Congenital Heart Disease - Dr. MacNeill

Heart Lesions

    1. Left to right - VSD, ASD, cushion defect, PDA

    2. Cyanotic - truncus, transposition, total anomalous, tricuspid atresia, tetralogy

            a. Cyanosis - decrease of deoxygenated hgb by 3-5 mg/dl

                  1. Shunting from lung

                  2. Mixing blue and red blood     

    3. Single ventricle

Break the left side of the heart (Hypoplastic left, aortic stenosis, coarct) --> hepatomegaly, gray, pulmonary edema, etc

Break the right side of the heart (hypoplastic right heart, tricupsid atresia, pulmonary atresia, tetrology of fallot) --> Blue, poor perfusion, acidosis  

Not all ductal dependent lesions are cyanotic - AS, coarct 

Not all cyanotic lesions are ductal dependent - truncus arteriosis, TAPR


Cyanotic Lesions 

    a. Truncus Arteriosus -

                Blue because they're mixing - mixing happens before duct, therefore not ductal dependent  -
                Pulmonary exam will vary;
                You can give them O2 - won't worsen cyanosis but won't help

    b. Transposition of Great Arteries -

                Cyanotic because you have mixing blood;
                 If you find these later (ie, in the ED and not immediately after birth) these kids will all have VSD;
                 Ductal dependent
                 O2 wont help but wont hurt

    c. Tricuspid Atresia -

                  Blue because not perfusing lungs; Right ventricle doesn't develop (Hypoplastic right heart)
                  Ductal dependent; only pulmonary artery flow will come through ductus from aorta
                  ECG will show LVH but only because right side isn't balancing it out
                  O2 will prob not help, but won't kill

    d. Tetrology of Fallot -

                  Cyanotic because of decreased pulmonary perfusion and mixing -
                  O2 can help

    e. Totally Anomalous Pulmonary Venous Return  -

                  Cyanotic because of mixing

     
Hyperoxia Test 

- 10 minutes of 100% O2 and see response > may help differentiate between pulmonary and cardiac etiology

* For cyanotic lesions oxygen is not going to kill - it just may not help*  
* O2 can hurt you on left to right shunts*
  
           Left to right shunts are usually dyspneic/hypoxic because they are over-perfusing the lungs and they get fluid overload.

           Oxygen will cause vasodilitation of the pulmonary vessels and increase left to right shunting worsening the problem.

Prostaglandins - 0.05-0.1 mcg/kg/min > will cause apnea - tube the kid   

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