The maternal mortality rate in HELLP syndrome is about 1% to 2% but may vary depending on location and access to medical care. In 32 published series involving 2346 patients with HELLP syndrome managed worldwide between 2001 and 2011, there were 77 deaths (3.3%), 53 strokes, and 178 cases of acute renal failure.[15]Martin JN Jr, Owens MY. Preeclampsia-eclampsia y syndrome de HELLP. In: Romero Arauz JF, Tena Alavez G, Jimenez Solis GA, eds. Preeclampsia - enfermedades hipertensivas del embarazo [in Spanish]. Mexico: McGraw Hill; 2012. A management plan involving the aggressive use of corticosteroids, magnesium sulfate and prevention of severe systolic hypertension was not systematically provided for these patients.
Disease course
One case control study using the UK Obstetric Surveillance System included the findings from 129 patients with HELLP syndrome and 81 patients with partial HELLP syndrome. In the study, 95% of patients were delivered within 48 hours. Patients with HELLP syndrome were significantly more likely to require blood products and to have severe maternal morbidity (13%) compared with women with partial HELLP syndrome (1%).[98]Fitzpatrick KE, Hinshaw K, Kurinczuk JJ, et al. Risk factors, management, and outcomes of hemolysis, elevated liver enzymes, and low platelets syndrome and elevated liver enzymes, low platelets syndrome. Obstet Gynecol. 2014;123:618-27.
http://www.ncbi.nlm.nih.gov/pubmed/24499757?tool=bestpractice.com
Recurrence
The recurrence risk is relatively low (2% to 6%), although in some populations the recurrence is 15% to 20%. However, up to 20% to 40% of normotensive patients with a history of prior HELLP syndrome will have some form of preeclampsia in a subsequent pregnancy. This rate increases to 75% in those with underlying hypertensive disorders.[99]Sibai BL, Ramadan MK, Chari RS, et al. Pregnancies complicated by HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets): subsequent pregnancy outcome and long-term prognosis. Am J Obstet Gynecol. 1995;172:125-129.
http://www.ncbi.nlm.nih.gov/pubmed/7847520?tool=bestpractice.com
[100]Chames MC, Haddad B, Barton JR, et al. Subsequent pregnancy outcome in women with a history of HELLP syndrome at ≤ 28 weeks of gestation. Am J Obstet Gynecol. 2003;188:1504-1507.
http://www.ncbi.nlm.nih.gov/pubmed/12824985?tool=bestpractice.com
[101]van Pampus MG, Wolf H, Mayruhu G, et al. Long-term follow-up in patients with a history of (H)ELLP syndrome. Hypertens Pregnancy. 2001;20:15-23.
http://www.ncbi.nlm.nih.gov/pubmed/12044310?tool=bestpractice.com
[102]Habli M, Eftekhari N, Wiebracht E, et al. Long-term maternal and subsequent pregnancy outcomes 5 years after hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome. Am J Obstet Gynecol. 2009;201:385;e1-e5.
http://www.ncbi.nlm.nih.gov/pubmed/19716544?tool=bestpractice.com
Currently, there is no effective intervention to prevent the recurrence of HELLP syndrome.
Long-term disease associations
Hypertensive disorders of pregnancy are associated with an increased risk of cardiovascular disease in later life.[103]O'Kelly AC, Michos ED, Shufelt CL, et al. Pregnancy and reproductive risk factors for cardiovascular disease in women. Circ Res. 2022 Feb 18;130(4):652-72.
https://www.ahajournals.org/doi/10.1161/CIRCRESAHA.121.319895
http://www.ncbi.nlm.nih.gov/pubmed/35175837?tool=bestpractice.com
However, there is currently a lack of understanding of preeclampsia subtypes, which include differences in the timing of onset in pregnancy, the presence of severe features and the association of intrauterine growth restriction. These features may have different long-term implications for cardiovascular risk.[104]Grandi SM, Filion KB, Yoon S, et al. Cardiovascular disease-related morbidity and mortality in women with a history of pregnancy complications. Circulation. 2019 Feb 19;139(8):1069-79.
https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.118.036748
http://www.ncbi.nlm.nih.gov/pubmed/30779636?tool=bestpractice.com
Regular assessment of cardiovascular risk factors in women who have had hypertensive disorders of pregnancy provides an opportunity for prevention interventions.[105]Wenger NK, Lloyd-Jones DM, Elkind MSV, et al. Call to action for cardiovascular disease in women: epidemiology, awareness, access, and delivery of equitable health care: a presidential advisory from the American Heart Association. Circulation. 2022 Jun 7;145(23):e1059-71.
https://www.ahajournals.org/doi/full/10.1161/CIR.0000000000001071
http://www.ncbi.nlm.nih.gov/pubmed/35531777?tool=bestpractice.com