Obstetric Medicine

This article summarizes and critiques four recent publications looking at preconception counseling, pregnancy outcomes, and cardiac complications in women with history of Fontan circulation. The Fontan procedure is a palliative strategy for single-ventricle type congenital heart disease and involves passive flow of venous return into the pulmonary circulation, bypassing the ventricles. Pregnancy in these patients is not without risk and preconception counseling and contraception practices vary widely. High rates of miscarriage, prematurity, and small-for-gestational-age babies are reported. Cardiac complications include mainly arrhythmias. Whether long-term prognosis in these patients is affected by pregnancy is not yet known.

Melanoma is one of the most common cancers diagnosed in pregnancy and has a high metastatic potential. As the incidence of melanoma increases, careful clinical evaluation of suspicious skin lesions remains the mainstay of early diagnosis. There is controversy in the literature as to whether pregnancy-associated melanoma has worse survival than other melanomas. Any changing-pigmented lesion should be biopsied, regardless of pregnancy hyperpigmentation. Increased lymphangiogenesis in pregnancy is associated with increased metastasis – timely diagnosis is therefore imperative. While the effect of oestrogen and progesterone on melanoma is under investigation, it is generally accepted that oral contraceptive use in not contraindicated after a diagnosis of melanoma in pregnancy. Subsequent pregnancy should be delayed for two to three years after a diagnosis of melanoma with a high risk of recurrence.

Reverse innovation, defined as the flow of ideas from low- to high-income settings, is gaining traction in healthcare. With an increasing focus on value, investing in low-cost but effective and innovative solutions can be of mutual benefit to both high- and low-income countries. Reverse innovation has a role in addressing maternal health challenges in high-income countries by harnessing these innovative solutions for vulnerable populations especially in rural and remote regions. In this paper, we present three examples of ‘reverse innovation’ for maternal health: a low-cost, easy-to-use blood pressure device (CRADLE), a diagnostic algorithm (mini PIERS) and accompanying mobile app (PIERS on the Move), and a novel method for mapping maternal outcomes (MOM).

Uncommon renal disorders in pregnancy can be challenging to manage given limited evidence in the literature to guide management. We present a series of three uncommon renal disorders in pregnancy: Von Hippel–Lindau disease, Gitelman syndrome, and Nutcracker syndrome. Previously published case reports with differing outcomes offer some guidance to the management of these disorders in pregnancy. In this case series, we address the management of these syndromes during pregnancy and discuss the maternal and fetal outcomes. All three of our patients had good maternal and fetal outcomes, which will contribute to current data on maternal and fetal outcomes in these rare diseases, which is limited.

Carcinoid tumours are rare slow growing tumours which arise from primitive neuroendocrine cells. The effect of the pregnant state on carcinoid tumours and vice versa remains unclear, as does the optimal management of carcinoid tumours during the pregnancy including labour. We report the rare case of a 36 year old primigravida woman with large bilateral pulmonary carcinoid tumours. The patient's disease was minimally symptomatic with no clinical suspicion of carcinoid syndrome. Under close observation, the pregnancy progressed well and the patient proceeded to a spontaneous vaginal delivery of a healthy child. We conduct the the first literature review in 30 years of all reported cases in this area and make suggestions as to assessment and monitoring of cases of carcinoid during pregnancy.

Pompe disease is an autosomal-recessive disorder caused by acid alpha-glucosidase deficiency due to mutations in the GAA gene. There are two forms of the disease: infantile-onset Pompe disease and late-onset Pompe disease. The worldwide incidence of both forms of the disease is commonly reported to be 1 in 40,000. Adult patients are affected by limb-girdle muscular weakness and respiratory insufficiency. Enzyme replacement therapy with alglucosidase-alpha is available since 2006. There is little knowledge about pregnant woman with Pompe disease. These women should be considered as high-risk pregnant women. Here, we aim to present Cesarean delivery and postpartum management of a case with an interrupted enzyme replacement therapy during pregnancy.