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

Atrioventricular Septal Defect, Complete: Follow-up

Author: Michael Pettersen, MD, Director of Echocardiography, Division of Cardiology, Children's Hospital of Michigan; Associate Professor of Pediatrics, Wayne State University School of Medicine
Contributor Information and Disclosures

Updated: Nov 10, 2009

Follow-up

Further Inpatient Care

  • Some children who have survived surgical repair of complete atrioventricular septal defect (AVSD) require prolonged hospitalization.
  • Causes are multifactorial but may include sepsis, pulmonary hypertension, residual left-to-right shunts through ventricular septal defects (VSDs), or significant atrioventricular valve insufficiency.
  • Associated noncardiac problems, including feeding difficulties, renal insufficiency, or pulmonary insufficiency, may require ongoing management and delayed discharge.

Further Outpatient Care

  • As needed, outpatient care for nonsurgical patients with atrioventricular septal defect should focus on providing adequate nutrition and medications to lessen congestive heat failure (CHF) symptoms.
  • Outpatient care should prepare the child for surgical intervention at the age appropriate for the institution where the operation will be performed.
  • Postoperative outpatient care depends on any clinically significant residual problems.

Inpatient & Outpatient Medications

  • Postoperative medications range from none to many of the medications used to treat CHF discussed in the Medication section.
  • Almost every child who has survived surgical repair of complete atrioventricular septal defect has some abnormality of an atrioventricular valve.
  • Antibiotic prophylaxis is recommended for patients during the first 6 months after complete repair and for patients who have a residual intracardiac shunt associated with prosthetic patch material.

Transfer

  • Patients with complete atrioventricular septal defect should be transferred to an institution skilled in successfully treating patients with complex congenital heart disease.

Complications

  • Postoperative complications include arrhythmias, low cardiac output, pulmonary hypertension, atrioventricular valve stenosis, and mitral insufficiency. Arrhythmias include heart block and junctional tachycardia; the latter usually subsides within 3-7 days after surgery. Postoperative left ventricular dysfunction may result in low cardiac output and even renal insufficiency. Inotropic drugs may be needed for several days after surgery.
    • In patients with pulmonary hypertension, sedation, paralysis, and mild hyperventilation with 100% oxygen may be required to prevent pulmonary hypertensive crisis and decreased right ventricular (RV) afterload. For pulmonary hypertension refractory to these measures, nitric oxide may be used to achieve pulmonary vasodilatation.
    • On occasion, patients may require long-term therapy, which might include phenoxybenzamine or calcium-channel–blocking drugs to manage an elevated pulmonary vascular resistance (PVR).
    • Severe mitral regurgitation may occur postoperatively and is ideally recognized on intraoperative transesophageal echocardiography (TEE). Residual mitral regurgitation is the most common indication for late reoperation after repair of complete atrioventricular septal defect. Approximately 5-10% of patients ultimately require mitral valve repair or replacment.28,29
    • Anomalous attachment of atrioventricular valve tissue occasionally causes left ventricular (LV) outflow tract (LVOT) obstruction, in addition to the known tendency for patients with atrioventricular septal defect to have a small LVOT.30 Such anomalous attachments may prevent complete relief of subaortic obstruction without mitral-valve replacement. Resection of some discrete obstructing tissue or, in some patients, a modified Konno procedure for tunnel-like LVOT obstruction or for obstruction caused by anomalous attachments of the mitral-valve apparatus may be performed. This procedure may complicate outflow-tract reconstruction and has had varied results.
  • A rare complication of the complete atrioventricular canal is subacute bacterial endocarditis. Successful repair during active endocarditis is reported.

Prognosis

  • Without operation, the survival of patients with this lesion is poor. Death may occur during infancy secondary to heart failure or pneumonia. Death later in childhood results from progressive pulmonary vascular obstructive disease (PVOD). PVOD tends to develop more rapidly than in other congenital heart defects. Intimal fibrosis (Heath-Edwards grade 3 lesions) have been demonstrated to appear between age 6-12 months. Vascular dilation and plexiform lesions (Heath-Edward grade 4 lesions) may occur by age 1 year.31 Evidence suggests that these changes develop earlier and progress more rapidly in infants with Down syndrome.
  • Risk factors for surgical and late mortality and morbidity are identified. Preoperative risk factors for mortality include small size, unbalanced ventricular size, New York Heart Association class IV, and severe atrioventricular valve insufficiency. The era of operation (before 1987), patient age at operation, presence of accessory atrioventricular valve orifices, and other congenital heart diseases also increase the surgical mortality risk. Down syndrome is surprisingly not an independent risk factor for morbidity and mortality and therefore should not limit intervention.
  • Infants can successfully undergo surgery, with a published mortality rate of 3.6% and with minimal long-term morbidity. Late survival is approximately 96%, and the reoperation rate is approximately 11%.
  • Published 10-year survival rates are 81-91%. Risk factors strongly associated with early death or the need for repeat operation include operation before 1987, postoperative pulmonary hypertensive crisis, immediate postoperative severe left atrioventricular valve regurgitation, and double-orifice left atrioventricular valve. In the past, death was significantly most common when complete atrioventricular septal defect with RV outflow tract obstruction was corrected in children younger than 5 years or weighing less than 15 kg. In the current era, most centers operate by age 6 months.

Patient Education

  • For excellent patient education resources, visit eMedicine's Heart Center.

Miscellaneous

Medicolegal Pitfalls

  • Failure to recognize common atrioventricular canal early in life is the primary medicolegal pitfall.
  • Common atrioventricular canal is often recognized early in the life of the patient, especially in an infant with Down syndrome, only because of abnormal precordial activity, a loud, single S2, or both.
  • Because data from most studies suggest that approximately 50% of children with Down syndrome have congenital heart disease and because the physical findings are often subtle, early referral of a patient with Down syndrome to a pediatric cardiologist is recommended.

Special Concerns

  • Women who undergo successful repair of common atrioventricular canal should be able to tolerate pregnancy well if they are asymptomatic. If the patient has clinically significant residual atrioventricular valve insufficiency, a clinically significant residual ventricular septal defect (VSD), or poor ventricular function, the risks to both the mother and the fetus rise. The risk of a fetal congenital heart disease may be as high as 14% (range, 10-15%).
  • Data from some studies suggest that patients with congenital heart disease have substantially more stress in their lives than patients without chronic diseases. Even if this is true, findings suggest that children who have congenital heart disease have educational and occupational rates higher than those of age-matched control patients. Children with Down syndrome or other syndromes that affect cognitive function perform less well than other children.
  • Common atrioventricular canal defect is an endocardial cushion malformation resulting in an atrial septal defect, a VSD, and a common atrioventricular valve. Causes are multifactorial. Although the natural history is somewhat ominous, technologic advances over the past 20 years have greatly aided diagnosis and surgical correction of this complex malformation, yielding promising results.
 
Acknowledgments

The authors and editors of eMedicine 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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References
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Further Reading

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Keywords

complete atrioventricular septal defect, complete AVSD, endocardial cushion defect, ECD, common AV canal defect, AVC defect, CAVC, congestive heart failure, CHF, treatment, diagnosis

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Contributor Information and Disclosures

Author

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

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 financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

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 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.

 
 
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