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Admix ppt

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Kapil Vasanth

This document discusses admixture lesions in congenital heart disease. It covers the physiology, classification, clinical features, and natural course of various conditions that involve mixing of oxygenated and deoxygenated blood, including total anomalous pulmonary venous return (TAPVC), common atrium, double outlet right ventricle (DORV), and double inlet left ventricle (DILV). Examples are provided to illustrate how to calculate pulmonary and systemic blood flows using oxygen saturation measurements. The limitations of oxygen saturation as a measure of physiology are also addressed.

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© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 1 KAPIL R DEPT OF CARDIOLOGY ADMIXTURE LESIONS
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 2 CONGENITAL HEART DISEASES Anatomico-pathologic classification (↑ /↓PBF) Physiological classification (transposition/TOF/Admixture/ Eisenmenger / ductus dependent )
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 3 PHYSIOLOGY TOF physiology Transposition physiology Admixture physiology Ductus dependent physiology Eisenmenger physiology Normal physiology TOF DORV+subaortic VSD +PS TA + PS SV+PS TGA+VSD +PS d-TGA DORV +sub pul VSD TA +TGA +VSD Shunt ASD/VSD/PDA ↑PVR  BD or flow reversal Pre TR-TAPVC Post TR-SV DDSC-HLHS DDPC-Pul.Atresia Pul. AV fistula Mild cyanosis Spells/squatting FTT Severe cyanosis at birth Early CCF Suck-rest-suck cycle RRTI  cyanosis (2/3/4 decade) Mild cyanosis RRTI Early CCF Intense cyanosis Cardiogenic shock Mod-severe cyanosis RRTI Early CCF
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 4 ADMIXTURE - DEFINTION AND GENERAL FEATURES • Complete mixing of deoxygenated blood (SV) + oxygenated blood from pulmonary veins (atria / ventricle / great artery ) • Cyanosis ~ 85 % • S aorta = S pul.art • RRTI , FTT / CHF infancy • Cardiomegaly with hyperdynamic heart • Evidence of PAH • Abundant murmurs – systolic and diastolic
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 5 LEVEL OF MIXING • Systemic Veins-supracardiac TAPVC • Atria- common atrium ,mitral atresia , tricuspid atresia • Ventricles – SV , DORV , TGA with large VSD • Great Vessels (Truncus arteriosus ) • DDSC- HLHS with aortic atresia • DDPC- Pulmonary atresia with intact IVS • Distal Pulmonary Vascular Bed (Pul. AV fistula)
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 6 IMPORTANT BASIC CONCEPTS  Systemic blood flow  neurohormonal mechanisms  c.o maintained within narrow range ( no condition with ↑ SBF )  PBF  no effective neurohormonal control ( PBF-SBF series ) PBF controlled by SBF ( PBF ↓ or ↑ depending on PVR & SVR )  Effective saturation 𝛼 pulmonary blood flow
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 7 Example .1 Ao = 90 % PA =90 % (TAPVC , CA , SV , TA) MV=70% PV = 100 % Qp = 1/ 100- 90 = 10 L/min Qs = 1/90-70 = 5 L/min Qep = 1/100-70 = 3.3 L/min LR shunt = ( 10-3.3 ) = 6.7 L/min RL shunt (5-3.3 ) =1.7 L/min LR > R L PBF = 2 x SBF(Qp/Qs = 2:1 ) roughly PVR = SVR / 2 Inference : Favourable PVR for surgical correction BSA 1.6 m2 V02(BSA X 125 ) = 200 ml/min Hb- 14.75 ) O2 carrying capacity = 14.75x 1.36/10= 200 ml O2 /L blood
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 8 Example .2 Ao = 80 % PA =80 % (TAPVC , CA , SV , TA) MV=60% PV = 100 % Qp = 1/ 100- 80 = 5 L/min Qs = 1/80-60 = 5 L/min Qep = 1/100-60 = 2.5 L/min LR shunt = ( 5-2.5 ) = 2.5 L/min RL shunt (5-2.5 ) =2.5 L/min LR = R L / Bidirectional shunt PBF = SBF (Qp / Qs=1.1 ) roughly PVR = SVR / 2 Inference : unfavourable PVR for surgical correction (caution :↑ vascular resistance – PVR / supravalvular / valvular / subvalvular – clinical & ECHO correlation
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 9 PV -100 % MV- 50 % When Qp/Qs - low  small ↑ PBF ∝ ↑ ↑ Saturation (DDPC ) High Qp/Qs – high  ↑ PBF - not much change in Saturation Increasing PBF  volume overload  CCF 8.5 7 4 2.5
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 10 ADVANTAGES  Simpe pulse oximetry – reasonable assessment of PBF , PVR and operability  Assesment of response to pulmonary vasodilators  since arterial sat ∝ PBF  PVR fall  saturation will improve (significant response  mean PA pressure fall >10 mm to < 40 mm , PVR ↓ > 20%  <5 wood units )
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 11 LIMITATION OF SYSTEMIC SATURATION ∝ SBF Eg .1 Ao- 85 % PA- 85% PV-100 % Normal AV o2 difference 25 % ( assuming normal c.o ) MV = Ao – AV = 60% Qp/Qs = 85-60 / 100-85 = 1.67 : 1 (PBF > SBF ) Eg . 2 Ao- 85 % PA- 85% PV-100 % ↑MV -70 % Qp/Qs= 85-70/100-85 = 1 : 1 (↓ SBF) For identical aortic saturation – change in MV saturation – alters PBF Always measure Systemic venous & systemic arterial saturation
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 12 STREAMING  Inadequate mixing  S ao≠S pa for similar pulmonary blood flow  Favourable(S ao > S pa ) & unfavourable streaming S pa > S ao
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 13 STREAMING (inadequate mixing ) – eg .,TAPVC unfavourable – supracardiac (PV TV RVPA ) S pa > S ao favourable - infracardiac(PV IVCFOLA ) S ao > S pa
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 14 SYSTEMIC SATURATION Pulmonary blood flow Mixed venous saturation Streaming
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 15 NEONATE – MODE OF PRESENTATION Admixture lesions Intense cyanosis & acidosis HF & Pulmonary edema Shock and acidosis HLHS Obstructed TAPVC Large Pul. AV fistula DDPC Pulmonary atresia with intact IVS DDSC HLHS
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 16 CHF -Neonate & infancy SYMPTOMS • Feeding difficulty • Suck-rest-suck cycle (interrupted feeds) • Forehead sweating • Laboured Tachypnea • Intercostal retraction • Difficult to Rx respiratory infection • FTT ( term relevant upto 5 yrs ) SIGNS • Tachycardia • Fast breathing • Presacral & preorbital edema • Cardiomegaly ( most consistent sign ) • Hepatomegaly • Lung crepitations
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 17 CLINICAL FEATURES AND NATURAL COURSE Admixture lesions - ↑ PBF (mild cyanosis) CLINICAL FEATURES • Pre-TRICUSPID (TAPVC, common atrium ) • TAPVC – with obstruction  failure at 1wk PND • Without obstruction – CCF 6mnths – infancy , FTT • Common atrium – mild cyanosis , hf rare NATURAL COURSE Progressive cyanosis ↓ 2-3 yrs – PVD ↓ PVR increases
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 18 CLINICAL FEATURES AND NATURAL COURSE Admixture lesions - ↑ PBF (mild cyanosis) CLINICAL FEATURES Post -TRICUSPID (SV , DORV+sub pul VSD + PS , TRUNCUS ) ↓ CCF early infancy ( 1– 6mnths )  progress to early severe heart failure NATURAL COURSE 2-3 yrs – PVD , PVR increases ↓ symptoms of heart failure improve
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 19 CLINICAL FEATURES AND NATURAL COURSE Admixture lesions - ↓ PBF (DDPC & DDPC) CLINICAL FEATURES DDPC – pulmonary atresia , PA with intact IVS ↓ moderate severe cyanosis at birth DDSC – HLHS ↓ Failure at birth & cardiogenic shock NATURAL COURSE Cyanosis / hypoperfusion worsens with • Closure of ductus ( 4-6 weeks ) • growth of infant
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 20 CLINICAL FEATURES PRETRICUSPID (TAPVC , common atrium ) POST TRICUSPID ( DORV, SV , Truncus, TGA + VSD ) JVP Mean JVP elevated RV dysfunction – both a & v waves can be prominent Mean JVP normal Adult – Mild elevation Precordium Hyperactive precordium / Prominent LPH RV impulse unimpressive Apex Moderate cardiomegaly Mild cardiomegaly Heart sounds P2 loud and palpable, close fixed split s2 Single loud P2 Murmur Usually silent TR murmur Usually silent Truncal valve regurgitation – EDM ECG RVH +RAD RVH +LAD- CA (counterclockwise q in I,aVL RVH +RAD RVH +LAD- DORV + subaortic VSD CXR Prominent MPA MPA often not seen
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 21 DORV  Location of VSD  Relationship of great arteries and VSD  Right ventricular outflow obstruction
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 22 Large unrestricted L R VSD with low PVR cf: post tricuspid shunt physiology –RRTI, early failure infancy Large unrestricted L R VSD with high PVR cf: eisenmenger physiology – ↓ PBF - cyanosis TOF physiology – 85% , cyanotic spells ,squatting , ↓ failure TGA physiology – severe cyanosis , RRTI , early failure infancy
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 23 OVERRIDING STRADDLING-anomalous insertion of chordae tendinae of AV valves
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 24 DORV(subaortic VSD + PS )- TOF type 1. Mild cardiomegaly 2. Upturned rounded apex 3. ↓ pulmonary vascularity 4. Concave MPA DORV(subpul VSD- TGA type 1. Mild cardiomegaly 2. ↑ pulmonary vascularity 3. egg-on-side appearance
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 25 PLAX-Subaortic conus aorto mitral discontinuity , aortic override > 50 % PSAX- side –side AORTA & PA originate al same level - double circle appearance
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 26 DORV –subaortic VSD with PS
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 27 50% 20% 20% 10% TAPVC
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 28 RA, RV, PA, LA, LV, and aortic oxygen saturation will be identical. Complete mixing & N c.o  systemic SpO2 ∝ PBF Or if desaturation  pulmonary vascular disease If PBF ↑ ↑ S Ao - > 90% cyanosis may be missed
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 29 PHYSIOLOGY  PFO/ASD  obligatory shunt  Fetal life – small PBF/ Birth – fall in PVR  ^PBF  ^ RA pressure  congestion  RA – spo2 > 90%  Streaming ( fetal persistence  IVC – PFO-LA , CS-RV )
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 30 Fetal circulation(↑PVR IVC – PFO-LA ) After birth  ↑ SVR  ↑LA pressure – PFO closes TAPVC↑ RA pressure  PFO ↓ STREAMING
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 31 CRAIG( ANATOMIC) BUROUGHS & EDWARDS( LENGTH) SMITH(PV OBSTRUCION) Supracardiac Long Supracardiac (Without Obstruction) Cardiac Intermediate Infra Diaphragmatic With Obstruction Infracardiac & Mixed Short
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 32 WITHOUT OBSTRUCTION • Asymptomatic at birth • Mild cyanosis • CHF -> 6mnths • RA /RV volume overload • Split S2/ RPH/ flow MDM TA • / ESM PA • Vertical vein  venous hum • RAD/ P pulmonale/RBBB • SNOWMAN -> 1st few mnths absent WITH OBSTRUCTION • Supracardiac 50% • Usually manifest >12 hrs after birth • Minimal ausc. findings despite stormy presentation • No murmur ( soft ESM) • RVH+/ no RA enlargement • No cardiomegaly
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 33 1. Pulmonary vascular disease  EISENMENGER 2. PV desaturation Obstruction in venous channel 3. Unfavourable Streaming 4. Passive pulmonary hypertension due to venous HT . SYSTEMIC DESATURATION 1 2 3 4
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 34 SURVIVAL  PFO/ASD  Venous obstruction  inter atrial septum/anomalous channel  Anomalous channelleft MPA and left mainstem bronchus, right pulmonary artery and trachea  Ductus venosus  Length of the vertical vein  Associated anomalies TGA / TOF / TA / TA  Visceral heterotaxy & polysplenia
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 35 TWO ADMIXTURE LESIONS IN TANDEM  TAPVC  complete ECD + Pulmonary atresia in tandem –clinical manifestations dominated by 2nd lesion  This is important when Glenn shunt is planned as TAPVC will interfere with rerouting of SVC to PA
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 36 PAPVC 1. Mild to moderate cardiomegaly 2. Right heart dilatation 3. Dilated SVC /Azygous V.  Rt heart dilatation DD- AIR BRONCHOGRAM • primary pulmonary disease(ARDS) • pneumonia, meconium aspiration SCIMITAR 1. Retrosternal soft tissue density 2. Severe RTlung hypoplasia+ mediastinal shift 3. Elevation of the right hemidiaphragm 4. Rt displacement of the heart + obscuration of right RT border 5. RT PA small or absent 1 3 2 2 3 4
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 37 WITHOUT OBSTRUCTION 1. ↑PBF & Large MPA 2. Moderate cardiomegaly 3. RA + RV enlargement WITH OBSTRUCTION 1. Lateral- pretracheal density (thymus-retrosternal ) 2. Venous hypertension - diffuse reticular pattern (interstitial edema, dilated pulmonary veins, and lymphatics ) 3. Normal MPA Normal heart size(↓PBF ) Small aorta 4. ↑ L.Vol Overinflated lung parenchyma ( ≠ ARDS ) 2 2 3 3
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 38 SUPRASTERNAL NOTCH – vertical vein
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 39 Subcostal BICAVAL VIEW- dilated SVC and R L shunt
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 40 3 pulmonary veins draining into vertical vein
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 41
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 42 CATH DATA
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 43
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 44 DILV – prototype of a single ventricle DDSC/DDPC Complete mixing If , S pv – 100 % , N c.o  systemic saturation α PBF Rough guide  Spo2 >85% - good PBF / <75% low PBF
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 45 FAVOURABLE STREAMING (DILV+L-TGA )– aorta from left & anterior RV (SVPA) UNFAVOURABLE STREAMING (DILV+d’TGA )-aorta from right &anterior RV (SVAo )
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 46 DILV RA LA TV MV LV
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 47 DILV-PLAX
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 48 Tricuspid atresia with dextrocardia Right apical 4 chamber with probe marker towards right L A L V R A RV
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 49 BULBOVENTRICULAR FORAMEN B V F Atretic TV LA L V
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 50 HLHS  Hypoplasia of the LV, atresia or critical stenosis of the aortic or mitral valves, and hypoplasia of the ascending aorta and aortic arch  CoA 75%/ CNS anomalies ( callosum agenesis)  Ductus dependent (Rx – PGE1 / DUCTAL STENTING )  Symptomatic 1st few hrs of life  failure  Sx neonatal period  NORWOOD  FONTAN
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 51 HLHS
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 52 PULMONARY ATRESIA WITH INTACT IVS
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 53 DILV + TGA ↑pul. Vasc. straight left heart border TRICUSPID ATRESIA ↓ pul.vascularity RA +LV enlargement Concave MPA (TOF )
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 54 HLHS + intact IAS Pul. Ven HT with reticular appearance ( obstructed TAPVC ) HLHS + ASD +PDA ↑pul.vasc + cardiomegaly
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 55 PA with INTACT IVS 1. Massive cardiomegaly disproportionate RA enlargement ( TR , dd : EBSTEINS) 2. ASD ( no RA enlargement ) 3. Pul. Vasc. N  ↓ vasc. (closing ductus ) 4. Egg-on-side (dd: TGA ) 5. Prominent aortic arch (↑ C.O ) 1 2 3
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 56 TRUNCUS ARTERIOSUS COLLART EDWARD CLASSIFICATION I - MPA from trunk II – RPA & LPA from posterior aspect III- RPA & LPA from lateral aspect IV- from decsending aorta
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 57 CLINICAL FEATURES PBF Mild cyanosis birth PVR ^ age  overcirculation TR Severe – failure neonatal period
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 58  VSD – infundibular (truncal tricuspid discontinuity) membranous septum intact  Combined infund.& memb. Septum – truncal tricuspid continuity
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 59 OTHER FEATURES  Truncal valve -tricuspid in (69%), quadricuspid (22%), bicuspid (9%), pentacuspid (0.3%), and unicommissural (0.3%)  Right sided aortic arch 35%  Ductus absetnt in ½ pts / size inverse to aorta( A4 – aortic underdeveloped )  LAD –small / CONUS- large – compensatory- RVOT / PDA –LCX  Incompetent truncal valve
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 60 CXR • moderate to severe cardiomegaly • an egg-on-side or boot-shaped appearance • a small thymic shadow (DIGEORGE’S syndrome ) • right aortic arch • prominent pulmonary vascularity • Concave pulmonary segment
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 61 Concave MPA with pulmonary venous congestion – (dd:TGA ) Boot shaped heart with concave MPA with pulmonary plethora (≠TOF)
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 62 TRUNCUS ARTERIOSUS Suprasternal view  interrupted aortic arch , RT aortic arch
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 63 AP window ( no VSD / RVOT & PV normal position) L R shunt ,CCF / large shunt -BD – cyanosis
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 64 DIFFERENTIAL DIAGNOSIS (cyanotic heart diseases  Cyanotic HD with ↓PBF (TOF physiology )  Cyanotic HD with ↑ PBF (Transpostion physiology)  Eisenmenger physiology  Post palliation for cyanotic HD ( BDG / non functioning aorto pulmonary shunt )
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 65 TOF PHYSIOLOGY TRANSPOSITIO N EISENMENGER (ASD/VSD/PDA) POST PALLIATION (BDG/ blocked AP shunt ) ADMIXTURE Mild cyanosis , 1 wk after birth HF rare Mod-sev cyanosis at birth , hf infancy Cyanosis 4th/3rd/2nd decade Hf 5th-6th decade BDG- cyanosis persist (80-85%) blocked AP shunt – worsening cyanosis Mild cyanosis Hf late infancy Progressive cyanosis ( ↓2 yrs symptoms may improve ) Hf symptoms ↓2yrs Cyanosis worsens PVR ↑ - PVD similar course BDG- symptoms of SV improve Blocked AP - similar course N JVP No cardiomegaly Prominent a Mild cardiomegaly Prominent a ASD- cardiomegaly VSD/PDA-no BDG- non pulsatile JVP Prom a , TR-V Mild cardiomegaly P2 single & loud Mild –ESM / severe – no mumur P2 single & loud No murmur ASD – P2 loud and close split VSD&PDA- systolic murmur with loud P2 TR murmur P2 single & loud No mumur P2 single & loud TR murmur AV valve regurgitant murmur MPA segment Inconspicous (branch PA’s small) MPA –not seen Prominent central PA Variable Prominent central PA (pretricuspid ) Post tricuspid- MPA- not seen
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 66 APPROACH TO A PATIENT WITH SUSPECTED ADMXITURE LESION cyanosis ≤ 85% + N-↑PBF ) At birth : HF (HLHS,obstr.TAPVC) / intense cyanosis(DDPC) / shock(DDSC ) Mild cyanosis  progressive cyanosis failure(late infancy )  PVD Mild cyanosis  early infancy CCF  hf ↓ - PVR ↑ (2yrs )  PVD ↑JVP , hyperactive precordium , loud & split P2 ↔JVP , silent precordium , single loud P2 PRETRICUSPID – TAPVC ,CA POSTTRICUSPID- SV , DORV , Truncus CXR Cardiomegaly MPA / LPA /RPA RA/RV/LV P.Venous HT Lung NO – obstr. TAPVC ,HLHS MILD- PAPVC , DORV-TOF, HLHS+ASD+PDA MOD- TAPVC , Truncus SEVERE – PA with IVS Concave – DORV , Tr atresia Absent RPA-SCIMITAR Prominent – TAPVC (no obstr. ) , PAPVC RA,RV-TAPVC , Truncus , PA IVS Straight LT border- DILV + LTGA Obstr.TAPVC HLHS Hypoplasia- SCIMITAR Overinflated – obstr.TAPVC N- ARDS, HMD
© 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 67 TAPVC New born – palliation – septostomy Obstructive – immediate corrective Sx Non obstructive (3-6 mnths ) S-S venous confluence  LA SV(HLHS/ DILV/TA ) Neonatal – Norwood / BT / banding/ ductal stenting BDG (3-6 mnths ) FONTAN 2-4 yrs DORV DORVTOF- BT  VSD-Ao tunnel+Rastelli /REV /Nikaidoh TGA –VSD-PA tunnel +ASO(3mnths ) TRUNCUS Complete repair 6 wks- 6mnths MPA – transection + VSD patch closure + RV –PA homograft /conduit (PEEL procedure – autologous tissue ) THANK U

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  • 1. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 1 KAPIL R DEPT OF CARDIOLOGY ADMIXTURE LESIONS
  • 2. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 2 CONGENITAL HEART DISEASES Anatomico-pathologic classification (↑ /↓PBF) Physiological classification (transposition/TOF/Admixture/ Eisenmenger / ductus dependent )
  • 3. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 3 PHYSIOLOGY TOF physiology Transposition physiology Admixture physiology Ductus dependent physiology Eisenmenger physiology Normal physiology TOF DORV+subaortic VSD +PS TA + PS SV+PS TGA+VSD +PS d-TGA DORV +sub pul VSD TA +TGA +VSD Shunt ASD/VSD/PDA ↑PVR  BD or flow reversal Pre TR-TAPVC Post TR-SV DDSC-HLHS DDPC-Pul.Atresia Pul. AV fistula Mild cyanosis Spells/squatting FTT Severe cyanosis at birth Early CCF Suck-rest-suck cycle RRTI  cyanosis (2/3/4 decade) Mild cyanosis RRTI Early CCF Intense cyanosis Cardiogenic shock Mod-severe cyanosis RRTI Early CCF
  • 4. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 4 ADMIXTURE - DEFINTION AND GENERAL FEATURES • Complete mixing of deoxygenated blood (SV) + oxygenated blood from pulmonary veins (atria / ventricle / great artery ) • Cyanosis ~ 85 % • S aorta = S pul.art • RRTI , FTT / CHF infancy • Cardiomegaly with hyperdynamic heart • Evidence of PAH • Abundant murmurs – systolic and diastolic
  • 5. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 5 LEVEL OF MIXING • Systemic Veins-supracardiac TAPVC • Atria- common atrium ,mitral atresia , tricuspid atresia • Ventricles – SV , DORV , TGA with large VSD • Great Vessels (Truncus arteriosus ) • DDSC- HLHS with aortic atresia • DDPC- Pulmonary atresia with intact IVS • Distal Pulmonary Vascular Bed (Pul. AV fistula)
  • 6. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 6 IMPORTANT BASIC CONCEPTS  Systemic blood flow  neurohormonal mechanisms  c.o maintained within narrow range ( no condition with ↑ SBF )  PBF  no effective neurohormonal control ( PBF-SBF series ) PBF controlled by SBF ( PBF ↓ or ↑ depending on PVR & SVR )  Effective saturation 𝛼 pulmonary blood flow
  • 7. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 7 Example .1 Ao = 90 % PA =90 % (TAPVC , CA , SV , TA) MV=70% PV = 100 % Qp = 1/ 100- 90 = 10 L/min Qs = 1/90-70 = 5 L/min Qep = 1/100-70 = 3.3 L/min LR shunt = ( 10-3.3 ) = 6.7 L/min RL shunt (5-3.3 ) =1.7 L/min LR > R L PBF = 2 x SBF(Qp/Qs = 2:1 ) roughly PVR = SVR / 2 Inference : Favourable PVR for surgical correction BSA 1.6 m2 V02(BSA X 125 ) = 200 ml/min Hb- 14.75 ) O2 carrying capacity = 14.75x 1.36/10= 200 ml O2 /L blood
  • 8. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 8 Example .2 Ao = 80 % PA =80 % (TAPVC , CA , SV , TA) MV=60% PV = 100 % Qp = 1/ 100- 80 = 5 L/min Qs = 1/80-60 = 5 L/min Qep = 1/100-60 = 2.5 L/min LR shunt = ( 5-2.5 ) = 2.5 L/min RL shunt (5-2.5 ) =2.5 L/min LR = R L / Bidirectional shunt PBF = SBF (Qp / Qs=1.1 ) roughly PVR = SVR / 2 Inference : unfavourable PVR for surgical correction (caution :↑ vascular resistance – PVR / supravalvular / valvular / subvalvular – clinical & ECHO correlation
  • 9. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 9 PV -100 % MV- 50 % When Qp/Qs - low  small ↑ PBF ∝ ↑ ↑ Saturation (DDPC ) High Qp/Qs – high  ↑ PBF - not much change in Saturation Increasing PBF  volume overload  CCF 8.5 7 4 2.5
  • 10. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 10 ADVANTAGES  Simpe pulse oximetry – reasonable assessment of PBF , PVR and operability  Assesment of response to pulmonary vasodilators  since arterial sat ∝ PBF  PVR fall  saturation will improve (significant response  mean PA pressure fall >10 mm to < 40 mm , PVR ↓ > 20%  <5 wood units )
  • 11. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 11 LIMITATION OF SYSTEMIC SATURATION ∝ SBF Eg .1 Ao- 85 % PA- 85% PV-100 % Normal AV o2 difference 25 % ( assuming normal c.o ) MV = Ao – AV = 60% Qp/Qs = 85-60 / 100-85 = 1.67 : 1 (PBF > SBF ) Eg . 2 Ao- 85 % PA- 85% PV-100 % ↑MV -70 % Qp/Qs= 85-70/100-85 = 1 : 1 (↓ SBF) For identical aortic saturation – change in MV saturation – alters PBF Always measure Systemic venous & systemic arterial saturation
  • 12. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 12 STREAMING  Inadequate mixing  S ao≠S pa for similar pulmonary blood flow  Favourable(S ao > S pa ) & unfavourable streaming S pa > S ao
  • 13. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 13 STREAMING (inadequate mixing ) – eg .,TAPVC unfavourable – supracardiac (PV TV RVPA ) S pa > S ao favourable - infracardiac(PV IVCFOLA ) S ao > S pa
  • 14. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 14 SYSTEMIC SATURATION Pulmonary blood flow Mixed venous saturation Streaming
  • 15. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 15 NEONATE – MODE OF PRESENTATION Admixture lesions Intense cyanosis & acidosis HF & Pulmonary edema Shock and acidosis HLHS Obstructed TAPVC Large Pul. AV fistula DDPC Pulmonary atresia with intact IVS DDSC HLHS
  • 16. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 16 CHF -Neonate & infancy SYMPTOMS • Feeding difficulty • Suck-rest-suck cycle (interrupted feeds) • Forehead sweating • Laboured Tachypnea • Intercostal retraction • Difficult to Rx respiratory infection • FTT ( term relevant upto 5 yrs ) SIGNS • Tachycardia • Fast breathing • Presacral & preorbital edema • Cardiomegaly ( most consistent sign ) • Hepatomegaly • Lung crepitations
  • 17. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 17 CLINICAL FEATURES AND NATURAL COURSE Admixture lesions - ↑ PBF (mild cyanosis) CLINICAL FEATURES • Pre-TRICUSPID (TAPVC, common atrium ) • TAPVC – with obstruction  failure at 1wk PND • Without obstruction – CCF 6mnths – infancy , FTT • Common atrium – mild cyanosis , hf rare NATURAL COURSE Progressive cyanosis ↓ 2-3 yrs – PVD ↓ PVR increases
  • 18. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 18 CLINICAL FEATURES AND NATURAL COURSE Admixture lesions - ↑ PBF (mild cyanosis) CLINICAL FEATURES Post -TRICUSPID (SV , DORV+sub pul VSD + PS , TRUNCUS ) ↓ CCF early infancy ( 1– 6mnths )  progress to early severe heart failure NATURAL COURSE 2-3 yrs – PVD , PVR increases ↓ symptoms of heart failure improve
  • 19. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 19 CLINICAL FEATURES AND NATURAL COURSE Admixture lesions - ↓ PBF (DDPC & DDPC) CLINICAL FEATURES DDPC – pulmonary atresia , PA with intact IVS ↓ moderate severe cyanosis at birth DDSC – HLHS ↓ Failure at birth & cardiogenic shock NATURAL COURSE Cyanosis / hypoperfusion worsens with • Closure of ductus ( 4-6 weeks ) • growth of infant
  • 20. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 20 CLINICAL FEATURES PRETRICUSPID (TAPVC , common atrium ) POST TRICUSPID ( DORV, SV , Truncus, TGA + VSD ) JVP Mean JVP elevated RV dysfunction – both a & v waves can be prominent Mean JVP normal Adult – Mild elevation Precordium Hyperactive precordium / Prominent LPH RV impulse unimpressive Apex Moderate cardiomegaly Mild cardiomegaly Heart sounds P2 loud and palpable, close fixed split s2 Single loud P2 Murmur Usually silent TR murmur Usually silent Truncal valve regurgitation – EDM ECG RVH +RAD RVH +LAD- CA (counterclockwise q in I,aVL RVH +RAD RVH +LAD- DORV + subaortic VSD CXR Prominent MPA MPA often not seen
  • 21. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 21 DORV  Location of VSD  Relationship of great arteries and VSD  Right ventricular outflow obstruction
  • 22. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 22 Large unrestricted L R VSD with low PVR cf: post tricuspid shunt physiology –RRTI, early failure infancy Large unrestricted L R VSD with high PVR cf: eisenmenger physiology – ↓ PBF - cyanosis TOF physiology – 85% , cyanotic spells ,squatting , ↓ failure TGA physiology – severe cyanosis , RRTI , early failure infancy
  • 23. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 23 OVERRIDING STRADDLING-anomalous insertion of chordae tendinae of AV valves
  • 24. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 24 DORV(subaortic VSD + PS )- TOF type 1. Mild cardiomegaly 2. Upturned rounded apex 3. ↓ pulmonary vascularity 4. Concave MPA DORV(subpul VSD- TGA type 1. Mild cardiomegaly 2. ↑ pulmonary vascularity 3. egg-on-side appearance
  • 25. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 25 PLAX-Subaortic conus aorto mitral discontinuity , aortic override > 50 % PSAX- side –side AORTA & PA originate al same level - double circle appearance
  • 26. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 26 DORV –subaortic VSD with PS
  • 27. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 27 50% 20% 20% 10% TAPVC
  • 28. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 28 RA, RV, PA, LA, LV, and aortic oxygen saturation will be identical. Complete mixing & N c.o  systemic SpO2 ∝ PBF Or if desaturation  pulmonary vascular disease If PBF ↑ ↑ S Ao - > 90% cyanosis may be missed
  • 29. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 29 PHYSIOLOGY  PFO/ASD  obligatory shunt  Fetal life – small PBF/ Birth – fall in PVR  ^PBF  ^ RA pressure  congestion  RA – spo2 > 90%  Streaming ( fetal persistence  IVC – PFO-LA , CS-RV )
  • 30. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 30 Fetal circulation(↑PVR IVC – PFO-LA ) After birth  ↑ SVR  ↑LA pressure – PFO closes TAPVC↑ RA pressure  PFO ↓ STREAMING
  • 31. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 31 CRAIG( ANATOMIC) BUROUGHS & EDWARDS( LENGTH) SMITH(PV OBSTRUCION) Supracardiac Long Supracardiac (Without Obstruction) Cardiac Intermediate Infra Diaphragmatic With Obstruction Infracardiac & Mixed Short
  • 32. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 32 WITHOUT OBSTRUCTION • Asymptomatic at birth • Mild cyanosis • CHF -> 6mnths • RA /RV volume overload • Split S2/ RPH/ flow MDM TA • / ESM PA • Vertical vein  venous hum • RAD/ P pulmonale/RBBB • SNOWMAN -> 1st few mnths absent WITH OBSTRUCTION • Supracardiac 50% • Usually manifest >12 hrs after birth • Minimal ausc. findings despite stormy presentation • No murmur ( soft ESM) • RVH+/ no RA enlargement • No cardiomegaly
  • 33. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 33 1. Pulmonary vascular disease  EISENMENGER 2. PV desaturation Obstruction in venous channel 3. Unfavourable Streaming 4. Passive pulmonary hypertension due to venous HT . SYSTEMIC DESATURATION 1 2 3 4
  • 34. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 34 SURVIVAL  PFO/ASD  Venous obstruction  inter atrial septum/anomalous channel  Anomalous channelleft MPA and left mainstem bronchus, right pulmonary artery and trachea  Ductus venosus  Length of the vertical vein  Associated anomalies TGA / TOF / TA / TA  Visceral heterotaxy & polysplenia
  • 35. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 35 TWO ADMIXTURE LESIONS IN TANDEM  TAPVC  complete ECD + Pulmonary atresia in tandem –clinical manifestations dominated by 2nd lesion  This is important when Glenn shunt is planned as TAPVC will interfere with rerouting of SVC to PA
  • 36. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 36 PAPVC 1. Mild to moderate cardiomegaly 2. Right heart dilatation 3. Dilated SVC /Azygous V.  Rt heart dilatation DD- AIR BRONCHOGRAM • primary pulmonary disease(ARDS) • pneumonia, meconium aspiration SCIMITAR 1. Retrosternal soft tissue density 2. Severe RTlung hypoplasia+ mediastinal shift 3. Elevation of the right hemidiaphragm 4. Rt displacement of the heart + obscuration of right RT border 5. RT PA small or absent 1 3 2 2 3 4
  • 37. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 37 WITHOUT OBSTRUCTION 1. ↑PBF & Large MPA 2. Moderate cardiomegaly 3. RA + RV enlargement WITH OBSTRUCTION 1. Lateral- pretracheal density (thymus-retrosternal ) 2. Venous hypertension - diffuse reticular pattern (interstitial edema, dilated pulmonary veins, and lymphatics ) 3. Normal MPA Normal heart size(↓PBF ) Small aorta 4. ↑ L.Vol Overinflated lung parenchyma ( ≠ ARDS ) 2 2 3 3
  • 38. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 38 SUPRASTERNAL NOTCH – vertical vein
  • 39. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 39 Subcostal BICAVAL VIEW- dilated SVC and R L shunt
  • 40. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 40 3 pulmonary veins draining into vertical vein
  • 41. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 41
  • 42. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 42 CATH DATA
  • 43. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 43
  • 44. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 44 DILV – prototype of a single ventricle DDSC/DDPC Complete mixing If , S pv – 100 % , N c.o  systemic saturation α PBF Rough guide  Spo2 >85% - good PBF / <75% low PBF
  • 45. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 45 FAVOURABLE STREAMING (DILV+L-TGA )– aorta from left & anterior RV (SVPA) UNFAVOURABLE STREAMING (DILV+d’TGA )-aorta from right &anterior RV (SVAo )
  • 46. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 46 DILV RA LA TV MV LV
  • 47. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 47 DILV-PLAX
  • 48. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 48 Tricuspid atresia with dextrocardia Right apical 4 chamber with probe marker towards right L A L V R A RV
  • 49. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 49 BULBOVENTRICULAR FORAMEN B V F Atretic TV LA L V
  • 50. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 50 HLHS  Hypoplasia of the LV, atresia or critical stenosis of the aortic or mitral valves, and hypoplasia of the ascending aorta and aortic arch  CoA 75%/ CNS anomalies ( callosum agenesis)  Ductus dependent (Rx – PGE1 / DUCTAL STENTING )  Symptomatic 1st few hrs of life  failure  Sx neonatal period  NORWOOD  FONTAN
  • 51. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 51 HLHS
  • 52. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 52 PULMONARY ATRESIA WITH INTACT IVS
  • 53. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 53 DILV + TGA ↑pul. Vasc. straight left heart border TRICUSPID ATRESIA ↓ pul.vascularity RA +LV enlargement Concave MPA (TOF )
  • 54. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 54 HLHS + intact IAS Pul. Ven HT with reticular appearance ( obstructed TAPVC ) HLHS + ASD +PDA ↑pul.vasc + cardiomegaly
  • 55. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 55 PA with INTACT IVS 1. Massive cardiomegaly disproportionate RA enlargement ( TR , dd : EBSTEINS) 2. ASD ( no RA enlargement ) 3. Pul. Vasc. N  ↓ vasc. (closing ductus ) 4. Egg-on-side (dd: TGA ) 5. Prominent aortic arch (↑ C.O ) 1 2 3
  • 56. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 56 TRUNCUS ARTERIOSUS COLLART EDWARD CLASSIFICATION I - MPA from trunk II – RPA & LPA from posterior aspect III- RPA & LPA from lateral aspect IV- from decsending aorta
  • 57. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 57 CLINICAL FEATURES PBF Mild cyanosis birth PVR ^ age  overcirculation TR Severe – failure neonatal period
  • 58. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 58  VSD – infundibular (truncal tricuspid discontinuity) membranous septum intact  Combined infund.& memb. Septum – truncal tricuspid continuity
  • 59. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 59 OTHER FEATURES  Truncal valve -tricuspid in (69%), quadricuspid (22%), bicuspid (9%), pentacuspid (0.3%), and unicommissural (0.3%)  Right sided aortic arch 35%  Ductus absetnt in ½ pts / size inverse to aorta( A4 – aortic underdeveloped )  LAD –small / CONUS- large – compensatory- RVOT / PDA –LCX  Incompetent truncal valve
  • 60. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 60 CXR • moderate to severe cardiomegaly • an egg-on-side or boot-shaped appearance • a small thymic shadow (DIGEORGE’S syndrome ) • right aortic arch • prominent pulmonary vascularity • Concave pulmonary segment
  • 61. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 61 Concave MPA with pulmonary venous congestion – (dd:TGA ) Boot shaped heart with concave MPA with pulmonary plethora (≠TOF)
  • 62. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 62 TRUNCUS ARTERIOSUS Suprasternal view  interrupted aortic arch , RT aortic arch
  • 63. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 63 AP window ( no VSD / RVOT & PV normal position) L R shunt ,CCF / large shunt -BD – cyanosis
  • 64. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 64 DIFFERENTIAL DIAGNOSIS (cyanotic heart diseases  Cyanotic HD with ↓PBF (TOF physiology )  Cyanotic HD with ↑ PBF (Transpostion physiology)  Eisenmenger physiology  Post palliation for cyanotic HD ( BDG / non functioning aorto pulmonary shunt )
  • 65. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 65 TOF PHYSIOLOGY TRANSPOSITIO N EISENMENGER (ASD/VSD/PDA) POST PALLIATION (BDG/ blocked AP shunt ) ADMIXTURE Mild cyanosis , 1 wk after birth HF rare Mod-sev cyanosis at birth , hf infancy Cyanosis 4th/3rd/2nd decade Hf 5th-6th decade BDG- cyanosis persist (80-85%) blocked AP shunt – worsening cyanosis Mild cyanosis Hf late infancy Progressive cyanosis ( ↓2 yrs symptoms may improve ) Hf symptoms ↓2yrs Cyanosis worsens PVR ↑ - PVD similar course BDG- symptoms of SV improve Blocked AP - similar course N JVP No cardiomegaly Prominent a Mild cardiomegaly Prominent a ASD- cardiomegaly VSD/PDA-no BDG- non pulsatile JVP Prom a , TR-V Mild cardiomegaly P2 single & loud Mild –ESM / severe – no mumur P2 single & loud No murmur ASD – P2 loud and close split VSD&PDA- systolic murmur with loud P2 TR murmur P2 single & loud No mumur P2 single & loud TR murmur AV valve regurgitant murmur MPA segment Inconspicous (branch PA’s small) MPA –not seen Prominent central PA Variable Prominent central PA (pretricuspid ) Post tricuspid- MPA- not seen
  • 66. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 66 APPROACH TO A PATIENT WITH SUSPECTED ADMXITURE LESION cyanosis ≤ 85% + N-↑PBF ) At birth : HF (HLHS,obstr.TAPVC) / intense cyanosis(DDPC) / shock(DDSC ) Mild cyanosis  progressive cyanosis failure(late infancy )  PVD Mild cyanosis  early infancy CCF  hf ↓ - PVR ↑ (2yrs )  PVD ↑JVP , hyperactive precordium , loud & split P2 ↔JVP , silent precordium , single loud P2 PRETRICUSPID – TAPVC ,CA POSTTRICUSPID- SV , DORV , Truncus CXR Cardiomegaly MPA / LPA /RPA RA/RV/LV P.Venous HT Lung NO – obstr. TAPVC ,HLHS MILD- PAPVC , DORV-TOF, HLHS+ASD+PDA MOD- TAPVC , Truncus SEVERE – PA with IVS Concave – DORV , Tr atresia Absent RPA-SCIMITAR Prominent – TAPVC (no obstr. ) , PAPVC RA,RV-TAPVC , Truncus , PA IVS Straight LT border- DILV + LTGA Obstr.TAPVC HLHS Hypoplasia- SCIMITAR Overinflated – obstr.TAPVC N- ARDS, HMD
  • 67. © 2003 By Default! A Free sample background from www.awesomebackgrounds.com Slide 67 TAPVC New born – palliation – septostomy Obstructive – immediate corrective Sx Non obstructive (3-6 mnths ) S-S venous confluence  LA SV(HLHS/ DILV/TA ) Neonatal – Norwood / BT / banding/ ductal stenting BDG (3-6 mnths ) FONTAN 2-4 yrs DORV DORVTOF- BT  VSD-Ao tunnel+Rastelli /REV /Nikaidoh TGA –VSD-PA tunnel +ASO(3mnths ) TRUNCUS Complete repair 6 wks- 6mnths MPA – transection + VSD patch closure + RV –PA homograft /conduit (PEEL procedure – autologous tissue ) THANK U

Editor's Notes

  • #3: Anatomico-pathologic- 5T’S / 2T