1. Congenital Heart Diseases: Atrial Septal Defect

2. Patient History This is a case of G.L., a 21 year old female, complaining of easy fatigability and occasional chest pain who was referred for cardiovascular evaluation prior to employment. She was diagnosed to have “heart disease” in childhood. Past medical history revealed frequent upper respiratory tract infections.

3. Physical Examination General: Hypoesthenic, narrow A-P diameter Vital Signs: BP: 100/80 mmHg PR: 75 bpm RR: 20 cpm BMI: 15 kg/m2

4. Physical Examination Thorax and Lungs: symmetrical chest expansion, resonant on all lung all fields, (-) crackles

5. Physical Examination Cardiovascular Examination: JVP and CAP – normal at base: S1 is normal followed by a grade 3/6 crescendo decrescendo murmur S2 wide with fixed splitting at apex: multiple clicks

6. InspirationExpiration 3/6 A2 P2 C C C JVP CAP

7. 12- Lead ECG Normal sinus rhythm Right ventricular hypertrophy Incomplete right bundle branch block Diffuse ST-T changes

8. Chest Radiograph Cardiomegaly with multi-chamber enlargement Pulmonary congestion

9. 2D- Echocardiography ASD, ostium secundum type Markedly dilated right bentricle with adequate wall motion and contractility with evidence of RV pressure and volume overload Dilated right atrium without thrombus Dilated main pulmonary artery TR, severe PR Moderate pulmonary hypertension Reverse E/A across mitral valve

10. Salient Features 21 y/o, female Easy fatigability and occasional chest pain Childhood “heart disease” Frequent URTI Hypoesthenic with BMI of 15 kg/m2

11. PECXRECG2D Echo ASD, ostium secundum type Left lower parasternal lift Cardiomegal y with multi- chamber enlargement Right ventricular hypertrophy Dilated right ventricle with RV pressure and volume overload Severe TR Systolic grade 3/6 crescendo- decrescendo murmur at base Dilated main pulmonary artery Pulmonary regurgitation

12. PECXRECG2D Echo Fixed and widely split S2 Pulmonary congestion Moderate pulmonary hypertension Multiple clicks at apex Reverse E/A across mitral valve Cardiomegal y with multi- chamber enlargement Right atrium and ventricle dilated

13. InspirationExpiration S1 C1 S2 3/6 JVP 3 cm at 30 0 CAP

15. Diagnosis Cardiac Diagnosis: Etiologic: Congenital Heart Disease Anatomic:ASD, TR, PR Dilated RA & RV, Dilated Main Pulmonary Artery Physiologic: not in failure Functional: Class II-B Pulmonary Hypertension

16. DIFFERENTIAL DIAGNOSIS

17. (+) upper respiratory tract infection Mitral stenosis -dyspnea and cough on exertion -orthopnea and PND -hemoptysis Mitral regurgitation -dyspnea and cough on exertion -orthopnea and PND -ankle edema premature birth, perinatal distress, or perinatal hypoxia may be present most children with small defects remain asymptoma-tic In patients with Eisenmenger syndrome, symptoms in adult life consist of exertional dyspnea, syncope and hemoptysis R-L shunt leads to cyanosis, clubbing, and erythorocy- tosis Prolonged symptom- free period Palpitations Fatigue Dyspnea on exertion Orthopnea Frequent respiratory infections Symptoms of right ventricular failure “heart disease ” in childhood frequent URTI easy fatigability occasional chest pain Hx RHDPDAVSDASDPatient

18. Mitral stenosis diastolic thrill at the apex S1 and P2 are accentuated S2 is split or fixed OS of the mitral valve on expiration (+) carvallo's sign Mitral regurgitation (+) systolic thrill at the apex holosystolic murmur -(+) S4 precordial activity is increased apical impulse is laterally displaced S1 normal, S2 typically obscured by murmur Continuous machinery- like murmur Bounding peripheral pulses loud, harsh, or blowing holosystolic murmur is heard best over the lower LSB in the 3rd or 4th ICS displaced cardiac apex with a similar holosystolic murmur apical diastolic rumble and third heart sound (S3) Prominent RV impulse S1 normal or split, with accentuation of TV closure sound Wide & fixed splitting of the S2 Systolic ejection murmur (heard in pulmonic area) Diastolic rumble across the tricuspid valve Neck vein distention Ascites Edema Hyposthenic, narrow AP chest diameter Normal JVP and CAP Left lower sternal lift Normal S1 ff. by gr. 3/6 crescendo- decrescendo murmur S2 wide with fixed splitting Multiple clicks at apex P.E. RHDPDAVSDASDPatient

19. LVHLVH with a larger PDA May be normal With larger defects there are various degrees of right axis deviation associated with right ventricular Enlargement Ostium secundum defects – incomplete right bundle block & right axis deviation Ostium primum defects – left anterior hemiblock & left axis deviation Normal sinus rhythm RVH Incomplete RBBB Diffuse ST-T changes ECG RHDPDAVSDASDPatient

20. Mitral stenosis -(+) kerley B lines -concentric hypertrophy of left atrium -cephalization -prominent main pulmonary artery and branches -constriction of arteries in the middle and peripheral lung zones Mitral regurgitation -eccentric hypertrophy of the left atrium -LA and LV enlargement -equalization, cephalization -mitral annulus calcification LAE LVE PAE PVE Right Ventricular enlargement Increased pulmonary vascular markings Shunt vascularity (inc. pulm. vascular markings) Right ventricular enlargement Enlargement of the pulmonary artery segment in P-A view Cardiomegaly with multi- chamber enlargement and pulmonary congestion CXR RHDPDAVSDASDPatient

21. Mitral stenosis -mitral orifice <4cm -concentric hypertrophy of left atrium -annular calcifications Mitral regurgitation -eccentric hypertrophy of the left atrium -LA enlargement -hyperdynamic LV -annular calcifications/ LV dyskinesis -ruptured chordae tendineae LAE Continuous flow from the aorta into the main pulmonary artery color flow can show the shunting of blood from the left ventricle to the right VSD's can result in a shunt from below the tricuspid valve to below the pulmonary valve. Enlargement of RV Negative-contrast image at the site of defect (saline injection) Doppler – abnormal pressure of left-to- right blood flow across the septum ASD, ostium secundum type Markedly dilated right ventricle with adequate wall motion and contractility with evidence of RV pressure and volume overload Dilated RA w/o thrombus Dilated MPA Severe TR PR Mod. Pul. HPN Reverse E/A across mitral valve Echo RHDPDAVSDASDPatient

22. Radiologic Findings L to R Shunt ASD, Ostium secundum Cardiomegaly Accentuated pulmonary vascular markings

23. ASD Accentuated pulmonary vascular markings MPA convex

24. L to R Shunting VSD Accentuated pulmonary vascular markings Convex MPA

25. R anterior oblique Displacement of the esophagus RA, RV and LV enlargement

26. Pulmonary Arterial Hypertension Causes –Primary/ Idiopathic Genetic –Secondary Cardiac Pulmonary 1.hypoxic vasoconstriction 2.decreased area of the pulmonary vascular bed 3.volume/pressure overload

27. Secondary Pulmonary Arterial Hypertension Hypoxic Vasoconstriction –COPD and obstructive sleep apnea –Due to down regulation of endothelial nitric oxide synthetase

28. Decreased Area of Pulmonary Bed –Occurs when loss of vessels exceed 60% of the total pulmonary vasculature –Occurs in patients with collagen vascular disease like CREST and scleroderma. And those with chronic emboli

29. Volume/ Pressure Overload –Seen in patients with left to right intracardiac shunts –May passively occur in patients with left atrial hypertension and left ventricular dysfunction, mitral valve disease and hose with aortic stenosis

30. Pulmonary Arterial Hypertension Common Features –Medial hypertrophy –Eccentric and concentric intimal fibrosis –Recanalized thrombi –Plexiform lesions

31. There is medial hypertrophy and marked intimal hyperplasia of this muscular pulmonary artery. These changes are the morphologic response to increased pulmonary vascular pressures, such as may be seen in patients with chronic obstructive lung disease, pulmonary thromboembolism, or left-sided heart disease (e.g., mitral stenosis). Occasionally, elevated pulmonary pressures are seen without identifiable cause (idiopathic or primary pulmonary hypertension).

32. A. PA chest film demonstrates mild cardiomegaly with normal pulmonary arterial markings. There is pulmonary venous congestion, fluid within the horizontal fissure and prominent Kerley B lines (indicative of lymphatic engorgement).

33. Lateral chest film show marked venous congestion with fluid visible in both the horizontal and oblique fissures.

34. Pulmonary Arterial Hypertension Chest radiograph –The classic finding on a chest radiograph from a patient with PAH is enlargement of central pulmonary arteries, attenuation of peripheral vessels, and oligemic lung fields. –Findings of right ventricular and right atrial dilatation are possible.

35. A posterior-anterior (PA) chest radiograph demonstrates enlargement of the main pulmonary artery and right pulmonary artery.The peripheral pulmonary arteries are reduced in caliber. The lung volumes are normal without evidence of underlying pulmonary parenchymal disease. A lateral radiograph also demonstrates enlargement of both the right and left pulmonary arteries. Cardiomegaly, with predominant right ventricular enlargement is present.

36. Pulmonary Venous Hypertension Occurs secondary to increased resistance to pulmonary venous drainage Associated with diastolic dysfunction of the left ventricle and valvular dysfunctions (aortic and mitral valve stenosis and regurgitation)

37. Pulmonary Venous Hypertension Features –Capillary congestion –Focal alveolar edema –Dilatation of interstitial lymphatics

38. The alveolar septa appear thickened, and the alveolar spaces contain numerous pigment-laden macrophages. A few alveolar spaces contain pink edema fluid. Note the thick-walled pulmonary vessel.

39. Acute Pulmonary Congestion and Edema

40. Management Medical Watch out for dev’t of complications »Arrhythmias, CHF, endocarditis Operative repair: definitive –Patch of pericardium or of prosthetic material –Percutaneous transcatheter device closure –Indications: symptomatic significant L to R shunting –Qp:Qs ≥ 1.5:1 –RA or RV enlargement – radiographic, cardiac catheterization think otherwise if: –PVR > 12 U/mL / significant pulmonary hypertension –Eisenmenger – R to L shunting with cyanosis

41. Management Results Surgical repair w/ mortality rates near zero Complications: atrial arrhythmias, left atrial hypertension Transcather closure –Air embolism (1-3%), thromboembolism from device (1- 2%), systemic, pulmonary venous obstruction (1%), perforation of atrium/aorta (1-2%), atrial arrhythmias (1- 3%) Schwartz’s Principles of Surgery, 8 th Ed.