View source for Atrial septal defect indications for surgical repair in adults

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{{Atrial septal defect}}
{{CMG}}; '''Associate Editor(s)-In-Chief:''' [[Priyamvada Singh|Priyamvada Singh, M.B.B.S.]] [mailto:psingh13579@gmail.com]; {{CZ}} '''Assistant Editor(s)-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]] [mailto:kfeeney@elon.edu]

==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 shunt]]ing, 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 shunt]]ing 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. <ref name="pmid7923709">{{cite journal|author=Driscoll D, Allen HD, Atkins DL, Brenner J, Dunnigan A, Franklin W et al.| title=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. | journal=Circulation | year= 1994 | volume= 90 | issue= 4 | pages= 2180-8 | pmid=7923709 | doi=| pmc= | url= }} </ref>

9) The Canadian Cardiac Society recommended a threshold  Qp/Qs >2:1, or >1.5:1in the presence of reversible [[pulmonary hypertension]] <ref name="pmid11586386">{{cite journal| author=Therrien J, Dore A, Gersony W, Iserin L, Liberthson R, Meijboom F et al.| title=CCS Consensus Conference 2001 update: recommendations for the management of adults with congenital heart disease. Part I. | journal=Can J Cardiol | year= 2001 | volume= 17 | issue= 9 | pages= 940-59| pmid=11586386 | doi= | pmc= | url= }} </ref>. 

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]], [[pedal edema|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) [[Arrhythmia]]s as an isolated symptom can occur in 1 out of 5 adults patients with [[atrial septal defect]]s.  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 [[Atrial septal defect surgical closure | 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 [[arrhythmia]]s and thromboembolic episodes and should be closely observed for these complications.<ref name="pmid7623878">{{cite journal| author=Konstantinides S, Geibel A, Olschewski M, Görnandt L, Roskamm H, Spillner G et al.| title=A comparison of surgical and medical therapy for atrial septal defect in adults. | journal=N Engl J Med | year= 1995 | volume= 333 | issue= 8 | pages= 469-73 | pmid=7623878 | doi=10.1056/NEJM199508243330801 | pmc= | url= }} </ref>

===Percutaneous versus Surgical Closure===
The ACC/AHA guidelines recommend different interventional and [[Atrial septal defect surgical closure | 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.  [[Atrial septal defect percutaneous closure | 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 [[Atrial septal defect sinus venosus|sinus venosus ASD]], [[Atrial septal defect coronary sinus|coronary sinus ASD]], or [[atrial septal defect ostium primum|primum ASD]]. With appropriate patient selection, percutaneous closure has been demonstrated to be as successful, safe and effective as surgical closure. Additionally, [[Atrial septal defect percutaneous closure | percutaneous closure]] has been associated with fewer complications and a reduced average length of hospital stay compared to surgical care. Surgical closure of 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.

== 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines<ref name="pmid30121240">{{cite journal| author=Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM | display-authors=etal| title=2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. | journal=J Am Coll Cardiol | year= 2019 | volume= 73 | issue= 12 | pages= 1494-1563 | pmid=30121240 | doi=10.1016/j.jacc.2018.08.1028 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30121240  }}</ref> ==

=== Recommendations for Atrial Septal Defect ===
{| class="wikitable"
|-
| colspan="1" style="text-align:center; background:LightGreen" |[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]
|-
| bgcolor="LightGreen" |'''1.''' "In adults with isolated secundum ASD causing impaired functional capacity, right atrial and/or RV enlargement, and net left- to-right shunt sufficiently large to cause physiological sequelae (eg, pulmonary– systemic blood flow ratio [Qp:Qs] ≥1.5:1) without cyanosis at rest or during exercise, transcatheter or surgical closure to reduce RV volume and improve exercise tolerance is recommended, provided that systolic PA pressure is less than 50% of systolic systemic pressure and pulmonary vascular resistance is less than one third of the systemic vascular resistance. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]]-NR)"''
|-
| bgcolor="LightGreen" |'''2.''' "Adults with primum ASD, sinus venosus defect or coronary sinus defect causing impaired functional capacity, right atrial and/ or RV enlargement and net left-to-right
shunt sufficiently large to cause physiological sequelae (eg, Qp:Qs ≥1.5:1) without cyanosis at rest or during exercise, should be surgically repaired unless precluded by comorbidities, provided that systolic PA pressure is less than 50% of systemic pressure and pulmonary vascular resistance is less than one third of the systemic vascular resistance. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]]-NR)"''
|}

{| class="wikitable"
|-
| colspan="1" style="text-align:center; background:LemonChiffon" |[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]
|-
| bgcolor="LemonChiffon" |'''1."'''In asymptomatic adults with isolated secundum ASD, right atrial and RV enlargement, and net left-to-right shunt sufficiently large to cause physiological sequelae (eg, Qp:Qs 1.5:1 or greater), without cyanosis at rest or during exercise, transcatheter or surgical closure is reasonable to reduce RV volume and/or improve functional capacity, provided that systolic PA pressure is less than 50% of systemic pressure and pulmonary vascular resistance is less than one third systemic resistance. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]]-LD)''<nowiki>"</nowiki>
|-
| bgcolor="LemonChiffon" |'''2.''' "Surgical closure of a secundum ASD in adults is reasonable when a concomitant surgical procedure is being performed and there is a net left-to-right shunt sufficiently large to cause physiological sequelae (eg, Qp:Qs 1.5:1 or greater) and right atrial and RV enlargement without cyanosis at rest or during exercise.''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]]-LD)<nowiki>"</nowiki>''
|-
| bgcolor="LemonChiffon" |'''3.''' "Percutaneous or surgical closure may be considered for adults with ASD when net left-to-right shunt (Qp:Qs) is 1.5:1 or greater, PA systolic pressure is 50% or more of systemic arterial systolic pressure, and/or pulmonary vascular resistance is greater than one third of the systemic resistance ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B-NR]])''<nowiki>"</nowiki>
|}

==2008 ACC/AHA Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)<ref name="pmid19038677">{{cite journal| author=Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA et al.| title=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. | journal=J Am Coll Cardiol | year= 2008 | volume= 52 | issue= 23 | pages= e1-121 | pmid=19038677 | doi=10.1016/j.jacc.2008.10.001 | pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19038677  }} </ref> ==
===Recommendations for Interventional and Surgical Therapy (DO NOT EDIT)<ref name="pmid19038677">{{cite journal| author=Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA et al.| title=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. | journal=J Am Coll Cardiol | year= 2008 | volume= 52 |issue= 23 | pages= e1-121 | pmid=19038677 | doi=10.1016/j.jacc.2008.10.001 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19038677  }} </ref>===

{|class="wikitable"
|-
| colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''1.''' Closure of an ASD either percutaneously or surgically is indicated for [[right atrial enlargement|right atrial]] and RV enlargement with or without symptoms. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])'' <nowiki>"</nowiki>
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''2.''' A [[sinus venosus]], [[coronary sinus]] or [[primum ASD]] should be repaired surgically rather than by percutaneous closure. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
|-
| bgcolor="LightGreen"|<nowiki>"</nowiki>'''3.''' Surgeons with training and expertise in [[CHD]] should perform operations for various ASD closures.  ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|}

{|class="wikitable"
|-
|colspan="1" style="text-align:center; background:LightCoral"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class III]] (Harm)
|-
|bgcolor="LightCoral"|<nowiki>"</nowiki>'''1.''' Patients with severe irreversible [[PAH]] and no evidence of a [[left-to-right shunt]] should not undergo ASD closure. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
|}

{|class="wikitable"
|-
| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]
|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''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. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])'' <nowiki>"</nowiki>
|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''2.''' Closure of an ASD, either percutaneously or surgically, is reasonable in the presence of:'' <nowiki>"</nowiki>
|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''a.''' [[Paradoxical embolism]]. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''b.''' Documented orthodeoxia-[[platypnea]]. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: B]])'' <nowiki>"</nowiki>
|}

{|class="wikitable"
|-
| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIb]]
|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' Closure of an ASD, either percutaneously or surgically, may be considered in the presence of net [[left-to-right shunt]]ing, 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). ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|-
|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''2.''' Concomitant Maze procedure may be considered for intermittent or chronic atrial [[tachyarrhythmias]] in adults with ASDs. ''([[ACC AHA guidelines classification scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
|}

==References==
{{reflist|2}}

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