Flashcards in Primer 9 - Congenital Heart Disease Deck (43):
What is an Ebstein anomaly?
Congenital defect when tricuspid leaflets are displaced into right ventricle, hypoplastic right ventricle, tricupid regurg or stenosis. Dilated right atrium causing increased risk of SVT and WPW.
Around 80% have a patent foramen ovale with a Right to Left shunt.
What is a left-to-right shunt?
Congenital anomaly that causes the blood flow from high pressure of the left (systemic) to go to the right low pressure (pulmonary system).
What are the 3 main left-to-right shunts?
1. Atrial septal defect (ASD)
2. Ventricular spetal defect (VSD)
3. Patent ductur arteriosus (PDA)
What is the most common type of atrial septal defect and why is it caused?
A patent foramen ovale. A hole in the atrium-atrium chambers, due to either the osteum secundum or the foramen ovale are just too big, they overlap, and they cannot close.
What pharmacological use is associated with Ebstein anomaly?
Associated with maternal lithium use.
How does an Atrial septal defect sound like?
Loud S1 and wide, fixed splitting S2.
What is the most common type of congenital heart defect?
Ventricular septal defect.
How does a Patent ductus arteriousus sound like?
A continuous machine-like murmur.
When does the Patent ductus arteriosus is supposed to close?
Within the first 24 hours of life.
What is the treatment of Patent Ductus Arteriosus?
Indomethacin: blocks prostaglandin synthesis.
When should we keep the Patent Ductus Arteriousus and what can we use to keep it open?
In certain right to left shunts such as transposition of the great vessels, use prostaglandins to keep it open.
What is Eisenmenger syndrome?
Severe longstanding uncorrected left to right shunts leads to this syndrome. A severe left to right shunt leads to an overflow into the pulmonary circulation, creating pulmonary hypertension. Since the right ventricle needs to work harder to pump against pulmonary resistance, leading to right ventricle hypertrophy. Once right ventricle pressure goes up enough to exceed the left side of the heart, the shunt switches to right to left shunt.
What is coarctation of the aorta and what are the two types.
Focal circumferential narrowing of the aorta. The adult distal to the ductus arteriosus. The infantile type is coarctation proximal to the insertion of the ductus arteriosus.
Which syndrome is related to the coarctation of the aorta?
What do we see in X-ray in coarctation of the aorta?
Notching of the arteries of the ribs on chest x-ray due to intercostal arteries becoming dilated, carving indentation on the inferior aspect of the ribs.
What three cardiac pathologies are associated with Turner syndrome?
Infantile coarctation, bicuspid aortic valves, and infective endocarditis.
What heart pathologies are associated with Down Syndrome?
Endocardial cushion defect.
What heart pathology is associated with DiGeorge syndrome?
Tetralogy of Fallot or Truncus arteriosus.
What heart pathology is associated with Congenital rubella?
PDA or Pulmonary artery stenosis.
What heart pathology is associated with Aortic insufficiency?
What happens in Right to left shunt?
Blood is being shunted from deoxygenated right side to the left side, bypassing the lung circulation.
What are the five causes of Right to left shunts AKA cyanotic diseases?
1. persistent Truncus arteriosus, 2. Transposition of the great vessels, 3. Tricuspid atresia, 4. Tetralogy of Fallot, 5. Total anomalous pulmonary venous return.
-They all start with T.
-The 5 Ts.
What is Persistent Truncus Arteriosus?
Failure of the trucus arteriosus to divide into the aorta and pulmonary trunk, mixing deoxygenated blood mixing in the circulation.
What is transposition of the great vessels?
When the aorta and the pulmonary artery have been switched; the left ventricle pumps into the pulmonary circulation and the right ventricle into the circulation. This creates two completely closed groups, needing a shunt to be compatible with life.
What pathologies are fetus of diabetic mothers more likely to suffer?
Transposition of the great arteries (TGA), shoulder dystocia (shoulder gets caught under the maternal pelvic bone), large for gestacional age (LGA).
What is tricuspid atresia?
When the tricuspid valve does not develop. There is no opening from the right atrium to the right ventricle. Needs two shunts to be compatible with life (ASD, VSD).
What are the two shunts needs for the fetus to be compatible with life?
Atrial septal defect and ventricular septal defect.
What is Ebstein anomaly?
It is when tricuspid leaflets are displaced inferior into right ventricle, there is hypoplastia of right ventricle and tricuspid regurgitation or stenosis. Their is 80% of patients have a patent foramen ovale with right to left shunt.
What increased risk does the dilated right atrium give in patients with Ebstein anomaly?
Increased risk of Supraventricular tachycardia (SVT) and Wolf-Parkinson-White (WPW).
What do we find in the physical exam of the heart of someone with Ebstein anomaly?
Widely split S2 and tricuspid regurgitation murmur.
What are the 4 defects of Tetralogy of Fallot?
1.Pulmonary valvular stenosis. 2. Right ventricular hypertrophy. 3.Ventricular septal defect. 4. Overriding aorta.
Why do kids with Tetralogy of Fallot squat?
Kids with Tet of Fallot have left to right shunt which is acyanotic, but there may be episodes when it turn to right to left shunt like when the cry or exercise, and become cyanotic. Older kids learn to squat on these cyanotic spells because squating increases the systemic vascular resistance, forcing it back into left to right shunt.
What is the classic X-ray finding in tetralogy of Fallot?
The boot-shaped heart due to right ventricular hypertrophy.
What is Total anomalous pulmonary venous return?
Where the pulmonary veins drain into the systemic circulation; instead of oxygenated blood coming from the pulmonary veins into the left atrium, it might empty to the superior vena cava, or the coronary sinus, but at the end empties into the right atria; a completely closed loop, needing a type of shunt to live.
What is the most common congenital cardiac anomaly?
Ventricular septal defect.
Explain how the great vessels are attached in a transposition of the great vessels?
The right ventricle is connected into the aorta and the left ventricle is connected to the pulmonary trunk.
A 45 y.o. man presents with a BP of 160/90 on the right arm and 170/92 on the left arm,. There are no palpable pulses in the feet/ankle. What problem does this patient most likely have?
Coarctation of the aorta: the hypertension of the upper extremities and hypotension in lower extremities.
RFF: Continuous machinery-like heart murmur.
Patent ductus arteriosus.
RFF: "Boot shaped heart"
Tetralogy of Fallot.
RFF: Rib notching.
Coarctation of the aorta.
RFF: Most common congenital cardiac anomaly.
Ventricular septal defect.
RFF: Most common congenital cause of early cyanosis.