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Case report
Fetal congenital midaortic syndrome with unilateral renal artery stenosis prenatally presenting with polyhydramnios and postpartum as hyponatremic hypertensive syndrome
  1. Sara Ardabili1,
  2. Vincent Uerlings1,
  3. Andrea Kaelin Agten2 and
  4. Markus Hodel1
  1. 1Department of Obstetrics and Gynecology, Cantonal Hospital Lucerne, Lucerne, Switzerland
  2. 2Fetal Medicine Unit, Nottingham University Hospitals NHS, Nottingham, UK
  1. Correspondence to Dr Sara Ardabili; sara.ardabili{at}gmx.ch

Abstract

The midaortic syndrome (MAS) is a rare anomaly, characterised by narrowing of the distal aorta and its major branches. The most common symptom is severe arterial hypertension. The combination of hyponatremia, polyuria and renovascular hypertension caused by a unilateral renal artery stenosis is described as hyponatremic hypertensive syndrome. We report a case of MAS with unilateral renal artery stenosis in a preterm female neonate. A pregnant woman at 34 weeks of gestation was referred with fast growing abdominal circumference and pain. The ultrasound revealed severe polyhydramnios and fetal myocardial hypertrophy. Within the first 48 hours of the neonatal period, the diagnosis of MAS was made. We conclude that symptomatic MAS, caused by unilateral renal artery stenosis, resulting in increased renin–angiotensin–aldosterone system activity and subsequent polyuria of the non-stenotic kidney, lead to clinically significant polyhydramnios.

  • congenital disorders
  • materno-fetal medicine
  • hypertension
  • pregnancy
  • neonatal and paediatric intensive care
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Footnotes

  • Contributors SA and MH conceived the presented idea. SA developed the theory and performed the literature search. SA wrote the manuscript with support from VU and AKA. All authors discussed the results and contributed to the final manuscript. MH supervised the project.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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