exam 1 - cardio Flashcards

Question

kawasaki disease

Answer 1

-Leading cause of acquired heart disease in children in the U.S. -8% of pts < 5 years, M:F ratio is 1.5:1 -Diagnostic Criteria -Fever > 4-5 days AND at least four of the following: -Bilateral, painless, nonexudative conjunctivitis -Lip or oral cavity changes (cracking, strawberry tongue, inflammation of oral mucosa) -Cervical lymphadenopathy > 1.5 cm in diameter (usually unilateral) -Polymorphous exanthem -Extremity changes (redness/swelling of hands/feet with desquamation) -Potential for cardiovascular complications -Myocarditis, pericarditis, valvular heart disease, coronary arteritis -Mild, transient dilation of CA to large aneurysms (most resolve spontaneously, others cause complication/require treatment) -Risk: Males, infants < 6 months, those not treated with IVIG -Treatment -IVIG + high-dose aspirin (followed by low-dose x 6-8 weeks) -2 g/kg IVIG over 10-12 hours and 80-100 mg/kg/day (or 30-50 mg/kg/day) divided QID -Dual therapy with IVIG + infliximab or corticosteroid -Reduces inflammation faster -Effective in reducing CA dilation/aneurysms -Consider for patients with persistent fever despite 1-2 infusions of IVIG

Answer 2

-MC of the cardiomyopathies in children -Causes: Primarily idiopathic; viral myocarditis, untreated tachyarrhythmias, L heart obstructive lesions, congenital abnormalities of the coronary arteries, medication toxicity, and genetic/metabolic diseases -Clinical Manifestations -Heart dilates > CO falls > decreased exercise tolerance, FTT, diaphoresis, and tachypnea -Continued failure > Congestive signs (hepatomegaly, rales), prominent gallop on auscultation -Imaging: Cardiomegaly with or without venous congestion -ECG: Sinus tachycardia with ST segment changes -Evaluation for supraventricular arrhythmias is critical (one of the few treatable and reversible causes of DCM in children -Echocardiogram -LA/LV enlargement with decreased ejection fraction -End-diastolic/systolic dimensions increased -Mitral insufficiency commonly seen -Other Testing -Cardiac catheterization: Evaluate hemodynamic stability and coronary artery anatomy -Endomyocardial biopsies/PCR testing: Inflammation consistent with acute myocarditis, abnormal myocyte architecture, and myocardial fibrosis -Electron micrographs: Mitochondrial/metabolic disorders -Cardiopulmonary stress test: Response to therapy, assessment of cardiac limitations on exercise -Treatment -Afterload-reducing agents + diuretics -Aspirin/warfarin to prevent thrombus formation -Anti-arrhythmic therapy possible -If medical management unsuccessful, heart transplant considered

Answer 3

-Leading cause of sudden cardiac death in children -Familial (MC) and non-familial forms -Familial: MC caused by mutation in one of several genes that encode proteins of the cardiac sarcomere -Non-familial: Glycogen storage disease, Noonan syndrome, Friedreich ataxia, maternal gestational diabetes, mitochondrial disorders, and other metabolic disorders -Asymptomatic -Symptoms of inadequate coronary perfusion or HF: Angina, syncope, palpitations, or exercise intolerance -Initial presentation may be SCD! – Precipitated by sporting activities -Exam: -L precordial bulge with diffuse PMI -LV heave or S4 gallop -If outflow obstruction > systolic ejection murmur -Provoked by exercise or positional maneuvers that decrease LV volume (standing) – increase outflow over obstruction

Answer 4

-Echocardiogram -Asymmetrical septal hypertrophy, concentric (metabolic/non-familial) -Possible LV outflow obstruction -Systolic function – hypercontractile; diastolic function almost always abnormal -ECG -Deep Q waves in inferolateral leads (increased mass of hypertrophied septum) -LVH/LAE -Other Testing -Stress testing to evaluate for provocable LV outflow tract obstruction, ischemia, and arrhythmias; to determine prognosis -Extreme LVH, blunted BP response to exercise > increased mortality -Cardiac MRI: Defining areas of myocardial fibrosis/scarring -Cardiac Catheterization -Hemodynamic findings: Elevated LA pressure (impaired diastolic filling) -Provocation of LV outflow obstruction, angiography, biopsy (myofiber disarray) -Treatment/Prognosis -Restricted from competitive athletics and isometric exercise -Resting/latent outflow obstruction: B-blockers, verapamil, disopyramide -Surgical myectomy with resection of -part of the hypertrophied septum -Ethanol ablation (HCM with outflow obstruction) -Ethanol into septal coronary artery > MI > reduction in septal size/outflow obstruction -Not often performed in children -Dual-chamber pacemaker/defibrillators not well studied in children

Answer 5

-BP should be determined at every pediatric visit beginning at 3 years of age -HTN prevalence approximately 3% -Pediatric standards for BP published based on age, gender, and ht: dont need to know -Normal: < 90th percentile AND < 120/80 -Pre-HTN: > 90th percentile OR > 120/80 and < 95th percentile -Stage I HTN: > 95th percentile and < 99th percentile + 5 mmHg -Stage II HTN: > 99th percentile + 5 mmHg -If properly measured BP exceeds 95th percentile, measurement should be repeated on at least 3 separate occasions over a 2-4 week interval -Ambulatory BP monitoring Performed over 24 hours with at least 40 readings during waking and sleeping hours -If BP persistently elevated, search for the cause should be undertaken -Most HTN is primary -Causes may include: Coarctation of the aorta, renal artery stenosis, chronic renal disease, pheochromocytoma, and medication side effects (steroids) -Treatment (primary HTN) -1st line: Lifestyle changes with diet and exercise!!!!!!!!!!!!!! -Anti-HTN agents: ACEIs, ARBs, CCBs, diuretics -Maximizing monotherapy prior to introducing a second agent remains official guideline

Answer 6

-Abnormal lipid levels are relatively common in children and adolescents (affects approx 1 in 5 adolescents) -Lipid disorders are less common -Severe hypercholesteremia (LDL > 190 mg/dL) – 1 in 250 adolescents -Occur in assoc with/ obesity and accompanied by other risk factors that contribute to increased rates of CV and metabolic morbidity/mortality -Abnormalities in lipid profile appearing early in childhood correlate with higher risk for CAD in adulthood

Answer 7

-Routine screening is controversial -Reasonable to check a fasting or non-fasting lipoprotein as early as 2 years of age with: family history of early CVD (< 55 in men, < 65 in women) or significant hypercholesterolemia (total cholesterol > 240 mg/dL), to detect familial hypercholesterolemia or other rare forms of hypercholesteremia -AHA guidelines: -Universal lipid screening at 9-11 years old and then at ages 17-21 years -Checking fasting lipid panel indicated in children and adolescents with obesity or metabolic risk factors (high blood sugar, low HDL, high triglycerides, high BP, large waist circumference)

Answer 8

-LDL > 130 mg/dL on two successive tests -First-line: Lifestyle changes with dietary counseling and regular physical activity -If unresponsive after 3-6 months and at extreme risk, drug therapy to decrease ASCVD risk -Statins (coenzyme A reductase inhibitors) most commonly used and safe as early as 8-10 years -Familial FH -Cholestyramine (bile acid sequestrant) as early as 6 years, colesevelam (10 years) -Ezetimibe (10 years) + statin -Niacin for hypertriglyceridemia

Answer 9

-Transient LOC and postural tone resulting from an abrupt, temporary decrease in cerebral blood flow -Majority of episodes self-limited and benign – vasovagal syncope -Disorder of HR and BP control by ANS > causes hypotension or bradycardia -Rarely first warning sign of arrhythmia, structural heart disease, or noncardiac disease

Answer 10

-History: Age, time of day, state of hydration/nutrition, environmental conditions, patient’s activity/body position, frequency/duration of episodes, aura/prodrome/symptoms prior to episode -Additional: Medications, history of viral illness, medical history (neurologic disorders, TBI, neurosurgical interventions), family history (cardiac, exertional, sudden death in children, seizures), witnesses -Physical -General: Hydration, nutritional status, thyroid disease manifestations -Orthostatic VS -Decrease in systolic BP of 20 mmHg or diastolic of 10 mmHg after 3 minutes of standing when compared to BP in supine or sitting position -Pulse strength, rate, and differences between U/L extremities -Heart murmurs > echocardiogram -Phenotype of inherited genetic disorders -ECG: Evaluate for HR, corrected QT interval, T-wave abnormalities, ventricular arrythmias, AV conduction disturbances, or Brugada syndrome (predisposes to VT)

Answer 11

-Response of cardiac-CNS system reflex -Prolonged/rapid assumption of an upright position > venous pooling in lower extremities > central hypovolemia leading to decreased venous return and SV -Emotional/physical stress (pain/fright) > sympathetic response > tachycardia/vasoconstriction > subsequent parasympathetic response characterized by bradycardia/asystole and decrease in BP/SV -Clinical Manifestations -Prodrome: Nausea, epigastric pain, clammy sensation, pallor, dizziness, lightheadedness, tunnel vision, and weakness -LOC > falls into supine state > restoration of venous return/central blood volume -LOC is short (< 1-2 minutes), with rapid return to baseline behavior -Myoclonic “jerks” common, loss of bowel/bladder uncommon -Treatment -Maintaining adequate intravascular volume -Clear urine 5 times daily -Increased salt intake/salt tablets -Leg pumping, leg crossing, and squatting with presyncopal symptoms -Regular aerobic exercise -Medications (limited studies) -Fludrocortisone (renal salt resorption), midodrine (alpha-agonist), B-blockers (target increased sympathetic output initiating reflex), vagolytic agents (disopyramide), SSRIs