Fetal Circulation

RBC  RA via SVC  RV or LA (through PFO vs foramen ovale)

-if coming from head, blood goes downwards to RV  pulm valve  pulm artery  mixed oxy/deoxy goes to ductus arteriosus to body
-blood from placenta  IVC  across foramen ovale  down into LV and ejected out of aorta
-high pulmonary vascular resistance (impediment to flow). Low SVR. 2 Right to Left Shunt (ductus arteriousus, foramen ovale)
-pulm vascular resistance is not same as high pulmonary HTN (pressure)

Birth:
-lungs expand, SVR inc, dec PVR, closure of foramen ovale (closes functionally bc R sided pressures less than L side). Closure of ductus arteriosus
begins to close (seals over time but closes after PFO, harder to reopen. Closes from oxygen tension).
-pulm vascular resistance over time is sigmoidal curve, still slowly coming down weeks to years after birth

Neonatal CV System
-ANS immature – parasympathetic tone dominant, low HR at baseline and little to no inc in HR for decreases in BP
-LV non compliant
-CO is heart rate dependent. Fewer contractile elements. Inadequate T tubules. Dependence on extracellular calcium for inotropy.
-High degree ventricular interdependence 2ndary to LV and RV having equal mass  failure of one leads to biventricular failure.

Preop Congenital Cardiac Patient

-did baby go home with mom
-was baby intubated
-general health, exercise tolerant
-current growth status curves
-recent URI (changes in PVR)
-signs of CHF – diaphoresis with feeding, tachypnea, poor/lengthy feeding, recurrent URI
Physical Exam
-weight gain
-cyanosis – perioral, periorbital, distal extremities
Tests: 2D Echo, SpO2, H/H (higher Hct in cyanotic patients as they need it to maintain o2 delivery)

PDA:
-cont machine like murmur
-can be closed transcatheter or surgical approach (premies or older kids with large PDA not amenable to closure via device)
-preop risk: SBE, inappropriate ligation, RV failure
-pre and post ductal saturation should be monitored

-which is NOT ductal dependent? Hypoplastic left heart syndrome (aortic atresia), tricuspid atresia, complete AV canal, pulm atresia intact
ventricular system, D transposition of great arteries

Coarctation of Aorta
-upper body HTN absent/diminished femoral pulses
-periop risk – neuro deficit, SBE
-monitor upper and lower body pressures

ASD
-pulm overiculation
-often diagnosed in adulthood (freq headaches)
-anatomic closure of PFO as early as 3 months

VSD
-most common congenital defect
-systolic murmur
-periop risks – air embolism, shunt reversal

AV Canal – systolic and diastolic murmur, cardiomegaly, high incidence with trisomy 21

Transposition of Great Arteries

-cyanosis CHF, egg shaped heart on CXR, LVH, RVH
-coronary abnl
-periop risk – maintain balance b/w systemic and pulm circulations
-head vessels are transposed;

Mustard/Senning Procedure:
-atrial baffles used to redirect blood flow
-involves long suture lines through atria leading to arrhythmias
-RV still systemic ventricle and prone to failure overtime
Truncus Arteriosus
-rare
-characterized by common outlet for aorta and main PA, single valve and VSD
-single ventricle physio

Total Anomalous Pulm Venous Return

-only neonatal surgical emergency
-partial vs total
-obstructed vs unobstructed
-TAPVR: supracardiac, cardiac, infracardiac, mixed

TOF:
-tetralogy includes: RVH, VSD, pulmonic stenosis, overriding aorta (RVOT b/c of VSD)
-cyanosis, hypercyanotic tet spells, boot shaped heart, RAD on EKG.
-periop risks: hypercyanotic spells, systemic emboli, polycythemia  thrombosis, RH failure
-kid squatting on bed, raise knees, maintain SVR, inc preload, dec infundibuliar spells
-most common seqelae of TOF repair is free pulmonary insufficiency/regurge

-What manipulations inc PVR?

-dec PO2, inc PCO2, very high hct (inc blood viscosity), high mechanical ventilation volumes, very low mechanical ventilation volumes,
atelectasis, excessive PEEP, low TV
-What spo2 are we aiming for in single ventricle? 85%! Mixed venous sat from jugular bulb (70-75%); if flow to lungs is same, and pulm vein sat
is 100  mixing -> sat should be between 80-85%.

Single ventricle physiology:

-If heart pumps and all blood going to lungs, none left to go to system (Excess pulm blood flow). If sat is too hgh, bad because too much pulm
blood flow. If sat is 95%, BP will be hypotensive, same with if sat is too low (no blood going to lungs b/c all going to system or more likely – no
blood moving anywhere because heart is failing  low CO state)

Stage I Palliation – Norwood:

-BT shunt, atrial septectomy, creation of neoaorta with homograft patch
-goals – provide systemic perfusion independent of ductus, preserve function of isngle ventricle, allow normal maturation of pulm vasculature
Stage III Fontan Palliation
-total cavopulm connection, series circulation recovered. Management: PRELOAD!
-loss RV as pump for pulm blood flow