Severe pulmonary hypertension in pulmonary alveolar microlithiasis: A comprehensive literature review.

This review focuses on Pulmonary Alveolar Microlithiasis (PAM), an autosomal recessive genetic disorder characterized by calcium crystal deposits (microliths) resulting from loss of function of the SLC34A2 gene. PAM is a rare disease with approximately 1100 reported cases globally. The historical context of its discovery and the genetic, epidemiological, and pathophysiological aspects are discussed. PAM falls under interstitial lung diseases and is associated with pulmonary hypertension (PH), primarily categorized as Group 3 PH. The clinical manifestations, diagnostic approaches, and challenging aspects of treatment are explored. A clinical case of PAM with severe pulmonary hypertension is presented, emphasizing the importance of comprehensive evaluation and the potential benefits of phosphodiesterase-5 inhibitors (PDE5i) therapy. Despite limited therapeutic options and challenging diagnosis, this review sheds light on recent developments and emerging treatments for PAM and associated pulmonary hypertension.

Prevalence and Prognosis of Hypothyroidism in Patients With Chronic Thromboembolic Pulmonary Hypertension: An Updated Review With Description of Case Series.

Hypothyroidism has been shown to have several effects on organs, including derangements in the coagulation system, impairing endothelial function, but data on the importance of hypothyroidism in the pathogenesis and development of chronic thromboembolic pulmonary hypertension (CTEPH) are limited. This report presents an updated review of the prevalence and prognosis of hypothyroidism in patients diagnosed with CTEPH, including a detailed retrospective description of the series. The descriptive case series included 34 adult patients diagnosed with CTEPH, of whom 11 patients were diagnosed with hypothyroidism. The prevalence of hypothyroidism in CTEPH was found to be 32.35%. All patients with hypothyroidism had NYHA functional Class II-III. Hemodynamic values obtained through right heart catheterization (RHC) showed that patients with hypothyroidism had significantly higher mean pulmonary arterial pressures (mPAP), with a mean of 56.91 mm Hg vs 43.93 mm Hg (p = 0.026), and the PVR in dynes/sec/cm5 was 932 vs 541 (p = 0.027). Significant differences in PVR were found in wood units (WU) 11.91 vs 7.11 (p = 0.042). The mean level of brain natriuretic peptide (BNP) between both groups was 797.3 pg/mL for patients with hypothyroidism vs 262.02 pg/mL in patients with euthyroidism (p = .032). Hypothyroidism may significantly affect patients' clinical and hemodynamic outcomes in patients with CTEPH. Hypothyroidism as a risk factor in the evaluation and treatment of these patients is vital to optimize outcomes in CTEPH; further research is warranted whether hypothyroidism therapies could alter such outcomes.