Pediatric Cardiology Flashcards
ECG changes indicative of hypertrophic cardiomyopathy include a tall […] wave in aVL and a deep […] wave in V3.
ECG changes indicative of hypertrophic cardiomyopathy include a tall R wave in aVL and a deep S wave in V3.
these changes represent LV hypertrophy
ECG in a newborn normally shows physiologic […] axis deviation.
ECG in a newborn normally shows physiologic right axis deviation.
i.e. mainly negative in lead I and positive in aVF
Patients with Tetralogy of Fallot may […] in response to cyanotic spells, which decreases right-to-left shunting by increasing systemic vascular resistance (SVR)
Patients with Tetralogy of Fallot may squat in response to cyanotic spells, which decreases right-to-left shunting by increasing systemic vascular resistance (SVR).
inhaled O2 may help stimulate pulmonary vasodilation and systemic vasoconstriction, which can further decrease right-to-left shunting
Patients with Tetralogy of Fallot often experience “[…] spells” which may be caused by crying, fever, and exercise due to exacerbation of RV outflow tract obstruction.
Patients with Tetralogy of Fallot often experience “tet spells” which may be caused by cry.
Tetralogy of Fallot is characterized by a […]-shaped heart on CXR.
Tetralogy of Fallot is characterized by a boot-shaped heart on CXR
boot shape due to RV hypertrophy
The degree of shunting in Tetralogy of Fallot is determined by the degree of […].
The degree of shunting in Tetralogy of Fallot is determined by the degree of right ventricular outflow tract obstruction (from pulmonary stenosis).
severe RVOT obstruction typically presents shortly after birth with cyanosis; moderate obstruction may present during childhood
Treatment for patients with prolonged QT intervals includes […] therapy and pacemaker placement.
Treatment for patients with prolonged QT intervals includes beta blocker therapy and pacemaker placement.
beta blockers limit extertional heart rate and shorten the QT interval; additional management should also include avoidance of electrolyte derangements, vigorous exercise, and medications that block K+ channels
Universal lipid screening is recommended for children ages […] - […] and again between ages […] - […].
Universal lipid screening is recommended for children ages 9 - 11 and again between ages 17 - 21.
lipid levels typically remain stable prior to and after puberty; future lipid screening should begin again at age 35 (men) or age 45 (women) in 5-year intervals
What congenital heart defect results from thickening of the tunica media in the aortic arch?
Coarctation of the aorta
What heart abnormalities (2) are associated with Turner syndrome?
What heart abnormalities (2) are associated with Turner syndrome?
bicuspid aortic valve (most common) and coarctation of the aorta
patients may also develop aortic root dilation
What is the likely diagnosis in a child that presents with fever and signs of heart failure after several days of a runny nose and nasal congestion?
Viral myocarditis
signs of heart failure include cardiomegaly, pulmonary edema, hepatomegaly, etc.
What is the likely diagnosis in a cyanotic newborn with truncus arteriosus and an absent thymic shadow on X-ray?
DiGeorge syndrome
DiGeorge syndrome is associated with conotruncal heart defects including truncus arteriosus, tetralogy of Fallot, and transposition of the great vessels
What is the likely diagnosis in a newborn that becomes cyanotic with left axis deviation on ECG and decreased pulmonary markings on CXR?
Tricuspid valve atresia
left axis deviation is seen as up-going R wave in lead I and down-going R wave in lead avF; lack of blood flow to the RV results in underdevelopment of the pulmonary valve and/or artery; ASD and VSD are necessary for survival
What is the likely diagnosis in a newborn that presents with cyanosis, a single loud S2 on auscultation (no murmur), and a narrow mediastinum on X-ray?
Transposition of the great vessels
the narrow mediastinum is described as an “egg on a string” appearance
What is the likely diagnosis in a newborn that presents with upper extremity hypertension and lower extremity hypoperfusion with a systolic interscapular murmur?
Coarctation of the aorta
often presents a couple days after birth when the ductus arteriosis closes
What is the likely diagnosis in a young child that presents with failure to thrive and easy fatigability with a grade II holosystolic murmur best heard over the left sternal border with a diastolic rumble over the cardiac apex?
Large ventricular septal defect (VSD)
larger VSDs tend to be quieter (grade II) due to less turbulence; the diastolic rumble is heard due to increased flow across the mitral valve
What is the likely diagnosis in an infant that presents with distant heart sounds and hypotension with cardiomegaly on X-ray one week after having cardiac surgery?
Postpericardiotomy syndrome (pericardial effusion after cardiac surgery)
What is the likely diagnosis in an infant that presents with one month of biphasic stridor that improves with neck extension, but not with prone positioning?
Vascular ring
due to compression of the trachea; compression of the esophagus can occur as well (e.g. dysphagia, vomiting, difficulty feeding)
What is the likely diagnosis in an infant with Down syndrome that presents with heart failure and a systolic ejection murmur at the left upper sternal border and holosystolic murmur at the left lower sternal border?
Complete AV septal defect
most common heart defect in Down syndrome patients; murmurs are due to ASD and VSD, respectively
What is the most common heart defect in patients with Down syndrome?
Complete AV septal defect
What is the next step in management for a child with a grade II systolic ejection murmur that decreases with standing?
Reassurance
benign murmurs are typically early or mid-systolic grade I or II murmurs that decrease in intensity with standing or Valsalva
What is the next step in management for a newborn with cyanosis that does not improve with 100% O2 and a continuous machine-like murmur on auscultation?
Administer prostaglandin E1
cyanosis that fails to improve with 100% O2 is indicative of a possible congenital heart defect; maintaining the patency of the PDA can be life-saving
What is the next step in management for a well-appearing infant with a harsh, grade III holosystolic murmur heard best over the left lower sternal border?
Echocardiography
this patient likely has a small VSD, but imaging is needed to determine the size and location; small VSDs typically close spontaneously with no treatment
What is the recommended treatment for a patient that presents with mitral stenosis secondary to suspected rheumatic fever?
Continous antibiotic prophylaxis (e.g. IM benzathine penicillin G every 4 weeks)
patients with a history of rheumatic fever have a high risk for recurrence and progression of rheumatic heart disease with repeated episodes of group A Streptococcus pharyngitis
What viral pathogens are most commonly associated with viral myocarditis?
coxsackie B and adenovirus
[…] is a congenital heart disease that is characterized by:
- Pulmonary infundibular stenosis
- Right ventricular hypertrophy
- Overriding aorta
- Ventricular septal defect (VSD)
Tetralogy of Fallot is a congenital heart disease that is characterized by:
- Pulmonary infundibular stenosis
- Right ventricular hypertrophy
- Overriding aorta
- Ventricular septal defect (VSD)
mnemonic: PROVe; overriding aorta is the positioning of the aorta directly over the VSD
Tetralogy of Fallot is a congenital heart disease that is characterized by:
- […]
- Right ventricular hypertrophy
- Overriding aorta
- Ventricular septal defect (VSD)
Tetralogy of Fallot is a congenital heart disease that is characterized by:
- Pulmonary infundibular stenosis
- Right ventricular hypertrophy
- Overriding aorta
- Ventricular septal defect (VSD)
mnemonic: PROVe; overriding aorta is the positioning of the aorta directly over the VSD
Tetralogy of Fallot is a congenital heart disease that is characterized by:
- Pulmonary infundibular stenosis
- […]
- Overriding aorta
- Ventricular septal defect (VSD)
Tetralogy of Fallot is a congenital heart disease that is characterized by:
- Pulmonary infundibular stenosis
- Right ventricular hypertrophy
- Overriding aorta
- Ventricular septal defect (VSD)
mnemonic: PROVe; overriding aorta is the positioning of the aorta directly over the VSD
Tetralogy of Fallot is a congenital heart disease that is characterized by:
- Pulmonary infundibular stenosis
- Right ventricular hypertrophy
- […]
- Ventricular septal defect (VSD)
Tetralogy of Fallot is a congenital heart disease that is characterized by:
- Pulmonary infundibular stenosis
- Right ventricular hypertrophy
- Overriding aorta
- Ventricular septal defect (VSD)
mnemonic: PROVe; overriding aorta is the positioning of the aorta directly over the VSD
Tetralogy of Fallot is a congenital heart disease that is characterized by:
- Pulmonary infundibular stenosis
- Right ventricular hypertrophy
- Overriding aorta
- […]
Tetralogy of Fallot is a congenital heart disease that is characterized by:
- Pulmonary infundibular stenosis
- Right ventricular hypertrophy
- Overriding aorta
- Ventricular septal defect (VSD)
mnemonic: PROVe; overriding aorta is the positioning of the aorta directly over the VSD
Patients with Tetralogy of Fallot may squat in response to cyanotic spells, which decreases right-to-left shunting by increasing […]
Patients with Tetralogy of Fallot may squat in response to cyanotic spells, which decreases right-to-left shunting by increasing systemic vascular resistance (SVR).
inhaled O2 may help stimulate pulmonary vasodilation and systemic vasoconstriction, which can further decrease right-to-left shunting
Atrial septal defect (ASD) results in a […]-to-[…] shunt.
Atrial septal defect (ASD) results in a left-to-right shunt.
Compared to right-to-left shunts, left-to-right shunts lead to cyanosis at a(n) […] age.
Compared to right-to-left shunts, left-to-right shunts lead to cyanosis at a(n) later age.
“blue kids”
D-transposition of great vessels is an example of a […]-to-[…] shunt.
D-transposition of great vessels is an example of a right-to-left shunt.
causes early cyanosis
Ebstein anomaly is associated with tricuspid […] and right heart failure.
Ebstein anomaly is associated with tricuspid regurgitation and right heart failure.
In the neonatal period, a patent ductus arteriosus becomes a […]-to-[…] shunt as pulmonary vascular resistance decreases.
In the neonatal period, a patent ductus arteriosus becomes a left-to-right shunt as pulmonary vascular resistance decreases.
Larger ventricular septal defects may lead to left ventricle overload and, consequently, […].
Larger ventricular septal defects may lead to left ventricle overload and, consequently, heart failure.
Patency of a patent ductus arteriosus is maintained by […] synthesis and low O2 tension.
Patency of a patent ductus arteriosus is maintained by PGE synthesis and low O2 tension.
includes both PGE1 and PGE2
Smaller ventricular septal defects are often […] and may close spontaneously.
Smaller ventricular septal defects are often asymptomatic and may close spontaneously.
Tetralogy of Fallot results in a […]-to-[…] shunt.
Tetralogy of Fallot results in a right-to-left shunt.
blood flows across the VSD into the aorta, causing early cyanosis
The combination of right ventricle hypertrophy and pulmonary hypertension in response to left-to-right shunting eventually results in shunt […] (Eisenmenger syndrome).
The combination of right ventricle hypertrophy and pulmonary hypertension in response to left-to-right shunting eventually results in shunt reversal (Eisenmenger syndrome).
cyanosis results due to deoxygenated blood from the right ventricle entering the systemic circulation
Total anomalous pulmonary venous return (TAPVR) is associated with […] and sometimes PDA to allow for right-to-left shunting.
Total anomalous pulmonary venous return (TAPVR) is associated with ASD and sometimes PDA to allow for right-to-left shunting.
shunting allows for maintenance of cardiac output
Treatment of a patent ductus arteriosus involves […], which decreases PGE, resulting in PDA closure.
Treatment of a patent ductus arteriosus involves indomethacin, which decreases PGE, resulting in PDA closure.
Uncorrected patent ductus arteriosus may eventually result in late cyanosis in the […] extremities.
Uncorrected patent ductus arteriosus may eventually result in late cyanosis in the lower extremities.
occurs in the lower extremities because PDA arises after the major branches of the aortic arch
Ventricular septal defect (VSD) results in a […]-to-[…] shunt.
Ventricular septal defect (VSD) results in a left-to-right shunt.
size of defect determines extent of shunting and age at presentation
Tricuspid atresia requires both […] and […] for viability.
Tricuspid atresia requires both ASD and VSD for viability.
Truncus arteriosus is an example of a […]-to-[…] shunt.
Truncus arteriosus is an example of a right-to-left shunt.
causes early cyanosis; deoxygenated blood from RV mixes with oxygenated blood from LV before pulmonary and aortic circulations separate.
What does the E in J♥NES criteria represent?
Erythema marginatum
annular, nonpruritic rash with erythematous borders, commonly involving trunk and limbs
What does the J in J♥NES criteria represent?
Joint (migratory polyarthritis)
swelling and pain in a large joint that resolves within days and “migrates” to involve another large joint
What does the N in J♥NES criteria represent?
Nodules in skin (subcutaneous)
What does the S in J♥NES criteria represent?
Sydenham chorea
rapid, involuntary muscle movements
What does the ♥ in J♥NES criteria represent?
Pancarditis (includes endocarditis, myocarditis, and pericarditis)
What effect does atrial septal defect (ASD) have on S2 splitting?
Causes a wide, fixed split
increased blood in right heart delays closure of pulmonary valve
What is the most common congenital heart defect?
Ventricular Septal Defect (VSD)
[…] is characterized by the pulmonary artery arising from the left ventricle and aorta from the right ventricle.
D-transposition of the great vessels is characterized by the pulmonary artery arising from the left ventricle and aorta from the right ventricle.
[…] is a congenital heart disease characterized by downward displacement of tricuspid leaflets into the right ventricle.
Ebstein anomaly is a congenital heart disease characterized by downward displacement of tricuspid leaflets into the right ventricle.
[…] is a congenital heart disease characterized by drainage of pulmonary veins into the right circulation.
Total anomalous pulmonary venous return (TAPVR) is a congenital heart disease characterized by drainage of pulmonary veins into the right circulation.
[…] is a congenital heart disease characterized by a single large vessel arising from both ventricles before eventually splitting.
Truncus arteriosus is a congenital heart disease characterized by a single large vessel arising from both ventricles before eventually splitting.
ASD and endocardial cushion defects
Down syndrome
PDA
Congenital rubella
Coarctation of the Aorta
Turner syndrome (many also have bicuspid aortic valve)
Coronary artery aneurysms
Kawasaki disease
Congenital heart block
Neonatal lupus
Supravalvular aortic stenosis
Williams syndrome
Conotruncal abnormalities
Tetrology of Fallot (overriding aorta), truncus arteriosus, DiGeorge syndrome (Tetrology of Fallot), velocardiofacial syndrome
Ebstein abnormality (displacement of the tricuspid valve leading to atrialization of the RV)
Maternal lithium use during pregnancy
Heart failure
Neonatal thyrotoxicosus
Asymmetric septal hypertrophy and transposition of the great vessels
Maternal diabetes
ASD associated syndromes
- Holt-Oram syndrome
- fetal alcohol syndrome
- Trisomy 21
VSD associated syndromes
- Apert syndrome
- Down syndrome
- fetal alcohol syndrome
- ToRCHeS infections
- Cri du chat syndrome
- Trisomies (13, 18, 21)
Holt Oram syndrome
Holt-Oram syndrome is characterized by skeletal abnormalities of the hands and arms (upper limbs) and heart problems. (absent radii, first degree heart block, ASD)
Apert syndrome
Apert syndrome is a rare genetic condition that is apparent at birth. People with Apert syndrome can have distinctive malformations of the skull, face, hands, and feet. Apert syndrome is characterized by craniosynostosis, a condition in which the fibrous joints (sutures) between bones of the skull close prematurely.
