Atrial septal defect ostium primum indications for surgical repair

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S. [2]; Cafer Zorkun, M.D., Ph.D. [3] Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [4]

Overview

The decision to surgically close an atrial septal defect depends upon many contributing factors including the type of defect, the size of defect, the amount of left-to-right shunting, the development or worsening of symptoms, the presence of pulmonary hypertension and the presence of any associated anomalies.

Indications for Surgical Repair in Adults

In general, an ASD should be closed when:

1. There is right ventricular overload.

OR

2. There is 1.5 times more pulmonary flow than systemic flow (i.e. the pulmonary flow [Qp] : systemic flow [Qs] ratio is >1.5).

If the patient meets these criteria, and has no symptoms, this is not a contraindication for repair.

Indications

Amount of Shunt

1) Size and amount of left-to-right shunting across the defect serve as a good indicator of the progression and worsening of the disease.

2) The pulmonary-to-systemic flow ratio Qp/Qs gives a good idea of the shunting.

3) Cardiac catheterization gives most accurate diagnosis of Qp/Qs.

4) Echocardiography and doppler ultrasound are more commonly used as they are convenient, non-invasive and cheap.

5) Qp/Qs is calculated as Qp/Qs = [PA diameter(2) x VTI-PA] ÷ [LVOT diameter(2) x VTI-LVOT] where

  • PA = Pulmonary artery
  • VTI-PA = Velocity time of the Doppler flow signal
  • LVOT = Left ventricular outflow tract
  • VIT-LVOT = Maximum doppler flow velocity apical to the aortic valve

6) In case the pulmonary arterial pressure is more than 2/3rd of the systemic systolic pressure, it could cause a net left-to-right shunt of at least 1.5:1 or evidence of reversibility of the shunt when given pulmonary artery vasodilators prior to surgery.

7) If Eisenmenger's syndrome has developed, it must be demonstrated that the right-to-left shunt is reversible with pulmonary artery vasodilators prior to surgery.

8) The American Heart Association has recommended a threshold Qp/Qs ≥1.5:1 for patients 21 years of age or older. [1]

9) The Canadian Cardiac Society recommended a threshold Qp/Qs >2:1, or >1.5:1 in the presence of reversible pulmonary hypertension. [2]

10) The Qp/Qs ratio can change as the disease progresses. Due to this it has been recommended that asymptomatic patients undergo echocardiography every 2-3 years.

Symptoms

1) Development and worsening of symptoms such as shortness of breath, exercise intolerance, fatigue, swelling of feet and ankle or abdomen (suggesting right sided heart failure), recurrent respiratory infections along with echocardiographic abnormalities are an indication for repair.

2) Arrhythmias as an isolated symptom can occur in 1 out of 5 adults patients with atrial septal defects. The surgical closure for patients presenting only with arrhythmia is controversial as not much benefit could be derived even after surgery.

Size of Defect

1) Secundum ASD <6 mm diameter in infants may close spontaneously by the end of two years of life. Thus, in asymptomatic patients with small defects early closure is not indicated.

2) Defects of moderate size (6 to 8 mm) are less likely to close spontaneously. Despite this surgical closure of these defects are not indicated before two years of age, in case these are asymptomatic.

Age

1) Closure of an ASD in individuals under age 25 has been shown to be associated with a low risk of complications, and individuals have a normal lifespan (comparable to a healthy age-matched population).

2) Closure of an ASD in individuals between the ages of 25 and 40 who are asymptomatic but have a clinically significant shunt is controversial. Those that perform the procedure believe that they are preventing long-term deterioration in cardiac function and preventing progression of pulmonary hypertension.

Clinical Trial Data

Surgical repair of an atrial septal defect in patients over 40 years of age, decreases all causes mortality, increases long-term survival and decreases complications like heart failure when compared with medical therapy. However, surgically treated patients may have an increased risk of arrhythmias and thromboembolic episodes and should be closely observed for these complications.[3]

Percutaneous versus Surgical Closure

The ACC/AHA guidelines recommend different interventional and surgical closure techiques in patients with atrial septal defect depending on the associated lesions, presence and absence of atrial and ventricular hypertrophy and amount of shunting across the lesions. Percutaneous closure is commonly performed for ostium secundum atrial septal defect. This procedure is still not FDA approved for the treatment of other types of atrial septal defects like sinus venosus ASD, coronary sinus ASD or primum ASD. With appropriate patient selection, percutaneous closure has been demonstrated to be as successful, safe and effective as surgical closure. Additionally, percutaneous closure has been associated with fewer complications and a reduced average length of hospital stay compared to surgical care. Surgical closure of ostium ostium secundum atrial septal defect can be done when a concomitant tricuspid valve repair is considered or when the anatomy of the defect doesn't favor a percutaneous device.

2008 ACC/AHA Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)[4]

Recommendations for Interventional and Surgical Therapy (DO NOT EDIT)[4]

Class I
"1. Closure of an ASD either percutaneously or surgically is indicated for right atrial and RV enlargement with or without symptoms. (Level of Evidence: B) "
"2. A sinus venosus, coronary sinus or primum ASD should be repaired surgically rather than by percutaneous closure. (Level of Evidence: B)"
"3. Surgeons with training and expertise in CHD should perform operations for various ASD closures. (Level of Evidence: C)"
Class III (Harm)
"1. Patients with severe irreversible PAH and no evidence of a left-to-right shunt should not undergo ASD closure. (Level of Evidence: B)"
Class IIa
"1. Surgical closure of secundum ASD is reasonable when concomitant surgical repair/replacement of a tricuspid valve is considered or when the anatomy of the defect precludes the use of a percutaneous device. (Level of Evidence: C) "
"2. Closure of an ASD, either percutaneously or surgically, is reasonable in the presence of:
"a. Paradoxical embolism. (Level of Evidence: C)"
"b. Documented orthodeoxia-platypnea. (Level of Evidence: B) "
Class IIb
"1. Closure of an ASD, either percutaneously or surgically, may be considered in the presence of net left-to-right shunting, pulmonary artery pressure less than two thirds systemic levels, PVR less than two thirds systemic vascular resistance, or when responsive to either pulmonary vasodilator therapy or test occlusion of the defect (patients should be treated in conjunction with providers who have expertise in the management of pulmonary hypertensive syndromes). (Level of Evidence: C)"
"2. Concomitant Maze procedure may be considered for intermittent or chronic atrial tachyarrhythmias in adults with ASDs. (Level of Evidence: C)"

References

  1. Driscoll D, Allen HD, Atkins DL, Brenner J, Dunnigan A, Franklin W; et al. (1994). "Guidelines for evaluation and management of common congenital cardiac problems in infants, children, and adolescents. A statement for healthcare professionals from the Committee on Congenital Cardiac Defects of the Council on Cardiovascular Disease in the Young, American Heart Association". Circulation. 90 (4): 2180–8. PMID 7923709.
  2. Therrien J, Dore A, Gersony W, Iserin L, Liberthson R, Meijboom F; et al. (2001). "CCS Consensus Conference 2001 update: recommendations for the management of adults with congenital heart disease. Part I.". Can J Cardiol. 17 (9): 940–59. PMID 11586386.
  3. Konstantinides S, Geibel A, Olschewski M, Görnandt L, Roskamm H, Spillner G; et al. (1995). "A comparison of surgical and medical therapy for atrial septal defect in adults". N Engl J Med. 333 (8): 469–73. doi:10.1056/NEJM199508243330801. PMID 7623878.
  4. 4.0 4.1 Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA; et al. (2008). "ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons". J Am Coll Cardiol. 52 (23): e1–121. doi:10.1016/j.jacc.2008.10.001. PMID 19038677.


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