digoxin and Familial-Primary-Pulmonary-Hypertension

Recent advances in pulmonary arterial hypertension (PAH) research have created a new era of PAH-specific therapies. Although these therapeutics have revolutionized PAH therapy, their innovation was predated by supportive but nonspecific medical therapies adapted from their use in more common cardiopulmonary diseases. These therapies include oxygen therapy, diuretics, digoxin, anticoagulation, and high-dose calcium channel blockers. Expert opinion continues to support the use of adjunct therapies based on current pathologic understandings of PAH combined with some evidence extrapolated from small studies. This article discusses why these therapies continue to play an important role in the treatment of patients with PAH.

Topics: Calcium Channel Blockers; Digoxin; Diuretics; Familial Primary Pulmonary Hypertension; Humans; Hypertension, Pulmonary; Oxygen Inhalation Therapy

Background Digoxin acutely increases cardiac output in patients with pulmonary arterial hypertension (PAH) and right ventricular failure; however, the effects of chronic digoxin use in PAH are unclear. Methods and Results Data from the Minnesota Pulmonary Hypertension Repository were used. The primary analysis used likelihood of digoxin prescription. The primary end point was a composite of all-cause mortality or heart failure (HF) hospitalization. Secondary end points included all-cause mortality, HF hospitalization, and transplant-free survival. Multivariable Cox proportional hazards analyses determined the hazard ratios (HR) and 95% CIs for the primary and secondary end points. Among 205 patients with PAH in the repository, 32.7% (n=67) were on digoxin. Digoxin was more often prescribed to patients with severe PAH and right ventricular failure. After propensity score-matching, 49 patients were digoxin users, and 70 patients were nonusers; of these 31 (63.3%) in the digoxin group and 41 (58.6%) in nondigoxin group met the primary end point during a median follow-up time of 2.1 (0.6-5.0) years. Digoxin users had a higher combined all-cause mortality or HF hospitalization (HR, 1.82 [95% CI, 1.11-2.99]), all-cause mortality (HR, 1.92 [95% CI, 1.06-3.49]), HF hospitalization (HR, 1.89 [95% CI, 1.07-3.35]), and worse transplant-free survival (HR, 2.00 [95% CI, 1.12-3.58]) even after adjusting for patient characteristics and severity of PAH and right ventricular failure. Conclusions In this retrospective, nonrandomized cohort, digoxin treatment was associated with greater all-cause mortality and HF hospitalization, even after multivariate correction. Future randomized controlled trials should assess the safety and efficacy of chronic digoxin use in PAH.

Topics: Digoxin; Familial Primary Pulmonary Hypertension; Heart Failure; Hospitalization; Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Retrospective Studies; Treatment Outcome

Red blood cell distribution width (RDW) has been shown to predict clinical outcomes in cardiopulmonary vascular diseases. We investigated whether RDW is useful to predict responsiveness of acute pulmonary vasodilator testing in patients with idiopathic pulmonary arterial hypertension (IPAH).. RDW was determined in 167 IPAH patients who underwent acute pulmonary vasodilator testing. All subjects were followed up for 20 ± 10 months.. Nineteen out of 167 patients (11.4%) were acute pulmonary vasodilator testing responders. Patients with lower RDW levels ≤ 13.65% (sensitivity 89.5%, specificity 52.7%; AUC: 0.747, 95% CI: 0.632 to 0.861) were more likely to have a positive response. Multivariate logistic regression analysis showed that RDW ≤ 13.65% independently predicted responsiveness of vasodilator testing in patients with IPAH (OR 18.453, 95% CI 2.279-149.391, p = 0.006). RDW correlated with disease severity evaluated by clinical parameters. Patients with increased RDW (> 13.65%) had significantly increased risk of all-cause death (Log-rank p = 0.007).. RDW independently predicts responsiveness of acute pulmonary vasodilator testing in patients with IPAH. RDW is associated with disease severity and all-cause death.

Topics: Adult; Biomarkers, Pharmacological; Calcium Channel Blockers; Digoxin; Diuretics; Erythrocyte Indices; Erythrocytes; Familial Primary Pulmonary Hypertension; Female; Follow-Up Studies; Humans; Logistic Models; Male; Prognosis; ROC Curve; Severity of Illness Index; Survival Analysis; Vasodilation; Vasodilator Agents

Topics: Acetylcholine; Blood Pressure; Blood Vessels; Cardiac Catheterization; Cardiomegaly; Digoxin; Familial Primary Pulmonary Hypertension; Humans; Hypertension; Hypertension, Pulmonary; Infusions, Parenteral; Lung; Lupus Erythematosus, Systemic; Metabolism; Prednisone; Pulmonary Heart Disease; Tolazoline