Related Experiment Video
Updated: Apr 26, 2026

Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
Published on: November 4, 2015
Plexiform pulmonary arteriopathy in a 2 year-old boy
Sabah Boudjemaa1, Valerie Meau-Petit, Fazia Hallalel
1Department of Pathology, Hopital Armand Trousseau, PARIS, FRANCE.
Abstract:
Idiopathic pulmonary arterial hypertension is a rare disease in children. We report a case of a 2-year old boy admitted to the intensive care unit of our hospital for severe dyspnea and epistaxis. Laboratory investigations showed hemolytic anemia with schizocytes and severe thrombocytopenia. Cardiac investigations diagnosed supra-systemic pulmonary arterial hypertension, which was refractory to maximal medical treatment. On evolution, he had several cardiac arrests and finally died 8 days after admission. Autopsy was performed and showed typical lesions of idiopathic pulmonary hypertensive arteriopathy characterized by plexiform lesions of the interlobular arteries containing numerous disseminated intravascular microthrombi. The rest of the family was screened, DNA was stored, and genetic study of BMPR2 was planned.
Related Concept Videos
Pulmonary Hypertension: Classification and Pathogenesis
There are various classifications for PH, each relating to different underlying causes and also...
Pulmonary Tuberculosis II
Here is a detailed explanation of its pathophysiology:
Transmission: The process begins when a person inhales droplet nuclei containing M. tuberculosis. These are typically released into the air when an individual with pulmonary or...
Chronic Obstructive Pulmonary Disease II: Emphysema
Atypical Pneumonia
Other Pulmonary Disorders
Chronic Obstructive Pulmonary Disease-II: Pathophysiology
Chronic Inflammation

