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TETRALOGY OF FALLOT. COMMONEST CYANOTIC CONGENITAL HEART DISEASE 10 % OF ALL CONGENITAL HEART DISEASES. MORPHOLOGY. FOUR MORPHOLOGICAL DEFECTS VENTRICULAR SEPTAL DEFECT RIGHT VENTRICULAR OUTFLOW TRACT OBSTRUCTION SUBVALVAR VALVAR SUPRAVALVAR OVERRIDING OF THE AORTA

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tetralogy of fallot
TETRALOGY OF FALLOT
  • COMMONEST CYANOTIC CONGENITAL HEART DISEASE
  • 10 % OF ALL CONGENITAL HEART DISEASES
morphology
MORPHOLOGY
  • FOUR MORPHOLOGICAL DEFECTS
    • VENTRICULAR SEPTAL DEFECT
    • RIGHT VENTRICULAR OUTFLOW TRACT OBSTRUCTION
      • SUBVALVAR
      • VALVAR
      • SUPRAVALVAR
    • OVERRIDING OF THE AORTA
    • RIGHT VENTRICULAR HYPERTROPHY
morphology in t o f
MORPHOLOGY IN T.o.F

Subvalvar Obstruction

altered physiology
ALTERED PHYSIOLOGY
  • OBSTRUCTION TO FLOW OF DEOXYGENATED BLOOD FROM THE RIGHT VENTRICLE TO THE PULMONARY ARTERY
  • DECREASED OXYGENATION DUE TO POOR PERFUSION OF THE BLOOD
altered physiology6
ALTERED PHYSIOLOGY
  • SHUNTING OF DEOXYGENATED BLOOD FROM THE RIGHT VENTRICLE TO THE AORTA ACROSS THE VENTRICULAR SEPTAL DEFECT (FACILITATED BY AORTIC OVERRIDE)
  • POOR SYSTEMIC OXYGENATION, LOW HEMOGLOBIN SATURATION AND CYANOSIS
clinical presentation
CLINICAL PRESENTATION
  • CYANOSIS NOT USUALLY NOTICED AT BIRTH
  • CAUSES
    • CHILD LESS ACTIVE IN THE INITIAL FEW MONTHS
    • FOETAL HEMOGLOBIN HAS MORE AFFINITY FOR OXYGEN THAN ADULT HEMOGLOBIN
clinical presentation8
CLINICAL PRESENTATION
  • CYANOSIS MANIFESTS MORE AS CHILD BECOMES MORE ACTIVE
  • PHYSICAL GROWTH IS USUALLY GOOD
  • MENTAL DEVELOPMENT MAY BE DELAYED IN SEVERE CASES DUE TO CHRONIC HYPOXIA OF THE BRAIN
cyanotic spells
CYANOTIC SPELLS
  • TYPICAL OF FALLOT’S TETRALOGY
  • USUALLY OCCURS WHEN THE CHILD CRIES OR IS VERY ACTIVE AS WHEN THE CHILD WAKES UP FROM SLEEP
cyanotic spells10
CYANOTIC SPELLS
  • ACTIVITY RESULTS IN
    • INCREASES OXYGEN DEMAND
    • DECREASES SYSTEMIC VASCULAR RESISTANCE
    • INCREASES SYMPATHETIC ACTIVITY WHICH CAUSES INFUNDIBULAR SPASM, I.E., INCREASE IN THE MUSCULAR OBSTRUCTION TO THE RIGHT VENTRICULAR OUTFLOW AT THE SUBVALVAR LEVEL
decreased s v r
DECREASED S.V.R.
  • MORE SHUNTING ACROSS THE VSD – MORE DESATURATION OF SYSTEMIC BLOOD – PERIPHERAL ACIDOSIS – FUTHER SYSTEMIC VASODILATATION – FURTHER DECREASE IN SVR – VICIOUS CYCLE
squatting
SQUATTING
  • TYPICAL OF FALLOT’S TETRALOGY
  • CHILD ASSUMES SQUATTING POSTURE VERY FREQUENTLY
  • SOME POSTURES MAY BE CALLED ‘SQUATTING EQUIVALENTS’
  • REASON IS THAT SQUATTING CAUSES AN INCREASE IN RESISTANCE TO SYSTEMIC FLOW – DECREASED SHUNTING ACROSS THE VSD – LESS DESATURATION OF SYSTEMIC BLOOD
natural history
NATURAL HISTORY
  • WIDE SPECTRUM OF CLINICAL MANIFESTATIONS DEPENDING ON SEVERITY OF ABNORMALITIES, I.E., DEGREE OF OBSTRUCTION TO RIGHT VENTRICULAR OUTFLOW, AND SIZE OF VSD
natural history14
NATURAL HISTORY
  • IN SEVERE CASES, CHILD MAY HAVE REPEATED CYANOTIC SPELLS
  • IN LESS SEVERE CASES, CHILD MAY BE FREE FROM SPELLS, BUT SEVERLY CYANOSED, WITH NORMAL PHYSICAL BY DELAYED MENTAL DEVELOPMENT
  • IN MILD CASES, CHILD MAY GROW NORMALLY, WITH CYANOSIS BEING ONLY MINIMAL
treatment options
TREATMENT OPTIONS
  • ONLY SURGICAL
    • PALLIATIVE SURGERY
    • DEFINITIVE SURGERY
palliative surgery
PALLIATIVE SURGERY
  • AIMED TO DIVERT SYSTEMIC BLOOD INTO THE PULMONARY CIRCULATION AND THUS ENHANCE PULMONARY FLOW AND OXYGENATION
  • STANDARD OPERATION IS THE MODIFIED BLALOCK-TAUSSIG SHUNT OR OTHER SYSTEMIC PULMONARY SHUNTS SUCH AS POTT’S SHUNT AND WATERSTON-COOLEY SHUNT
definitive surgery
DEFINITIVE SURGERY
  • RELIEF OF RIGHT VENTRICULAR OUTFLOW TRACT OBSTRUCTION
  • SEPARATION OF SYSTEMIC AND PULMONARY CIRCULATIONS BY CLOSURE OF THE VSD
treatment strategies
TREATMENT STRATEGIES
  • PALLIATIVE SURGERY IN EARLY CHILDHOOD FOLLOWED BY DEFINITIVE SURGERY IN THE LATER YEARS, USUALLY AFTER 3 – 4 YEARS OF AGE
  • DEFINITIVE SURGERY IN THE NEONATAL PERIOD OR EARLY CHILDHOOD
t o f in adult cardiac surgical hospital
T.O.F IN ADULT CARDIAC SURGICAL HOSPITAL
  • DELAYED DEFINITIVE REPAIR FOLLOWING SHUNT IN EARLY CHILDHOOD
  • DELAYED PRESENTATION, FOR DEFINITIVE REPAIR
  • RE-OPERATION FOR DELAYED COMPLICATIONS AFTER DEFINITVE REPAIR
postoperative nursing issues
POSTOPERATIVE NURSING ISSUES
  • MOST WILL HAVE A NORMAL CIRCULATION POSTOPERATIVELY
  • C.V.P MAY RUN HIGH DUE TO STIFF RIGHT VENTRICLE AND HIGH RVEDP
  • GAS EXCHANGE IS USUALLY NORMAL
  • MAY HAVE HEART BLOCKS OR BRADYARRYHTHMIAS