Cyanotic Heart Lesions Case presentation (pink kid turns blue, blue ki)d comes out What would you think about Where does cyanotic heart disease come in? Emailing Dr. Meadows How would you approach this patient? Indications/Contraindications Which kids to start PGE Neonatal Intensive Care Nursery Night Curriculum Series

Cyanosis Arterial saturation less than 90% and a PO2 less than 60 torr In all cyanotic heart lesions the amount of cyanosis seen is dependent on the amount of pulm blood flow Decreased PBF- increased cyanosis Increased PBF- minimal cyanosis but CHF may develop With 100% oxygen PO2 >250 is not congenital heart disease PO2 <100 is cardiac disease Turn into questions

5 “T’s” Most common cyanotic lesions of the newborn Tetralogy of Fallot Transposition of the Great Arteries Truncus Arteriosus Total Anomalous Venous Return Tricuspid Atresia Make this a question and animate it

General Sources of Arterial Saturation Decreased Pulmonary Blood Flow Admixture Lesions Increased Pulmonary Blood Flow Tetralogy of Fallot Transposition of Great Vessels Truncious Arteriosus Tricuspid Atresia Anomalous pulmonary venous return Pulmonary Atresia

PGE For cyanotic heart lesions with reduced blood flow RE-opens PDA and prevents it from closing Allows partially desaturated systemic arterial blood to enter the pulmonary artery and be oxygenated Initial dose 0.1mg/kg/min Side effects- apnea, fever, hypotension Ask what is PGE? How does it work? And What are potential Complications of it?

Complete Transposition of the Great Arteries 5% of all CHD Boys 3:1 Most common cyanotic condition that requires hospitalization in the first two weeks of life

Complete Transposition of the Great Arteries Aorta arises from the right ventricle Pulmonary artery arises from the left ventricle

Complete Transposition of the Great Arteries Complete separation of the 2 circuits Hypoxemic blood circulating in the body Hyperoxemic blood circulating in the pulmonary circuit

Complete Transposition of the Great Arteries Defect to permit mixing of 2 circulations- ASD, VSD, PDA. VSD is present in 40% of cases Necessary for survival

Clinical Symptoms Depend on anatomy present No mixing lesion and restrictive PFO Profound hypoxia Rapid deterioration Death in first hours of life Absent respiratory symptoms or limited to tachypnea Single second heart sound, no murmurs animate

Clinical Symptoms Mixing lesion present (VSD or large PDA) Large vigorous infant Cyanotic Little to no resp distress Most likely to develop CHF in first 3-4 months of life excessive sweating (a cold, clammy sweat often noted during feeding); poor feeding, slow weight gain, irritability or lethargy, and/or rapid breathing animate

CXR Egg shaped cardiac silhouette Narrow superior mediastinum Describe the findings on this CXR, and animate

Management Prostaglandin to establish patency of the ductus arteriosus Increases shunting from aorta into the pulmonary artery Increases pulmonary venous return distending the left atrium Facilitates shunting from the left to the right atrium of fully saturated blood across the foramen ovale. Ask what physiologically happens

Management Therapeutic balloon atrial septostomy (Rashkind Procedure) if surgery is not going to be performed immediately Improves mixing and pulmonary venous return at the atrial level

Treatment Surgery consists of switching the right and left sided structures at the atrial level, at the ventricular level, or at the great artery level.

Tetraology of Fallot VSD RVOT Obstruction RVH Overriding aorta Ask what are the 4 compents and animate

Two Important Abnormalities NonRestrictive VSD Large enough to equalize pressures in both ventricles Degree of RVOT Because of the non-restrictive VSD, systolic pressures in the RV and the LV are identical. Depending on the degree of RVOT obstruction, and L-R, bidirectional, or r-L shunt is present. Example: Mild RVOT- there is a left to right shunt and infant is acyanotic Severe RVOT- there is a predominant R-L shunt and the infant is cyanotic Degree of cyanosis depends on the degree of right outflow tract obstruction

Clinical Presentation of Cyanotic TOF Cyanosis, clubbing, dyspnea on exertion, squatting, hypoxic spells. Loud systolic ejection murmur, systolic thrill at middle LSB Soft murmurs are associated with less blood flow and more hypoxia

CXR- boot shaped Ask what are the classic CXR findings

Hypoxic Spell (“TET Spell”) Peak incidence of 2-4 months Characterized by: Hyperapnea (Rapid and deep respirations) Irritability and prolonged crying Inc cyanosis Decreased heart murmur

Pathophysiology Lower SVR or inc resistance of RVOT can increase the R-L shunt Stimulates the respiratory center to produce hyperapnea Results in an increase in systemic venous return In turn, increases R-L shunt through VSD

TET Spell Treatment Hold infant in knee-chest position Morphine Sodium bicarbonate to treat acidosis- decreases resp stimulating effect of acidosis Vasoconstrictor (phenylephrine) Propranolol

Treatment Early surgical repair depending on pt’s weight VSD is closed and obstructing ventricular muscle is removed

Total Anomalous Pulmonary Venous Return The pulmonary veins drain into the RA or its venous tributaries rather than the LA A interatrial communication (ASD or PFO) is necessary for survival Pulmonary venous return reaches the RA Systemic and pulmonary venous blood are completely mixed

4 Types 1. Supracardiac Common pulmonary vein drains into the SVC via the left SVC and left innominate vein.

2. Cardiac The common PV drains into the coronary sinus

3. Infracardiac The common PV drains into the portal vein, ductous venosus, hepatic vein, or IVC. Infracardiac-type TAPVC. Pulmonary venous blood draining through the liver to reach the IVC and right atrium.

4. Mixed A combination of the other types

Clinical Signs for Unobstructed Veins Mild cyanosis, signs of CHF in infancy, history of pneumonia Widely split S2, Grade 2-3/6 systolic murmur heard at the ULSB CXR- marked cardiomegaly

Clinical Signs for Obstructed Veins Profound desaturation Acidosis PGE1 administration does not improve oxygenation because elevated pulmonary pressures in the right side of the heart (due to obstructed pulmonary outflow) will result in right to left shunting across an open ductus further decreasing arterial saturation.

Treatment Digitalis and diuretics to treat heart failure Intubation and inc PEEP for those with severe pulm over load Corrective surgery

Tricuspid Atresia Tricuspid valve is absent RV and PA are hypoplastic Associated defects- ASD, VSD, or PDA (necessary for survival) Dilation of LA and LV Essentially single ventricle physiology LA and LV are large since they handle both systemic and pulmonary venous return

Clinical Signs Severe cyanosis, poor feeding, tachypnea Single S2, grade 3/6 systolic murmur at LLSB if VSD is present CXR- boot shaped heart

Treatment PGE IV infusion Blalock-Taussig shunt in infancy systemic to pulmonary arterial shunt Provide stable blood flow to the lungs A gortex tube is sewen between the subclavian artery and the right pulmonary artery PGE infusion in severely cyanotic lesions to maintain patency of PDA

Bidirectional Glenn Superior vena cava is connected to the pulmonary arteries IVC continues to be connected to the heart

Fontan Procedure Redirects IVC to lungs Now oxygen-poor blood from upper and lower body flows through the lungs without being pumped and corrects cyanosis The single ventricle is only responsible for supplying blood to the body.

Truncus Arteriosus A single trunk leaves the heart Gives rise to pulm, systemic, and coronary circulations Large VSD is always present

Clinical Signs Cyanosis immediately after birth Early signs of CHF 2-4/6 systolic murmur at LSB suggestive of VSD

Treatment Anticongestive medications (diuretics and digitalis) Corrective surgery VSD is closed Pulmonary artery is separated from the truncus Continuity is then established between the right ventricle and the pulmonary artery utilizing a valved homograft conduit

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