Right Heart Catheterization (RHC): A Comprehensive Review of Provocation Tests and Hepatic Hemodynamics in Patients With Pulmonary Hypertension (PH).

The classic definition of pulmonary arterial hypertension (PAH) is a mean pulmonary artery pressure (mPAP) of 20 mmHg. The gold standard for assessing pulmonary hemodynamics is right heart catheterization (RHC), which is necessary to confirm the diagnosis of PH. In some instances, RHC evaluates the degree of hemodynamic dysfunction and performs vasoreactivity tests. Measurement of the hepatic venous pressure gradient remains the gold standard diagnostic for identifying portal hypertension. This review aims to describe the procedure of RHC and the hemodynamic measurement in patients with PAH and Portopulmonary hypertension (PoPH). The RHC remains the gold standard for diagnosing PAH and PoPH.

Novel and robust treatment of pulmonary hypertension with netrin-1 and netrin-1-derived small peptides.

Limited medical therapies have been implemented for the treatment of the devastating cardiorespiratory disease of pulmonary hypertension (PH) while none of which is sufficiently effective to stop or regress development of PH. We have previously shown that netrin-1, an axon-guiding protein during development, protects against ischemia reperfusion injury induced myocardial infarction via modest and stable production of nitric oxide (NO) and attenuation of oxidative stress. Since NO deficiency and oxidative stress-mediated vascular remodeling play important roles in the pathogenesis of PH, our present study investigated therapeutic effects on PH of netrin-1 and its derived small peptides. Infused into mice for 3 weeks during exposure to hypoxia, netrin-1 and netrin-1 derived small peptides V1, V2 or V3 substantially alleviated pathophysiological and molecular features of PH, as indicated by abrogated increases in mean pulmonary artery pressure (mPAP) and right ventricular systolic pressure (RVSP), attenuated right ventricular hypertrophy, diminished vascular remodeling of medial thickening and upregulation in smooth muscle alpha-actin (SMA) and proliferative cell nuclear antigen (PCNA), and alleviated perivascular and peribronchial fibrosis reflected by collagen deposition. NO bioavailability was substantially improved by treatment with netrin-1 and netrin-1 derived small peptides, while hypoxia induced increases in total superoxide production and eNOS uncoupling activity were all attenuated. These dual mechanisms of increasing NO bioavailability and decreasing oxidative stress at the same time, underlie robust protective effects on PH of netrin-1 and its derived small peptides, which are different from existing medications that primarily target NO signaling alone. Our data for the first time demonstrate intriguing findings that netrin-1 and netrin-1 derived small peptides can be used as novel and robust therapeutics for the treatment of PH.

Acute vasodilator testing: An opportunity to advance the precision care of pulmonary hypertension.

The pulmonary arterial pressure of a child with severe pulmonary arterial hypertension immediately normalized while breathing nitric oxide during heart catheterization at 8 years of age. Her acute pulmonary vascular response to nitric oxide has persisted throughout her life. Her acute response to other medications has been similar to her long-term response to medications in the same class. Acute vasodilator testing with inhaled nitric oxide and other medications may be an opportunity to refine study design and advance precision care for patients with pulmonary hypertension.

Frequency and predictors of pulmonary hypertension in patients with Systemic Lupus Erythematosus.

OBJECTIVE: To determine the frequency and predictors of pulmonary hypertension in patients with Systemic Lupus Erythematosus in a Pakistani population, presenting at a tertiary care hospital. METHODS: This cross-sectional study was conducted at the Department of Rheumatology, Shiekh Zayed Hospital, Lahore from March to June 2018. A total of 97 patients, who fulfilled the Systemic Lupus Erythematosus (SLE) criteria of American College of Rheumatology (ACR) 1992 were enrolled. Pulmonary Arterial Hypertension (PAH) was measured by calculating pulmonary arterial systolic pressure through echocardiography by a single consultant cardiologist. Disease characteristics and demography was collected in a self-administered proforma. PAH was defined as mean pulmonary arterial pressure of 25mmHg or above by calculating with a formula. SPSS version 20 was used for analysis of data. RESULTS: Out of 97 patients, 89.7% (n=87) were females and 10.3% (n=10) were males, with mean age of 31.29± 8.824 years. The mean disease duration was 24.21 ± 30.46 months. PAH was found in 23.3% (n=23) patients, including 19 females and 4 males. On further analysis of data, Raynaud phenomenon, rheumatoid factor and nephritis were assessed as predictors of PAH and all of these showed statistical significance for presence of PAH as per Chi-square test (p<0.05). CONCLUSION: In this study, 23.3% SLE patients showed evidence of PAH and positive statistical significance was found between predictors like Raynaud phenomenon, rheumatoid factor, nephritis and presence of PAH. So it is imperative to detect PAH early and start prompt treatment to achieve better quality of life.

Evaluation of therapeutic effects of spironolactone on schistosomal pulmonary arterial hypertension by echocardiography / 中国血吸虫病防治杂志

Objective To evaluate the therapeutic effect of spironolactone on schistosomal pulmonary arterial hypertension(SPAH). Methods A total of 62 patients suffered from hepatosplenic schistosomiasis with pulmonary arterial hypertension were divided into the spironolactone group(n=31) and control group (n=31). All the patients underwent serial echocardiography and the clinical effect before and after the treatment was evaluated by assessing the mean pulmonary arterial pressure (mPAP) and pulmonary arterial diameter (PAD). At the same time, the varieties of the clinical symptoms, signs and the distance of the 6-minute walking test (6-MWT) were investigated. Results In spironolactone group, mPAP(-x±s) decreased from (31.8±7.1) mmHg to (21.2±2.1) mmHg, PAD(-x±s) decreased from (28.0±5.0) mm to (20.0±3.5) mm before and after the treatment respectively(P<0.01). There were significant differences in mPAP, PAD, the distance of 6-MWT and the heart function before and after the treatment in the spironolactone group. However, the data did not show the significant difference in the control group. Conclusion The therapeutic effect of spironolactone in the treatment of SPAH is satisfactory.