Congenital Heart Disease with Early Cyanosis (R → L shunt)

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🌱 來自: Huppert’s Notes

Congenital Heart Disease with Early Cyanosis (R → L shunt)🚧 施工中

Congenital Heart Disease with Early Cyanosis (R → L shunt)

Tetralogy of Fallot

•   Etiology: Associated with Down syndrome; includes four anatomic components: 1) VSD; 2) Right ventricular hypertrophy; 3) Pulmonary stenosis; 4) Overriding aorta

•   Clinical presentation: Progressive hypoxemia in childhood

•   Heart sound: Harsh crescendo-decrescendo systolic ejection murmur best heard at the left upper sternal border (due to RVOT obstruction), single S2 (because pulmonic component rarely audible).

•   CXR: Boot-shaped heart

•   Treatment: Early surgical repair

Transposition of the great vessels

•   Etiology: Congenital defect involving the abnormal spatial rearrangement of any of the great vessels (SVC, IVC, pulmonary artery, pulmonary vein). If involving only the pulmonary artery and aorta, belongs to a subtype called transposition of the great arteries (TGA) which is the most common congenital heart condition. Causes are largely unknown (some association with genetics, viral illness in pregnancy, advanced maternal age)

•   Clinical presentation: Cyanotic newborn, tachypnea; requires shunt (e.g., VSD, PDA, PFO) to allow mixing of blood

•   Treatment: Surgical repair

Persistent truncus arteriosus

•   Etiology: Congenital defect where there is a single vessel leaving the heart rather than both the pulmonary artery and aorta; typically occurs spontaneously

•   Clinical presentation: Cyanosis at birth, heart failure may occur within weeks

Tricuspid atresia

•   Etiology: Congenital defect where there is complete absence of the tricuspid valve. Therefore, there is no right atrioventricular connection and it requires ASD and VSD for viability. Causes unknown

•   Clinical presentation: Congenital cyanosis

•   Treatment: Administer PGE to maintain patent ductus arteriosus and surgical repair