Primary pulmonary hypertension in pregnancy

Abstract

Primary pulmonary hypertension (PPH) is an uncommon but serious disease. Most patients with PPH are young women and the disease is more serious and eventful in pregnant women. We have experienced a patient with PPH in pregnancy, who was delivered successfully but died suddenly on the 7th day after the delivery. We report the obstetric course and the clinical management for the delivery of the patient with PPH.

Primary pulmonary hypertension and pre‐eclampsia: a successful pregnancy

“…A maternal mortality of approximately 50% was described in 1964 (McCaffrey & Dunn 1964) in the largest review published. Based upon recently reported cases (Nelson et al 1988; Robinson & Leicht 1988; Takeuchi et al 1988; Atanasoff et al 1989), this high figure has not substantially improved over the last three decades. With a risk of this magnitude it is reasonable to advise women affected by this condition against starting or continuing a pregnancy.…”

“…Though low birthweight for gestational age has been quite frequently described in fetuses of mothers with primary pulmonary hypertension (Takeuchi et al 1988; Leduc et al 1990) no special attention has been drawn to this issue. Inadequate blood supply to the uterine circulation may be responsible for fetal growth retardation.…”

“…Vaginal delivery for pregnant women with primary pulmonary hypertension has been preferred in a majority of the recently reported cases (Nelson et al 1988; Robinson & Leicht 1988; Takeuchi et al 1988; Atanasoff et al 1989; Leduc et al 1990). However, excessive duration of labour may be associated with important haemodynamic alterations (Walcott et al 1970; Metcalfe et al 1981) and sudden increases of pulmonary arterial pressure with right ventricular failure and subsequent left failure have been described (Robinson & Leicht 1988; Takeuchi et al 1988; Leduc et al 1990). Our patient was in her 35th week of gestation with unfavourable cervical conditions and a rapid labour was not warranted; a short and controlled procedure, such as caesarean section, was therefore preferred.…”

Birth Control and Pregnancy Management in Pulmonary Hypertension

“…Several case reports or case series and one prospective registry have described the successful use of contemporary PAH therapy in this patient population. 9,10,17,[41][42][43][44][45][46][47][48][49][50][51][52][53][54][55][56] Increased mortality has been associated with both severe PH and late diagnosis 6 ; therefore, early treatment with PAH therapies is indicated.…”

Pulmonary hypertension and pregnancy: a series of eight cases

“…Since 1982 eight cases monitored with PAC have been reported. 7,8,20,[22][23][24][25][26] Only two patients survived giving a mortality rate of 75%. Roberts et al concluded that the outcome had not improved in parturients with severe pulmonary hypertension, despite the introduction of invasive cardiovascular monitoring and aggressive circulatory management.…”