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VSD Case Discussion. Patient Data. 23 y/o female Underline Disease: 1. Large VSD 2. Pulmonary hypertension, secondary 3. moderate TR and PR. Patient Data. heart murmur which was noted in LMD since childhood exercise intolerance (compared with other children) when she was a child
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Patient Data • 23 y/o female • Underline Disease: 1. Large VSD 2. Pulmonary hypertension, secondary 3. moderate TR and PR
Patient Data • heart murmur which was noted in LMD since childhood • 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 • exertional dyspnea deteriorated in recent 2 years but no orthopnea, PND has ever occurred. In addition, no chest tightness or chest pain was noted
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 • 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
Cardiac ECHO • 95/01/15 1. Pulmonary hypertension2. Large VSD, type 2 with bidirectional shunt3. MV prolapse with mild MR 4. TV prolapse with moderate TR PR, moderate5. Dilated RA, LV & RV, Fair LV contractility
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 Cardiac ECHO
Cardiac Cath • cardiac cath on 95/03/24 and it showed 1. large VSD, perimembranous type, Qp/Qs: 5.1 2. severe pulmonary HTN
Cardiac Cath • 95/3/24
Cardiac Cath • Angiography • LV showed a large shunt from LV to RV via a defect of IVS at perimembranous area • AAo showed L’t aortic arch without morphological CoA
Image CXR • 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.
Image -- CT • D-loop, large VSD +RVH + LVH, • engorged PAs. Thymus (+); LPA[mm]=18.1 ; RPA=28.5 ; McGoon=2.89 • 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.
Image -- MR • A interventricular defect, measureing about 2.7 cm in diameter, is found in the subaortic region . • Hypertrophy of RV wall, and engorged MPA with AP ratio = 2.1/4.1 are noted, indicating presence of significant arterial pulmonary hypertension. • 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
Operation • OP method: PA banding • OP finding: 1. VSD, large bidirectional shunt 2. Ao 20mm, PA 40mm 3. post-banding: ABP 102/54, mean 69 PAP 48/28, mean 39 CVP 9 SpO2 93%, FiO2 40%, SvO2 80%
Post-OP 7/14: extubation, O2 mask used 7/15 cardiac ECHO: PG40-48 mmHg Sever PR with PG 44mmHg Sever TR with PG 68mmHg VSD size 1.86cm, bidirectional shunt 7/19 cardiac echo: LEVF 36-42% PA banding, diameter 0.9-1.17 cm PG 42mmHg Qp/Qs = 1.1 Suggest adequate PA banding
95/07/16 (after OP) LVEDD4.47 cm LVESD3.65 cm AO2.31 cm LA2.17 cm EF37.8 % four chamber enlargement, poor LV function with flat LV septal motion and decrease posterior wall excursion, LVEF 37.8% Alarge interruption of IVS at perimembranous area with bidirectioanl shunt,size 2.0cm mild to moderate TR, PG 101mmHg mild posterior bowing of anterior leaflet of mitral valve without MR s/p PA banding with banding diameter 8.2mm, PG 39-44mmHg, dilater MPA with mild PR left arch, no CoA, no PDA catheter in RV thru PA Cardiac ECHO
Swan-Ganz • 95/7/19 PA O2 saturation : 65.8 % SVc O2 saturation: 53.3 % Ao O2 saturation : 85.7 % Qp / Qs = 85.7 -53.3 / 99 – 65.8 = 0.975
physiologic effects of VSDs depend upon the size of the defect and the PVR • Large VSD: size approximately the size of aortic orifice • Moderate VSD: size ≦ ½ aortic orifice • Small VSD: < 1/3 aortic orifice
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
Natural Hsitory • Small VSD: • 75% spontaneous closure (< 2 y/o) • left-to-right shunt less than 33 percent (Qp/Qs <1.5) • No evidence of left ventricular volume overload • Normal pulmonary artery pressure • No VSD-related aortic regurgitation or symptoms
Natural History • Large VSD • rarely close spontaneously • elevated pulmonary artery vascular resistance leads to RV pressure overload • RV hypertrophy. • Eisenmenger syndrome.
Clinical Feature • S/S: Tachypnea, Poor feeding, Poor weight gain, Tachycardia, Hepatomegaly , Pulmonary rales, grunting, and retractions ,Pallor • PE: holosystolic murmur diastolic murmurs in infants may indicate increased left-to-right shunting or the development of aortic or pulmonary regurgitation
Diagnosis • ECG • CXR • ECHO • MRI • Cardiac Cath
EKG • The ECG is normal in patients with small VSDs. • moderate or large left-to-right shunts : left atrial enlargement and LV hypertrophy (LVH) • RV hypertrophy in addition to LVH.
CXR • small defects: normal CXR • 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
Surgical Indication • Hemodynamic indication Qp/Qs > 1.5 Rp/Rs < 0.75 Pulmonary arteriolar resistance < 7 wood unit
Surgical Indication • Anatomical indication VSDI (doubly commited subarterial type, muscular outlet type) Perimembranous type • others Recurrent IE Large VSD : < 3 months – CHF Small VSD : Cardiac Enlargement Failure to trive
PA banding a) Swiss cheese type + symptomatic b) VSD w/ straddling A-V valvec) Contraindication to surgery i) Fixed pulmonary HTN (greater than 8 Wood units) [Wood units = mm Hg/L/min/m2]
Measurement of left-to-right shunt • Cardiac Cath • MRI • RNA
Cardiac Cath • Pulmonary resistance = Mean PAP – mean LAP / pulmonary folw • Pulmonary flow ≒ O2 consumption / PVO2 – PAO2 • Qp / Qs ≒ sys A-V O2 difference / pul A-V O2 difference = (aortic O2 sat - central venous O2 sat)/ (pulm venous O2 sat - pulm art sat)
Cardiac Cath – Pul flow • Pulmonary flow ≒ O2 consumption / PVO2 – PAO2 • 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 • 邱XX: pul flow = 138.1 – 17.04 ln(23) +0.378 x 80 / 99-90 = 12.66
Cardiac Cath – Pul resistance • Pulmonary resistance = Mean PAP – mean LAP / pulmonary folw • 邱XX pulmonary risistance = 70 – 30 / 12.6 = 3.16 wood unit
Cardiac Cath – L-to-R shunt • Qp / Qs ≒ sys A-V O2 difference / pul A-V O2 difference = (aortic O2 sat - central venous O2 sat)/ (pulm venous O2 sat - pulm art sat) • 邱XX Qp/Qs = 96.6-59 / 97.6-92 = 4