NBME 10. 200Q Cardiac pathologies + 87Q marfan Flashcards
200 - visi ats; 87 - tik case marfano, kitu ats nera cia
Tetralogy of Fallot is the most likely diagnosis in … CP?
Infant with paroxysmal cyanosis during crying spells and a 3/6 systolic ejection murmur on examination.
Tetralogy of Fallot consists of pulmonary infundibular stenosis, overriding aorta, ventricular septal defect (VSD), and right ventricular hypertrophy => RESULTS IN WHAT SHUNT?
right-to-left shunt with cyanosis first noted in infancy.
In tetralogy: Right ventricular outflow tract (RVOT) obstruction eventually leads to right ventricular hypertrophy.
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In tetralogy: murmur?
holosystolic murmur best heard in the left lower sternal border, is caused by RVOT obstruction, not as often as by the VSD.
In tetralogy: Paroxysmal cyanosis that occurs during periods of crying is a classic presentation and these episodes are referred to as “tet” spells.
Patients in the midst of one of these spells often exhibit rapid, deep breathing, agitation, and progressive cyanosis. These periods of cyanosis occur as a result of transient obstruction or occlusion of the RVOT leading to decreased blood flow through the pulmonary circulation, although the cause of the underlying transient obstruction is unclear.
In tetralogy: Tx?
surgical correction. Surgery aims to relieve the RVOT obstruction, close the VSD, and separate the pulmonary and systemic circulation. Surgery is usually performed in the first 6 months of life and almost always within the first year.
Kiti ats: Aortic stenosis, prevalence in kids?
is uncommon in children.
Kiti ats: Aortic stenosis, CP?
Systolic murmur that radiates to the carotids. It can present with exertional syncope when severe but does not cause cyanosis.
Kiti ats: Atrial septal defect. shunt?
Common congenital malformation of the interatrial septum that results in a left-to-right shunt between the atria
Kiti ats: Atrial septal defect. What happens with blood?
Mixing of oxygenated blood from the left atrium with deoxygenated blood returning from the superior vena cava results in higher oxygen concentration in the right atrium and ventricle than normal. It does not cause cyanosis.
Kiti ats, Coarctation of the aorta. CP?
Often presents in childhood with hypertension in an upper extremity and a systolic murmur. It is associated with weak lower extremity pulses and differentially lower blood pressure in the lower extremities.
kiti ats. Patent ductus arteriosus, connection between what?
is a persistent conduit between the aorta and the pulmonary artery that has failed to obliterate. It results in a continuous, machine-like murmur heard in the left second intercostal space, radiating to the clavicle.
Kiti ats: Transposition of the great arteries.
Results in aberrant reversal of the normal anatomical association of the aorta and pulmonary artery to the cardiac chambers secondary to failure of the aorticopulmonary septum to spiral.
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Kiti ats: Transposition of the great arteries.
This results in separate systemic and pulmonary circulations, with the aorta leaving the right ventricle and the pulmonary trunk leaving the left ventricle. This is incompatible with life unless an additional shunt allowing admixture of blood is present.
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Kiti ats. Truncus arteriosus.
results from failure of the aorticopulmonary septum to divide the aorta and pulmonary trunk. Most patients present with a concomitant ventricular septal defect and cyanosis noted in the neonatal period, but cyanosis is not paroxysmal.
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Kiti ats. Truncus arteriosus. what cyanosis?
cyanosis noted in the neonatal period, but cyanosis is not paroxysmal.
Kiti ats. Ventricular septal defect.
murmur, cianosis?
It is characterized by a holosystolic murmur best heard in the left lower sternal border. It does not cause cyanosis.
87Q. marfan syndrome, what evaluation need prior surgery (in the case was hermioplasty).
Cardioecho
87Q. marfan - how often cardioecho?
Patients with Marfan syndrome are at increased risk for vascular complications, such as aortic root dilation, aortic aneurysm, and aortic dissection, which may lead to abrupt death at an early age.
Patients with Marfan syndrome should undergo annual echocardiography to screen for these complications.
87Q. marfan - what other evaluation apart cardio?
Annual ophthalmic examination should be performed to identify characteristic features such as ectopia lentis, as well as myopia, iris transillumination defects, iridodonesis, or phacodonesis, and to prevent complications such as cataract, glaucoma, or retinal detachment.