midterm condensed - heart, heme, tumors Flashcards
acyanotic vs cyanotic defects
-left to right shunts- acyanotic
-right to left shunts- cyanotic
know this for cardio part of exam
Indomethacin
Exam findings
Prostaglandins to maintain patency
Patterns of cyanotic and acyanotic CHDs
VSD
-MC
-Small VSD: asymptomatic; high-frequency, loud murmur
-Moderate-large- HF
-Auscultation: Pansystolic murmur at LLSB
-Large shunts increase flow over mitral valve causing a mid-diastolic murmur at apex
-Splitting of S2 and intensity of P2 depend on PA pressure
-Imaging:
-ECG: LAE/LVH
-CXR: Cardiomegaly, LVH, increase PA size/blood flow
-PHTN -> RVH
-Echo: Location/size of defect + hemodynamic information (magnitude of shunt and R/PA pressure)
-Tx:
-1/3rd close spontaneously
-Small: Close spontaneously; if not -> surgical closure
-Initial for moderate-severe: Diuretics + digoxin/afterload reducers -> if still poor growth/PHTN -> surgical closure (closure device)
ASD
-asymptomatic, even if large
-RV impulse at LLSB palpable
-Auscultation: Soft (grade I/II) systolic ejection murmur in region of RV outflow tract and a fixed split S2 (due to overload of RV with prolonged ejection into pulmonary circuit)
-Larger shunt: Mid-diastolic murmur at LLSB from increased volume
across tricuspid valve
-Imaging:
-ECG- RV enlargement
-CXR- cardiomegaly, RAE, prominent pulmonary artery
-Echo- location/size and magnitude of shunt
-Tx: Significant shunt still at 3yo -> closure (cath lab – closure device)
patent ductus arteriosus
-Small- asymptomatic
-moderate-large- HF
-Widened pulse pressure
-Auscultation: Continuous, machine-like murmur at L infraclavicular area, radiating along pulmonary arteries
-Larger shunts: Increased flow across MV -> mid-diastolic murmur at apex and hyperdynamic precordium
-Splitting of S2/intensity of P2 depend on PA pressure
-Imaging:
-ECG- LVH -> RVH if PHTN present
-CXR- full pulmonary artery silhouette and increased pulmonary vascularity
-Echo- size/anatomy of PDA and magnitude of shunt and PA pressure
-Tx:
-Moderate-large/symptomatic: Indomethacin!!!!!!, diuretics, eventual closure (cath lab with coil embolization or PDA closure device)
tetralogy of fallot
-MC cyanotic
-4 structural defects:
-1. VSD
-2. Overriding aorta (over VSD)
-3. Pulmonary stenosis
-4. RVH
-Pulmonary stenosis murmur -> initial finding
-R-L shunting increases
-Single S2 and right ventricular impulse at L sternal border
-Hypoxic/Tet spells
-Infant: Restless, agitated, inconsolable crying
-Toddler: Squatting -> increase venous return
-Hyperpnea with gradually increasing cyanosis
-blue mucous membranes -> BAD
-Severe spells: Prolonged unconsciousness and convulsions, hemiparesis, or death
-Imaging:
-ECG: RAD, RVH
-CXR: Boot-shaped heart (small main pulmonary artery and upturned apex from RVH)
-Echo: Anatomy of pulmonary stenosis, coronary anomalies
-Tx:
-Progressive pulmonary stenosis/cyanosis
-Hypoxic spells: Oxygen, knee-chest position, ketamine/phenylephrine (increased SVR)
-Indication for surgical repair
-Complete surgical repair with VSD closure and removal/patching of pulmonary stenosis
transposition of the great arteries
-Cyanosis, tachypnea, single S2
-Imaging:
-ECG: RAD/RVH
-CXR: Increased pulmonary vascularity, “egg on a string” cardiac shadow (narrow superior mediastinum)
-Echo: transposition, sites/amounts of mixing, assoc lesions
-Tx:
-Prostaglandin E1 -> maintain patency/relax pulmonary vasculature
-Balloon atrial septostomy
-Arterial switch (complete surgical repair)
tricuspid atresia
-Severely cyanotic, single S2
-Imaging:
-ECG: LVH
-CXR: Normal/slightly enlarged cardiac silhouette with decreased pulmonary blood flow
-Echo: Anatomy, lesions, source of pulmonary blood flow
-Tx:
-Prostaglandin E1 (maintains pulmonary blood flow until surgery)
-Surgery in stages: (dont need to know)
-Blalock-Taussig procedure
-Glenn procedure
-Fontan procedure
truncus arteriosus
-HF as pulmonary vascular resistance decreases
-Tachypnea, difficulty feeding, poor growth
-Bounding peripheral pulses
-Auscultation: Possible systolic murmur with click at L sternal border, single S2 (single valve)
-Imaging:
-ECG/CXR: LVH + RVH, cardiomegaly, increased pulmonary vasculature
-Echo: Anatomy, truncal valve function, and origin of PAs
-Tx:
-Medical management: Anti-congestive medications
-Surgical repair: VSD closure with placement of conduit between RV and PAs
total anomalous pulmonary venous return
-Most important determinant is presence or absence of obstruction to pulmonary venous drainage
-Absence of obstruction:
-Minimal cyanosis/asymptomatic
-Hyperactive RV impulse, widely split S2 (increased RV volume) and a systolic ejection murmur at LUSB
-Mid-diastolic murmur at LLSB from increased flow across the tricuspid valve
-Presence of obstruction:
-Cyanosis, marked dyspnea/tachypnea, and signs of R sided HF
-Imaging:
-Absence of obstruction:
-ECG: RV overload
-CXR: Cardiomegaly with increased pulmonary blood flow
-Presence of obstruction:
-ECG: RAD/RVH (from increased pulmonary congestion)
-CXR: Heart normal/mildly enlarged with varying degrees of pulmonary edema
-Echo: Extent of volume overloaded R side of heart, R-L atrial level shunting, and common pulmonary vein site of drainage/degree of obstruction
-Tx:
-Surgery: Common pulmonary vein opened into the LA, ligation of any vein/channel that had been draining the common vein
hypoplastic left heart syndrome
-Ductus arteriosus constriction -> S&S of HF from excessive pulmonary blood flow/obstruction of systemic blood flow
-Diffusely weak/absent pulses, usually no heart murmur, S2 single/loud
-Low CO -> grayish color to cool, mottled skin
-Imaging:
-ECG: RVH with decreased LV forces
-CXR: Cardiomegaly (R-sided enlargement) and pulmonary venous congestion/edema
-Echo: Small L heart, degree of mitral/aortic valve stenosis, hypoplastic ascending aorta, adequacy of L-R atrial flow and R-L ductal flow
-Tx:
-Medical: PGE1, correction of acidosis, BP support as needed
-Surgery is staged (newborn, 4-6 months, 2-3 years)
-Heart transplant for failed surgical palliation or if systemic RV fails
coarctation of the aorta
-Infants:
-Hypoplastic aortic arch, abnormal aortic valves, and VSDs
-Sx develop when ductus closes (2 wks)
-Poor feeding, respiratory distress, and shock possibly sooner
-Femoral pulses are weaker compared to R radial pulse
-BP in LE < UE
-Older children:
-Leg discomfort with exercise, headache, epistaxis
-Decreased/absent LE pulses, HTN of UE
-Auscultation: L interscapular area of back, continuous (if collateral vessels have formed)
-Systolic ejection murmur with click if abnormal aortic valve (50% of time) is present
-Imaging
-Infants:
-ECG/CXR: RVH, marked cardiomegaly, pulmonary edema
-Older children:
-ECG/CXR: LVH, mildly enlarged heart; rib notching (from collateral vessels)
-Echo: Site/degree of coarctation, presence of LVH, aortic valve morphology/function
-Tx:
-Infant with cardiac decompensation: IV prostaglandin (chemically opens ductus – closure would otherwise worsen coarctation), inotropic agents, diuretics, supportive care
-Balloon angioplasty < surgical repair
rheumatic fever
-Group A B-hemolytic streptococcal infection
-Carditis
-Pancardiac inflammation
-Mitral valve MC affected -> insufficiency
-Aortic valve 2nd MC affected
-Early decrescendo diastolic murmur consistent with aortic insufficiency
-Polyarthritis (80% of pts)
-Large joints MC -> typically migratory
-Joint swelling/limitation of movement
-Sydenham Chorea
-emotional lability, ataxia/slurring of speech, Muscular weakness
-up to 3mo, may not be apparent for months to years after acute episode of rheumatic fever
-Erythema Marginatum: Macular, serpiginous, erythematous rash with sharply demarcated border on trunk/extremities
-Subcutaneous Nodules (severe cases)
-Occur over joints, scalp, and spinal column
-Few mm – 2 cm in diameter, nontender, free mobile
-Jones- 2 major or 1 major + 2 minor PLUS evidence of streptococcal injection
-Acute Treatment:
-Anti-Infective tx:
-Benzathine PCN: Single IM injection
-Alternative: PCN V or amoxicillin
-Allergic to PCN? Narrow-spectrum cephalosporins, clindamycin, azithromycin, or clarithromycin
-Anti-inflammatories:
-Aspirin
-Tx for HF as indicated, activity limitations
-Tx after acute episode:
-Prevention
-Regular, long-acting IM injections of benzathine PCN q 4 weeks x 5-10 years of therapy (or d/c at 21yo whichever is longer)
-Alternatives: PCN V, sulfadiazine, erythromycin less effective
-Residual Valvular Damage: Replacement, if indicated
endocardial cushion / AV septal defect
-May be complete, partial, or transitional
-Complete: ASD, posterior/inlet VSD, common AV valve
-Partial/transitional: 2, separate AV valves
-May have AV valve insufficiency at either level
-common with downs syndrome
-Sx of HF by 6-8 wks of life (decreased PVR)
-PHTN present due to increased pulmonary blood flow, and pulmonary vascular obstructive ds may develop early
-Murmurs vary
-Imaging:
-ECG: LAD and combined atrial enlargement/ventricular hypertrophy
-CXR: Cardiomegaly with enlargement of all chambers and presence of increased vascularity
-Echo is dx
hypertension
-start checking at 3yo
-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
syncope
-Hx:
-Age, time of day, state of hydration/nutrition, environmental conditions, activity/body position, frequency/duration of episodes, aura/prodrome/symptoms prior to episode
-Additional: Meds, hx of viral illness, medical hx (neuro disorders, TBI, neurosurgical interventions), family hx (cardiac, exertional, sudden death in children, seizures), witnesses
-NO loss of urine, NO AMS
-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: HR, QT interval, T-wave abnormalities, ventricular arrythmias, AV conduction disturbances, or Brugada syndrome (predisposes to VT)
-Tx:
-adequate intravascular volume
-Clear urine 5x daily
-Increase salt
-Leg pumping, leg crossing, and squatting with presyncopal sx
-Regular aerobic exercise
-Meds (limited studies)
iron deficiency anemia
-ID- Insufficient iron to maintain normal functions such that iron stores are reduced
-IDA- Hmg > 2 SDs below normal for age and gender, 2ndary to ID
-Normal term infants born with sufficient iron to prevent ID x 4mo
-Premature, LBW, neonatal anemia, perinatal blood loss, hemorrhage –> reduce iron stores
-Breast milk is low in iron relative to cow’s milk and fortified formulas
-Exclusively breast-fed -> 1 mg/kg/day of supplemental iron x 6mo
-Pallor, fatigue, irritability
-Hx of pica is common
-Assoc with increased lead absorption – neurotoxicity
-screening for anemia @ 12mo with hmg concentration and review of RF
-RF: Low socioeconomic status, premature/LBW, exclusively breast-fed > 4 mo w/o iron supp, lead exposure, weaning to whole milk or complementary foods low in iron, feeding/growth problems, inadequate nutrition
-Hmg < 11 = high-risk ID –> iron eval (iron saturation, ferritin, CRP, reticulocyte hemoglobin concentration)
-Tx:
-Hmg 10-11 at screening: Closely monitored/empirical tx with iron supplementation and a re-check in 1 mo
-If ID/IDA: PO iron 3 mg/kg/day x 3 mo
-supplementation -> increased reticulocyte hmg x 48 hrs; maximal between 5-7 days
-Rise in hmg of 1 or more g/dL after 1mo is a good response
-Therapy continued x 3 months to replenish stores of iron
-Parenteral iron for those with CKD and erythrocyte stimulants maybe celiac or IBD as well
idiopathic thrombocytopenic purpura (ITP)
-MC bleeding disorder of kids
-Often after viruses (rubella, varicella, measles, parvovirus, influenza, EBV, or HIV)
-Thrombocytopenia from clearance of circulating IgM/IgG-coated platelets by reticuloendothelial system
-Most pts recover spontaneously x several mo
-Acute petechiae and ecchymosis
-Epistaxis and gingival bleeding are common
-Dx Blood:
-Platelets markedly reduced (< 50,000 and often < 10,000)
-Large platelets on blood smear (accelerated new platelet production)
-WBC/diff and Hb usually normal
-PT and aPTT normal
-Bone marrow: Megakaryocyte hyperplasia with normal erythroid and myeloid cellularity
-Complications:
-Severe hemorrhage and bleeding
-RF: Platelets < 10,000/uL and mean platelet volume < 8 fL
-Tx:
-Observation in absence of bleeding, regardless of platelet count
-Avoid NSAIDs, physical activities
-Corticosteroids
-Severe acute bleeding- intravenous immunoglobulin (IVIG) is tx of choice (alt or adjunct to corticosteroids)
-Anti-Rh(D) IG: Binds to D antigen on RBCs -> spleen clears anti-D-coated RBCs versus platelets -> Only effective for Rh (+) pts
-Splenectomy:
-Considered if persists x 12mo and failure of preferred/alt 2nd-line tx
-Thrombopoietin receptor agonists (eltrombopag, romiplostim); FDA-approved for > 12mo
-Typically postponed to > 5yo if possible
-Complete response in 70%, partial in 20%
-Prognosis:
-80% remission
-Tx with combination steroid and IVIG better remission rates at 12 and 24mo than either alone
immunoglobulin A vasculitis/Henoch Schonlein Purpura (HSP)
-MC type of small vessel vasculitis in kids
-URI precedes dx in 2/3
-Antigens from GABHS, viruses, drugs, foods, and insect bites
-Small vessels of skin, GI tract, and kidneys MC affected
-Skin: Urticarial -> maculopapular -> symmetrical, palpable, purpuric rash on legs, buttocks, and elbows
-Migratory polyarthralgia or polyarthritis (ankles/knees)
-Intermittent, sharp abdominal pain (50%)
-Renal involvement in 2nd-3rd weeks with nephritic or nephrotic picture, often high BP (25-50%)
-Intussusception, hemorrhage/edema of small intestine, testicular torsion, neurologic symptoms also assoc
-Dx:
-Platelets normal/elevated,
-hemostasis and platelet function are normal
-UA with hematuria, sometimes proteinuria
-Stool with occult blood possible
-ASO titer elevated and throat culture + for group A B-hemolytic streptococci
-Serum IgA may be elevated
-Tx/Prognosis:
-Supportive, NSAIDs, corticosteroids
-If ASO titer elevated or + strep culture –> PCN
-Good prognosis: Recurrence of symptoms in 25-50%, progressive renal failure in < 5%
Acute lymphoblastic Leukemia (ALL)
-MC malignancy of kids (25% of cancer dx < 15yo)
-Peak age- 4yo; 85% dx between 2-10yo
-Trisomy 21– 10-20x increase in leukemia
-Uncontrolled proliferation of immature lymphocytes -> Unknown cause
-> 25% malignant hematopoietic cells (blasts) in bone marrow aspirate
-> 70% receiving aggressive combo chemotherapy and early pre-symptomatic tx to CNS are cured
-decreased production of RBCs, WBCs, or platelets and to leukemic infiltration of extramedullary sites
-Intermittent fevers, bruising/pallor, bone pain (pelvis, back, legs)
-PE- normal to highly abnormal
-Pallor, petechiae, purpura
-Hepatomegaly/splenomegaly (> 60%)
-Lymphadenopathy, testicular enlargement, SVC syndrome (mediastinal adenopathy), tachypnea/orthopnea/respiratory distress (mediastinal mass), CN palsies (leukemic infiltration), leukemic infiltration/hemorrhages of optic fundi, flow murmur/tachycardia (anemia)
-Labs:
-CBC w/ diff- most useful
-95% have decrease in at least one cell type (neutropenia, thrombocytopenia, or anemia)
-WBC count is low or normal (50%), with neutropenia (< 1000 uL)
-Peripheral smear: RBC abnormalities (teardrop shapes)
-Uric acid/LDH elevated as a result of cell breakdown
-Bone marrow bx: Infiltration of leukemic blasts replacing normal marrow
-Imaging:
-CXR: Mediastinal widening/mass, tracheal compression due to lymphadenopathy or thymic infiltration
-AUS: Kidney enlargement, intra-abdominal adenopathy
-Plain films of long bones/spine: Demineralization, periosteal elevation, growth arrest lines, compression of vertebral bodies
-Tx:
-Induction (1st month)– > 95% of pts exhibit remission (on BM aspirates by morphology)
-Consolidation:
-Intrathecal CTX with continued systemic therapy and sometimes cranial radiation therapy
-Several months of intensive CTX follows consolidation (intensification)
-Maintenance:
-PO, pulses of IV, Intrathecal
-Tumor lysis syndrome should be anticipated when tx is started
-Hyperkalemia, hyperuricemia, and hyperphosphotemia
-Maintaining UOP with IV fluids treating with PO allopurinol
-Hyperleukocytosis (with hyperviscosity and respiratory distress, AMS): Leukopheresis
-During tx, fever/neutropenia requires prompt assessment -> blood cultures/tx with empiric broad-spectrum abx
-Prophylaxis for Pneumocystis jirovecii (trimethoprim-sulfamethaxzole)
-Prognosis- Most important features: WBC count and age
-1-9yo w/ WBC count < 50,000– survival rate > 90% range
-Ages > 10 - ~ 88%
acute myeloid leukemia (AML)
-25% of all leukemias kids -> but 1/3 of deaths
-RF: Congenital, acquired (ionizing radiation, cytotoxic CTX agents, benzenes)
-Aggressive induction therapy results in 75-85% remission rate
-Anemia (44%), thrombocytopenia (33%), and neutropenia (69%)
-Hyperleukocytosis: Venous stasis, sludging of blasts in small vessels (hypoxia, hemorrhage, infarction)
-CNS leukemia present in 5-15% of pts at dx (higher than ALL)
hodgkin lymphoma
-Better response to tx than adults
-5-10 year survival rate of > 90%
-15% in children < 16yo (3% < 5yo)
-4:1 M:F predominance in 1st decade
-Painless cervical lymphadenopathy
-Firm, rubbery texture; not fixed to surrounding tissues; variable growth rate
-Bx: Lack of identifiable infection in region drained by the enlarged node, > 2 cm in size, supraclavicular/abnormal CXR, lymphadenopathy increasing in size after 2 wks or failing to resolve within 4-8 wks
-Constitutional symptoms (33%): Fever, wt loss of 10% in prior 6mo, drenching night sweats (Ann Arbor staging)
-Asymptomatic mediastinal disease (adenopathy, mass) in 50%: Sx with compression of vital structures
-Labs: CBC usually normal (although anemia, neutrophilia, eosinophilia, and thrombocytosis may be present), ESR and other acute phase reactants often elevated
-Staging:
-Ann Arbor classification (determines tx and prognosis)
-CT of neck, chest, abdomen, and pelvis, as well as PET scan (with or w/o bone marrow bx)
-Pathologic Findings:
-Reed-Sternberg cell (germinal-center B cells with malignant transformation)
-20% tumors in developed countries are + for EBV
-Tx/Prognosis:
-Based on stage, presence of B symptoms, tumor bulk, and # of involved nodal regions
-Chemotherapy and/or immunotherapy alone – less often by radiation therapy
-5-year survival rate- 90-95% in Stages I and II (slightly lower in Stages III and IV)
-2/3rds of all relapses within 2 years after dx, relapse rarely beyond 4 years
non-hodgkin lymphoma
-5-10% of malignancies in < 15yo
-Incidence increases with age
-3:1 male predominance
-in Africa ~50% due to EBV and assoc w/ Burkitt lymphoma
-congenital or acquired immune deficiencies -> 100-10K x greater risk
-rapidly proliferating, high-grade, diffuse malignancies -> very responsive to tx though
-Arise at any site of lymphoid tissue as well as extra-lymphatic sites (bone, bone marrow, CNS, skin, and testes)
-S&S determined by location of lesions and degree of dissemination
-Dx:
-Bx of involved tissue with histology immunophenotyping, and cytogenetic studies
-CBC, LFTs, and biochemical profile -> Elevated LDH reflects tumor burden
-CXR, CT scan- neck/abdomen/pelvis, PET scan
-Bone marrow and CSF exams
-Tx/Prognosis
-Management of life-threatening problems at presentation is critical -> Acute TLS, SVC syndrome, airway compromise, cardiac tamponade
-Systemic CTX
-Intensive intrathecal CTX for CNS prophylaxis x 3-9mo
-LL: Tx protocols for ALL x 2 years
-BL/BLL, LCBL, ALCL: Alkylating agents/methotrexate
-Prognosis based on extent of ds at dx
-90% with localized disease – long-term, disease-free survival
-Disease on both sides of diaphragm, CNS involvement, or BM involvement – 70-80% failure-free survival rate
-Relapses early – may have chance for cure with HSCT
brain tumors
-MC solid tumors of childhood
-misdx or dx late
-Infratentorial tumors (<2):
-Sx: Vomiting, unsteadiness, lethargy, irritability
-Signs: Macrocephaly, ataxia, hyperreflexia, cranial nerve palsies
-Supratentorial tumors (older kids):
-Headache, visual symptoms, seizures, and focal neurologic deficits (personality changes)
-Cerebellar/posterior fossa tumors: Morning vomiting
-Brainstem tumors: Facial and extraocular muscle palsies, ataxia, hemiparesis, hydrocephalus
-Dx:
-MRI- study of choice (with and w/o contrast)
-Imaging of entire neuraxis and CSF cytologic exam for medulloblastoma, ependymoma, and pineal region tumors
-MRI of spine for midline tumors of 4th ventricle/cerebellum
-Lumbar CSF»_space;» ventricular CSF
-Biomarkers (hcg and a-fetoprotein) helpful in dx and f/u
-2 categories (based on cell origin):
-1. Glial tumors: Astrocytomas, ependymomas
-2. Embryonal tumors: Medulloblastomas, atypical teratoid/rhabdoid (AT/RT) tumors
-Astrocytoma !!!
-MC brain tumor of childhood
-Low-grade may be curable by complete surgical excision alone
-CTX effective alone in 40-50% of low-grade cases (multiple courses)
-Tx:
-Supportive care: Dexamethasone, anti-convulsants (levetiracetam)
-Specific therapy
-Maximal safe surgical resection is preferred initial approach
-Radiation use varies
-CTX effective in tx low-grade and malignant astrocytomas and medulloblastomas
-Older children with malignant gliomas: Combination of above
-Prognosis:
-Low-grade astrocytomas: 5 and 10-year survival rates 60-90%
-Low-stage medulloblastoma: 10-year survival rate ~ 40-60%
-High-risk: 5-year survival rate ~ 25-40%
-Glioblastoma: Cure rates poor (survival rates < 10%)
