Atrial septal defects

Highlights
Summary Overview

Basics
Definition Epidemiology Aetiology Pathophysiology Classification

Diagnosis
History & examination Tests Differential Step-by-step Guidelines Case history

Treatment
Details Step-by-step Guidelines

Follow Up
Recommendations Complications Prognosis

Resources
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History & exam

Key factors
systolic ejection murmur fixed splitting of the second heart sound

Other diagnostic factors

presence of risk factors mid-diastolic murmur congestive cardiac failure failure to thrive

symptoms of atrial arrhythmias cyanosis finger clubbing

History & exam details

Diagnostic tests
1st tests to order
echocardiogram ECG chest x-ray

Tests to consider
chest CT/MRI cardiac catheterisation Diagnostic tests details

Treatment details
Acute
left-to-right shunt observation corrective closure prophylactic antibiotics right-to-left shunt o o o o o o reversible corrective closure prophylactic antibiotics irreversible (Eisenmenger's syndrome) supportive medical therapy with pulmonary vasodilators monitoring and treatment of hyperviscosity prophylactic antibiotics heart-lung transplantation

Treatment details

Summary
There are 4 types of ASD: ostium secundum, ostium primum, sinus venosus, and unroofed coronary sinus. Secundum defects are the most common.

Most patients are asymptomatic. Untreated defects can produce right atrial enlargement, cardiac arrhythmias, and heart failure over time.

Secundum, primum, and coronary sinus defects with small shunts (ratio of pulmonary flow to systemic flow, Qp:Qs, <1.5) do not require treatment. Corrective closure is required if the shunt is larger (Qp:Qs ratio 1.5), there is right atrial enlargement, or the patient has a sinus venosus defect.

Corrective closure, if required, is usually performed at 2 to 4 years of age but can be performed at a younger age in symptomatic patients.

Percutaneous device closure is the preferred treatment for secundum defects, and surgical closure is reserved for larger secundum defects, technically challenging cases, and other defects.

If right-to-left shunting (Eisenmenger's syndrome) occurs, the ASD is operable if the shunt is reversible with pulmonary vasodilators. If the shunt is irreversible, the treatment is largely supportive.

Definition
An atrial septal defect (ASD) is an opening in the atrial septum, excluding a patent foramen ovale. [1] There are 4 types of ASD: ostium secundum, ostium primum, sinus venosus, and unroofed coronary sinus.

Epidemiology
ASDs occur in 1 of 1500 live births. Secundum-type ASDs constitute 6% to 10% of congenital heart defects [1] [8] and have a 2:1 female-to-male predominance. [9] Secundum defects account for 75% of ASDs. The other types of ASDs occur far less frequently: ostium primum (15% of ASDs), sinus venosus (5% of ASDs), and unroofed coronary sinus defects (1% of ASDs). ASDs can also occur in conjunction with more significant congenital heart defects.

Aetiology
Most cases of ASDs occur sporadically with no family history of congenital heart defects. Girls are more likely than boys to develop ASDs. Maternal alcohol consumption increases the risk of developing a range of congenital heart conditions, including ASDs. Some causative mutations have been identified. An association between ASDs and upper extremity anomalies was first reported in 1960. [10] This particular association has been linked to mutations of TBX5. [11]Rare cases of familial ASDs have been identified, caused by mutations in the transcription factors NKX2.5 and GATA4, as well as the structural protein MYH6. [12] [13]

Pathophysiology
Inter-atrial blood flow is maintained throughout cardiac embryogenesis while two different septal structures form. The first septum to develop is septum primum. Programmed cell death in the anterosuperior aspect of septum primum results in an ostium secundum. Septum secundum then develops to the right of septum primum as an infolding of the right atrial wall and ultimately forms the limbus of the fossa ovalis, while septum primum functions as the valve of the fossa ovalis. [2] An abnormality during the development of these structures can result in an ASD. Ostium secundum-type ASDs develop due to a lack of development of septum secundum. Ostium primum-type ASDs develop due to lack of closure of ostium primum by the endocardial cushions. Sinus venosus-type ASDs are thought to occur because of resorption of the wall between the superior vena cava and pulmonary veins, which also explains why this type of ASD is associated with anomalous drainage of the right upper pulmonary vein. [1] Occasionally, sinus venosus ASDs can develop between the inferior vena cava and the right atrium. The direction of blood flow through the defect is related to the relative compliances of the two ventricles. In infancy, the right ventricle is relatively thick and non-compliant; therefore, the amount of left-to-right shunt is minimal. However, as the pulmonary vascular resistance decreases, the right ventricle becomes more compliant, increasing the left-to-right shunt. With age, the left-to-right shunt is exacerbated by increasing stiffness of the left ventricle associated with ageing and systemic hypertension. The right atrium, right ventricle, and pulmonary arteries may enlarge due to the increased volume load. The pulmonary and tricuspid valves may become incompetent due to enlargement of the valves' annuli. Despite the increased volume load, the pulmonary artery pressures are usually only slightly increased. However, the chronic volume overload can cause engorgement of the pulmonary arteries, capillaries, and veins. Some patients develop medial hypertrophy of the pulmonary arteries and pulmonary vascular obstructive disease. Patients become cyanotic if the shunt reverses.

Classification
Classification according to location ASDs are classified according to their location relative to the fossa ovalis and the proposed embryogenesis. [2] [3] Although no true defect in the atrial

septum is present with sinus venosus and unroofed coronary sinus defects, they have historically been classed as ASDs due to the left-to-right shunt they produce.
Secundum ASDs are found in the region of the fossa ovalis. Ostium primum ASDs are found anterior to the fossa ovalis and superior to the atrioventricular valves. These defects are a type of atrioventricular septal defect and are usually associated with a so-called "cleft" in the anterior leaflet of the "mitral valve". Morphologically, this is better described as a zone of apposition of the left atrioventricular valve of the atrioventricular septal defect, and distinctly different from a true cleft in the anterior leaflet of the mitral valve. [4] [5] Sinus venosus defects usually occur superior and posterior to the fossa ovalis, outside its confines, and are associated with an anomalous connection of the right pulmonary veins, especially the right superior and middle pulmonary veins, with the caval vein (superior or inferior) overriding the defect. [6] Unroofed coronary sinus defects are found near the os of the coronary sinus and are associated with a

persistent left superior vena cava.

Subtypes of ASD. A: sinus venosus; B: ostium secundum; C: ostium primum; D: unroofed coronary sinusMayo Clinic Foundation

Classification by size ASDs can be small (3 to <6 mm), medium (6 to <12 mm), or large (>12 mm), although a single measurement can be flawed. The most clinically useful measure of size is the magnitude of the shunt produced by an ASD. This used to be described as the ratio of the pulmonary to systemic blood flow (Qp:Qs ratio). However, shunt quantification is seldom used; symptoms, clinical signs, and evidence of right heart enlargement are used to make clinical decisions.

History & examination

Key diagnostic factorshide all
systolic ejection murmur (common)

Best heard at the left upper sternal border. The murmur often radiates to the back. Produced by excess volume of blood crossing the pulmonary valve.

Shortens in patients with Eisenmenger's physiology. fixed splitting of the second heart sound (common)

Second heart sound does not become single with expiration.

Fixed splitting disappears in patients with Eisenmenger's physiology. Other diagnostic factorshide all presence of risk factors (uncommon)
Key risk factors include female sex and maternal alcohol consumption. mid-diastolic murmur (uncommon)

Best heard along the lower sternal border. Due to excess volume of blood crossing the tricuspid valve. Can only be heard if there is a moderate or greater degree of left-to-right shunt.

Disappears in patients with Eisenmenger's physiology. congestive cardiac failure (uncommon)

Rare presentation that usually does not occur until after the fifth decade of life. Symptoms include fatigue, decreased exercise tolerance, dyspnoea on exertion, orthopnoea, paroxysmal nocturnal dyspnoea, and oedema.

Rare in infants; the main presenting symptom is tachypnoea. failure to thrive (uncommon)

Occurs in <10% of infants with ASD.

CHD should always be excluded in infants and children with unexplained failure to thrive. symptoms of atrial arrhythmias (uncommon)

Include rapid palpitations, fluttering in the chest, dizziness, or shortness of breath. Can occur in adult patients with undiagnosed ASD. Incidence of atrial arrhythmias increases with age, especially between the fifth and seventh decades of life. [7]

cyanosis (uncommon) Occurs in cases of shunt reversal (Eisenmenger's syndrome). finger clubbing (uncommon)

Risk

Associated with a range of cyanotic CHDs, including Eisenmenger's ASDs. factorshide all

Strong female sex Ostium secundum ASDs have a female-to-male predominance of 2:1. maternal alcohol consumption

During the first trimester, doubles the risk of having a child with an ASD. [14]

Weak positive FHx

Most cases of ASDs are sporadic. However, rare cases of familial ASDs have been identified, caused by mutations in the transcription factors NKX2.5 and GATA4, and the structural protein MYH6. [12] [13]

Diagnostic tests
1st tests to orderhide all
Test

echocardiogram The preferred imaging modality. View imageView imageView image adults.

Transthoracic echocardiography is usually useful in demonstrating a defect in children. Image quality may be poo

Useful to identify all types of ASDs, to determine defect size and adequacy of the rim for device placement in sec defects, and to ensure normally connected pulmonary veins. Used to characterise shunt volume and to determine flow pattern through the defect.

Contrast media or "bubble study" can help to demonstrate a right-to-left shunt, especially with Valsalva manoeuv ECG Not a diagnostic test, but diagnostic clues may be present. Often normal if the shunt is small.

P waves >2.5 mm, suggesting right atrial enlargement, or R-wave voltages in lead V1 greater than the upper limi wave in the inferior limb leads, known as the crochetage pattern, is also found in patients with ASDs. Primum-type ASDs frequently produce a counterclockwise frontal-plane loop and left-axis deviation. Sinus venosus-type defects are associated with a p-wave axis <30.[16] chest x-ray Not diagnostic, but diagnostic clues may be present.

normal for age, suggesting right ventricular hypertrophy, may be present in larger defects. A notch near the apex

May be normal if left-to-right shunt is small. With larger shunt volumes, heart may be enlarged and pulmonary va markings may be increased. Cannot differentiate between ASD types.

Tests to considerhide all

Test

chest CT/MRI May be needed to help define pulmonary venous anatomy if echocardiography is insufficient. cardiac catheterisation pulmonary vascular obstructive disease.

Not required for diagnosis, but is used to detect pulmonary vascular resistance in the subset of patients at risk fo

If patients develop a right-to-left shunt, reversibility of the shunt with pulmonary vasodilators should be assessed right-to-left shunt is irreversible, surgery is not an option.

therapy. If the right-to-left shunt is reversible with pulmonary vasodilators, the ASD can be surgically closed. How

Differential diagnosis
Condition Partial anomalous pulmonary venous drainage Differentiating signs/symptoms Differentiating tests

Signs and symptoms similar to atrial septal defect

Atrial septum intact with dilated right heart structures: iden echocardiography in children, and MRI or CT in adults.

Ventricular septal defect

Small ventricular septal defects (VSDs) are associated with a holosystolic murmur and normal first and second heart sounds. With a larger shunt volume (ratio of pulmonary to systemic blood flow, Qp:Qs, >2), a middiastolic mitral valve flow murmur may be heard in addition

VSD identified by Doppler echocardiography.

to a holosystolic mid-frequency murmur. Patent ductus arteriosus

Associated with prematurity. Patent ductus arteriosus is also associated with bounding peripheral pulses due to wide pulse pressure. The left ventricular impulse may be prominent. The murmur associated with a patent ductus arteriosus is often described as a machinerylike continuous murmur. However, in some premature and newborn infants the murmur can be heard only in systole. A middiastolic mitral valve flow murmur may be heard.

PDA identified by Doppler echocardiography.

Pulmonary stenosis

A click follows

Pulmonary stenosis identified by Doppler echocardiograp

the first heart sound, which is best heard at the left upper sternal border.

The second heart sound may be normal, split, or single depending on the severity of the stenosis.

Right bundle-branch block

Can produce a widely split second heart sound similar to that heard in patients with an ASD.

ECG shows right bundle-branch block. Echocardiography shows no evidence of an ASD.

The characteristic ASD murmurs are absent.

Other congenital heart defects

ASDs occur in the context of other congenital heart conditions, including transposition of the great arteries, pulmonary atresia, and Ebstein's

Abnormal anatomy identified by Doppler echocardiograph

anomaly.

Step-by-step diagnostic approach

Clinical history and presentation can vary significantly depending on the age of the patient and the size of the defect. Patients are usually asymptomatic and are evaluated for an ASD because of a heart murmur, or an abnormal ECG or chest x-ray ordered for other clinical reasons.

History
Most patients with ASDs are asymptomatic. [15] If the degree of shunting is large, the child may experience dyspnoea, fatigue, failure to thrive, or recurrent lower respiratory infections. Symptoms of CHF and failure to thrive occur in less than 10% of infants. If the ASD occurs in combination with other defects associated with a left-toright shunt, such as a patent ductus arteriosus or a ventricular septal defect, the defect is often found much earlier due to a higher incidence of CHF. Adults are also typically asymptomatic, although they may complain of palpitations, fluttering in the chest, or dizziness due to atrial arrhythmias, fatigue, dyspnoea, or exercise intolerance. Pregnancy may precipitate atrial arrhythmias in women with ASDs.

Physical exam
The physical findings of an ASD are related to the size of the shunt. Patients may have a hyperdynamic right ventricular impulse by palpation, especially in older children and adults with a large left-to-right shunt. The first heart sound is usually normal, but the second heart sound is often split and does not become single with expiration (fixed split). Patients usually have a systolic ejection murmur best heard at the left upper sternal border and radiating to the back. This murmur is caused by the excess volume of blood crossing the pulmonary valve. With moderate or larger defects, an additional mid-diastolic murmur may be appreciated along the lower sternal border due to the volume of blood crossing the tricuspid valve. A murmur of left atrioventricular valve insufficiency is often heard in patients with an ostium primum ASD. When pulmonary hypertension has developed, the volume of the left-to-right shunt decreases and results in loss of fixed splitting of the second heart sound, increased intensity of the pulmonary component of the second heart

sound, shortening of the systolic murmur, and disappearance of the diastolic murmur. If the shunt reverses, the patient will appear cyanosed and may develop finger clubbing.

Initial tests
Echocardiography
Echocardiography is the preferred imaging modality. Secundum-type ASDs can often be diagnosed using transthoracic echocardiography, especially in children. Trans-oesophageal echocardiography is used to help assess the size of the defect, to determine the adequacy of tissue rims for device closure in secundum-type defects, to diagnose sinus venosus-type ASDs, and to ensure normally connected pulmonary veins. Transoesophageal echocardiography is often necessary in older patients if the transthoracic image quality is poor. Two-dimensional echocardiography demonstrates right atrial and ventricular enlargement, as well as the defect itself, especially for secundum-type defects. View image This is best seen in subcostal views due to the septum being orthogonal to the ultrasound beam. Secundum defects result in dropout of the mid portion of the atrial septum, and primum defects result in dropout of the inferior portion, in a four-chamber view from the apex. View imageSinus venosus defects are defined by a deficiency in the posterosuperior portion of the atrial septum with the superior caval vein overriding the defect. Coronary sinus defects have a communication at the orifice of the coronary sinus. Doppler examination is used to characterise the shunt volume and to determine the flow pattern through the defect. Non-invasive calculation of the ratio of pulmonary to systemic blood flow, Qp:Qs, can be done with Doppler echocardiography but is no longer used stringently to make clinical decisions. Contrast echocardiography may be used to help determine the presence of a right-to-left shunt.

Chest x-ray
Chest x-ray is not needed to diagnose ASD. It may be normal if there is only a small left-to-right shunt. With larger shunt volumes, the heart may be enlarged and pulmonary vascular markings may be increased. Chest x-ray cannot differentiate between the types of ASD.

ECG
An ECG is not needed to diagnose ASD. It is often normal in secundum, coronary sinus, and sinus venosus-type ASDs if the shunt is small. If the shunt is moderate to severe, there may be p waves taller than 2.5 mm, suggesting right atrial enlargement, or R-wave voltages in lead V1 greater than the upper limit of normal for age, suggesting right ventricular hypertrophy. A notch near the apex of the R wave in the inferior limb leads, known as the crochetage pattern, is also found in patients with ASDs. Some ECG findings are specific for particular defects. Primum-type ASDs frequently produce a counterclockwise frontal-plane loop and left-axis deviation indicating the morphological substrate of a common atrioventricular junction. Sinus venosus-type defects are associated with a p-wave axis less than 30. [16]

Subsequent tests
Chest CT/MRI
This may be needed to help define pulmonary venous anatomy if all pulmonary veins cannot be shown to enter the left atrium by transthoracic or trans-oesophageal echocardiography.

Cardiac catheterisation
Cardiac catheterisation is not required for diagnosis. However, it is important in the evaluation of pulmonary vascular resistance in patients with suspected pulmonary vascular obstructive disease. If patients develop a right-to-left shunt, reversibility of the shunt with pulmonary vasodilators should be assessed to guide therapy. If the right-to-left shunt is reversible with pulmonary vasodilators, the ASD can be surgically closed. However, if the right-to-left shunt is irreversible, surgery is not an option.

Click to view diagnostic guideline references.

Case history #1

A 6-year-old girl is brought to her paediatrician for routine well-child care. She is doing well and has never experienced chest pain, palpitations, or syncope. She actively participates in a dance class and reports being able to keep up with her peers. There is no family history of congenital heart defects. She is well-appearing with no apparent distress. Her left ventricular impulse is normal, and there is a subtle right ventricular lift. Her second heart sound is widely split and does not vary with respiration. She has a soft 1-2/6 systolic ejection murmur best heard along the left upper sternal border. The remainder of her physical examination is normal.

Case history #2
A 45-year-old woman seeks treatment for frequent palpitations. The patient did well throughout the first 2 decades of life. In her mid-20s she noticed that she became slightly short of breath with exertion. She has recently been seen twice in the hospital emergency department for atrial tachyarrhythmias. Her left ventricular impulse is normal. Her right ventricular impulse is 2+. She has a 2/6 systolic ejection murmur at her left upper sternal border. The murmur radiates to her back. The remainder of her physical examination is normal.

Other presentations
The clinical presentation depends on the age of the patient and the size of the left-to-right shunt. Children can be completely asymptomatic. Less than 10% of patients with ASD have CHF and poor growth in infancy, particularly

those born prematurely. This presentation is more common if the patient has an additional source of pulmonary blood flow, such as a patent ductus arteriosus, or in association with partial anomalous pulmonary venous drainage. Adult patients with an untreated ASD can commonly present with CHF, atrial arrhythmias, right ventricular failure, paradoxical embolisation, cerebral abscess, or pulmonary vascular obstructive disease. Adults typically do not develop CHF until their fifth decade of life. Patients with sinus venosus-type defects are more prone to developing CHF. The incidence of atrial arrhythmias increases with age, especially between the fifth and seventh decades of life. [7] Pulmonary vascular obstructive disease develops in 5% to 10% of patients and is more common in women. Some patients develop irreversible right-to-left shunting (Eisenmenger's physiology). Most women with ASD usually tolerate pregnancy well, including those with a sizable left-to-right shunt. However, patients who have developed pulmonary vascular obstructive disease do not tolerate pregnancy well. Idiopathic pulmonary arterial hypertension may co-exist with atrial septal defect. Pulmonary vein stenosis should be ruled out in the setting of associated pulmonary hypertension.

Treatment Options
Treatment Patient group left-to-right shunt line 1st Treatmenthide all

observation

All patients with an ASD are observed, as the defect may close or shrink. If the ratio of pulmonary to systemic blood flow, Qp:Qs, is <1.5, the defect is of little prognostic importance and does not require closure.

adjunct [?]

corrective closure

If the ratio of pulmonary to systemic blood flow, Qp:Qs, is or remains 1.5, or there is evidence of right atrial enlargement, the defect requires closure to prevent heart failure, atrial arrhythmias, and pulmonary vascular obstructive disease. [20] This is usually performed at age 2 to 4 years. [1] Patients who develop unremitting heart failure require earlier closure.

Ostium secundum ASD: device closure is the

Treatment Patient group left-to-right shunt line 1st Treatmenthide all

observation

All patients with an ASD are observed, as the defect may close or shrink. If the ratio of pulmonary to systemic blood flow, Qp:Qs, is <1.5, the defect is of little prognostic importance and does not require closure. preferred method if rims are adequate to secure a device. If the rims are inadequate (due to a deficiency in the posterior/inferior rim) or there are large septal aneurysms or multiple fenestrations of the atrial septum, surgical closure is required.

Ostium primum ASD: surgical closure with repair of the left atrioventricular valve is the most commonly used procedure.

Coronary sinus ASD: surgical closure of the os of the coronary sinus is the preferred procedure. Sinus venosus ASD: anomalous right pulmonary veins are baffled via the defect to the left atrium. A Warden operation may also be performed, whereby the superior vena cava is transected and then reconnected to the right atrium while a patch is placed in the right atrium to route anomalous right pulmonary vein flow to the left atrium.

adjunct [?]

prophylactic antibiotics

Required for the first 6 months after device or surgical closure, to prevent endocarditis. The antibiotics are given before a procedure that may cause bacteraemia. Guidelines on which procedures require prophylaxis vary between countries, and local guidelines on endocarditis prophylaxis should be adhered to. For example, many guidelines no longer recommend prophylaxis for dental procedures, or for procedures involving the upper and lower GI tract, genitourinary tract, or

Treatment Patient group left-to-right shunt line 1st Treatmenthide all

observation

All patients with an ASD are observed, as the defect may close or shrink. If the ratio of pulmonary to systemic blood flow, Qp:Qs, is <1.5, the defect is of little prognostic importance and does not require closure. the upper and lower respiratory tract.

Clindamycin is an alternative to penicillin in patients with a penicillin allergy. Primary Options amoxicillin : children: 50 mg/kg orally one hour before procedure; adults: 2 g orally one hour before procedure OR clindamycin : children: 20 mg/kg orally one hour before procedure; adults: 600 mg orally one hour before procedure

right-to-left shunt reversible 1st

corrective closure

The ASD is still operable if the right-to-left shunt is reversible with pulmonary vasodilators. Ostium secundum ASD: device closure is the preferred method if rims are adequate to secure a device. If the rims are inadequate (due to a deficiency in the posterior/inferior rim) or there are large septal aneurysms or multiple fenestrations of the atrial septum, surgical closure is required.

Ostium primum ASD: surgical closure with repair of the left atrioventricular valve is the most commonly used procedure.

Coronary sinus ASD: surgical closure of the os of the coronary sinus is the preferred procedure.

Treatment Patient group left-to-right shunt line 1st Treatmenthide all

observation

All patients with an ASD are observed, as the defect may close or shrink. If the ratio of pulmonary to systemic blood flow, Qp:Qs, is <1.5, the defect is of little prognostic importance and does not require closure.

Sinus venosus ASD: anomalous right pulmonary veins are baffled via the defect to the left atrium. A Warden operation may also be performed, whereby the superior vena cava is transected and then reconnected to the right atrium while a patch is placed in the right atrium to route anomalous right pulmonary vein flow to the left atrium.

plus [?]

prophylactic antibiotics

Required for the first 6 months after surgical closure, to prevent endocarditis. The antibiotics are given before a procedure that may cause bacteraemia. Guidelines on which procedures require prophylaxis vary between countries, and local guidelines on endocarditis prophylaxis should be adhered to. For example, many guidelines no longer recommend prophylaxis for dental procedures, or for procedures involving the upper and lower GI tract, genitourinary tract, or the upper and lower respiratory tract.

Clindamycin is an alternative to penicillin in patients with penicillin allergy. Primary Options amoxicillin : children: 50 mg/kg orally one hour before procedure; adults: 2 g orally one hour before procedure OR clindamycin : children: 20 mg/kg orally one hour

Treatment Patient group left-to-right shunt line 1st Treatmenthide all

observation

All patients with an ASD are observed, as the defect may close or shrink. If the ratio of pulmonary to systemic blood flow, Qp:Qs, is <1.5, the defect is of little prognostic importance and does not require closure.

before procedure; adults: 600 mg orally one hour before procedure

irreversible (Eisenmenger's syndrome) 1st

supportive medical therapy with pulmonary vasodilators

The ASD is inoperable if the right-to-left shunt is irreversible with pulmonary vasodilators. Supportive medical therapy with pulmonary vasodilators is the mainstay of treatment. Pulmonary vasodilators that have been shown to be of benefit include the endothelin antagonist bosentan, the phosphodiesterase-5 inhibitor sildenafil, and, in limited studies, infusions of the prostanoid epoprostenol. Primary Options bosentan : adults: 62.5 mg orally twice daily for 3-4 weeks, increase to 125 mg twice daily according to response OR sildenafil : adults: consult specialist for guidance on dose Secondary Options epoprostenol : adults: consult specialist for guidance on dose

plus [?]

monitoring and treatment of hyperviscosity

Patients with Eisenmenger's syndrome frequently develop erythrocytosis to compensate for the hypoxaemia, which can lead to hyperviscosity.

Treatment Patient group left-to-right shunt line 1st Treatmenthide all

observation

All patients with an ASD are observed, as the defect may close or shrink. If the ratio of pulmonary to systemic blood flow, Qp:Qs, is <1.5, the defect is of little prognostic importance and does not require closure.

Hyperviscosity can produce symptoms of headache, fatigue, and sometimes mental status changes. Treatment of symptomatic patients involves phlebotomy and intravenous infusion of saline.

Routine phlebotomy is not indicated in asymptomatic patients.

plus [?]

prophylactic antibiotics

All patients with unrepaired cyanotic heart disease require prophylaxis against endocarditis. The antibiotics are given before a procedure that may cause bacteraemia. Guidelines on which procedures require prophylaxis vary between countries, and local guidelines on endocarditis prophylaxis should be adhered to. For example, many guidelines no longer recommend prophylaxis for dental procedures, or for procedures involving the upper and lower GI tract, genitourinary tract, or the upper and lower respiratory tract.

Clindamycin is an alternative to penicillin in patients with penicillin allergy. Primary Options amoxicillin : children: 50 mg/kg orally one hour before procedure; adults: 2 g orally one hour before procedure OR clindamycin : children: 20 mg/kg orally one hour

Treatment Patient group left-to-right shunt line 1st Treatmenthide all

observation

All patients with an ASD are observed, as the defect may close or shrink. If the ratio of pulmonary to systemic blood flow, Qp:Qs, is <1.5, the defect is of little prognostic importance and does not require closure.

before procedure; adults: 600 mg orally one hour before procedure

2nd

heart-lung transplantation

May be considered for some patients who are severely symptomatic. When feasible, surgical correction of the ASD together with lung transplantation may be considered.

Acute

Treatment approach
Small ASDs close spontaneously. If closure of the ASD is required, device closure in the catheterisation laboratory or surgical closure can be used depending on the type of ASD. [17]Surgical repair is usually performed through a median sternotomy, but a procedure using a limited sternal split and skin incision has been reported. [18] ASDs should be treated by physicians experienced in the management of CHD. [19]

Left-to-right shunt
Patients with all types of ASD are initially observed, as the defect may close or shrink spontaneously. If the ratio of pulmonary vascular to systemic blood flow, Qp:Qs, is <1.5, the defect is of little prognostic importance and does not require closure. If the Qp:Qs ratio is or remains 1.5, or there is evidence of right atrial enlargement, the defect requires closure to prevent heart failure, atrial arrhythmias, and pulmonary vascular obstructive disease. [20] This is usually performed at age 2 to 4 years. [1] Patients who develop unremitting heart failure require earlier repair.

Right-to-left shunt
The ASD is still operable if the shunt is reversible with pulmonary vasodilators. However, if the shunt is irreversible, the ASD is inoperable and the patient has Eisenmenger's syndrome. Patients with Eisenmenger's syndrome should avoid pregnancy, dehydration, and high altitudes. Endocardial pacing is contra-indicated. Care must be taken with intravenous lines to avoid an air embolus. All patients with unrepaired cyanotic heart disease require prophylaxis against endocarditis. Patients with Eisenmenger's syndrome frequently develop erythrocytosis to compensate for the hypoxaemia, which can lead to hyperviscosity. Hyperviscosity can produce symptoms of headache, fatigue, and sometimes mental status changes. Treatment of symptomatic hyperviscosity involves phlebotomy and intravenous infusion of saline. Routine phlebotomy is not indicated in asymptomatic patients. Pulmonary vasodilator therapy may be used to improve quality of life. Those demonstrated to be of benefit include the endothelin antagonist bosentan, the phosphodiesterase-5 inhibitor sildenafil, and, in limited studies, infusions of the prostanoid epoprostenol. Heart and lung transplantation, or lung transplantation with repair of the cardiac defect, may be beneficial in selected patients with severe disease.

Options for corrective closure

Treatment involves either an operation using direct sutures to close the defect or a patch of pericardium to close larger defects, or percutaneous device closure. Device closure is used to treat secundum defects but cannot be used in other types of ASD due to the closeness of the defects to other cardiac structures. Prophylactic antibiotics to prevent endocarditis are required for the first 6 months after device or surgical closure.
Ostium secundum ASD: device closure is the preferred method if rims are adequate to secure a device. View image If the rims are inadequate (due to a deficiency in the posterior/inferior rim) or there are large septal aneurysms or multiple fenestrations of the atrial septum, surgical closure is required. Ostium primum ASD: surgical closure with repair of the left atrioventricular valve is the most commonly used procedure. Coronary sinus ASD: surgical closure of the os of the coronary sinus is the preferred procedure. Sinus venosus ASD: anomalous right pulmonary veins are baffled via the defect to the left atrium. A Warden operation may also be performed, whereby the superior vena cava is transected and then reconnected

to the right atrium while a patch is placed in the right atrium to route anomalous right pulmonary vein flow to the left atrium.

Monitoring
Routine follow-up for patients with small ASDs that do not require surgical closure should include assessment of symptoms, especially arrhythmias and paradoxical embolic events in adults. Pulmonary pressures, right ventricular function, and residual atrial shunting should be assessed by echocardiography. Patients who have had device closure of a secundum ASD should be assessed for symptoms of atrial arrhythmias, chest pain, or embolic events. [24]Echocardiography is usually performed at 24 hours, 1 month, 3 months, and 1 year. Further monitoring is elective. Attention should be paid to device placement, residual shunting, thrombus formation, and pericardial effusion. [15] Post-operative fever, chest or abdominal pain, vomiting, and fatigue may indicate pericardial effusions or cardiac tamponade and should be investigated immediately. Chest pain or syncope after device closure may be due to device erosion and should be investigated immediately.

Patient Instructions
Patients require endocarditis prophylaxis for the first 6 months after device or surgical closure of an ASD, and indefinitely if they have unrepaired cyanotic heart disease. Patients should report symptoms of fever, chest or abdominal pain, vomiting, syncope, or fatigue immediately. Restrictions on physical activities are not required.

Complications
Complicationhide all

device erosion Presents with chest pain or syncope after device closure. pericardial effusion/tamponade Presents after repair of an ASD with postoperative fever, chest or abdominal pain, vomiting, and fatigue. paradoxical embolisation Caused by embolism of a venous thrombus into the systemic arterial circulation through the ASD. Requires the presence of right-to-left shunt through the ASD. A right-to-left shunt can occur intermittently in patients with coughing or a Valsalva manoeuvre, or may be present at rest in patients with Eisenmenger's physiology. This complication is usually seen in adults with an undiagnosed ASD. It may present as a stroke or transient ischaemic attack. Additionally, there can be peripheral emboli. congestive cardiac failure see our comprehensive coverage of Chronic congestive heart failure Occurs in <10% of infants with ASD. In infants, the main presenting symptom is tachypnoea. Usually does not occur until after the fifth decade of life. In adults, symptoms include fatigue, decreased

exercise tolerance, dyspnoea on exertion, orthopnoea, paroxysmal nocturnal dyspnoea, and oedema. infective endocarditis see our comprehensive coverage of Infective endocarditis Can occur as a complication of device closure or surgery. Antibiotic prophylaxis is recommended for the first 6 months after repair of an ASD, and indefinitely in patients with unrepaired cyanotic heart disease. Often presents non-specifically, and most commonly involves fever with possible physical signs of peripheral emboli: Osler's nodes, Roth's spots, or Janeway's lesions. A cardiac murmur may sometimes be noted. atrial fibrillation see our comprehensive coverage of Chronic atrial fibrillation Includes rapid palpitations, fluttering in the chest, dizziness, or shortness of breath. Occurs in large defects as a result of atrial enlargement and hypertrophy. Treatment of ASD may help in controlling atrial arrhythmias; however, incidence of recurrent arrhythmias is higher with delayed closure of ASD. Pregnancy can precipitate atrial fibrillation due to the increased volume load. The incidence of atrial arrhythmias increases with age, especially between the fifth and seventh decades of life. [7] Atrial arrhythmias should be treated to restore and maintain sinus rhythm if possible. [23] Cardioversion after appropriate anticoagulation is recommended to attempt restoration of sinus rhythm if atrial fibrillation occurs. Rate control and anticoagulation are recommended if sinus rhythm cannot be maintained by medical or interventional means. [15]

Prognosis
The prognosis of ASDs without pulmonary vascular obstructive disease is excellent. The risk of death after surgical closure of an uncomplicated ASD is <1%. Long-term survival for patients aged <24 years at surgery is similar to that of age-matched controls. Patients who have surgery at age >24 years have poorer survival than age-matched controls. [21] Five- to 10-year follow-up after device closure is excellent, but long-term follow-up data are not available for device closure. The incidence of device erosion is 0.1% in the US, with most erosions occurring within 72 hours after placement. [22] However, erosion occurred in at least one case more than 5 years after device placement. Shunt reversal with inoperable Eisenmenger's physiology is associated with a poor prognosis.