Diagnosis and Management of Pulmonary Arterial Hypertension

Abstract

Pulmonary arterial hypertension is a rare disease, which requires a high index of suspicion to diagnose when patients initially present. Initial symptoms can be nonspecific and include complaints such as fatigue and mild dyspnea. Once the disease is suspected, echocardiography is used to estimate the pulmonary arterial (PA) pressure and to exclude secondary causes of elevated PA pressures such as left heart disease. Right heart catheterization with vasodilator challenge is critical to the proper assessment of pulmonary hemodynamics and to determine whether patients are likely to benefit from vasodilator therapy. Pathologically, the disease is characterized by deleterious remodeling of the distal pulmonary arterial and arteriolar circulation, which results in increased pulmonary vascular resistance. In the last fifteen years, medications from three different classes have been approved for the treatment of pulmonary arterial hypertension. These include the prostanoids, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors.

The challenge of breathlessness in the detection of pulmonary hypertension

“…Defined by an elevated resting mean pulmonary artery pressure .25 mmHg, PH is a complex, multifactorial group of disorders that leads to progressive right ventricular failure [14][15][16]. As well as breathlessness, presenting symptoms include fatigue, weakness, angina pectoris, syncope and abdominal distension [17]. While very few patients with dyspnoea consulting their general practitioner or referred to the pulmonology outpatient clinic will be diagnosed with PH, early diagnosis is so important that it must be always considered in the differential diagnosis.…”

“…Consequently, misdiagnosis of PH as asthma is fairly common. In addition, patients may interpret an insidious onset of breathlessness and fatigue as being due to their lifestyle, being unfit or ageing, and so delay seeking medical attention until their daily activities are severely impaired [17]. Thus, diagnosis is often delayed by at least 2 yrs after the onset of symptoms [21,22].…”

“…While some clinicians rely on an echocardiogram to make a diagnosis of PAH because it is less invasive, it is necessary to demonstrate that the elevation in pulmonary artery pressure is due to increased pulmonary venous resistance, which can only be achieved by catheterisation [17]. It is also important to be aware that the resting physiological range of pulmonary artery pressure observed by echocardiography is dependent on age, sex and body mass index, with higher pressures often observed in older or obese individuals [10].…”

Obesity Hypoventilation Syndrome and Pulmonary Hypertension Case with Excessive Daytime Sleepiness

“…Multiple studies have shown a higher prevalence of SRBD in patients with pulmonary hypertension as well as an increased prevalence of pulmonary hypertension in patients with SRDB (17% to 53%); and factors such as daytime PO 2 , BMI, and AHI are significantly associated with both [3].…”

“…In 2008 PH update; classified SRBD in group 3 as PH due lung diseases and hypoxia [2]. Pulmonary hypertension defined by a mean pulmonary artery pressure exceeding 25 mmHg on right heart catheterization, Is more common (50% vs. 15%) and more severe in patients with OHS than in patients with OSAS [3][4][5][6][7].…”