Congenital Heart Disease 2 Flashcards
Pink hands and blue feet. Dx?
Eisenmenger’s with PDA
Large shunt (usu VSD)--> VSD--> reverse to R to L shunt w/ cyanosis. Sxs = SOB, Syncope, hemoptysis, stroke Dx?
Eisenmenger’s
Cyanosis/clubbing, a-wave JVP, RV heave, click, diastolic murmur of PR from PA dilation, loud P2. Dx?
Eisenmenger’s
Eisenmenger’s CXR
Big “mogul #2” from big PA, calcified PA
Drug class to avoid in eisenmenger’s
Vasodilators (no NTG)
Rx for eisenmenger’s
PAH Rx: Bosentan, sildenafil, prostacyclin
Tet of fallot
- large sub aortic VSD
- RVOT obstruction (+/-PS)
- overriding Ao
- RVH
DDR of overriding Ao
TOF, Pulm atresia, truncus
Which lesion in TOF determines security of TOF?
RVOT obstruction (b/c blue blood sneaks over from RV to LV and causes cyanosis)
Difference between RV and LV pressures in TOF
RVp = LVp
Name 3 associated lesions with TOF
- R- Ao arch on CXR
- anomalous coronary artery
- Secundum ASD
TOF PE
Cyanosis RV lift Thrill if PS severe Systolic murmur over pulmonic area ABSENT P2
Most common problem after TOF repair
PR–> RV inc–> inc QRS–> VT–> SCD
EKG findings in TOF
RBBB
Wide QRS
What is the repair for TOF?
RVOT patch
Close VSD
Excise PV
What is next step if pt with TOF repair comes in with arrhythmia ?
Look for Hemodynamic abnormality (most likely PR which should be promptly corrected with PVR)
What is the purpose of creating a shunt for congenital heart diseases?
Increase blood flow through pulmonary vasculature
Glenn
SVC–> RPA
Bidirectional Glenn
SVC to RPA and LPA
Blalock-Tausigg
Subclavian Artery–> PA
Waterston shunt
Asc Ao–>RPA
Potts Shunt
Desc Ao–> LPA
Complications of creating shunts for congenital heart disease
Distortion of PA
Large shunt= Pulm HTN and LV overload
No heart/lung Tx if Lateral thoracotomy
When do you consider phlebotomy for a patient with cyanosis HD?
Hgb> 20 , Hct > 65
If pt has iron deficiency and cyanotic CHD, what are they at risk for?
Stroke
What do you do if pt has sxs of hyperviscosity and Hct> 65? <65?
Hydrate
Suspect iron def, give gentle iron suppl.
How much should you phlebotomize a pt with cyanotic CHD?
Max 1 u
Are cyanotic CHD pt’s at risk for thromboemboli or bleeding?
Bleeding
What should be done for all IV lines attached to cyanotic CHD pt’s?
Place air filters to avoid air emboli
Name a spinal problem that cyanotic CHD pt’s get and can you do Sx?
Scoliosis
No- unacceptable bleed risk
Name a rheumatologic dz that cyanotic CHD patients get
Gout
Mortality rate for patients with cyanotic CHD who get pregnant
50%
D-loop
RV on R
Ao- anterior and to R
L-loop
RV on Left
Ao- anterior and to left
What is an arterial switch and what dz do you use it for?
TGA
just switch arteries back to correct ventricles
Main step in Mustard procedure for TGA
Take blue blood from SVC/IVC into LA
Long term complication of Mustard procedure for TGA
RV Failure needing OHT
Name the lesion in L-TGA
RV and LV switched
Echo for L-TGA
TV is on Left. And as always apically displaced
What is L-TGA associated with?
VSD, PS, TR, CHB, RV Dysfxn
L-TGA ECG
Q waves in II, III, aVF
AVB
3 types of single ventricle, atrioventricular connections
- double inlet
- single inlet (tricuspid atresia)
- common inlet (one AV valve)
What lesion must a single inlet ventricle have to survive?
ASD
Rx for single ventricle?
Fontan
What is goal of very contain procedure?
To separate blue and red blood
What arrhythmias are our tan patients prone to? Why?
Atrial
Inc RA size
What is the major complication of increased RA size in fontan?
RA Thrombus–> PE
What to remember about treating atrial arrhythmias in fontan patients?
Always r/o clot with TEE first
Fontan pt comes in with pleural effusion, ascites, diarrhea, JVD. Suspected Dx and next tests?
Protein Losing Enteropathy
Serum albumin, Upr, STOOL alpha1 AT
Rx for protein losing Enteropathy after fontan
Repair fontan obstruction
Pathophysiology of protein losing Enteropathy in fontan. Mortality rate?
Inc’d systemic venous P–> inc’d thoracic duct P
50% at 5yrs
Another name for congenitally corrected transposition
L-TGA
