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TAPVR: Total Anomalous Pulmonary Venous Return

2% of all CHD
Male-female ratio 4:1
No communication between pulmonary veins and left atrium (LA)
Supracardiac: 50%. Right SVC
Cardiac: 20%. Coronary sinus or RA
Infracardiac: 20%. Portal vein, ductus venosus, hepatic vein, or IVC
Mixed: 10%
ASD >>>>> RIGHT to LEFT (low pressure)
Pulmonary venous return flowing away from the heart


TAPVR: Without Pulmonary Venous Obstruction

CHF and growth retardation. Mild cyanosis
Precordial bulge with hyperactive RV
Right ventricular and atrial hypertrophy (RVH – RAH)
Mod to marked cardiomegaly. “Snowman” sign: both SVC and vertical vein enlarged, fills out mediastinum more than usual: pulmonary vv dumping into SVC, vertical vein (anomalous, should be into LA)


TAPVR: With Pulmonary Venous Obstruction

Marked cyanosis and respiratory distress in the newborn period

plenty of blood flow, but can't get out of venous system due to obstruction

without pulmonary venous obstruction:
may enter into RA from pulmonary vein confluence
ASD: R-->L, some systemic circulation (mixed blood), some to pulmonary artery

may sat 90-91%, eventually RHF, CHF


RAPVR infradiaphragmatic (with pulmonary venous obstruction)

dumping in hepatic vein, prevents oxygenated blood from entering systemic circulation

nothing will help them, plenty of blood flow to lungs, but can't get out of venous system: needs sx


Tricuspid Atresi

1-3% of CHD
Absent tricuspid valve and hypoplastic RV
All have inter-atrial communication
VSD in 90%, or PDA are necessary
Right ventricle is hypoplastic
Severe cyanosis from birth
Single S2. Grade 2-3/6 systolic murmur of VSD


Tricuspid Atresia BF

LA receives both the entire systemic and pulmonary venous return (from ASD)
The entire mixture flows into the LV which is the sole pumping chamber for the pulmonary and systemic circulation.
(no communication btw RA and RV)
fairly cyanotic, need PGE
dependent on LV being sole pumping mech for lungs and system, nothing to be done sx, LV pumping ability determines survivability


Ebstein's Anomaly

The tricuspid valve is downwardly displaced and adherent to the interventricular septum
Cyanosis and CHF during the first few days of life
Improvement in oxygenation with reduction of pulmonary vascular resistance
*high incidence SVT*
*Cardiomegaly, balloon shaped heart*

ballooning of RA (and "atrialized" RV) RV contracts, tricuspid valve did not close-->inc. pressure/flow in RA, continues to flow, little blood goes to pulmonary circuit
RA blood-->ASD-->LA: mixed blood in system, improves in 1st few days of life with dec. in pulmonary resistance
-need single ventricle repair, RV will never have normal function: make VSD and LV will act as pump to system and lungs


HPI: Baby is a 4 day old infant that is brought in by parent to the ER you are working
Mom states
‘baby was born last week and went home after 2 days in the hospital’
‘Baby has been breathing fast and is not eating well’

While all of these are possible, what is first on your differential?
Metabolic Disease
Congenital Heart Disease

but don't forget about Congenital Heart Disease (5 out of 100, 95 will be septic)


Case: Born at full term. Normal prenatal US.
Didn’t awake for 6 am feeding, and had slept for 8 hours through the night with no wet diaper.
At 4pm parents noted his breathing to be labored and quick.
He was taken to the ER

Physical Exam
*36.3 (hypothermic! more worrisome than hyperthermic)*, 170, 80, 64/45, *O2 sat undetectable*
Not dysmorphic, Dusky with poor tone, Coarse breath sounds, no murmur , No liver edge palpable (not bad CHF), Abdomen firm and full, without bowel sounds, Extremities cool with thready pulses and delayed capillary refill (5-6 sec)

What is your next step in managing this baby?
B. Start oxygen
C. Obtain labs and chest x-ray
D. Start IV fluids

B. Start oxygen

Labs obtained on 100% Oxygen
Glucose less than 10 (normal 40)

pH: 6.73 (normal 7.4)
paCO2: 25 (normal 40)
paO2: 20 (normal 60)
bicarb: 6 (normal 24)
*base deficit: -25 (normal 0)*
(lack of bicarb is causing metabolic acidosis)
paCO2 is low due to hyperventilating, not enough to compensate

CBC: HCT: 51, WBC: 26.1 (not worried about infection)
75% segs 5% bands 20% lymphs

*Lactate 24.5 (normal less than 2)*
Ammonia 545 (normal less than 20)
Creatinine 2.2
Blood culture sent


2: What is your next step in managing this baby?
B. Start Amp and Gent after IV access
C. Give Dextrose bolus after IV access
D. Intubate baby and secure airway

D. Intubate baby and secure airway (with O2)

then C. Give Dextrose bolus after IV access (to avoid brain cell death)


3: What is your next step in managing this baby?
B. Start Amp and Gent
C. Give Dextrose bolus
D. Start Prostaglanding(PGE) infusion

all except E.
feasibility issue, don't take down to radiology, close to coding



ABC’s first:
*Oxygen* and intubation
Dextrose regulation

then Evaluation
Arterial blood gas
Blood culture
Chest x-ray

abx and PGE
Volume resuscitation


4: What is your next step in managing this baby?
B. Start Amp and Gent
C. Start Prostaglanding(PGE) infusion
E. All of the above
F. A, B, C

F. A, B, C

bicarb might temporarily help, but need to solve problem


Why are we starting PGE ?

to keep PDA open or reopen it


Case: Echocardiogram revealed ??

Hypoplastic Left Heart (HLHS) with a closing PDA

small/nonexistant LV, everything after will be compromised (coronaries!)

PDA will fill the coronaries retrograde


Case: Management

After baby is stabilized from a cardiovascular perspective, the evaluation of the cause can be more complete
-Antibiotics and PGE must be started because they can be life saving
-A baby presenting 2-10 days after birth in shock PGE should be started in addition to antibiotics


Obstructive Lesions: Ductal Dependent

Critical PS/AS
Critical CoA/IAA
Hypoplastic Left Heart

Presents in CV shock at 2-3 days of age when PDA closes
Generally cyanotic
Needs PGE1

Without a PDA there is no blood flow to the abdomen and lower extremities.

(Blue blood is better than no blood.)


Obstructive Lesions: Non-Ductal Dependent

Mild-moderate PS/AS
Mild-moderate CoA

Presents in older child w/ murmur, exercise intolerance, or HTN (in CoA)
Not cyanotic, may not need sx


Hypoplastic Left Heart Syndrome

A ductal-dependent lesion
One ventricle pumps both PBF and SBF
Difficult to balance PBF and SBF

oxygenated blood goes from (tiny) LV across ASD to RA-->RV-->pulmonary artery-->lungs or PDA
if PDA-->system

if kiddo sat at 100% but BP 60/30 ??
too much blood going to lungs, not enough to system

what to do? inc. pulmonary resistance: inc. CO2, dec O2, sedate baby
blend in N2 to vasoconstrictor lungs
more to make more acidotic

if 70/35 and not statting well..enough to system, not enough to lungs,
give pulmonary vasodilator to improve sat

more invasive tx: heart transplant


palliative procedure for HLHS: Norwood Procedure (7-10 days of life)

rebuilding aorta
What is the purpose of the Blalock-Taussig shunt? pulmonary artery to subclavian (pulm. artery was cut off)
Is there a murmur? yes, lots of turbulent BF across shunt
Mortality rate 12%

if shunt clots off, cath emergency, goes to OR


next procedure after Norwood: HLHS: Bidirectional Glenn (4-5 months of life)

pulmonary BF from SVC
wouldn't do early due to high pulmonary, no arterial level pressure yet in neonate (SVC is passive flow vs. subclavian artery)
What is the purpose of the Glenn?
Is there a murmur? NO, less turbulence
Mortality rate 6%


last step HLHS: Fontan circuit

SVC and IVC into pulmonary artery
little pop off valve to release pressure (2)
done at 2-3 years
can't play contact sports, but do fine, mortality rate 6%


Our Patient

PGE was started and the PDA reopened allowing improved systemic perfusion
All electrolyte and acid-base abnormalities were corrected over several hours
The baby was managed on the ventilator and had the Norwood procedure on DOL 5