Characteristics and survival of adult Swedish PAH and CTEPH patients 2000-2014.

OBJECTIVES: The Swedish Pulmonary Arterial Hypertension Register (SPAHR) is an open continuous register, including pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) patients from 2000 and onwards. We hereby launch the first data from SPAHR, defining baseline characteristics and survival of Swedish PAH and CTEPH patients. DESIGN: Incident PAH and CTEPH patients 2008-2014 from all seven Swedish PAH-centres were specifically reviewed. RESULTS: There were 457 PAH (median age: 67 years, 64% female) and 183 CTEPH (median age: 70 years, 50% female) patients, whereof 77 and 81%, respectively, were in functional class III-IV at diagnosis. Systemic hypertension, diabetes, ischaemic heart disease and atrial fibrillation were common comorbidities, particularly in those >65 years. One-, 3- and 5-year survival was 85%, 71% and 59% for PAH patients. Corresponding numbers for CTEPH patients with versus without pulmonary endarterectomy were 96%, 89% and 86% versus 91%, 75% and 69%, respectively. In 2014, the incidence of IPAH/HPAH, associated PAH and CTEPH was 5, 3 and 2 per million inhabitants and year, and the prevalence was 25, 24 and 19 per million inhabitants. CONCLUSION: The majority of the PAH and CTEPH patients were diagnosed at age >65 years, in functional class III-IV, and exhibiting several comorbidities. PAH survival in SPAHR was similar to other registers.

Effect of spironolactone on vascular stiffness in hemodialysis patients: a randomized crossover trial.

Background: The role of spironolactone treatment in hemodialysis patients is debated, but a survival benefit is suggested. Mineralocorticoids and chronic kidney disease have been linked to cardiovascular fibrosis. Therefore, we hypothesized that spironolactone would affect vascular stiffness, cardiac systolic, and diastolic function in hemodialysis patients. Methods: This was a randomized crossover study in hemodialysis patients supplemented with an echocardiographic case series. All outcomes reported here were secondary in the trial and were assessed without blinding. Block randomization and allocation determined treatment order. Participants received 50 mg spironolactone daily for 12 weeks and untreated observation for another 12 weeks. Pulse wave velocity (PWV) was measured before and after treatment and observation. Doppler-echocardiography was conducted before and after treatment. Systemic arterial compliance indexed to body surface area (SACi), left ventricular ejection fraction (LVEF), the peak early diastolic mitral inflow velocity (E), the peak late diastolic mitral inflow velocity (A), and the peak early diastolic myocardial lengthening velocity (E') were measured. E/A and E/E' were then calculated. Statistical analyses were conducted per protocol. A generalized linear mixed model with random participant effects was used for PWV. The Wilcoxon signed-rank test was used for echocardiographic variables. Results: Thirty participants were recruited, 18 completed follow-up, and 17 were included in PWV-analyses. Spironolactone treatment showed a tendency toward an increase in PWV of 1.34 (95% confidence interval: -0.11 to 2.78) m/s, which was not statistically significant (P = 0.07). There were no significant changes in any of the other variables (LVEF, E/A, E/E', or SACi). Conclusions: We found no evidence supporting an effect of 12-week administration of spironolactone 50 mg daily on vascular stiffness, cardiac systolic, or diastolic function in hemodialysis patients.

[Platypnea-orthodeoxia syndrome: a neglected cause of dyspnea?] / Platypné­ortodeoxisyndrom ­ förbisedd orsak till dyspné.

A 68-year-old woman presented with progressive and severe effort-related dyspnea. Her history included an ischemic stroke at age 43. Routine exams were normal. Cardiopulmonary exercise testing (CPET) showed a reduction in PaO2 from 11.8 kPa to 4.8 kPa. Repeated CPET with 100 % inhaled O2 improved the drop in PaO2 marginally. Transesophageal echocardiography revealed a shunt from right to left through a patent foramen ovale (PFO). A right heart catheterization showed normal pressures and no signs of intrapulmonary shunting. The PFO was closed percutaneously and the patient's symptoms resolved almost completely. Platypnea-orthodeoxia syndrome is an uncommon disorder where the pathophysiological mechanisms include a right-to-left shunt, either intracardiac or pulmonary. The most common intracardiac shunt related to the syndrome is a PFO. Platypnea-orthodeoxia syndrome, although rare, merits our attention, since it is often easily treatable. The key finding is desaturation on standing up.

Thirty-year electrocardiographic follow-up after repair of tetralogy of Fallot or atrial septal defect.

BACKGROUND AND PURPOSE: Knowledge about long-term electrocardiographic changes after surgery for congenital heart disease is limited. METHODS: Eleven patients with corrected tetralogy of Fallot (ToF) and 14 with corrected atrial septal defect (ASD) were followed up at 20 and 30 years after surgery. RESULTS: Approximately 50% of the ASD group developed prolonged QRS duration. In the ToF group, 7 increased QRS duration by more than 20 milliseconds. Nearly all had right bundle-branch block, and 30% of them also had bifascicular block. Two in the ASD group developed first grade atrioventricular block. Five ASD and 6 ToF had prolonged corrected QT duration in the late postoperative phase. CONCLUSIONS: Even after primarily good results of surgery in congenital heart disease, unknown late effects may occur not only in complex lesions such as ToF but also after ASD correction. Regular medical checkups are important after surgical correction in congenital heart disease.