EM Conference Pearls (9/20)

Pediatric congenital heart disease

  • Congenital HD: Two types: Neonates with ductal dependent lesions and infants (2-6months) presents with CHF
  • Cyanosis presentation: When ductal-dependent lesion is required for pulmonary blood flow (Will not respond to oxygen)
  • Shock presentation: When ductal-dependent lesion is required for systemic blood flow (appear septic and not response to fluids, may get worse with fluids)
  • Hypoxic/cyanotic or shocky/acidotic baby treatment = Prostaglandin E1 (PGE1) and transfer to facility with pediatric cardiovascular surgeon.
  • PGE1 treatment may cause apnea (monitor closely and consider intubation)
  • CHF in infants = wheezing, retractions, tachypnea, sweating/crying, difficulty feeding

EBM in the ED

  • EBM = What the evidence shows in the literature + What the physician wants for the patient + what the patient wants for themselves
  • Just like we need to practice intubation, central lines –> Learning to read and interpret literature is a skill that needs to be practiced.

Aortic Dissection

  • AD: Chest pain plus disease (ex: CP + Neurodeficit)
  • If you find your self giving large amounts of strong pain meds (narcotics) while treating what is seemingly ACS…STOP..think about AD or alternative diagnosis of chest pain
  • The 3 important questions, aortic dissection is the subarachnoid hemorrhage of the torso, migrating pain, colicky pain + opioids = badness and pain that comes and goes can still be a dissection.
  • Treatment: Treat pain, HR, BP
  • Pain: Fentanyl 25 – 50 mcg bolus
  • HR: Goal of 60 bbpm
  • Esmolol 0.5 mg/kg bolus then 50 – 300 mcg/kg/min or
  • Labetalol 10 – 20 mg bolus then 0.5-2 mg/minor
  • BP control: Goal SBP =110
  • Nitroprusside 0.25 – 0.5 mcg/kg/min then titrate (CN toxicity)
  • Nicardipine 5 mg/hr
  • Warning: Giving a vasodilator without concomitant reduction in ionotropy may cause progression of dissection. Start BB first before vasodilation meds.

Case of the Week COW #7

CC: Numbness and palpitations

HPI: 21-year-old Female presents to the Emergency Department (ED) complaining of palpitations and left arm weakness with perioral numbness, which began just prior to arrival. The patient states the episode lasted 30 minutes before completely resolving on its own. In the ED, she denies any other complaints except for a mild headache. Patient notes she experienced a similar episode of palpitations yesterday afternoon, while resting, which she described as “skipping beats”. On further questioning, the patient admitted to being hospitalized to a NYC hospital 2 weeks ago where she had a Cardiac Echocardiogram done which showed “hypertrophy.” Patient never followed up with cardiologist as instructed. In the past, a doctor in her country prescribed her an unknown antihypertensive medication, which she took for one year but stopped taking it once she moved to NJ. Denies fever, dizziness, chest pain, and shortness of breath, recent travels, calf pain or swelling.

Physical Exam:

BP 109/72   HR 82     RR 18     SpO2 100% on RA    Temp 97.0F

General: Well appearing female, in non-acute distress

HEENT: NCAT, pupils PERRLA, neck supple

Respiratory: CTA B/L, no wheezing, rales or rhonchi

Cardiac: +S1/S2, no MRG, regular rhythm

Abdomen: soft NT ND

Neuro exam: AAO X 3, No focal deficits

Extremities: no edema, no tenderness or swelling, 5/5 strength in all extremities. Sensory intact

Skin: pink and warm, No diaphoresis, no rashes, lacerations, or abrasions

Pertinent Labs: Troponin 0.308

Pertinent Imaging and other tests:

  • Chest X-Ray: Cardiomegaly with a boot shaped heart, which may indicated right heart failure.
  • CT Head: Normal 
  •  ECG: Normal Sinus Rhythm, Bi-atrial enlargement, RBBB, LVH

Working Diagnosis: Hypertrophic Cardiomyopathy (HCOM)

ED/Hospital course:  Patient was given 324 mg of ASA and admitted to Telemetry with a diagnosis of Hypertrophic Cardiomyopathy. While still in the ED waiting for a bed on Telemetry the patient had multiple runs of non-sustained V-Tach and Cardiology was consulted. The patient was started on ASA and Metoprolol PO. A 2D ECHO was done which was consistent with HCOM. Patient remained stable on Telemetry for 3 days prior to discharge. The patient’s Troponin was trended daily, 0.308 in the ED, 0.288 on day 1 of admission, and 0.314 on day 2 of admission. Patient was told to follow up with Cardiology Clinic for possible AICD placement planning.

Pearls:

Hypertrophic cardiomyopathy (HCM) is one of the most common inherited cardiac disorders (affecting ~ 1 in 500 people) and is the number one cause of sudden cardiac death in young athletes. Annual mortality is estimated at 1-2 %.

  • Pathology and Pathophysiology:
    • Dynamic Obstruction of the Left Ventricular Outflow Obstruction (LVOT)
    • Primarily Autosomal Dominant Inheritance
    • Left Ventricular diastolic dysfunction resulting from impaired relaxation and filling of the stiff and hypertrophied left ventricle (often associated with increased filling pressure)
    • Abnormal intramural coronary arteries with thickened walls and narrow lumens
    • Chaotic, disorganized left ventricular architecture (“ cellular disarray’) predisposing to abnormal transmission of electrical impulses and thus serving as a substrate for the formation of arrhythmia.
  • Clinical Manifestation
    • Exertional syncope or pre-syncope – this is the most worrisome symptom, suggesting dynamic LVOT obstruction with or without dysrhythmia, with the potential for sudden cardiac death.
    • Symptoms of pulmonary congestions due to left ventricular dysfunction (e.g. exertional dyspnea, fatigue, orthopnea, paroxysmal nocturnal dyspnea)
    • Chest pain – may be typical angina pain due to increased demand (thicker myocardial walls) and reduced supply (aberrant coronary arteries).
    • Palpitations due to supraventricular or ventricular arrhythmias.
  • ECG Features:
    • Left atrial enlargement
    • Left ventricular hypertrophy with associated ST segment / T-wave abnormalities
    • Deep, narrow (“dagger-like”) Q waves in the Lateral (V5-6, I, aVL) and inferior (II, III, aVF) leads. Most common in Lateral vs. Inferior
    • Giant precordial T-wave inversions in apical HCM
    • Signs of WPW (Short PR, delta wave)
    • Dysrhythmias: Atrial Fibrillation, supraventricular tachycardia, PACs, PVCs, VT

*** Infarction Q Waves are wider with a different morphology compared to HCM.

Pathologic Q Waves:

  • Usually > 40 ms (1mm) wide
  • > 2mm Deep
  • > 25% of depth of QRS Complex
  • Best seen in V1-V3

 

Post by Tyler Manis, MD, PGY4

Case of the week COW #6

CC: Vomiting for 2 days

HPI: 32 yo female with PMH of Romano Ward Syndrome s/p AICD, previous cardiac arrest, Atrial Fibrillation s/p Ablation, renal artery thrombosis on Coumadin and deafness presents to the Emegency Department (ED) via ALS after being found unresponsive at home and with ventricular tachycardia. Paramedics state they were called to the patient’s home for an “unconscious” person. They arrived to find the patient lethargic but arousable, cool, pale, and diaphoretic with BP 90/50. The patient soon became unresponsive and was in Ventricular tachycardia on the monitor. Patient underwent synchronized cardioversion with 150 Joules and converted to sinus tachycardia. Patient also received 150 mg Amiodarone bolus and 250 mL of Normal Saline IVF. Patient’s sister arrived to ED approximately 10 minutes after patient’s arrival and states that patient had multiple episodes of becoming unconscious at home earlier today. Denied recent illness, fever/chills, vomiting, diarrhea

Physical Exam:

BP 133/105,   HR 115 Sinus tachycardia,   RR 20,   SpO2 100% on 3 lpm NC,    Temp 97.0F

General: Lethargic but arousable to painful stimuli

HEENT: NCAT, pupils PERRLA, neck supple

Respiratory: Moderate respiratory distress with bibasilar rales, otherwise clear, no wheezing

Chest: AICD in left anterior chest

Cardiac: +S1/S2, no MRG, tachycardia, regular rhythm

Abdomen: soft NT ND

Neuro exam: arousable to painful stimuli, pt reading lips, answering appropriately

Extremities: weak thready pulses throughout, no edema

Skin: cool, pale, diaphoretic, no rashes, lacerations, or abrasions

 Labs:

CBC: Unremarkable     CMP: K 3.2   Glucose 595     BUN/Cr 17/1.62

AST/ALT 359/401       INR: 1.7             Troponin 0.122

ED Course: Approximately 5 minutes after arrival to the ED during the initial exam, patient became unresponsive and was noted to be in Ventricular Fibrillation on the monitor. CPR/ACLS was started and patient was intubated immediately. Her defibrillator appeared to be delivering shocks however there was no conversion of the rhythm. ACLS was performed for 30 minutes and pulses were regained multiple times. Patient was in V-fib, then PEA, then V-tach arrest. 100mg Lidocaine and 2 grams Magnesium were also given. Defibrillation with external pads ultimately led to Return of Spontaneous circulation. Bloody, frothy sputum was also noted from the ETT post-arrest. A right IJ central line was placed

EKG:

Pre-arrest: Atrial-paced with PACs. QTc 503      Post-arrest: Atrial tachycardia. QTc 609

DX: VFib & VTach arrest s/p ROSC in the setting of Congenital Long QT syndrome and AICD non-capture.

ED/Hospital course:  Post-ROSC EKG did not show STEMI. Post-ROSC Chest XR demonstrated diffused pulmonary edema. Patient was hypoxic with SpO2 in the 80s on 100% FiO2. Decreased tidal volume and increased PEEP improved oxygenation. Bedside cardiac Ultrasound showed global hypokinesis and no pericardial effusion. CT Head w/o contrast was normal. Amiodarone drip was started. Hypothermia protocol was initiated. The patient was admitted to the CCU. She did not code again while in the ED. Her AICD was interrogated and it showed she had been in sustained Vtach to which her AICD gave multiple shocks without capture. She was in the hospital for 11 days where she initially was improving and was therefore extubated after 7 days. Unfortunately she again went into Vtach arrest multiple times and then PEA arrest and was pronounced dead.

Pearls:

Congenital Long QT Syndrome (LQTS)

  • The major variant of congenital prolonged QT syndrome (there are 6 total). Affects estimated 1/2500 to 1/7000 people worldwide
  • Can have varying degrees of penetrance
  • Thought to account for ~3000/year of sudden deaths in children
  • May play a roll in prevalence of SIDS
  • Autosomal dominant inheritance, results in mutation of genes responsible for creating ion-channel proteins. The impaired transport of ions in cardiac tissue causes prolonged QT à impaired repolarization à ventricular arrhythmias (symptomatically syncope, seizure, cardiac arrest). May also present with AV blocks and bradydysrhythmias.
  • Romano-Ward is the more common, autosomal dominant (AD) form with purely cardiac phenotype.
  • The Jervell and Lange-Nielsen syndrome refers to the autosomal recessive (AR) phenotype of congenital LQTS that is associated with profound sensorineural hearing loss and a high risk for sudden death. MORE MALIGNANT CLINICAL COURSE. Our patient likely had this variation since she also had deafness.

Management and treatment

  • Beta- blockers = 1st line treatment in symptomatic patients. Blunts catecholaminergic response
  • Whenever syncopal episodes recur despite full-dose beta-blocking therapy, left cardiac sympathetic denervation (LCSD) should be considered. Cardiac pacing is only rarely indicated (e.g. in infants or young children with 2:1 atrioventricular block).
  • Implantable cardioverter defibrillators (ICDs) are always indicated after cardiac arrest, or when requested by the patient, and whenever syncope recurs despite beta-blockade and LCSD.
  • Potassium supplementation or spironolactone
  • Other antiarrhythmic therapy based on genotype

Risk Factors  for syncope or SCD

  • Congenital deafness
  • Hx/o syncope
  • Hx/o Ventricular arrhythmia
  • Family hx/o SCD
  • Female gender
  • QTc > 600ms
  • Medical non-compliance

Event triggers (vary depending on which genetic mutation)

  • Exercise-related
  • Auditory stimuli (alarm clock, telephone ringing!)
  • Acute emotional events
  • Catecholamine-induced
  • Pregnancy and up to 9 months post-partum
  • Onset of menopause

Post by Katrina D’Amore, DO.

 

Case of the Week (COW) #4

 

 

CC: Generalized Weakness

HPI: 55 y/o male presents complaining of 4 days of gradually worsening weakness in bilateral arms and legs. The patient also reports lower extremity pain one week ago, which resolved. Patient also complains of intermittent left sided back pain for the past couple of months that is exacerbated by walking. ROS: Positive for non-bloody diarrhea 2 weeks prior to presentation. Denies fever and vomiting at any time. No recent travels or sick contacts. Denies HA, dizziness, CP, SOB, or abdominal pain

Pertinent PE and Vitals: BP 172 / 82; Pulse 89; Resp 16; Temp 97.1; Pulse Ox 100% on R/A

GEN: Awake NAD.

EYES: PERRL, EOMI

NECK: supple, FROM, no meningismus

PULM: CTA in all fields, no tachypnea CARD: S1/S2 Normal. Normal rate.

NEURO: AAOX3. Lucid. Follows commands. CN 2-12 intact. No dysmetria bilaterally. No focal neurological deficits appreciated. Sensory intact. No drifts present in upper extremities. No dysarthria. Back: Mild painful ROM of lumbar region. No midline vertebral tenderness. No deformities. Deep tendon reflexes intact. 5/5 strength in all extremities.

Pertinent Labs (if any):

WBC 15.8 without any shift.

ED & Hospital Course: Patient was walked to evaluate gait in the setting of generalized weakness. She was able to ambulate with no ataxia. However, she was moving very slow and appeared globally weak. Patient stated this was not how she usually walks. A CT Head w/o Contrast followed by a lumbar puncture was then performed.

Lumbar Puncture (CSF Labs): RBC : 17,515   WBC: 21    Glucose: 103    Total Protein : 109

*LP was a traumatic 2nd attempt PLUS the samples were accidentally sent in a tube to the lab and dropped by the receiving lab tech. This contributed to the high RBC. Bonus Pearl – CSF tubes should always be hand delivered.

Working Diagnosis at time of Disposition : Guillain Barre Syndrome (GBS)

Neurology was consulted. Negative Inspiratory Force was performed with normal results. Patient was admitted to Neurology Floor with diagnosis of GBS. The patient improved after multiple doses of IVIG and was discharged from the hospital 1 week later with outpatient Neurology follow up

Pearls & Takeaways

Get a thorough history. Diarrhea is usually never important but in this case it was helpful in making the diagnosis. Ø Always walk your patients prior to discharge. Ø GBS is typically followed by a viral illness. Signs and symptoms include symmetric motor weakness that is usually ascending with little to no sensory involvement. Ø Progression of disease is over days to weeks. Ø Paralysis can ascend to the diaphragm. A Negative Inspiratory Force (NIF) test should be performed to evaluate the respiratory muscles. Intubate if FVC <15 mL/kg or negative inspiratory pressure < -25 cm H2O. Ø Albumin-cytological dissociation of CSF (high protein (>45) and low WBC count

 

Recent Pubs

We’ve had a bunch of publications in both peer-reviewed and non peer-reviewed sources over the past few weeks! Check them out when you get a chance:

Traficante and Kashani in the Journal of Clinical Toxicology talking about a Massive Calcium Channel Blocker OD

McGovern and D’Amore in Annals of Emergency Medicine talking about Peds EM Education 

D’Amore, McGovern and McNamee in ACEP Now talking about End-tidal for DKA and COPD

Pena, Mota and McGovern in AAEM/RSA Blog going over the elusive Porphyria diagnosis and management 

DeFranco and McGovern in ACOEP’s Fast Track talking about Isolated Bandemia