate Professor, Department of Pediatrics, University of Kentucky College of Medicine; Chief, Division of Pediatric Cardiology, Medical Director, Kentucky Children's Hospital
Louis I Bezold, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Society of Echocardiography, and Society of Pediatric Echocardiography
Disclosure: Nothing to disclose.

Medical Editor

Paul M Seib, MD, Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital
Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Alvin J Chin, MD, Professor of Pediatrics, Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine
Alvin J Chin, MD is a member of the following medical societies: American Association for the Advancement of Science and American Heart Association
Disclosure: Nothing to disclose.

CME Editor

Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College
Gilbert Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine
Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association
Disclosure: Nothing to disclose.

 
 
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