Atrial Septal Defect, Ostium Primum Follow-up

  • Author: Shannon M Rivenes, MD; Chief Editor: Steven R Neish, MD, SM   more...
 
Updated: Aug 11, 2010
 

Further Inpatient Care

  • Low-risk patients with ostium primum atrial septal defects (ASDs) who undergo successful intracardiac repairs generally do well after surgery. Fluid restriction is liberalized and diuretic therapy weaned over a period of 3-5 days after surgery. Initially, electrolytes are closely monitored, with frequency decreasing as diuretic therapy is weaned. Patients on preoperative ACE inhibition may remain on continued therapy for a period of time. Activity and diet are advanced. A postoperative transthoracic echocardiography is generally performed before discharge or at the first postoperative visit. Care must be taken to avoid trauma to the chest for 8-12 weeks postoperatively.
  • Some patients may develop a postpericardiotomy syndrome manifested by chest pain, fever, pericardial inflammation with a rub, and pericardial effusion. High-dose salicylates or nonsteroidal anti-inflammatory drugs (NSAIDs) generally improve symptoms. Hemodynamically significant effusions may require pericardiocentesis. Failure to respond to salicylates may warrant pulsed steroid therapy.
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Further Outpatient Care

  • Follow-up is warranted within 1-2 weeks of surgery. Vital signs, history, and physical examination are assessed, and sutures are removed. An ECG generally is obtained to rule out conduction abnormalities or arrhythmias. Chest radiography is performed to assess potential pleural or pericardial fluid and to assess heart size and pulmonary vasculature. Echocardiography may be performed as a limited study to assess function and effusion or performed on an inpatient basis as a complete postoperative study. Further outpatient care is dictated by findings from the initial visit.
  • Long-term follow-up is required for all patients. Both the tricuspid and mitral valves tend to be abnormal, with the potential for deterioration with advancing age. Subacute bacterial endocarditis (SBE) prophylaxis is warranted for a minimum of 6 months postoperatively and may be prudent for life because of the abnormal atrioventricular (AV) valve tissue adjacent to suture/patch material. The development and/or progression of AV conduction abnormalities also warrant continued observation.
  • In patients who have not undergone repair for isolated small-to-moderate ASDs, follow-up in clinic is usually every 6 months to a year. Exercise intolerance, increasing fatigue, palpitations, and frequent lower respiratory infections or wheezing may merit referral for earlier repair. Palpitations may need to be evaluated with a Holter monitor and ECG. Chest radiography is warranted to follow heart size and pulmonary vascular markings.
  • For patients with mitral regurgitation, closer follow-up is usually necessary. In addition to the above, monitoring for progression of mitral regurgitation via physical examination, chest radiography, and echocardiography is important. It is important to time surgery before a deterioration in ventricular function if ventricular dilatation is noted. Remember that left ventricular function may appear near normal in the setting of moderate-to-severe mitral regurgitation because of the reduced afterload related to mitral regurgitation. Ventricular function may worsen significantly when a competent mitral valve is in place.
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Inpatient & Outpatient Medications

  • Immediately following surgery, patients generally receive intravenous (IV) diuretic therapy, traditionally furosemide. During recovery, IV preparations are changed to oral (PO) formulations and the dose is decreased. Patients are typically discharged on twice-daily dosing. Diuretics are weaned over a period of weeks to months, dictated by physical findings and roentgenographic assessment.
  • Patients often require inotropic support and/or afterload reduction in the early postoperative period. Ongoing support may be warranted with oral ACE inhibition following discharge. As heart size and systolic function normalize, ACE inhibition may be reduced or discontinued in the outpatient setting. With persistent or evolving significant mitral regurgitation, afterload reduction should be continued.
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Deterrence/Prevention

  • Prevention of congenital heart defects lies in continued research at the molecular genetics level. No effective preventive therapies are available at this time.
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Complications

  • Infective endocarditis remains both a preoperative and a postoperative complication. In a study from the Oregon Health Sciences University, the 30-year postoperative incidence of infective endocarditis was 2.8% among patients with ostium primum ASDs.[2]
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Prognosis

  • Pediatric patients with small left-to-right shunts and no significant mitral regurgitation who have not undergone surgery are at relatively low risk for complications. In these patients, adult survival is expected, but complications can develop as age advances. Untreated patients with large shunts and/or significant mitral regurgitation are at significant risk of morbidity and mortality. Death, arrhythmia, heart block, refractory heart failure, and advanced pulmonary vascular disease are the most common complications and tend to increase with advancing age. Pulmonary vascular obstructive disease may develop in a subset of patients, with patients with Down syndrome at highest risk. Prognosis is guarded, and morbidity and mortality are high regardless of therapy.
  • Surgical repair generally improves life expectancy and alters the natural course of the disease. Long-term outcome of 180 children with ostium primum ASDs from 1982-1996 was assessed by the group at the Hospital for Sick Children in Toronto.[3] Mean age at repair was 4.6 years, with 23 patients younger than 1 year. Absent or mild symptoms were reported in 145 patients (80%), and severe symptoms or congestive heart failure (CHF) were reported in 34 patients (20%). Follow-up ranged from 2 months to 14.5 years (mean, 6 ± 4.2 y). Early mortality occurred in 3 patients (1.6%); 2 were infants. Seventeen patients (9%) underwent reoperation (5 infants). Five patients underwent reoperation for subaortic obstruction, 12 for left AV valve regurgitation (1 required valve replacement). Actuarial survival was 98% at 10 years with no late deaths. Age and preoperative moderate-to-severe left AV valve regurgitation werepredictors of reoperation. Age at repair younger than 1 year was a predictor of death.
  • A smaller study from the Oregon Health Sciences University assessed 38 consecutive patients aged 3-58 months who underwent correction between 1981 and 1997. Moderate-to-severe mitral regurgitation was present in 45% of patients, and CHF was present in 41%. Closure of the mitral cleft was performed in 92% of patients, and 28% underwent a mitral annuloplasty. The early 30-day mortality was 7.9%. A low incidence of late mitral regurgitation (0.9%) with only one late reoperation was noted on a follow-up lasting 14 years. Eighty-seven percent of patients remained asymptomatic at the last follow-up visit. The study concluded that an aggressive approach to operating at an early age is safe, effective, and yields excellent long-term results.
  • The conclusions of the Oregon Health Sciences University study were supported by Italian data, which documented 93.5% (±2%) freedom from reoperation at 12.3 years for partial AV canal defects.[4] Rates were highest in patients with preoperative AV valve regurgitation and a double orifice left AV valve and were statistically lower for patients who had early repair using a bifoliate approach. Results were attributed to the prevention of progressive mitral annular dilatation.
  • The experience in Sapporo, Japan was more recently reviewed, spanning the years of 1983-2002.[1] Repair was performed in 61 patients with AV canal defects, including 7 with a transitional form. Age of operation ranged from 1 month to 62 years, with a median age of 5.3 years. All patients underwent patch closure of the ASD. The cleft was left at least partially open prior to 1995 and completely closed after 1996. The 10-year actuarial survival rate was 91%. Actuarial freedom from reoperation for mitral regurgitation was 91% at 10 years, notably decreased to 89% at 5 years and 78% at 10 years in patients with grade III or higher postoperative mitral insufficiency. Age at operation, preoperative degree of mitral regurgitation, and method of cleft repair (all previously assumed to be significant) were found not to be significant risk factors.
  • The adult experience was reviewed from 1958-1990 at the Mayo Clinic, encompassing 31 patients aged 40-71 years at the time of repair;[5] 23 had repair of the mitral cleft, 2 required mitral valve replacements, and 6 warranted mitral reoperation. Early mortality was 6%; 19 patients were followed for a mean of 14 years, with 14 reporting a sustained postoperative improvement.
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Patient Education

  • For excellent patient education resources, visit eMedicine's Heart Center. Also, see eMedicine's patient education article Palpitations.
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Contributor Information and Disclosures
Author

Shannon M Rivenes, MD  Assistant Professor, Department of Pediatrics, Division of Pediatric Cardiology, Texas Children's Hospital and Baylor College of Medicine

Shannon M Rivenes, MD is a member of the following medical societies: Alpha Omega Alpha, 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; Pharmacy Editor, eMedicine

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.

References

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  2. Morris CD, Reller MD, Menashe VD. Thirty-year incidence of infective endocarditis after surgery for congenital heart defect. JAMA. Feb 25 1998;279(8):599-603. [Medline].

  3. Najm HK, Williams WG, Chuaratanaphong S, et al. Primum atrial septal defect in children: early results, risk factors, and freedom from reoperation. Ann Thorac Surg. Sep 1998;66(3):829-35. [Medline].

  4. Michielon G, Stellin G, Rizzoli G, Milanesi O, Rubino M, Moreolo GS, et al. Left atrioventricular valve incompetence after repair of common atrioventricular canal defects. Ann Thorac Surg. Dec 1995;60(6 Suppl):S604-9. [Medline].

  5. Bergin ML, Warnes CA, Tajik AJ, Danielson GK. Partial atrioventricular canal defect: long-term follow-up after initial repair in patients > or = 40 years old. J Am Coll Cardiol. Apr 1995;25(5):1189-94. [Medline].

  6. Agny M, Cobanoglu A. Repair of Partial Atrioventricular Septal Defect in Children Less than Five Years of Age: Late Results. Ann Thorac Surg. May 1999;67(5):1412-4. [Medline].

  7. Arky, Ronald. Physicians' Desk Reference. 52nd ed. Montvale, NJ: Medical Economics Co Inc; 1998:784-7, 1051-1062, 1219-1221.

  8. Castaneda AR, Jonas RA, Mayer JE. Atrioventricular canal defect. In: Cardiac Surgery of the Neonate and Infant. 1994:167-86.

  9. Cheitlin MD, Douglas PS, Parmley WW. 26th Bethesda conference: recommendations for determining eligibility for competition in athletes with cardiovascular abnormalities. Task Force 2: acquired valvular heart disease. J Am Coll Cardiol. Oct 1994;24(4):874-80. [Medline].

  10. Del Nido PJ, Bichell DP. Minimal-access surgery for congenital heart defects. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 1998;1():75-80. [Medline].

  11. Garson A Jr, Bricker JT, Fisher DJ. The Science and Practice of Pediatric Cardiology. 2nd ed. Williams & Wilkins; 1998:1158-179.

  12. Giamberti A, Mazzera E, Di Chiara L, Ferretti E, Pasquini L, Di Donato RM. Right submammary minithoracotomy for repair of congenital heart defects. Eur J Cardiothorac Surg. Dec 2000;18(6):678-82. [Medline].

  13. Gilman AG, Goodman LS, Nies AS. Goodman and Gilman's The Pharmacological Basis of Therapeutics. 8th ed. 1990:721-5, 749-63, 814-839.

  14. Graham TP Jr, Bricker JT, James FW, Strong WB. 26th Bethesda conference: recommendations for determining eligibility for competition in athletes with cardiovascular abnormalities. Task Force 1: congenital heart disease. J Am Coll Cardiol. Oct 1994;24(4):867-73. [Medline].

  15. Kaur A, Srivastava S, Lytrivi ID, Nguyen K, Lai WW, Parness IA. Echocardiographic evaluation and surgical implications of common atrioventricular canal defects with absent or diminutive ostium primum defect. Am J Cardiol. Jun 1 2008;101(11):1648-51. [Medline].

  16. Lange A, Mankad P, Walayat M, et al. Transthoracic three-dimensional echocardiography in the preoperative assessment of atrioventricular septal defect morphology. Am J Cardiol. Mar 1 2000;85(5):630-5. [Medline].

  17. Marino B, Digilio MC, Toscano A, et al. Congenital heart diseases in children with Noonan syndrome: An expanded cardiac spectrum with high prevalence of atrioventricular canal. J Pediatr. Dec 1999;135(6):703-6. [Medline].

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  20. Sadler TW. Langman's Medical Embryology. 5th ed. Baltimore, MD: Williams & Wilkins; 1985:176-84.

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  22. Zanchetta M, Rigatelli G, Pedon L, et al. Role of intracardiac echocardiography in atrial septal abnormalities. J Interv Cardiol. Feb 2003;16(1):63-77. [Medline].

 
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ECG from a patient with a partial atrioventricular septal defect. The PR interval is mildly prolonged. Left axis deviation with Q waves in leads I and aVL are present, consistent with a counterclockwise loop in the frontal plane. Right atrial enlargement and an rsR' pattern in the right chest leads also are noted.
Two-dimensional, apical, 4-chamber echocardiogram of a partial atrioventricular (AV) canal defect. The asterisk (*) delineates an area of dropout in the inferior atrial septum at the site of the primum atrial septal defect. The AV valves are separate but aligned at the same horizontal level, consistent with a 2-orifice common AV valve. In systole, the medial leaflets of the right- and left-sided AV valves demonstrate attachments to the crest of the interventricular septum, allowing no ventricular level shunting. RA = Right atrium; LA = Left atrium; RV = Right ventricle; LV = Left ventricle.
Gross pathology specimen viewed from the opened left atrium and left ventricle, demonstrating a partial atrioventricular (AV) canal defect. An ostium primum atrial septal defect (ASD) marked by an asterisk (*) is visualized in the inferior aspect of the interatrial septum. An ostium secundum ASD marked by 2 asterisks (**) is also noted. The mitral valve is cleft and the leaflets are thickened and rolled, suggestive of chronic mitral regurgitation. LA = Left atrium; LV = Left ventricle; MV = Mitral valve.
 
 
 
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