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congenital heart disease 2


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nml sequence with shunt (ostium secundum or primum) acyanosis, increased PBF, asymptomatic in childhood, systolic murmur, L to R shunt, complications involve pulmonary htn, CHF, arrythmia, HF,
sponatenous closure, surgical repair
nml sequence with shunt, acyanosis with increased PBF, perimembranous or muscular, physiologic consequence depends on size, no symptoms until 4-6 wks, increased resp effort and fatigue, therapy to surgery
nml sequence with shunt, acyanosis with increased PBF,
incomplete AVSD (partial AV canal, ostium premum ASD with cleft mitral)
transitional AVSD large ostium primum ASD and smal inlet VSD
Complete AVSD complete AV canal, large ostium primum ASD and large inlet VSD with common AV valve, 2/3 of pt with Downs
most need surgery
nml sequence with shunt, acyanosis with increased PBF, normally closes within 10-18 hrs of life, consquence depends on size, could end up with eisenmenger, CHF,
Aortic Stenosis and Atresia
nml sequence with obstructino, acyanosis with nml PBF, valvar (uni, bi, tri cuspid), subvalvar (membrane tissue or thickened ring TOF), supravalvar (hour class, medial hypertrophy, athersclerosis, rubella), atresia (males, asso with HPLHS)
sudden death, murmur, DOE, angina, syncope, sugical repair
Pulmonic Stenosis/Atresia
nml sequence with obstruction, acyanosis with nml PBF,
valvar (dome shaped, bicuspid)
subavalvar (asso w VSD or williams)
supravalvar (rubella)
atresia (imperforate valve, PBF requires PDA)
murmur, dyspnea, surgical repair
Aortic coarctation
nml sequence with obstruction, acyanosis with nml PBF, shelflike narrowing opposite DA, tubular hypoplasia (elongated narrowinh of aorta), CHF, hypertension, murmur, medical therapy, surgery
Hypoplastic Left heart Syndrom
nml sequence with obstruction, cyanosis with increased PBF, underdevelopment of left side of heart, often coarctation, RV hypertrophy, pulm return through FO (L to R), systemic flow dependant on PDA, coronary flow is retrograde through DA
shunt is necessary for life, as it closes death ensues unles intervention, PGE2 or surgery
Transposition of Great Arteries
abnml ventriculo arterial connection (discordance), cyanosis with increased PBF, aorta arises from RV and pulm art from LV, circulation is in parallel, tissue ox depends on blood mix through ASD, VSD, or PDA.
d-TGA: aorta is ant and to rt of pulm art, AV concordance parallel circ
l-TGA: aorta is ant and to left of pulm art, AV discordance "corrected" transposition (series circ)
coronary abnml,
arterial switch procedure
Truncus Arteriosis
abnl ventriculo arterial connection, cyanosis with increased PBF, single arterial trunk arises from single semilunar valve and supplies aorta, CA and PA, from incomplete or failed sept of embryonic TrArteriosus, truncal vessel overrides VSD, semilunar cusp is 3/4, DA is absent (not necessary), surgical repair
Tetralogy of Fallot
abnl ventriculo arterial connection, cyanosis with increased PBF,
VSD, Rigt ventricular outflow obstruction (subvalvar pulm stenosis), overriding aorta, rt ventricular hypertrophy
(R to L shunt)
"tet" spells
surgical repair
fetal circulatory path and postnatal changes
UV to FO to LH out aorta to cornoary, head, neck, brain, up ex. SVC through pulm art through PDA to dec aorta to placenta.
placental circ eliminated, pulmonary resistance drops and pulm flow increases 10x, DA closes functionally, FO closes
appropriate connection of atria and ventricle, and origin of pulmonary trunk and aorta
RA connectis via mitral to LV and LA connectis via tricuspid to RV, or pulmonary trunk arises from LV and aorta from RV
eisenmenger syndrome
when pulmonary pressure reaches systemic levels the shunt (L to R) may swtich (R to L) and result in cyanosis

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