Doctorese Babble -- Cyanotic Heart Lesions

I. Tetralogy of Fallot


A. Facts

        1. MC congenital cardiac abnormality

        2. Cyanotic lesion

        3. 1% prevalence of CDH – 1% total worldwide will receive care

        4. R-> L shunt

        5. Typically no observed immediately at birth – some decreased sats don’t always present with obviously blue appearance



B. Features

       1. RVOT – pulmonic stenosis is supra or infravalvular – clinically most important component

       2. VSD

       3. RVH

       4. Overiding aorta

C. Tet spell

       1. Infundibular spasm -- less blood circulates to the lungs  hypoxia and increased deoxygenated blood through VSD

       2. Present as grey babies with difficulty breathing – increased anaerobic respiration  lactic acid, which affects contractility and leads to cardiogenic shock

D. Treatment

      1. Sedate

      2. Fluid bolus to increase blood volume going to heart – getting the IV can be difficult

      3. Increase SVR – can reduce shunting

             a. Knee-chest positioning – compresses femoral artery and vein increasing SVR and venous return
             b. Alpha-1 receptor agonists – phenylephrine



       4. CxR shows boot-shaped heart (RVH) – decreased pulmonary blood flow  very black lungs  oligemia

       5. Echo

       6. Corrective surgery

             a. Transanular patch – pulmonic stenosis

             b. Don’t completely close VSD

                    (1) Must incise RV

                   (2) Slight VSD serves as pressure release valve, otherwise heart can fail – “blue blood is better  than no blood”

                   (3) If baby is too small, an ASD can improve saturation

           c. BTT shunt if baby is very tiny – often performed in developing countries, and can result in pulmonary hypertension if more comprehensive treatment not pursued later



II. Transposition of the Great Vessels

A. PDA can close late

B. Patients arrive cyanotic and in shock

C. Can make ASD or VSD

         1. ASD is easier

         2. PFO balloon atrial septostomy

D. “Switch procedure” ultimately curative

III. Tricuspid Atresia

IV. Truncus Arteriosus

A. Present with overcirculated lungs – edema and RSD

B. VSD murmur

C. Truncus becomes new aorta

D. RV to PA conduit


V. TAPVR

A. Peripheral veins don’t return to left atrium

B. Those that cross diaphragm frequently obstructed – pulmonary edema and RSD

          1. Bilateral edema

          2. RSD

          3. GSB pneumonia

C. Treatment – return aberrant vein to LA

VI. Hypoplastic Left Heart