Cyanotic Heart Lesions

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
o Decreased PBF- increased cyanosis o Increased PBF- minimal cyanosis but CHF may develop

With 100% oxygen

o PO2 >250 is not congenital heart disease o PO2 <100 is cardiac disease

5 Ts
Most common cyanotic lesions of the newborn
o o o o o Tetralogy of Fallot Transposition of the Great Arteries Truncus Arteriosus Total Anomalous Venous Return Tricuspid Atresia

General Sources of Arterial Saturation

Decreased Pulmonary Blood Flow Admixture Lesions Increased Pulmonary Blood Flow

Tetralogy of Fallot
Tricuspid Atresia Pulmonary Atresia

Transposition of Great Vessels

Anomalous pulmonary venous return

Truncious Arteriosus

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

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
o Hypoxemic blood circulating in the body o Hyperoxemic blood circulating in the pulmonary circuit

Complete Transposition of the Great Arteries

Defect to permit mixing of 2 circulations- ASD, VSD, PDA.
o VSD is present in 40% of cases

Necessary for survival

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

Clinical Symptoms
Mixing lesion present (VSD or large PDA)
o o o o 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

CXR
Egg shaped cardiac silhouette Narrow superior mediastinum

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

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
1. 2. 3. 4. VSD RVOT Obstruction RVH Overriding aorta

Two Important Abnormalities

NonRestrictive VSD
o Large enough to equalize pressures in both ventricles

Degree of RVOT

 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

Clinical Presentation of Cyanotic TOF

CXR- boot shaped

Hypoxic Spell (TET Spell)

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

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

1. Hold infant in knee-chest position 2. Morphine 3. Sodium bicarbonate to treat acidosis- decreases resp stimulating effect of acidosis 4. Vasoconstrictor (phenylephrine) 5. Propranolol

TET Spell Treatment

Treatment
Early surgical repair depending on pts 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
o Systemic and pulmonary venous blood are completely mixed

Common pulmonary vein drains into the SVC via the left SVC and left innominate vein.

4 Types 1. Supracardiac

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

 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 Unobstructed Veins

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

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
o systemic to pulmonary arterial shunt o Provide stable blood flow to the lungs o A gortex tube is sewen between the subclavian artery and the right pulmonary artery

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

Truncus Arteriosus
A single trunk leaves the heart o Gives rise to pulm, systemic, and coronary circulations o 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
o VSD is closed o Pulmonary artery is separated from the truncus o Continuity is then established between the right ventricle and the pulmonary artery utilizing a valved homograft conduit