Successfully reported this slideshow.
We use your LinkedIn profile and activity data to personalize ads and to show you more relevant ads. You can change your ad preferences anytime.
Approach to a patient with
Congenital Cyanotic Heart Disease
-
Speakers:Dr. Debasis Maity
Dr.Md.Samim Shikari
Chittaranjan...
Nightmare:
• It’s 2A.M
• Posted in NICU
• Your 1st night therein
• You get a callbook from C.S OT
• You rush to the spot
•...
Congenital Heart Disease
• Prevalence
• 0.8% of live births
• 3-4% of stillborns, 10-25% of spontaneous
abortuses
• 2% of ...
Cyanotic Heart Disease
• Patient appears blue (cyanotic), due to
deoxygenated blood bypassing the lungs and
entering the s...
Acyanotic or Cyanotic
• Normal saturations are 95%
• Visual diagnosis below 85%
• Pulse oximetry useful in cases with Sp...
Common Cyanotic Lesions
Decreased Pulmonary Blood
Flow
Increased Pulmonary Blood
Flow
Tetralogy of Fallot
Tricuspid Atresi...
History
• Development and weight gain
• Poor feeding
• Cyanosis and cyanotic spells
• Squatting
• Tachypnea, dyspnea
• Fre...
Antenatal and Family History
• Maternal Infections
– Rubella, CMV, Herpes, Coxsackie, HIV
• Maternal Medications, Alcohol ...
Physical Examination
• General appearance, weight and nutrition
• Association with chromosomal syndromes and
other systemi...
Associations
Syndrome Associations
Trisomy 21 (Down syndrome) Endocardial cushion defect, VSD, ASD
X0 (Turner Syndrome) Bi...
Associations
Syndrome Associations
DiGeorge sequence, CATCH 22 (cardiac
defects, abnormal facies, thymic
aplasia, cleft pa...
Cyanosis
• Cyanosis: Bluish discoloration of skin and mucous
membrane due to reduced Hb concn more than
5gm/1ooml in cutan...
• Differential Cyanosis-Hands RED ,feet BLUE-as
in PDA
• Reverse differential cyanosis-Hands BLUE,feet
RED-as in Transposi...
Causes of cyanosis
CNS Depression Irregular respiration, poor muscle tone
Improves with stimulation or mechanical
ventilat...
Pulse and BP
• Examine pulse and BP in all four limbs
• Weak lower limb pulses suggestive of
coarctation
• BP compared aga...
Systemic Examination
system wise but mainly-
• First and second heart sounds
• Loud or muffled heart sounds
• Splitting
MU...
Chest Radiography
• Cardiac size(CTR to be measured) and
silhouette
• Cardiac chambers and great vessels
• Pulmonary vascu...
Normal Cardiac Silhouette
Hyperoxia Test
Appreciable specificity, sensitivity when
matched clinically.
• Measurements to be obtained by TCOM or by
a...
• We can also check preductal(rt radial art) and
post ductal(umbilical art/post.tibial artery)
arterial difference in po2....
Arterial Blood Gas
• Confirm or reject cyanosis
• Elevated pCO2 suggests respiratory pathology
• Low pH in sepsis, shock o...
Echocardiogram
• Definitive diagnositic modality for structural
heart disease
• M-mode, 2D Echo and Doppler
• Windows comm...
CHD – Age of Presentation
Common Congenital Cyanotic Heart
Diseases
Case scenario 01:
• A 2yr old boy presented with episodes of
becoming blue mostly in the morning.
• O/E-central cyanosis,c...
Xray chest
Tetralogy Of Fallot
• High VSD
– Large enough to equalize
pressure
• Pulmonic stenosis
– Usually infundibular,
sometimes v...
TOF – Other Anomalies
• Right aortic arch – 25%
• Pulmonary atresia – 15 – 20%
• Left superior vena cava
• Complete AV sep...
TOF – Critical component
Degree of pulmonary stenosis
– Determines degree of R L shunt(proportional
relation)
– Determine...
TOF - Clinical Features
• Dyspnea/Dyspnea on exersion
• Cyanosis with tachypnea and clubbing
• Failure to thrive
• Severit...
TOF - Investigations
• ECG
– Right axis deviation and
RVH
• CXR
– Decreased pulmonary
vasculature
– Concave PA segment
– R...
Echocardiography
Parasternal long axis view Parasternal short axis view
TOF – Cyanotic spells
• Hyperpnea and cyanosis, progressing to
limpness and syncope, might terminate in
convulsions or eve...
Cyanotic spells - Pathophysiology
• The highly accepted theory justifying
pathophysiology of TOF with cyanotic spells:
• kothari et al: suggests role of
mec...
Cyanotic spells – Treatment
• Always check ABC,vitals as and when
necessary.
• A) Knee chest position- To increase systemi...
• E) Propanolol- 0.01-0.25 mg/kg intravenously
over 5 min. Reduces dynamic RV outflow
obstruction.
• Slows HR (↓ R→ L Shun...
• H) Methoxamine- 0.10mg/kg IV over 5-10
min. Leads to ↑ SVR.
• I) IV fluids- preferably initially as bolus of 10-
20cc/kg...
• J) Inj NaHCO3-(1-2 meq/kg intravenously slowly)
with 1:1 dilution with 5%D. To correct metabolic
acidosis.
• K) Transfus...
TOF – Surgical Management
• Complete surgical repair
– As early as 3 – 4 months of age
• Palliative surgery
– TOF with pul...
Palliative surgeries in TOF
• Waterston’s shunt: between ascending aorta
& right pulmonary artery.
• Blalock-Taussig shunt...
Surgery - TOF
CASE SCENARIO 02:
• A 2month old INFANT OF DIABETIC MOTHER(full
term delivery) presented to ER with marked
respiratory dis...
CXR of that infant
Dignosed:
• TGA with VSD in Cngestive failure
Transposition of Great Vessels
• Aorta arises from right
ventricle, pulmonary
artery from left
ventricle(d-TGA)
• Systemic...
Transposition of Great Vessels
• Also known as D-TGA
• Normal relation – Aorta
posterior and to the right
of pulmonary art...
TGA - Variants
• TGA with intact
Interventricular septum
• TGA with VSD
• TGA with VSD and LVOT
obstruction (Pulmonary
ste...
TGA - Diagnosis
• ECG usually normal –
Right dominant
• CXR shows mild
cardiomegaly, narrow
mediastinum, and
normal to inc...
TGA - Echo
TGA - Treatment
• PGE1 (start with 0.05-0.1mcg/kg/min,once the
desired effect is achieved the dose is gradually
reduced to...
Case scenario 03:
• A 1month old boy,presented with-
• Bluish discoloration of extremiteis and tounge
• h/o- dusky appeara...
Tricuspid Atresia
• Circulation through
Foramen Ovale/ASD
• Associated VSD or PDA
• Presentation depends
on relation betwe...
Tricuspid Atresia - Presentation
• 50% present on D1,
80% by 1st month
• Infants with
pulmonary oligemia
present early wit...
Diagnosis
• CXR depends on pulmonary blood flow
• ECG – Right atrial enlargement, left axis
deviation, LVH
• Echo –
– Abse...
Management
• Corrective surgery – Fontan procedure, usually
after 2 years of age
• Palliation –
• BT shunt for oligemia
• ...
Case scenario 04
• A 2.5months old MALE infant, presented with mild
cyanosis since birth,
• o/e-Now underweight, mild cent...
Dignosis?
Total Anomalous Pulmonary Venous
Return
• Pulmonary veins drain
anomalously into RA or
systemic venous
tributaries
– Supra...
TAPVR
• Presentation and
severity depends on
pulmonary venous
obstruction
• ASD or PFO essential for
survival
TAPVR - Management
• Treat CHF with diuretics, digitalis
• Correction of metabolic acidosis, pulmonary
edema
• Corrective ...
CHD – Ductus dependent lesions
Ductus Dependent Systemic
Circulation
• Hypoplastic Left Heart
Syndrome
• Crictical AS
• “S...
Case scenario 05:
• A 2yr old child presented to OPD with-
• h/o-effort intolerance,easy fatiguability,with new onset
blui...
Dignosis?
Diagnosis?
• EBSTEIN’S anomaly with
• WPW syndrome
CHD – Management Summary
Ductus dependent Pulmonary Circulation
• TOF and PA/PS represent central cyanosis
• Cyanosis aggr...
Ductus Dependent Systemic Circulation
• Signs of poor perfusion, acidosis, mimics sepsis
• Maintain ductus patency with PG...
Medical Management
• When a congenital cyanotic heart defect or
duct dependent lesion is suspected –
– Start PGE1 infusion...
Antenatal Diagnosis
• Fetal echo – usually done around 18 – 24
weeks, as early as 12 weeks
• High risk approach –
– Siblin...
Fetal Echo
• May be done by
transabdominal or
transvaginal approach
• 4 chamber, 5 chamber
and 3 vessel views
Practical Approach to a cyanotic
patient
• Rule out acrocyanosis,cyanosis d/t extracardiac
causes and treat accordingly to...
Contd.
• By Echocardiography
• Maintain ductus dependent flow with PGE1 infusion to buy
time for corrective surgeries if c...
A BREIF DISCUSSION
Take home messages
• Maintain a high index of suspicion in neonates
and infants with respiratory or feeding
difficulties, ...
• Early institution of treatment to prevent long
term morbidity and mortality
• Seek opinion from cardiologists and
cardio...
Knee chest positions
Thank You
Upcoming SlideShare
Loading in …5
×

Approach to congenital cyanotic heart diseases

11,165 views

Published on

Published in: Health & Medicine
  • Be the first to comment

Approach to congenital cyanotic heart diseases

  1. 1. Approach to a patient with Congenital Cyanotic Heart Disease - Speakers:Dr. Debasis Maity Dr.Md.Samim Shikari Chittaranjan Sishu Sadan,Kolkata
  2. 2. Nightmare: • It’s 2A.M • Posted in NICU • Your 1st night therein • You get a callbook from C.S OT • You rush to the spot • See a just delivered “Blue Baby”. • Take all reversible measures to resuscitate • Excluded all causes ,remaining only cardiac causes left • Baby dies • Postmortem done to find out the cause • And yes you were right ,it was a case of TGA with Intact septum.
  3. 3. Congenital Heart Disease • Prevalence • 0.8% of live births • 3-4% of stillborns, 10-25% of spontaneous abortuses • 2% of premature infants (Excluding PDA) • Leading cause of death among children with congenital malformations
  4. 4. Cyanotic Heart Disease • Patient appears blue (cyanotic), due to deoxygenated blood bypassing the lungs and entering the systemic circulation. • Can be caused by right-to- left or bidirectional shunting, or malposition of the great arteries.
  5. 5. Acyanotic or Cyanotic • Normal saturations are 95% • Visual diagnosis below 85% • Pulse oximetry useful in cases with SpO2 between 85% and 94% • Important to measure pre and post ductal saturations and po2
  6. 6. Common Cyanotic Lesions Decreased Pulmonary Blood Flow Increased Pulmonary Blood Flow Tetralogy of Fallot Tricuspid Atresia Pulmonary Atresia with intact Septum Double Outlet Right Ventricle with PS TGA with PS Ebstein Anomaly Transposition of the Great Arteries Total Anomalous Pulmonary Venous Return Hypoplastic Left Heart Syndrome Persistent Truncus Arteriosus
  7. 7. History • Development and weight gain • Poor feeding • Cyanosis and cyanotic spells • Squatting • Tachypnea, dyspnea • Frequent respiratory infections • Exercise intolerance • Chest pain, syncope, palpitations • Neurological Symptoms
  8. 8. Antenatal and Family History • Maternal Infections – Rubella, CMV, Herpes, Coxsackie, HIV • Maternal Medications, Alcohol and Smoking – Amphetamines, Lithium, Valproate • Maternal Conditions – Diabetes, SLE • Hereditary Disease – Marfan, Long QT syndrome, Holt Oram Syndrome
  9. 9. Physical Examination • General appearance, weight and nutrition • Association with chromosomal syndromes and other systemic malformations • Colour • Vital Signs – Pulse, BP, respiration and temperature
  10. 10. Associations Syndrome Associations Trisomy 21 (Down syndrome) Endocardial cushion defect, VSD, ASD X0 (Turner Syndrome) Bicuspid Aortic Valve, Coarctation of Aorta Trisomy 18, Trisomy 13 VSD, ASD, PDA, coarctation of aorta, bicuspid aortic or pulmonary valve Fragile X Mitral valve prolapse, aortic root dilatation Deletion 5p (cri du chat syndrome) VSD, PDA, ASD CHARGE association (coloboma, heart, atresia choanae,retardation, genital, and ear anomalies) VSD, ASD, PDA, TOF, endocardial cushion defect
  11. 11. Associations Syndrome Associations DiGeorge sequence, CATCH 22 (cardiac defects, abnormal facies, thymic aplasia, cleft palate, and hypocalcemia) Aortic arch anomalies, conotruncal anomalies Asplenia syndrome Complex cyanotic heart lesions with decreased PBF, TGA, TAPVR Polysplenia syndrome Acyanotic lesions with increased PBF, PAPVR, dextrocardia, single ventricle Congenital rubella PDA, peripheral pulmonic stenosis Fetal hydantoin syndrome VSD, PDA, ASD Fetal Alcohol Syndrome ASD, VSD Maternal Diabetes Hypertrophic Cardiomyopathy, VSD, TGA
  12. 12. Cyanosis • Cyanosis: Bluish discoloration of skin and mucous membrane due to reduced Hb concn more than 5gm/1ooml in cutaneous veins • Central Cyanosis – Right to left shunts – Respiratory or CNS Pathology • Peripheral Cyanosis - Exposure to cold, congestive heart failure, polycythemia, shock
  13. 13. • Differential Cyanosis-Hands RED ,feet BLUE-as in PDA • Reverse differential cyanosis-Hands BLUE,feet RED-as in Transposition of the great vessels (TGA) + PDA + Pulmonary hypertension • Intermittent Cyanosis-Ebstain’s anomalies. • Cyanosis is recognized at higher level of spo2(80-85%)in polycythemia and at a lower level of spo2(45-50%) in severe anaemia.
  14. 14. Causes of cyanosis CNS Depression Irregular respiration, poor muscle tone Improves with stimulation or mechanical ventilation Pulmonary Disease Tachypnea, respiratory distress Crepitations, decreased breath sounds, CXR findings, Improvement with O2 Cardiac disease Tachypnea without retractions Lack of respiratory findings Little or no improvement with O2 CXR abnormal cardiac silhouette
  15. 15. Pulse and BP • Examine pulse and BP in all four limbs • Weak lower limb pulses suggestive of coarctation • BP compared against age specific percentile curves
  16. 16. Systemic Examination system wise but mainly- • First and second heart sounds • Loud or muffled heart sounds • Splitting MURMUR • Location • Timing Signs of congestive cardiac failure(eg-hepatomegaly etc) Presence of murmur not necessarily pathological BUT Absence of murmur does not rule out cardiac lesions
  17. 17. Chest Radiography • Cardiac size(CTR to be measured) and silhouette • Cardiac chambers and great vessels • Pulmonary vascular markings • Differentiate with pulmonary disease
  18. 18. Normal Cardiac Silhouette
  19. 19. Hyperoxia Test Appreciable specificity, sensitivity when matched clinically. • Measurements to be obtained by TCOM or by arterial po2. • If arterial Po2>250 torr:PASSED:excludes critical structural heart disease. • <100:FAILED: diagnostic of cyanotic heart disease(in absence of obvious lung disease) • 100-250: may be intracardiac mixing….
  20. 20. • We can also check preductal(rt radial art) and post ductal(umbilical art/post.tibial artery) arterial difference in po2.Difference of more than 20 torr is to be taken as significant.(remember differential cyanosis/reverse differential cyanosis)
  21. 21. Arterial Blood Gas • Confirm or reject cyanosis • Elevated pCO2 suggests respiratory pathology • Low pH in sepsis, shock or severe hypoxemia • Pre and post ductal (umbilical artery or lower limb) useful to confirm differential saturations
  22. 22. Echocardiogram • Definitive diagnositic modality for structural heart disease • M-mode, 2D Echo and Doppler • Windows commonly used – Apical 4 Chamber, Parasternal long and short axis, Suprasternal, Subclavicular and Subcostal
  23. 23. CHD – Age of Presentation
  24. 24. Common Congenital Cyanotic Heart Diseases
  25. 25. Case scenario 01: • A 2yr old boy presented with episodes of becoming blue mostly in the morning. • O/E-central cyanosis,clubbing. • No pallor,oedema or respiratory distress. • CVS-heart was of normal size,parasternal heave +ve, systolic thrill palpable over lt.middle sternal border.S1-Normal,S2-only A2 is audible. • Liver –not enlarged. • CXR given below. • ECG-Rt axis deviation. Dignosis????????
  26. 26. Xray chest
  27. 27. Tetralogy Of Fallot • High VSD – Large enough to equalize pressure • Pulmonic stenosis – Usually infundibular, sometimes valvular • Overriding of the aorta • Right ventricular hypertrophy
  28. 28. TOF – Other Anomalies • Right aortic arch – 25% • Pulmonary atresia – 15 – 20% • Left superior vena cava • Complete AV septal defect – 2% • Tricuspid valve anomalies • Anomalous coronary arteries – Ant descending artery from RCA; passes across RVOT – 5% • Anomalies of Pulmonary Artery and branches
  29. 29. TOF – Critical component Degree of pulmonary stenosis – Determines degree of R L shunt(proportional relation) – Determines duration and grade of murmur(inverse relation)
  30. 30. TOF - Clinical Features • Dyspnea/Dyspnea on exersion • Cyanosis with tachypnea and clubbing • Failure to thrive • Severity of PS determines age of presentation • Cyanotic spells /“tet spells”.
  31. 31. TOF - Investigations • ECG – Right axis deviation and RVH • CXR – Decreased pulmonary vasculature – Concave PA segment – Right aortic arch, right atrial enlargement
  32. 32. Echocardiography Parasternal long axis view Parasternal short axis view
  33. 33. TOF – Cyanotic spells • Hyperpnea and cyanosis, progressing to limpness and syncope, might terminate in convulsions or even CVA • Right ventricular infundibular spasm and decreased systemic vascular resistance – Increased catecholamines – Increased respiratory rate
  34. 34. Cyanotic spells - Pathophysiology
  35. 35. • The highly accepted theory justifying pathophysiology of TOF with cyanotic spells: • kothari et al: suggests role of mechanoreceptors in right ventricular infundibulum(spasm of RVOT) due to increased catecholamine,decreased RV size and hyperpnoea which ultimately precipitates a tet spell. • Associated reduction in SVR is also postulated.
  36. 36. Cyanotic spells – Treatment • Always check ABC,vitals as and when necessary. • A) Knee chest position- To increase systemic vascular resistance • B) Oxygen- Oxygen to improve oxygenation. • C) Morphine- Depresses respiratory center→ abolish hyperpnoea. While giving this, facilities for ventilation should be available.dose-0.2mg/kg sc/im
  37. 37. • E) Propanolol- 0.01-0.25 mg/kg intravenously over 5 min. Reduces dynamic RV outflow obstruction. • Slows HR (↓ R→ L Shunting). Slight ↑ in SVR. Blocks hyperpnea response. • F) Ketamine- 1-3mg/kg. IV over 1min→ has dual benefit causes sedation and ↑ SVR • G) Phenylephrine Hydrochloride- 0.01 mg/kg IV (slowly) or 0.1 mg/kg SC or IM (↑ SVR – dose to be titrated to BP response).
  38. 38. • H) Methoxamine- 0.10mg/kg IV over 5-10 min. Leads to ↑ SVR. • I) IV fluids- preferably initially as bolus of 10- 20cc/kg which may be increased to 60cc/kg. Bolus fluid should be isotonic saline or colloid. Extra volume can be given in cyanotic spell as the physiology is not inductive to CCF and also because of a restrictive RV physiology.
  39. 39. • J) Inj NaHCO3-(1-2 meq/kg intravenously slowly) with 1:1 dilution with 5%D. To correct metabolic acidosis. • K) Transfuse PRBC’s- 5-10 ml / kg IV over 5 hrs. • L) Correct Tachyarrhythmia- Improve diastolic filling and cardiac output. • M) RSI and mechanical ventillation as and when necessary.Some people recommend availability of atleast temporary pacing facilty before instituting beta blockers.
  40. 40. TOF – Surgical Management • Complete surgical repair – As early as 3 – 4 months of age • Palliative surgery – TOF with pulmonary atresia/hypoplastic PA – Unfavourable coronary artery anatomy – Young infants with severe cyanotic spells
  41. 41. Palliative surgeries in TOF • Waterston’s shunt: between ascending aorta & right pulmonary artery. • Blalock-Taussig shunt: between subclavian artery & pulmonary artery. • Pott’s shunt: descending aorta and left pulmonary artery.
  42. 42. Surgery - TOF
  43. 43. CASE SCENARIO 02: • A 2month old INFANT OF DIABETIC MOTHER(full term delivery) presented to ER with marked respiratory distress.o/e –central cyanosis+,b/l- creps,HR-180/min,RR-58/min,Liver palpable- 5.5cm.H/O-repeated episodes of cough,resp.distress,fever since birth.CVS-gr 3 ESM @ lt.parasternal area,CXR-cardiomegaly with narrow base and plethoric lung fields.Also there is a H/O exacerbation of cyanosis during cry.Diagnosis??????
  44. 44. CXR of that infant
  45. 45. Dignosed: • TGA with VSD in Cngestive failure
  46. 46. Transposition of Great Vessels • Aorta arises from right ventricle, pulmonary artery from left ventricle(d-TGA) • Systemic and pulmonary circulations exist as parallel circulations • PDA and PFO enable survival; 30 - 40% have VSD
  47. 47. Transposition of Great Vessels • Also known as D-TGA • Normal relation – Aorta posterior and to the right of pulmonary artery • In TGA aorta is anterior • Presents with cyanosis and tachypnea within first few hours or days of life • Severity inversely proportional to presence of shunts
  48. 48. TGA - Variants • TGA with intact Interventricular septum • TGA with VSD • TGA with VSD and LVOT obstruction (Pulmonary stenosis)
  49. 49. TGA - Diagnosis • ECG usually normal – Right dominant • CXR shows mild cardiomegaly, narrow mediastinum, and normal to increased pulmonary blood flow,rt upper field shows maximum plethora. • Egg on end appearance.
  50. 50. TGA - Echo
  51. 51. TGA - Treatment • PGE1 (start with 0.05-0.1mcg/kg/min,once the desired effect is achieved the dose is gradually reduced to 0.01mics/kg/min)) to maintain PDA • Rashkind balloon atrial septostomy in severely hypoxic or acidotic infants • Arterial switch procedure (Jatene) performed as early as first 2 weeks of life • Rastelli operation another alternative • Previously, atrial switch operations were done by creating atrial baffles
  52. 52. Case scenario 03: • A 1month old boy,presented with- • Bluish discoloration of extremiteis and tounge • h/o- dusky appearance since birth,poor feeding,rapid respiration since birth. • o/e- poor weight gain,LV type of apical impulse +ve,prominent large “a” wave in JVP, holosystolic murmur of grade 3/6 at lt lower sternal border, s1 normal S2 single.Liver-enlarged . • ECG-LAD+LVH. Dignosis?????
  53. 53. Tricuspid Atresia • Circulation through Foramen Ovale/ASD • Associated VSD or PDA • Presentation depends on relation between great arteries and presence or absence of PS • Commonest – Type 1b (No TGA, small VSD, PS)
  54. 54. Tricuspid Atresia - Presentation • 50% present on D1, 80% by 1st month • Infants with pulmonary oligemia present early with cyanosis, hyperpnea, acidosis • Infants with pulmonary plethora present with CHF 51% 18%
  55. 55. Diagnosis • CXR depends on pulmonary blood flow • ECG – Right atrial enlargement, left axis deviation, LVH • Echo – – Absence of Tricuspid valve – Relation of great arteries – RVOT – VSD, PDA
  56. 56. Management • Corrective surgery – Fontan procedure, usually after 2 years of age • Palliation – • BT shunt for oligemia • Manage CHF in infants with pulmonary plethora • Consider PA banding if medical measures fail, remember VSD might close spontaneously in Type 1 • Palliative measures meant to try to attain normal LV function, adequate PBF at low PA pressure
  57. 57. Case scenario 04 • A 2.5months old MALE infant, presented with mild cyanosis since birth, • o/e-Now underweight, mild central Cyanosis.tachypnea ,tachycardia, precordial bulge+ve,hyperactive rt ventricular impulse,cardiac impulse is highest at xiphoid process and lower lt sternal border. • S1-normal,S2-wide split and fixed,P2 accentuated,mid- diastolic rumble +nt at lower lt sternal border • Hepatomegaly +nt • ECG-RVH pattern(rsR´ IN v1) • Cxr-given below
  58. 58. Dignosis?
  59. 59. Total Anomalous Pulmonary Venous Return • Pulmonary veins drain anomalously into RA or systemic venous tributaries – Supracardiac – 50% – Cardiac – 20% – Infracardiac - 20%
  60. 60. TAPVR • Presentation and severity depends on pulmonary venous obstruction • ASD or PFO essential for survival
  61. 61. TAPVR - Management • Treat CHF with diuretics, digitalis • Correction of metabolic acidosis, pulmonary edema • Corrective surgery – earlier in obstructed TAPVR • Aim to return blood to LA and close ASD • Obstructed TAPVR – symptoms exacerbated or precipitated by PGE1
  62. 62. CHD – Ductus dependent lesions Ductus Dependent Systemic Circulation • Hypoplastic Left Heart Syndrome • Crictical AS • “Shone” complex variants • Coarctation of aorta • Interrupted Aortic Arch Ductus Dependent Pulmonary Circulation • TOF with PA • Pulmonary Atresia with intact interventricular septum • Critical PS • Tricuspid Atresia with PS/PA • Single ventricle with PS/PA • Severe Ebstein anomaly • Complete TGA with intact IVS
  63. 63. Case scenario 05: • A 2yr old child presented to OPD with- • h/o-effort intolerance,easy fatiguability,with new onset bluish discolaration of body and tounge which is gradually increasing • Mother has manic depressive psychosis*7yrs.father puts forward a h/o of rapid heart beats of the child occasionally which he has noticed. • Significant history??-One child died after similar type of presentation and had sudden death.(coincidental?) • o/e- moderate central cyanosis,dominant “v” wave in jvp,S1-normal,S2-wide split but variable,TR murmur(systolic) +nt. • ECG- RBBB+RAH pattern and has a characteristic clinch
  64. 64. Dignosis?
  65. 65. Diagnosis? • EBSTEIN’S anomaly with • WPW syndrome
  66. 66. CHD – Management Summary Ductus dependent Pulmonary Circulation • TOF and PA/PS represent central cyanosis • Cyanosis aggravated with closure of ductus • Aim to keep ductus open till palliative shunt surgery; Eg. BT shunt • PGE1 infusion indicated
  67. 67. Ductus Dependent Systemic Circulation • Signs of poor perfusion, acidosis, mimics sepsis • Maintain ductus patency with PGE1 • Ventilatory strategies to increase pulmonary vascular resistance, avoid pulmonary over circulation • Ideal systemic:pulmonary flow 1:1 • Adjust PEEP, insp. Rate, Tidal Volume • Avoid too much O2 , pO2 around 80% • Decrease SVR – Phenoxybenzamine, Milrinone
  68. 68. Medical Management • When a congenital cyanotic heart defect or duct dependent lesion is suspected – – Start PGE1 infusion (0.05 – 0.1 mcg/kg/min) – Maintenance dose of 0.01 mcg/kg/min – May cause apnea (12%), fever (10%) and flushing. – Less commonly brady/tachyarrythmia, shock, cardiac arrest
  69. 69. Antenatal Diagnosis • Fetal echo – usually done around 18 – 24 weeks, as early as 12 weeks • High risk approach – – Sibling/Family history of CHD – Maternal infection, autoimmune, metabolic conditions, drug intake – Fetal chromosomal anomaly, arrythmia – Abnormal 4 Chamber view on USG
  70. 70. Fetal Echo • May be done by transabdominal or transvaginal approach • 4 chamber, 5 chamber and 3 vessel views
  71. 71. Practical Approach to a cyanotic patient • Rule out acrocyanosis,cyanosis d/t extracardiac causes and treat accordingly to revert . • Crying,free flow of 100% o2,warming,Rx of circulatory compromise revert those situations CYANOSIS • Conduct HYPEROXIA TEST accordingly(po2 less than 100torr) • Measure pre+post ductal po2 difference ,if any(significant) CAYNOYSIS PERSISTS!!!!! • Cardiological evaluation • CXR • ECHOCARDIOGRAPHY • ECG Po2<100 torr: FAILED HYPEROXIA TEST
  72. 72. Contd. • By Echocardiography • Maintain ductus dependent flow with PGE1 infusion to buy time for corrective surgeries if compatible with life RULE OUT DUCTUS DEPENDENT FLOW • By Echocardiography • Conservative management+corrective cardiac surgeries as recommended if any + antifailure measures if needed DUCTUS INDEPENDENT LESION • For opinion from cardiologists & cardiothoracic surgeons • We must not hesitate to call help from others REFERRAL AS AND WHEN NECESSARY
  73. 73. A BREIF DISCUSSION
  74. 74. Take home messages • Maintain a high index of suspicion in neonates and infants with respiratory or feeding difficulties, and failure to thrive and cyanosis • Basic investigations that are regularly done give a good idea about the presence as well as type of CHD even before an Echo is available • One should not ignore the role of ECHOCARDIOGRAPHY because pure clinics may not be enough to definitely reach at proper diagnosis.
  75. 75. • Early institution of treatment to prevent long term morbidity and mortality • Seek opinion from cardiologists and cardiothoracic surgeons without hesitation • Maintain nutrition,hydration,prevent infection,anemia . • Train and demonstrate “knee-chest” position to mother/attendents to abolish cyanotic spells.
  76. 76. Knee chest positions
  77. 77. Thank You

×