SIGNS AND SYMPTOMS
History
- History and physical exam with attention to symptoms of abdominal pain, nausea, vomiting, and headache
- Obstetric history:
- Parity
- Deliveries
- History of hypertensive disorder during pregnancy
- Estimated gestational age
- Prenatal care
- May present with flulike symptoms, such as fatigue or malaise
- Nausea, usually with vomiting
- Right upper quadrant or epigastic pain:
- Pain increases with severity of disease
- Headache, often with visual changes
- Symptoms which carry higher morbidity:
- Dyspnea and/or fluid overload to suggest cardiogenic/noncardiogenic pulmonary edema
- Dyspnea associated with pulmonary embolus
- Chest pain suggestive of myocardial ischemia
- Altered mental status, seizures of focal neurologic deficit:
- Hypertensive encephalopathy
- Cerebral edema
- Hemorrhagic cerebrovascular accident
- Peripheral edema
- Ascites
- Hematuria
- Low urine output
ALERT
Determination of gestational age and fetal viability is critical in HELLP.
Physical-Exam
- Vital signs with attention to BP
- May not have systolic or diastolic HTN
- Many patients will have right upper quadrant pain, concern for liver subcapsular hematoma
- Evidence of fluid overload
- Careful neurologic exam
- Fetal heart tones
ESSENTIAL WORKUP
- Immediate CBC with platelet count and smear, BUN, creatinine, LFTs, coagulation profile, and magnesium level
- Urinalysis for protein; screen for UTI
- Weigh patient to determine recent weight gain
DIAGNOSIS TESTS & NTERPRETATION
Lab
- CBC:
- Anemia
- Thrombocytopenia
- Peripheral smear demonstrates microangiopathic hemolytic anemia (burr cells or schistocytes)
- Other hemolysis markers are elevated lactate dehydrogenase (LDH) levels, increased reticulocyte count, and elevated bilirubin levels
- Platelet count and smear:
- Disseminated intravascular coagulation screen
- Coagulation profile:
- BUN, creatinine, and magnesium levels
- LFTs to assess hemolysis markers and hepatic dysfunction:
- Elevated aspartate aminotransferase level: >40 IU/L
- Elevated alanine aminotransferase level: >40 IU/L
- Elevated LDH: >600 IU/L
- Elevated serum bilirubin: >1.2 mg/dL
Imaging
- CXR:
- Suspected pulmonary edema
- CT of head:
- Mental status changes or focal neurologic deficit
- US of the pelvis (transabdominal or transvaginal):
DIFFERENTIAL DIAGNOSIS
- GI:
- Cholecystitis
- Cholelithiasis
- Biliary colic
- Pancreatitis
- Hepatitis
- Ulcer disease
- Acute fatty liver of pregnancy
- Acute gastritis
- Hiatal hernia
- Severe gastroesophageal reflux
- Hematologic:
- Preeclampsia-associated thrombocytopenia
- Gestational thrombocytopenia
- Idiopathic thrombocytopenic purpura
- Thrombotic thrombocytopenic purpura
- Hemolytic uremic syndrome
- Neurologic:
- Epilepsy
- Encephalitis
- Meningitis
- Encephalopathy
- Brain tumor
- Intracranial hemorrhage
- Other:
- Drug abuse
- Pyelonephritis
- Sepsis
TREATMENT
PRE HOSPITAL
Cautions:
- Transport patient in left lateral decubitus position to prevent inferior vena cava syndrome
- Venous access for anticipated seizure activity
- Routine seizure management (preferably with magnesium sulfate) if the patient seizes
ALERT
Transport to a facility capable of providing high-risk obstetric care.
INITIAL STABILIZATION/THERAPY
- ABC management
- Left lateral decubitus position to prevent inferior vena cava syndrome
- High-flow oxygen via face mask
- Maternal monitoring:
- Cardiac
- Pulse oximetry
- Tocography
- Fetal monitoring
ED TREATMENT/PROCEDURES
- Control HTN with antihypertensives (see Medication):
- Avoid ACE inhibitors because of fetal side effects
- Heparin should be avoided because of bleeding complications
- Treat preeclampsia or eclampsia with IV magnesium sulfate:
- Magnesium sulfate is not given to treat HTN
- Order type and screen for possible transfusion
- Call for emergent obstetric consult, consider neonatology consult:
- Consider emergent delivery
- Early plasma exchange therapy has shown promise in postpartum patients with severe disease
- Discuss administration of glucocorticoid with consultant:
- Helps fetal lung maturity
- IV dexamethasone more effective than IM betamethasone
- Depends on gestational age of fetus
- Does not reduce disease severity or duration, but improves platelet counts
- Limit IV fluid administration unless clinical evidence of dehydration:
- Excess fluids promote further capillary leak
- Lactated Ringers or NS at 60 mL/hr (no more than 125 mL/hr)
- Monitor urine output with Foley catheter
- Correct thrombocytopenia by platelet transfusion in women with platelet counts <20,000 platelets/μL, even without active bleeding, as risk of postpartum bleeding is significantly increased
- Platelet counts >40,000 platelets/μL are safe for vaginal delivery
- Correct thrombocytopenia to platelet counts >50,000 platelets/μL if cesarean delivery planned
- If coagulation dysfunction is present, transfusion with fresh frozen plasma and packed RBCs in consultation with obstetrics
- Transfusion with packed RBCs for hemoglobin <10 g/dL
MEDICATION
First Line
- Hydralazine: 2.5 mg IV, then 5–10 mg q15–20min:
- Up to 40 mg total dose, to keep diastolic BP <110 mm Hg
- IV drip 5–10 mg/hr titrated
- Labetalol: 10 mg IV, then 20–80 mg IV q10min:
- Up to 300 mg total dose
- IV drip 1–2 mg/min titrated
Second Line
- Nitroprusside: 0.25 μg/kg/min as a drip:
- Increase 0.25 μg/kg/min q5min
- Use only if no response to hydralazine or labetalol
- Magnesium sulfate: 4–6 g in 100 mL IV over 15–20 min as loading dose:
- Maintenance drip starting at 2 g/hr
- Titrate to clinical effect
- Watch for toxicity (antidote is calcium gluconate 10%, 10 mL IV over 3 min).
- Measure magnesium sulfate level at 4–6 hr; adjust drip to achieve levels between 4 and 7 mEq/L.
FOLLOW-UP
DISPOSITION
Admission Criteria
- Admit all patients to obstetric service for continuous monitoring of mother and fetus
- ICU admission:
- Pulmonary edema
- Respiratory failure
- Cerebral edema
- GI bleeding with hemodynamic instability
Discharge Criteria
Patients with HELLP syndrome should always be admitted. Discharge should be a decision of the OB Consultant
Issues for Referral
After stabilization in the ED, transfer to facility capable of managing high-risk obstetric conditions unless delivery is imminent.
FOLLOW-UP RECOMMENDATIONS
Patients should be followed closely by OB:
- May develop HELLP after delivery, usually within 48 hr
PEARLS AND PITFALLS
- Hypertensive pregnant women with abdominal pain, elevated LFTs, and decreased platelets need emergent treatment and OB consultation
- Patients with HELLP syndrome may have a normal BP
- Transport to a facility capable of caring for these patients after stabilization is essential
ADDITIONAL READING
- Ciantar E, Walker JJ. Pre-eclampsia, severe pre-eclampsia and hemolysis, elevated liver enzymes and low platelets syndrome: What is new?
Women’s Health.
2011;7(5):555–569.
- Deak TM, Moskovitz JB. Hypertension and pregnancy.
Emerg Med Clin North Am.
2012;30:903–917.
- Giannubilo SR, Bezzeccheri V, Cecchi S, et al. Nifedipine versus labetalol in the treatment of hypertensive disorders of pregnancy.
Arch Gynecol Obstet.
2012;286:637–642.
- Woudstra DM, Chandra S, Hofmeyr GJ, et al. Corticosteroids for HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome in pregnancy (Review).
Cochrane Database Syst Rev
. 2010; (9):CD008148.
- Yoder SR, Thornburg LL, Bisognano JD. Hypertension in pregnancy and women of childbearing age.
Am J Med
. 2009;122:890–895.
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