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111
Congenital Heart Disease
69
Medical
Professional
05/11/2012

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Term
Cardioplegia:
Definition
is the intentional and temporary cessation of the heart, to produce asystole .Cardioplegia is a speclized solution that is introduced into the coronary circulation. This is done to slow the metabolism of the heart and prevent damage to the LV and the RV.
Term
Levocardia:
Definition
Normal cardiac position; ant. Inferior and to the left
Term
Dextrocardia:
Definition
heart on the other side (not on left on right) apex still to the left
Term
Mesocardia:
Definition
Heart in the middle (portion or all of lung removed)
Term
Concordance:
Definition
normal alignment (LV w/ LA; RV w/ RA)
Term
Disconcordance:
Definition
abnormal alignment w/ great vessels & chambers
Term
Cardiac Situs:
Definition
Cardiac position
Term
Atrial Situs:
Definition
normal atrial position
Term
Situs Inversus:
Definition
all organs reversed
Term
Bovine:
Definition
Cow
Term
Porcine
Definition
Pig
Term
Cadaver
Definition
Dead human
Term
How long does a tissue valve replacement typically last?
Definition
<15 years
Term
How long does a mechanical valve replacement typically last?
Definition
~12-15 years
Term
What type of valve typically lasts longer, tissue or mechanical?
Definition
Mechanical typically lasts longer than tissue
Term
How long does it usually take fetal hemoglobin to convert to adult hemoglobin?
Definition
Usually up to 6 months
Term
How long does it usually take fetal blood saturation to convert to adult blood saturation?
Definition
2 hours to 24 hours to reach normal saturation levels
Term
What is a LVAD and what does it do?
Definition
Left Ventricular Assist Device; a kind of mechanical heart that is placed inside a person's chest to help the heart pump oxygen-rich blood throughout the body.
Term
What is the Impella device and what does it do?
Definition
A small rotary pump attached to the end of a catheter which is passed retrograde through the aortic valve into the left ventricle from which it aspirates blood which it delivers immediately above the aortic valve. Inpatient use only.
Term
Name 4 types of Atrial Septal Defects; which one is most common?
Definition
1. Ostium Secundum: the most common type of ASD (accounting for 50-70% of all cases). The hole is located near the center of the atrial septum.

2. Sinus Venosus: the hole is located near one of the two places where the vena cava enters the right atrium. The two kinds of sinus venosus defect are distinguished by whether the hole is near the entry point of the SVC or of the IVC.

Ostium Primum: the hole is located in the lower part of the atrial septum, near the tricuspid valve

4. Common Atrium: absence of interatrial septum
Term
What is an ASD?
Definition
Atrial Septum Defect: a hole in the atrial septum, that separates the right and left atria. Because of the lower pressure in the right atrium, this hole typically allows oxygenated blood from the lungs to shunt from the left into the right atrium. This blood proceeds into the right ventricle, which pumps it back to the lungs rather than to the body. ASDs vary in size and in the severity of symptoms they may cause. They account for between 5%-10% of all cases of congenital heart disease and are twice as prevalent among girls as boys.
Term
What is the surgical correction for ASDs?
Definition
Transcatheter septal occluder or surgically sealed with pericardium patach or gortex
Term
Which ASD is an Endocardial Cushion Defect?
Definition
Ostium Primum; EC did not meet the Septum Primum during fetal development. They did not arch and/or fuse properly. This type of defect can effect the AV valves (cleft valve).
Term
How are ASDs graded?
Definition
Mild, moderate, severe

*mild grade is insignificant hemodynamically
Term
What is the surgical correction for a Common Atrium?
Definition
Gortex patch or multiple graphs
Term
How would an ASD effect the heart? What chambers are impacted?
Definition
Physical findings vary due to age, weight, and medical history of the baby. ASDs can go undetected for years; however, can lead to symptoms such as, feeding difficulties, fatigue, SOB, sleepy, noticable change in activity, right side arhythmias, systolic murmurs, pulmonary congestion, Eisenmenger's.
Term
What is Eisenmenger's PYSIOLOGY?
Definition
Hemodynamic significant VSD; volume overload then pressure overload, the R heart will enlarge. ↑ volume ↑ pressure→pulmonary hypertension
Term
Name 3 types of Ventricular Septal Defects
Definition
Supercristal: lies just below the Pulmonary valve in the infundibular septum

Perimembraneous:occur beneath the aortic valve on the left side of the septum, extending to the right under the septal leaflet of the tricuspid valve

Muscular: divided into separate distinct regional groups, including midmuscular, apical, anterior, and posterior. Gives a "Swiss cheese" look.
Term
What is absent with a common ventricle?
Definition
IVS
Term
What is the grading criteria for a VSD?
Definition
Insignificant, small, moderate, large
Term
What is a Gerbode VSD?
Definition
A ventricular septal defect communicating directly between the left ventricle and right atrium.
Term
What are possibel clinical finding for a Gerbode VSD?
Definition
Depending on age and the underlying risks, there maybe AS with a murmer at 2nd or 3rd LICS, AV valve regurge, early stages of RHF such as distended neck veins, SOB (may need to be inubated), increased HR, decreased O2 Sats, enlarged RH and/or dilated PA in x-ray
Term
With an Endocardial Cushion Defect, what are two ways that this defect can be termed?
Definition
Compete or partial AV Canal Defect
Term
What are some clinical findings for Endocardinal Cushion Defects?
Definition
Failure of EC to form IAS septum; usually combined with other defects such as valve defects. Partial involves usually one valve. Complete will envolve 2 or more valves.
Term
What are some associated defects with a Partial ECD?
Definition
Primum ASD,cleft MV, EC partially fused resulting in the division of the AV Canal, R/L ostium, volume overload to RH
Term
What are some associated defects with a Complete ECD?
Definition
Involves all 4 valves and chambers, large membranous VSD and large primum ASD, MV and TV can be merged together so there is one valve, shunting at both levels, usually the AV is incompetent, can be AI, LVOT slightly narrowed
Term
What is "Failure to thrive"?
Definition
Means that the way things are, there is not chance for survival
Term
State some possible critical findings for ECDs.
Definition
Failure to thrive, fatigue, dyspnea, PHTN→↑PRV, large cardiac silhouette, abnormal EKG, L-axis deviation, signs of CHF
Term
Why could there be a split S2 with ECDs?
Definition
Volume overload in RH → delay P2
Term
What would produce a thrill with ECD?
Definition
Jets of blood
Term
What is the survival rate if ECD is not repaired within 1 year?
Definition
20%, mortality rate at 80%
Term
What are some surgical processes for ECDs?
Definition
Close ASD & VSD, narrow flow rate with pulmonary band, reconstruct MV/TV (reset, suture, anular ring), repair any additional defects
Term
What is TV Atresia and what are some anomalies that might come with it?
Definition
A cyanotic lesion, with complete failure of the TV to form; there is only thickened annulus/membrane. The RV is small and underdeveloped. The PA and Ao may be transposed or normal. This is usually an ASD, VSD, PDA which the baby needs.
Term
How is TV Atresia initially treated?
Definition
Surgical treatment is first palliative (relieving pain or alleviating a problem without dealing with the underlying cause), blood flow to the lungs needs to be established or increase. ProstagladinE' is administered to help keep PDA open. The baby then is taken to Cath Lab for a balloon septostomy.
Term
What are the 3 surgeries used to treat TV Atresia/Hypoplastic Left Heart Syndrome? Describe them in detail and when the surgies typically take pace.
Definition
1. Stage I: Within 4-7 weeks, the first procedure is the Norwood Procedure uning a Blalock-Tausigg shunt; an ASD/VSD is created and the L. Subclavian Artery is divided and connected to the L. PA allowing blood to flow into the lungs to pick up O2. An alternative is to use the Modified Blalock-Tausigg utilizing a conduit between the PA and a branch off the Ao, typically the Inominate Artery.

2. Stage II: Typically 4-6 months, the next procedure is the Glen Shunt Procedure where the SVC and the RPA are surgically connected allowing venous return to flow passively into the RPA/LPA. This allows a bridge for the 3rd sugical procedure...

3. Stage III: Within 18 months to 3 years, the Fontane Procedure completes the process. A graft re-routes blood flow from the SVC to bypass the RA directly to the PA, then a baffle reroutes IVC blood flow directly to the PA or the IVC is connected to the RPA by a tunnel-like patch to the PA. A PA band maybe used to slow flow. ASD/VSD are closed.
Term
What is Ebstein's anomaly
Definition
A rare cyanotic lesion defect involving an abnormality in the TV, which connects the RA with the RV. The valve forms abnormally and is displaced inferiorly toward the apex causing the RV to become atrialized nd the RVOT becomes the main pumping chamber. The leaflets of the TV are usually thickened and incompetant which leads to PHTN→PVR.
Term
What are some associated lesions and clinical presentations you might find with Ebsteins Anomaly?
Definition
ASD/VSD tritology of flow, coarctation of the Ao, PV atrisia, signs and symptoms of CHF(SOB, ↑HR, ↓activity, PHTN), murmur (TVI, MVI), dyspnea, feeding difficulties, arrhythmias (SVT, WPW)
Term
What is the surgical procedure to repair Ebstein's Anomaly?
Definition
Repair or replace the TV with a tissue valve, close ASD & PDA, correct any associated CHDs with PA band, diuretics to control PHTN, and possible ventilator to ↑ SaO2
Term
What is Tretalolgy of Fallot (TET) and the 4 characteristic?
Definition
A most common cyanotic lesion caused by the a maldevelopment of the conus septum→division of septum moved too far anterior

1) A large VSD (L→R predominately but can be bi-directional)

2) An infundibular stenosis RVOT into the pulmonary artery and/or pulmonary valve narrowing. (PA atresia most severe).

3) The aorta is enlarged and "overrides," or sits directly above, the VSD. (malalignment of Ao/PA)

4) RVH because of volume overload and PHTN
Term
What are some common symptoms of Tetralogy of Fallot?
Definition
1. cyanosis

2. clubbing

3. squatting
Term
What is the most severe form or Tetralogy of Fallot?
Definition
Pulmonary Atresia
Term
What are some possible associated congenital lesions in Tetralogy of Fallot?
Definition
Bicuspid PV, right-sided Ao Arch, coronary anomalies, right to left shunt
Term
What is the surgical repair for Tetralogy of Fallot and what are some surgical complications?
Definition
1. Blalock-Tausigg removed if pallative measures were taken, ASD/VSD closed, PDA closed, in PS the RVOT can be surgically reconstructed

2. Infection, arrythmias, CHF, subacute bacterial endocarditis, pacemaker, death
Term
Mortality:
Definition
# of deaths from disease
Term
Morbidity:
Definition
rate of disease
Term
What is Eisenmenger's COMPLEX?
Definition
Characterized by: large VSD, over-riding Ao, no RVOT obstruction, PA can be dilated, TV thickened and stenotic, ↑PVR, Eisenmenger's Physiology if blood flow reverses
Term
What are some physical signs and symptoms associated with Bicuspid Ao?
Definition
2 cusps of unequal size or 3 cusps with raphe (fused) between two cusps, valve maybe stenotic with varying degrees of AI, coarchtation of the descending Ao, Ao root diated, sbe prophylaxis (treat with anitbiotics), CHF or LV failure, murmur, AS or AI, SOB, PHTN, Ventricular arrythmias
Term
What is the ratio males to females for Bicuspid Ao Valve?
Definition
2:1 males
Term
What are some options to surgically repair Bicuspid Ao?
Definition
1. Do nothing

2. Ao valvuplasty

3. Ao valve replacement

*may need pacemaker, antiarrhythmic agents
Term
What is coarctation of the Ao?
Definition
an abnormality of the tunica media of decending Ao (thickening) causing a stenosis of Ao→HTN→↑afterload
Term
What is the most popular prosthetic valve?
Definition
St. Jude mechanical
Term
What is Congenital AS?
Definition
Ao cusps are fused and not thickened (can become calcific later in life), can be well tolerated if there are no symptoms.
Term
What are some symptoms and physical findings of Congenital AS?
Definition
Evidence of PHTN, CHF/LV failure, SBE, fatigue, chest pain, arrthymias, dyspnea, murmur, wide mediastinum and large LV in x-ray silhouette, pulmonary congestion, repiratory infections
Term
What are some sugical options for Congenital AS?
Definition
valvuplasty, Ao valve replacement, Ross procedure (replace Ao with PV and leave PA open or install new PV)
*repair other lesions if present
Term
What is Hypoplastic Left Heart Syndrome and some of its characteristics?
Definition
The LV is severely underdeveloped; cyanotic lesion, RVE, RVH, hypoplastic LV maybe filled with clot, coronary circulation maybe insufficient, AoV hypoplastic, AI, MS or Coarctation. NOT COMPATIBLE WITH LIFE, NEED A VERY LARGE ASD!
Term
What type of medical care is required for Hypoplastic Left Heart Syndrome?
Definition
Keep PDA open for Coronary Arterty flow (retrograde), correct metabolic acidosis, manipulate PVR with medications, intubation, cardiac medication management, O2 suppliment
Term
What is Hypertrophic Cardiomyopathy (HOCM)and some characteristics?
Definition
a condition in which the heart muscle becomes thick causing a small LV cavity with a degree of LVOT obstruction. The thickening makes it harder for blood to leave the heart, forcing the heart to work harder to pump blood. (EF @ 75-80%). Venturi effect (the reduction in fluid pressure that results when a fluid flows through a constricted section of pipe/vessel.)→Systolic anterior motion of the MV or chordae (SAM). Diastolic failure due to hypertrophy. ↑Intracardiac pressures; ↑LVP reflected in LA back to RH
Term
What are the 2 classifications of HOCM?
Definition
Obstructive or non-obstructive
Term
What is ASH?
Definition
Asymetrical Hypertrophy (refers to muscular septum)
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