Pediatric Complete Atrioventricular Septal Defects Treatment & Management

  • Author: Michael D Pettersen, MD; Chief Editor: Steven R Neish, MD, SM   more...
 
Updated: Oct 12, 2011
 

Medical Care

Although their effectiveness in complete atrioventricular septal defect (AVSD) has been questioned, diuretics, digoxin, and ACE inhibitors have all been used to alleviate tachypnea and failure to thrive.

In many medical centers, the surgical mortality rate at age 2-3 months is 5% or less. Therefore, unless symptoms are dramatically relived, medical treatment for children with symptoms of congestive heart failure (CHF) is not pursued for more than a few weeks before definitive repair.

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Surgical Care

Treatment for a complete atrioventricular septal defect is surgical.

Single-stage complete repair is currently preferred, but occasional cases of refractory CHF in a low-birth-weight infant may be palliated with the placement of a pulmonary artery band.

Some patients with complete atrioventricular septal defect have additional muscular ventricular septal defects (VSDs). Banding of the pulmonary artery may alleviate their CHF for 6-12 months, during which time the VSDs may spontaneously close and thus simplify eventual complete repair.

The surgical mortality rate should be low. In a published review of surgical outcomes of 363 patients with atrioventricular septal defects who were treated between 1982 and 1995, the early mortality rate was 10.3%, and the 10-year survival rate was 83%.[25]

The Pediatric Heart Network Investigators recently published a multicenter observational study on the contemporary results after repair of complete atrioventricular septal defect. In this series of 120 children, in-hospital and 6-month mortality rates were 2.5% and 4%, respectively. The incidence of residual septal defects and the degree of left atrioventricular valve regurgitation was independent of repair type, presence of trisomy 21, and age of operation, although younger age of operation was associated with a longer hospital stay.[26]

Another study, also from the Pediatric Heart Network Investigators, assessed the influence of atrial VSD subtype on outcomes after repair. Preoperatively, transitional patients showed the highest prevalence of moderate or severe left atrioventricular valve regurgitation (LAVVR). In data obtained 1 and 6 months post AVSD repair, the results noted that complete atrial VSD and canal-type VSD patients showed the highest prevalence of trisomy 21 and were younger, had lower weight-for-age z scores, and had more associated cardiac defects. Annuloplasty was similar among all subtypes, while complete atrial VSD showed a longer duration of ventilation and hospitalization. At 6 months, weight-for-age z scores improved and improvement was similar in all subtypes.[27]

A pulmonary artery band, placed on the main pulmonary artery by means of a small lateral or anterior thoracotomy incision, obviates cardiopulmonary bypass in a premature neonate or small infant. However, it has the risks of distorting the origins of the branch pulmonary arteries if it migrates and of complicating the eventual definitive surgery if it erodes through the media and intima. Pulmonary artery banding is best used, when deemed necessary, for only a few months in a patient who then undergo complete intracardiac repair and pulmonary artery band takedown.

A major aspect of atrioventricular septal defect repair involves creating a competent mitral valve. A pericardial patch can be used for this augmentation and for tricuspid valve repair. Repair is occasionally done with 2 patches: a pericardial patch for the atrial septal defect and a polytetrafluoroethylene patch (Gore-Tex patch; W.L. Gore & Associates, Inc, Newark, DE) for the VSD, with routine closure of the mitral valve cleft. The 2-patch technique with routine cleft closure and atrial septal incision may lower the incidence of residual mitral regurgitation.

Ten Harkel et al reported intermediate follow-up results in patients who underwent surgical repair of atrioventricular septal defects.[28] During a mean follow-up of 66 months, 19% had severe mitral valve regurgitation, and 9% required reoperation. Of note, 13% of patients with severe mitral valve regurgitation in the immediate postoperative period had significantly improved mitral valve function. For this reason, the authors cautioned against reoperation in the early postoperative period unless it is absolutely necessary.

Additional aspects of complete repair of common atrioventricular canal may include relief of associated left ventricular (LV) outflow tract (LVOT) obstruction, patent ductus arteriosus (PDA) ligation, removal of a previously placed pulmonary artery band, repair of stenosis of a pulmonary arterial branch, or relief of aortic arch obstruction. Recent data suggest that children with atrioventricular septal defects and Down syndrome have a prognosis better than that of children with the same cardiac lesion but not Down syndrome.

Residual atrioventricular valve insufficiency or stenosis is a major determinant of long-term outcome.

Total circular annuloplasty is a simple procedure to help reduce atrioventricular valve regurgitation, although most patients with severe atrioventricular valve insufficiency or stenosis require more complex mitral valvuloplasty techniques. The need for mitral-valve replacement is not rare over the course of long-term follow-up but is ideally delayed until an adult-size prosthetic valve can be implanted.

Atrioventricular septal defect may be associated with other surgical conditions, including subaortic stenosis, coarctation of the aorta, tetralogy of Fallot (TOF), and total anomalous pulmonary venous return. Each associated lesion may complicate complete repair and make it difficult to achieve a good hemodynamic result. In addition, these defects may add potential risk over follow-up (eg, recoarctation after coarctation of the aorta repair or pulmonary insufficiency after repair of TOF).

Surgically induced atrioventricular block is a known complication of atrioventricular septal defect repair. Permanent pacing is required if atrioventricular conduction does not return postoperatively.

For complex cardiac lesions involving an unbalanced atrioventricular septal defect, total cavopulmonary connection, otherwise known as a Fontan operation, may be indicated.

In the presence of TOF, an aortic monocusp is used to compensate for deficient right atrioventricular valve tissue. Right-dominant, unbalanced biventricular repair can be successfully completed in patients with mild LV hypoplasia. However, careful preoperative evaluation of the adequacy of the LV to support the systemic circulation is imperative.

De Oliveira et al reported their experience with 2-ventricular repair of patients with unbalanced atrioventricular septal defect and small right ventricles (RVs). Patients with a small RV had a high mortality rate, with an 87% 10-year survival, compared with a 100% survival rate in surgical patients with balanced atrioventricular septal defect. Although a median sternotomy is the usual surgical approach, the thoracotomy approach was safely used for common atrioventricular canal repair in some centers.

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Consultations

Given the complexity of atrioventricular septal defect, a multidisciplinary team is usually required. This could include pediatric cardiologists, pediatric cardiothoracic surgeons, pediatricians, neonatologists, and pediatric intensivists, as well as a nurse coordinator and supportive ancillary staff.

Additional consultants might include a geneticist for genetic counseling and a nutritionist.

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Diet

A high-energy diet is needed because cardiac shunting results in high metabolic demands. Even at 125 kcal/kg/d, children still may not appropriately gain weight. Some children have such high metabolic demands that extraordinary energy intake, exceeding 150 kcal/kg/d, is necessary for growth.

Pulmonary edema can lead to tachypnea that makes oral intake of nourishment too difficult. A nasogastric tube may be needed in severe cases of congestive heart failure (CHF) with failure to thrive.

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Activity

After the patient recovers from surgery, normal daily activities should be allowed.

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Proceed to Medication
 
 
Contributor Information and Disclosures
Author

Michael D Pettersen, MD  Director of Echocardiography, Division of Cardiology, Children's Hospital of Michigan; Associate Professor of Pediatrics, Wayne State University School of Medicine

Michael D Pettersen, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, and American Society of Echocardiography

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

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  Professor of Pediatrics, University of Pennsylvania School of Medicine; Attending Physician, Cardiology Division, Children's Hospital of Philadelphia

Alvin J Chin, MD, is a member of the following medical societies: American Association for the Advancement of Science, American Heart Association, and Society for Developmental Biology

Disclosure: Nothing to disclose.

Gilbert Z Herzberg, MD  Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center

Gilbert Z 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.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors Michael McConnell, MD, and John Scheitler, MD, to the original writing and development of this article.

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Atrioventricular (A-V) valve leaflets viewed from the cardiac apex in normal valves (A) and in the Rastelli type A complete form of common A-V canal (B). In A, the normal tricuspid valve (TV) has anterior (AL), septal (SL), and posterior (PL) leaflets. A normal mitral valve (MV) has ALs and PLs.In B, the superior cushion–derived leaflet bridges the ventricular septum and attaches to the papillary muscle of the conus at its rightmost extent. A right superior leaflet (RSL) typically attaches to the papillary muscle of the conus and to the anterior papillary muscle of the right ventricle (RV), and a right lateral leaflet (RLL) attaches to the anterior papillary muscle of the RV and to the posterior papillary muscle of the RV. The inferior cushion–derived bridging leaflet is usually cleft, giving the appearance of a right inferior leaflet (RIL) and a left inferior leaflet (LIL).
Apical 4-chamber echocardiographic image demonstrating a complete atrioventricular septal defect. A large primum atrial septal defect, a large inlet ventricular septal defect, and a single common orifice atrioventricular valve are noted.
Apical 4-chamber echocardiographic image with color Doppler demonstrating moderately-severe insufficiency of the common atrioventricular valve.
Parasternal long axis echocardiographic image of a complete atrioventricular septal defect. A large inlet ventricular septal defect is seen. Accessory atrioventricular valve tissue is visualized within the left ventricular outflow tract.
Subcostal sagittal echocardiographic image demonstrating the common atrioventricular valve. The anterior bridging leaflet inserts onto the interventricular septum consistent with a Rastelli type A valve.
 
 
 
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