Congenital Flashcards
Bicuspid
Moderate to severe no sports
Asymptomatic As 2d echo
> 50 mm hg echo every year
<50 every 2 years
AS gradient more than 50
Valvuloplasty before pregnancy
Or sports
Sub aortic stenosis repaired
Residual lesions Restenosis Endocarditis vSD Heart block
Supra valvular
Coronary obstruction Williams tumor Peripheral pulmonary stenosis Surgery for mean gradient 50 and peak 70 HTN No pregnancy and screen all relatives
Noonan syndrome
Pulmonary stenosis
Repair if the mean gradient is 40 and peak 60
Ebstein
Accessary pathway in 25% Asd 50% Cyanosis due to ASD RV dysfunction Afib Peak T Pregnancy risk 6% Cardio thoracic ratio >65 need surgery
Aortopathies
Lost-dietz 4.5 cm
Marfan syndrome pregnancy 4
Usually >5cm
Coarctation
Bicuspid > 50% VSD Cerebral aneurysm 10% Aortic rupture Endocarditis ICH HTN MI High risk pregnancy
D transposition
Aorta from RV Coronary ostial abnormalities vSD 45% Lot obstruction 25% Treatment rastelli if PS present Atrial switch mustard and senning Coronary switch
D transposition complications After atrial switch Baffle obstruction SVC syndrome Baffle leak Sub pulm stenosis Residual VSD Arrhythmias RV failure
Arterial switch Coronary stenosis and LV dysfunction new aortic leak aortic dilatation RVOT stenosis After rastelli Rvot conduit obstruction RV failure Sudden death Branch pulm stenosis
Congenitally corrected L transposition
Just the ventricles switch positions
Complications
Compete heart block
Vsd
RV failure
Mustard and senning switch VSD repair aV replacement Rastelli Complications arrhythmias, LV dysfunction RV dysfunction AI
Single ventricle tricuspid atresia
Avoid air emboli
Hemodynamics are problematic
False BP readings
Bidirectional Glenn Modified fontan 1 1/2 ventricle repair Reconstruct 2 ventricle Fontan late complications arrhythmias RV thrombus protein losing enteropathy. Cirrhosis
Asd adults 25-50% Secundum 75% Arrhythmias 20% Reversible pulm HTN Closure improves morbidity not mortality
Vsd most common defect at birth 80% peri membranous Loud p2 Diastolic mitral flow Close large shunts Endocarditis AI PAH
Vsd closure
Do not for severe pulm HTN and eisenmenger
Post infarct vsd 50% mortality with surgery and 30% with device closure
PFO closure
No benifit closure 1
PC trial
Respect
And mist for migraine
Platypnea
Positional dyspnea treatment PFO closure
Eisenmenger
Edema Palpitations Polycythemia Hemoptysis Syncope Avoid pregnancy exercise heat high altitudes and iron deficiency
Tetralogy
Qrs > 180 risk for VT and sudden death
Branch pulm stenosis RV dysfunction VT may need icd RVOT obstruction LV dysfunction
PVR in adult for following reasons
Rvef 180
Lvef 20%
Mod to sev PR
Tetralogy no pregnancy
Severe pr
Arrhythmias VT usually 15% of peopke
Before repair
Association with 22q 11 micro deletion
Trisomy 21
50% CHD and 50% AVSD
Pulm HTN
Vsd
Heart block
Holt Oran
First degree AV block
aSD
Upper limb abnormalities
Turners
CHD 25%
Coarctation and bicuspid
Early root replacement
Williams
Spontaneous deletion ch7 Elfin face Supra valvular AS Branch pA stenosis Coronary artery stenosis
Digeorge
22q11 micro deletion Conotruncal tetralogy, truncus, cleft palate Hypocalcemia Immunodeficiency Right sided arch
Need holter
Tof
Tga
Ebstein
Fontan
Tetralogy of fallout
Pa pressures are normal
TGA
Baffle leak detection with bubble study
Patch on secundum defect
Need endocarditis prophylaxis
