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Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
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Chronic Thromboembolic Pulmonary Hypertension in a Child With Sickle Cell Disease

Robert Spencer1, Gerson Valencia Villeda2, Koji Takeda3

  • 1Division of Pediatric Cardiology, Morgan Stanley Children's Hospital, New York, NY, United States.

Frontiers in Pediatrics
|August 28, 2020
PubMed

Insights

Chronic thromboembolic pulmonary hypertension (CTEPH) is a curable condition. A case study shows an 11-year-old with sickle cell disease successfully treated with surgery, highlighting the need for increased awareness in high-risk pediatric patients.

Area of Science:

  • Cardiology
  • Hematology
  • Pulmonology

Background:

  • Chronic thromboembolic pulmonary hypertension (CTEPH) is a potentially curable pre-capillary pulmonary hypertension (PH) caused by unresolved pulmonary emboli.
  • Sickle cell disease (SCD) is a risk factor for developing PH, though CTEPH in this population is underdiagnosed.

Observation:

  • An 11-year-old boy with homozygous sickle cell disease and an indwelling catheter presented with severe PH detected via routine echocardiography.
  • Diagnostic workup included CT scans, ventilation-perfusion scintigraphy, and right heart catheterization to confirm the diagnosis.

Findings:

  • The patient received medical management followed by a successful pulmonary thromboendarterectomy.
  • Post-surgery, the patient experienced complete resolution of his pulmonary hypertension.

Implications:

  • This case underscores the importance of recognizing CTEPH in pediatric patients, especially those with risk factors like sickle cell disease.
  • Pediatric healthcare providers should consider screening for CTEPH in at-risk children to enable timely diagnosis and treatment.

Keywords:
CTEPH—chronic thromboembolic pulmonary hypertensionhematologypediatric cardiologypulmonary hypertensionsickle cell disease

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