Congenital Heart Defects - Bell Flashcards
(93 cards)
1
Q
Changes at birth:
- First breath
- Clamped placental vessel
- Others
A
- Decrease in pulmonary resistance
- Sys. vascular resistance increases
- FO closes
- PG dehydrogenase destoys PGE2 from placenta
- Decreased m coat of pulmonary vascular resistance over the next 6 weeks
2
Q
Cyanosis can be ___ or ____
A
acrocyanosis, central cyanosis
3
Q
_____ is a sign of serious abnormality
A
Central cyanosis
4
Q
Difference in limbs that is cause for evaluation
A
3%
5
Q
Central cyanosis ______ Hb
A
3-5g/dL desaturated
6
Q
Abnormality cardiac physiology that causes hypoxia without hypercarbia
A
5 terrible T’s with PS
(TAVPR, Pulm atresia, Single ventricle)
7
Q
Cardiac defects show this level even on 100% oxygen
A
Usually less than 50
PCO2 doest change (still low, normal level)
8
Q
CHF sx in infants
A
- tachypnea
- poor feeding
- tachycardia, diaphoresis
- Hyperdynamic precordium, tachy/tachy
- Hepatomegaly
- Edema
9
Q
Younger children with CHF may appear to have _______
A
gastroenteritis, with N/V/D
10
Q
Older children with CHF may present with
A
exercise intolerance, cough, anorexia, fatigue
11
Q
Pathophys. causes of chf
A
- decreased C.O.
- Increased SY tone and increased mineralocorticoids due to high RAAS
- Also Increased ANP, BNP, IGF1 ,GH
- Both lead to cardiac remodeling
12
Q
4 physiologic mechanisms that reult in HF
A
- Increased fluid load to heart (Increased preload)
- Obstructed ventricular emptying (increased afterload)
- Decreased contractility
- Abnormal rhythms
13
Q
Cyanotic lesions that lead to increased volume load
A
Decreased pulmonary blood flow
obstruction to pulm blood flow and R>L shunt
Tricuspid atresia, Single ventrical with pulmonary stenosis, tetrology
14
Q
Cyanotic lesions with increased pressure load
A
Increased pulmonary blood flow
intracardiac mixing or abnormal ventricular-arterial connection
Transposition, TAPVR, TA, Common atria or ventricle
15
Q
Top 3 Congenital HDs’
A
VSD (25-30)
ASD (Secundum) (6-8)
PDA (6-8)
16
Q
___________ associated with VSD
A
NKx2.5, GATA4, TBX5
17
Q
Two others assoc’d with VSD
A
22q11 deletion, Holt-Oram syndrome
18
Q
Gender ratio of VSD
A
equal
19
Q
4 types of VSD
A
Supracristal
Peri-membranous
Posterior
Muscular
20
Q
Large VSD can progress to…(6)
A
- CHF
- Holosystolic murmur (2 weeks) with diastolic rumble at apex
- Prominent L precurdium
- Palpable Sternal lift
- Systolic thrill
- Apical Thrust
21
Q
Small VSD’s…
A
close spontaneouslyl
22
Q
Complications of large VSD
A
Growth fail, pulm infctns (untreated CHF)
Eisenmonger’s physiology
23
Q
Long-term VSD risk
A
bacterial endocarditis
24
Q
Eisenmonger’s physiology
A
Dilated pulmonary artery secondary to pulmonary HTN
25
VSD Treatment
Diuretics, ACEi, ARB, nitrate, milrinone (or CCB)
If refractory, pulmHTN, or A regurg = close surgically
26
Complications of Percutaneous VSD closure
late (1 week) AV block
hemolysis
sepsis
device embolization
arrhythmia
thrombosis
27
Atrial septal defect gender ratio
Female to male 3:1
28
\_\_\_ is one third of the CHD detected in adults
ASD
29
\_\_\_\_is most common defect in Holt Oram syndrome
ASD
30
TF's involved in ASD
NKx,
TBX5, GATA4 (activate MYH6)
31
ASD associated with
PAPVR, MVP
32
Ausculation of ASD
First heart sound is loud (possible pulmonic ej. click)
Soft systolic ejection murumur at the 2nd interspace
*fixed widely split second heart sound*
33
ASD on ECG
RVH, R axis deviation
34
Echo in ASD
septal motion with increased RV will move **anterior in systole** rather than posterior in systole/anterior in diastole
35
\_\_\_\_ may trigger ASD complications
pregnancy
36
\_\_\_ rare with ASD
bact. endocarditis
37
Can delineate lesion and shunt in ASD
Cardiac cath
38
Surgical closure of ASD indicated with
2:1 shunt (regardless of sx)
39
Most ASD closures done when?
1-5
40
PDA female to male
2:1
41
TF associated with PDA
TFAP2B (6p12)
neural crest TF associated with Char syndrome and PDA
42
\_\_\_\_\_\_ have high incidence of PDA
preterm infants (
43
PDA - what arch artery?
VI pharyngeal arch artery
44
PDA caused by either
Deficiency of the endothelial and musclar layer of vessel, regulatory proteins that direct fibrosis
OR
Another cardiac lesion that requires this to remain open
45
Flow through PDA is initially..
in systole, then in both systole and diastole
46
PDA presentation at birth
normal but with increased peripheral pulses
47
Large PDA presentation
2-6 weeks will be signs of CHF
LV thrust
Continuous murmur at ULSB, radiating to the back
Hepatomegaly
Peripheral bounding pulse
48
Small PDA will have normal \_\_\_\_\_\_\_
CXR and EKG
49
PDA Tretment
surgical closure for ALL lesions
50
Indomethacin is used when?
Premature infants with PDA
-\> everyone else gets surgical closure
51
Most common Coarc
juxtaductal
(below the origin of the subclavian artery at the ductus)
52
Gender ratio of Coarc
males to female 2-5:1
53
Coarc associated with
Turner's
(bicuspid AV, VSD, PDA, Mitral stenosis or regurg, CoW aneurysms)
54
LVOTO association
Coarc
55
HTN in coarc due to
decreased renal perfusion
56
Adult presentation of coarc
HTn
HA
epistaxis
dizziness
palpitation
57
What makes the Diagnosis in Coarc
higher BP in arms with bounding pulse, while LE pulses distant
58
Ausc. of coarc
thrill or systolic ejection click from bicuspid AV
May be systsolic murmur of AS at 3rd R interspace
OR
systolic murmur of collateral circulation over R and L sides of the chest
59
BA esophagram in coarc
Displacement of the esophagus
60
Unoperated coarc may cause
early death from UE HTN
Intracranial hemorrhage
Aneurysms in brain, descending aorta, collat. circ
Hypertensive encephalopathy
61
PGE1 infusion for whom?
coarc
repair after
62
\_\_\_\_ is major problem in coarc
bacterial endocarditis
63
\_\_\_\_ stents may decrease the recoarctation rate
Endovascular stents
64
Coarc need what?
SBE prophylaxis
65
coarc complications
postcoarctecctomy synd.
HTN and abdominal pain from inability of mesenteric arteries to regulate BP with new perfusion
66
Most common cyanotic HD diagnosed in adults
tetrology
67
22q11 testing indicated for TOF if...
associated with absent pulmonic valve, arch abnormalities, or aorto-pulmonary collaerals
68
Short TOF presentation
sleep and muscle weakness
69
Prolonged TOF presentation
unconciousness and convulsions
70
TOF older child presentation
Progressive cyanosis, dyspnea, cyanosis
Squatting episodese (helps compress LE arteries and increase aortic resistance so pulmonary flow is increased)
71
Murmur in TOF
systolic ej. murmur of pulmonic stenosis
holosystolic murmur at LLSB of VSD
72
CXR of TOF
Boot shaped heart
73
TOF tx
PGE1, O2, hypoglycemia and anemia Tx
Corrective surgery
If underdev. pulm arteries, palliative procedures like Blalock-Taussig shunt or palliative balloon valvuloplasty
74
Administer ___ if O2 doesn't help with tet spell
Morphine
Causes vagal stimulation, increased PSy tone
75
Other Tet spell treatments
Acidosis Tx
Phenylephrine
Propranolol
76
If TOF patient is febrile before surgery, consider
brain abscess or endocarditis
77
40-50% of TOF have
ventricular arrhythmias
78
\_\_\_\_\_\_\_\_\_\_\_\_ occurs when aorta arises from RV and is anterior to the pulmonary artery
D transposition (Complete transposition)
79
TGA requires
PDA and PFO
80
TGA clinical presentation
Cyanotic
## Footnote
**Loud single second heart sound**
81
CXR for TGA
Egg shaped wih narrow mediastinum, cardiomegaly,
increased pulmonary vascular markings if VSD present
82
Mortality of untreated TGA
90% by 6 months
83
Jatene =
Arerial switch procedure
performed within 1-2 weeks
84
TGA treatment - After 2 weeks
Two stage switch can be completed
85
TGA infants with VSD may have
switch and VSD closure after 2 weeks
because LV pressures are high with flow through VSD
86
Arterial treatment for TGA
Morbidity?
Jatene switch
Morbidity: Supravalvular stenosis at reanastomosis sites, dilatation of aortic root with valcular insufficiency
87
Atrial repair of TGA
Morbidity?
Atrial switch (Mustard/Sennig)
Morbidity: arrhythmias, **baffle obstruction** of flow
88
LTGA
present in HF and cyanosis
89
TGA gene
ZIC3
90
Gene for PDA
TFAP2B
91
Pulmonic stenosis gene
GATA4, ZIC3
92
TOF gene
NKX, TX5, FOG2
93
\_\_\_\_\_\_\_ can be normal in ped exam
early systolic vibratory ej. murmur
