It is unclear whether pregnancy is a trigger or accelerant for idiopathic pulmonary arterial hypertension (PAH). Alternatively, its frequency close to the onset of symptoms and diagnosis in the idiopathic PAH population may represent a coincidence in a disease that predominates in young women. We describe a carrier of a BMPR2 gene mutation who had an uneventful first pregnancy but had aggressive PAH during her second pregnancy and now has ongoing heritable PAH. The possible role of pregnancy as a trigger in this vulnerable patient is discussed. Databases of patients with heritable PAH should be explored to see whether pregnancy is related to overt manifestation of the disease.
WeirEK. Diagnosis and management of primary pulmonary hypertension. In: WeirEK, ReevesJT, eds. Pulmonary hypertension. Mount Kisco, NY: Futura, 1984: 115–168.
3.
RichS, DantzkerDR, AyresSM, BergofskyEH, BrundageBH, DetreKM, FishmanAP. Primary pulmonary hypertension: A national prospective study. Ann Intern Med1987; 107: 216–223.
4.
SimonneauG, GatzoulisMA, AdatiaI, CelermajerD, DentonC, GhofraniA, SanchezMA Gomez. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol2013;62(25 suppl): D34–D41.
5.
GrunigE, WeissmannS, EhlkenN, FijalkowskaA, FischerC, FourmeT, GaliéN. Stress Doppler echocardiography in relatives of patients with idiopathic and familial pulmonary arterial hypertension: Results of a multicenter European analysis of pulmonary artery pressure response to exercise and hypoxia. Circulation2009; 119(13): 1747–1757.
6.
NahapetianA, OudizRJ. Serial hemodynamics and complications of pregnancy in severe pulmonary arterial hypertension. Cardiology2008; 109(4): 237–240.
7.
HemnesAR, KielyDG, CockrillBA, SafdarZ, WilsonVJ, Al HazmiM, PrestonIR, MacLeanMR, LahmT. Statement on pregnancy in pulmonary hypertension from the Pulmonary Vascular Research Institute. Pulm Circ2015; 5(3): 435–465.
8.
AustinED, CoganJD, WestJD, HedgesLK, HamidR, DawsonEP, WheelerLA, ParlFF, LoydJE, PhillipsJA3rd.Alterations in oestrogen metabolism: Implications for higher penetrance of familial pulmonary arterial hypertension in females. Eur Respir J2009; 34(5): 1093–1099.
9.
MairKM, YangXD, LongL, WhiteK, WallaceE, EwartMA, DochertyCK, MorrellNW, MacLeanMR. Sex affects bone morphogenetic protein type II receptor signaling in pulmonary artery smooth muscle cells. Am J Respir Crit Care Med2015; 191(6): 693–703.
10.
FesselJP, ChenX, FrumpA, GladsonS, BlackwellT, KangC, JohnsonJ. Interaction between bone morphogenetic protein receptor type 2 and estrogenic compounds in pulmonary arterial hypertension. Pulm Circ2013; 3(3): 564–577.
