Postnatal diagnosis of maternal congenital heart disease: missed opportunities

Abstract

A 30-year-old primigravida with no known comorbidities presented to the emergency department at 29+6/40 gestation, with breathlessness. The initial diagnosis was pulmonary embolism, which was later revised following initial investigations and considered to be pre-eclampsia/HELLP (haemolysis, elevated liver enzymes, low platelets) syndrome. Following caesarean section and delivery of a live baby, the patient had episodes of cyanotic hypoxia and was admitted to intensive care. A provisional diagnosis of idiopathic pulmonary hypertension was performed. Decompensation led to transfer to a specialist intensive care unit for extracorporeal membrane oxygenation, where a diagnosis of patent ductus arteriosus and Eisenmenger's syndrome was made. Heart disease is the leading indirect cause of maternal death, and Eisenmenger's syndrome in pregnancy carries a 50–65% mortality. A literature review demonstrated that this is the only reported case of a postnatal diagnosis of Eisenmenger's syndrome. We considered missed opportunities to make an earlier diagnosis, so that patients and doctors will benefit from the lessons we learnt.

Patent Ductus Arteriosus in Pregnancy: Cardio-Obstetrics Management in a Late Presentation

“… 4 Additionally, there have been many cases of pregnant women presenting late in their pregnancy with complications such as preeclampsia, HELLP (hemolysis, elevated liver enzymes, low platelet) syndrome, and decompensated heart failure due to previously undiagnosed PDA. 1 This illustrates the importance of a coordinated effort to both diagnose and manage a patient with an unrepaired PDA early in pregnancy.…”

“…When unrepaired, they can lead to Eisenmenger syndrome, which carries a 50%-65% mortality rate during pregnancy. 1 Physiologic changes in pregnancy impose further stress on those with congenital heart disease; for example, the decreased systemic vascular resistance that occurs during pregnancy increases right-to-left shunting, leading to reduced pulmonary perfusion and subsequent hypoxemia. 2 This will lead to further decompensation and an increase in maternal and fetal morbidity and mortality.…”

Extracorporeal Membrane Oxygenation for Pulmonary Embolism During Pregnancy and Postpartum

“…Another publication concerned a patient initially thought to have pulmonary embolism but subsequently diagnosed with pre-eclampsia and undergoing ECMO. That report was also excluded from the present study (21). Another case report involving peripartum heart failure and ECMO application was also excluded (22).…”

Where on the Differential Is Eisenmenger Syndrome in a Patient Without Prior Cardiopulmonary Disease?

“…However, due to the chronic nature of the cardiopulmonary dysfunction and underlying hypoxia, subjective symptoms and physical examination findings may be unremarkable or absent, as the body can compensate for some degree of hypoxia [10]. As a result, patients may be undiagnosed for months or years due to physicians having an appropriately high threshold for proceeding with further diagnostic evaluation in the absence of clinical manifestations [11].…”