Pathophysiology of cyanotic chd
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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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