title: The mystery of persistent pulmonary hypertension: an idiopathic infantile arterial calcification.
abstract: BACKGROUND
  Idiopathic infantile arterial calcification (IIAC) is a rare autosomal recessive disorder, characterized by wide spread calcifications in arterial walls, leading to vaso-occlusive ischaemia of multiple organs. Mortality is high, and there is no definitive treatment.
  
  
  METHODS
  A male neonate, 36+5 weeks gestation, 2.81 kg, was admitted to NICU for respiratory distress. At one hour of age, he was noted to be pale, hypoperfused, with weak pulses, a hyperdynamic precordium and a grade IV/VI pansystolic murmur. The rest of his examination was normal. A chest X-ray showed massive cardiomegaly and pulmonary oedema. An echocardiogram (ECHO) indicated moderate persistent pulmonary hypertension (PPHN) of unclear etiology. A diagnosis of Idiopathic infantile arterial calcification was made and a trial of Editronate therapy was given without success.
  
  
  CONCLUSIONS
  IIAC is a rare disorder, it should be considered whenever a neonate presents with unexplainable cardiac failure, PPHN, echogenic vessels on X-ray/ultrasound and, or concentric hypertrophic ventricles on ECHO. Serial antenatal ultrasound findings of echogenic cardiac foci should raise the suspicion of IIAC. Further studies to determine the long term effects of Editronate on vascular calcifications, disease outcome, and other treatment options are needed.
authors.affiliation: Section of Neonatology, Department of Pediatrics, University of Calgary, Calgary, Alberta, Canada.
authors.forename: Huma
authors.initials: H
authors.lastname: Shaireen
authors.forename: Alexandra
authors.initials: A
authors.lastname: Howlett
authors.forename: Harish
authors.initials: H
authors.lastname: Amin
authors.forename: Kamran
authors.initials: K
authors.lastname: Yusuf
authors.forename: Majeeda
authors.initials: M
authors.lastname: Kamaluddeen
authors.forename: Abhay
authors.initials: A
authors.lastname: Lodha
mesh_terms: D017809:Fatal Outcome; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology*; D007231:Infant, Newborn; D008297:Male; D061205:Vascular Calcification / Q000150:complications / Q000175:diagnosis*