Abstract
Pulmonary hypertension (PH) is recognized to be associated with a number of comorbid conditions. Based on these associations, PH is classified into 5 groups, considering common pathophysiologic drivers of disease, histopathologic features, clinical manifestations and course, and response to PH therapy. However, in some of these associated conditions, these characteristics are less well-understood. These include, among others, conditions commonly encountered in clinical practice such as sarcoidosis, sickle cell disease, myeloproliferative disorders, and chronic kidney disease/end stage renal disease. PH in these contexts presents a significant challenge to clinicians with respect to disease management. The most recent updated clinical classification schemata from the 6th World Symposium on PH classifies such entities in Group 5, highlighting the often unclear and/or multifactorial nature of PH. An in-depth review of the state of the science of Group 5 PH with respect to epidemiology, pathogenesis, and management is provided. Where applicable, future directions with respect to research needed to enhance understanding of the clinical course of these entities is also discussed.
Highlights
Pulmonary hypertension (PH) is characterized by elevated mean pulmonary artery pressure of >20 mmHg as determined by right heart catheterization (RHC)
Patients with SCD, for example, may have recurrent chest pains, fatigue, and chronic shortness of breath. These symptoms can be related to anemia, acute chest syndrome, obstructive airway disease, thromboembolism, and/or PH [30,31,32]
Asymptomatic patients remain at greater risk of developing PH; screening with Doppler echocardiography is recommended
Summary
Pulmonary hypertension (PH) is characterized by elevated mean pulmonary artery pressure (mPAP) of >20 mmHg as determined by right heart catheterization (RHC). According to the new classification, causes of PH include pulmonary arterial hypertension (PAH, Group 1 PH), pulmonary venous hypertension due to elevated filling pressure of the left heart (Group 2 PH), PH due to chronic pulmonary disease or hypoxemia (Group 3 PH), chronic thromboembolic disease (Group 4), whereas PH that develops due to multiple or unclear mechanisms is referred to as Group 5 PH (Table 1). Within Group 5, several clinical disorders are implicated in association with the development of PH (Table 2). 1.1 Idiopathic PAH 1.2 Heritable PAH 1.3 Drug- and toxin-induced PAH 1.4 PAH associated with: 1.4.1 Connective tissue disease 1.4.2 HIV infection 1.4.3 Portal hypertension 1.4.4 Congenital heart disease 1.4.5 Schistosomiasis 1.5 PAH long-term responders to CCB 1.6 PAH with overt signs of venous/capillaries (PVOD/PCH) involvement 1.7 Persistent PH of the Newborn syndrome
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