CYANOTIC

CONGENITAL HEART
DISEASE
Approach to the PatientGive 100% oxygen: "The Oxygen Test'If PaO inereases > 100 tor
think Lung Disease!If PaO does not change.. think Heart Disease!
• Disease-Approach to the PatientThink about the famous "T" defectsTetralogy of FallotL
Transposition of the Great ArteriesTricuspid (and/or Pulmonary) AtresiaTruncus
ArteriosusTotal Anomalous Pulmonaryy Venous Return
• For these babies it is essential to consider PGE1(0.05 mcg/kg/min) to open the ductus to
providepulmonary blood fulllWatch for flushing, low-grade T,and apnea (1) as side
effecis.o
• CHD with Decreased Pulmonary Blood FlowPulmonary Atresia Tricuspid AtresiaVery
cyanotic Very cyanoticwhen PDA coses when PDA closesDiminished flow on x-ray
.Diminished flow on x-rayECG shows ECG shows characteristic"normal axis LVH, left
axis deviation!!Little or no murmur Little or no murmur
• CHD with Decreased PulmonaryBlood Flow-Management.PGE1 in NICU T SConfim Dx
by echoor cathSurgery to providepulmonary blood flow
• CHD with Decreased PulmonaryBlood Flow Management.For most patientswith
puimonar Siae(and all with tricuspid atresia!!),the RV is too small to ever beusedA Fontan
Procedure(RV bypass) is performedin the first 1-2 years of life!
• CHD with Decreased Pulmonary Blood FlowTetralogy of Fallot(10% of aii CHD)
RedcdBlood F lowHarsh systolic ejecuon Thc Tolungsmurmur, Single S2 Puinonery
Amat LM-USB in a cyanotic Voe VSDbaby (clubbing and NorwSquatting older child;
rarenow!l)
• CHD with Decreased Pulmonary Blood FlowTetralogy of Fallot JV Hearl(10% of all
CHD)ReduadUsually complete repair Blood Fkow(close /SD, open RVOT) Thec
Tolungsat about 6 months of age Pulrnonnny AotaValNorow VSDKnow about the Tet
spell Ottreat with O2, knee-chestposition, bicarb, morphine,phenylephrine (Neo- Ih R
VerdSynephrine), andbeta-blocker!
• Cyanotic CHD with Normal orIncreased Pulmonary Blood FlowTransposition of Great
Arteries (TGA)Most common cause of (intense) cyanosis in newbornperiod (5% of all
CHD)Male infant more commonLoud single 8aNo mumur often (unless VSD
present)"Egg-shaped heart on chest xray
• HD WebcamHO Video ConterencimTransposition of the Great Arte2 circulattons i
paiallelMay improve witn PGE1(open ductus) AoPANeed to providefor
• Surgical Repair of TGACurrent ManagentArterial Switch AoOperatioon PAPerformed at
RA2-3 weeks of age
• Cyanotic CHD with Normal orIncreased Pulmonary FlowTruncus Arteriosus(1% of
CHD)VSD+ one large vesselcoming off the heart; TrunPulmonary arteries branchoff of
thisTruncal valve often4-5 leaflets
• Cyanotic CHD with Normal orIncreased Pulmonary FlowTruncus Arteriosus(1% of
CHD)Usually mild cyanosis becauseof normal-to-increasedpulmonary flow TruncalMay
show signs of CHF Vave.Px: "Click" and SEM VSOECG: BVH RV LV.Remember FISH
test ASOfor 22 q11-!!
• Cyanotic CHD with Normal orIncreased Pulmonary FlowTruncus Arteriosus Truncus
Repair(1% of CHD)Treat CHF medically 1 ard C'und atfren(cliuretics, digoxin) P
CondawwwSurgical repair 1sD patcbrd(the Rastelli operation)at about 6 months of age
• HD WebcamHD Video ConierencangCyanotic CHD with Normal orIncreased Pulmonary
FlowTotal Anomalous LeartIinePulmonar enau S VerbcmReturn (TAPVR) Vein(1% of
CHD) VC40% to RA or CCS Fught AumCoronnryYSinus30% to left SVC nlaidRught
gob VinVentncd25% to IVC TRTA ANOMALOUSPULMONAYVENOUS
DRANAGE147:21
• Total Anomalous PulmonaryVenous ReturnIf venous returm is."Unobstrectesee increased
flowWon the x-ray, and therewill be minimal cyanosisTypical "snowman heart'in TAPVR
to the left SVC
• Total Anomalous PulmonaryVenous ReturnIf venous retum is:"Obstructedvouwillceea
small heart and pulmonaryyedema on x-ray, and therewill be intense cyanosisIn this case,
urgent(vs. elective) operation isneeded, i.e., connectpulmonary veins to the
• Complex" Cyanotic Heart DefectsDoube Outhet iDouble Otett dSingle Ventricle DORV
RADegree of cyanosisdepends on degreeof PSWith PS, a harsh SEMis heard
SingleSurgical repair Ventricle ate Windowat1 year of age
• CHF in the First Neek of LifeNewboms presentingwith decreasedperfusion, acidosispoor
pulses, enlargedliver, tachypnea,gallop rhythm..Think:Obstruction to leftheart flow