Introduction
Kartagener’s syndrome (KS) is an autosomal recessive genetic disorder
characterized by a triad syndrome: chronic sinusitis, bronchiectasis and
situs inversus. KS results from congenital ciliary dysfunctions and has
an incidence of 1 in 25,000 [1]. Congenital ciliary dysfunctions in
the respiratory tract, fallopian tube and flagella of spermatozoa can
cause chronic or recurrent upper respiratory diseases and infertility.
Gene mutations such as DNAI1 and DNAH5 have been indicated
in KS patients [2]. Therapeutic strategies in KS patients are
primarily focused on the control of respiratory infection, and lung
transplantation is an option for end-stage lung disease.
Females with KS are not necessarily infertile, although males with KS
are infertile due to abnormal sperm motility [1]. Previous reports
have presented courses of pregnancies in females with KS without
aggravation of the lung disease after natural conception [3] as well
as in vitro fertilization and embryo transfer (IVF-ET) [4, 5].
Regarding pregnancy following lung transplantation, careful management
is essential for contraception, timing of conception, maintenance of
immunosuppression, adverse perinatal complications and risk of
breastfeeding by neonatal exposure to immunosuppressants [6].
In this report, we describe a case of successful pregnancy in a patient
with KS with DNAH5 mutations who had been offered an indication
for lung transplantation before conception, despite aggravation of the
lung disease during pregnancy, by a multidisciplinary approach.