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Heart transplantation
What is called the final frontier in the surgical management of congenital heart disease
Philippines
In which country is pediatric heart transplantation currently not available
Two to three decades
For how many decades have more resource-rich countries been conducting pediatric heart transplantation
Pig hearts into baboons
What animal models are used in current breakthrough research on heart xenotransplantation
Lack of donors
What is the primary problem with heart transplantation that drives research into xenotransplantation
Expanding the donor pool
What is the current cutting-edge research focus to shorten the heart transplant list
Non-humans
Which group of donors is being considered to expand the donor pool for heart transplantation
Baby Fae
What is the name of the historic infant in the 1984 xenotransplant case
1984
In what year did the historic infant xenotransplant case of Baby Fae occur
Loma Linda, CA, USA
In which location did the historic infant xenotransplant case of Baby Fae take place
Baboon heart
What animal organ was used in the first pediatric heart transplant involving Baby Fae
Hypoplastic left heart syndrome
With what end-stage condition was Baby Fae born
Transplantation
What was the only therapeutic resort for infants born with hypoplastic left heart syndrome in 1984
One to two months
How long did Baby Fae survive after her xenotransplant surgery
Acute rejection
What immunological complication caused Baby Fae to not survive beyond one to two months
Traditional classification
Which classification gives an idea of the pathophysiology of a congenital heart lesion by placing it as cyanotic or non-cyanotic
Non-cyanotic heart disease
Under which traditional classification is a ventricular septal defect categorized
Cyanotic heart disease
Under which traditional classification is tetralogy of fallot categorized
Right-to-left shunting of deoxygenated blood
What mechanical flow characterizes cyanotic congenital heart disease
Deoxygenated blood
What type of blood is shunted into systemic circulation in cyanotic congenital heart disease
Pulmonary congestion
What is the primary mechanism of heart failure in non-cyanotic congenital heart disease
Operative classification and analysis
Which practical classification scheme leads surgeons to analyze congenital heart lesions based on shunts and obstructions
Determine the level of the shunt
What is the first analysis step in evaluating shunt lesions under the operative classification
Shunt
What is a hole or communication between the systemic left side and the pulmonary right side circulation called
Left side
Which side of the heart represents systemic circulation in a shunt lesion
Right side
Which side of the heart represents pulmonary circulation in a shunt lesion
Atrial, ventricular, and post-aortic valve
What are the three potential levels of communication for shunt lesions
Post-aortic defect
What level of shunt is represented by a patent ductus arteriosus
Severity and natural history
What clinical insights are gained by determining the level of a shunt lesion
Obstructive lesions
Which lesions are evaluated by determining whether they obstruct the left or right ventricular outflow
Interactions between the shunt lesion and the obstructive lesion
What must be determined in operative analysis to predict pulmonary and systemic blood flow changes
Reduced or increased pulmonary blood flow, or reduced systemic blood flow
What three flow abnormalities can result from interactions between shunts and obstructions
Shunt
What cardiovascular abnormality connects one chamber or circulatory system to another
Obstructions
What cardiovascular abnormality blocks outflow from one system to the other
Heart failure or circulatory collapse
What are the two major systemic outcomes of interactions between shunts and obstructions
Cyanosis
What state is defined by the cardiac output coming from the heart being unoxygenated
Blue blood or blue babies
What is the clinical manifestation of unoxygenated cardiac output entering systemic circulation
Heart failure
What chronic clinical event is characterized by reduced cardiac output and congested lungs
Congested lungs
What is the primary clinical manifestation of heart failure due to damming of blood back to the lungs
Circulatory collapse
What acute event is defined by zero cardiac output and represents a true surgical emergency
Alleviate the lesion's effects on the heart
What is the primary goal of surgery during an acute circulatory collapse
18 years old
What is the age of the female patient presenting with palpitations and occasional shortness of breath in Case 1
Palpitations and occasional shortness of breath
What are the clinical presentations of the 18-year-old female patient in Case 1
Clear breath sounds with no signs of pulmonary congestion
What were the lung exam findings in the 18-year-old female patient in Case 1
Systolic murmur on the left upper parasternal area
What was the key physical exam finding on the chest of the patient in Case 1
Pulmonary valve
Which valve's anatomical location corresponds to the left upper parasternal systolic murmur heard in Case 1
No left ventricular enlargement
What did the chest X-ray show regarding left ventricular size in the patient in Case 1
Apex
Which part of the cardiac silhouette on chest X-ray represents the left ventricle
Displaced to the left side
Where is the cardiac apex displaced if there is left ventricular enlargement
No cardiac waist
What is the term for a chest X-ray finding where the normal depression in the area of the pulmonary artery and aorta is lost
Depression in the area of the pulmonary artery and aorta
What anatomical feature defines the normal cardiac waist on a chest X-ray
Bump on the pulmonary artery
What did the chest X-ray demonstrate in Case 1 to indicate an abnormal cardiac waist
Normal right atrium and ventricle
What did the blue arrow on the chest X-ray in Case 1 indicate about the right heart chambers
Right ventricular enlargement
What does a bulging right heart contour on chest X-ray indicate
Atrial septal defect
What does the red box highlight on the 2D echo in Case 1
Under the breastbone
Where is the right ventricle located relative to the chest wall during a 2D echocardiogram
Right atrium
Which right-sided chamber drains directly into the right ventricle
Posteriorly
Where are the left chambers of the heart located anatomically relative to the right chambers
Roughly 2 cm
What was the size of the atrial septal defect in Case 1
Noticeably enlarged
What was the state of the right-sided chambers on the echocardiogram in Case 1
Left ventricle
In a normal heart, which ventricle is larger than the other
Right ventricle cavity and contour larger than left ventricle
What was the abnormal ratio of ventricular sizes seen on 2D echo in Case 1
Shunt connecting LA to RA
Why does right-sided cardiomegaly occur in an atrial septal defect
Atrial septal defect
What is the term for a communication between the right and left atria
LA to RA
In what direction does blood shunt through an atrial septal defect
Pressure gradient
What physical force drives blood from the left atrium to the right atrium in an ASD
Left atrium
Which atrium has the higher pressure in a simple atrial septal defect
10-15 mmHg
What is the normal pressure of the left atrium
5-8 mmHg
What is the normal pressure of the right atrium
Central venous pressure or CVP
What is another clinical term for the normal right atrial pressure of 5-8 mmHg
Oxygenated blood
What type of blood moves from the left atrium to the right atrium through an ASD
Right-sided heart enlargement
What anatomical change is caused by increased blood volume on the right side of the heart in an ASD
Flooding them with already oxygenated blood
What is the consequence of increased blood flow from the right ventricle into the lungs in an ASD
5 years old
What is the age of the boy presenting in the VSD case
Short stature, poor weight gain, and shortness of breath on exertion
What are the clinical presentations of the 5-year-old boy in the VSD case
Clear breath sounds
What were the pulmonary physical exam findings in the 5-year-old VSD patient
Left upper parasternal area
Where was the systolic murmur heard in the 5-year-old VSD case
Displaced towards the left
Where was the cardiac apex positioned on the chest X-ray of the VSD patient
Grossly enlarged
What was the overall size of the heart compared to the thoracic cavity in the VSD patient
More than 50 percent
What was the width ratio of the heart compared to the thoracic cavity in the VSD patient
Left ventricular enlargement
What does a displaced apex towards the left suggest on chest X-ray in a VSD patient
Exaggerated pulmonary artery
What did the red arrow highlight on the chest X-ray of the VSD patient
Smaller
How does the cardiac waist appear when the pulmonary artery knob is enlarged on chest X-ray
Not much bigger than normal
What was the size of the right side of the heart on the chest X-ray of the VSD patient
Communication between the right and left ventricles
What does the yellow box show on the 2D echo in the VSD case
Left ventricle is dilated
What is the ventricular finding on 2D echo in a patient with a VSD
Back to the lungs and into the left side
Where does the shunted oxygenated blood find its way after passing through the VSD into the right side
Goes in circles
How does the shunted blood flow repeatedly in a patient with a VSD shunt
Volume-loading the left side
What hemodynamic change strains the left heart and ultimately causes heart failure in VSD
Small fraction
What fraction of blood actually goes out into the systemic circulation in a large VSD
Double-time
How hard must the heart work in a patient with a VSD shunt
Degree of pulmonary congestion
What is significantly different when comparing ASD versus VSD shunt lesions
VSD
Between ASD and VSD, in which condition does pulmonary congestion present earlier
VSD
Between ASD and VSD, in which condition is there more pulmonary congestion
VSD
Between ASD and VSD, where is the severity of heart failure increased
High systemic pressure of 120 mmHg
Why is there more heart failure in VSD compared to ASD
Systemic pressure
What type of pressure is pumped through the VSD and into the lungs, causing early damage
Hypertensive
What do the lungs become earlier in VSD compared to ASD due to the high-pressure shunt
Pressure and volume overload
What dual hemodynamic overload does VSD cause to the lungs
Size of the defects
Upon what does the degree of lung congestion depend in shunt lesions