eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology
Aortopulmonary Septal Defect: Treatment & Medication
Updated: Mar 16, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- Medical palliation of aortopulmonary septal defect (APSD) may be performed for several days to weeks to allow elective surgical scheduling. Because an aortopulmonary window does not spontaneously close, surgical repair is necessary to prevent the development of pulmonary vascular obstructive disease (PVOD). Meanwhile, digoxin and diuretics may provide some symptomatic benefit before surgical repair.
- In rare instances (eg, active sepsis), postponing surgery may be desired. In this situation, provide medical therapy (ie, digoxin, inotropic drugs, diuretics) for a brief time in anticipation of surgery.
- The effect of vasodilator agents (eg, angiotensin-converting enzyme [ACE] inhibitors, phosphodiesterase inhibitors, nitrates) is uncertain because these drugs all affect pulmonary resistance in addition to systemic resistance.
- Intubation and positive pressure ventilation with permissive hypercarbia and limiting inspired oxygen concentration to 21% may help limit pulmonary blood flow in infants with torrential pulmonary flow requiring medical palliation. Consider using lower inspired oxygen concentrations (15-19%) to elevate pulmonary vascular resistance (PVR) in more extreme cases. Sedation and muscle relaxants may prove necessary to limit spontaneous ventilation.
- Previous common practice consisted of medically treating a small infant with congestive heart failure (CHF) with the expectation that he or she would grow and become a "better" surgical candidate. This approach is seldom successful because caloric demands of an infant with CHF typically exceed the amount of nutrition delivered by even the most aggressive means.
- Case reports and small case series report closure of small aortopulmonary septal defects in the cardiac catheterization (CC) lab. The Rashkind double umbrella device,16 the Amplatzer duct occluder,17 the Amplatzer septal occluder, muscular ventricular septal defect (VSD) occluder, and perimembranous VSD occluder have all been used to close small (type I) defects.18 The limiting factor to catheter closure of these defects is the anatomy. Only relatively small defects with circumferential tissue rims are amenable to transcatheter device closure, limiting this therapeutic option to a relatively small number of patients.
Angiogram of a small-to-moderate aortopulmonary septal defect in a 4 year-old child. Complete occlusion of the aortopulmonary septal defect with an Amplatzer Duct Occluder. Ao = Ascending aorta; PA = Pulmonary artery.
Surgical Care
Various surgical techniques allow correction of this lesion.19,20,4,21,22,23
- Most centers use techniques that involve cardiopulmonary bypass.
- The aorta and pulmonary artery may be divided, and the defects in the walls may be closed primarily or with patch material. Alternatively, the aorta or pulmonary artery may be opened and the defect patched using autologous, homologous, xenograft, or synthetic material. Two larger case series have reported that transaortic repair is associated with a more favorable outcome and has less risk of causing late pulmonary artery stenosis.
- Other defects may be addressed at the same operation; however, in rare instances, a staged approach may be undertaken.
- Specific techniques for unique anatomy must be individualized.
- Postoperatively, evidence of a good surgical repair should be confirmed. Residual anatomic problems may be anticipated from preoperative anatomy and include, but not be limited to, pulmonary artery stenosis or distortion, residual left-to-right shunt at the aortopulmonary septal defect site, and ascending aortic obstruction or distortion. Postoperative data should be consistent with a complete repair.
- If a pulmonary artery catheter was left in place, it should indicate low pulmonary artery pressure and pulmonary artery oxygen saturation less than 80%.
- An elevated pulmonary artery pressure may indicate pulmonary artery vasoreactivity or a persistent left-to-right shunt. Pulmonary artery saturation and left atrial pressure should differentiate the two conditions. If concerns persist, transthoracic or transesophageal echocardiography may be informative. Rarely, CC may be needed to detect residual abnormalities.
- Apart from anatomic concerns, an older infant or child with elevated preoperative PVR is at risk for postoperative pulmonary hypertension that may require aggressive management.
- Inhaled nitric oxide may be useful in the management of postoperative pulmonary hypertension by acting as a selective pulmonary arteriolar vasodilator.
- Other drugs such as sildenafil or calcium channel blockers may provide ongoing pulmonary vasodilatation.
Consultations
- Consult a pediatric cardiologist for diagnosis. Then, refer the patient to a competent cardiovascular surgical team experienced in the repair of congenital heart disease.
Diet
- High-calorie formula may be needed for infants with CHF perioperatively.
Activity
- Activity generally is not restricted in patients with this defect, except in those with Eisenmenger syndrome.
Medication
Digitalis and diuretics may be used to palliate this condition for a short time before surgical repair as discussed in Medical Care.
Cardiac Glycoside
Digitalis may be used in the management of congestive heart failure (CHF). It exerts positive inotropic effect, which increases the force of contraction of the myocardium. The mode of action by which digitalis improves symptoms is complex but probably results from both increased cardiac contractility and neurohormonal actions.
Digoxin (Lanoxicaps, Lanoxin)
Cardiac glycoside with direct inotropic effects in addition to indirect effects on the cardiovascular system. Acts directly on cardiac muscle, increasing myocardial systolic contractions. Its indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.
May be given as a loading dose followed by a maintenance dose or simply as a maintenance regimen. Digitalis loading increases hazards of this drug. In management of CHF, little, if any, indication for digoxin loading is warranted. For more immediate inotropy, use IV beta-agonists.
Adult
0.125-0.375 mg PO qd
Pediatric
Premature neonates (<37 wk of gestation): 5-8 mcg/kg/d PO divided bid
Neonates: 10 mcg/kg/d PO qd or divided bid
IV dose is 80% of PO dose
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; 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; adjust dose with renal insufficiency
Diuretics
These agents improve symptoms by decreasing total body water, thereby decreasing pulmonary fluid and improving breathlessness. 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 has resulted in edema or ascites. Use multiple strategies to medically manage CHF in infancy. Carefully monitor fluid status and electrolyte balance of infants on anticongestive medications.
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. It is a very effective diuretic yet may cause significant potassium loss.
Adult
20-80 mg/dose PO/IV qd to q6h, titrated prn
Pediatric
1-2 mg/kg/dose PO/IV qd to q6h, titrated prn
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
Documented hypersensitivity; hepatic coma, anuria, and 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, carbon dioxide, glucose, creatinine, uric acid, calcium, and BUN determinations during first few months of therapy and periodically thereafter
Chlorothiazide (Diuril)
Thiazide diuretic acts at the distal part of the nephron to inhibit sodium and chloride reabsorption. Used alone, this agent typically elicits a modest diuresis; however, when combined with furosemide, effects of both agents are potentiated with a potent diuretic effect.
Adult
250-1000 mg/d PO/IV qd or divided bid
Pediatric
20-40 mg/kg/d PO/IV divided bid
May decrease effectiveness of anticoagulants, antigout agents, and sulfonylureas; effectiveness may be decreased by bile acid sequestrants, methenamine, and NSAIDs; may increase the toxicity of allopurinol, anesthetics, antineoplastics, calcium salts, diazoxide, digitalis, lithium, loop diuretics, methyldopa, muscle relaxants, and vitamin D
Documented hypersensitivity; anuria; hypokalemia
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
When used with a loop diuretic, may have very potent diuresis; additional risks include hypercalcemia and hypomagnesemia; adult concerns include increased plasma cholesterol levels and diabetogenic effects in diabetes
Spironolactone (Aldactone)
Potassium-sparing diuretic that works on the distal tubule to inhibit sodium/potassium exchange at the aldosterone site. Although a weak diuretic alone, it helps limit potassium loss when used with other potent diuretics.
Adult
25-200 mg PO qd
Pediatric
1-3.5 mg/kg/d PO qd
May decrease effect of anticoagulants; potassium and potassium-sparing diuretics may increase toxicity of spironolactone
Documented hypersensitivity; anuria, renal failure, hyperkalemia
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Caution in renal and hepatic impairment
More on Aortopulmonary Septal Defect |
| Overview: Aortopulmonary Septal Defect |
| Differential Diagnoses & Workup: Aortopulmonary Septal Defect |
 Treatment & Medication: Aortopulmonary Septal Defect |
| Follow-up: Aortopulmonary Septal Defect |
| Multimedia: Aortopulmonary Septal Defect |
| References |
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References
Kutsche LM, Van Mierop LH. Anatomy and pathogenesis of aorticopulmonary septal defect. Am J Cardiol. Feb 15 1987;59(5):443-7. [Medline].
Mert M, Paker T, Akcevin A, et al. Diagnosis, management, and results of treatment for aortopulmonary window. Cardiol Young. Oct 2004;14(5):506-11. [Medline].
Dipchand AI, Giuffre M, Freedom RM. Tetralogy of Fallot with non-confluent pulmonary arteries and aortopulmonary septal defect. Cardiol Young. Jan 1999;9(1):75-7. [Medline].
Erez E, Dagan O, Georghiou GP, et al. Surgical management of aortopulmonary window and associated lesions. Ann Thorac Surg. Feb 2004;77(2):484-7. [Medline].
Carminati M, Borghi A, Valsecchi O, et al. Aortopulmonary window coexisting with tetralogy of Fallot: echocardiographic diagnosis. Pediatr Cardiol. Jan 1990;11(1):41-3. [Medline].
Marangi D, Peterson RJ, Ceithaml EL, Marvin WJ Jr. Surgical repair of d-transposition with aortopulmonary window: a case report. J Thorac Cardiovasc Surg. Mar 1996;111(3):671-2. [Medline].
Nelson AW. Aorticopulmonary window in a dog. J Am Vet Med Assoc. May 1 1986;188(9):1055-8. [Medline].
Kiran VS, Singh MK, Shah S, John C, Maheshwari S. Lessons learned from a series of patients with missed aortopulmonary windows. Cardiol Young. Oct 2008;18(5):480-4. [Medline].
Valsangiacomo ER, Smallhorn JF. Images in cardiovascular medicine. Prenatal diagnosis of aortopulmonary window by fetal echocardiography. Circulation. Jun 18 2002;105(24):E192. [Medline].
Waters BL, Allen EF, Gibson PC, Johnston T. Autopsy findings in a severely affected infant with a 2q terminal deletion. Am J Med Genet. Nov 15 1993;47(7):1099-103. [Medline].
Bergstrom CS, Saunders RA, Hutchinson AK, Lambert SR. Iris hypoplasia and aorticopulmonary septal defect: a neurocristopathy. J AAPOS. Jun 2005;9(3):264-7. [Medline].
Balaji S, Burch M, Sullivan ID. Accuracy of cross-sectional echocardiography in diagnosis of aortopulmonary window. Am J Cardiol. Mar 15 1991;67(7):650-3. [Medline].
Horimi H, Hasegawa T, Shiraishi H, et al. Detection of aortopulmonary window with ventricular septal defect by Doppler color flow imaging. Chest. Jan 1992;101(1):280-1. [Medline].
Garver KA, Hernandez RJ, Vermilion RP, Goble M. Images in cardiovascular medicine. Correlative imaging of aortopulmonary window: demonstration with echocardiography, angiography, and MRI. Circulation. Aug 5 1997;96(3):1036-7. [Medline].
Singh A, Mehmood F, Romp RL, Nanda NC, Mallavarapu RK. Live/Real time three-dimensional transthoracic echocardiographic assessment of aortopulmonary window. Echocardiography. Jan 2008;25(1):96-9. [Medline].
Stamato T, Benson LN, Smallhorn JF, Freedom RM. Transcatheter closure of an aortopulmonary window with a modified double umbrella occluder system. Cathet Cardiovasc Diagn. Jun 1995;35(2):165-7. [Medline].
Naik GD, Chandra VS, Shenoy A, et al. Transcatheter closure of aortopulmonary window using Amplatzer device. Catheter Cardiovasc Interv. Jul 2003;59(3):402-5. [Medline].
Trehan V, Nigam A, Tyagi S. Percutaneous closure of nonrestrictive aortopulmonary window in three infants. Catheter Cardiovasc Interv. Feb 15 2008;71(3):405-11. [Medline].
Backer CL, Mavroudis C. Surgical management of aortopulmonary window: a 40-year experience. Eur J Cardiothorac Surg. May 2002;21(5):773-9. [Medline].
Di Bella I, Gladstone DJ. Surgical management of aortopulmonary window. Ann Thorac Surg. Mar 1998;65(3):768-70. [Medline].
Hew CC, Bacha EA, Zurakowski D, et al. Optimal surgical approach for repair of aortopulmonary window. Cardiol Young. Jul 2001;11(4):385-90. [Medline].
Prasad TR, Valiathan MS, Shyamakrishnan KG, Venkitachalam CG. Surgical management of aortopulmonary septal defect. Ann Thorac Surg. Jun 1989;47(6):877-9. [Medline].
Tkebuchava T, von Segesser LK, Vogt PR, et al. Congenital aortopulmonary window: diagnosis, surgical technique and long-term results. Eur J Cardiothorac Surg. Feb 1997;11(2):293-7. [Medline].
Nishimura RA, Carabello BA, Faxon DP, et al. ACC/AHA 2008 guideline update on valvular heart disease: focused update on infective endocarditis: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines: endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation. Aug 19 2008;118(8):887-96. [Medline]. [Full Text].
Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. Oct 9 2007;116(15):1736-54. [Medline]. [Full Text].
Chopra PS, Reed WH, Wilson AD, Rao PS. Delayed presentation of anomalous circumflex coronary artery arising from pulmonary artery following repair of aortopulmonary window in infancy. Chest. Dec 1994;106(6):1920-2. [Medline].
Grunenfelder J, Zund G, Vogt PR, Turina MI. Aortopulmonary window with anomalous origin of the right coronary artery. Ann Thorac Surg. Jan 1999;67(1):233-5. [Medline].
Izumoto H, Ishihara K, Fujii Y, et al. AP Window and Anomalous Origin of the Right Coronary Artery From the Window. Annals of Thoracic Surgery. 1999;68:557-9. [Medline].
McElhinney DB, Reddy VM, Tworetzky W, et al. Early and late results after repair of aortopulmonary septal defect and associated anomalies in infants <6 months of age. Am J Cardiol. Jan 15 1998;81(2):195-201. [Medline].
Tulloh RM, Rigby ML. Transcatheter umbrella closure of aorto-pulmonary window. Heart. May 1997;77(5):479-80. [Medline].
van Son JA, Puga FJ, Danielson GK, et al. Aortopulmonary window: factors associated with early and late success after surgical treatment. Mayo Clin Proc. Feb 1993;68(2):128-33. [Medline].
Further Reading
Keywords
aortopulmonary septal defect, APSD, aorticopulmonary septal defect, aortopulmonary window, AP window, aortopulmonary fenestration, heart disease, treatment, diagnosis, patent ductus arteriosus, PDA, interrupted aortic arch, ventricular septal defect, VSD, tetralogy of Fallot, aortic atresia, transposition of the great arteries, double aortic arch, congestive heart failure, pulmonary vascular obstructive disease, coarctation of the aorta, cyanosis, hepatomegaly, failure to thrive, VACTERL association
