Robosa, Dino Rodas, Francis Rodriguez, Shereen Rogelio, Ma. Gracella Salazar, Riccel Salcedo, Von

 Etiology: Congenital Heart Disease  Anatomy: atrial septal defect, ostium secundum, dilated right atrium, markedly dilated and hypertrophied right ventricle, dilated main pulmonary artery, anterior mitral valve prolapse  Physiology: NSR, incomplete right bundle branch block, diffuse ST-T changes, moderate pulmonary hypertension, increased right ventricular pressure and overload  Functional Capacity: Class II  Objective Assessment: C

2. How do you explain the auscultatory findings?

a. At the base, S1 is normal followed by a grade 3/6cresendo-decresendo murmur – Increased flow across the pulmonic valve is responsible for a midsystolic pulmonary outflow murmur – Grade 2–3 mid-systolic murmur at the mid to upper left sternal border with fixed splitting of S 2 Ostium secundum ASDs are most common

b. S2 is wide with fixed splitting

Wide splitting – The split becomes wider when there is delayed activation of contraction or emptying of the right ventricle resulting in a delay in pulmonic closure Fixed splitting – This occurs with delayed closure of the pulmonic valve when output of the right ventricle is greater than that of the left ventricle (such as occurs in large atrial septal defects, a ventricular septal defect with left to right shunting, or right ventricular failure)

c. At the apex, multiple clicks are heard ◦ Midsystolic clicks, occurring with or without a late systolic murmur, often denote prolapse of one or both leaflets of the mitral valve  Results from the chordae tendineae that are functionally unequal in length  Best heard along the lower left sternal border and at the left ventricular apex ◦ Systolic clicks usually occur later than the systolic ejection sound.

 The right atrium is enlarged due to volume overload. ◦ Tall P waves (more than 2-3 mm) ◦ RVH is also noted, typically as rsR’ pattern of the QRS complexes in the right chest leads

 Acyanotic  Includes: ◦ Ventral septal defect ◦ Atrial septal defect ◦ Patent ductus arteriosus

Left-to- Right Shunt Chambe rs Enlarged Prominen t aortic knob Dilated MPA Pulmonar y Vascularit y Ventral Septal Defect LA and LV NoYes↑ Atrial Septal Defect RA and RV NoYes (convex) ↑ Patent Ductus Arterios us LA and LV Yes ↑

Left-to- Right Shunt Chambe rs Enlarged Prominen t aortic knob Dilated MPA Pulmonar y Vascularit y Ventral Septal Defect LA and LV NoYes↑ Atrial Septal Defect RA and RV NoYes (convex) ↑ Patent Ductus Arterios us LA and LV Yes ↑

 RV Enlargement ◦ PA view: lateral upward displacement of the cardiac apex ◦ Lateral view: fullness of retrosternal space  RA Enlargement ◦ PA view: increased convexity of the lower right cardiac border

Normal PA viewPA view (ASD) SV C RV IVC Aortic knob MPA LV Dilated MPA Increased pulmonary vascularity

Right ventricular enlargement 2/3 1/3 Retrosternal space Normal Lateral view Lateral view (ASD) Retrosternal space

 Acyanotic  Includes: ◦ Ventral septal defect ◦ Atrial septal defect ◦ Patent ductus arteriosus

Left-to- Right Shunt Chambe rs Enlarged Prominen t aortic knob Dilated MPA Pulmonar y Vascularit y Ventral Septal Defect LA and LV NoYes↑ Atrial Septal Defect RA and RV NoYes (convex) ↑ Patent Ductus Arterios us LA and LV Yes ↑

Left-to- Right Shunt Chambe rs Enlarged Prominen t aortic knob Dilated MPA Pulmonar y Vascularit y Ventral Septal Defect LA and LV NoYes↑ Atrial Septal Defect RA and RV NoYes (convex) ↑ Patent Ductus Arterios us LA and LV Yes ↑

 RV Enlargement ◦ PA view: lateral upward displacement of the cardiac apex ◦ Lateral view: fullness of retrosternal space  RA Enlargement ◦ PA view: increased convexity of the lower right cardiac border

Normal PA viewPA view (ASD) SV C RV IVC Aortic knob MPA LV Dilated MPA Increased pulmonary vascularity

Right ventricular enlargement 2/3 1/3 Retrosternal space Normal Lateral view Lateral view (ASD) Retrosternal space

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)

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

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

 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.

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.

Prevention of Infective Endocarditis: Guidelines From the American Heart Association

 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.

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.

Device closure may now be offered as an alternative to surgical closure to patients with secundum ASD of up to 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

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

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%)

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