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2 Peds Cardiology

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Question
Answer
Most common congenital heart defect   bicuspid aortic valve. Incidence of congential heart disease: 8-10/1000 (.8%), but if you take into consideration bicuspid aortic valves, the incidence is: 15/1000  
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4 heart defects   Hypoplastic left heart syndrome, congenital aortic stenosis, Coarctation of aorta, transposition of the great heart vessels. Almost always symptomatic when born.  
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Most defects occur in   otherwise healthy, well-developed, term infants.Transition from fetal to more adult circulation unmasks the defects. Hypoplastic left and right heart is perfectly protected in utero b/c of fetal circulation.  
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Heart has finished its formation at about   60 days post conception. 60% of spontaneous abortions occur due to lethal heart disease  
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Heart Disease associated with Down's Syndrome   AVSD (no true tricuspid or bicuspid valve, only an AV valve), TOF. (50% of all Down's Syndrome kids will have a heart disease). These kids tend to age faster: never drop pulmonary resistance to nl. Can go from birth to pulmonary vascular dz in 5 months.  
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Heart Dz associated with Turner's Syndrome   Bicuspid aortic valve, coarctation of the aorta (COA)  
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Heart Dz associated wtih DPH syndrome   PS  
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Heart Dz associated with Trisomy 13/18   VSD, DORV  
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Heart Dz associated with Marfan's syndrome   MVP (mitral valve prolapse), MR (mitral regurgitation), AI, DAA  
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First thing to do in Down's   EKG (in nl kids, you will see a right axis deviation b/c they have a very thick R ventricle) in down's: Left axis deviation. Good marker for AVSD. Then do Echo to find extent of defect  
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First Breath   clears out all the fluid from the lung. Pulmonary vascular resistance drops but doesn't get to normal until 3-6 months of life. PO2 blood levels increase b/c you are bleeding, Pulmonary blood flow increases, pulmonary venous return to atrium goes up.  
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Increased PO2 causes   closure of the vaso vasorum around the ductus arteriosus. remnant of ductus arteriosus is the ligament arteriosum  
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Disconnecting placenta   increases vascular resistance, Left ventricle dilates. Now able to provide more output to the periphery!  
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3 presentations of heart disease   cyanosis, CHF, murmur/abnormal physical exam  
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Early recognition is   first few days of life. late - a week out  
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Most common cause of V/Q mismatch in premies   lack of surfactant. Other causes: meconium, pneumonia, retained amniotic fluid, etc.  
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reversible form of heart failure in DM moms.   neonatal hypoglycemia (high circulating insulin levels, with no way to put out glucose fast).  
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First sign of CHF in babies   Tachypnea, then tachycardia, then hepatomegaly (not edema and rales like in adults!).  
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Cost of tachypnea   myocardial oxygen consumption  
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Sequence of evaluation   History, Physical Examination, Electrocardiogram, Chest Radiograph, Echocardiogram, Other Studies including stress test, 24-Holter, ambulatory ECG monitoring, cardiac cath, angiocardiography, nuclear imaging, cardiac MRI  
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Where is the thrill of aortic stenosis felt?   In the suprasternal notch  
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In patients with severe pulmonary htn, where is palpable pulmonary closure (P2) frequently noted?   at the upper left sternal border  
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Sx of decreased pulmonary blood flow in an infant/toddler   cyanosis, squatting, loss of consciousness  
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Sx of Increased Pulmonary blood flow in an infant/toddler   tachynpnea with activity/feeds, Diaphoresis, poor weight gain  
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Sx of Decreased pulmonary blood flow in an older child   dizziness, syncope  
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Sx of Increased Pulmonary blood flow in an older child   exercise intolerance, dyspnea on exertion, diaphoresis  
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S1   First heart sound is teh atrioventricular (AV) valve closure. Best heard at the lower left sternal border  
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S2   semilunar valve closure. Best heard at the upper left sternal border. Two sounds A2 and P2 (aortic and pulmonary valve closure). Normal splitting: widening w/ inspiration and narrowing with expiration. Abnl splitting of S2 may be an indication of heart dz  
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S3   the sound of rapid filling of the left ventricle. A persistent S3 often heard in the presence of a dilated LV caused by cardiomyopathy or large L-to-R shunt  
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S4   Associated with atrial contraction and is normally not audible. Occurs before S1. It is heard in the presence of atrial contraction into a noncompliant ventricle as in hypertrophic or restrictive cardiomyopathy or hypertrophied LV from other causes  
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____% of children have an innocent murmur at some time during childhood   40-45%  
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How do describe murmurs   Location and radiation, Relationship to cardiac cycle and duration (systolic, pansystolic, diastolic, and continuous), Intensity, Quality (harsh, musical, rough; high, medium, or low in pitch), Variation with position (supine, sitting, standing,squatting)  
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Murmurs heard during systolic ejection   semilunar valve stenosis (AS/PS/truncal stenosis), ASD, Coarctation  
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Causes of Pansystolic murmurs   VSD, AVVR (atrioventricular valve regurgitation)  
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Causes of diastolic murmurs   Semilunar valve regurgitation (AI/PI/truncal insufficiency), AV valve stenosis (MS/TS)  
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Causes of continuous murmurs   Runoff lesions (PDA/AVM/aortopulmonary collaterals)  
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6 Most common Functional murmurs in childhood are:   Newborn murmur, Functional murmur of peripheral arterial pulmonary stenosis, still murmur, pulmonary ejection murmur, venous hum, innominate or carotid bruit  
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Where is a newborn murmur heard?   lower left sternal border; first few days of life, usually disappears by age 2-3 weeks.  
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Systolic ejetion murmur heard with equal intensity at the upper left sternal border, at the back and in both axillae; usually disappears before age 2   Functional murmur of peripheral arterial stenosis; results from the normal branching of the pulmonary artery  
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Most common innocent murmur of early childhood   still murmur. Usually heard between ages 2-7 years. Musical, vibratory, short, high-pitched, systolic.  
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Most common innocent murmur in older children   Pulmonary ejection murmur. Heard from age 3 years onward. Usually a soft systolic murmur, well localized to the upper left sternal border  
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This functional murmur is continous and is produced by turbulence at the confluence of the subclavian and jugular veins   venous hum  
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long harsh systolic murmur that is heard in the right supraclavicular area. Functional   Innominate or carotid bruit  
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Bounding pulses are characteristic of   run-off lesions, including patent ductus arteriosus (PDA), aortic regurgitation, arteriovenous malformation (AVM), or any condition with low diastolic pressure (fever, anemia, or septic shock)  
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Extracardiac Examination   Arterial Pulse, Quality and amplitude of pulse, arterial BP (UE & LE), Extremities (cyanosis, clubbing, edema), Abdomen  
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Cardinal sign of right heart failure   hepatomegaly. Hepatomegaly can also be seen in a child with pulmonary edema from lesions causing L-R shunting or left heart failure. Splenomegaly may be present in pts with longstanding CHF & is characteristic of infective endocarditis  
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heart size in relation to chest in child older than 1 yr   heart size should be less than 50% of the chest diameter  
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Components to check on CXR   1. position of cardiac apex, 2.Position of abdominal viscera, 3. Cardiac Size, 4. Cardiac configuration, 5. Character of the pulmonary vasculature  
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Dextrocardia   heart on the right side of the chest. Levocardia - on the left, mesocardia-in the middle  
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This imaging technique is useful in assessing fixed and reversible areas of ischemia   Nuclear imaging. Valuable in evaluating myocardial perfusion in patients with Kawasaki dz, etc.  
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What causes the closure of the ductus arteriosus?   Pulmonary vascular resistance falls below that of the systemic circuit, resulting in a change in blood flow across the ductus arteriosus from left to right which closes the flap. Usually closes between 7-14 days  
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The one-way valve in fetal life that shunts blood from the inferior vena cava to through the right atrium to the left atrium   foramen ovale. At birth, pulmonary and systemic vascular resistance increase; left atrial pressure rises above that of the right atrium.This functionally closes the flap preventing flow across the septum. 10-15% of adults have a probe patent foramen ovale  
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Persistent pulmonary htn is a clinical syndrome that occurs in   full-term infants  
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In the nl newborn, pulmonary vascular resistance and the pulmonary arterial pressure (Decrease/increase)   Decrease; this phenomenon results from demusclularization of the pulmonary arterioles  
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HF   condition in which the heart fails to meet the circulatory and metabolic needs of the body. Usually occurs in infants before 6 months. Common causes in infancy: VSD, PDA, COA, AVSD, Large AVMs, and chronic atrial tachyarrhythmias  
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Children with HF may present with   irritability, diaphoresis with feeds, fatigue, exercise intolerance, or evdienceof pulmonary congestion, tachypnea  
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In CHF, when ventricular systolic dysfunction presents, what neurohormone is activated?   catecholamines from the adrenal glands are released and results in tachycardia, diaphoresis, and indirectly through activation of the renin-angiotensin system, peripheral vasoconstriction, and salt and water retention  
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Acyanotic heart disorders   ASD & VSD, PDA, Coarctation of the Aorta, Aortic stenosis, MVP  
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ASD: Rarely presents as CHF. Peripheral pulses nl and equal.   RV heave felt best at the mid to lower left sternal border. Ejection-type systolic murmur heard best at left sternal border at 2nd intercostal space. Murmur caused by increased flow across pulmonic valve  
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Most common congenital heart malformation   Simple VSD, accounting for about 30% of all cases of congenital heart dz. 80-85% are small and close spontaneously.  
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Consequence of Large VSD   CHF. Large: 6-10mm in diameter  
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VSD presentation   small to moderate: no CV sx. Large: ill early in infancy. Frequent upper and lower respiratory infxns. Gain weight slowly. Dyspnea, diaphoresis, exercise intol and fatigue common.  
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AVSD   Common in Down's kids.CHF develops in infancy, recurrent bouts of pneumonia common. Heart is significantly enlarged. Echo is the diagnostic test of choice.  
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PDA    
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