The prevalence and pregnancy outcomes of intrahepatic cholestasis of pregnancy: A retrospective clinical audit review.

BACKGROUND: To determine the prevalence and outcomes of intrahepatic cholestasis of pregnancy. METHODS: A review comparing intrahepatic cholestasis of pregnancy pregnancies to all other pregnancies in three tertiary care Australian hospitals over a 36-month period. RESULTS: There were 43,876 pregnancies. The prevalence of intrahepatic cholestasis of pregnancies (n = 319) was 0.7%. There were differences between intrahepatic cholestasis of pregnancy and non-intrahepatic cholestasis of pregnancy mothers including higher prevalence of South Asian (22.6% versus 3.1%, p < 0.001), Indigenous Australian (3.8% versus 1.8%, p < 0.05), and Asian ethnicity (8.4% versus 5.7%, p < 0.05), mothers with a body mass index >35 kg/m2 (10.6% versus 5.5%, p < 0.001), those with diabetes mellitus (25.7% versus 9.8%, p < 0.001), and those with twin births (8.7% versus 2.2%, p < 0.001). The primary clinical outcomes of intrahepatic cholestasis of pregnancy included a median gestational age at delivery of 36.4 (SE 0.09) weeks compared to 38.6 (SE 0.01) weeks (p < 0.001), a lower birth weight (3.12 (SE 0.03) versus 3.31 kg (SE 0.03), p < 0.001), and an increase in special care nursery admissions (44.5% versus 15.3%, p < 0.001). CONCLUSION: Treated intrahepatic cholestasis of pregnancy in the population described here had similar mortality outcomes although increased special care nursery admission as compared to the general population.

Long-term outcomes after first-onset arrhythmia in Fontan physiology.

OBJECTIVES: Patients living with a Fontan circulation are prone to develop arrhythmias. However, their prognostic impact has been seldom studied. As such, we aimed to determine the incidence and predictors of arrhythmias after the Fontan procedure and the long-term outcomes after the first onset of arrhythmias. METHODS: Of the 1034 patients who have undergone a Fontan procedure as recorded in the Australian and New Zealand Fontan Registry, we identified those in whom a tachyarrhythmia or bradyarrhythmia developed. We evaluated the incidence and predictors of developing arrhythmias and their prognostic impact on late outcomes. RESULTS: Arrhythmia developed in 195 patients. Tachyarrhythmia was present in 162 patients, bradyarrhythmia was present in 74 patients, and both forms were present in 41 patients. At 20 years, freedom from any arrhythmia, tachyarrhythmia, and bradyarrhythmia was 66% (95% confidence interval [CI], 59-72), 69% (95% CI, 62-75), and 85% (95% CI, 80-90), respectively. On multivariable analyses, patients with an extracardiac Fontan (hazard ratio [HR], 0.23; 95% CI, 0.10-0.51; P < .001) were less likely to develop an arrhythmia, whereas those with left atrial (HR, 3.18; 95% CI, 1.45-6.95; P = .004) and right atrial (HR, 4.00; 95% CI, 2.41-6.61; P < .001) isomerism were more likely to have an arrhythmia. After onset of any arrhythmia (tachyarrhythmia or bradyarrhythmia), 10- and 15-year survivals were 74% (65%-83%) and 70% (60%-80%), respectively, and freedom from Fontan failure was 55% (44%-64%) and 44% (32%-56%), respectively. The development of any arrhythmia (HR, 2.20; 95% CI, 1-44-3.34; P < .001), tachyarrhythmia (HR, 2.56; 95% CI, 1.60-4.11; P < .001), and bradyarrhythmia (HR, 1.85; 95% CI, 1.16-2.95; P = .01) were all independent predictors of late Fontan failure on multivariable analyses. CONCLUSIONS: The development of an arrhythmia is associated with a heightened risk of subsequent failure of the Fontan circulation.

Unsatisfactory Early and Late Outcomes After Fontan Surgery Delayed to Adolescence and Adulthood.

The ideal age to perform the Fontan procedure is still unknown. The aim of this study is to determine outcomes after Fontan surgery delayed to adolescence and adulthood in Australia and New Zealand. Patients who had undergone a Fontan procedure at 15 years of age or older were identified in the 1133 patients registered in the Australia and New Zealand Fontan Registry until December 2012. A total of 45 patients underwent the following Fontan procedure at a median age of 18.3 years (16-21 years): 24 atriopulmonary connections, 10 lateral tunnel, and 11 extracardiac conduits. Hospital mortality was 13% (6 of 45). After a mean follow-up of 15.5 ± 9 years, there were 8 late deaths. Survival rates after 10, 20, and 25 years were 79% (95% CI: 64-89), 70% (95% CI: 51-83), and 70% (95% CI: 51-83), respectively. Freedom from Fontan failure (death, heart transplantation, Fontan takedown, protein-losing enteropathy, and poor functional status) after 10 and 20 years was 63% (95% CI: 47-76) and 35% (95% CI: 19-52), respectively. Patients with a single left ventricle had a lower risk of failure (hazard ratio = 0.25, 95% CI: 0.10-0.59; P = 0.002). Arrhythmias developed in 29 patients after a median of 0.1 years (0-9.3 years) and 10 required a permanent pacemaker. Freedom from all adverse events at 10 years was 30% (95% CI: 16-45). Outcomes of the Fontan procedure in adolescents and adults are poor, with disproportionately high hospital mortality and late adverse events. The Fontan procedure should not be delayed to adolescence and adulthood and should be performed electively in childhood.

Effectiveness and safety of 1 vs 4†h blood pressure profile with clinical and laboratory assessment for the exclusion of gestational hypertension and pre-eclampsia: a retrospective study in a university affiliated maternity hospital.

OBJECTIVE: We asked whether 60 compared with 240 min observation is sufficiently informative and safe for pregnancy day assessment (PDAC) of suspected pre-eclampsia (PE). DESIGN: A retrospective study of 209 pregnant women (475 PDAC assessments, 6 months) with routinely collected blood pressure (BP), symptom and laboratory information. We proposed a 60 min screening algorithm comprising: absence of symptoms, normal laboratory parameters and ≤1high-BP reading (systolic blood pressure, SBP 140 mm Hg or higher or diastolic blood pressure, DBP 90 mm Hg or higher). We also evaluated two less inclusive screening algorithms. We determined short-term outcomes (within 4 h): severe hypertension, proteinuric hypertension and pregnancy-induced hypertension, as well as long-term outcome: PE-related diagnoses up to the early puerperium. We assessed performance of alternate screening algorithms performance using 2×2 tables. RESULTS: 1 in 3 women met all screen negative criteria at 1 h. Their risk of hypertension requiring treatment in the next 3 h was 1.8% and of failing to diagnose proteinuric hypertensive PE at 4 h was 5.1%. If BP triggers were 5 mm Hg lower, 1 in 6 women would be screen-negative of whom 1.1% subsequently develops treatment-requiring hypertension and 4.5% demonstrate short-term proteinuric hypertension. We present sensitivity, specificity, negative and positive likelihood ratios for alternate screening algorithms. CONCLUSIONS: We endorse further research into the safest screening test where women are considered for discharge after 60 min. Safety, patient and staff satisfaction should be assessed prospectively. Any screening test should be used in conjunction with good clinical care to minimise maternal and perinatal hazards of PE.