Congenital Flashcards
BAV screening
First degree relatives
Secundum ASD anatomy
Absence of tissue in region of fossa ovalis (center of septum)
Secundum ASD shunt
Left to right shunt
RA and RV enlargement
Holt Oram Syndrome
Heart-hand syndrome
TBX5 mutation, AD
Associated with secundum ASD
Secundum ASD EKG
RAD, RBBB from RV overload
R wave notches in inferior limb leads
Secundum ASD closure indications
Symptoms class I No symptoms class IIa No PVD - PASP < 50% systemic, PVR < 1/3 SVR, no R to L shunt on pulse ox
Sinus venous defect
Superior portion of septum, posterior to fossa ovalis
A/w anomalous RUPV
L-> R -> RV overload
Sinus venous defect treatment
Surgical closure if
impaired functional capacity
R enlargement
No cyanosis or PVD
Primum ASD
Partial A-V canal defect, inferior portion of septum
A/w Downs
AV valves abnormal, on same level, MV usually cleft
R enlargement
RBBB and LAD
Primum ASD treatment
Surgical closure if
impaired functional capacity
R enlargement
No cyanosis or PVD
Primum ASD associated lesions
- Secundum ASD
- VSD
- PS
- Subaortic stenosis - abnormal MV insertion in LVOT
- Left SVC
- Coarctation
Complete AV canal defect
Atrial defect above valve, ventricular below
One big AV valve
L->R shunt -> PVD
Huge R sided enlargement
Must be repaired in infancy or Eisenmenger’s develops
Look for anomalous PVs
RV volume overload, no ASD
Anomalous PV repair if
Qp:Qs > 1.5
Symptoms
RV enlargement
Indication to close VSD
LV enlargement
Muscular VSD
bordered by myocardium
Perimembranous VSD
Beneath commissure between R and posteirior cusps and remote from L cusp
Infundibuluar, outlet supracristal VSD
- Lies beneath commissure between R and L cusps
- Aortic prolapse
- Earlier closure to prevent AR
Sub-aortic and sub-pulmonary VSD
Should be closed to prevent AR
PDA closure
- LA or LV enlargement with net L to R shunt
- PASP < 50% systemic & PVR < 1/3 systemic
Pulmonary stenosis associated with
Noonan’s syndrome
PS severe
PG > 65
Peak vel > 4
MG > 35
PS treatment
Balloon if mod-severe and HF, cyanosis or exercise intolerance without other reasons
Surgical if ineligible or failed balloon
20 years after Pulm balloon valvotomy complications
Arrhythmias
RV enlargement
TR
PR
Indications for coarctation repair
HTN + significant coarctation
Arm/leg peak-peak grad >20 or mean doppler systolic > 20
Arm/leg >10 or mean doppler systolic > 10 with collateral flow
Arm/leg or doppler > 10 + decreased LV function or AF
Ebstein’s indications for repair
Decreased exercise capacity Cyanosis (risk for stroke) Severe TR Bypass tract Severe RV enlargement with onset of dysfunction
Holt Oram a/w
Secundum ASD
Down syndrome a/w
AV septal defects (partial or complete AV canal)
Noonan syndrome a/w
Pulmonary stenosis
Turner syndrome a/w
coarctation
Phlebotomy for complex CHD indications
Symptomatic hyper viscosity with Hb > 20, Hct > 0.65
Fluids
If symptoms and Hit <0.65, suspect iron deficiency
Treatment of complex CHD iron deficiency
Ferrous sulfate once daily
Discontinue when Hct rises rapidly
Therapy for Eisenmenger’s
PH meds
ERAs
PDE5i
Prostacyclin agonists
Tetralogy of Fallot
Large sub aortic VSD
RVOT obstruction +/- PS
Overriding aorta
RVH
ToF associations
DiGeorge syndrome (low nasal bridge and ears, wide eyes)
Right aortic arch
Anomalous coronary arteries
Secundum ASD
ToF simple repair
Close VSD
Resect RVOT muscle
+/- Pulm valvotomy
ToF complex repair
RVOT patch
Excision of Pulm valve
RV to PA conduit
Issues after ToF repair
RV dysfunction if late Residual RVOT gradient, VSD Severe PR most common Post-op RBBB Arrhythmias
Replace PV in ToF
Before irreversible RV dysfunction
Shunts for ToF
Cynatoic heart disease with low pulmonary blood flow
Improves oxygenation, makes small PAs grow
Problems: distortion of PAs, pHTN and LV overload, no heart-lung transplant if lateral thoracotomy
Classic Glenn ToF
SVC -> R PA
Bidirectional Glenn ToF
SVC -> both PAs
Blalock-Taussig ToF
Subclavian -> PA
Waterston ToF
Ascending Ao -> R PA
Potts ToF
Descending Ao -> L PA
dTGA
Morphologic RV on right
Aorta anterior and to the right, from RV
L TGA
RV on left
Aorta anterior and to left
Congenitally corrected transposition, blood going to right places
dTGA surgery
Arterial switch
Atrial switch
Arterial switch dTGA
Great arteries switched, coronaries reimplanted
Mustard dTGA
Baffle from IVC -> SVC -> LV
RV becomes systemic ventricle
Mustard dTGA complications
- Eventual RV failure, TR
- Atrial arrhythmias
- Baffle obstruction, leak (ASD) - beware PPM
lTGA associated with
- VSD
- PS
- Tricuspid / left systemic AV valve regurgitation
- Systemic ventricular dysfunction (may be from leaky AV valve)
- Conduction abnormalities >75%
- Inversion of bundles, absence of septal Q waves, have inferior Q waves
- CHB
Doublet inlet ventricle
Two AV valves into single ventricle
Single inlet ventricle
One AV valve entering ventricle
TV atresia, ASD
Common inlet ventricle
One big AV valve entering ventricle
Fontan
For single ventricle
Diverts blood from vena cave, RA -> PA
BAV surveillance with ascending aortic aneurysm >4.5 cm
Annual
BAV surveillance with mild AS, no aneurysm
Every 3-5 years
Coarctation surveillance after intervention without symptoms
MRA or CTA every 3-5 years
Takayasu arteritis criteria (look for 3+)
Age < 40 Claudication Dec brachial artery pulse Subclavian artery or aortic bruit SBP variation > 10 mm Hg between arms Angiographic (CT, MR) evidence of aorta or branch vessel stenosis
Platypnea Orthodeoxia Syndrome
Dyspnea and hypoxemia when upright
Resolves when supine
Intracardiac shunt, pulmonary conditions
agitated bubble study in supine and upright positions
Causes of Playpnea-orthodeoxia
PFO
ASD
Atrial septal aneurysm with fenestration
Pulm - interstitial lung disease, HPS, Pulm AVMs
PFO and platpnea orthodeoxia syndrome occur if
Distortion of intertribal septum in upright position Post-pneumonectomy, lung transplant Right hemidiaphragm paralysis Kyphoscoliosis Ascending aortic dilatation
