Disease States Flashcards

1
Q

Achondroplasia

A
  • airway: craniofacial and spinal abnormalities, limited neck extension, large tongue, large mandible, A-A instability
  • kyphosis/scoliosis/spinal stenosis: difficult and unpredictable epidurals and spinals
  • comorbidities: OSA, obesity
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2
Q

Acromegaly

A
  • GH hypersecretion: usually from GH secreting pituitary adenoma, may have panhypopituitarism (need hydrocortisone and thyroxine)
  • potentially difficult airway/mask: gigantism and facial bone hypertrophy, large tongue, mucosal hypertrophy, prominent jaw, OSA
  • comorbidities: OSA, HTN, cardiac arrhythmias, diastolic dysfunction, CAD, glucose intolerance, renal failure
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3
Q

Acute Porphyrias

A
  • group of inherited enzymatic defects of heme synthesis
  • overproduction of heme precursors and intermittent symptomatic attacks
  • multisystem manifestations: neuro, renal and CV
  • S&S: abdominal pain, N/V, autonomic and peripheral neuropathies, electrolyte disturbances, hypovolemia, bulbar dysfunction, respiratory failure, mental status changes, seizures, coma
  • drug triggers: barbiturates, Etomidate, ketorolac, metoclopramide, steroids
  • nondrug triggers: dehydration, fasting, stress, infection
  • Preop
  • severity and susceptibility of disease, precipitating factors, treatments, and current symptomatology
  • ensure adequate hydration, glucose, and anxiolytics
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4
Q

Adrenal Insufficiency

A
  • primary (cortisol/aldosterone) versus secondary (ACTH)
  • labs: hyponatremia, hyperkalemia, hypoglycemia
  • Addisonian crisis S&S (life threatening!): hypotension, dehydration, circulatory collapse, vomiting/diarrhea, hypoglycemia, acidosis, hyponatremia, hyperkalemia, abdominal pain, loss of consciousness
  • Addisonian crisis rx: hydrocortisone 100 mg q6h
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5
Q

Adrenocortical Excess (Cushing Syndrome)

A
  • source of corticosteroid: pituitary (Cushing disease), adrenal, paraneoplastic, or exogenous
  • S&S: HTN, increased ICP, LVH, OSA
  • labs: hypokalemia, hypocalcemia, increased hgb, hyperglycemia
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6
Q

Alcoholism

A
  • multisystem disease: neuro (peripheral neuropathy, Wernicke-Korsakoff), CV (tachycardia, HTN, cardiomyopathy), respiratory (pneumonia, smoking), GI (reflux, gastritis), liver (fatty, hepatitis, cirrhosis), pancreatitis, heme (pancytopenia)
  • withdrawal: BZs (diazepam) and thiamine
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7
Q

Amyotrophic Lateral Sclerosis (ALS)

A
  • aspiration risk (bulbar palsy)
  • altered responses to NMBDs: hyperkalemia w/sux, prolonged response to NDMR
  • increased risk for postop PPV
  • potential autonomic dysfunction
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8
Q

Anterior Mediastinal Mass

A
  • 4 T’s: thymus, thyroid, terrible lymphoma, teratoma and germ cell tumors
  • lymphoma most common
  • other options for dx: CT guided needle biopsy, awake anterior mediastinoscopy w/LA
  • consider empiric chemo/rad/steroids to decrease size of mass
  • may require ECMO or CPB (severe positional symptoms)
  • H&P: chest pain, dyspnea, orthopnea and position changes, syncope
  • SVC syndrome: stridor, cyanosis, venous engorgement of neck, edema of head and neck
  • labs/imaging: CBC, ECG, CT, echo, +/- flow-vol loops (intra-thoracic obstruction)
  • MAINTAIN SPONTANEOUS VENTILATION
  • Airway and/or vascular compression
    1. if possible, awake patient
    2. reposition (determine preoperatively what relieves compression)
    3. rigid branch and ventilation distal to obstruction
    4. sternotomy and surgical elevation of mass off compressed airway and/or vessels
  • Avoid muscle relaxants until manual ventilation ensures ability to give PPV
  • > 50% tracheobronchial compression precludes safe GETA
  • Flow volume loops: risk of airway collapse during induction supposed to correlate with increase in mid-expiatory phase plateau when going from upright to supine, but NOT borne out with studies
  • Fixed obstruction (intra or extra thoracic): limited during inspiration and expiration (hamburger)

***Flow volume loops not mandatory: CT combined with H&P observing patient in sitting, supine, and possibly prone positions gives all the required information

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9
Q

Aortic Dissection

A
  • intimal tear in aorta
  • Stanford types: A begins in ascending, B begins in descending
  • DeBakey types: 1 involves whole aorta, 2 is just ascending, 3 is just descending
  • Type A requires surgery (+/- AV and coronaries), risk of stroke
  • Type B may be treated medically, endovascular; risk of spinal cord ischemia
  • Risks: atherosclerosis, HTN, Ehlers-Danlos, Marfan
  • strict control of BP (SBP <120 mmHg)
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10
Q

Burns

A
  • Body surface area “rule of 9’s”: head 9%, arms 9% each, legs 18% each, chest 18%, back 18% (head is 18% for children)
  • Parkland formula: 4 cc/kg/%BSA over 24 hours (half in first 8 hrs, half in next 16 hours)
  • avoid sux after 24 hours
  • consider CO poisoning and hyperbaric O2 therapy
  • concerns: airway, IV access, fluid resuscitation and UO, hypothermia, infection, compartment syndrome, blood product needs

CV changes

  • First 48 hours: CO decreased due to circulating myocardial depressants, increased SVR, contracted plasma volume (increased capillary permeability and third spacing), decreased response to catecholamines
  • After 48 hours: capillary integrity returns (if adequate fluid resuscitation), interstitial fluid reabsorption, increased metabolic demands, and increased circulating catecholamines lead to a hyper dynamic state (increased CO and decreased SVR from circulating inflammatory mediators)

*airway edema (3rd spacing, inhalation injury), hypovolemia/shock (increased vascular permeability, edema, 3rd spacing, extravasation of proteins, fluid shifts to interstitial compartments), hyperkalemia (tissue destruction), myocardial depression (circulating depressants from tissue injury), diminished response to catecholamines

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11
Q

Carcinoid

A
  • tumors of GI tract (also bronchi/lungs)
  • contain a variety of hormones: serotonin, histamine, substance P, PGs, kallikrein, etc
  • carcinoid syndrome: systemic release of serotonin, histamine, etc. (usually from liver mets or non-GI tumors)
  • ->flushing, edema, diarrhea, bronchoconstriction–>can progress to TR/PR and PS leading to RV failure
  • carcinoid crisis: d/t handing of tumor during surgery–>bronchoconstriction, hypotension (sometimes HTN), hyperglycemia
  • tx: octreotide (somatostatin analogue), antihistamines
  • caution: ketamine, morphine, catecholamines (epi), mivacurium, atracurium
  • caution: sux (risk of mediator release–>contractions/histamine)
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12
Q

Cardiac Tamponade

A
  • obstructive shock: increased intrapericardial pressures leads to cardiac compression and obstructive shock (decreased CO/BP)
  • compensation: tachycardia, increased SVR, increased contractility (sympathetic activation)
  • pulsus paradoxus: inspiratory fall in SBP >10 mmHg
  • Kussmaul sign: JVP distends with inspiration
  • ECG: may have electrical alternans (alteration of QRS axis or amplitude between beats)
  • Echo: compressed chambers, diastolic collapse of RA/RV
  • Avoid PPV: decreased venous return–>decreased CO
  • Induction with ketamine or etomidate
  • Spontaneous ventilation until tamponade relieved
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13
Q

Cerebral Aneurysm

A
  • SAH complications: rebreeding, mass effect, brain edema, seizures, vasospasm, cardiac arrhythmias, neurogenic pulmonary edema
  • Triple H treatment for vasospasm: HTN, hypervolemia, and hemodilution (also early angioplasty, nimodipine/nicardipine)
  • Brain protection: maintain CPP >60 mmHg; normocapnia; avoid hyperglycemia/acidosis/hypoxia; mild hypothermia (32-34 degrees); seizure prophylaxis
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14
Q

Chronic Renal Failure

A
  • comorbidities common: CAD, HTN, DM
  • considerations: volume overload, electrolytes abnormalities (hyperK, hyperMag, hypoCa), uremia, pharmacokinetic changes due to decreased elimination and decreased albumin
  • uremia: encephalopathy, autonomic/peripheral neuropathy, pleural/pericardial effusions, anemia, platelet dysfunction
  • Cr>2.0 independent risk factor for cardiac complications
  • avoid really excreted drugs: morphine, meperidine, pancuronium
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15
Q

Cystic Fibrosis

A
  • genetic mutation for protein (CF transmembrane regulator) found on most exocrine glands
  • changes in pulmonary, GI, GU, sweat glands
  • resp: obstructive disease, viscous mucous secretions w/reduced mucociliary clearance leading to airway inflammation and chronic infection and chronic hypoxia/hypercarbia (PAH/cor pulmonale)
  • pancreas: protein/fat malabsorption (vit K), DM
  • hepatobiliary: fatty liver, cirrhosis, portal HTN
  • labs: CBC, lytes, LFTS, coags, glucose
  • other: CXR, echo?, PFTs
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16
Q

Down Syndrome

A
  • airway/pulmonary: AAI, mid-face hypoplasia, macroglossia, subglottic stenosis, OSA, PAH
  • CV: ASD, VSD, endocardial cushion defects, tetralogy of Fallot
  • GI: higher incidence of GERD, TEF
  • Endocrine: hypothyroidism
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17
Q

Duchenne Muscular Dystrophy

A
  • most common childhood muscular dystrophy
  • affects skeletal, cardiac, and smooth muscle
  • death due to respiratory failure
  • cardiac (cardiomegaly, cardiomyopathy), resp (PNA, aspiration, failure), and GI (gastroparesis, smooth m.)
  • kyphoscoliosis can lead to pulmonary hypoplasia, RLD, and V/Q mismatching–>hypoxia, hypoxic pulmonary vasoconstriction, and hypercapnia lead to pulmonary HTN and RV failure
  • anesthetic risk: rhabdomyolysis and hyperkalemia (triggered by inhalation agents and succinylcholine)
  • NOT at elevated risk of MH (but makes ddx challenging)
  • MH (hyper metabolism): increased CO2, metabolic acidosis, muscle rigidity, elevated temp >38.8
  • trigger free anesthetic (no volatiles or sux)–>no inhalation induction
  • NDMRs: more sensitive and longer duration
  • rhabdo/hyperK tx: calcium chloride, bicarb, insulin/dextrose, hyperventilation, IV hydration, mannitol to promote diuresis
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18
Q

Eisenmenger Syndrome

A

Goals

  • maintain SVR and avoid decreases in SVR (increased R–>shunt)
  • avoid increases in PVR: avoid dehydration, pain, acidosis, hypoxia, hypercarbia, hypothermia, high PEEP, increased intrathoracic pressure
  • CHF mgmt: digoxin, diuretics
  • avoid air bubbles: paradoxical air embolus
  • pulmonary vasodilator: prostacyclin, epoprostenol, bosentan, or sildenafil; inhaled NO may be useful intro
  • IV induction preferred over inhalation if CHF
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19
Q

Epiglottitis and Croup

A
  • epiglottitis: minimal cough, sudden stridor, “toxic” appearance, dysphagia, drooling
  • epiglottitis: associated w/H. influenza
  • croup: barky, unproductive cough, hoarseness, gradual stridor, mild fever, minimal dysphagia, no drooling; often improves w/nebulized epinephrine and steroids
  • croup: associated with parainfluenza viruses
  • ddx: FB aspiration, tracheitis, tonsillitis, retropharyngeal abscesses, vascular rings, allergic reaction, laryngeal diphtheria
  • imaging: epiglottitis w/”thumb sign”, croup w/”steeple sign”
  • tx: steroids, nebulized epi, heliox, broad spectrum antibiotics for epiglottitis
  • airway: ETT w/inhalation induction, surgical backup for trach
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20
Q

Geriatric Patient

A

Age-related changes

  • CV: LVH, diastolic dysfunction, blunted HR response, atherosclerosis, wide PP,
  • Resp: increased dead space, increased V/Q mismatch, FRC increases but closing capacity also increases and approaches FRC, reduced response to hypoxia/hypercapnia, chest wall stiffness, muscle atrophy
  • Renal: reduced RBF/GFR
  • GI: decreased GI motility, GERD

-POCD risks: preop cognitive impairment, opioids/BZs, infection, hyponatremia

21
Q

Hemophilia

A

Hemophilia A

  • factor VIII deficiency
  • normal PT, normal bleeding time, prolonged PTT
  • tx: recombinant factor VIII (older treatments- FFP, cryo, DDAVP)

Hemophilia B

  • factor IX deficiency
  • normal PT, normal bleeding time, prolonged PTT
  • tx: recombinant factor IX

*regional usually avoided, requires factor replacement and monitoring of factor levels

22
Q

HIV

A
  • HIV virus infects helper T cells
  • multisystem dz: airway (dysphagia, infections), neuro (dementia, neuropathy), CV (ACS, dilated CM, pericardial effusion), GI (diarrhea, fatty liver, pancreatitis), renal (CKD), heme (pancytopenia), endocrine (DM, thyroid)
  • strict aseptic technique
  • caution: antiretrovirals may prolong effect of fentanyl and midazolam (cytochrome p450 inhibition)
  • regional: concern w/infection, CNS infection/neoplasm, and coagulopathy
23
Q

Hyperthyroidism

A
  • thyroid storm: tachycardia, fever, altered mental status
  • enlarged thyroid/goiter: stridor, airway obstruction
  • CV: a fib, CHF
  • propranolol preferred beta blocker: non-selective beta 1 and beta 2 blocker, may also inhibit peripheral T4-T3 conversion
  • Thyroid storm treatment
  • BBs for tachycardia
  • IVFs and electrolyte correction
  • PTU, sodium iodide, and hydrocortisone (reduces release of thyroid hormone)
24
Q

Hypothyroidism

A
  • myxedema coma: coma, seizure, CHF, respiratory failure, hypothermia, hyponatremia, hypoglycemia, ileus, adrenal insufficiency, and coagulopathy
  • airway: may require AFOI
25
Q

Liver Cirrhosis

A
  • Child-Pugh: albumin, PT, bilirubin, ascites, encephalopathy (avoid elective surgery in class C)
  • MELD: bilirubin, creatinine, INR

Multisystem disease

  • neuro: encephalopathy
  • CV: increased CO, decreased SVR
  • pulmonary: hepatopulmonary syndrome (shunting), portopulmonary hypertension
  • GI: varices, ascites, malnutrition
  • renal: renal failure (prerenal, hepatorenal)
  • heme: coagulopathy, anemia
  • metabolic: hyponatremia

*hepatopulmonary syndrome: orthodeoxia and platypnea (desaturation and dyspnea when upright vs supine)

Goals

  • avoid further decrease in hepatic blood flow: hypotension, hypoxemia, hemorrhage
  • maintain volume (albumin) and electrolytes (hyponatremia)
  • reverse coagulopathy: FFP, plts, cryo
  • manage ascites: diuretics, paracentesis
  • avoid renal failure: maintain preload, avoid NSAIDs
  • encephalopathy: caution w/sedatives/analgesics, lactulose as required (reduces ammonia absorption)

*cisatracurium and fentanyl avoids liver metabolism

26
Q

Autonomic Hyperreflexia

A
  • syndrome of widespread reflex sympathetic discharge in response to stimuli below spinal cord lesion
  • usually at or above T7 level
  • descending inhibitory signals from supraspinal centers unable to module afferent input from below lesion
  • sympathetic stimulation below lesion and a reflex arc leading to massive sympathetic efferent discharge below lesion (HTN, vasoconstriction, pallor, spasticity)
  • corrective reflexes (parasympathetic) above lesion (bradycardia, flushing of face/neck, headache, mucous membrane and conjunctival congestion, nasal stuffiness, sweating, miosis)
27
Q

OSA

A

STOP BANG

  • S: snore
  • T: tired
  • O: observed apnea
  • P: high blood Pressure
  • B: BMI >35
  • A: age >50
  • N: neck circumference >40 cm
  • G: gender male

<3 RFs = low risk OSA
3 RFs = high risk OSA
5-8 RFs = likely mod-severe OSA

28
Q

MS

A
  • axonal inflammation and demyelination of CNS (not peripheral)
  • triggers: infection, stress, trauma, hyperthermia
  • potential respiratory impairment
  • neuraxial anesthesia implicated in postop relapse (spinal»>epidural)
  • PNBs okay, epidurals probably okay (stress can worsen MS)
  • avoid sux if motor nerve involvement (spasticity, flaccidity, hyperreflexia)
  • unpredictable effect of NDMR: resistance in some (up regulation of Each receptor) and sensitivity in others (wasting, weakness)
  • if using epidural, use low concentration local (eg. 0.125% bupivicaine) since high doses may exacerbate MS
29
Q

Myasthenia Gravis

A
  • autoimmune disease targeting post junctional acetylcholine receptors
  • potential respiratory and bulbar involvement
  • sensitive to NDMRs, resistant to sux
  • thymoma and anterior mediastinal mass
  • potential for myasthenia crisis or cholinergic crisis, both of which can cause respiratory failure
  • plasmapheresis preop if severe weakness, effects can last weeks
  • regional anesthesia when possible

*RFs for post-op mechanical ventilation: pyrido >750 mg/day, duration >6 years, VC <2.9 L, coexisting COPD, upper abdominal surgery

  • avoid giving more cholinesterase inhibitor than usual daily dose–>may precipitate cholinergic crisis
  • 1 mg IV neostigmine = 120 mg PO pyridostigmine
30
Q

Myasthenic (Eaton-Lambert) Syndrome

A
  • autoimmune disease targeting presynaptic voltage-gated calcium channels –> decrease acetylcholine release
  • associated with lung carcinoma
  • extremely sensitive to both sux and NDMRs
  • autonomic dynsfunction in 30%
  • proximal lower limb weakness that improves with exercise
  • no improvement with neostigmine
  • IVIG pre-op may help
  • regional good option
  • Patients extremely sensitive to both NDMRs and sux
  • Reversal of muscle relaxation is less effective, especially in patients treated with 3,4-diaminopyridine (works on potassium channels to increase action potential duration)
31
Q

Pheochromocytoma

A
  • associated w/MEN, NFM, and VHL disease (CNS hemangiomas)
  • dx: free NE in 24 hour urine (most sensitive) +/- urine VMA
  • some believe free metanephrines in plasma is most accurate
  • alpha 1 blockade: doxazosin po 10-14 days
  • may need beta blockade, but only after alpha blockade
  • most secrete NE (some secrete epi)
  • in case of epinephrine secreting tumors, tachycardia may accompany HTN–>labetalol drug of choice
  • **metanephrine = epi metabolite
  • **VMA = norepi metabolite
32
Q

Preeclampsia

A

Multisystem Disease: endothelial dysfunction

  • CV: HTN, CHF, volume contraction
  • Pulm: pulmonary edema
  • CNS: ICH, cerebral edema, headache, seizures
  • GU: oliguria/anuria
  • Heme: thrombocytopenia, hemolysis, DIC
  • Airway: increase pharyngeal/laryngeal edema and friability
  • Uteroplacental insufficiency

Dx:

  • BP >140/90 at 20 weeks or later
  • 300 mg or more of protein in 24 hour urine

Severe Features:

  • BP >160/110, 24 hour urine protein >5 g
  • multiorgan involvement: pulmonary edema, seizures, oliguria (<500 ml/d), plts <100,000, abnormal liver enzymes, epigastric/RUQ pain, CNS sxs (headaches, blurred vision, AMS)

*Consider epidural over spinal for c/s- higher risk of cerebral edema and pulmonary edema with volume loading in these patients (single shot spinal harder to manage sympathectomy without aggressive volume loading)

33
Q

Premature (LBW) Infant

A

Considerations: impaired temp regulation, respiratory distress and bronchopulmonary dysplasia, postop apnea, persistent fetal circulation (PDA, PFO) w/L-to-R shunt/RVH/CHF, retinopathy of prematurity, IVH, increased risk of NEC/sepsis

34
Q

Progressive Systemic Sclerosis (Scleroderma)

A

Considerations

  • skin: difficult IVs, decreased mouth opening
  • CV: cardiac fibrosis (CHF), conduction defects, CAD
  • pulm: PAH, ILD
  • GI: esophageal dysmotility (aspiration risk)
  • CNS: neuropathy
  • renal: renal artery fibrosis (CKD)
35
Q

Hypertension

A

Causes
-essential, CKD, renovascular disease, steroids/Cushing’s, OSA, drugs (cocaine, amphetamine, OCP), EtOH abuse, obesity/metabolic syndrome, thyroid/parathyroid disease, pheo, coarctation

Signs of end-organ ischemia
-LVH, angina, MI, CHF, CAD, CVA, TIA, CKD, retinopathy, PVD

36
Q

Pulmonary Hypertension

A

Goals

  • avoid increased PVR (N20, hypoxia, hypercarbia, acidosis, hypothermia, pain)
  • avoid high airway pressures, PEEP, alpha agonists
  • avoid decreased RV preload
  • maintain RV contractility (early inotropic support)
  • main LV after load (care w/neuraxial)
37
Q

Pulmonic Stenosis

A
  • avoid increased RV O2 consumption (high HR, contractility)
  • maintain preload (right CO fixed)
  • maint sinus rhythm
  • caution w/neuraxial (decreased preload–>decreased CO)
38
Q

Pituitary Adenoma

A
  • prolactinoma most common functional tumor (amenorrhea, galactorrhea, infertility)
  • larger, nonfunctioning tumors cause mass/compression symptoms (N/V, headache, visual disturbance, rhinorrhea, panhypopituitarism, hydrocephalus)
  • other tumors: ACTH (Cushing’s), GH (acromegaly, HTN, insulin resistance, skeletal/soft tissue overgrowth), TSH (hyperthyroidism)
  • anterior pituitary (6 tropic hormones): ACTH, prolactin, GH, TSH, FSH, LH
  • posterior pituitary (2 hormones- synthesized in hypothalamus and then transported): ADH, oxytocin
  • bromocriptine: dopamine agonist (inhibits secretion of GH and prolactin)
  • octreotide: somatostatin analog (used to treat acromegaly and reduce GH secretion)

*transphenoidal approach: close proximity to cavernous sinus and internal carotid artery, increasing risk of massive hemorrhage and hemodynamic instability

39
Q

Pyloric Stenosis

A

Considerations

  • hypochloremic metabolic alkalosis
  • hyponatremia, hypokalemia
  • hypovolemia (cap refill, skin turgor, mucous membranes, fontanelle)
  • tendency to postoperative depression of ventilation

*modified RSI (true RSI w/apnea not tolerated in this population)

Pre-op Optimization

  • NS
  • Endpoints: Cl > 90, HCO3 < 30
40
Q

Rheumatoid Arthritis

A

Considerations

  • airway: TMJ, AA sublaxation
  • CV: conduction abnormalities, myocarditis/pericarditis, aortic insufficiency
  • Resp: pleural effusions, pulmonary fibrosis, RLD
  • CNS: peripheral neuropathy
  • GI: hepatosplenomegaly
  • Drugs: steroids, NSAIDs, DMARDs (methotrexate[hepatotoxicity], sulfasalazine), and immunosuppressives
41
Q

Sarcoidosis

A

Multisystem granulomatous disease with:

  1. Airway: laryngeal, nasal, mucosal sarcoidosis
  2. CV: heart block, pericardial disease, restrictive cardiomyopathy
  3. Pulmonary: RLD, alveolar fibrosis, decreased DLCO
  4. CNS: encephalopathy, seizures
42
Q

Scoliosis

A
  • potential respiratory involvement and need for postop PPV
  • potential pulmonary HTN and right heart failure
  • difficult airway?
  • difficult regional?
  • associated with neuromuscular disease and connective tissue disorders (CP, muscular dystrophy, Marfan)
43
Q

SLE

A

Considerations

  • CV: conduction abnormalities, CAD, pericarditis/effusion, noninfective vegetations
  • Pulm: RLD, pulmonary HTN, pleuritis
  • Renal: lupus nephritis
  • CNS: seizures
  • Heme: decreased hgb, WBC, plts
  • antiphospholipid antibody–>arterial and venous thrombosis (MI, PE, fetal loss)
  • Meds: steroids, NSAIDs, heparin, cyclophosphamide
44
Q

Tetralogy of Fallot

A

Most common congenital heart lesion with R-to-L shunt

  1. VSD
  2. Overriding aorta
  3. Pulmonic stenosis
  4. RVH

Blalock-Taussig shunt: subclavian artery to PA

Pathophysiology depends on:

  1. Degree of RVOT obstruction
    - increased RVOT obstruction–>increased shunting via VSD
  2. Degree of SVR relative to RVOT obstruction
    - decreased SVR–>increased shunting via VSD

“Tet Spell”: acute hypoxemia and acidemia and/or syncope (from increased shunting)

  • Treatment = reduce shunt
  • Increase SVR: phenylephrine, knee-chest position
  • Decrease PVR: oxygen, decrease CO2
45
Q

Von Willebrand Disease

A

vWF: synthesized by endothelium and platelets

  • adhesion protein for platelets to injured vessel wall
  • forms a complex w/factor VIII in plasma
  • Deficiency affects both platelet plug formation and fibrin formation
  • *Oozing and prolonged bleeding after surgery (typical of coagulopathies) and mucosal hemorrhages (typical of platelet disorders)

Type 1: vWF/Factor VIII at 5-30% normal levels
Type 2: qualitative defect of vWF
Type 3: low or undetectable vWF (severe symptoms, like spontaneous hemorrhage)

Dx: assay for vWF (unreliable with PT/PTT or bleeding time)

Tx:

  • desmopressin increases levels 2x-4x by endogenous release of vWF and FVIII into plasma
  • desmopressin doesn’t work in type 2 (defective vWF)
  • vWF/FVIII concentrates
46
Q

WPW

A
  • congenital pre-excitation syndromes w/tendency to RVR and VF w/a fib due to accessory pathway conduction
  • avoid techniques and drugs that increase HR
  • careful w/desflurane, ketamine
  • in setting of a fib–>avoid agents that increase AV node refractory period (BBs, CCBs, digoxin, adenosine)

dx: EKG- shortened PR, wide QRS w/ delta wave

tx: amiodarone, procainamide
* synchronized cardio version treatment of choice for any question of instability

47
Q

Methemoglobinemia

A

<30% = no hypoxia
30-50% = signs and symptoms of hypoxia
>50% = coma and death
Treatment: methylene blue

-Ferric (Fe3+) form of iron in hgb–>increased affinity for oxygen–>left shift of oxy-hgb DC–>reduced tissue offloading of oxygen

S&S
>10%: SOB, cyanosis, AMS, headache, exercise intolerance, dizziness
>50%: seizures, coma, and death (>70%)
*Cyanosis and low SpO2 in the presence of a normal arterial PO2 by ABG
*Classic SpO2 85%
*Presence of “chocolate, dark-red, brownish to blue “ colored arterial blood (color does not change with addition of O2) and brown urine

Causes
-LAs (benzocaine, prilocaine, lidocaine), antibiotics (TMP-SMX), nitrates

Treatment

  • methylene blue (reduces hgb to normal Fe2+ state)
  • contraindicated in patients with G6PD deficiency (can cause hemolysis)
  • hyperbaric O2 and exchange transfusions can also be utilized
48
Q

Congenital Diaphragmatic Hernia

A

Pathophysiology

  • herniation of abdominal contents into the thorax–>pulmonary hypoplasia–>decreased alveoli, decreased surfactant, abnormal pulmonary vasculature–>impaired gas exchange, intrapulmonary shunting, pulmonary HTN
  • pulmonary HTN impairs transition to fetal circulation- resultant extra pulmonary shunting through PFO and PDA leads to worsening hypoxia, hypercarbia, and acidosis which in turn exacerbates pulmonary HTN (a vicious cycle)

A-a gradient

  • normal 10 mmHg (young, adult, non-smoker)
  • caused in CDH by intra and extra pulmonary shunting
  • A-a >500 predicts non-survival

Treatment

  • surgery delayed until neonate medically stable
  • goal is to reduce pulmonary HTN causing the R-to-L shunt through PFO and PDA
  • avoid mask ventilation (distends intrathoracic viscera and worsens repertory function) and excessive suctioning (hypoxia)
  • IV access, supplemental oxygen
  • intubate (awake or RSI)
  • NG/OG for stomach decompression
  • ventilator- resolve hypoxia, hypercarbia, and acidosis (all of which exacerbate PAH)
  • sedation w/opioids and BZs to minimize release of catecholamines (increased PVR)
  • muscle relaxant to reduce oxygen consumption
  • correct hypothermia, acidosis
  • order ECHO to assess congenital heart defects and right heart dysfunction
  • If all these fails, pulmonary vasodilators like nitric oxide or milrinone, surfactant administration, or high-frequency oscillatory ventilation
  • Last resort: ligation of PDA to reduce shunting or ECMO

***Maintain oxygen saturation of about 87-94% and a PaO2 of 50-80 mmHg

Intra-op
-cautious as surgeon puts abdominal contents from thorax into abdomen–>compression of IVC and/or aorta–>hypoxia and hypotension

Disposition

  • keep intubated
  • controlled ventilation reduces oxygen requirements
  • helps to prevent and treat hypoxemia, hypercarbia, and acidosis, all of which can induce or exacerbate pulmonary HTN (rebound pHTN common after this surgery)