High prevalence of respiratory ciliary dysfunction in congenital heart disease patients with heterotaxy.

ABSTRACT:

Background

Patients with congenital heart disease (CHD) and heterotaxy show high postsurgical morbidity/mortality, with some developing respiratory complications. Although this finding is often attributed to the CHD, airway clearance and left-right patterning both require motile cilia function. Thus, airway ciliary dysfunction (CD) similar to that of primary ciliary dyskinesia (PCD) may contribute to increased respiratory complications in heterotaxy patients.

Methods and results

We assessed 43 CHD patients with heterotaxy for airway CD. Videomicrocopy was used to examine ciliary motion in nasal tissue, and nasal nitric oxide (nNO) was measured; nNO level is typically low with PCD. Eighteen patients exhibited CD characterized by abnormal ciliary motion and nNO levels below or

SUBMITTER: Nakhleh N 

PROVIDER: S-EPMC3770728 | biostudies-literature | 2012 May

REPOSITORIES: biostudies-literature