Epoprostenol-associated ascites in pulmonary arterial hypertension.

The development of ascites in pulmonary arterial hypertension (PAH) in the absence of pre-existing hepatic dysfunction is usually associated with decompensated right heart failure or cardiac cirrhosis. Ascites in PAH has rarely been associated with intravenous epoprostenol, a synthetic form of the prostaglandin PGI2.

Diagnosis and Treatment of Pulmonary Arterial Hypertension: A Review.

Importance: Pulmonary arterial hypertension (PAH) is a subtype of pulmonary hypertension (PH), characterized by pulmonary arterial remodeling. The prevalence of PAH is approximately 10.6 cases per 1 million adults in the US. Untreated, PAH progresses to right heart failure and death. Observations: Pulmonary hypertension is defined by a mean pulmonary artery pressure greater than 20 mm Hg and is classified into 5 clinical groups based on etiology, pathophysiology, and treatment. Pulmonary arterial hypertension is 1 of the 5 groups of PH and is hemodynamically defined by right heart catheterization demonstrating a mean pulmonary artery pressure greater than 20 mm Hg, a pulmonary artery wedge pressure of 15 mm Hg or lower, and a pulmonary vascular resistance of 3 Wood units or greater. Pulmonary arterial hypertension is further divided into subgroups based on underlying etiology, consisting of idiopathic PAH, heritable PAH, drug- and toxin-associated PAH, pulmonary veno-occlusive disease, PAH in long-term responders to calcium channel blockers, and persistent PH of the newborn, as well as PAH associated with other medical conditions including connective tissue disease, HIV, and congenital heart disease. Early presenting symptoms are nonspecific and typically consist of dyspnea on exertion and fatigue. Currently approved therapy for PAH consists of drugs that enhance the nitric oxide-cyclic guanosine monophosphate biological pathway (sildenafil, tadalafil, or riociguat), prostacyclin pathway agonists (epoprostenol or treprostinil), and endothelin pathway antagonists (bosentan and ambrisentan). With these PAH-specific therapies, 5-year survival has improved from 34% in 1991 to more than 60% in 2015. Current treatment consists of combination drug therapy that targets more than 1 biological pathway, such as the nitric oxide-cyclic guanosine monophosphate and endothelin pathways (eg, ambrisentan and tadalafil), and has shown demonstrable improvement in morbidity and mortality compared with the previous conventional single-pathway targeted monotherapy. Conclusions and Relevance: Pulmonary arterial hypertension affects an estimated 10.6 per 1 million adults in the US and, without treatment, typically progresses to right heart failure and death. First-line therapy with drug combinations that target multiple biological pathways are associated with improved survival.

Intraoperative changes and prognostic implications of pulmonary hypertension in patients with hypertrophic obstructive cardiomyopathy undergoing surgical septal myectomy.

OBJECTIVES: Patients with hypertrophic cardiomyopathy often have concomitant pulmonary hypertension, which has a negative prognostic effect in patients undergoing myectomy. Our objective was to investigate the effect of myectomy on pulmonary artery pressure obtained via Swan-Ganz catheter and characterize how changes in pulmonary artery systolic pressure may indicate outcomes in these patients. METHODS: We performed a single-center retrospective analysis of 271 patients with recordings of intraoperative pulmonary artery pressures during surgical myectomy. We analyzed primary composite outcomes as 30-day or in-hospital major cardiopulmonary adverse events. RESULTS: There was a 5.17% adverse event rate. Patients with adverse events were older, were more likely to be female, had chronic obstructive pulmonary disease, and had longer cardiopulmonary bypass times. Some 35.7% of those with adverse events had moderate to severe pulmonary hypertension (pulmonary artery systolic pressure ≥50 mm Hg) on postbypass stress test, compared with 4.3% of those without adverse events (P < .001). Further, 21.4% of patients with adverse events had pulmonary artery systolic pressure 50 mm Hg or greater at the end of surgery, compared with 1.9% of patients without adverse events (P < .001). The pulmonary artery systolic pressure decrease after surgery in those without adverse events was on average 5 mm Hg more than in those with adverse events. CONCLUSIONS: Postoperative pulmonary hypertension was associated with a higher rate of adverse cardiopulmonary events. This may influence the decision to use Swan-Ganz catheters in patients undergoing septal myectomy in monitoring pulmonary artery pressures to better risk stratify and manage these patients postoperatively.

Long-term tolerability of phosphodiesterase-5 inhibitors in pulmonary hypertension of sickle cell disease.

OBJECTIVES: Sickle cell disease-related pulmonary hypertension (SCD-PH) is a complex disorder with multifactorial contributory mechanisms. Previous trials have evaluated the efficacy of pulmonary arterial hypertension (PAH) therapies in SCD-PH with mixed results. We hypothesized that a subset of patients with right heart catheterization (RHC) confirmed disease may benefit from PAH therapy. METHODS: We performed a retrospective chart review of patients with SCD-PH diagnosed by RHC who were treated with phosphodiesterase 5 inhibitor (PDE5-I) therapy for ≥4 months between 2008 and 2019 at two institutions. RESULTS: Thirty-six patients were included in the analysis. The median age (IQR) upon PDE5-I initiation was 47.5 years (35-51.5 years); 58% were female and twenty-nine (81%) had HbSS disease. Of these, 53% of patients had a history of acute chest syndrome, 42% had a history of venous thromboembolism, and 38% had imaging consistent with chronic thromboembolic PH. Patients were treated for a median duration of 25 months (IQR 13-60 months). Use of PDE5-I was associated with a significant improvement in symptoms as assessed by NYHA Class (P = .002). CONCLUSIONS: In SCD patients with PH defined by RHC, PDE5-I therapy was tolerated long-term and may improve physical activity.

Group 3 Pulmonary Hypertension: A Review of Diagnostics and Clinical Trials.

Group 3 pulmonary hypertension (PH) is a known sequelae of chronic lung disease. Diagnosis and classification can be challenging in the background of chronic lung disease and often requires expert interpretation of numerous diagnostic studies to ascertain the true nature of the PH. Stabilization of the underlying lung disease and adjunctive therapies such as oxygen remain the mainstays of therapy, as there are no Food and Drug Administration-approved therapies for group 3 PH. Referral to PH centers for individualized management and clinical trial enrollment is paramount.