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Segmental Pulmonary Hypertension in Children with Congenital Heart Disease

Bibhuti B Das1, Benjamin Frank2, Dunbar Ivy2

  • 1Department of Pediatric Cardiology, Baylor College of Medicine, Texas Children's Hospital Austin Specialty Care, Austin, TX 78759, USA.

Medicina (Kaunas, Lithuania)
|September 29, 2020
PubMed

Insights

Segmental pulmonary hypertension in children, often linked to congenital heart defects, requires advanced imaging for diagnosis. Management involves interventional and targeted therapies for improved outcomes.

Area of Science:

  • Pediatric Cardiology
  • Cardiovascular Imaging
  • Pulmonary Hypertension

Background:

  • Segmental pulmonary hypertension (PH) is a complex pediatric condition.
  • It is associated with various congenital heart diseases (CHDs) and major aorto-pulmonary collaterals.
  • Accurate assessment of PH requires multimodality imaging.

Purpose of the Study:

  • To describe cases of segmental PH in children with complex CHDs.
  • To discuss management strategies, including interventional and targeted therapies.
  • To highlight the role of imaging in diagnosing and assessing PH in this population.

Main Methods:

  • Case series of 3 children with complex congenital heart defects and segmental PH.
  • Multimodality imaging for assessment of pulmonary vascular disease.
  • Review of management strategies and therapeutic interventions.

Main Results:

  • Children with complex CHDs presented with variable degrees of segmental PH.
  • Multimodality imaging was crucial for evaluating pulmonary vascular disease.
  • Management involved a combination of interventional procedures and targeted therapies.

Conclusions:

  • Segmental PH in children with complex CHDs is a challenging clinical entity.
  • A tailored approach combining advanced imaging, interventional strategies, and PH-targeted therapies is essential.
  • Further research into optimal management protocols is warranted.

Keywords:
congenital heart diseasedigital subtraction angiographypulmonary hypertension in childrensegmental pulmonary hypertension

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