Objectives Hereditary haemorrhagic telangiectasia (HHT) affects 1 in 5-8000 individuals. Pregnancy outcomes are rarely reported. The major reason is that most women do not have their HHT diagnosed prior to pregnancy. Using a large well-characterised series, we studied all pregnancies known to have occurred in HHTaffected women, whether or not their diagnosis was known at the time of pregnancy. Our aim was to estimate rates and types of major complications of HHT in pregnancy, to guide management decisions.Design Cohort study, with prospective, retrospective and familial components.Setting/Population Tertiary referral centre population.Methods All 262 pregnancies in the 111 women with HHT and pulmonary arteriovenous malformations (PAVMs) reviewed between 1999 and 2005 were studied. Eighty-two women (74%) did not have a diagnosis of HHT/PAVM at the time of pregnancy. 222 pregnancies in their 86 HHT-affected relatives were also studied.Main outcome measures PAVM bleed, stroke and maternal death.Results Thirteen women experienced life-threatening events during pregnancy: 1.0% (95% CI 0.1-1.9) of pregnancies resulted in a major PAVM bleed; 1.2% (0.3-2.2%) in stroke (not all were HHT related); and 1.0% (0.13-1.9%) in maternal death. All deaths occurred in women previously considered well. In women experiencing a life-threatening event, prior awareness of HHT or PAVM diagnosis was associated with improved survival (P = 0.041, Fisher's exact test).Conclusions Most HHT pregnancies proceed normally. Rare major complications, and improved survival outcome following prior recognition, means that pregnancy in a woman with HHT should be considered high risk. Recommendations for pregnancy management are provided.Keywords Arteriovenous malformations, cerebral, haemorrhage, myocardial infarction, nose bleeds, pulmonary.Please cite this paper as: Shovlin C, Sodhi V, McCarthy A, Lasjaunias P, Jackson J, Sheppard M. Estimates of maternal risks of pregnancy for women with hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): suggested approach for obstetric services.

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Summary: Seventy-three pregnancies in 43 women with SPK have now been described by the US National Transplantation Pregnancy Registry (NTPR) (established in 1991), which contains self-reported data from questionnaires and hospital records. These women have high rates of complications despite normoglycaemia and restoration of renal function. We describe the pregnancies of three SPK recipients in the UK managed in joint renal obstetric clinics and discuss the antenatal and postnatal complications specific to SPK transplants.Keywords: pregnancy, pancreas -kidney transplant CASE 1A 36-year-old Caucasian woman presented at seven weeks gestation in her first pregnancy. Four years earlier she had a simultaneous pancreas-kidney transplant (SPK) for type 1 diabetes and nephropathy with pancreatico-duodenal anastomosis to the small bowel. Initial immunosuppression included tacrolimus and mycophenolate mofetil (MMF) but sirolimus was started following neutropenia secondary to MMF. The pregnancy was unplanned. Prior to pregnancy laboratory markers were as follows: serum creatinine (Cr) 110 mmol/L (estimated Glomerular Filtration Rate [eGFR] 52 mL/minute/1.73 m 2 ), HbA1C 5.1% and serum amylase 58 mmol/L. Atorvastatin and lansoprazole were discontinued and immunosuppression was left unchanged (tacrolimus 3 mg twice daily and sirolimus 1 mg once daily). She continued taking aspirin 75 mg once daily, which she had been taking since transplantation. She was counselled about the unknown risks of sirolimus in pregnancy and relative safety of tacrolimus. Following discontinuation of lansoprazole she developed heartburn and was prescribed gaviscon and ranitidine.She was normotensive and protein:creatinine ratios (PCR) (65 mg/mmol Cr) remained stable throughout the pregnancy. Tacrolimus was sequentially increased to 5 mg twice daily between 13 and 23 weeks gestation because levels were repeatedly low (2.7-5.8 ng/mL). She had a normal glucose tolerance test at 27 weeks. Sirolimus levels were adequate (1.5-3.0 ng/ mL). Serum Cr initially fell to 80 mmol at 13 weeks but increased to 114 mmol/L at 34 weeks gestation. An ultrasound of the allograft excluded obstruction and Cr peaked at 121 mmol/L at 35 weeks. Fetal ultrasound showed normal fetal growth. An elective caesarean section with Pfannensteil incision was performed at 37 þ 1 weeks when Cr was 95 mmol. A 2594 g male infant was delivered (Apgar scores at 1, 5 and 10 minutes: 8, 8 and 10) and fetal arterial and venous cord blood were 7.255 and 7.326, respectively. The baby was transferred to the neonatal unit for temporary non-invasive ventilation. Good perfusion of the maternal kidney and pancreas allografts was demonstrated on ultrasound postpartum. Serum Cr postpartum was 103 mmol/L and amylase 59 mmol/L. She was discharged home with the baby on day 4 and received thromboprophylaxis with enoxaparin (40 mg once daily) for 10 days. Most recent serum Cr is 107 mmol/L (eGFR 58 mL/ minute/1.73 m 2 ) at 16 weeks postpartum. CASE 2A 29-year-old Caucasian woman with type 1 diabetes devel...

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