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Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
Published on: November 4, 2015
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Plexiform pulmonary arteriopathy in a 2 year-old boy
Sabah Boudjemaa1, Valerie Meau-Petit, Fazia Hallalel
1Department of Pathology, Hopital Armand Trousseau, PARIS, FRANCE.
Turk Patoloji Dergisi
|August 12, 2014
Summary
Idiopathic pulmonary arterial hypertension is a rare pediatric disease. Autopsy revealed characteristic arteriopathy lesions, prompting genetic testing for BMPR2 mutations in this fatal case.
Area of Science:
- Pediatric Cardiology
- Hematology
- Pathology
Background:
- Idiopathic pulmonary arterial hypertension (IPAH) is a rare and severe condition in children.
- Early diagnosis and treatment are crucial for managing pediatric IPAH.
- Understanding the underlying pathophysiology is key to improving outcomes.
Observation:
- A 2-year-old boy presented with severe dyspnea and epistaxis.
- Laboratory findings included hemolytic anemia with schizocytes and severe thrombocytopenia.
- Cardiac investigations revealed supra-systemic pulmonary arterial hypertension refractory to treatment.
Findings:
- The patient experienced cardiac arrests and died 8 days after admission.
- Autopsy confirmed idiopathic pulmonary hypertensive arteriopathy with plexiform lesions and microthrombi.
- Genetic analysis of BMPR2 was planned for the patient's family.
Implications:
- This case highlights the aggressive nature of pediatric IPAH.
- Autopsy findings provide critical insights into the disease's pathology.
- Family screening and genetic studies are essential for identifying potential hereditary factors.
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