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Aortopulmonary Septal Defect Treatment & Management

  • Author: Barry A Love, MD; Chief Editor: Stuart Berger, MD  more...
 
Updated: Feb 05, 2015
 

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,[21] the Amplatzer duct occluder,[22] the Amplatzer septal occluder, muscular ventricular septal defect (VSD) occluder, and perimembranous VSD occluder have all been used to close small (type I) defects.[23] 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. See the image below.

Angiogram of a small-to-moderate aortopulmonary se 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.

Important considerations

The following are essential issues to keep in mind:

  • Early recognition of serious heart disease with associated pulmonary hypertension and high pulmonary vascular resistance (PVR) is important, as irreversible pulmonary vascular obstructive disease (PVOD) may develop (one of the most feared scenarios in pediatrics and pediatric cardiology)
  • Realization that heart diseases, including large ventricular septal defects (VSDs), patent ductus arteriosus (PDA), and aortopulmonary septal defect (APSD), may present in this fashion (When PVR does not fall after birth, children may not have symptoms of congestive heart failure [CHF] and may feed and grow normally with a paucity of cardiac findings.)
  • Recognition of loud and single second heart sounds that indicate the existence of pulmonary hypertension, thereby warranting further workup
  • Ensuring clearly discernment of a splitting of the second heart sound with respiration on each cardiac examination on every child, and referral of the patient to a pediatric cardiologist for evaluation if the heart sounds are suspicious
  • Identification of aortopulmonary septal defect as the cause of a large left-to-right shunt causing CHF

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.

Transfer

Transport patients, if needed, to a facility with the appropriate pediatric and/or pediatric cardiac surgical services.

Diet and activity

A high-calorie formula may be needed for infants with CHF perioperatively. Generally, activity is not restricted in patients with this defect, except in those with Eisenmenger syndrome.

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Surgical Care

Various surgical techniques allow correction of this lesion.[5, 24, 25, 26, 27, 28]

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.

A retrospective review (2002-2011) reported good outcomes associated with the use of a single pericardial patch technique for primary repair of an aortopulmonary window with an interrupted aortic arch.[29] In 6 of 11 patients, the interrupted aortic arch was type A; the remaining 5 patients had type B. Median age at the time of surgery was 11 days, with a m ean weight of 2.6 kg; mean follow-up was 6 ± 3 years. There were no early and late deaths nor reoperations. There was one case of postoperative stroke without late sequelae. All patients at last follow-up visit had no recurrent aortic arch obstruction or pulmonary artery branch stenosis.[29]

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. A case report indicated successful transcatheter closure of a late-onset residual shunt following repair of an aortopulmonary septal defect with the use of a muscular ventricular septal occlude.[30]

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.

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Contributor Information and Disclosures
Author

Barry A Love, MD Assistant Professor , Department of Medicine, Division of Cardiology, Assistant Professor, Division Pediatric Cardiology, Director, Pediatric Electrophysiology Service, Department of Pediatrics, Division of Pediatric Cardiology, Mount Sinai School of Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Alvin J Chin, MD Emeritus Professor of Pediatrics, 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, Society for Developmental Biology, American Heart Association

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

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, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

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Echocardiographic image of a 1-month-old infant with a large isolated aortopulmonary septal defect (APSD). The image is a parasternal short-axis view just below the pulmonary artery bifurcation. Aorta at this level is to the right and in the same anterior-posterior plane as the main pulmonary artery (MPA). Right pulmonary artery is seen posterior to the aorta at this level, but the origin of the pulmonary arteries is not visible; it is more superior than this axial image. Normally, a complete wall should be visible for both aorta and pulmonary artery. This image shows the absence of that wall, resulting in the large defect between aorta and pulmonary artery.
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.
 
 
 
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