PH Grand Rounds: Confronting the Challenge of Sarcoidosis-Associated Pulmonary Hypertension

Sarcoidosis Associated Pulmonary Hypertension

Hypertension Grand Rounds Gitelman Syndrome

Gitelman syndrome (GS) is an autosomal-recessive renal tubular disorder characterized by hypokalemia, hypomagnesemia, hypocalciuria, metabolic alkalosis, secondary hyperreninemic aldosteronism, and low blood pressure.1–3 GS patients are usually diagnosed relatively late, because malaise, low blood pressure, hypokalemia, hypocalciuria, and hypomagnesemia are difficult to categorize clinically. I...

Pulmonary hypertension associated with sarcoidosis

Pulmonary hypertension (PH) affects approximately 6% of unselected patients with sarcoidosis but its prevalence is much higher in advanced pulmonary fibrosis. Although destruction of the distal capillary bed and resultant hypoxaemia are central, the underlying mechanisms are multifactorial: left heart dysfunction, specific pulmonary vasculopathy (which can cause pulmonary veno-occlusive disease...

Pulmonary hypertension associated with sarcoidosis

Pulmonary involvement is common in sarcoidosis, an immune-mediated inflammatory disorder that is characterized by non-caseating granulomas in tissue. Sarcoid patients with advanced pulmonary disease, especially end-stage pulmonary fibrosis, risk developing pulmonary hypertension (World Health Organization group III pulmonary hypertension secondary to hypoxic lung disease). Increased levels of e...

Thalassemia Associated Pulmonary Hypertension

Cardiac disease is the main cause of death in both forms of thalassemia; thalassemia major (TM) and thalassemia intermedia (TI). Pulmonary hypertension (PH) is one of the cardiopulmonary morbidities with high mortality that, if not treated, may trigger right-sided heart failure and premature death. PH is defined as a mean pulmonary artery pressure of ≥25 mmHg at rest or ≥30 mmHg during exercise...