Myocardial inflammation is a common finding in patients with autoimmune diseases and cardiac symptoms. The diagnosis can be confirmed by CMR, which is a noninvasive and reliable tool for the investigation of these patients.

Background-The prevention of contrast-induced nephropathy, which accounts for considerable morbidity and mortality, remains a vexing problem. Contrast-induced renal vasoconstriction is believed to play a pivotal role in the pathogenesis of contrast-induced nephropathy. The aim of this study was to examine the efficacy of the prostacyclin analog iloprost in preventing contrast-induced nephropathy in patients with renal dysfunction undergoing a coronary procedure. Methods and Results-We conducted a randomized, double-blind, placebo-controlled trial of iloprost in 208 patients with a serum creatinine concentration Ն1.4 mg/dL who underwent coronary angiography and/or intervention. Iloprost 1 ng · kg Ϫ1 · min Ϫ1 or placebo was administered intravenously beginning 30 to 90 minutes before and ending 4 hours after the procedure. Contrast-induced nephropathy was defined by an absolute increase in serum creatinine Ն0.5 mg/dL or a relative increase Ն25% measured 2 to 5 days after the procedure. Contrast-induced nephropathy occurred in 23 of the 105 patients (22%) in the control group and in 8 of the 103 patients (8%) in the iloprost group (odds ratio, 0.29; 95% confidence interval, 0.12 to 0.69; Pϭ0.005). In the control group, the estimated glomerular filtration rate declined from 49.7Ϯ15.5 to 46.6Ϯ16.6 mL · min Ϫ1 · 1.73 m Ϫ2 (Pϭ0.01). In the iloprost group, the estimated glomerular filtration rate increased marginally from 47.5Ϯ14.5 to 48.6Ϯ16.1 mL · min Ϫ1 · 1.73 m Ϫ2 (Pϭ0.26). The mean absolute estimated glomerular filtration rate decline in the control group was greater than its change in the iloprost group (difference, 4.2 mL · min Ϫ1 · 1.73 m Ϫ2; 95% confidence interval, 1.1 to 7.3; Pϭ0.008). Conclusion-Prophylactic

SummaryPulmonary arterial hypertension (PAH) is a disease of small pulmonary arteries, characterized by vascular proliferation and remodeling. Progressive increase in pulmonary vascular resistance ultimately leads to right ventricular heart failure and death. PAH-specific drug therapy has improved clinical outcomes and survival. While the survival is better, progression of pulmonary vasculopathy contributes to pulmonary artery dilatation. Left main compression syndrome, pulmonary artery dissection, pulmonary artery rupture, and severe hemoptysis are reported as complications leading to sudden cardiac death, an event encountered more often in PAH patients. The advent of PAHtargeted drug therapy has reduced referral for lung transplantation; however, severe complications require rapid diagnosis, decision making, and possible registration on a lung transplantation waiting list. PAH referral centers provide multidisciplinary emergency care and specific therapeutic management, contributing to improved quality of life and survival for PAH patients. We review the complications leading to sudden death in PAH. Key words: pulmonary hypertension; pulmonary artery dissection; pulmonary artery rupture; left main compression syndrome. [Respir Care 2013;58(7):1246 -1254.

BackgroundIn patients with Duchenne Muscular Dystrophy (DMD), the absent or diminished dystrophin leads to progressive skeletal muscle and heart failure. We evaluated the role of myocardial inflammation as a precipitating factor in the development of heart failure in DMD.Methods20 DMD patients (aged 15-18 yrs) and 20 age-matched healthy volunteers were studied and followed-up for 2 years. Evaluation of myocarditis with cardiovascular magnetic resonance imaging (CMR) was performed using STIR T2-weighted (T2W), T1-weighted (T1W) before and after contrast media and late enhanced images (LGE). Left ventricular volumes and ejection fraction were also calculated. Myocardial biopsy was performed in patients with positive CMR and immunohistologic and polymerase chain reaction (PCR) analysis was employed.ResultsIn DMD patients, left ventricular end-diastolic volume (LVEDV) was not different compared to controls. Left ventricular end-systolic volume (LVESV) was higher (45.1 ± 6.6 vs. 37.3 ± 3.8 ml, p < 0.001) and left ventricular ejection fraction (LVEF) was lower (53.9 ± 2.1 vs. 63 ± 2.4%, p < 0.001). T2 heart/skeletal muscle ratio and early T1 ratio values in DMD patients presented no difference compared to controls. LGE areas were identified in six DMD patients. In four of them with CMR evidence of myocarditis, myocardial biopsy was performed. Active myocarditis was identified in one and healing myocarditis in three using immunohistology. All six patients with CMR evidence of myocarditis had a rapid deterioration of left ventricular function during the next year.ConclusionsDMD patients with myocardial inflammation documented by CMR had a rigorous progression to heart failure.

Remote postconditioning consisted of 1 min isc/1 min rep protects the ischemic rabbit heart in vivo, independently of the site of the remote artery. This intervention seems to confer a stronger protection than the classic postconditioning.

Pulmonary arterial hypertension (PAH) is a heterogenous clinical entity with poor prognosis, despite recent major pharmacological advances. To increase awareness about the pathophysiology, epidemiology, and management of the disease, large national registries are required. The Hellenic pulmOnary hyPertension rEgistry (HOPE) was launched in early 2015 and enrolls patients from all pulmonary hypertension subgroups in Greece. Baseline epidemiologic, diagnostic, and initial treatment data of consecutive patients with PAH are presented in this article. In total, 231 patients with PAH were enrolled from January 2015 until April 2018. At baseline, about half of patients with PAH were in World Health Organization functional class II. The majority of patients with PAH (56.7%) were at intermediate 1-year mortality risk, while more than one-third were low-risk patients, according to an abbreviated risk stratification score. Half of patients with PAH were on monotherapy, 38.9% received combination therapy, while prostanoids were used only in 12.1% of patients. In conclusion, baseline data of the Greek PAH population share common characteristics, but also have some differences with other registries, the most prominent being a better functional capacity. This may reflect earlier diagnosis of PAH that in conjunction with the increased proportion of patients with atypical PAH could partially explain the preference for monotherapy and the limited use of prostanoids in Greece. Nevertheless, early, advanced specific therapy is strongly recommended.

No abstract

Pulmonary arterial hypertension (PAH) is characterized by an insult in the pulmonary vasculature, with subsequent right ventricular (RV) adaptation to the increased afterload that ultimately leads to RV failure. The awareness of the importance of RV function in PAH has increased considerably because right heart failure is the predominant cause of death in PAH patients. Given its wide availability and reduced cost, echocardiography is of paramount importance in the evaluation of the right heart in PAH. Several echocardiographic parameters have been shown to have prognostic implications in PAH; however, the role of echocardiography in the risk assessment of the PAH patient is limited under the current guidelines. This review discusses the echocardiographic evaluation of the RV in PAH and during therapy, and its prognostic implications, as well as the potential significant role of repeated echocardiographic assessment in the follow-up of patients with PAH.