1. Workshop 11: CHD-ASD D4 Saldana, Sales, Salonga, San Diego, San Pedro, Sanez, Sanidad, Santos E., Santos J., Santos J., Santos K., Santos M.

2. GL, 21 y/o female Chief Complaints – Easy fatigability – Occasional chest pain Past Medical History – Diagnosed to have “heart disease” in childhood – Frequent upper respiratory tract infection

3. Physical Examination Hyposthenic, narrow AP chest diameter BP 100/80 PR 75/min RR 20/min BMI 15 JVP and CAP normal Left lower sternal lift Auscultation – Base: normal S1 followed by grade 3/5 crescendo- decrescendo murmur, S2 is wide with fixed splitting – Apex: multiple clicks are heard Lungs – Equal expansion, resonant no crackles

4. 12- Lead ECG – Normal sinus rhythm – Right ventricular hypertrophy – Incomplete bundle branch block – Diffuse ST-T changes CXR – Cardiomegaly with multichamber enlargement – Pulmonary Congestion

5. Echo-Doppler ASD, ostium secundum type Markedly dilated RV with good wall motion and contractility – With evidence of RV pressure and volume overload Dilated RA with no evidence of thrombus Dilated main pulmonary artery Mitral valve prolapse, anterior mitral valve leaflet PR Moderate pulmonary hypertension

6. Hemodynamic Studies

7. Congenital Heart Disease Etiology ASD ostium secundum, Dilated RA w/o evidence of thrombus and MPA RV hypertrophy Mitral valve prolapse, PR, Pulmonary hypertension Anatomic Incomplete bundle right bundle branch block Physiologic Class III Functional CARDIAC DIAGNOSIS

8. AUSCULTATORY FINDINGS

9. EXPIRATIONINSPIRATION S1S2S1S2 CAP JVP a v y x

10. Grade 3/6 Crescendo Decrescendo Murmur Midsystolic Murmur Begins just after the S 1 heart sound and terminates just before the P 2 heart sound 3/6 = Moderately loud Increased flow across the pulmonic valve

11. Widened Split S2 I: RBBB Delay in the closure of the pulmonic valve No effect on the closure of aortic valve Occurs during inspiration

12. Fixed Split S2 Rare I: ASD Delay in the closure of pulmonary valve Same amount of splitting throughout the entire respiratory cycle

13. Multiple Clicks Mitral valve prolapse Sudden tensing of the valves as they reach their elastic limit

14. CHEST X-RAY FINDINGS: Left-to-Right Shunt

15. Uncomplicated ASD: Left-to-Right shunt enlargement of central and all segments of pulmonary arteries increased pulmonary vascularity, prominent hilar markings “shunt vascularity”

16. RA enlargement RV enlargement: filling in of retrosternal space; posterior displacement of the LV toward the spine Uncomplicated ASD: Left-to-Right shunt

17. RV enlargement: increased opacification posterior to the sternum Uncomplicated ASD: Left-to-Right shunt

18. enlargement of the cardiac silhouette enlarged central and peripheral pulmonary arteries normal- to small-sized aorta absent SVC shadow- bec. of rotation of the heart from right-sided cardiac enlargement Uncomplicated ASD: Left-to-Right shunt

19. Long Standing Shunt Leads to pulmonary arterial hypertension Eisenmeger Physiology – when pulmonary arterial pressure exceeds systemic arterial pressure, reversal of shunting of blood from left-to-right to right-to-left occurs CXR findings: marked central pulmonary artery dilatation narrowing of peripheral pulmonary artery branches central pulmonary arteries become aneurysmal and rarely, be calcified

20. Long Standing Shunt (Eisenmeger Physiology) enlargement of the right heart absence of the SVC shadow aneurysmal enlargement and calcification central pulmonary arteries

21. Pulmonary ARTERIAL Congestion vs. Pulmonary VENOUS Congestion

22. Pulmonary Arterial Congestion Pulmonary Venous Congestion active congestion -arteriolar dilation leads to increased blood flow passive congestion - dilation of veins and capillaries due to impaired venous outflow Affected tissues turn red (erythema) because of the engorgement of vessels with oxygenated blood tissues take on a dusky reddish-blue color (cyanosis) due to red cell stasis and the accumulation of deoxygenated hemoglobin

23. Pulmonary Arterial Hypertension Medial hypertrophy, eccentric and concentric intimal fibrosis, recanalized thrombi appearing as fibrous webs, and plexiform lesions Abnormalities in molecular pathways regulating the pulmonary vascular endothelial and smooth-muscle cells – loss of apoptosis of the smooth-muscle cells allowing their proliferation – emergence of apoptosis-resistant endothelial cells which can obliterate the vascular lumen

24. Pulmonary Arterial Hypertension three types of changes in the pulmonary arteries: – Muscular walls of the arteries may tighten up  narrower lumen – Walls may thicken as the amount of muscle increases in some arteries. Scar tissue may form in the walls of arteries. As the walls thicken and scar, the arteries become increasingly narrow. – Tiny blood clots may form within the smaller arteries, causing blockages

25. Pulmonary Venous Hypertension occurs in the setting of elevated left sided filling pressure often associated with diastolic dysfunction of the left ventricle; diseases affecting the pericardium or mitral or aortic valves; or rare entities such as cor triatriatum, left atrial myxoma, extrinsic compression of the central pulmonary veins from fibrosing mediastinitis, and pulmonary venoocclusive disease. the degree of elevation in pulmonary artery pressure is concordant with the degree of elevation in left atrial pressure.

26. Pulmonary Venous Congestion arterialization of the external elastic lamina, medial hypertrophy, and focal eccentric intimal fibrosis Microcirculatory lesions: capillary congestion, focal alveolar edema, and dilatation of the interstitial lymphatics

27. MANAGEMENT

28. Operative repair – Patch of pericardium – Prosthetic material – Percutaneous transcatheter device closure Indicated for all patients with uncomplicated secundum atrial septal defects w/ significant left to right shunting – Qp:Qs ≥ 1.5:1 – RA or RV enlargement

29. Post operative Management Aspirin w/ or w/out clopidogrel 6 months Prevent thrombus formation

30. Closure should not be carried out: – Small defects – Trivial left to right shunts – Severe pulmonary vascular disease w/out significant left to right shunt

31. Follow Up At least 1 follow-up echocardiogram to confirm complete closure of the ASD Yearly appointment after the immediate postoperative period is adequate

32. THANK YOU