Vikas Kohli MD FAAP FACC American Board Certified Pediatric CardiologistSenior Consultant, Indraprastha Apollo  Director, ...
Classification of CHD   ACYANOTIC            CYANOTIC   Increased Flow       Decreased Flow      ATRIAL: ASD         ...
ACYANOTIC   Vs.   CYANOTIC     HOW TO DECIDE ?
Acyanotic Vs. Cyanotic Is history and physical exam reliable ? Normal Saturations are >94% Between 93 and 85% human eye...
Acyanotic Vs. Cyanotic  ACYANOTIC         CYANOTIC                     Sats less than 93%  Pulse Oximeter  Sats >94%
HISTORY Sweating while feeding Shortness of breath Failure to thrive Recc infections (Lower) chest  INCREASED PULMONAR...
HISTORY Cyanosis Spells Squatting Syncope, Chest Pain
CLINICAL DIAGNOSIS HISTORY          : Increased flow or not PULSE OXIMETER   : Acyanotic Vs. Cyanotic CHEST X-RAY      ...
Classification of CHD   ACYANOTIC            CYANOTIC   Increased Flow       Decreased Flow      ATRIAL: ASD         ...
Classification of CyCHD  CYANOTIC  Decreased Flow     TOF     Pulm Atresia  Increased Flow     TAPVD     TGA     T...
ToF: Relevance of murmur Longer the murmur : less the RVOT obstruction More the obstruction: shorter the murmur In a sp...
Murmur and other clinical signs To and Fro murmur: Absent Pulmonary Valve Syndrome
Truncus Systolic ejection murmur if truncal valve is mildly  stenotic If truncal valve is regurgitant, murmur becomes to...
Conditions with RV/Pulm HTN RV systemic Pressures    Pulm Hypertension ToF                      TAPVD Pulm Atresia, V...
CYANOTC CONGENITAL HEART DISEASE
X-ray Chest - Assessment Situs Cardiac Configuration Cardiac Borders Chamber size   LV   RV   RA   LA
TOF                  1. RV APEX                  2. SMALL PULM BAY                  3. NARROW PEDICLE      3          2   ...
TOF RT AO ARCH                 • ALL FINDINGS OF                   TOF                 • LOOK ALONG                   RIGH...
TOF ABSENT PULM VALVE
PULM ATRESIA VSD                   1. RV APEX                   2. NARROW PEDICLE                   3. DECREASED PBF      ...
SINGLE VENTRICLE                   1. SINGLE                      VENTRICLE                      CAN HAVE                 ...
TGA      • RV APEX      • DIFFICULT TO        MAKE OUT IN        NEWBORNS      • NARROW PEDICLE      • INCREASED FLOW     ...
TGA 5 MONTHS OLD                   • RV APEX                   • NARROW PEDICLE                   • CARDIAC ENLARGEMENT   ...
TGA 6 MO OLD               • RV APEV               • NARROW                 PEDICLE               • MILD CARDIAC          ...
TRUNCUS          • RV APEX          • INCREASED PBF          • MILD CARDIAC            ENLARGEMENT          • PEDICLE/BASE...
SUPRACARDIAC TAPVD                     • WIDE SUP                       MEDIATINUM                     • NOT MUCH CARDIAC ...
INFRADIAPHRAGMATIC TAPVD                           • DIFFUSE                             HAZINESS                         ...
TAPVD-INFRADIAPHRAGMATIC OBSTRUCTED
HLHS       • INCREASED PBF       • SMALL HEART       • MAYBE NORMAL XRAY
X-RAYS CONCLUSION X-Rays allow one to get an idea of the PBF Additionally the dominant ventricle can be commented on at ...
Presentations Prenatal Diagnosis Asymptomatic Newborn Postnatal Cardiac Murmur Postnatal Critical Illness Presentation...
Which Conditions ? Duct dependent pulmonary circulation   PULMONARY ATRESIA   CRITICAL PS Duct Dependent Systemic Circ...
WHY THEY DON’T PRESENTEARLIER TYPICALLY BABY STABLE FOR 1-2 WEEKS THEN CRASHES DUE TO DUCT CLOSURE SO, FETAL CIRCULATIO...
The Baltimore-Washington Study           It was observed that, of all infants with              cardiovascular malformatio...
What are the consequences of late misseddiagnosis ? Mortality Brain Damage Multiorgan failure Perioperative mortality
Can we screen for CHD ? 1990’s:    4 extremity BP    Palpation of pulses in lower extremity This was anticipated to he...
Screening for duct-dependant congenital heart disease withpulse oximetry: a critical evaluation of strategies to maximizes...
The reliability of a single pulse oximetry reading as a    screening test for congenital heart disease in otherwise    asy...
Strategies for implementing screening forcritical congenital heart disease   AAP WORKING GROUP SUBCOMITTEE ON P-OX        ...
NEONATAL PRESENTATIONS THE BLUE BABY THE PINK BABY IN DISTRESS THE SHOCKY BABY
INDICATORS OF CARDIAC PROBLEM      PRIMARY           SECONDARY    Desaturation     Murmur    Shock            Cardiac ...
DESATURATION PPHN is presumed to be the cause Surprisingly: CHD can have exactly same presentation Low saturation witho...
DIFFERENTIAL DIAGNOSIS Cyanosis all over    Intra-cardiac Mixing: Single Ventricle    Intrapulmonary mixing: PPHN Diff...
ESPECIALLY IF ANY OF THE SECONDARY FACTORS                                   PRESENT
INDICATORS OF CARDIAC PROBLEM                      SECONDARY       PRIMARY      Murmur  Desaturation                   ...
CHD (Obstructive) Vs SEPSISCoarctation, Aortic Stenosis, Hypopl LV (HLHS) Most NB’s transferred to tertiary centers are a...
ESPECIALLY IF SECONDARY SIGNS PRESENT
HOW TO REACH A DIAGNOSIS ? SUSPECT: KNOWLEDGE BASE REQUIRED DIAGNOSTIC MODALITY:  ECHOCARDIOGRAM: Ready availability of ...
PROSTAGLANDIN
WHO SHOULD BE GIVEN PROSTAGLANDIN  The threshold for starting prostaglandin is directly   proportional to severity of the...
Physical Exam helps in deciding  THE CLINICALLY USEFUL FINDING IS CYANOSIS  IN A CRITICALLY ILL NEWBORN:    IF MURMUR+CY...
Physical Exam helps in deciding The clinically useful finding IN A NON-  CYANOSED CRITICALLY ILL NEWBORN IS: PRESENCE OF...
STARTING PROSTIN USUALLY THE DECISION TO START IS EASY IN CRITICALLY ILL “DYING” NEWBORN RESPONSE IS DRAMATIC
TIPS EARLY INSTITUTION OF PROSTIN CY NB’s WITH MURMUR ACY NB’s WITH ABNORMAL PULSES EVEN WITHOUT ECHO EARLY TRANSPORT...
STARTING PROSTIN Prior to starting, one needs to have Prostin available This also remains a critical step outside the NC...
STARTING PROSTIN Ease of use of Prostin is directly proportional to:    Severity of Illness    Ease of availability Co...
DOSAGE START WITH 0.05 mcg/Kg/Min 500 mcg/ml Put 1 ml in 25 ml Infuse at 0.5ml/hr = 0.05mcg/Kg/Min To allow 0.1 mcg/k...
SIDE EFFECTS APNEA    DOSE RELATED    OVERCOME WITH AMINOPHYLLINE    NO NEED TO INTUBATE Aminophylline: Bolus dose of...
 PROSTIN VR    Rs 7-8000  (Pfizer) ALPROSTADIL   Rs 5000  (Samarth) BIOGLANDIN    Rs 4000  (United)
CONCLUSION Screening by pulse ox is imperative and standard care  now Familiarity with prostaglandin is important Echo ...
SINGLE VENTRICLE PATHWAYS INCREASED PULM BLOOD FLOW    WITHOUT PS DECREASED PULM BLOOD FLOW   WITH PS OR PULM ATRESIA
BASIC AIM OF MANAGEMENT IS TO ISOLATE THE SINGLE VENTRICLE ONLY FOR SYSTEMIC FLOW IE TO RECEIVE PULM VENOUS RETURN AND D...
FIRST STEP IN EARLY INFANCY MAKE SURE PULM PRESSURES ARE LOW   THAT IS DONE BY      BANDING THE PULM ARTERY IN INCREASE...
NEXT 2 STEPS INVOLVE ISOLATING THE SYSTEMIC VENOUS RETURN FROM SINGLE VENTRICLE GLENN OPERATION: SVC CONNECTED TO PULM A...
DR VIKAS KOHLI FETAL NEONATAL PEDIATRIC CARDIOLOGIST OPD: DELHI CHILD HEART CENTER INPATIENT/PROCEDURES/SURGERY: APOLLO
Approach to a child with cyanotic chd
Approach to a child with cyanotic chd
Approach to a child with cyanotic chd
Approach to a child with cyanotic chd
Approach to a child with cyanotic chd
Approach to a child with cyanotic chd
Approach to a child with cyanotic chd
Approach to a child with cyanotic chd
Upcoming SlideShare
Loading in...5
×

Approach to a child with cyanotic chd

5,551

Published on

Approach to CHD; Approach to cyanotic CHD; Separate section on approach to neonatal CHD. Prostaglandin and other methods of managing the critically ill newborn

Published in: Health & Medicine
0 Comments
24 Likes
Statistics
Notes
  • Be the first to comment

No Downloads
Views
Total Views
5,551
On Slideshare
0
From Embeds
0
Number of Embeds
1
Actions
Shares
0
Downloads
279
Comments
0
Likes
24
Embeds 0
No embeds

No notes for slide

Approach to a child with cyanotic chd

  1. 1. Vikas Kohli MD FAAP FACC American Board Certified Pediatric CardiologistSenior Consultant, Indraprastha Apollo Director, Delhi Child Heart Center
  2. 2. Classification of CHD  ACYANOTIC  CYANOTIC  Increased Flow  Decreased Flow  ATRIAL: ASD  TOF  VENTR: VSD  Pulm Atresia  ARTERIAL: PDA  Increased Flow  COMBINED:  TAPVD VSD+PDA  TGA  No Shunts  Truncus  Pulm or Aortic  Tricuspid Atresia Stenosis
  3. 3. ACYANOTIC Vs. CYANOTIC HOW TO DECIDE ?
  4. 4. Acyanotic Vs. Cyanotic Is history and physical exam reliable ? Normal Saturations are >94% Between 93 and 85% human eye cannot detect desaturation Below 85% we can visually diagnose Cyanosis
  5. 5. Acyanotic Vs. Cyanotic  ACYANOTIC  CYANOTIC  Sats less than 93%  Pulse Oximeter  Sats >94%
  6. 6. HISTORY Sweating while feeding Shortness of breath Failure to thrive Recc infections (Lower) chest INCREASED PULMONARY BLOOD FLOW ACYANOTIC OR CYANOTIC
  7. 7. HISTORY Cyanosis Spells Squatting Syncope, Chest Pain
  8. 8. CLINICAL DIAGNOSIS HISTORY : Increased flow or not PULSE OXIMETER : Acyanotic Vs. Cyanotic CHEST X-RAY : increased Flow or not
  9. 9. Classification of CHD  ACYANOTIC  CYANOTIC  Increased Flow  Decreased Flow  ATRIAL: ASD  TOF  VENTR: VSD  Pulm Atresia  ARTERIAL: PDA  Increased Flow  COMBINED:  TAPVD VSD+PDA  TGA  No Shunts  Truncus  Pulm or Aortic  Tricuspid Atresia Stenosis
  10. 10. Classification of CyCHD  CYANOTIC  Decreased Flow  TOF  Pulm Atresia  Increased Flow  TAPVD  TGA  Truncus  Tricuspid Atresia
  11. 11. ToF: Relevance of murmur Longer the murmur : less the RVOT obstruction More the obstruction: shorter the murmur In a spell almost no murmur This is diagnostic and a very important clinical sign
  12. 12. Murmur and other clinical signs To and Fro murmur: Absent Pulmonary Valve Syndrome
  13. 13. Truncus Systolic ejection murmur if truncal valve is mildly stenotic If truncal valve is regurgitant, murmur becomes to and fro This is one of the few causes to and fro murmur including ToF with Aortic Insufficiency Pulse volume is good and maybe bounding in truncus
  14. 14. Conditions with RV/Pulm HTN RV systemic Pressures  Pulm Hypertension ToF  TAPVD Pulm Atresia, VSD  Truncus  Single Ventricle Incr PBF
  15. 15. CYANOTC CONGENITAL HEART DISEASE
  16. 16. X-ray Chest - Assessment Situs Cardiac Configuration Cardiac Borders Chamber size  LV  RV  RA  LA
  17. 17. TOF 1. RV APEX 2. SMALL PULM BAY 3. NARROW PEDICLE 3 2 4. PULM VASCULATURE NOT SEEN 4 1 5. NO CARDIAC ENLARGEMENT
  18. 18. TOF RT AO ARCH • ALL FINDINGS OF TOF • LOOK ALONG RIGHT HEART BORDER • LACK OF PULM BF • 25% TOF PTS • 33% TRUNCUS PTS • TRUNCUS HAS INCREASED PBF
  19. 19. TOF ABSENT PULM VALVE
  20. 20. PULM ATRESIA VSD 1. RV APEX 2. NARROW PEDICLE 3. DECREASED PBF 4. MAY BE INCREASED PBF W MANY COLLATERALS 5. NOT NECESSARILY VERY DIFFERENT FROM TOF
  21. 21. SINGLE VENTRICLE 1. SINGLE VENTRICLE CAN HAVE INCREASED OR DECREASED PBF 2. CAN HAVE VARIED SHAPES OF HEART 3. BUT MAY AT TIMES LOOK NORMAL ALSO
  22. 22. TGA • RV APEX • DIFFICULT TO MAKE OUT IN NEWBORNS • NARROW PEDICLE • INCREASED FLOW IN NEWBORNS LOOKS LIKE THIS
  23. 23. TGA 5 MONTHS OLD • RV APEX • NARROW PEDICLE • CARDIAC ENLARGEMENT • MASSIVE PULM BLOOD FLOW
  24. 24. TGA 6 MO OLD • RV APEV • NARROW PEDICLE • MILD CARDIAC ENLARGEMENT • INCREASED PBF BUT NOT MASSIVE
  25. 25. TRUNCUS • RV APEX • INCREASED PBF • MILD CARDIAC ENLARGEMENT • PEDICLE/BASE NOT AS NARROW
  26. 26. SUPRACARDIAC TAPVD • WIDE SUP MEDIATINUM • NOT MUCH CARDIAC ENLARGEMENT • NON SPECIFIC APEX (USUALLY RV) • INCREASED PBF • MAYBE ASSOC W CARDIAC ENLARGEMENT
  27. 27. INFRADIAPHRAGMATIC TAPVD • DIFFUSE HAZINESS • NOT PROMINENT PA’S • TINY NODULAR SHADOWS • HILUM PROMINENCE > PERIPHERY
  28. 28. TAPVD-INFRADIAPHRAGMATIC OBSTRUCTED
  29. 29. HLHS • INCREASED PBF • SMALL HEART • MAYBE NORMAL XRAY
  30. 30. X-RAYS CONCLUSION X-Rays allow one to get an idea of the PBF Additionally the dominant ventricle can be commented on at times Some detail like Rt Ao Arch may be helpful PA dominance (eg TGA) Vs PA subsided (Pulm Atresia, TOF) are helpful findings
  31. 31. Presentations Prenatal Diagnosis Asymptomatic Newborn Postnatal Cardiac Murmur Postnatal Critical Illness Presentation:  The Cyanotic Newborn  The Newborn with Respiratory Distress  The Newborn presenting with Shock
  32. 32. Which Conditions ? Duct dependent pulmonary circulation  PULMONARY ATRESIA  CRITICAL PS Duct Dependent Systemic Circulation  HYPOPLASTIC LEFT HEART  INTERRUPTED AORTIC ARCH  CRITICAL COA (ACYANOTIC)
  33. 33. WHY THEY DON’T PRESENTEARLIER TYPICALLY BABY STABLE FOR 1-2 WEEKS THEN CRASHES DUE TO DUCT CLOSURE SO, FETAL CIRCULATION ALLOWS CHILD TO BE ASYMPTOMATIC QUESTION IS CAN WE PICK THEM UP WHEN THEY ARE ASYMPTOMATIC ?
  34. 34. The Baltimore-Washington Study It was observed that, of all infants with cardiovascular malformations who died in the first week of life, one in four did not have the cardiovascular malformation identified before death.Kuehl KS et al. Failure to diagnose congenital heart disease in infancy. Pediatrics 1999;103:743-747
  35. 35. What are the consequences of late misseddiagnosis ? Mortality Brain Damage Multiorgan failure Perioperative mortality
  36. 36. Can we screen for CHD ? 1990’s:  4 extremity BP  Palpation of pulses in lower extremity This was anticipated to help But still diagnosis was missed 2000’s  Pulse Oximeter Saturation
  37. 37. Screening for duct-dependant congenital heart disease withpulse oximetry: a critical evaluation of strategies to maximizesensitivity Acta Paediatr. 2005 Nov;94(11):1590-1596. CTO2 Vs NGO2 200 normals compared with 66 with critical CHD IF ONLY ONE MEASUREMENT WAS MADE 7/66 PTS HAD A FALSE NEGATIVE BUT WHEN CRITERIA WERE CHANGED TO:  > 3% DIFFERENCE BET UL & LL  BOTH UL AND LL SATS < 95% Then sensitivity of 98.5%, specificity of 96.0%, positive predictive value of 89.0% and negative predictive value of 99.5%
  38. 38. The reliability of a single pulse oximetry reading as a screening test for congenital heart disease in otherwise asymptomatic newborn infants. Pediatr Cardiol. 2008 Sep;29(5):885-9. Routine pulse oximetry was neither reliable nor an important diagnostic tool in our cohort.
  39. 39. Strategies for implementing screening forcritical congenital heart disease AAP WORKING GROUP SUBCOMITTEE ON P-OX SCREENING The work-group members found sufficient evidence to begin screening for low blood oxygen saturation through the use of pulse-oximetry monitoring to detect CCHD in well-infant and intermediate care nurseries. Pediatrics. 2011 Nov;128(5):e1259-67. Epub 2011 Oct 10
  40. 40. NEONATAL PRESENTATIONS THE BLUE BABY THE PINK BABY IN DISTRESS THE SHOCKY BABY
  41. 41. INDICATORS OF CARDIAC PROBLEM PRIMARY SECONDARY  Desaturation  Murmur  Shock  Cardiac Enlargement  Resp Distress  Peripheral Pulse Abn
  42. 42. DESATURATION PPHN is presumed to be the cause Surprisingly: CHD can have exactly same presentation Low saturation without resp cause: r/o cardiac
  43. 43. DIFFERENTIAL DIAGNOSIS Cyanosis all over  Intra-cardiac Mixing: Single Ventricle  Intrapulmonary mixing: PPHN Differential Cyanosis  Upper Limb Blue: TGA+PDA  Lower Limb Blue: Duct Shunting R to L with normally related GA’s Combination: PPHN  Intrapulmonary + PDA + PFO shunting
  44. 44. ESPECIALLY IF ANY OF THE SECONDARY FACTORS PRESENT
  45. 45. INDICATORS OF CARDIAC PROBLEM SECONDARY PRIMARY  Murmur  Desaturation  Cardiac Enlargement  Shock  Peripheral Pulse  Resp Distress Abn
  46. 46. CHD (Obstructive) Vs SEPSISCoarctation, Aortic Stenosis, Hypopl LV (HLHS) Most NB’s transferred to tertiary centers are already on ABx Differentiating Sepsis is very difficult ! Most markers dont have reliable sensitivity 22/47 such cardiac newborns did not have murmur Majority with fulminant sepsis had weak pulses due to low cardiac outputDifferentiation of systemic infection and congenital obstructive left heart disease in the very young infant. Pediatr Emerg Care 1998;14:263-267
  47. 47. ESPECIALLY IF SECONDARY SIGNS PRESENT
  48. 48. HOW TO REACH A DIAGNOSIS ? SUSPECT: KNOWLEDGE BASE REQUIRED DIAGNOSTIC MODALITY: ECHOCARDIOGRAM: Ready availability of reliable Bedside Pediatric/Neonatal Echo remains the single most important hurdle in the diagnosis of neonatal CHD
  49. 49. PROSTAGLANDIN
  50. 50. WHO SHOULD BE GIVEN PROSTAGLANDIN  The threshold for starting prostaglandin is directly proportional to severity of the childs illness  In the critically ill child: start Prostin without wasting time  Go by clinical findings
  51. 51. Physical Exam helps in deciding  THE CLINICALLY USEFUL FINDING IS CYANOSIS IN A CRITICALLY ILL NEWBORN:  IF MURMUR+CYANOSIS IS PRESENT START PROSTIN
  52. 52. Physical Exam helps in deciding The clinically useful finding IN A NON- CYANOSED CRITICALLY ILL NEWBORN IS: PRESENCE OF ABNORMAL PULES: IF PRESENT START PROSTIN Danford DA et al. Application of information theory to decision analysis in potentially prostaglandin-responsive neonates. JACC 1986;8:1125-1130
  53. 53. STARTING PROSTIN USUALLY THE DECISION TO START IS EASY IN CRITICALLY ILL “DYING” NEWBORN RESPONSE IS DRAMATIC
  54. 54. TIPS EARLY INSTITUTION OF PROSTIN CY NB’s WITH MURMUR ACY NB’s WITH ABNORMAL PULSES EVEN WITHOUT ECHO EARLY TRANSPORT SUSPECT CHD WHEN:  PPHN TYPE PRESENTATION  SEPSIS
  55. 55. STARTING PROSTIN Prior to starting, one needs to have Prostin available This also remains a critical step outside the NCR Since cost issues involved Pediatricians may not want to use it without a confirmation
  56. 56. STARTING PROSTIN Ease of use of Prostin is directly proportional to:  Severity of Illness  Ease of availability Confounding Factor: Echo should be done or not ?
  57. 57. DOSAGE START WITH 0.05 mcg/Kg/Min 500 mcg/ml Put 1 ml in 25 ml Infuse at 0.5ml/hr = 0.05mcg/Kg/Min To allow 0.1 mcg/kg/min increase drip rate to 1ml/hr
  58. 58. SIDE EFFECTS APNEA  DOSE RELATED  OVERCOME WITH AMINOPHYLLINE  NO NEED TO INTUBATE Aminophylline: Bolus dose of 6 mg/kg before or during initiation of PGE1, and continued at 2 mg/kg dose every 8 hours for 72 hours. PEDIATRICS Vol. 112 No. 1 July 2003, pp. e27-e29
  59. 59.  PROSTIN VR Rs 7-8000 (Pfizer) ALPROSTADIL Rs 5000 (Samarth) BIOGLANDIN Rs 4000 (United)
  60. 60. CONCLUSION Screening by pulse ox is imperative and standard care now Familiarity with prostaglandin is important Echo not needed in a critically ill child Reliable Pediatric echo is extremely helpful
  61. 61. SINGLE VENTRICLE PATHWAYS INCREASED PULM BLOOD FLOW  WITHOUT PS DECREASED PULM BLOOD FLOW  WITH PS OR PULM ATRESIA
  62. 62. BASIC AIM OF MANAGEMENT IS TO ISOLATE THE SINGLE VENTRICLE ONLY FOR SYSTEMIC FLOW IE TO RECEIVE PULM VENOUS RETURN AND DELIVER IT TO AORTA THE PULM BLOOD FLOW BYPASSES THIS AND GOES TO LUNGS WITHOUT A PUMP IN BETWEEN
  63. 63. FIRST STEP IN EARLY INFANCY MAKE SURE PULM PRESSURES ARE LOW  THAT IS DONE BY  BANDING THE PULM ARTERY IN INCREASED PBF GROUP  MAINTAINNG PBF IN DECREASED PBF GROUP BY BT SHUNT IF NEEDED
  64. 64. NEXT 2 STEPS INVOLVE ISOLATING THE SYSTEMIC VENOUS RETURN FROM SINGLE VENTRICLE GLENN OPERATION: SVC CONNECTED TO PULM ARTERY DIRECTELY FONTAN OPERATION: IVC CONNECTED TO PULM ARTERY DIRECTELY
  65. 65. DR VIKAS KOHLI FETAL NEONATAL PEDIATRIC CARDIOLOGIST OPD: DELHI CHILD HEART CENTER INPATIENT/PROCEDURES/SURGERY: APOLLO

×