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. Differential Diagnosis

15. ASDVSDPDAPAPVR CARDIAC SIZE↑↑↑↑ Pulmonary Vascularity ↑↑↑↑ Main Pulmo Artery Segment Prominent LVNormalEnlarged Normal LANormalEnlarged Normal RVEnlargedNormal Enlarged RAEnlargedNormal Enlarged AortaSmall EnlargedSmall Charactistic feature Absent LAE Enlarged AortaSchimitar Sign

16. Partial Anomalous Pulmonary Venous Return blood flow from a few of the pulmonary veins return to the right atrium instead of the left atrium pulmonary venous flow enters the systemic venous circulation The most common type of PAPVR is one in which a right upper pulmonary vein connects to the right atrium or the superior vena cava. almost always associated with a sinus venosus type of ASD

17. Anatomical Variations in PAPVR A.PAPVR with ASD -In 80-90% of cases, most common type -anomalous drainage -true anomalous connection B. Isolated PAPVR -Intact atrial septum -mostly involves the anomalous drainage of the right upper pulmonary vein into the superior vena cava

18. Anatomical Variations in PAPVR C. PAPVR with complex congenital heart disease (heterotaxia) -Left atrial isomerism with a common atrium Because of -right-sided pulmonary veins and IVC to the right- sided atrium -ipsilateral pulmonary hypoplasia D. Scimitar syndrome -right pulmonary vein to inferior vena cava with lung sequestration

20. FINDINGSASD in the PatientPAPVR HISTORY Asymptomatic in childhood Easy Fatiguability Chest Pain Previous Upper Respi Infections Asymptomatic in childhood Dyspnea & Easy Fatiguability Chest Pain PHYSICAL EXAM JVP & CAP normal Narrow AP diameter Left Lower Sternal Lift Grade 3/6 cresendo decresendo murmur S2 wide with fixed splitting Normal Respirations JVP & CAP (?) Narrow AP diameter Left Parasternal Lift Soft Systolic Ejection Murmur & Mid- Diastolic Murmur S2 wide splitting Normal Respirations X-ray Cardiomegaly with Multi- chamber Enlargement Pulmonary Congestion Possible 2D Echo- Doppler ASD-Ostium Secundum Dilated RV, RA, MPA Pulmonary HPN May present with ASD in 10% of cases Dilated RV, RA, MPA Pulmonary HPN

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

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

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

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

25. 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

26. 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

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

28. Pulmonary ARTERIAL Congestion vs. Pulmonary VENOUS Congestion

29. 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

30. 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

31. 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

32. 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.

33. 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

34. MANAGEMENT

35. 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

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

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

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

39. THANK YOU