1. VSD Case Discussion

2. Patient Data 23 y/o female 23 y/o female Underline Disease: Underline Disease: 1. Large VSD 2. Pulmonary hypertension, secondary 3. moderate TR and PR

3. Patient Data heart murmur which was noted in LMD since childhood heart murmur which was noted in LMD since childhood exercise intolerance (compared with other children) when she was a child exercise intolerance (compared with other children) when she was a child 2 years ago, she had a cold and treated at LMD. Dyspnea of exertion was noted 2 years ago, she had a cold and treated at LMD. Dyspnea of exertion was noted exertional dyspnea deteriorated in recent 2 years but no orthopnea, PND has ever occurred. In addition, no chest tightness or chest pain was noted exertional dyspnea deteriorated in recent 2 years but no orthopnea, PND has ever occurred. In addition, no chest tightness or chest pain was noted

4. Patient Data 亞東 hospital and received a series work up. EKG, cardiac echo and catheterization were done there and large VSD with pulmonary hypertension was told 亞東 hospital and received a series work up. EKG, cardiac echo and catheterization were done there and large VSD with pulmonary hypertension was told Sep, 2004. Large VSD with pulmonary hypertension, moderate TR and PR, LVEF: 61% were noted Sep, 2004. Large VSD with pulmonary hypertension, moderate TR and PR, LVEF: 61% were noted MR study was performed on 2005/4/15 and showed VSD with pulmonary arterial hypertension and RV hypertrophy MR study was performed on 2005/4/15 and showed VSD with pulmonary arterial hypertension and RV hypertrophy

5. Cardiac ECHO 95/01/15 95/01/15 1. Pulmonary hypertension 2. Large VSD, type 2 with 1. Pulmonary hypertension 2. Large VSD, type 2 with bidirectional shunt 3. MV prolapse with mild MR bidirectional shunt 3. MV prolapse with mild MR 4. TV prolapse with moderate TR 4. TV prolapse with moderate TR PR, moderate 5. Dilated RA, LV & RV, Fair LV PR, moderate 5. Dilated RA, LV & RV, Fair LV contractility contractility

6. Cardiac ECHO 94/01/26 94/01/26 LVEDD5.78 cm LVESD3.87 cm AO 2.00 cm LA 2.76 cm EF 61 % 1.RV, LV chamber enlargement, LVEF 61% 2. An interruption of IVS at perimembranous area with bidirectioanl shunt,size 1.7cm 3. moderate TR, PG 69mmHg 4. posterior bowing of anterior leaflet of mitral valve with mild MR 5. dilater MPA with moderate PR, PG 55mmHg 6. left arch, no CoA, no PDA

7. Cardiac Cath cardiac cath on 95/03/24 and it showed cardiac cath on 95/03/24 and it showed 1. large VSD, perimembranous type, Qp/Qs: 5.1 1. large VSD, perimembranous type, Qp/Qs: 5.1 2. severe pulmonary HTN 2. severe pulmonary HTN

8. Cardiac Cath 95/3/24 95/3/24 SitePressureSaturation IVC68.667.0 RA271.170.8 SVC61.359.3 RV110/674.976.1 MPA109/467090.791.3 LPA106/46709090.7 AAo107/72879696.6 LV102/597.698.2 LPAW5

9. Cardiac Cath Angiography Angiography 1. LV showed a large shunt from LV to RV via a defect of IVS at perimembranous area 2. AAo showed L ’ t aortic arch without morphological CoA

10. Image -- CXR

12. Image CXR 3/23 Cardiomegaly. Prominent pulmonary conus and engorged bilateral perihilar vasculature. 3/23 Cardiomegaly. Prominent pulmonary conus and engorged bilateral perihilar vasculature. 7/13 Cardiomegaly. Prominent pulmonary conus and bil. perihilar vascular lung markings. Sharp CP angles. 7/13 Cardiomegaly. Prominent pulmonary conus and bil. perihilar vascular lung markings. Sharp CP angles.

13. Image -- CT 1. D-loop, large VSD +RVH + LVH, 2. engorged PAs. Thymus (+); LPA[mm]=18.1 ; RPA=28.5 ; McGoon=2.89 3. Bil. clear lungs but mild emphysema of the anterior basal seg. of the bil. lower lungs, normal pattern of the tracheobronchial tree without definite stenosis. 3. Bil. clear lungs but mild emphysema of the anterior basal seg. of the bil. lower lungs, normal pattern of the tracheobronchial tree without definite stenosis.

14. Image -- MR 1. A interventricular defect, measureing about 2.7 cm in diameter, is found in the subaortic region. 2. Hypertrophy of RV wall, and engorged MPA with AP ratio = 2.1/4.1 are noted, indicating presence of significant arterial pulmonary hypertension. 3. The shunt in the VSD is from LV to RV during systole, and from RV to LV during diastole. * pulmonary flow quantification: windkessel volume (cc): 510 (norm: 176 +/- 45) acceleration volume (cc): 35 (norm: 25 +/- 5) max Q / acceleration vol (sec^2): 342 (norm: 154 +/- 24) windkessel vol./acceleration vol: 15 (norm: 7 +/- 2) * LV and RV function

15. Operation OP method: PA banding OP method: PA banding OP finding: OP finding: 1. VSD, large bidirectional shunt 1. VSD, large bidirectional shunt 2. Ao 20mm, PA 40mm 2. Ao 20mm, PA 40mm 3. post-banding: 3. post-banding: ABP 102/54, mean 69 ABP 102/54, mean 69 PAP 48/28, mean 39 PAP 48/28, mean 39 CVP 9 CVP 9 SpO2 93%, FiO2 40%, SvO2 80% SpO2 93%, FiO2 40%, SvO2 80%

16. Post-OP 7/14: extubation, O2 mask used 7/15 cardiac ECHO: PG40-48 mmHg Sever PR with PG 44mmHg Sever PR with PG 44mmHg Sever TR with PG 68mmHg Sever TR with PG 68mmHg VSD size 1.86cm, bidirectional shunt VSD size 1.86cm, bidirectional shunt 7/19 cardiac echo: LEVF 36-42% PA banding, diameter 0.9-1.17 cm PA banding, diameter 0.9-1.17 cm PG 42mmHg PG 42mmHg Qp/Qs = 1.1 Qp/Qs = 1.1 Suggest adequate PA banding Suggest adequate PA banding

17. Cardiac ECHO 95/07/16 (after OP) 95/07/16 (after OP) LVEDD4.47 cm LVESD3.65 cm AO2.31 cm LA2.17 cm EF37.8 % 1. four chamber enlargement, 2. poor LV function with flat LV septal motion and decrease posterior wall excursion, LVEF 37.8% 3. Alarge interruption of IVS at perimembranous area with bidirectioanl shunt,size 2.0cm 4. mild to moderate TR, PG 101mmHg 5. mild posterior bowing of anterior leaflet of mitral valve without MR 6. s/p PA banding with banding diameter 8.2mm, PG 39-44mmHg, dilater MPA with mild PR 7. left arch, no CoA, no PDA 8. catheter in RV thru PA

18. Swan-Ganz 95/7/19 95/7/19 PA O2 saturation : 65.8 % PA O2 saturation : 65.8 % SVc O2 saturation: 53.3 % SVc O2 saturation: 53.3 % Ao O2 saturation : 85.7 % Ao O2 saturation : 85.7 % Qp / Qs = 85.7 -53.3 / 99 – 65.8 Qp / Qs = 85.7 -53.3 / 99 – 65.8 = 0.975 = 0.975

19. VSD

21. physiologic effects of VSDs depend upon the size of the defect and the PVR physiologic effects of VSDs depend upon the size of the defect and the PVR Large VSD: size approximately the size of aortic orifice Large VSD: size approximately the size of aortic orifice Moderate VSD: size ≦ ½ aortic orifice Moderate VSD: size ≦ ½ aortic orifice Small VSD: < 1/3 aortic orifice Small VSD: < 1/3 aortic orifice

22. Physiology In the normal case, where no connection exists, the ratio Qp:Qs is 1:1. Left-to-right shunting results in a Qp:Qs >1, while right-to-left shunting results in a Qp:Qs 1, while right-to-left shunting results in a Qp:Qs <1

23. Natural Hsitory Small VSD: Small VSD:  75% spontaneous closure (< 2 y/o)  left-to-right shunt less than 33 percent (Qp/Qs <1.5) percent (Qp/Qs <1.5)  No evidence of left ventricular volume overload overload  Normal pulmonary artery pressure  No VSD-related aortic regurgitation or symptoms symptoms

24. Natural History Large VSD Large VSD  rarely close spontaneously  elevated pulmonary artery vascular resistance leads to RV pressure overload  RV hypertrophy.  Eisenmenger syndrome.

25. Clinical Feature S/S: Tachypnea, Poor feeding, Poor weight gain, Tachycardia, Hepatomegaly, Pulmonary rales, grunting, and retractions,Pallor S/S: Tachypnea, Poor feeding, Poor weight gain, Tachycardia, Hepatomegaly, Pulmonary rales, grunting, and retractions,Pallor PE: PE: holosystolic murmur holosystolic murmur diastolic murmurs in infants may indicate increased left-to-right shunting or the development of aortic or pulmonary regurgitation diastolic murmurs in infants may indicate increased left-to-right shunting or the development of aortic or pulmonary regurgitation

26. Diagnosis ECG ECG CXR CXR ECHO ECHO MRI MRI Cardiac Cath Cardiac Cath

27. EKG The ECG is normal in patients with small VSDs. The ECG is normal in patients with small VSDs. moderate or large left-to-right shunts : left atrial enlargement and LV hypertrophy (LVH) moderate or large left-to-right shunts : left atrial enlargement and LV hypertrophy (LVH) RV hypertrophy in addition to LVH. RV hypertrophy in addition to LVH.

28. CXR small defects: normal CXR small defects: normal CXR moderate to large defects : pulmonary vascular markings are increased, and the left atrium, LV, and PA may be enlarged. moderate to large defects : pulmonary vascular markings are increased, and the left atrium, LV, and PA may be enlarged. PVR increases: RV enlargement becomes more prominent and the LV decreases in size; anterior bulging of the lower sternum may be present PVR increases: RV enlargement becomes more prominent and the LV decreases in size; anterior bulging of the lower sternum may be present

29. Surgical Indication Hemodynamic indication Hemodynamic indication Qp/Qs > 1.5 Rp/Rs < 0.75 Pulmonary arteriolar resistance < 7 wood unit

30. Surgical Indication Anatomical indication Anatomical indication VSDI (doubly commited subarterial type, muscular outlet type) VSDI (doubly commited subarterial type, muscular outlet type) Perimembranous type Perimembranous type others others Recurrent IE Recurrent IE Large VSD : < 3 months – CHF Large VSD : < 3 months – CHF Small VSD : Cardiac Enlargement Small VSD : Cardiac Enlargement Failure to trive Failure to trive

31. PA banding a) Swiss cheese type + symptomatic b) VSD w/ straddling A-V valve c) Contraindication to surgery i) Fixed pulmonary HTN (greater than 8 Wood units) (greater than 8 Wood units) [Wood units = mm Hg/L/min/m2] [Wood units = mm Hg/L/min/m2]

32. Measurement of left-to-right shunt Cardiac Cath Cardiac Cath MRI MRI RNA RNA

33. Cardiac Cath Pulmonary resistance = Pulmonary resistance = Mean PAP – mean LAP / pulmonary folw Mean PAP – mean LAP / pulmonary folw Pulmonary flow ≒ Pulmonary flow ≒ O2 consumption / PVO2 – PAO2 O2 consumption / PVO2 – PAO2 Qp / Qs ≒ Qp / Qs ≒ sys A-V O2 difference / pul A-V O2 difference = sys A-V O2 difference / pul A-V O2 difference = (aortic O2 sat - central venous O2 sat)/ (aortic O2 sat - central venous O2 sat)/ (pulm venous O2 sat - pulm art sat) (pulm venous O2 sat - pulm art sat)

34. Cardiac Cath – Pul flow Pulmonary flow ≒ Pulmonary flow ≒ O2 consumption / PVO2 – PAO2 O2 consumption / PVO2 – PAO2 Estimated VO2 (female) = 138.1 – 17.04 x ln(age) + 0.378 x HR Estimated VO2 (female) = 138.1 – 17.04 x ln(age) + 0.378 x HR Estimated VO2 (male) = 138.1 – 11.49x ln(age) + 0.378 x HR Estimated VO2 (male) = 138.1 – 11.49x ln(age) + 0.378 x HR 邱 XX: pul flow = 138.1 – 17.04 ln(23) +0.378 x 80 / 99-90 = 12.66 邱 XX: pul flow = 138.1 – 17.04 ln(23) +0.378 x 80 / 99-90 = 12.66

35. Cardiac Cath – Pul resistance Pulmonary resistance = Pulmonary resistance = Mean PAP – mean LAP / pulmonary folw Mean PAP – mean LAP / pulmonary folw 邱 XX pulmonary risistance = 邱 XX pulmonary risistance = 70 – 30 / 12.6 70 – 30 / 12.6 = 3.16 wood unit = 3.16 wood unit

36. Cardiac Cath – L-to-R shunt Qp / Qs ≒ Qp / Qs ≒ sys A-V O2 difference / pul A-V O2 difference = sys A-V O2 difference / pul A-V O2 difference = (aortic O2 sat - central venous O2 sat)/ (aortic O2 sat - central venous O2 sat)/ (pulm venous O2 sat - pulm art sat) (pulm venous O2 sat - pulm art sat) 邱 XX Qp/Qs = 96.6-59 / 97.6-92 = 4 邱 XX Qp/Qs = 96.6-59 / 97.6-92 = 4

37. MRI

38. RNA

39. summary