1. How do you manage this patient?

2. Diagnostic An adequate diagnostic workup: Documents the presence and type of ASD(s) Determines the size (diameter) of the defect(s) Determines the functional importance of the defect either by: – shunt size (Qp/Qs) – right ventricular size, function and volume overload and right atrial size – pulmonary artery pressures and if elevated, pulmonary vascular resistance Identifies other associated conditions that may influence management (e.g. anomalous pulmonary venous connection, significant valve disease; or coronary artery disease)

3. The initial workup should include at a minimum: A thorough clinical assessment ECG Chest x-ray Transthoracic echo-Doppler evaluation by an appropriately trained individual Transesophageal (TEE) echo/Doppler examination to prove the existence of an ASD, better define its/their location(s) and size(s) and shape(s), assess pulmonary venous connections, and to evaluate the cardiac valves, if this information is not provided by transthoracic echocardiography (TTE) – A transesophageal examination is essential to determine if the ASD is suitable for device closure and must be performed prior to the procedure Resting oxygen saturation

4. The diagnostic workup may require: Heart catheterization (if determination of pulmonary artery pressures and resistances is of concern; to assess pulmonary vascular reactivity; or delineate anomalous pulmonary venous connections) Coronary angiography in patients at high risk of coronary artery disease or in patients over the age of 40 years if surgical repair is planned Magnetic resonance imaging (MRI) to prove the existence of an ASD or to assess pulmonary venous connections if doubts remain after other imaging modalities. MRI can also be used to estimate Qp/Qs Oxygen saturation with exercise if there is any suggestion of pulmonary hypertension. If there is severe pulmonary hypertension or resting desaturation of < 85%, the patient should not be exercised Open lung biopsy should only be considered when the reversibility of the pulmonary hypertension is uncertain from the hemodynamic data. It is potentially hazardous and should be done only at centres with substantial relevant experience in CHD

5. Medical management should include treatment of possible complications: – Respiratory tract infections – Arrhythmias, atrial fibrillation, supraventricular tachycardia – Pulmonary hypertension, coronary artery disease, heart failure – Infective endocarditis Harrison’s Principles of Internal Medicine 17th ed.

6. Pulmonary Hypertension Because the pulmonary artery pressure in PAH increases dramatically with exercise, patients should be cautioned against participating in activities that demand increased physical stress O2 supplementation helps to alleviate dyspnea and RV ischemia in patients whose arterial O2 saturation is reduced Anticoagulant therapy (Warfarin) Phosphodiesterase 5 inhibitors (Sildenafil) Prostacyclins (Treprostinil) Harrison’s Principles of Internal Medicine 17th ed.

7. Infective Endocarditis Prevention of Infective Endocarditis: Guidelines From the American Heart Association

8. Surgical management Operative repair – definitive management with a patch of pericardium OR prosthetic material OR percutaneous transcatheter device closure should be advised for all patients with uncomplicated secundum atrial septal defects with significant left-to-right shunting Harrison’s Principles of Internal Medicine 17th ed.

9. Indications The mere presence of an ASD may warrant intervention especially if there is a significant shunt (> 2:1) symptomatic pulmonary hypertension is present [pulmonary artery pressure (PAP) > 2/3 systemic arterial blood pressure (SABP) or pulmonary arteriolar resistance > 2/3 systemic arteriolar resistance net left-to-right shunt (Qp:Qs) of at least 1.5:1 RA or RV enlargement – radiographic, cardiac catheterization or there is evidence of pulmonary artery reactivity when challenged with a pulmonary vasodilator (e.g. oxygen, nitric oxide and/or prostaglandins) or lung biopsy evidence shows that pulmonary arterial changes are potentially reversible Schwartz ‘s Principles of Surgery, 9 th ed. http://www.achd-library.com/index.html

10. Device closure may now be offered as an alternative to surgical closure to patients with secundum ASD of up to 36-38 mm in diameter Surgical closure may also be offered, and may be especially attractive should the patient prefer the surgical approach, or especially if atrial arrhythmia surgery (atrial maze procedure for atrial fibrillation and radiofrequency or cryoablation for atrial flutter) may be offered concurrently http://www.achd-library.com/index.html

11. Device closure Early and intermediate follow-up is excellent after device closure The intermediate results are comparable to surgery with a high rate of shunt closure and few major complications Longer follow-up is needed to determine the incidence of arrhythmias and thromboembolic complications late after device closur Functional capacity improves and supraventricular arrhythmias are better tolerated and more responsive to pharmacologic management Surgical closure Following surgical repair, pre-operative symptoms, if any, should decrease or abate Pre-existing atrial flutter and fibrillation may persist. Likewise, atrial flutter and/or fibrillation may arise after repair, but are better tolerated and often more responsive to antiarrhythmic therapy Post-operative ASD patients are especially prone to cardiac tamponade for the first several weeks after surgery

12. Complications with transcatheter closure include: – air embolism (1 to 3%) – thromboembolism from the device (1 to 2%) – disturbed AV valve function (1 to 2%) – systemic/pulmonary venous obstruction (PVO) (1%) – perforation of the atrium or aorta with hemopericardium (1 to 2%) – atrial arrhythmias (1 to 3%) – and malpositioning/embolization of the device requiring intervention (2 to 15%)

13. The following ASD patients require periodic follow up by an ACHD cardiologist Those repaired as adults Elevated pulmonary artery pressures at the time of repair Atrial arrhythmias pre- or post-operatively Ventricular dysfunction pre-operatively Co-existing heart disease (e.g. coronary artery disease, valvular heart disease, hypertension) Those with device closure need follow-up in specialized centers with serial ECGs and echocardiograms to determine the late outcomes of these new techniques Endocarditis prophylaxis and aspirin are recommended for 6 months following device closure http://www.achd-library.com/index.html