The risk
of pregnancy-related death in women with PAH is substantial with risk
of dying reported to be 30 to 50%. For this reason alone, pregnancy
is contraindicated in patients with PAH. In addition, warfarin (Coumadin)
which is teratogenic (harmful) to the developing fetus, is used by most
PAH patients, and adds to the risk of pregnancy. Because of the risk
to both the patient and the fetus, use of some form of birth control
and avoidance of pregnancy is strongly advised in women of childbearing
age with PAH.
There
are no published guidelines for birth control use in PAH, and there
is no consensus regarding the best form of birth control. The two safest
methods of birth control are 1) the barrier method which may include
condoms in men and/or a diaphragm with spermacide in women, and 2) a
vasectomy in the male partner for woman with PAH in a monogamous (one
partner) relationship. The failure rate in preventing pregnancy with
barrier methods, when used properly is quite low. Tubal ligation was
felt by many PH specialists to be an acceptable option in patients who
were not in severe heart failure. In the sickest patients, consideration
of other birth control methods is recommended.
In a survey
of 23 PH specialists from North America and Europe, the majority felt
that use of estrogen-containing birth control pills (BCP) was acceptable
as long as patients were anticoagulated with warfarin. Birth control
pills containing the lowest amount of estrogen are recommended. However,
nearly half of the specialists did not advocate using BCP for their
patients, and some actively discouraged patients from doing so because
of concern over the possible role of estrogen in worsening PAH. Sixty-five
percent of specialists indicated that use of hormonal therapy in
post-menopausal
women with PAH was acceptable. Progesterone alone, taken orally (by
mouth), can be as effective as estrogen in combination with progesterone
in preventing pregnancy. However, when using progesterone alone,
the
pills must be taken at the same time every day to be effective. Intramuscular
injections of progesterone (Depo-Proveraâ) is another option,
however this is often accompanied by fluid retention which may be
harmful
to patients with right heart failure. There is also a 20 to 30% incidence
of irregular, heavy menstrual bleeding associated with the use of
progesterone
injections that may be worsened by the use of warfarin. Furthermore,
intramuscular injections may be associated with localized bleeding
in patients receiving warfarin.
A potential interaction exists between endothelin receptor antagonists
such as bosentan (Tracleer) and hormonal birth control, although no
studies have demonstrated this to date. Women should not rely on hormonal
birth control alone when taking bosentan. Also, many antibiotics can
decrease the effectiveness of the hormonal forms of birth control.
Thus,
if a woman with PAH requires antibiotics, an additional method of
contraception should be used during the entire menstrual cycle until
the next period
occurs.
Intrauterine
devices (IUDs) can be used for birth control, although this is not
recommended as the first choice for birth control. Traditional IUDs
may be associated
with excessive bleeding at the time of menses that may be exacerbated
by the use of warfarin. The device should be removed if excessive
bleeding
occurs. A newer IUD that releases progesterone (Mirenaâ) has
a lower risk of bleeding (and has been used to treat excessive menstrual
bleeding) and may be an option for some patients. Prophylactic antibiotic
use should be used at the time of insertion and removal in most patients
with PAH related to congenital heart disease.
The use
of surrogate mothers (women who have fertilized eggs inserted into their
uterus to carry a fetus), is an option that some PH specialists have
considered for selected patients, although this has been done in only
a few instances. There are many ethical, practical, and medical issues
associated with the use of surrogate mothers for PAH patients that should
be discussed not only with treating physicians but also with experienced
reproductive counselors.
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