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PATHOPHYSIOLOGY OF CYANOTIC CHD. BY J. A. AL-ATA COSULTANT & ASSISTANT PROFESSOR OF PEDIATRIC CARDIOLOGY. CYANOSIS. BLUISH discoloration of SKIN & MM due to doxyhemoglobin. Desaturated arterial blood Central cyanosis. Peripheral cyanosis Normal Art. Sat.

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pathophysiology of cyanotic chd

PATHOPHYSIOLOGY OFCYANOTIC CHD

BY

J. A. AL-ATA

COSULTANT & ASSISTANT PROFESSOR OF PEDIATRIC CARDIOLOGY

cyanosis
CYANOSIS
  • BLUISH discoloration of SKIN & MM due to doxyhemoglobin.
  • Desaturated arterial blood Central cyanosis.
  • Peripheral cyanosis Normal Art. Sat.
  • Detected; clinically ( sat below 85% ), pulse oximetry, or ABG.
causes of cyanosis
CAUSES OF CYANOSIS
  • Central cyanosis:
      • Respiratory
      • CNS
      • Muscular
      • Cardiac
  • Peripheral cyanosis:
      • CHF
      • Shock
      • Acrocyanosis
      • Abnormal hemoglobin
types of cyanotic chd
Types of Cyanotic CHD
  • With pulmonary blood flow;
      • D-TGA
      • Truncus arteriosus
      • TAPVD
      • DORV
      • Single ventricle no ps.
  • With pulmonary blood flow;
      • TOF
      • Critical pulmonary stenosis
      • Pulmonary Atresia
d tga
D-TGA
  • Parallel circulation not in series
      • Body----RA----RV----AO----Body
      • Lungs---LA----LV----PA----Lungs
  • Poor mixing
  • Hypoxia & Acidemia
  • Hyperventilation
  • Increased pulmonary flow
  • CHF
  • Myocardial depression
truncus arteriosus
TRUNCUS ARTERIOSUS
  • Complete mixing so cyanosis is minimal
  • VSD always present
  • Identical pressures in both ventricles
  • Systemic saturation is proportional to pulmonary blood flow ( PBF )
  • PVR & PA.s caliber determine PBF
  • CHF is a usual presentation
  • Eisenmenger syndrome may develop
  • AS & AI are complicating factors
tapvr
TAPVR
  • NON-Obstructed TAPVR
      • Similar hemodynamics to a large ASD
      • Lt to Rt shunt magnitude is determined by RV compliance & ASD size
      • Rt heart & pulmonary volume overload
      • Complete mixing at RA level
      • Minimal cyanosis due to large PBF
      • Slight PA pressure elevation
tapvr cont
TAPVR, CONT;
  • Obstructed TAPVR
      • Pulmonary venous hypertension & secondary PA & RV hypertension
      • Less RV & PA volume overload
      • Pulmonary venous oedema
      • More cyanosis & respiratory distress
      • Complete mixing
tricuspid atresia
TRICUSPID ATRESIA
  • Increased RA pressure & size
  • Dependant on the ASD or PFO
  • Dilated LA & LV
  • Complete mixing in LV
  • With TGA PBF & SAT.
  • Variable degree of cyanosis
ebstein anomaly
EBSTEIN ANOMALY
  • Variable degrees of cyanosis depending on amount of Rt to Lt shunt across ASD or PFO
  • Hugely dilated RA
  • Incompetent TV
  • Atrialized & small poorly functional RV
  • RVOTO & PS can be associations
  • SVT ( WPW ) can be associated
tetralogy of fallot
TETRALOGY OF FALLOT
  • Decreased PBF & Amount of RT to LT shunt determine degree of cyanosis
  • PBF is mainly determined by RVOTO & PS
  • Pink TOF = mod RVOTO & balanced shunting across the VSD
  • Increased PBF can result from PDA or MAPCAS
  • Equal ventricular pressures
pulmonary atresia
PULMONARY ATRESIA
  • With VSD
      • An extreme form of TOF
      • Early cyanosis when PDA closes
      • Ductal dependant
      • CHF with MAPCAS / PDA
      • Balanced form
pulmonary atresia cont
PULMONARY ATRESIA CONT;
  • Without VSD
      • Early marked cyanosis ( extremely decreased PBF )
      • Ductal dependant
      • RV sinusoids
      • RV decompression by TR
      • ASD or PFO mandatory
summary
SUMMARY
  • Central cyanosis is the hallmark of cyanotic CHD
  • Cyanosis is a serious symptom or sign
  • Variable degrees of cyanosis occur due to variability in PBF, PVR, RVOTO, presence & size of shunting site e.g. ASD and hemoglobin concentration
  • CHF can occur in cyanotic CHD due to increased PBF
  • Pulmonary AVMs & Methemoglobinemia are non-cardiac causes of central cyanosis.
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