Quiz III Flashcards
(60 cards)
Define cardiothoracic ratio and normal value
ratio for heart diameter to chest diameter at widest points
-should be less than 50%
Where does pericardial effusion occur?
b/w the visceral and parietal layers of the pericardium
Length of normal aortic arch on frontal CXR
<4cm from left border of trachea to lateral border of the aortic arch
Age or atherosclerotic disease can have what morphological effect on the aorta
Can become elongated and tortuous
Appearance of dilated ascending aorta on CXR?
Will appear as a CONVEX shape above the right atrium where there should be straight line representing SVC
Signs of dilated main pulmonary artery on CXR
- larger than aortic arch
- extends to the left of line drawn tangentially to aortic arch and apex of heart
Descending pulmonary artery should be less than?
16 mm (seen on the right side)
Left atrial enlargement on CXR
- left side
- right side
LEFT - left atrial contour becomes straight or convex (normally concave)
RIGHT - double density sign
Also elevation of left main bronchus
PPH puts increased pressure on?
Right ventricle -> enlargement
Match the following conditions with their respective SEPTAL defects
Down Syndrome
Trisomy 13
22q11
Down Syndrome: AVSD, primum defects
Trisomy 13: VSD, ASD
22q11: Posterior malalignment VSD
Match the following conditions with their respective VALVULAR defects
Trisomy 18
Noonan Syndrome
Trisomy 18: polyvalvular disease, VSD
Noonan Syndrome: pulmonary valve stenosis, hypertrophic cardiomyopathy
Match the following conditions with their respective CONOTRUNCAL defects
22q11 Deletion Syndrome
CHARGE Syndrome
Turner Syndrome
Williams Syndrome
22q11 Deletion Syndrome: Interrupted Aortic Arch type B, truncus arteriosus, tetralogy of Fallot, right aortic arch
CHARGE Syndrome: Tetralogy of Fallot, double outlet right ventricle, right aortic arch
Turner Syndrome: Coarctation, bicuspid aortic valve, hypoplastic left heart syndrome
Williams Syndrome: Supravalvular aortic stenosis, Branch PA stenosis
Down syndrome
- which CHD
- why at risk for AML?
- AVSD
- GAT1 mutation -> transient myeloproliferative disorder
Trisomy 18
- mutation
- maternal or paternal
- survival?
- CHD?
- features?
- 50% meiosis II nondisjx
- 95% maternal (age correlation)
- 5-10% survival after 1 year
- 50% w/in 1st week
-CHD -> polyvalvular abnormalities
FEATURES
- prominent occiput
- feet rounded at bottom w/ prominent heels
- central apnea
Trisomy 13
- features
- CHD
- survival
FEATURES
- Giant sore on top of head
- Bilateral cleft lip and palate
- Dysplastic malformed ear, small head, microcephaly, polydactyly
- CHD -> VSD
- survival - median = 7 days
What 3 syndromes is TOF associated with?
22q11.2 deletion -> 16%
Alagille Syndrome -> 12%
Down’s -> 5%
22q11.2 features
What testing is important?
- Hooded eyes
- Low set, rotated ears
- Micrognathia
- HYPOcalcemia – no PTH
- Immune deficiency (DiGeorge’s)
- Feeding problems, cleft palate
- Learning probs, behavior issues, psychosis
- TEST PARENTS!!!
Williams Syndrome
- CHD
- gene
- characteristics
- risk of?
- mutation?
- unique characteristics
CHD - supravalvular aortic stenosis
Gene -> 7q11 and ELN-1
CHARACTERISTICS
-Elevated Serum calcium, urine calcium output
-FTT
-Developmental delays
-Supraorbital fullness, stellate iris, long
philtrum, large mouth with full lips
-Risk of sudden cardiac death with anesthesia due to decreased elastin in coronary arteries
Coarctation of aorta associated with?
Characteristics of this syndrome
Turner Syndrome
-NORMAL IQ but visuaspatial difficulties
• Tiny hyper-convex fingernails
• Lymphedema of feet and hands
• Webbed neck
Noonan Syndrome
- CHD
- Mutation
- Features
CHD -> PVS + HCM
-mutation -> RAS/MAP kinase pathway
Features • Wooly/curly hair with family members that have different hair • Pectus excavatum • Widely spaced • Ptosis of the eyes • Eyes slanting DOWN (DOWN’S SLANT UP) • Broad forehead and small chin • Webbed neck • Scoliosis • Low posterior hairlines • Bleeding diathesis
CHARGE syndrome
- define
- heart defect
- can be associated with
- cranial nerve dysfx includes
- mutations or deletions in
- Coloboma, Heart defect, choanal atresia, mental/growth retardation, GU anomalies, ear anomalies
- conotruncal defects
- associated w/ Kallmann’s
- CN I, VII-X
- mutation -> CHD7 -> needed for neural crest cells
Describe autonomic control of HR and SV
SV (contractility) -> ONLY under SNS control
HR - influenced by PNS and SNS
Venous return and what go hand in hand?
Stroke Volume
Blood volume adjustment occurs at the level of the?
KIDNEY - long term
-regulate size of the peripheral venous pool -> venous return -> cardiac output
