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Clinical approach to congenital heart disease diagnosis

approaching diagnosis of congenital heart diseases clinically

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Clinical approach to congenital heart disease diagnosis

  1. 1. CLINICAL APPROACH TO THE DIAGNOSIS OF CONGENITAL HEART DISEASE
  2. 2. INCIDENCE OF CHD  6 PER 1000 LIVE BIRTHS - FOR MODERATE AND SEVERE FORMS OF CHD.  19 PER 1000 LIVE BIRTHS - IF POTENTIALLY SERIOUS BICUSPID AORTIC VALVE IS INCLUDED.  75 PER 1000 LIVE BIRTHS -IF VERY SMALL MUSCULAR VSDS ARE INCLUDED.
  3. 3. CLINICAL DIAGNOSIS OF CHD  REPRESENTS THE EPITOME OF APPLIED LOGIC.  DIAGNOSES EMERGE WITH GRATIFYING FREQUENCY WHEN CORRECT INFERENCES ARE DRAWN FROM ACCURATE OBSERVATIONS.  IT IS A STIMULATING CHALENGE AND A CONSTANT SOURCE OF SELF EDUCATION.
  4. 4. CLINICAL CLASSIFICATION OF CONGENITAL HEART DISEASE 1.ACYANOTIC WITHOUT A SHUNT (NORMAL OR DECREASED PULMONARY ARTERIAL BLOOD FLOW) A.MALFORMATIONS ORIGINATING IN THE RIGHT SIDE OF THE HEART (FROM MOST PROXIMAL TO MOST DISTAL.) B.MALFORMATIONS ORIGINATING IN THE LEFT SIDE OF THE HEART (FROM MOST PROXIMAL TO MOST DISTAL.) 2.ACYANOTIC WITH A SHUNT (LEFT TO RIGHT, INCREASED PULMONARY ARTERIAL BLOOD FLOW) SHUNT AT ATRIAL LEVEL SHUNT AT VENTRICULAR LEVEL SHUNT BETWEEN AORTIC ROOT AND RIGHT SIDE OF HEART SHUNT AT AORTOPULMONARY LEVEL SHUNT AT MORE THAN ONE LEVEL
  5. 5. CLINICAL CLASSIFICATION OF CONGENITAL HEART DISEASE CYANOTIC  INCREASED PULMONARY ARTERIAL BLOOD FLOW  NORMAL OR DECREASED PULMONARY ARTERIAL BLOOD FLOW  DOMINANT LEFT VENRICLE  DOMINANT RIGHT VENTRICLE  WITH PULMONARY HYPERTENSION  WITHOUT PULMONARY HYPERTENSION  NORMAL OR NEARLY NORMAL VENTRICLES.
  6. 6. DIAGNOSIS OF CHD- FIVE BASIC QUESTIONS  IS THE PATIENT ACYANOTIC (OR) CYANOTIC?  IS PULMONARY ARTEIAL BLOOD FLOW INCREASED (OR) NOT ?  DOES THE MALFORMATION ORIGINATE IN THE LEFT (OR) RIGHT SIDE OF THE HEART ?  WHICH IS THE DOMINANT VENTRICLE ?  IS PULMONARY HYPERTENSION PRESENT (OR) NOT ?
  7. 7. DIAGNOSIS OF CONGENITAL HEART DISEASE  HISTORY  GENERAL EXAMINATION  EXAMINATION OF PRECORDIUM  CHEST SKIAGRAM  ECG
  8. 8. HISTORY  DEVELOPMENT AND WEIGHT GAIN  FEEDING DIFFICULTY, TACHYPNEA, DYSPNEA  FREQUENT RESPIRATORY INFECTIONS  CYANOSIS AND CYANOTIC SPELLS, SQUATTING  EXERCISE INTOLERANCE  CHEST PAIN, SYNCOPE, PALPITATIONS  NEUROLOGICAL SYMPTOMS  ANTENATAL HISTORY  FAMILY HISTORY
  9. 9. HISTORY- ONSET OF CYANOSIS EARLIEST ONSET • D-TGA • PA,INTACT SEPTUM • PA,VSD • OBSTRUCTED TAPVC • TRICUSPID ATRESIA • EBSTEIN'S ANOMALY LATE ONSET • EISENMENGER PHYSIOLOGY • TAPVC • EBSTEIN'S ANOMALY • PS, STRETCHED PFO
  10. 10. PAROXYSMAL HYPOXIC SPELL FOUND IN  TETROLOGY OF FALLOT  OTHER DISEASES WITH FALLOT’S PHYSIOLOGY  PULMONARY ATRESIA WITH VSD COMMENST AGE  4MONTH TO 12 MONTH OF AGE RARE BEYOND 2YEARS OF AGE FEAUTURES  OCCURS USALLY IN THE MORNING, AFTER A GOOD SLEEP.  PRECIPITATED BY FEEDING , CRYING & BLADDER AND BOWEL MOVEMENTS  HYPERPNEA INCREASES, CYANOSIS DEEPENS. MAY DEVELOP SYNCOPE - CONVULSION-CVA MACHANISMS  INFUNDIBULAR PULMONARY SPASM  OVER REACTION OF IMMATURE RESPIRATORY CENTER.  PARAOXYSMAL ATRIAL TACHYCARDIA.
  11. 11. SQUATTING  AGE OF OCCURRENCE  MACHANISMS RIGHT - LEFT SHUNT IS DECREASED DUE TO 1. DECREASED VENOUS RETURN 2. INCREASD SVR DUE TO COMPRESSION OF FEMORAL ARTERY  SQUATTING EQUIVALENTS 1. KNEE-CHEST POSITION 2. SITTING WITH LEGS DRAWN UNDERNEATH 3. STANDING WITH CROSSED LEGS.
  12. 12. Squatting Position
  13. 13. SYMPTOM EVALUTION FOR CHD SYMPTOM CONGENITAL HEART DISEASES EXERTIONAL ANGINA AS, PS, PPH, ALCOPA EXERTIONAL SYNCOPE AS, PS. CYANOSIS WITH SYNCOPE TOF DYSPHAGIA DOUBLE AORTIC ARCH, ANOMALOUS ORIGIN OF RIGHT SUBCLAVIAN ARTERY PASSING BELOW OESOPHAGUS. CVA IN A CYANOTIC CHILD CYANOTIC CHD COMPLICATED BY CEREBRAL ABSCESS, CEREBRAL THROMBI & PARADOXICAL
  14. 14. ANTENATEL HISTORY MATERNAL DISEASES CARDIAC MALFORMATIONS IN THE NEW BORN MATERNAL RUBELLA (IN THE FIRST TRIMESTER OF PREGNANCY) CONGENITAL RUBELLA SYNDROME - PDA, PERIPHERAL PULMONARY ARTERY STENOSIS, VPS, ASD. MATERNAL LUPUS ERYTHMATOSUS CONGENITAL COMPLETE HEART BLOCK MATERNAL DIABETUS TGA, VSD, COMMON ATRIUM, CARDIOMEGALY,CARDIOMYOPATHY
  15. 15. ANTENATAL HISTORY – TERATOGENIC DRUGS TERATOGENIC DRUGS CARDIAC MALFORMATIONS EXTRA CARDIAC ABNORMALITIES THALIDOMIDE VARIABLE -TF,VSD,ASD,TA PHOCOMELIA LITHIUM EBSTEIN'S ANOMALY OF TRICUSPID VALVE, TRICUSPID ATRESIA NONE ISORETINOIN VSD ALCOHOL ABUSE FOETAL ALCHOL SYNDROME - VSD (45% OF INFANTS), PDA, ASD MICROCEPHALY, GROWTH AND MENTAL RETARDATION, SMOOTH PHILTRUM, THIN UPPERLIP EPTOIN FOETAL HYDANTION SYNDROME - PS, AS, COARCTATION OF AORTA, PDA MICROCEPHALY, GROWTH AND MENTAL RETARDATION, SHORT PALPABERAL FISSURES, SMOOTH PHILTRUM, THIN UPPERLIP
  16. 16. FOETAL ALCOHOL SYNDROME AFFECTS 30-40% OF CHILDREN BORN TO ALCOHOLIC MOTHER CLINICAL FEATURES - UNDER DEVELOPED- APPEARING CENTRL FACE DUE TO MAXILLARY HYPOPLASIA - SMALL AND UPTURNED NOSE - INDISTINCT (OR) SMOOTH PHILTRUM - MICROGNATHIA - THIN UPPER LIP AND VERMILON CARDIAC ANOMALIES - VSD - ASD
  17. 17. Physical Examination • General appearance, weight and nutrition • Association with chromosomal syndromes and other systemic malformations • Colour • Vital Signs – Pulse, BP, respiration and temperature
  18. 18. GENDER PREDISPOSITION OF CHD MALE 1. VALVULAR A.S 2. CORACTATION OFAORTA 3. TGA 4. HYPOPLASTIC LEFT HEART SYNDROME 5. PULMONARY AND TRICUSPID ATRESIAS 6. TOF FEMALE 1. ASD 2. PDA 3. EBSTENIS ANOMALY OF TRICUSPID VALVE.
  19. 19. GENERAL EXAMINATION GENERAL APEARANCE 1. SHORT STATURE -TURNER SYNDROME, NOONAN SYNDROME, -ELIS–VAN CREVELD SYNDROME. 2. POORLY DEVELOPED - COARCTATION OF AORTA LOWER EXTREMITIES HEAD & FACE 1. HYPERTELORISM [WIDELY SET EYES] -NOONAN SYNDROME -TURNER SYNDROME (BCAV, COARC.AORTA) -MULTIPLE LENTIGENES SYNDROME (P.S, HOCM) -WILLIAM SYNDROME (SUPRA VALVULAR A.S) 2. WEBBING OF THE NECK -TURNER SYNDROME, NOONAN SYNDROME 3. LOW SET EARS - DOWN SYNDROME, WILLIAM SYNDROME, - NOONAN SYNDROME 4. LOW POSTERIOR HAIRLINE -TURNER SYNDROME, NOONAN SYNDROME 5. TYPICAL FACIAL APPEARANCE -MONGOLIAN FACIES -DOWN SYNDROME - ELFIN FACIES -WILLIAM SYNDROME
  20. 20. HYPERTELORISM  CLINICAL MEASURMENTS 1. INTERPUPILLARYDISTANCE (IPD) . 2. INNER INTERCANTHAL DISTANCE (ICD) 3. OUTER INTERCANTHAL DISTANCE (OCD)  RADIOLOGICAL MEASUREMENTS 1. INNER INTERORBITAL DISTANCE (IID). 2.OUTER INTERORBITAL DISTANCE (OID)
  21. 21. HYPERTELORISM  ORBITAL HYPERTELORISM INCREASED OUTER CANTHAL DISTANCE (OR) OUTER INTER ORBITEL DISTANCE.  OCCULAR HYPERTELORISM INCREASED IPD.  TELECANTHUS 1. LARGE DISTANCE BETWEEN TWO MEDIAL CANTHI, WHEN COMPARED TO INTER ORBITAL DISTANCE. (NORMAL ADULT-30MM) 2. MUSTARDE RATIO ICD/IPD > 0.55 3. SUSPECTED WHEN LOWER LID PUNCTA IS LATERAL TO THE MEDIAL EDGE OF IRIS IN STRAIGHT GAZE.
  22. 22. LOW SET EAR - MEASUREMENTS  AN IMAGINARY LINE IS DRAWN FROM THE OUTER CANTHUS TO EXTERNAL OCCIPITAL PROTUBERANCE (OR)  DRAW A LINE JOINING INNER AND OUTER CANTHUS AND EXTENDING IT.  IF THE SUPERIOR ATTACHMENT OF THE PINNA IS -AT (OR) ABOVE THIS LINE → NORMAL -BELOW THIS LINE → LOW SET EARS
  23. 23. LOW HAIRLINE - MEASUREMENTS TWO CRITERIAS  POSTERIOR HAIRLINE IS BELOW THE LEVEL OF 5TH CERVICAL SPINOUS PROCESS.  RATIO OF DISTANCE BETWEEN EXT OCCIPITAL PROTUBERANCE TO POSTERIOR HAIR LINE & DISTANCE BETWEEN POSTERIOR HAIRLINE TO C7 SPINOUS PROCESS. = > 1/6 IN MEN > 1/4 IN WOMEN
  24. 24. SHORT NECK BIRD’S INDEX  RATIO BETWEEN THE HEIGHT AND DISTANCE BETWEEN THE EXTERNAL OCCEPITAL PROTRUBERANCES TO THE C7 SPINOUS PROCESS.  NORMAL RATIO – BELOW 12.8  SHORT NECK IS WHEN THE RADIO IS > 13.6.
  25. 25. TURNER SYNDROME Row 1 Row 2 Row 3 Row 4 0 2 4 6 8 10 12 Column 1 Column 2 Column 3 CHROMOSOMAL PATTERN - 45 XO CLINICAL FEATURES -PHENOTYNE- FEMALE, SHORT STATURE,WEBBED NECK, LOW HAIR LINE,SMALL CHIN - PROMINENT EARS (LARGE AURICLES) - BROAD CHEST WITH WIDELY SPECED NIPPLES, CUBITUS VALGUS, SHORT 4TH METECARPALS AND METATARSALS, NARROW HYPER CONVEX NAILS, PIGMENTED NEVI, ABSENT AUXILLARY AND PUBIC HAIR (OVARIAN DYSGENESIS) CARDIAC ANOMALIES - COARCTATION OF AORTA (MORE COMMEN IN TURNER WITH WEBBING OF THE NECK.) - BICUSPID AORTIC VALVE WITH AS.
  26. 26. NOONAN SYNDROME CLINICAL FEATURES - PTOSIS, DOWNWARD SLANTING EYES - HYPERTELORISM. LOW SET EARS, - LOW POSTERIOR HAIR LINE, - MICROGNATHIA, WEBBED NECK, FLAT CHEST, SHORT STATURE, MENTAL RETARDATION, HYPOSPADIASIS,SMALL UNDESCENDED (OR) CRYPTORCHID TESTES. - NORMAL CHROMOSOMES. CARDIAC ANOMALIES - DYSPLASTIC PULMONARY VALVE STENOSIS (60%) - HYPERTROPHIC CARDIOMYOPATHY(20%).
  27. 27. EXAMINATION OF EYES EYES EXTERNAL APPEARANCE 1. PTOSIS - NOONAN SYNDROME 2. BRUSHFIELD SPOTS - DOWN SYNDROME 3. COLOBOMA OF IRIS - CAT'S EYE SYNDROME (TOF,TRICUSPID ATRESIA, ASD & VSD) 4. PREMATURE CATARACT – CONGENITAL RUBELLA SYNDROME INTERNAL APPERANCE RETINA 1. TORTOUS RETINAL ARTERIES - COARCTATION OF AORTA WITH 'U' TURNS WITHOUT CHARACTERISTIC CHANGES OF HYPERTENSIVE RETINOPATHY
  28. 28. CONGENITAL RUBELLA SYNDROME DUE TO 1ST TRIMESTER MATERNAL RUBELLA INFECTION CLINICAL FEATURES -CONGENITAL CATARACT -CONGENITAL DEAFNESS -MENTAL DEFICENCY - MICROCEPHALY - PROLEFERATIVE VASCULAR LESIONS. CARDIAC ANOMALIES - PDA , ASD. - PULMONARY VALVULAR AND ARTERIAL STENOSIS.
  29. 29. EXAMINATION OF MOUTH MOUTH LIPS 1. ABSENT PHILTRUM - FOETAL ALCOHOL SYNDROME 2. LONG PHILTRUM - WILLIAM SYNDROME 3. BLUISH DISCOLOURATION - CYANOSIS MUCUS MEMBRANE 1.CLUSTERS OF SMALL RUBY PATCHES - HERDITARY TELANGIECTASIA (RENDU – OSLER- WEBER SYNDROME) TEETH 1. MALFORMED TEETH - WILLIAM SYNDROME 2. PREMATURELY ERUPTED TEETH - ELIS VAN CREVELD SYNDROME (PRESENT AT BIRTH + GINGIVAL HYPERTROPHY + MULTIPLE FRENULA TONGUE 1. LONG PROTRUDING TONGUE - DOWN SYNDROME 2. HIGH ARCHED PALATE - NOONAN SYNDROME 3. CLEFT OF SOFT PALATE - VELOCARDIOFACIAL SYNDROME (VSD)
  30. 30. WILLIAMS SYNDROME NON FAMILIAL SUPRA VALVULAR AS CLINICAL FEATURES - FACE IS DIAGNOSTIC - HEAD IS SMALL (ELF – LIKE APPERANCE) - CHEEKS ARE FULL AND BAGGY - MOUTH AND FOREHEAD ARE LARGE - CURVED LIPS, PEG SHAPED, WIDELY SPACED TEETH - MENTAL RETARDATION CARDIAC ABNORMALITES - SUPRA VALVULAR AS - PULMONARY ARTERY BRANCH STENOSIS.
  31. 31. HEREDITARY TELANGIECTASIA
  32. 32. EXAMINATION OF EXTREMITIES A. COLOUR 1. CYANOSIS & CLUBBING - CYANOTIC CONGENITAL HEART DISEASES 2. DIFFERENTIAL CYANOSIS - PDA WITH SEVERE PHT WITH Rt to Lt SHUNT 3. REVERSED DIFFERENTIAL - D- TGA WITH PHT WITH PREDUCTAL COARCTATION OF CYANOSIS AORTA WITH REVERSED FLOW THROUGH PDA. B. STRUCTURE 1. POLYDACTYLY - ELIS – VAN – CREVELD SYNDROME. - LAWRENCE MOON BIDEL SYNDROME. -TURNER SYNDROME. 2. SYNDACTYLY - ELIS- VAN CREVELD SYNDROME. 3. FINGERIESD THUMB - HOLD- ORAM SYNDROME. 4. BROAD THUMB & TOES - RUBINSTEIN – TAYBI SYNDROME (PDA) 5. SIMIAN PALMAR CREASE - DOWN SYNDROME. 6. CUBITUS VALGUS DEFORMITY - TURNER SYNDROME. 7. ROCKER – BOTTOM FEET - TRISOMY – 18 (PDA , VSD)
  33. 33. CARDIAC DEFECTS CAUSING CENTRAL CYANOSIS  TRANSPOSITION OF THE GREAT ARTERIES  TETRALOGY OF FALLOT  TRICUSPID ATRESIA  TRUNCUS ARTERIOSUS  TOTAL ANOMALOUS PULMONARY VENOUS RETURN.  EBSTEIN ANOMALY  EISENMENGER PHYSIOLOGY  CRITICAL PULMONARY STENOSIS (OR) ATRESIA FUCTIONALLY SINGLE VENTRICLE.
  34. 34. SYMMETRIC CYANOSIS
  35. 35. DIFFERENTIAL CYANOSIS
  36. 36. DOWN SYNDROME CHROMOSOMAL ABNORMALITY-TRISOMY 21 CLINICAL FEATURES - SHALLOW ORBITS - EPICANTHAL FOLDS, HYPERTELORISM - BRUSHFIELD SPOTS (SPECKLED IRIS) - PROTRUDING TONGUE, MENTAL RETARDATION - LOWSET EARS, TRANSVERSE PALMAR CREASE. CARDIAC ABNORMALITIES - ENDOCARDIAL CUSHION DEFECTS (2/3 OF CASES) - VSD.
  37. 37. TRISOMY-18 SYNDROME
  38. 38. HOLT–ORAM SYNDROME INHERITENCE: - AUTOSOMAL DOMINENT CLINICAL FEAUTURES - -HYPOPLASTIC THUMB WITH AN ACCESSORY PHALANX (TRIPHALANGISM) --THUMB MAY BE RUDIMENTARY (OR) ABSENT --METACARPAL BONE MAY BE SMALL (OR) ABSENT --HYPOPLASTIC RADIUS. CARDIAC ANOMALY - OSTIUM SECUNDOM ASD.
  39. 39. ELLIS – VAN CREVELD SYNDROME INHERITENCE  AUTOSOMAL RECESSIVE CLINICAL FEATURES  DWARFISM WITH POLYDACTYLY OF THE HANDS (INVARIABLE)  POLYDACTYLY OF THE FEET (10%)  POLYCARPALY (9 OR 10TH CARPEL BONE)  CLINODACTYLY (BENT FINGERS)  SYNDACTYLY (INTERDIGITAL WEBBING)  HYPOPLASIA OF THE NAILS  PREMATURE ERUPTION OF MALFORMED MAXILLARY INCISORS; GINGIVAL HYPETORPHY AND MULTIPLE FRENULA CARDIAC ANOMALY  COMMON ATRIUM, LARGE ASD.
  40. 40. Pulse and BP • Examine pulse and BP in all four limbs. • Weak lower limb pulses suggestive of coarctation of aorta. • BP compared against age specific percentile curves.
  41. 41. JUGULAR VENOUS PLUSE (JVP)  TOF: INCONSPICUOUS; AGEING , SYSTEMIC HTN, ETC. CAN PRODUCE PROMINENT A WAVE. POSTOPERATIVE : A AND V WAVES.  PROMINENT A WAVE: TRICUSPID ATRESIA, PULMONARY ATRESIA WITH INTACT SEPTUM AND PS WITH STRETCHED PFO. EISENMENGER PHYSIOLOGY: PROMINENT A AND V WAVES; SOMETIMES V WAVE CAN BECOME VERY PROMINENT, SIMULATING VENOUS CORRIGAN.  TAPVC: PROMINENT V WAVE.  EBSTEIN’S ANOMALY: PROMINENT A AND V WAVES ARE OCCASIONALLY FOUND DUE TO HYPOKINETIC TR AND COMMODIOUS RIGHT ATRIUM.
  42. 42. EXAMINATION OF THORAX- INSPECTION 1.PRECORDIAL BULGE - CARDIAC ENLARGEMENT BEFORE PUBERTY. 2.PECTUS EXCAVATUM & CARINATUM - ASD, NOONAN SYNDROME 3.FEMALE HYPOMASTIA - TURNER'S SYNDROME (SHIELD CHEST) 4.HARRISON'S GROOVE -CHRONIC DYSPNOEA DUE TO LARGE LEFT TO RIGHT SHUNT LESIONS 5.HEAVY MUSCULAR THORAX WITH - CO–ARCTATION OF AORTA LESS DEVELOPED EXTREMITES (VISIBLE COLLATERAL ARTERIES IN
  43. 43. PALPATION OF PRECORDIUM THRILL  PS, STRETCHED PFO.  DORV, RESTRICTIVE VSD.  TRICUSPID ATRESIA, PS RESTRICTIVE VSD.  UNIVENTRICULAR HEART, ACROSS THE BULBOVENTRICULAR COMMUNICATION.  TOF WITH ABSENT PV.
  44. 44. APICAL IMPULSE (COMMONEST IS RV TYPE APEX) LV TYPE APEX • TRICUSPID ATRESIA. • PULMONARY ATRESIA WITH INTACT SEPTUM • UNIVENTRICULAR (LV) HEART • EBSTEIN’S ANOMALY • LSVC TO LEFT ATRIAL COMMUNICATION. ABSENT RV ACTIVITY • HYPOPLASTIC RIGHT HEART SYNDROME.
  45. 45. HEART SOUND – S1  WIDE SPLIT WITH LOUD T1 COMPONENT: EBSTEIN’S ANOMALY.  ABSENT T1 COMPONENT : TRICUSPID ATRESIA.
  46. 46. HEART SOUNDS - SINGLE S2 ABSENT P2 1. PULMONARY ATRESIA 2. SEVERE PS AND DYSPLASTIC PULMONARY VALVE 3. TRUNCUS ARTERIOSUS 4. ABSENT PULMONARY VALVE. INAUDIBLE P2 1. D–TGA 2. MPGA. ABSENT A2 1. AORTIC ATRESIA. INAUDIBLE A2 1. LOUD P2 IN PULMONARY AREA (SEVERE PHT.) SYNCHRONOUS A2 AND P2 1. VSD WITH BIDIRECTIONAL FLOW 2. SINGLE VENTRICLE.
  47. 47. HEART SOUND – S2  FIXED, WIDE : TAPVC, ASD WITH SHUNT REVERSAL.  NORMAL / NARROW / SINGLE : PDA WITH SHUNT REVERSAL.  SINGLE LOUD S2 (A2) : ANY ENTITY IN TETRALOGY PHYSIOLOGY WITH SEVERE PS, CCHD WITH MALPOSED GREAT ARTERIES.  SINGLE LOUD S2 (P2) : VSD WITH SHUNT REVERSAL.  WHEN CYANOSIS IS MILD AND GREAT ARTERIES ARE NORMALLY RELATED, P2, THOUGHT SOFT, MAY BE PRESERVED IN TETRALOGY PHYSIOLOGY.  A PRESERVED P2, WHATEVER MAY BE THE DEGREE OF CYANOSIS, EXCLUDES TRUNCUS ARTERIOSUS, PULMONARY ATRESIA AND TOF WITH ABSENT PULMONARY VALVE.
  48. 48. HEART SOUNDS – S3/S4  MULTIPLE HEART SOUNDS : EBSTEIN’S ANOMALY.  S4 : PS WITH STRETCHED PFO, ASD/ EP.  S4 (LV) : CORRESPONDS THE RIGHT ATRIAL A WAVE AND DENOTING A NONRESTRICTIVE ASD IN TRICUSPID ATRESIA.
  49. 49. EJECTION CLICK  PULMONARY CLICK : EISENMENGER PHYSIOLOGY.  AORTIC CLICK : MORE SEVERE THE PULMONARY STENOSIS, MORE IS THE PROBABILITY OF GETTING IT IN TETRALOGY PHYSIOLOGY.  VERY PROMIENT, HIGH PITCHED : TRUNCUS ARTERIOSUS.
  50. 50. MURMUR SYSTOLIC MURMUR  SEVERE THE CYANOSIS, LESSER IS THE GRADE OF THE MURMUR.  EXCEPTION IS THE OBLIGATORY MURMURS IN DORV/ VSD/ PS, TRICUSPID ATRESIA/ VSD (MORE SO WHEN VSD IS RESTRICTIVE) AND IN UNIVENTRICULAR HEART (BULBOVENTRICULAR FLOW).
  51. 51. DIASTOLIC MURMUR  TRUNCUL REGURGITION.  TOF WITH ABSENT PULMONARY VALVE.  TOF WITH AORTIC REGURGITION.  GRAHAM STEEL MURMUR IN EISENMENGER PHYSIOLOGY.
  52. 52. DD OF A CONTINOUS MURMUR WITH OR WITHOUT CYANOSIS ? CONTINOUS OR A TO & FRO MURMUR ?
  53. 53. CONTINOUS MURMURS WITHOUT CYANOSIS  PDA  AP WINDOW  VENOUS HUM  CORONARY AV FISTULA  ALCAPA  RSOV • PERIPH PULM. STENOSIS • SYSTEMIC AV FISTULA • COLLATERALS IN COA • MAMMARY SOUFFLE • AORTICO-LV TUNNEL
  54. 54. CONTINOUS MURMUR WITH CYANOSIS DUCT IN TETRALOGY PULM ATRESIA WITH DUCT MAPCAS IN PULM ATRESIA SUPRACARD. TAPVR PULM AV FISTULAE POST BT SHUNT (THOMAS-BLALOCK-TAUSSIG SHUNT) POST - POTT’S, WATERSTON, CENTRAL SHUNTS
  55. 55. MAPCAS
  56. 56. TO & FRO MURMUR WITHOUT CYANOSIS WITH CYANOSIS  VSD AR  MR AR  AS AR  PS PR  POST OP TETRALOGY  MR AR  TR PR ETC • TETRALOGY WITH AR • TRUNCUS WITH REGURG • ABSENT PV SYNDROME
  57. 57. DIAGNOSIS OF CYANOTIC CHD
  58. 58. CCHD WITH DECREASED PULMONARY BLOOD FLOW  TETRALOGY OF FALLOT  VSD - PS  DORV – VSD – PS  TRICUSP. ATRESIA - PS  SINGLE VENTRICLE - PS  TGA WITH VSD – PS  CORR.TRANSP.-VSD-PS  ASD - PS
  59. 59. CCHD - ↓ PBF - INSPECTION /PALPATORY FINDINGS  CYANOSIS & CLUBBING  POLYCYTHEMIA  QUIET PRECORDIUM TO INSPECTION & PALPATION  NO HARRISONS SULCUS (OR) PRECORDIAL BULGE  APEX WELL WITHIN LIMITS IF VISIBLE  NO PALPABLE SOUNDS (OR) THRILLS
  60. 60. CCHD WITH ↓ PB FLOW AUSCULTATORY FINDINGS  NORMAL FIRST HEART SOUND  SINGLE SECOND HEART SOUND  PULM COMPONENT INAUDIBLE  STENOTIC PULMONARY MURMUR SLIGHTLY AFTER S1 STOPS SHORT OF S2  OTHER MURMURS – DUCTAL/ MAPCA/ AR
  61. 61. Tetralogy of Fallot (TOF)  TOFTOF: Cyanosis: Cyanosis proportional to RVOTproportional to RVOT obstructionobstruction  RV apex, parasternalRV apex, parasternal heave ,heave , Single S2,Single S2, EjectionEjection systolic murmur at Leftsystolic murmur at Left upper sternal edgeupper sternal edge  TOF with PATOF with PA: Single S2: Single S2 but soft murmurbut soft murmur sometimessometimes continuouscontinuous from the MAPCASfrom the MAPCAS.. Occasionally CCFOccasionally CCF
  62. 62. EJECTION MURMUR IN FALLOT PHYSIOLOGY  LENGTH & LOUDNESS INVERSELY PROPORTIONAL TO SEVERITY OF STENOSIS IN ISOLATED PVS – THE OPPOSITE  ABSENT MURMUR – ACQUIRED PULM ATRESIA - MAPCA MURMUR OVER BACK - SOFT DUCTAL MURMUR  TO & FRO – AORTIC REGURG / ABS PV SYNDROME
  63. 63. TETROLOGY OF FALLOT
  64. 64. Tetralogy of Fallot TETRALOGY OF FALLOT
  65. 65. TRICUSPID ATRESIATRICUSPID ATRESIA • Clinical feautures:Clinical feautures: cyanosiscyanosis, LV, LV impulse,impulse, S2 single,S2 single, Holosystolic murmurHolosystolic murmur along left sternalalong left sternal edgeedge
  66. 66. TRICUSPID ATRESIA
  67. 67. TRICUSPID ATRESIA TRICUSPID ATRESIA
  68. 68. CYANOSIS WITH INCREASED FLOW  TRANSPOSITION PHYSIOLOGY  TAPVC  COMMON MIXING LESIONS WITH UNOBSTRUCTED PULMONARY FLOW  PA, VSD WITH SEVERAL MAPCAS.
  69. 69. CCHD WITH ↑ P B FLOW - SYMPTOMS  RESPIRATORY SYMPTOMS PREDOMINATE  GROWTH RETARDED – WEIGHT & HEIGHT  SCRAWNY, SICK, DYSPNOEIC PATIENT  RECURRENT LRTI/PNEUMONIAS  CHRONIC LUNG DISEASE- BRONCHIECTASIS ETC  DIAPHORESIS/ BREATHLESSNESS AT REST  EXERTIONAL DYSPNOEA, LIMITED ACTIVITY.
  70. 70. CCHD WITH ↑ P B FLOW INSPECTION FINDINGS SICKLY UNDERWEIGHT INDIVIDUAL CYANOSIS & CLUBBING -MILD TO MODERATE SEVERE PHT, EISENMENGER – MODIFIES FINDINGS HARRISSON’S SULCUS, PRECORDIAL BULGE ACTIVE PRECORDIUM, RV, LV, PA PULSATIONS OBVIOUS CARDIOMEGALY
  71. 71. CCHD WITH ↑ P B FLOW PALPATORY FINDINGS ACTIVE PRECORDIUM RV IMPULSE – DORV, TAPVR, TGA VSD PS LV IMPULSE – SINGLE VENTRICLE, AVSD-AV REGURG PALPABLE SECOND SOUND / THRILLS RARE
  72. 72. Transposition of the Great Arteries (TGA)  D-TGA with IVS-D-TGA with IVS- cyanosis and tachypnea,cyanosis and tachypnea, S2 single and loudS2 single and loud, soft, soft or absent MURMUR.or absent MURMUR.  D-TGA with VSD-D-TGA with VSD- presents with cardiacpresents with cardiac failure, subtle cyanosisfailure, subtle cyanosis and holo systolic VSDand holo systolic VSD murmurmurmur..
  73. 73. D-TGA- EGG ON END APPERANCE
  74. 74. RVH IN D-TGA RVH IN D-TGA
  75. 75. Tetralogy of Fallot Total Anomalous Pulmonary Venous Return • Pulmonary veins drain anomalously into RA or systemic venous tributaries – Supracardiac – 50% – Cardiac – 20% – Infracardiac - 20% • Presentation and severity depends on pulmonary venous obstruction • ASD or PFO essential for survival
  76. 76. TAPVR- Snowman Appearance TAPVR- SNOWMAN APPEARNCE
  77. 77. TRUNCUS ARTERIOSUS • CLINICALCLINICAL PRESENTATIONPRESENTATION -in neonates-in neonates murmurmurmur and mild cyanosis,and mild cyanosis, -later develops-later develops CardiacCardiac failure, valvefailure, valve insufficiency, single S2,insufficiency, single S2, Loud ESM with thrill andLoud ESM with thrill and MDM due to mitral flowMDM due to mitral flow murmurmurmur
  78. 78. Truncus Arteriosus TRUNCUS ARTERIOSUS
  79. 79. Ebstein’s Anomaly of TVEbstein’s Anomaly of TV  Clinical feautures-Clinical feautures- - depends on degree of- depends on degree of displacement ofdisplacement of Tricuspid ValveTricuspid Valve, can be, can be mild till teenage ormild till teenage or severe with cyanosis insevere with cyanosis in neonate.neonate.  WPW syndromeWPW syndrome is anis an association, multipleassociation, multiple clicks, holo systolic TRclicks, holo systolic TR murmur, gallop.murmur, gallop.
  80. 80. Ebstein’s anomaly EBSTEIN ANOMALY
  81. 81. Ebstein’s with WPW Syndrome EBSTEIN’S WITH WPW SYNROME
  82. 82. DIAGNOSIS OF ACYANOTIC CHD
  83. 83. ATRIAL SEPTAL DEFECT (ASD) A.S.D[O.S type] PRECORDIUM -hyper dynamic RV apical impulse -systolic pulsation in 2nd LICS AUSCULATION -fixed splitting of S2 -split S1 [loud T1] -ESM in pulmonary area -tricuspid mid-diastolic flow murmur X-RAY -pulmonary plethora -markeoly dilated pulmonary trunk and its branches -dilated RA and RV ECG -RSR’ in V1
  84. 84. Ventricular Septal Defect (VSD) • Clinical representation depends on size of VSD &PVR. • Moderate sized defect with low PVR. -CCF in infancy -murmur detected at first well baby examination -retarded growth & development -hyper dynamic LV -PSM in L+ 2 to 4 ICS with thrill -flow MDM across mitral valve
  85. 85. Patent Ductus Arteriosus (PDA) • HISTORY : Premature birth, maternal rubella, birth at high altitude. • CLINICAL SIGNS: 1.Continuous murmur -peak around S2 -maximal in 1st or 2nd LICS -contains eddy sounds[later systole and early diastole] 2.LV apical impulse 3.Brisk arterial pulse with wide pulse pressure
  86. 86. Coarctation of the Aorta • Systemic hypertension • Abnormal differences in upper and lower limb pulses and systolic BP[Radio femoral delay] • Prominent carotid and suprasternal pulsations • Systolic murmur over posterior interscpular region, Lt sternal border and suprasternal notch • Co-existing BCAV- AEC,ejection systolic murmur/EDM in aortic or 2nd aortic area. • X-RAY -3RD to 8th posterior ribs [Rib notching] -seldom before 6yrs of age
  87. 87. COARCTATION OF AORTA ASYMPTOMATIC ADULTS – COLLATERALS HYPERTENSION ! FEMORALS !! BICUSPID AV IN 80% - EJECTION CLICK ! COLLATERAL MURMUR OVER BACK
  88. 88. COARCTATION OF AORTA- RIB NOTCHING
  89. 89. KEYS TO CLINICAL DIAGNOSIS  WORK IN ORDER  COLOUR - CYANOSIS, PALLOR, POLYCYTHEMIA  EXAMINE THE PATIENT HEAD TO FOOT FOR CONGENITAL DEFECTS  PALPATE ALL PERIPHERAL PULSES & RECORD BOTH UL & LL BP  INSPECT – FOR CHEST FORM, PULSATIONS  PALPATE TO DETERMINE – WHICH VENTRICLE ?  FORGET THE MURMUR !!  LISTEN FIRST TO S1, AND THEN TO S2  CAN YOU SPLIT THE SECOND SOUND ??  THEN CONCENTRATE ON THE COMPONENTS  FINALLY THE MURMURS – SYSTOLIC – EJECTION OR PANSYST.  IS THERE A DIASTOLIC MURMUR (OR) CONTINOUS MURMUR
  90. 90. CONCLUSION - -INTELLIGENT SELCTION OF INVESTIGATIVE - PROCEDURES FROM AN EVER - INCREASING ARRAY - REQUIRES FAR MORE SOPHISTICATED DECISION - MAKING. -THE BASIC CLINICAL ASSESSMENT PROVIDES THE INFROMATION NECESSARY FOR MOST OF THESE DECISIONS. -WITH INCREASING EMPHASIS ON THE COST OF MEDICAL CARE, A RESURGENCE OF INTEREST IN THE INEXPENSIVE AND SAFE CLINICAL EXAMINATION IS LIKELY.
  91. 91. S.H.P

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