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Flashcards in Congenital Heart Disease Deck (13):

1. Explain the basic development of the heart from a single tube to a four-chambered heart with two great vessels. (in order to assess congenital anomalies).

heart of a 5 week old fetus is basically a contractile tube without valves that propels blood through the embryo, during development, the tube lengthens and loops (normally to the right) atrial and ventricular septa develop and great vessels differentiate


Be able to recognize how patients with congenital disease might present as adults in clinic.



Endocardial cushion defect can effect which heart structures?

lower atrial septum (meets with septum primum), upper ventricular septum and AV valves, particularly anterior leaflet of mitral valve

(complete endocardial cushion defect seen often in trisomy 21- Down's syndrome)


Contrast osmium primum and secundum atrial septal defects

primum ASD rests from partial deficiency of endocardial cushion, resulting in incomplete development of the lower atrial septum and MV

secundum ASD results from failure of septum secundum to reach the remnant septum primum


What might an amplatzer device be used to repair?

amplatzer is a ventricle closure device

note most defects are in the upper (more membranous?) portion of the ventricular septum, there are rare defects in the septum itself (muscular defects)


What are the consequences of a patent ductus arteriosus?

if large PDA can result in significant increase in pulmonary blood flow (lower resistance in pulmonary bed)

results in diversion of aortic blood into the pulmonary arteries


In a baby born with great vessel transposition may undergo which procedures (one to create an atrial defect and one to transpose the great vessels)?

tear in atrial septum (Rashkind)
surgical ASD (Blalock Hanlon)

transposition of great vessels to correct position (Jatene)


What is a mustard procedure used for?

removal of the atrial septum and use of synthetic material to reverse the atrial return with transposed great vessels

atrial arrhythmias and SA node failure is common, the right ventricle is also likely to fail under systemic pressure over the years


What is the primary defect in tetralogy of Fallot?

aorta is displaced to the right resulting in big ventricular septal defect, right ventricular outflow is squished (decreased blood to the lungs and RV hypertrophy) and shunting of blood from right to left heart


What is the treatment for tetralogy of Fallot?

surgery to repair the VSD and enlarging the right ventricular outflow (often means cutting open abnormal pulmonic valve which might ultimately have to be replaced)


Describe the effects of tricuspid atresia

tricuspid valve does not develop
right ventricle is underdeveloped
pulmonary arteries are small

survival depends on crating a shunt from the systematic circulation to the pulmonary circulation


How would you treat someone with tricuspid atresia?

bidirectional glenn anatosmose the pulmonary artery and the superior vena cava

font an anatomies the inferior vena cava is attached to the pulmonary artery


What are complications of a Fontan procedure?

right atrium dilates and can produce arrhthymias
potential for blood stagnation due to atrial dilation

(lateral tunnel approach avoids some atrial stretching; extra cardiac connection to avoid damage to the RA all together)