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Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
Approach to Cyanotic Congenital Heart Diseases
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Approach to Cyanotic Congenital Heart Diseases

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  • 1. - CSNVittal Approach to Cyanotic Congenital Heart Diseases
  • 2. Cyanosis Bluish discoloration of the mucous membrane and skin Visible clinically when arterial O2 saturation falls to 60-65% Primarily depends on the amount of reduced Hb – must be at least 5g % - provided absolute Hb is normal (Lunds Gaard & Vanslyke) Physiological cyanosis may be normal till 20 min. but beyond that – it is always abnormal Central cyanosis desaturation of arterial blood. Peripheral cynosis normal arterial oxygen circulation.
  • 3. Cyanosis - Types Central – cyanotic CHD Peripheral – hypothermia, CCF Mixed Cyanosis – CHD in Shock Differential cyanosis – PDA with reversal Reverse differential cyanosis – TGA with PDA with reversal Intermittent Cyanosis – Ebsteins anomaly Circum oral cyanosis Cyclical cyanosis – Bilateral choanal atersia
  • 4. Complications of Cyanosis / Cyanotic CHD Clubbing Cyanotic Spell Depressed IQ Infective endocarditis Polycythemia Embolic phenomenon
  • 5. Cyanosis - Etiology A] Cyanotic Congenital Heart Diseases with  Pulm. Vascularity 1. With RVH  Severe PS with ASD  Pulm atresia (with or without VSD)  TOF 1. With LVH  Tricuspid atresia  Pulm. Atresia and hypoplastic Rt. Ventricle 1. With combined ventricular hypertrophy  TGA with PS  Truncus arteriosus with hypoplastic pulm. Arteries 1. With neither ventricle predominant  Ebstein’s anomaly
  • 6. Cyanosis - Etiology B] Cyanotic Congenital Heart Diseases with  Pulm. Vascularity 1. With RVH  Hypoplastic Lt. Heart syndrome  TAPVR  TGA  Double outlet Single (Rt.) ventricle 1. With Lt. / Combined ventricular Hypertrophy  TGA + VSD  Single ventricle  Tricuspid atresia with transposition  Truncus arteriosus
  • 7. Cyanosis - Etiology C] Non Cardiac Causes 1. Pulmonary  RDS in newborns  Pneumonia  Meconium aspiration  Pneumothorax  Chylothorax  Diagrphmatic hernia  Hemothorax  Lobar emphysema  Pleural effusion  Pulm. Telangectasia  Bronchogenic cyst
  • 8. Cyanosis - Etiology C] Non Cardiac Causes 1. Hematological  Abnormal hemoglobins o Hb Kansas o HB Beth Isreal o Hb M variants  Hereditary methemoglobinemia
  • 9. Cyanosis : D D CardiacCardiac RespiratoryRespiratory Tachypnoea RR Less ( 40 – 60) More ( > 60 ) Respiratory distress Absent Present 100 % Oxygen No response PO2 > 150 torr
  • 10. Cyanosis with NO CCF TOF Pulmonary atresia with VSD Tricuspid atresia Transposition of great vessels with pulmonary stenosis
  • 11. Cyanosis with Heart Failure Complete Transposition of great arteries Truncus arteriosus Single ventricle
  • 12. Foetal Circulation
  • 13. Cyanotic Congenital Heart Disease Increased Pulmonary Blood Flow Cyanosis, Clubbing, Polycythemia Decreased Pulmonary Blood Flow Transposition of Great arteries (3-5%) Truncus Arteriosus (1-2%) Single Ventricle (1-2%) TAPVC (1-2%) HLHS (1-3%) Tetralogy of Fallot (5-7%) Tricuspid Atersia Ebstein’s Anomaly Pulmonary Atersia % given for 100 CHDs
  • 14. Approach to a Child with cyanotic CHD History:  H/o. cyanotic spells (TOF, Ebstein anomaly)  Cyanosis increased during defecation, feeding.  H/o. squatting  Easy fatigability  Failure to thrive  Increased metabolic activity  Recurrent infections due to  pulm flow  Decreased intake  Associated chromosomal anomaly  Chest pain  Syncope : TOF, Eisenmenger’s  Hemoptysis : Eisenmenger’s  Cough, breathlessness & repeated chest infections due to  pulm flow  TAPVC, TGA, Truncus arteriosus  Convulsions : - Cerebral abscess – TOF after 2 yrs.
  • 15. Approach to a Child with cyanotic CHD Prenatal History: Consanguinity Age of parents (esp. mother) Down’s – more in maternal age > 35 (endocardial cushion defects) Maternal medication during pregnancy Sod. valproate – TOF, VSD Lithium – ASD, tricuspid atresia, Ebstein’s Marijuana – Ebstein’s Clomiphene – TOF Antitussive - ASDs Sex hormones – VSD, TGA, TOF
  • 16. Approach to a Child with cyanotic CHD Age of the Patient: 1. Cyanosis during newborn period 1. Hypoplastic LV 2. TGA with intact ventricular septum 3. Tricuspid atresia 4. Hypoplastic Rt. Ventricle with pulm. Atresia 5. TOF (severe type) 2. Cyanosis develops during 1st week : 1. Pulmonary atresia 2. Tricuspid atresia 3. TGA 3. Cyanosis after 1 month 1. TOF 2. TGA, TAPVC
  • 17. Approach to a Child with cyanotic CHD Sex of the Patient: 1. Equal in both sexes 1. Ebstein’s anomaly 2. Pulmonary atresia 3. Tricuspid atresia 4. Truncus arteriosus 5. TOF 6. Cong. venacaval – Lt atrial communication 2. Females – more common: 1. Endocardial cushion defects 3. Males – more common 1. Univentricular heart 2. TGA 3. Hypoplastic left heart
  • 18. Approach to a Child with cyanotic CHD General Examination: Trisomy 21 – Endocardial cushion defects Cat cry syndrome – Tricuspid atresia Di George syndrome – Truncus arteriosus (hypoplastic mandible, defective ears and short filtrum) Marfan’s and Down syndrome – TOF Anomalies of 16 – 18 chromosomes – DORV Clubbing of fingers – takes 6 mo. to develop Differential clubbing – Eisenmenger’s syndrome – only in toes Short stature, kyphoscoliosis, arthropathy, congested eyes, bad teeth, growth retardation
  • 19. Approach to a Child with cyanotic CHD Jugulars Examination: Enlarged Tricuspid atresia Hypoplastic left ventricle TAPVR Endocardial cushion defect TGA with increased pulm. blood flow DORV with increased pulm. blood flow Truncus arteriosus Normal jugulars Fallot’s tetrology TGA with PS DORV with PS
  • 20. Approach to a Child with cyanotic CHD Pulse Collapsing Truncus arteriosus AV malformations Severer TOF with collaterals Taussig-bing operation fort OF Decreased Lt. carotid and Lt. brachial pulses Hypoplastic Lt. heart syndrome Decreased femoral pulses DORV
  • 21. Approach to a Child with cyanotic CHD Precardial Examination Normal TOF, DORV, Pulm. atresia with intact ventricular septum, tricuspid atresia Precardial bulge TAPVR, Hypoplastic Lt. ventricle, PS with intact IVS TGA
  • 22. Approach to a Child with cyanotic CHD Auscultation Splitting of S 1 : Ebstein’s anamoly Loud P 2 : TAPVR, Endocardial cushion defect, Eisenmenger syndrome, DORV with  pulm. flow Single S 2 : TOF, TOF with PS, DORV with PS, Tricuspid atresia, Truncus arteriosus Muffled S 2 : Ebstein’s anamoly Continuous murmur : TOF with collaterals, Truncus arteriosus
  • 23. Approach to a Child with cyanotic CHD Radiology Boat shaped heart : TOF (coeur en sabot) Enlarged heart : Severe TOF with collaterals, associated anemia or hypertension Concavity in pulm. artery area : PS Rt. sided aortic arch : 25% cases of TOF Rib notching : TOF with collaterals Egg on end appearance : TGA Figure of Eight appearance : TAPVR
  • 24.  Commonest Cyanotic HD  10% of CHDs Nada’s dictum: If a cyanotic heart disease child over 2 years is admitted in ward, if it is diagnosed as TOF it is 75% correct Taussing’s axiom: If a patient is having cyanosis with normal sized heart and oligemic lung fields it is definitely TOF
  • 25. 1. Pulmonary stenosis (infundibular) 2. VSD 3. Rt. Ventricular hypertrophy 4. Overriding aorta Fallot’s Pentology TOF + ASD Fallot’s Trilogy ASD with patent foramen ovale RVH PS
  • 26. History  Nicholas Steno 17th century – originally described TOF  In 1948, Etinne, Louis Arthur Fallot described the four anatomical features of TOF  In 1947 Taussig described various positions for relief of the dyspnoea
  • 27. Etiology:  Extracardiac manifestations in 10%  Syndromes associated with  Apert’s anomaly (TOF, VSD, CA)  Silver syndrome (TOF, VSD)  Goldenhar syndrome (TOF, VSD, ASD)  De Lange syndrome ( VSD, TOF, PDA, DORV)  Fetal hydantoin syndrome  Fetal carbamazepine syndrome  Fetal alcohol syndrome  Maternal phenylketonuria (PKU) birth defects  CATCH 22 – Cardiac defects, abnormal facies, thymic hypoplasia, cleft palate, hypocalcemia  Maternal drugs/diseases associated with  Trimethadone, Sex hormones, Thalidomide
  • 28. Associations:  RVOT  infundibular 45%  valve level 10%  combined 30%  atretic 15%  Pulmonary annulus and MPA hypolastic.  Right aortic 25%.  5% cases abnormal coronary arteries.
  • 29. Classification:  Group I  Severe PS or pulm. atresia  Pt. symptomatic at birth  Mortality high  Group II  Classical TOF.  Pt. becomes symptomatic from 6 mo. of life  Group III  Pink Fallot’s tetrology  Pt. becomes symptomatic between 4 – 5 years
  • 30. Hemodynamcis:  Pressure in both ventricles equal  Pulm blood flow depends on severity of PS  In VSD with mild PS, blood flow from Lt to Rt ventricle throughout systole  In TOF with severe PS, most of Rt. ventricular blood enters into aorta resulting in cyanosis and hypoxia
  • 31. Tetrology of Fallot
  • 32. Hemodynamics - Squatting & TOF During activity → respiration (hyperpnea)↑ Venous return increases but Pulmonary→ flow cannot increase Right to Left shunt→ → Cyanosis On squatting, venous return decreases → Systemic vascular resistance increases, therefore less or no right to left shunt → decreases cyanosis
  • 33. Clinical Symptoms:  Dyspnoea – not associated with cough  Cyanosis – at birth or from 6 mo. of age  Cyanosis on exertion  Squatting  Cyanotic spells  Growth retardation
  • 34. Polycythemia
  • 35. Clubbing
  • 36. Squatting Position
  • 37. Presence of CCF ExcludesPresence of CCF Excludes Tetralogy Physiology exceptTetralogy Physiology except when complicated bywhen complicated by Anemia Infective Endocarditis Valvar Regurgitation Surgically created or naturally occurring large left to right shunts Systemic hypertension Unrelated or coincidental myocardial disease
  • 38. Investigations:- CXR  coeur en sabot  Rt. sided aortic arch  Aneurysmal bulging of Pulm. artery (with absence of pulm. valves)  Oligemic lung fields  Rib corrosions – with collaterals  Unilateral lesions – after Blalock- Taussig operation
  • 39. Investigations:- ECG  RAD  RVH  Sudden transition from V1 to V2 (R wave in V1 is suddenly replaced by S wave in V2)
  • 40. Electrocardiography Right axis deviation, Right ventricular hypertrophy
  • 41. Echocardiogram Apical Parasternal
  • 42. Echocardiograp hy
  • 43. Chest roentgenogram Normal size heart Pulmonary vascular markings are decreased Concave main pulmonary artery segment with an upturned apex – BOOT shaped heart or coeur en sabot Right atrial enlargement (25%) Right aortic arch (25%)
  • 44. Investigations: Blood  Polycythemia  Spontaneous thromboses  Microcytic hypochromic anemia
  • 45. Other Complications 1. Cerebral venous thrombosis 2. Cerebral abscess  Entering of contaminated blood in to systemic circulation without being filtered in lungs  Infarction of brain due to polycythemia  Hypoxia – leading to anaerobic infection  Infective endocarditis can disseminate infection in cerebrum 1. Infective endocarditis
  • 46. Other Complications 5. Arthropathy  Gout arthropathy due to production of uric acid from RBC due to polycythemia 5. Acute renal failure  Uric acid nephropathy 7. Bleeding diathesis  Decrease in coagulants due to shrinkage of plasma  Decreased platelet adhesion and production 7. Delayed puberty
  • 47. Natural history of Fallot’s Tetrology Acyanotic TOF – gradually become cyanotic Patients already cyanotic become deeply cyanosed as infundibular stenosis increases and polycythemia develops Hypoxic spells develop in infants Severe TOF patients may develop AR Growth retardation in severe cases Coagulopathy develops in long standing cases Iron deficiency anemia develops slowly
  • 48. Tetrology of Fallot Medical Therapy : Maintain Hb > 14 g /dL (oral iron or bl. Transfusion) Beta blockers to be given in highest tolerated doses ( 1-4 mg/kg/d in 2-3 divided doses)
  • 49. Tetrology of Fallot  Timing of Surgery :  Stable, minimally cyanosed : Total correction at 1-2 yrs of age  Significant cyanosis (SaO2 < 70%) or H.o spells despite therapy  < 3 mo. : systemic to pulm. art shunt (class I)  > 3 mo. : shunt or correction depending on anatomy and surgical center’s experience (class I)  VSD with pulm. Atresia. Adequate Pas: Repair at 3-4 yrs, if RV to pulm. art. conduit required (class I) Systemic to pulm. artery shunt if symptomatic earlier and repair without conduit is not possible
  • 50. 1. Blalock – Taussig Operation: Anastamosis between subclavian artery with ipsilateral pulmonary artery. In Rt. sided aortic arch, anastamosis is done between Rt. subclavian artery and pulm. artery. 1. Waterston’s Operation: Anastamosis between ascending aorta and pulm. artery. 1. Pott’s Operation: Anastamosis between descending aorta and left branch of pulm. artery. 1. Gore-Tex Interposition shunt: Placed between the subclavian and ipsilateral PA, done even in small infants younger than 3 months
  • 51. Blalock Taussig Shunt
  • 52. Corrective Surgery:  Reconstruction of infundibular area  Closure of VSD  Trabeculotomy Indications 1. In symptomatic patients between 3-4 months 2. In asymptomatic mildly cyanosed patients between 3 – 24 months 3. Mildly cyanosed patients with Blalock-Taussig shunt correction between 1-2 years 4. Asymptomatic acyanotic patients – between 1-2 years
  • 53. Tetrology of Fallot Timing of Surgery :  Stable, minimally cyanosed : Total correction at 1-2 yrs of age  Significant cyanosis (SaO2 < 70%) or H.o spells despite therapy  < 3 mo. : systemic to pulm. art shunt (class I)  > 3 mo. : shunt or correction depending on anatomy and surgical center’s experience (class I)  VSD with pulm. Atresia. Adequate Pas: Repair at 3-4 yrs, if RV to pulm. art. conduit required (class I) Systemic to pulm. artery shunt if symptomatic earlier and repair without conduit is not possible
  • 54. Postoperative complications Congestive heart failure (right or left, residual outflow obstruction, VSD, and/or pulmonic regurgitation Atrial flutter, ventricular arrhythmias, right bundle-branch block, or left anterior hemiblock Infective bacterial endocarditis
  • 55. If the patient is severely cyanosed with group I Fallot’s tetrology should be given prostaglandin infusion to keep ductus arteriosus patent for augmentation of blood supply to pulmonary artery.
  • 56. Cyanotic spell (tet spell)  Medical emergency  6 mo. To 2 years  Early hours  Crying, feeding, defecation and relative anemia precipitate Mechanism :  Local intracardiac production of catecholamines increased due to stress – which increase infundibular spasm leading to more R > L shunt  Vulnerable resp. centre  Paroxysmal attack of arrhythmias
  • 57. Cyanotic spell (tet spell) Treatment: 1. Morphine : 0.1 mg/kg SC (stabilizes resp. centre and calms) 2. Propronalol : 0.1 mg /kg IV dil. in 50 ml of 5% D/W 3. Methoxamine : 40 mg. dil in 250 ml of 5% D/W - IV 4. Ketamine : 1 to 3 mg/kg IV over 60 min (increases systemic vascular resistance and calms patient) 5. Phenylephrine : 0.1 to 0.2 mg / kg IV – (increases systemic vascular resistance)
  • 58. Cyanotic spell (tet spell) Supportive Treatment: 1. Knee chest position 2. O2 inhalation 3. Sodium bicarbonate for acidosis Prevention : 1. Propronalol : 1 - 4 mg/kg up to 2 years 2. Iron supplementation : 1 mg/kg/d
  • 59. Polycythemia – Management: 1. Avoid dehydration 2. Iron therapy 3. Phlebotomy – if Hct. > 65 – 70%
  • 60. Prophylaxis for Infective Endocarditis 1. Maintain oral hygiene 2. Administer prophylactic antibiotic IV immediately before cardiac surgery and continue for 1-2 days postoperatively 3. Administer appropriate antibiotic for any dental procedure or surgical procedures
  • 61. Infective Endocarditis Prophylaxis Every child with CHD must be advised to maintain good oral hygiene and regular dental check up Unrepaired CCHDs are high risk conditions for IE. So prophylaxis is mandatory ASD (secundum type) and valvular PS are low risk conditions for IE – prophylaxis not recommended Other acyanotic CHDs including bicuspid aortic valve are moderate risk & prophylaxis is recommended Repaired CHDs with prosthetic material need prophylaxis for first 6 mo. after procedure Device placement by transcatheter route also require prophylaxis for the first 6 mo.

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