Abstract
Pulmonary hypertension (PH) is a disease, which targets the pulmonary vasculature affecting the heart and the lungs, and is characterized by a vast array of signs and symptoms. These manifestations of PH in pregnancy are highly variable and non-specific hence, it is prudent to have a very keen and high index of suspicion while evaluating these patients. This rare disease can be extremely debilitating and can be associated with a poor overall prognosis. Pregnancy in women with PH puts them at an elevated risk because the physiological changes associated with pregnancy are not well endured leading to even higher morbidity and mortality in these patients. Although there are various modalities for evaluation and workup of PH, right heart catheterization (RHC) remains the gold standard. A mean pulmonary artery pressure (PAP) of more than 20 mm of Hg is considered diagnostic. It is indeed heartening to see that in the past decade many novel therapeutic modalities have emerged and along with a better understanding of the disease process have proved to be promising in terms of reducing the adverse outcomes and preventing death in this population of patients.
Highlights
Pulmonary arterial hypertension (PAH), which is classified as group 1 PH (Pulmonary hypertension) is defined as an increase in pulmonary artery pressure (PAP) >20 mm of Hg at rest accompanied by an elevated pulmonary vascular resistance (PVR) of >3 Wood units as assessed by right heart catheterization (RHC) as per the sixth World Symposium on Pulmonary Hypertension [1]
As the pressure increases in the pulmonary vasculature, compensatory mechanisms come into play and cause the pulmonary arteries to become constricted and stiff due to abnormal endothelium, which causes a mild increase in the PVR and subsequently a moderate decrease in perfusion
After reviewing the basic physiological changes associated with pregnancy and pathophysiology of PH it should not be difficult to determine that the most pressing challenge in patients with PH during pregnancy is the reduced ability to accommodate the significant changes that occur in the cardiovascular and pulmonary system due to a compromised vascular bed
Summary
Pulmonary arterial hypertension (PAH), which is classified as group 1 PH (Pulmonary hypertension) is defined as an increase in PAP >20 mm of Hg at rest accompanied by an elevated pulmonary vascular resistance (PVR) of >3 Wood units as assessed by RHC as per the sixth World Symposium on Pulmonary Hypertension [1]. As the pressure increases in the pulmonary vasculature, compensatory mechanisms come into play and cause the pulmonary arteries to become constricted and stiff due to abnormal endothelium, which causes a mild increase in the PVR and subsequently a moderate decrease in perfusion. This leads to a hypertrophied right ventricle with normal CO. Further deterioration leads to more cellular proliferation in the wall of the pulmonary arteries leading to obliterative modeling, which in turn leads to an increase in PVR and a decrease in perfusion.
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