Algorithmic Approach To Congenital Heart Disease

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Published on January 31, 2010

Author: praveenks

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Slide 1: Thiruvananthapuram GREETINGS F r o m An Algorithmic Approach To Congenital Heart Disease in The Neonate : An Algorithmic Approach To Congenital Heart Disease in The Neonate M.Zulfikar Ahamed Professor and HOD , Pediatric Cardiology Govt. Medical College, Thiruvananthapuram Kerala Why is Newborn Dear to Us ? : Why is Newborn Dear to Us ? Because He (She) Heralds Dawn of a New Generation Why Are Newborns Precious To Us ? : Why Are Newborns Precious To Us ? U5MR CMR IMR NeoMR India 95 18 58 40 Kerala 18 4 12 8 Slide 5: NEONATAL SURVIVAL & IMR IMR IN KERALA - 12 /1000 8 4 Neonatal Death - Causes / CHD no. 311 SATH : Neonatal Death - Causes / CHD no. 311 SATH Number CHD % Overall 311 38 12.2 Early Neo 258 29 11.2 Late Neo 53 9 16.9 CHD : CHD Beyond 1 Year In Infancy Neonatal Post Neonatal Slide 8: Prevalence of CHD : 6-8 /1000 Live births Critical CHD : 3 /1000 Live births Majority of Critical CHD : In Newborn NEWBORN WITH CHD Why Should Neonatologist (Pediatrician ) be interested in CHD in Neonate ? : Why Should Neonatologist (Pediatrician ) be interested in CHD in Neonate ? Critical CHD manifest Primarily in Newborn CHD is a major component of Neonatal Mortality Three of major manifestations of CHD - Cyanosis CHF and Shock can also be non cardiac in origin Slide 10: NMR accounts for 2/3 of IMR WHY DO WE NEED A DIAGNOSTIC ALGORITHM FOR NEWBORN CHD ? CHD accounts for 15- 20 % IMR 1/3 of CHD are critical Majority of Critical CHD Present in Newborn Early Diagnosis favors Better Survival Neonatal CHD : Neonatal CHD Defects Behavior Prognosis Therapeutics Outcome Differ from CHD at other ages THREE THINGS HAPPEN IN THE NEONATE : THREE THINGS HAPPEN IN THE NEONATE There is a transitional circulation in series Ductal closure Fall in Pulmonary Vascular Resistance NEWBORN CHD PRESENTS if there is : NEWBORN CHD PRESENTS if there is Unfavorable transitional circulation - Parallel instead of series Obstruction of series circulation and - PDA closes Fall in PVR and - Shunting & Mixing occurs Slide 14: DAYS PATHOPHYSIOLOGY SITUATIONS CHD in NEONATE 0-3 Transitional Circulation Parallel circulation Critical Obstruction 3-14 PDA Closes Obstruction SBF Obstruction PBF Fall in PVR Pulmonary Edema 14-28 Fall in PVR Pulmonary Edema Slide 15: Parallel Circulation : d –TGA Obstruction Left : HLHS. AS. CoA. IAAS . Obstr. TAPVC Right : HRHS. TOF c PA. PS . Ebstein Symptomatic Life threatening CHD 0-3 Days Slide 16: a. Obstructed systemic Flow : AS. CoA. b. Obstructed Pulmonary Flow : TOF. TOF c PA TA. SV c PS c. Pulmonary Edema : TAPVC. Truncus SV. PDA. CHD 4-14 Days Slide 17: L R : VSD. PDA. AVSD. AP window Admixture - TAPVC . SV. DORV Obstructive - AS . CoA Others - ALCAPA . Pompe. EFE 1. PVR Falls CHD 14-28 Days Slide 18: PDA Closes Admixture lesions AVSD Large Post Tricuspid shunts Admixture AVSD Alcapa Pompe . EFE Obstr .TAPVC HLHS CoA IAAS AS TGA PA tr TA PS PVR Falls // l 1 2 3 4 wks Slide 19: 1 2 3 4 weeks Shock CHF Cyanosis TIMING of PRESENTATION TOP 5 CHD in NEWBORN (NERICP) : TOP 5 CHD in NEWBORN (NERICP) d- TGA HLHS TOF CoA VSD Others CoAVSD HLHS d-TGA Others VSD TOF CoA d-TGA PDA Others 0-7D 7-14D 14-28D Slide 21: d-TGA TOF Cyanotic Stenotic Shunt TOP CHD in NEWBORN HLHS CoA VSD PDA Slide 22: Deliveries : 8786 CHD : 110 Percentage : 1.2 90 % picked up : < 1 wk Critical CHD : 16 ( 14.5%) CHD in NEWBORNSAT Experience2002 - 2004 Slide 23: Prevalence in NB : 1.2 / 1000 Critical CHD : 16 ( 14.5 %) CHD in IBN Slide 24: Cyanotic TOP IO CHD in NEONATE ASD VSD PDA CoA Others d- TGA TOF TA SV Others Acyanotic Slide 25: Symptomatic Asymptomatic NEWBORN with CHD CHF Shock Cyanosis Arrhythmia Murmur No Murmur CHD IN NEONATE : CHD IN NEONATE CHF CYANOSIS SHOCK Arrhythmia Murmur CHD and SHOCK : CHD and SHOCK Low Systemic output HLHS IAAS Severe AS CoA Obstructed TAPVC 3 days old male baby. FTND .3.0 kg Suddenly became bad. Hypo perfusion & Hypotension : 3 days old male baby. FTND .3.0 kg Suddenly became bad. Hypo perfusion & Hypotension Possibilities HLHS Aortic Atresia CoA Critical AS Hypotension; Hypo perfusionCardiomegaly. PAH : Hypotension; Hypo perfusionCardiomegaly. PAH All pulses weak : HLHS . A atresia. AS Radials Ok, Femorals Not palpable : CoA Slide 30: NEWBORN WITH CHF CHF CHF CYANOSIS No Cyanosis Cyanosis chf Presentation Eg: Large VSD Eg: Single Ventricle Eg: d-TGA Pulse asymmetry CVS Findings CHF IN NEWBORN : CHF IN NEWBORN Tachypnea Tachycardia Hepatomegaly Cardiomegaly S3 gallop Crepn Wheeze Capillary Refill Sweating Edema JVP ? CYANOSIS : CYANOSIS Environment dependent The physician Lighting Colour of baby Wall painting Temperature of room Most difficult of all signs in newborn ! Slide 33: 1st week > 1 week D TGA TOF TGA Pulmonary Atresia Admixture lesions Tricuspid atresia TAPVC Ebstein SV Critical PS DORV Truncus NEWBORN WITH CYANOSIS IVSVSD Slide 34: NEWBORN CYANOSIS 35% of CCHD in childhood 22 29 13 Slide 35: NEWBORN CYANOSIS Within 24 hours n - 22 9 7 2 2 1 1 Slide 36: NB CCHD 1 - 7 days 16 5 3 1 4 Slide 37: NB CCHD 7 – 28 days CYANOSIS & CHF : CYANOSIS & CHF CYANOSIS & chf - TGA Complexes Obstructed TAPVC PPHN HLHS CHF + Cyanosis - Admixture Lesions TAPVC Single ventricle Truncus TGA with VSD Large with L R shunts ? PULSE ASYMMETRY : PULSE ASYMMETRY Coarctation IAAS Isolated Complex CoA WHEEZE / STRIDOR Absent PV syndrome Vascular Ring Large L - R Shunt CHD - NEWBORN - PULSES : CHD - NEWBORN - PULSES Upper limb Lower limb Anomalies ++ ++ Normal CCHD; No collaterals L - R shunt , PS +++ +++ PDA . AP window CCHD with collat / PDA Truncus + + AS . HLHS +++ + Coarctation IAAS ABNORMAL RHYTHM & CHD : ABNORMAL RHYTHM & CHD L- TGA - Heart Block ; SVT Ebstein - SVT Newborn and Murmur : Newborn and Murmur Neonate with critical CHD No murmur Neonate with murmur No critical CHD Murmur no critical CHD Critical CHD no murmur Newborn ASYMPTOMATIC MURMUR : ASYMPTOMATIC MURMUR Mostly Benign Mild PS Mild AS Tiny VSD Small PDA TR murmur ( transient RV Ischemia ) ‘Potentially Malignant’ PDA m in a complex CHD m of AS in severe CoA LOUD MURMUR IN NEWBORN : LOUD MURMUR IN NEWBORN Absent pulmonary Valve syndrome Ebstein Anomaly Congenital MR Loud murmurs are in Neonate RARE Slide 45: The Case of The ‘Missing Murmur’ in the newborn Delayed presentation Significant VSD ; PDA Large ASD ALCAPA AVSD Slide 46: EVALUATION OF CHD IN NEONATE History Examination CXR chest ECG S PO 2/ ABG Non Definitive Definitive Echo cardiography Cath + Angiography MRI Slide 47: DIAGNOSTIC PATHWAYCHD in NEWBORN Clinical Diagnosis Chest X - ray ; ECG Echo ( + Fetal Echo) [MRI – Cath Angio – Nuclear Imaging] NEONATAL CHD : CVS Examination Chest XRAY Electrocardiogram Hyperoxia test Echocardiography Relative merits of NEONATAL CHD Slide 49: “Infant … no language but a cry” A. Tennyson HISTORY Slide 50: NB WITH CHD Pregnancy Family history Perinatal history Chronology of Presentation History CHF Cyanosis ? Slide 51: 1. Color / Cyanosis 2. Perfusion 3. Respiration 4. Edema 5. Pulse 6. JVP Physical Examination NEWBORN with CHD 7. BP 8. Precordium 9. Apex 10. Liver 11. Heart sounds; S2 12. Murmur Slide 52: PHYSICAL EXAMINATION Edema uncommon; ? Turner Pulse crucial Apex and Liver : Malposition ? Flush method : Not useful S1 Normally single S2 Single at birth Split at 24 hrs (50%) Split at 48 hrs (80%) POINTS TO PONDER Slide 53: Acrocyanosis ?! Persistent Cyanosis CYANOSIS IN NEWBORN Transient Cyanosis on cry R L shunt PFO PDA CCHD Respiratory OR CYANOSIS IN NEWBORN : CYANOSIS IN NEWBORN Cyanosis - Is it present ? If so Why ? Is it Cardiac or Respiratory ? ‘ It is all Greek to Me ” “Kaunosis” Cyanosis ( Ancient Greek) (Modern English) Slide 55: Variable clinical assessment Physicians often disagree on Cyanosis Even if they agree, SPO2 varies ! IS THE BABY BLUE Or NOT ? Cyanosis picked up at saturation < 80 -85 % Saturation < 95 means CHD Is there Cyanosis ? : Is there Cyanosis ? Clinical Assessment & Pulse Oxymetry Cyanosis S po2 < 90% CCHD Slide 57: CHD NEONATAL CYANOSIS Cause ? RESPIRATORY Lung : MAS. RDS. Pneumonia Airway : choanal atresia; Web Compr of Lung : CLE .DH Hypoventilation : Sepsis . CNS PPHN Slide 58: Heat rate Respiratory rate Grunt Murmur CHF Abnormal Pulses Response to O2 CARDIAC RESPIRATORY Faster Fast Not Present Prominent Present Yes Not much Fast Faster Present Can be present Less likely No Good Slide 59: HYPEROXIA TEST Give 100 % Oxygen Assess PO2 PO2 > 200 mm HG PO2 < 150 mm Hg No CCHD LIKELY CCHD Pass Fail 150 - 200 ? CCHD c PBF ; PPHN Slide 60: HYPEROXIA TEST Administer 100% Oxygen for 15 minutes Assess oxygenation. Upper limb. Lower limb How ? ABG PO TCMO Slide 61: HYPEROXIA TEST PO2 (Jones) > 250 - No SH < 150 - Likely CCHD > 150 - Unlikely CCHD) Slide 62: Cyanotic Neonate ? Cardiac ? Respiratory Hyperoxia test PO2 < 150mmHg Po2 > 200 mmHg Respiratory PPHN CCHD CXR ECG Echocardiography ABG - Radial / Femoral + Contrast Echo + D I A G N O S I S Slide 63: CCHD in NeonateSATH Exp d-TGA TOF/PA TOF UVH TAPVC TRUNCUS 32 18 24 3 5 2 Slide 64: A 5 days old baby. Male . 3.2 kg Blue color noticed at home on crying . Feeding - ok Brought to Pediatric consultant 5 Days old baby with CCHD : 5 Days old baby with CCHD Not much CHF CHF 3 Major Possibilities D- TGA Pulmonary Atresia. VSD Tricuspid Atresia CCHD ; High Volume Pulse : CCHD ; High Volume Pulse Think of Ductus Dep. CCHD PA. VSD PA. IVS d-TGA Or Truncus Arteriosus BLUE BABY 5 DAYS ; NOT MUCH CHF : BLUE BABY 5 DAYS ; NOT MUCH CHF Mild CE. Not much murmur TGA No CE. High Volume Pulse. No murmur PA.VSD No CE. N Volume pulse. Murmur TA TOF also can present by 5 days Slide 69: d -TGA TRICUSPID ATRESIA Recognition : TRICUSPID ATRESIA Recognition Cyanotic Infant - Early Cyanosis Spell + No CHF / Cardiomegaly / Single S2 / Murmur . ‘A’ wave ? Slide 72: TRICUSPID ATRESIA Slide 73: TOF PA Slide 74: TETRALOGY OF FALLOT WITH PA Slide 75: PULMONARY ATRESIA WITH INTACT SEPTUM CHD - NEWBORN - PULSES : CHD - NEWBORN - PULSES Upper limb Lower limb Anomalies ++ ++ Normal CCHD; No collaterals L - R shunt , PS +++ +++ PDA . AP window CCHD with collat / PDA Truncus + + AS . HLHS +++ + Coarctation IAAS Slide 77: PPHN Clinical Evaluation Chest XRAY ECG ABG ECHO HYPEROXIA test Cardiac PPHN Respiratory Background substrate Cyanosis CHF PAH SPO2 Slide 78: DIAGNOSIS OF PPHN PPHN ? – Hyperoxia | Hyperoxia - HV test ABG ECHO UL LL PAH No SHD R-L P P H N Slide 79: BP in NEWBORN Optimal : 4 limb BP How : Dinamap (Oscillometry) Doppler Manual Suboptimal : Flush Method Upper limb BP > 10mmHg than Lower limb BP CoA Slide 80: MURMUR IN NEONATE Symptomatic Asymptomatic Abnormal Innocent PDA. VSD. PS. AS. PABS. AV Valve regurgitation 5 days old baby. Asymptomatic. No Cyanosis. Good 3/6 murmur, LS Border : 5 days old baby. Asymptomatic. No Cyanosis. Good 3/6 murmur, LS Border Possibilities 1. Valvar PS 2. Valvar AS 3. Pink TOF Also Small VSD | PDA Obstructive Lesions Slide 82: MURMUR ULSB : PS AS MID / LLSB : Pink TOF VSD EXAMINE FOR : EXAMINE FOR Tinge of cyanosis - Pink TOF Femorals weak - AS + CoA Femorals full - PDA small Everything ok - PS ASYMPTOMATIC LOUD MURMUR & CXR : RV Apex : PS RV Apex; Rt Arch : TOF Normal Apex : Mild PS | AS | VSD ASYMPTOMATIC LOUD MURMUR & CXR ASYMPTOMATIC LOUD MURMUR & ECG : ASYMPTOMATIC LOUD MURMUR & ECG RVH - PS RVH Early Transition – Pink TOF Normal RV – PS| AS - Small VSD | PDA Slide 86: ASYMPTOMATIC MURMUR IN NEWBORNSAT Hospital experience Screened : 6846 Murmur : 134 [ 1.96% ] Murmur at 6 weeks : 74 [ 1.08% ] (125) Become symptomatic : 9 [ 7.2% ] Slide 87: 134 0 6wks Murmur + Symptomatic ASYMPTOMATIC MURMUR IN NEWBORN 125 74 9 Slide 88: Normal : 10 Abnormal : 61 14.1% ECHO AT 6 Wks 85.9% 85.9% N - 71 Abnormal N Slide 89: Non critical Critical CHD CHD ASYMPTOMATIC MURMUR ABN echo at 6 wks 31 11 Slide 90: ECHO AT 6 Wks Common CHD ASYMPTOMATIC MURMUR IN NEWBORN Critical CHD ASD VSD PDA PS HLHS TOF SV.PS AS Slide 91: Complementary Poor screening test Sensitivity – 26 – 60 % Correlation of interpretation - Modest Correlation to Echo - Poor CHEST X RAY New born with Big Heart(CTR > 65% ) : New born with Big Heart(CTR > 65% ) Think of Ebstein Critical PS with IVS Pericardial Effusion Intra uterine Myocarditis New born with Big Heart : New born with Big Heart 1. Funny ECG : Ebstein [ Tall P; WPW; rSR; 10 AV block ] 2. RVH c stain : PS 3. Low Voltages : Pericardial effusion Slide 94: EBSTEINS ANOMALY Slide 95: CHDCXR ECG a. PBF : d-TGA. TAPVC. DORV. AVSD. PDA . CoA syndrome b. PBF : TOF c PA. PS. TGA PS. AVSD. PS c. NDBF : PS. CoA d. PVH : Obstr. TAPVC. HLHS. Cor Triatriatum I. RVH Slide 96: a. LVH : TA c TGA. SV. PAH b. PBF : TA. PS c IVS. PA c IVS c. N PBF : AS. CoA. ALCAPA. MR d. PVH : MR.AS CHDCXR ECG II. LVH Slide 97: a. PBF : Truncus. AVSD. TGA c VSD. PDA. VSD. b. PBF PS.IVS. DORV PS. TGA . VSD. PS CHDCXR ECG III. BVH 7 days old baby. In CHF. Tinge of Cyanosis . Has ARI : 7 days old baby. In CHF. Tinge of Cyanosis . Has ARI Admixture Lesions Large L R c Pneumonia ? TAPVC Single Ventricle DORV Truncus Arteriosus Possibilities CHF; cyanosis : CHF; cyanosis RVH. RAE . RV Strain : TAPVC Funny ECG : SV or DORV Prolonged PR; LAD; r/S in v1-v6; No Q in V6] BVH : Truncus DORV (Large VSD ?) LAD : AVSD.PAH Slide 102: HLHS Slide 103: COARCTATION – EARLY INFANCY 14 days old with CHF. HR 250/mt : 14 days old with CHF. HR 250/mt Only one possibility SVT Look for Shock | Hypo perfusion CHF NEWBORN WITH POTENTIAL CHD 7 Questions : NEWBORN WITH POTENTIAL CHD 7 Questions 1. Is the Baby Pink or Blue ? 2. Is there CHF ? 3. If both CHF / Cyanosis are present, which is more ? 4. How are the Limb pulses - Radial ; Femoral ? 5. Is there Cardiomegaly / Malposition of Apex ? 6. How is S2 ? ( click / S3 ? ) 7. Are there murmurs ? Slide 107: Cyanosis CHF Shock Arrhythmia Symptomatic Asymptomatic NEWBORN No murmur Murmur Evaluate Screen ? Evaluate ? wait Examination POx Both Slide 108: Non Definitive Work up CHD in NEONATE Physical Examination Four limb BP SPO2 Chest Xray Electrocardiography Slide 109: CHD LIKELY Clinical Cyanosis ; POx ? Cyanotic Acyonotic CHD CHD ABG + Hyperoxia Test Echocardiography Echocardiography CXR ECG CXR ECG Slide 110: Asymptomatic CHF Cyanosis Shock Arrhythmia Others CXR . ECG. SPo2 Echocardiography NEWBORN Symptomatic ? Non Definitive Evaluation Slide 111: ASYMPTOMATIC NEWBORN Cyanosis . Perfusion. Respiration Pulse. JVP. BP. Apex Heart sounds. Murmur Normal Abnormal Low Probability High Probability CHD Physical Examination ?POx Regular Follow up Evaluate Slide 112: OF TITANIC, SONAR,SOUND , ULTRASOUND and CARDIAC ULTRASOUND Cardiac ultrasound DIAGNOSTIC SENTINEL for CHD Slide 113: ECHOCARDIOGRAPHY & NEONATOLOGIST Functional Echo Consultant Echo Anatomy Function ? Screening Echo Slide 114: Segmental Analysis -ECHO Cardiac Position Associated Anomalies Ventricular Loop Atrioventricular Alignment Infundibulam Great Vessel Orientation Atrial situs Ventriculo Arterial Alignment Slide 115: “Most Newborns with CHD are Asymptomatic at Birth” “Baby’s primary activity is feeding: so analyze that” Slide 116: PULSE OXYMETRY Will pick up critical CHD esp. Duct dependent Advantage over physical examination – Increased sensitivity Prevent delayed diagnosis of critical CHD Early s improves outcome Slide 117: System Review 8 studies 35960 Newborn Sensitivity : 63% Specificity : 99% False + ve : 0.2% Sensitivity when looking for CCHD PULSE OXYMETRY in Asymptomatic newborn Arch Dis. Child 2007 Slide 118: PO2 + Clinical Exam in Detecting Duct dep CHD BMJ n: 39821 Picked up 29 duct dep CHD Sensitivity : 90 % False +ve : 0.2 % Near 100% detection of duct dep CHD Slide 119: PULSE OXYMETRY When ? > 24 hrs Cut off ? < 955 Where ? Post ductal ( Lower Limb) Slide 120: At 24 hours : Pre discharge Foot Disposable Probe Cut off < 95 % POx Slide 121: AHA /AAP Statement POx Circulation Aug 2009 Sensitivity for critical CHD 70 % At 24 hrs Cut off < 95 % Slide 122: CUT OFF ? Normal 97.3 + 1.3 ( Foot) So cut off < 95% Can it be < 92% ? Slide 123: Combining POx & Clinical Exam Sensitivity : 77% Specificity : 100% Cases detected N: 5211 TAPVC TA PA c VSD L R Slide 124: PULSE OXYMETRY issues Reduced sensitivity in Acyanotic CHD May miss critical AS. LOVOT Obstruction Cost of Machine + Probe Overanxious Parents False Positivity Slide 125: SCREEN Physical Exam 50 % ? Pox 65 % 95 % Both 77 % 99 % Methods Sensitivity Specificity Slide 126: NEWBORN Symptom No Symptom History. Examination CXR. ECG. SPO2 ECHO SCREEN (?) PHYSICAL EXAM POx Both ? High Prob Low Prob Slide 127: CHD in Neonate Symptomatic Asymptomatic CHF Cyanosis Shock Arrhythmia Definitive Murmur No murmur Non definitive Wait ? SCREEN Pox PE BOTH Abnormal Normal CXR ECG SPO2 Echocardiography ( Cath; MRI ) I Owe Tot g abhilash senior resident pediatrics : I Owe Tot g abhilash senior resident pediatrics www.pedheartsat.org : www.pedheartsat.org Please visit our Website ! Slide 130: THANK YOU . You have been very kind & patient. !

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