Triple vasodilator therapy in pulmonary arterial hypertension associated with congenital heart disease.

OBJECTIVE: This study assessed the long-term effects of triple therapy with prostanoids on patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), as there is limited information on the safety and efficacy of this treatment approach. METHODS: A retrospective cohort study was conducted on patients with PAH-CHD who were actively followed up at our centre. All patients were already receiving dual combination therapy at maximum doses. Clinical characteristics, including functional class (FC), 6-minute walking test distance (6MWTD) and N-terminal pro-brain natriuretic peptide (NT-proBNP) levels, were documented before initiating triple therapy and annually for a 2-year follow-up period. RESULTS: A total of 60 patients were included in the study, with a median age of 41 years and 68% being women. Of these, 32 had Eisenmenger syndrome, 9 had coincidental shunts, 18 had postoperative PAH and 1 had a significant left-to-right shunt. After 1 year of triple combination initiation, a significant improvement in 6MWTD was observed (406 vs 450; p=0.0027), which was maintained at the 2-year follow-up. FC improved in 79% of patients at 1 year and remained stable in 76% at 2 years. NT-proBNP levels decreased significantly by 2 years, with an average reduction of 199 ng/L. Side effects were experienced by 33.3% of patients but were mostly mild and manageable. Subgroup analysis showed greater benefits in patients without Eisenmenger syndrome and those with pre-tricuspid defects. CONCLUSIONS: Triple therapy with prostanoids is safe and effective for patients with PAH-CHD, improving FC, 6MWTD and NT-proBNP levels over 2 years. The treatment is particularly beneficial for patients with pre-tricuspid defects and non-Eisenmenger PAH-CHD.

Feasibility of a Noninvasive Operability Assessment in Chronic Thromboembolic Pulmonary Hypertension under Real-World Practice.

This study aimed to evaluate the feasibility of a noninvasive operability assessment of chronic thromboembolic pulmonary hypertension (CTEPH) based on multidetector computed tomographic angiography (MCTA). Up to 176 patients were evaluated from January 2016 to April 2018. Throughout the first phase, the initial surgical decision was made based on MCTA with further analysis of pulmonary angiography (PA) in order to evaluate in which cases the initial decision was not modified by PA. During the second phase, PA was limited to patients judged inoperable based on MCTA or those whose assessment was not possible. Patients deemed operable (50%) based on MCTA along the first phase had been adequately classified, as PA did not modify the initial decision in all but one patient. Comparable results were obtained throughout the implementation phase. Regarding operated patients, the decision of operability was based solely on MCTA in 94% of those with level I disease, in 75% with level II, and 54% with level III. This approach enabled shorter periods of time to complete surgical assessment and the avoidance of PA-related morbidity. Baseline parameters, postoperative measures, and survival rates at 1 year after surgery were comparable in both phases. Noninvasive operability assessment is feasible in a subset of CTEPH patients and optimizes surgical candidacy evaluation.

Foot health and quality of life in patients with rheumatoid arthritis: a cross-sectional study.

OBJECTIVE: The aim of this study is to identify foot health factors related to the quality of life in patients with rheumatoid arthritis (RA). SETTING: In this cross-sectional study, a total of 293 subjects were analysed, 229 of whom were in the RA group and 64 in the control group. In the RA group, 173 patients were female, and 50 in the control group. PARTICIPANTS: Patients with foot pain and RA (according to the American College of Rheumatology/European League Against Rheumatism 2010 rheumatoid arthritis classification criteria) and with foot pain but no RA were recruited (Granada, Spain). INTERVENTION: Two researchers independently interviewed the patients to obtain data for the study. PRIMARY AND SECONDARY OUTCOME MEASURES: Clinical data were obtained using the Short Form 12-Item questionnaire (quality of life) (primary outcome), Visual Analogue Scale for pain (VAS pain), the Manchester Foot Pain Disability Index (MFPDI) and the Foot Function Index (FFI). Anthropometric measurements were obtained using a foot measurement platform, the Foot Posture Index and the Manchester Scale of Hallux Valgus (secondary outcomes). RESULTS: Of the 293 subjects, 76.1% were female. Significant differences were observed between the RA and the control group (p<0.001) with regard to VAS pain (general, foot and hand), MFPDI and FFI. In terms of anthropometric measurements, significant differences were only recorded for midfoot and forefoot width (p=0.03). For the physical health component, multivariable linear regression with the parameters age, gender, VAS pain (general) and the presence of RA presented an R2 value of 48.8%, while for the mental health component the corresponding value was 5.6%. CONCLUSION: Morphological and structural characteristics of the foot are not necessarily associated with pain, disability and loss of function. The presence of RA, a higher score on VAS pain (general), female gender and older age are all associated with the physical component of the quality of life of patients with RA.

Pulmonary endarterectomy outputs in chronic thromboembolic pulmonary hypertension. / Resultados de la tromboendarterectomía pulmonar en la hipertensión pulmonar tromboembólica crónica.

BACKGROUND AND OBJECTIVE: Pulmonary thromboendarterectomy surgery is the treatment of choice for patients with chronic thromboembolic pulmonary hypertension; extremely high pulmonary vascular resistance constitutes a risk factor for hospital mortality. The objective of this study was to analyze the immediate and long-term results of the surgical treatment of chronic thromboembolic pulmonary hypertension in patients with very severe pulmonary hypertension. MATERIAL AND METHODS: Since February 1996, we performed 160 pulmonary thromboendarterectomies. We divided the patient population in 2 groups: group 1, which included 40 patients with pulmonary vascular resistance≥1090dyn/sec/cm-5, and group 2, which included the remaining 120 patients. RESULTS: Hospital mortality (15 vs. 2.5%), reperfusion pulmonary edema (33 vs. 14%) and heart failure (23 vs. 3.3%) were all higher in group 1; however, after one year of follow-up, there were no significant differences in the clinical, hemodynamic and echocardiographic conditions of both groups. Survival rate after 5 years was 77% in group 1 and 92% in group 2 (P=.033). After the learning curve including the 46 first patients, there was no difference in hospital mortality (3.8 vs. 2.3%) or survival rate after 5 years (96.2% in group 1 and 96.2% in group 2). CONCLUSIONS: Pulmonary thromboendarterectomy is linked to significantly higher morbidity and mortality rates in patients with severe chronic thromboembolic pulmonary hypertension. Nevertheless, these patients benefit the same from the procedure in the mid-/long-term. In our experience, after the learning curve, this surgery is safe in severe pulmonary hypertension and no level of pulmonary vascular resistance should be an absolute counter-indication for this surgery.

Pulmonary thromboendarterectomy in 106 patients with chronic thromboembolic pulmonary hypertension.

INTRODUCTION: Pulmonary thromboendarterectomy is the treatment of choice in chronic thromboembolic pulmonary hypertension. We report our experience with this technique. METHODS: Between February 1996 and June 2014, we performed 106 pulmonary thromboendarterectomies. Patient population, morbidity and mortality and the long-term results of this technique (survival, functional improvement and resolution of pulmonary hypertension) are described. RESULTS: Subjects' mean age was 53±14 years. A total of 89% were WHO functional class III-IV, presurgery mean pulmonary pressure was 49±13mmHg and mean pulmonary vascular resistance was 831±364 dynes.s.cm(-5). In-hospital mortality was 6.6%. The most important post-operative morbidity was reperfusion pulmonary injury, in 20% of patients; this was an independent risk factor (p=0.015) for hospital mortality. With a 31-month median follow-up (interquartile range: 50), 3- and 5-year survival was 90 and 84%. At 1 year, 91% were WHO functional class I-II; mean pulmonary pressure (27±11mmHg) and pulmonary vascular resistance (275±218 dynes.s.cm(-5)) were significantly lower (p<0.05) than before the intervention. Although residual pulmonary hypertension was detected in 14 patients, their survival at 3 and 5 years was 91 and 73%, respectively. CONCLUSIONS: Pulmonary thromboendarterectomy offers excellent results in chronic thromboembolic pulmonary hypertension. Long-term survival is good, functional capacity improves, and pulmonary hypertension is resolved in most patients.