Congenital Heart Disease Exam Solution Manual (Already Passed)
What is pulmonary stenosis? - Answers Pulmonary stenosis (PS) is an obstruction at the level of the
pulmonary valve or RV outflow tract.
What are the consequences of increased resistance to RV outflow in pulmonary stenosis? - Answers
Increased resistance to RV outflow leads to elevated RV pressure, reduced pulmonary blood flow, and
potential RV hypertrophy (RVH).
What can occur in severe cases of pulmonary stenosis? - Answers Severe cases of pulmonary stenosis
can lead to eventual right-sided heart failure.
What are some congenital causes of pulmonary stenosis? - Answers Congenital causes of pulmonary
stenosis are frequently associated with Noonan syndrome, rubella syndrome, and other cardiac
anomalies.
What are some acquired causes of pulmonary stenosis? - Answers Acquired causes of pulmonary
stenosis include homograft stenosis, conduit degeneration, or degeneration of bioprosthetic pulmonary
valves.
What are dysplastic pulmonary valves often associated with? - Answers Dysplastic pulmonary valves are
often seen in syndromic presentations like Noonan syndrome, which is characterized by short stature,
webbed neck, and other systemic features.
What are the clinical manifestations of mild Pulmonary Stenosis (PS)? - Answers Often asymptomatic.
What are the clinical manifestations of moderate-to-severe Pulmonary Stenosis (PS)? - Answers Dyspnea
on exertion, fatigue, cyanosis in critical cases if shunting occurs, and signs of right-sided heart failure
including peripheral edema and ascites.
What type of murmur is associated with Pulmonary Stenosis (PS)? - Answers High-pitched systolic
ejection murmur best heard at the second left intercostal space.
What are the characteristics of the murmur in Pulmonary Stenosis (PS)? - Answers Accompanied by a
delayed, soft, or absent P2 and an ejection click that diminishes with inspiration.
What is the gold standard diagnostic finding for Pulmonary Stenosis (PS)? - Answers
Echocardiography/Doppler.
What peak gradients suggest intervention in Pulmonary Stenosis (PS)? - Answers Peak gradients >64
mmHg or mean gradients >35 mmHg.
What does an ECG show in Pulmonary Stenosis (PS)? - Answers Right-axis deviation, RVH, and peaked P
waves indicative of right atrial overload.
, What does a chest X-ray (CXR) show in Pulmonary Stenosis (PS)? - Answers Prominent RV and
pulmonary arteries with potential post-stenotic dilatation of the pulmonary artery.
What are the indications for treatment of Pulmonary Stenosis? - Answers Symptomatic patients and
evidence of RV dysfunction or high transvalvular gradients.
What is the first-line treatment for domed pulmonary valves in Pulmonary Stenosis? - Answers Balloon
valvuloplasty.
What treatment is required for dysplastic pulmonary valves or complex anomalies in Pulmonary
Stenosis? - Answers Surgical repair.
What is an option for conduit stenosis in Pulmonary Stenosis? - Answers Percutaneous pulmonary valve
replacement.
What are the four primary defects of Tetralogy of Fallot? - Answers Ventricular Septal Defect (VSD),
Right Ventricular Hypertrophy (RVH), Pulmonary Stenosis, Overriding Aorta.
What is the nature of the Ventricular Septal Defect (VSD) in Tetralogy of Fallot? - Answers It is large and
non-restrictive.
What causes Right Ventricular Hypertrophy (RVH) in Tetralogy of Fallot? - Answers Increased right
ventricular pressure.
Where does Pulmonary Stenosis occur in Tetralogy of Fallot? - Answers At the infundibulum or valve
level, obstructing right ventricular outflow.
What is the significance of the Overriding Aorta in Tetralogy of Fallot? - Answers It is displaced over the
VSD, allowing mixing of oxygenated and deoxygenated blood.
What type of shunt is caused by the defects in Tetralogy of Fallot? - Answers A right-to-left shunt.
What are the consequences of the right-to-left shunt in Tetralogy of Fallot? - Answers Systemic
hypoxemia and cyanosis.
What genetic factor is often linked to Tetralogy of Fallot? - Answers 22q11.2 deletion (DiGeorge
syndrome).
What embryologic issue is associated with Tetralogy of Fallot? - Answers Abnormal development of the
conotruncal region of the heart.
What are the clinical manifestations of Tetralogy of Fallot? - Answers Cyanosis and clubbing, with
cyanotic spells during stress, feeding, or crying.
What are 'Tet spells' in Tetralogy of Fallot? - Answers Sudden hypoxia episodes relieved by squatting.
What is pulmonary stenosis? - Answers Pulmonary stenosis (PS) is an obstruction at the level of the
pulmonary valve or RV outflow tract.
What are the consequences of increased resistance to RV outflow in pulmonary stenosis? - Answers
Increased resistance to RV outflow leads to elevated RV pressure, reduced pulmonary blood flow, and
potential RV hypertrophy (RVH).
What can occur in severe cases of pulmonary stenosis? - Answers Severe cases of pulmonary stenosis
can lead to eventual right-sided heart failure.
What are some congenital causes of pulmonary stenosis? - Answers Congenital causes of pulmonary
stenosis are frequently associated with Noonan syndrome, rubella syndrome, and other cardiac
anomalies.
What are some acquired causes of pulmonary stenosis? - Answers Acquired causes of pulmonary
stenosis include homograft stenosis, conduit degeneration, or degeneration of bioprosthetic pulmonary
valves.
What are dysplastic pulmonary valves often associated with? - Answers Dysplastic pulmonary valves are
often seen in syndromic presentations like Noonan syndrome, which is characterized by short stature,
webbed neck, and other systemic features.
What are the clinical manifestations of mild Pulmonary Stenosis (PS)? - Answers Often asymptomatic.
What are the clinical manifestations of moderate-to-severe Pulmonary Stenosis (PS)? - Answers Dyspnea
on exertion, fatigue, cyanosis in critical cases if shunting occurs, and signs of right-sided heart failure
including peripheral edema and ascites.
What type of murmur is associated with Pulmonary Stenosis (PS)? - Answers High-pitched systolic
ejection murmur best heard at the second left intercostal space.
What are the characteristics of the murmur in Pulmonary Stenosis (PS)? - Answers Accompanied by a
delayed, soft, or absent P2 and an ejection click that diminishes with inspiration.
What is the gold standard diagnostic finding for Pulmonary Stenosis (PS)? - Answers
Echocardiography/Doppler.
What peak gradients suggest intervention in Pulmonary Stenosis (PS)? - Answers Peak gradients >64
mmHg or mean gradients >35 mmHg.
What does an ECG show in Pulmonary Stenosis (PS)? - Answers Right-axis deviation, RVH, and peaked P
waves indicative of right atrial overload.
, What does a chest X-ray (CXR) show in Pulmonary Stenosis (PS)? - Answers Prominent RV and
pulmonary arteries with potential post-stenotic dilatation of the pulmonary artery.
What are the indications for treatment of Pulmonary Stenosis? - Answers Symptomatic patients and
evidence of RV dysfunction or high transvalvular gradients.
What is the first-line treatment for domed pulmonary valves in Pulmonary Stenosis? - Answers Balloon
valvuloplasty.
What treatment is required for dysplastic pulmonary valves or complex anomalies in Pulmonary
Stenosis? - Answers Surgical repair.
What is an option for conduit stenosis in Pulmonary Stenosis? - Answers Percutaneous pulmonary valve
replacement.
What are the four primary defects of Tetralogy of Fallot? - Answers Ventricular Septal Defect (VSD),
Right Ventricular Hypertrophy (RVH), Pulmonary Stenosis, Overriding Aorta.
What is the nature of the Ventricular Septal Defect (VSD) in Tetralogy of Fallot? - Answers It is large and
non-restrictive.
What causes Right Ventricular Hypertrophy (RVH) in Tetralogy of Fallot? - Answers Increased right
ventricular pressure.
Where does Pulmonary Stenosis occur in Tetralogy of Fallot? - Answers At the infundibulum or valve
level, obstructing right ventricular outflow.
What is the significance of the Overriding Aorta in Tetralogy of Fallot? - Answers It is displaced over the
VSD, allowing mixing of oxygenated and deoxygenated blood.
What type of shunt is caused by the defects in Tetralogy of Fallot? - Answers A right-to-left shunt.
What are the consequences of the right-to-left shunt in Tetralogy of Fallot? - Answers Systemic
hypoxemia and cyanosis.
What genetic factor is often linked to Tetralogy of Fallot? - Answers 22q11.2 deletion (DiGeorge
syndrome).
What embryologic issue is associated with Tetralogy of Fallot? - Answers Abnormal development of the
conotruncal region of the heart.
What are the clinical manifestations of Tetralogy of Fallot? - Answers Cyanosis and clubbing, with
cyanotic spells during stress, feeding, or crying.
What are 'Tet spells' in Tetralogy of Fallot? - Answers Sudden hypoxia episodes relieved by squatting.
