12. Occurs most commonly while person is standing, especially for long periods of time, but can occur with emotional stress or noxious stimuli (like blood). Usually have a prodrome of dizziness, nausea, change in hearing and vision.
13. MUST get a good famhx to rule out potential familial syndromes (like long QT)
44. Less commonly due to actual problems with the myocardial performance, which can arise fro an inflammatory or infectious process that directly affects the myocardium and affects its contractility
47. Identify the importance of physical findings (e.g. edema, hepatomegaly, JVD, cardiomegaly, gallop rhythm) in CHF in older kids
48. The PREP questions like to point out this gallop rhythm; it is due to diminished systolic and diastolic function of the LV
49. As LV dysfunction increases, the right heart becomes affected as the increased pressure from the LV is transmitted through the pulmonary vascular system and then to the right heart (increased pressure against which the RV must pump); R sided heart failure leads to these classis findings of the hepatomegaly, SOB, crackles (less commonly seen in kids that adults), JVD
50. In older kids a dilated cardiomyopathy is a dx to consider with presentation of new CHF; for boards purposes think of this when they mention the kid having a recent URI
51. Know how an imaging study of the chest may help dx CHF
60. Understand the role of the pulmonary vascular bed in the presentation of CHF in infants with large LR shunts
61. As the pulmonary vascular bed resistance decreases during the initial days/weeks of life, and the pulmonary pressures drop below the systemic pressures, the L R shunting will increase and therefore the condition will worsen. The left heart faces a higher afterload, and therefore there is increased back pressure to the pulmonary vasculature, and when it has a lowered resistance, the increased pressure from the LV is transmitted against the pulm vasculature and then to the R heart
62. Signs of R heart failure become evident as this occurs
79. Since cardiogenic shock is so immediately critical and life threatening, need to rule it out as quickly as possible, therefore doing the EKG and echo early and fast is warranted
80. Other studies, like a CBC and blood cx, can wait per PREP, and LPs can make the condition worse
83. Gallop rhythm, hepatomegaly, tachypnea, lethargy, dehydration later signs, which are c/w the actual shock, are tachycardia, hypotension, decreased pulses/perfusion at which point the shock has gone from compensated to uncompensated
84. Know the tx of cardiogenic shock in the newborn infant
85. Giving fluids will worsen cardiogenic shock because it causes worsening congestion in the pulmonary circuit and adds more volume load to the already failing/dilated LV
86. If fluids are given to a pt in cardiogenic shock, will likely see and increase in HR as the heart attempts to increase CO (but is unable to increase SV due to poor pump)
87. The treatment = inotropics (dopamine) to increase contractility and thereby increase SV and CO
88. DO NOT GIVE MORE FLUIDS AND DO NOT OBTAIN FURTHER DIAGNOSTIC TESTS; you need to save the baby.
91. Acrocyanosis = transient bluish discoloration of the hands and feet in response to vasomotor instability or a cold environment. The perioral region can also be involved (spares the lips and mucous membranes), and the extremities may be cool on examination
92. Central cyanosis = due to deoxygenated blood or reduced hgb concentration (usually in the range of 4-6g/dL). Affected infants have bluish discoloration of the lips, tongue, mucous membranes, and occasionally nail beds. Cyanosis is usually clinically apparent when the O2 sat <70-80%
93. Causes = right to left shunt due to CHD or respiratory disease, hypoventilation, methemogobinemia
100. Tet spell in an infant = distress, crying, inconsolability, hyperpnea, increased cyanosis, also during a tet spell, the murmur will be diminished or absent b/c the murmur is due to pulm stenosis (decreased volume or RH output therefore = decreased murmur)
101. Often occur in the morning or times of dehydration the spell is brought on by diminished pulmonary blood flow
103. Can be accomplished immediately by placing the patient in a knee to chest or a squatting position which will increase venous return and also increase systemic resistance
105. Know the cardiac causes of cyanosis in a newborn (cyanotic hours to days after birth)
106. TOF will present with cyanosis in the newborn period only when the pulmonary stenosis is severe and causes a significant RL shunt at the VSD
107. Transposition of Great Arteries (TAG) most common form of cyanotic heart disease to present in the first day after birth (overall is the second most common cyanotic heart disease, after TOF); the LV connects to the pulm artier and the RV connects to the aorta so that deoxygenated blood returning via the SVC into the RA enters the RV and then back out the aorta (not oxygenated). They can be profoundly cyanotic if there is no reliable method for mixing left and right sided blood (left sided/oxygenated blood into the right side via and ASD or VSD) These babies are often otherwise normal, healthy appearing babies
108. Tricuspid atresia (anatomy review: they do not have a tricuspid valve, therefore blood from the RA cannot get to the RV, and the only way it can get out of the RA would be through an ASD, taking blood from the RA to the LA to the LV; once in the LV the only way for blood to get oxygenated is via a left to right shunt across a VSD into the RV where it can then go to the lungs). The obligate right to left shunt of desaturated blood from the RA to the LA , then to the LV and then systemic causes the early cyanosis
109. Truncus Arteriosusoccurs when a single arterial outlet supports the coronary, systemic and pulmonary blood flow. Cyanosis occurs when the origins of the pulm arteries from the truncus are narrowed
110. Total Anomalous Pulm Venous Return usually lack a direct connection between the pulm veins and the LA; the pulm veins drain into the RA or into veins that eventually drain into the RA. There are three forms, depending on where the connection occurs. Supracardiac will drain into the left innominate vein and then into the SVC. Cardiac drain directly to the RA or coronary sinus. Infracardiac (subdiaphragmatic) go below the diaphragm, connect with the ductus venosus and then into the IVC. This third kind is most likely to have obstructed flow and therefore early cyanosis. Usually FTT and tachypnea are presenting signs, but if they have a severe obstruction, the cyanosis may be the presenting sign and pulm edema can develop rapidly
111. Recognize the absence of improvement in arterial oxygen content with 100% oxygen in comparison with room air is compatible with the dx of cyanotic CHD
114. If a pt has CHD that cannot be corrected, partial volume exchange transfusions may be necessary to maintain Hct <65% or lower depending on signs and symptoms of complications of polycythemia
117. Understand the prognosis for cognitive development in patients with cyanotic congenital heart dz
118. Most kids who’ve had cyanotic CHD, even after early repair, are likely to have some degree of cognitive impairment
119. Outcome is related to degree of pre-op hypoxia, intraoperative management (cardiac bypass time), and post op complications
120. If they have abnormal findings on neurologic exam they are more likely to have poorer school performance than kids with same lesions and no neurologic findings
121. If their lesions do not require surgical repair they are less likely to have poorer school performance
122. Kids with acyanotic heart disease are less likely to have poorer school performance than those with cyanotic heart disease
123. Know that relative anemia can be associated with a stroke in a patient with cyanotic CHD
124. Can be related to iron-deficient blood with decreased O2 carrying capacity as well as structural changes in the erythrocytes made under the iron-poor environment that can cause a higher viscosity despite the anemia
129. Recognize the major clinical findings in patients with cardiac anomalies such as VSD, ASD, PDA and aortic stenosis or pulmonic stenosis
130. ASD exercise intolerance due to left to right shunting, systolic murmur without a click (due to increased flow across the pulmonic valve, not due to flow through the ASD), fixed and split second heart sound due to increased time required for the dilated right ventricle to empty its contents
131. VSD usually a holosystolic murmur caused by the left to right shunt from LV to RV beginning with onset of systole. If small, the murmur may be high pitched and heard best along the sternal border. The second heart sound is normal.
132. This is the most common congenital heart defect
134. Symptoms would arise from increased strain on the RV due to increased volume/pressure as the systemic resistance increases and the LR shunt across the defect increases during the first few weeks of life, usually sx develop at about 4-8 weeks of age
135. PDAproduces a continuous machinery-like murmur that is usually loudest at the left infraclavicular area. If the opening is large there may be increased aortic pulse pressure and bounding pulses
136. Aortic stenosisis associated with a systolic ejection click and a murmur best heard at the RUSB with radiation to the neck. The findings do not change with position. An infant with severe congenital AS may present fairly early with the findings of the murmur, diminished perfusion and pulses, signs of pulmonary edema and also may appear to be in septic shock. If it does not present in newborn period it will present at some point in later infancy or childhood because the problem gets worse as the kid gets bigger and the stenosis becomes more critical and the LV wears out
137. Pulmonic stenosissystolic ejection murmur at the LUSB with an associated opening click, the murmur often radiates to the back and the axillae as the sound of turbulence flows through the branch pulmonary arteries. This is the second most common CHD after VSD. A critical stenosis can present shortly after birth because the RV cannot effectively pump against the stenotic valve. Cyanosis can be present due to right to left shunting at the level of the atria. They are ductal dependent in order for blood to get to the lungs, it has to traverse the PDA in order to enter the pulmonary artery; therefore they present as/when the duct is closing with severe cyanosis and cardiac collapse, but in general it is not a cyanotic lesion except in the critical newborn. The majority of affected children have no other associated sx but need to be monitored over time
138. Know the importance of PDA in the presentation of hypoplastic left heart syndrome and in coarctation of the aorta
139. In cases severe coarctation, keeping the PDA open can help relieve an acute aortic obstruction (since the coarctation is often due to a posterior shelf into the aorta that is created by the closure of the PDA due to a defect in the vessel media)
141. Hypoplastic left heartan underdeveloped left heart is basically nonfunctional and so the RV must pump for both pulmonary and systemic blood delivery via the PDA. If the PDA is not kept open, then there is no way for blood to go systemic
158. Procedures that require ppx = any dental procedure that require extensive manipulation of the gingival or when perforation of the oral mucosa is likely; it is also reasonable to provide ppx for procedures involving the respiratory tract, infected skin, or musculoskeletal tissue; GI and GU tract procedures do not require ppx
159. Know the drugs of choice for the ppx of infective endocarditis
172. (HACEK = Haemophilus sp, Actinobacillus actinomycetemcomitants, cardiobacterium hominis, elkenella corrodens, kingella kingae) – these are gram negative oral and pharyngeal flora that are fastidious and slow growing and require special media to culture
212. Chest pain = substernal, positional (worse when laying down) and can be severe and worsen with deep inspiration, coughing, or movement of the upper torso. Pts usually prefer to sit up or lean forward
232. If pt meets dx criteria for KD then the echo is required in order to look for evidence of carditis
233. If it is an atypical case, then echo findings can help in confirming dx
234. Identify the cardiac complications of KD and the timing of onset; understand the prevention and treatment of the complications and the importance of follow up evaluation
235. About 50% of cases will show carditis by the end of the first week (acute phase)of sx, dilation (ectasia) may also appear at this phase
236. Coronary artery aneurysm usually appears at week 2-4 (subacute phase); its incidence is reduced greatly (from about 25% to 3% if pt is txed with IVIg during the acute phase)
255. Recognize that pts with Marfan syndrome may have associated cardiac dz that precludes participation in sports
256. Cardiac abnormalities can include dilation or aneurysm of arterial vessels, mitral valve dysplasia/prolapse/dysfxn, possible also to have same probs with tricuspid valve
257. Need to have their heart okayed before they can play or practice sports
258. Know the CV conditions associated with Turner syndrome
265. Have signs of superficial venous distention, venous congestion and facial and upper extremity swelling; as it progresses the jugular veins may become affected and there can be cerebral edema/seizures/death
266. Know the importance of CV evaluation when there is a fam hx of hypertrophic cardiomyopathy, MD, or Marfan
267. Hypertrophic cardiomyopathy is autosomal dominant inheritance, so if a parent has it all the kids need to be screened early and regularly as they have a 50% chance of inheriting it