First diagnosis of severe coarctation of the aorta necessitating percutaneous intervention during pregnancy: a case report

Background: Coarctation of the aorta (CoA) is a common congenital heart defect that affects about 3-4 in every 10 000 live births. Despite clear signs on clinical examination, the diagnosis is sometimes not made until adulthood. An increasing number of patients with CoA are reaching child-bearing age. Unrepaired CoA or severe recurrent stenosis during pregnancy is a significant concern, as it poses a high risk of maternal and foetal complications and even death.

Case summary: A 21-year-old woman was referred to the cardiology department at 17 weeks' gestation for management of arterial hypertension and unexplained systolic murmur. She had been diagnosed with hypertension elsewhere the year before presentation, but unfortunately, this remained unexplored. She had been started on labetalol early in the pregnancy. Clinical examination showed a loud systolic heart murmur extending from parasternal to subclavicular and scapular areas. Pulses in the lower extremities were very weak, and blood pressure was slightly elevated with a significant gradient between the upper and lower extremities. Echocardiography showed remarkable absence of pulsatile flow in the abdominal aorta and narrowing just distal to the subclavian artery with typical diastolic tail pattern on suprasternal imaging. Cardiac magnetic resonance confirmed the presence of a severe coarctation distal to the subclavian artery and presence of multiple collaterals allocating this patient in the extremely high-risk category with a risk of up to 40%-100% of maternal cardiac event during pregnancy. An extensive multidisciplinary team meeting was convened. After initial medical optimization, increased claudication and signs of placental hypoperfusion necessitated an endovascular procedure under general anaesthesia at 23 weeks' gestation. A Bentley BeGraft Plus stent (16 × 38 mm) was successfully placed. Postoperative ultrasound showed biphasic placental perfusion and normalization of blood pressure and ankle-brachial indices. At 36 weeks' gestation, the patient gave birth to a healthy child.

Discussion: Coarctation of the aorta should be considered in any young patient with arterial hypertension. Altered maternal haemodynamics during pregnancy resulted in severe symptomatic CoA and reduced placental flow necessitating percutaneous intervention during pregnancy. A multidisciplinary pregnancy heart team is essential for optimal treatment management in these high-risk patients.