Author:
Michael J.Bono
Description
- HELLP syndrome:
- Hemolysis
- Elevated Liver Enzymes
- Low Platelets
- No universally accepted definition of HELLP
- Continuum with severe preeclampsia as most patients will be hypertensive
- Liver involvement is hallmark:
- Other organs may be involved (e.g., brain, kidneys, lungs)
- Most maternal deaths occur with severe preeclampsia
- Increased mortality rate is associated with hepatic hemorrhage or CNS or vascular insult to the cardiopulmonary or renal systems
- Incidence: 0.2% of all pregnancies
- 12-18% have normal BP
- Occurs in 20% of pregnancies with severe preeclampsia or eclampsia
- At diagnosis:
- 52% preterm
- 18% term
- 32% postpartum
Risk Factors
Frequently white, multiparous, older
Pediatric Considerations |
| Infant mortality is greater in women with HELLP |
Etiology
- Unclear, but linked to the etiology of preeclampsia which has 4 proposed etiologies:
- Abnormal trophoblastic invasion of uterine vessels
- Immune reaction to maternal, paternal, and fetal tissues
- Maternal response to cardiovascular or inflammatory changes of pregnancy
- Genetic factors
- Proposed mechanism of HELLP:
- Fetal-placental debris is released into maternal circulation, causing systemic inflammatory response
- Vascular constriction causes resistance to blood flow and HTN
- Vasospasm probably damages vessels directly
- Endothelial cell is damaged and interendothelial cell leaks are the result
- Small-vessel leaks:
- Platelets and fibrinogen get deposited in the subendothelium
- Fibrin deposition develops in severe cases
- Vascular changes and local tissue hypoxia lead to hemorrhage, necrosis, and end-organ damage
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 epigastric 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; normal BP is <140/90
- BP normally decreases in the second and early third trimesters
- 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
Diagnostic Tests & Interpretation
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
Prehospital
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:
- Controversial, so discuss with OB consultant
- May help fetal lung maturity
- IV dexamethasone more effective than IM betamethasone
- Depends on gestational age of fetus
- Does not reduce disease severity or duration of HELLP, but improves platelet counts
- Limit IV fluid administration unless clinical evidence of dehydration:
- Excess fluids promote further capillary leak
- Lactated Ringer 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: 5 mg IV, then 5-10 mg q15-20min:
- Up to 40 mg total dose, to keep diastolic BP
<
90-110 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
- Nifedipine: 10 mg initial dose, repeat in 30 min if necessary
- Do not give sublingual dose
Second Line
- Nitroprusside: 0.25 mcg/kg/min as a drip:
- Increase 0.25 mcg/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:
- Start maintenance drip starting at 2 g/hr
- Titrate to clinical effect, check deep tendon reflexes periodically
- Watch for toxicity (antidote is calcium gluconate 10%, 10 mL IV over 3 min)
- Measure magnesium level at 4-6 hr; adjust drip to achieve levels between 4-7 mEq/L
- Measure serum magnesium levels if creatinine in >1.0 mg/dL
Disposition
Admission Criteria
- Admit all patients with suspected HELLP 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
- Hypertensive Disorders. In: CunninghamF, LevenoKJ, BloomSL, et al., eds. Williams Obstetrics. 24th ed.New York: McGraw-Hill; 2013. http://accessmedicine.mhmedical.com.evms.idm.oclc.org/content.aspx?bookid=1057§ionid=59789106. Accessed February 5, 2018.
- NakamuraK, InokuchiR, SonooT, et al. Late postpartum HELLP syndrome over 10 days after delivery . Am J Emerg Med. 2016;34:2237.
- PourratO, DoreyM, RagotS, et al. High-dose methylprednisolone to prevent platelet decline in preeclampsia: A rand omized controlled trial . Obstet Gynecol. 2016;128:153-158.
- RyanD, CruciataG, MontiS, et al. Peripartum patient with epigastric pain . Ann Emerg Med. 2017; 70:301-307.
- WoudstraDM, Chand raS, HofmeyrGJ, et al. Corticosteroids for HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome in pregnancy (Review) . Cochrane Database Syst Rev. 2010;(9):CD008148.
- YoungJ. Maternal emergencies after 20 weeks of pregnancy and in the postpartum period. In: TintinalliJE, StapczynskiJ, MaO, et al., eds. Tintinalli's Emergency Medicine: A Comprehensive Study Guide. 8th ed.New York: McGraw-Hill; 2016. http://accessmedicine.mhmedical.com.evms.idm.oclc.org/content.aspx?bookid=1658§ionid=109431050. Accessed February 1, 2018.
See Also (Topic, Algorithm, Electronic Media Element)
Preeclampsia