A case of vasculopathy of unknown etiology associated with fatal hydrops fetalis and review of the literature on intimomedial mucoid degeneration

Michael P. Zaleski, Youngmin Chu, Elizabeth Frauenhoffer, Jozef Malysz, Negar Rassaei, Catherine Abendroth, Erik Washburn, Wayne Ross, Francesca Ruggiero, Karmaine Millington

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1 Citation (Scopus)

Abstract

Non-immune hydrops fetalis (NIHF) has a high mortality rate [1]. Many etiologies of NIHF have been identified, including cardiovascular abnormalities, severe anemia, and genetic defects. In patients with cardiovascular etiology, structural malformations lead to fluid accumulation resulting in increased intravascular hydrostatic pressure. We report a fatal case of NIHF in a 31 week gestational age, Caucasian neonate with heart remodeling associated with a stenotic vasculopathy of the right pulmonary artery. The artery revealed partial occlusion with vascular wall abnormalities, including disarrayed smooth muscle fibers, hyperplasia within the tunica media, and myxoid change within the media and intima. Identical vasculopathy was also identified within a mesenteric artery, and this contributed to hemorrhage and early ischemic necrosis of the small intestine, discovered on postmortem examination.

Original languageEnglish (US)
Pages (from-to)39-44
Number of pages6
JournalCardiovascular Pathology
Volume33
DOIs
StatePublished - Mar 1 2018

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Hydrops Fetalis
Cardiovascular Abnormalities
Tunica Media
Hydrostatic Pressure
Mesenteric Arteries
Pulmonary Artery
Gestational Age
Small Intestine
Hyperplasia
Smooth Muscle
Blood Vessels
Anemia
Autopsy
Necrosis
Arteries
Newborn Infant
Hemorrhage
Mortality

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine
  • Cardiology and Cardiovascular Medicine

Cite this

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title = "A case of vasculopathy of unknown etiology associated with fatal hydrops fetalis and review of the literature on intimomedial mucoid degeneration",
abstract = "Non-immune hydrops fetalis (NIHF) has a high mortality rate [1]. Many etiologies of NIHF have been identified, including cardiovascular abnormalities, severe anemia, and genetic defects. In patients with cardiovascular etiology, structural malformations lead to fluid accumulation resulting in increased intravascular hydrostatic pressure. We report a fatal case of NIHF in a 31 week gestational age, Caucasian neonate with heart remodeling associated with a stenotic vasculopathy of the right pulmonary artery. The artery revealed partial occlusion with vascular wall abnormalities, including disarrayed smooth muscle fibers, hyperplasia within the tunica media, and myxoid change within the media and intima. Identical vasculopathy was also identified within a mesenteric artery, and this contributed to hemorrhage and early ischemic necrosis of the small intestine, discovered on postmortem examination.",
author = "Zaleski, {Michael P.} and Youngmin Chu and Elizabeth Frauenhoffer and Jozef Malysz and Negar Rassaei and Catherine Abendroth and Erik Washburn and Wayne Ross and Francesca Ruggiero and Karmaine Millington",
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T1 - A case of vasculopathy of unknown etiology associated with fatal hydrops fetalis and review of the literature on intimomedial mucoid degeneration

AU - Zaleski, Michael P.

AU - Chu, Youngmin

AU - Frauenhoffer, Elizabeth

AU - Malysz, Jozef

AU - Rassaei, Negar

AU - Abendroth, Catherine

AU - Washburn, Erik

AU - Ross, Wayne

AU - Ruggiero, Francesca

AU - Millington, Karmaine

PY - 2018/3/1

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N2 - Non-immune hydrops fetalis (NIHF) has a high mortality rate [1]. Many etiologies of NIHF have been identified, including cardiovascular abnormalities, severe anemia, and genetic defects. In patients with cardiovascular etiology, structural malformations lead to fluid accumulation resulting in increased intravascular hydrostatic pressure. We report a fatal case of NIHF in a 31 week gestational age, Caucasian neonate with heart remodeling associated with a stenotic vasculopathy of the right pulmonary artery. The artery revealed partial occlusion with vascular wall abnormalities, including disarrayed smooth muscle fibers, hyperplasia within the tunica media, and myxoid change within the media and intima. Identical vasculopathy was also identified within a mesenteric artery, and this contributed to hemorrhage and early ischemic necrosis of the small intestine, discovered on postmortem examination.

AB - Non-immune hydrops fetalis (NIHF) has a high mortality rate [1]. Many etiologies of NIHF have been identified, including cardiovascular abnormalities, severe anemia, and genetic defects. In patients with cardiovascular etiology, structural malformations lead to fluid accumulation resulting in increased intravascular hydrostatic pressure. We report a fatal case of NIHF in a 31 week gestational age, Caucasian neonate with heart remodeling associated with a stenotic vasculopathy of the right pulmonary artery. The artery revealed partial occlusion with vascular wall abnormalities, including disarrayed smooth muscle fibers, hyperplasia within the tunica media, and myxoid change within the media and intima. Identical vasculopathy was also identified within a mesenteric artery, and this contributed to hemorrhage and early ischemic necrosis of the small intestine, discovered on postmortem examination.

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