Pulmonary Hypertension in the Population with Down Syndrome

Douglas S. Bush, D. Dunbar Ivy

Research output: Contribution to journalReview articlepeer-review

3 Scopus citations


Persons with Down syndrome (DS) have an increased reported incidence of pulmonary hypertension (PH). A majority of those with PH have associations with congenital heart disease (CHD) or persistent pulmonary hypertension of the newborn (PPHN); however, there are likely multifactorial contributions that include respiratory comorbidities. PH appears to be most commonly identified early in life, although respiratory challenges may contribute to a later diagnosis or even a recurrence of previously resolved PH in this population. Currently there are few large-scale, prospective, lifetime cohort studies detailing the impact PH has on the population with DS. This review will attempt to summarize the epidemiology and characteristics of PH in this population. This article will additionally review current known and probable risk factors for developing PH, review pathophysiologic mechanisms of disease in the population with DS, and evaluate current screening and management recommendations while suggesting areas for additional or ongoing clinical, translational, and basic science research.

Original languageEnglish
Pages (from-to)33-47
Number of pages15
JournalCardiology and Therapy
Issue number1
StatePublished - Mar 2022


  • Congenital heart disease
  • Down syndrome
  • Pulmonary arterial hypertension
  • Pulmonary hypertension
  • Trisomy 21


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