Cardiac magnetic resonance findings predicting mortality in patients with pulmonary arterial hypertension: a systematic review and meta-analysis

Objectives: To provide a comprehensive overview of all reported cardiac magnetic resonance (CMR) findings that predict clinical deterioration in pulmonary arterial hypertension (PAH). Methods: MEDLINE and EMBASE electronic databases were systematically searched for longitudinal studies published by April 2015 that reported associations between CMR findings and adverse clinical outcome in PAH. Studies were appraised using previously developed criteria for prognostic studies. Meta-analysis using random effect models was performed for CMR findings investigated by three or more studies. Results: Eight papers (539 patients) investigating 21 different CMR findings were included. Meta-analysis showed that right ventricular (RV) ejection fraction was the strongest predictor of mortality in PAH (pooled HR 1.23 [95 % CI 1.07–1.41], p = 0.003) per 5 % decrease. In addition, RV end-diastolic volume index (po

Echocardiographic findings associated with mortality or transplant in patients with pulmonary arterial hypertension: A systematic review and meta-analysis

Background Identification of patients at risk of deterioration is essential to guide clinical management in pulmonary arterial hype

PulmoCor: National registry for pulmonary hypertension

Contains fulltext : 171308.pdf (publisher's version ) (Open Access

Characterization of a lung epithelium specific E-cadherin knock-out model: Implications for obstructive lung pathology

The airway epithelium regulates responses to aeroallergens, acting as a physical and immunological barrier. In asthma, epithelial barrier function and the expression of adherens junction protein E-cadherin is compromised, but it is unknown whether this is cause or consequence of the disease. We hypothesized that airway epithelial loss of E-cadherin is a critical step in the development of manifestations of asthma. We generated a transgenic mouse model with conditional loss of E-cadherin in lung epithelial cells at birth and onwards. We observed normal lung development at the time of birth in mice lacking E-cadherin in the lung epithelium. However, E-cadherin deficiency led to progressive epithelial damage in mice growing into adulthood, as evidenced by airway epithelial denudation, decreased zonula occludens (ZO)-1 expression, loss of ciliated cells, and enlarged alveolar spaces. In addition, spontaneous goblet cell metaplasia with mucus production was observed. These epithelial changes were accompanied by elevated levels of the epithelial-derived chemokine CCL17, infiltration of eosinophils and dendritic cells, and mucus production. In conclusion, loss of E-cadherin induces features in the lung reminiscent of those observed in asthma, indicating that the disruption of E-cadherin-mediated cell-cell contacts may play a key role in the development of asthma manifestations

A randomised comparison of the effect of haemodynamic monitoring with CardioMEMS in addition to standard care on quality of life and hospitalisations in patients with chronic heart failure: Design and rationale of the MONITOR HF multicentre randomised clinical trial

Background: Assessing haemodynamic congestion based on filling pressures instead of clinical congestion can be a way to further improve quality of life (QoL) and clinical outcome by intervening before symptoms or weight gain occur in heart failure (HF) patients. The clinical efficacy of remote monitoring of pulmonary artery (PA) pressures (CardioMEMS; Abbott Inc., Atlanta, GA, USA) has been demonstrated in the USA. Currently, the PA sensor is not reimbursed in the European Union as its benefit when applied in addition to standard HF care is unknown in Western European countries, including the Netherlands. Aims: To demonstrate the efficacy and cost-effectiveness of haemodynamic PA monitoring in addition to contemporary standard HF care in a high-quality Western European health care system. Methods: The current study is a prospective, multi-centre, randomised clinical trial in 340 patients with chronic HF (New York Heart Association functional class III) randomised to HF care including remote monitoring with the CardioMEMS PA sensor or standard HF care alone. Eligible patients have at least one hospitalisation for HF in 12 months before enrolment and will be randomised in a 1:1 ratio. Minimum follow-up will be 1 year. The primary endpoint is the change in QoL as measured by the Kansas City Cardiomyopathy Questionnaire (KCCQ). Secondary endpoints are the number of HF hospital admissions and changes in health status assessed by EQ-5D-5L questionnaire including healt

Echocardiographic right ventricular remodeling after percutaneous atrial septal defect closure

Background: In order to prevent right ventricular (RV) dysfunction, closure of secundum type atrial septal defects (ASD) is often indicated and percutaneous closure is the preferred treatment modality to do so. The magnitude and time course of RV remodeling is still incompletely understood. Methods: This retrospective cohort study included consecutive patients who underwent percutaneous secundum ASD closure in two tertiary referral centers in The Netherlands. Main study parameters were RV and right atrial dimensions measured with transthoracic echocardiography before and after percutaneous ASD closure. Secondary outcome was change in New York Heart Association (NYHA) functional class at follow-up. Results: From the 454 patients who underwent secundum ASD closure, 88 patients (median age 46 [range 17–84]) were included. The majority of RV and right atrial dimensional improvement occurred within 24 h. After a median follow-up of 569 days (IQR: 280–772) a further decrease in dimensions was observed. Comparing baseline and latest follow-up, end-diastolic RV basal diameter decreased from 4.5 SEM 0.1 to 3.9 SEM 0.1 cm (p < 0.001) and end-systolic right atrial area from 22.9 SEM 1.0 to 17.9 SEM 0.7 cm2 (p < 0.001). No significant changes in RV function were observed. NYHA functional class improved from 1.5 at baseline (IQR: 1.0–2.0) to 1.0 (IQR: 1.0–1.5) at latest follow-up (p < 0.001). Conclusion: Remodeling of the RV heart dimensions commences within 24 h after percutaneous secundum ASD closure for the majority of patients, followed by a further gradual recovery. A concurrent improvement of NYHA functional class was observed during follow-up

Hybrid myocardial perfusion SPECT/CT coronary angiography and invasive coronary angiography in patients with stable angina pectoris lead to similar treatment decisions

Objectives: To evaluate to what extent treatment decisions for patients with stable angina pectoris can be made based on hybrid myocardial perfusion single-photon emission CT (SPECT) and CT coronary angiography (CCTA). It has been shown that hybrid SPECT/CCTA has good performance in the diagnosis of significant coronary artery disease (CAD). The question remains whether these imaging results lead to similar treatment decisions as compared to standalone SPECT and invasive coronary angiography (CA). Methods: We prospectively included 107 patients (mean age 62.8±10.0 years, 69% male) with stable anginal complaints and an intermediate to high pre-test likelihood for CAD. Hybrid SPECT/CCTA was performed prior to CA in all patients. The study outcome was the treatment decision categorised as: no revascularisation, percutaneous coronary intervention (PCI) or coronary artery bypass grafting (CABG). Treatment decisions were made by two interventional cardiologists and one cardiothoracic surgeon in two steps: first, based on the results of hybrid SPECT/CCTA; second, based on SPECT and CA. Results: Revascularisation (PCI or CABG) was indicated in 54 (50%) patients based on SPECT and CA. Percentage agreement of treatment decisions in all patients based on hybrid SPECT/CCTA versus SPECT and CA on the necessity of revascularisation was 92%. Percentage agreement of treatment decisions in patients with matched, unmatched and normal hybrid SPECT/CCTA findings was 95%, 84% and 100%, respectively. Conclusions: Panel evaluation shows that patients could be accurately indicated for and deferred from revascularisation based on hybrid SPECT/CCTA