peds cardiology2 Flashcards
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»_space;»> 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? Sepsis Trauma Metabolic Disease Anemia Congenital Heart Disease
*Sepsis
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
PE:
Cyanotic
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? A. STAT ECHO B. Start oxygen C. Obtain labs and chest x-ray D. Start IV fluids E. STAT Head CT
B. Start oxygen
Labs obtained on 100% Oxygen
Glucose less than 10 (normal 40)
ABG: 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? A. STAT ECHO B. Start Amp and Gent after IV access C. Give Dextrose bolus after IV access D. Intubate baby and secure airway E. STAT Head CT
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? A. STAT ECHO B. Start Amp and Gent C. Give Dextrose bolus D. Start Prostaglanding(PGE) infusion E. STAT Head CT
all except E.
feasibility issue, don’t take down to radiology, close to coding
Management
ABC’s first:
Oxygen and intubation
Dextrose regulation
then Evaluation Arterial blood gas CBC, CMP Glucose Blood culture Chest x-ray ECHO
abx and PGE
Volume resuscitation
4: What is your next step in managing this baby? A. STAT ECHO B. Start Amp and Gent C. Start Prostaglanding(PGE) infusion D. STAT Head CT 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