Practicum III (Peds) Test 2: Congenital Heart Disease 2

Question Answer
Hypoplastic Left Heart Syndrome (HLHS): What has happened anatomically? Marked hypoplasia or absence of the left ventricle and severe hypoplasia of the ascending aorta.
Hypoplastic Left Heart Syndrome (HLHS): Common accompanying disorders aortic valve atresia with either mitral atresia or stenosis (theres no opening into the left atrium – the only flow they have is the ductus arteriosus
Hypoplastic Left Heart Syndrome (HLHS): S/S infants develop cyanosis and tachypnea within 24-48 hours after birth
Hypoplastic Left Heart Syndrome (HLHS): Why would the patient develop cyanosis/tachypnea The ductus arteriosus is closing ( which is their primary flow of oxygenated blood )
Hypoplastic Left Heart Syndrome (HLHS): what happens if the ductus arteriosus closes metabolic acidosis, renal failure, shock, death
Hypoplastic Left Heart Syndrome (HLHS): How to keep the patient alive (supportive) give prostaglandins, keep SaO2 around 75-80% – to high of O2 will vasodilate the lungs and pull the blood away from the heart (where it needs to be), avoid hyperventillation/sedation, correct acidosis, inotrops, infusions as directed
Hypoplastic Left Heart Syndrome (HLHS): what can be done to correct the problem (vs just keeping them alive) surgery: cardiac transplantation, staged reconstruction (norwood, glenn, fontan), or hybrid procedure
Hypoplastic Left Heart Syndrome (HLHS): post-bypass considerations Maintain balance between pulmonary and systemic circulations, watch out for the Blaalock-Taussig shunt, prone to pulmonary overcirculation, myocardial ischemia, systemic hypoperfusion, renal failure, metabolic acidosis, CV collapse
Hypoplastic Left Heart Syndrome (HLHS): What are the concerns about the Blaalock-Taussig shunt? decreased coronary perfusion, decreased RV function, decreased end-organ perfusion
Second stage palliation for single ventricle defects Cavopulmonary(Glenn) Shunt
Cavopulmonary(Glenn) Shunt – what is it done for and what do they do? done to help Hypoplastic Left Heart Syndrome (HLHS) – They disconnect the superior vena cava from the right atrium and connect it to the pulmonary artery
Cavopulmonary(Glenn) Shunt: how does it work pulmonary blood flow is passive and driven by gradient between CVP and LA pressure
Obstructive lesions (5) Aortic stenosis, Mitral stenosis, Pulmonic stenosis, Coarctation of the aorta, Interrupted Aortic Arch
Obstructive lesions: monitoring all basic + the 2's: 2 extra leads (5 lead EKG), 2 pulse oximeter probes (pre and post ductal), 2 temperature site (nasopharyngeal and bladder), 2 invasive hemodynamic monitors (A-line and CVP)
Anesthesia machine should be able to deliver ______ AIR as well as O2 and NO2
What pharmaceuticals should you have labeled and ready to go? calcium chloride, sodium bicarb, atropine, pheylephrine, lidocaine, epinephrine
inotropic infusions good to have when they come off bypass surgery dopamine, milrinone, and epinephrine
What temperature interventions are important essential to be able to rapidly alter temp is critical: heating/cooling water mattress, bair hugger, efficient room cooling/heating system. Ice can (and often is) applied to the head during circulatory arrest
Used to decrease PVR nitric oxide
Nitric oxide: make sure your connecting it to the _____ of the circuit and not the _____ side inspiratory, expiratory
Where should the focus of the preoperative evaluation be? cardiopulmonary system
What is it that is a good indication of the childs cardiorespiratory reserve general health and ACTIVITY
Patients with cyanotic lesions often have ______ that result in _____ abnormally high hematocrits, significantly increased blood viscocity and coaguapathies
Anyone with congenital heart disease weather repaired, palliated or unrepaired should have _____ a cardiology evaluation (by a cardiologist) documented prior to any elective surgery
What kind of drugs would be contraindicated for these patients? Anything that would make the pt tachycardic
Inhaled anesthetics cause_______ in patients with R to L shunts peripheral vasodialation, increased shunting, and cyanosis
______ (drug) a good choice in children with R to L shunts Ketamine
______ decreases BP and SVR but may be preferable to ketamine for _____ Propofol, short procedures
Connect IV tubing to ______ while there is free flowing IV fluid or blood venous cannulas
There shoud be Meticulous _____ of IV tubing “debubbling”
syringe implications Eject small amount of fluid from the syringe to clear air from the syringe
patient has RSV – what to do? delay the sx 6 weeks (50% increase in morbidity with RSV)
Patient with congenital heart disease has a URI – what happens usually cancelled
End tidal CO2 ____ (under/overestimates) PaCO2 underestimates
The gradient between ETCO2 and PaCO2 is significantly greater in ______ (cyanotic/acyanotic) patients than in ______ (cyanotic/acyanotic) patients cyanotic, acyanotic
T/F -PaCO2 is not always stable in patients with cyanotic heart disease true
PaCO2 in CHD: The gradient increases by 2-3 torr for every_____ drop in SaO2 10%
The current practice of giving patients antibiotics prior to a dental procedure is no longer recommended EXCEPT for ______ patients with the highest risk of adverse outcomes resulting from IE (infective endocarditis)
T/F – The Committee recommends administering antibiotics to prevent IE in patients who undergo a GI or GU tract procedure False – The Committee no longer recommends administering antibiotics solely to prevent IE in patients who undergo a GI or GU tract procedure
Patients that WOULD receive prophylactic antibiotics prior to a dental procedure Patients with: prosthetic cardiac valve, previous infective endocarditis, CHD (unrepaired cyanotic, completely repaired with prosthesis for 6 months post-sx, repaired with residual defects on or around prosthesis), Cardiac transplantation w/valvulopathy

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