Acyanotic Congenital Heart Disease Flashcards
Congenitial heart disease occurs in how many births per 1000?
- 8/1000
What are the three pathophysiological groups that congenital heart disease can fall into?
1) Left-Right shunt
2) Right-Left shunt
3) Obstructive, stenotic lesions
Which pathophysiological groups can cause acyanotic heart disease?
1) Left-Right shunt (increased pulmonary blood flow)
- PDA
- VSD
- ASD
2) Obstructive lesions
- Aortic stenosis
- Pulmonary stenosis
- Coarctation of aorta
What is the most common congenital heart disease?
- Ventricular septal defect
- Accounts for 25% of all
Large VSDs are typically asymptomatic at birth. Explain why. When will this be picked up?
Large VSDs:
- Less resistance to flow so murmur may be absent
- Pulmonary vascular resistant is high at birth resulting in less of a left to right shunt
- First 6-8 weeks of life this pulmonary vascular resistance decreases and shunting increases
How would you describe the murmur associated with a small VSD?
- Loud pansystolic murmur
- usually loudest over L sternal border
- May have Thrill
How would you describe the murmur associated with a large VSD?
- Increased flow across the mitral valve
- Mid-diastolic murmur at the apex
What investigations can be done to look for VSD?
1) ECG
- Findings of L atrial and ventricular enlargement
2) CXR
- Cardiomegaly
- Enlarged L ventricle
- Pulmonary HTN may lead to R heart enlargement
How are VSDs managed?
- 1/3 will close spontaneously (small more often)
2) Surgical closure
3) Initial tx for mod-sev VSD includes: - Diuretics +/- digoxin
- Afterload reduction
What is the name of the most common type of atrial septal defect? Where is the defect?
- Secundum defect
- Defect in region of the foramen ovale
What clinical findings are associated with a VSD?
- Small: Assymptomatic
- Mod-severe: Pulmonary overcirculation, heart failure
What are the clinical manifestations of an atrial septal defect?
- Rarely symptomatic
What may you see on ECG and CXR with Atrial septal defect?
1) ECG
- Right axis deviation
- Right ventricular enlargement
2) CXR
- Cardiomegaly
- Right atrial enlargement
- Prominant pulmonary artery
What is the treatment for a ASD?
- Rarely indication
- If significant shunt still present around age 3 can be closed surgically or by cath lab
What is the ductus arteriosis?
- A blood vessel in the developing fetus that connects the pulmonary artery to the descending aorta
What is the function of the ductus arteriosus?
- Allows blood from the right ventricle to bypass the fetuses fluid filled lungs
What happens to the ductus arteriosus after birth?
- It becomes the ligamentum arteriosum
- Ductus arteriosus typically closes within a few days after birth
What happens if the ductus arteriosis remains patent?
- Left to Right shunting
- Higher pressure in aorta shunts blood (left to right) to the pulmonary artery resulting in higher pulmonary blood flow
What are the clinical manifestations of a PDA?
- Can be asymptomatic
- Larger shunts can produce symptoms of heart failure
- Resp distress
- poor feeding
- irritability
- poor growth
What physical exam findings might you see with a PDA?
- Widened pulse pressure (due to runoff of blood into pulmonary circulation during diastole)
- Continuous machine like murmur in L intraclavicular area (often heard well over L side of the back)
What might be seen on imaging studies in a patient with a PDA?
1) ECG
- Full pulmonary artery silhouette
- Increased pulmonary vascularity
2) CXR
- Normal or evidence of L ventricular hypertrophy (compensating for poor output)
3) Doppler Echo
- showing blood flow from Aorta to L pulmonary artery (L-R shunt)
What is the medical treatment for PDA?
- NSAID (indomethacin I.v)
- Cannot use with renal failure, necrotizing enterocolitis, or overt bleeding.
If NSAIDs fail or are contraindication what is your next step in managing a PDA?
- Surgical occlusion
- If PDA too small for occlusion, diuretics and ACE inhibitors can be used to treat congestive symptoms until it grows larger
What is an endocardial cushon defect?
- AKA: Atrioventricular canal defect
- Failure of the septum to fuse with endocardial cushion
What are the symptoms of an endocardial cushion defect?
- Symptoms of heart failure as pulmonary vascular resistance decreases
- poor feeding
- tachypnea
- poor growth
- irritability
- Pulmonary HTN from increased pulmonary circulation
Complete endocardial cushion defects are most commonly seen in children with this genetic condition?
- Trisomy 21
A complete endocardial cushion defect is typically diagnosed how?
- Echocardiogram
- Other things that may be seen:
- enlargement of all chambers of heart and increased vascularity on CXR
- L axis deviation, and combined ventricular enlargement on ECG
How is an endocardial cushion defect managed?
- Initial management with diuretics +/- digoxin
- Surgical repair is ultimately needed
What is pulmonary stenosis?
- Obstruction to blood flow from the right ventricle to the pulmonary artery
- The stenosis can be valvular, subvalvular, or supravalvular
What is valvular pulmonary stenosis?
- Stenosis in the pulmonary valve
- Can’t open wide enough limiting the flood flow from the right ventricle to the lungs
Due to:- Thick leaflets
- Narrow fusion of leaflets (leaves narrow hole in center)
What is subvalvular pulmonary stenosis?
- The muscle under the valve area is thickened which creates a smaller outflow tract from the right ventricle into the pulmonary artery
What is supravalvular pulmonary stenosis?
- Pulmonary artery, just above the pulmonary valve, is narrow
What are the clinical manifestations of pulmonary stenosis
1) Mild: Asymptomatic
2) Mod-Severe:
- Tachypnea
- Fatigue/Fainting
- Tachycardia
- Swelling in feet, face, eyelids, and/or abdomen
- cyanosis in end stages
True or False: Spontaneous closure of a PDA after a few weeks of age in a full term baby is common?
- False uncommon after a few weeks of age in full term baby
- High oxygen and decreasing levels of prostaglandins associated with fully developed lungs and distance from maternal prostaglandins should have closed it by a few weeks of age
What helps to keep the ductus arteriosus open? What can help close it?
1) Open: Prostaglandin, low O2 environment
2) Close: High O2 environment, removal of prostaglandins (NSAIDS - indomethacin)
What ECG and CXR findings may you expect in a patient with pulmonic stenosis?
- Mild: None
- CXR: No cardiomegaly but dilation of the main pulmonary artery may be seen
- ECG: Right axis deviation and right ventricular hypertrophy
What is the treatment for pulmonic stenosis?
- Note that pulmonic stenosis typically is not progressive and does not worsen
- Balloon valvuloplasty is usually sucessful
- Otherwise surgical repair
What is aortic stenosis?
- Obstruction to blood flow from the left ventricle to the aorta
- The stenosis can be valvular, subvalvular, or supravalvular
describe the murmur associated with pulmonic stenosis?
- systolic ejection murmur
- Second left intercostal space
- Radiation to back
Describe the murmur associated with aortic stenosis?
- Systolic ejection murmur
- Second right intercostal space
- Radiation to neck
What are the clinical manifestations of aortic stenosis?
- Mild to moderate : Asymptomatic
- Severe: easy fatigue, exertional chest pain, syncope
- Symptoms of heart failure when critical
What can be seen on CXR, ECG and Echo with aortic stenosis?
CXR: LV hypertrophy, dilation of ascending aortic knob due to intrinstic aortopathy may be seen
ECG: LV hypertrophy
Echo: Site of stenosis, valve morphology, presence of LVH and can estimate pressure gradient
Is aortic stenosis typically a progressive disease?
- Yes, frequently progresses with growth and with age
How is aortic stenosis managed?
- Serial follow-up with echo
- Balloon valvuloplasty usually first interventional procedure for severe stenosis
- Surgical management is another option is previous methods insufficient
What is coarctation of the aorta? Where does it typically occur?
- A congenital condition where there is narrowing of the aorta
- Typically occurs in the area where the ductus arteriosus inserts
What other abnormalities are commonly seen in a patient with coarctation of the aorta?
- Hypoplastic (small) aortic arch
- Abnormal aortic valve
- VSDs
Is a patient with coarctation of the aorta affected by the patency of the ductus arteriosus? explain?
- Yes (Sort of)
Depends on two things:
1) Degree of coarctation (how narrow is the vessel), the more severe the more likely that a patient DA is needed
2) Location of coarctation, if coarctation is preductal (above the DA) then a patient DA allows additional blood to perfuse the lower extremities
20% of patients with a 45 X,0 karyotype will have this congenital cardiac malformation? Also what is the genetic condition?
- Coarctation of the aorta
- Turner syndrome
True or False: Coarctation of the aorta occurs equally in male and female patients
- False
- 2:1 male to female ratio
What are the clinical manifestations of coarctation of the aorta?
- Femoral pulses weaker and delayed compared to right radial
- Blood pressure in lower extremities lower than upper
- Poor feeding
- Respiratory distress
- Older children presenting are typically asymptomatic, may have leg discomfort with exertion *
What is rib notching? What causes it and what is it associated with?
- Notching of the upper ribs seen on CXR
- Collateral intercostal arteries have increased blood volume causing them to erode the ribs
- Seen in coarctation of the aorta in children over 8
How would you manage an infant with coarctation of the aorta?
1) Prostaglandin E1 (keep ductus arteriosus open)
2) Inotropic medication or diuretics for BP control
3) Surgical repair ultimately needed
Which ventrical is enlarged in an infant presenting with coartation of the aorta? What about in a young adult presenting with the same?
1) Infant: Right (right heart pumping in womb against narrow artery)
2) Adult: Left (left ventricle pumping against narrow artery)
