Assessing the spectrum of pulmonary hypertension identified at an Egyptian expert referral center

Background and objective Pulmonary hypertension is an assorted state that encompasses a spectrum of diseases and is categorized into five groups. The registries are necessary for the identification of risk factors, progression of the disease, outcomes, and effect of treatment strategies on the progression of the disease. The aim of this work was to identify different groups of PH identified at an Egyptian referral center and to compare the demographic and clinical characteristics of each group. Patients and methods This study included 132 patients who were diagnosed with a right heart catheter in the Chest Department, Kasr El-Aini Hospital, Faculty of Medicine, Cairo University, in the period from January 2017 to January 2019. Patients were classified into different groups, then received medical treatment accordingly. Demographic and clinical data were documented. Arterial blood gases, spirometry, and 6-minute walk test (6MWT) were performed. Results The mean age of cases was (43.9±13.69) years; the majority of them were females (72.7%). More than half of the patients (57.5%) had pulmonary artery hypertension (PAH), while 22.7% and 18.3% had pulmonary hypertension due to chronic thrombo-embolic cause and lung cause respectively. The 1-year survival rate was 81.8%. Conclusions The results of the study showed female predominance, the PAH type was the commonest, and the overall 1-year survival rate was 81.8%.

Comparison of pulmonary vascular resistance and pulmonary artery compliance during exercise between IPAH and CTEPH with normal pulmonary artery pressure

Background Pulmonary vascular resistance (PVR) and pulmonary artery compliance (PAC) are inversely related. However, the little is known about dynamics during exercise by disease difference. The aim of this study was to reveal the relationships of PVR and PAC during exercise between idiopathic pulmonary arterial hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH) patients. Methods Sixty-two IPAH patients (45±9 y.o) and 359 CTEPH patients (63±13 y.o) with normal mean PAP and PAWP at rest underwent symptom-limited exercise test using supine cycle ergometer with right heart catheter. Results There were no differences between baseline mean PAP and PAWP in 2 groups, however, cardiac output, SaO2 and SvO2 were lower in CTEPH group. Lower PAC (2.9±1.1 vs. 3.7±1.7 ml/mmHg, p<0.001) and higher PVR (2.3±1.0 vs. 1.9±1.0 wood.unit, p=0.016) were observed in CTEPH group. These trends were also seen at peak exercise. PVR-PAC relationship in CTEPH group was leftward shift compared with IPAH group (Figure 1). Conclusion Resting and exercise PVR and PAC in CTEPH patients were worse than those in IPAH patients who had normal PAP and PAWP at rest. Figure 1 Funding Acknowledgement Type of funding source: None

Exercise and fluid challenge during right heart catheterisation for evaluation of dyspnoea

This prospective study compared exercise test and intravenous fluid challenge in a single right heart catheter procedure to detect latent diastolic heart failure in patients with echocardiographic heart failure with preserved ejection function. We included 49 patients (73% female) with heart failure with preserved ejection function and pulmonary artery wedge pressure ≤15 mmHg. A subgroup of 26 patients had precapillary pulmonary hypertension. Invasive haemodynamic and gas exchange parameters were measured at rest, 45° upright position, during exercise, after complete haemodynamic and respiratory recovery in lying position, and after rapid infusion of 500 mL isotonic solution. Most haemodynamic parameters increased at both exercise and intravenous fluid challenge, with the higher increase at exercise. Pulmonary vascular resistance decreased by –0.21 wood units at exercise and –0.56 wood units at intravenous fluid challenge ( p = 0.3); 20% (10 of 49) of patients had an increase in pulmonary artery wedge pressure above the upper limit of 20 mmHg at exercise, and 20% above the respective limit of 18 mmHg after intravenous fluid challenge. However, only three patients exceeded the upper limit of pulmonary artery wedge pressure in both tests, i.e. seven patients only at exercise and seven other patients only after intravenous fluid challenge. In the subgroup of pulmonary hypertension patients, only two patients exceeded pulmonary artery wedge pressure limits in both tests, further five patients at exercise and four patients after intravenous fluid challenge. A sequential protocol in the same patient showed a significantly higher increase in haemodynamic parameters at exercise compared to intravenous fluid challenge. Both methods can unmask diastolic dysfunction at right heart catheter procedure, but in different patient groups.

Changes in pulmonary exercise haemodynamics in scleroderma: a 4-year prospective study

Pulmonary arterial hypertension (PAH) is a feared complication of systemic sclerosis. In this prospective cohort study, we monitored the changes in resting and exercise pulmonary haemodynamics of scleroderma patients without initial PAH over a mean follow-up period of ∼4 years.All patients underwent exercise echocardiography and cardiopulmonary exercise testing at baseline and follow-up. A subgroup underwent exercise right heart catheter (RHC) investigations. The primary end-point was the echocardiographic systolic pulmonary arterial pressure at 50 W exercise (sPAP50).We included 99 patients, of whom 58 had a complete dataset. Three out of 99 patients developed RHC-confirmed PAH (0.75 cases per 100 patient-years). sPAP50 increased (p<0.001) and peak oxygen uptake (secondary end-point) decreased significantly (p=0.001) during follow-up, but there was no significant change in resting sPAP (p=0.38). In the RHC subgroup (n=28), mean (m)PAP and pulmonary vascular resistance at 50 W increased significantly (p=0.02 and p=0.002, respectively), but resting mPAP was unchanged.Scleroderma patients without PAH develop a mild but significant deterioration of pulmonary exercise haemodynamics and exercise capacity over a 4-year follow-up period, indicating a progression of pulmonary vascular disease. The manifestation rate of RHC-confirmed PAH was 0.75 cases per 100 patient-years.

Pulmonary Hemodynamics and Six-Minute Walk Test Outcomes in Patients with Interstitial Lung Disease

Background. Six-minute walk test (6MWT) has 3 measurement outcomes, which are walk distance, desaturation, and symptom. The aim of this study was to examine whether routinely measured right-heart catheter (RHC) data correlate with 6MWT outcomes in patients with interstitial lung disease (ILD).Methods. Between June 2010 and December 2012, consecutive patients with ILD who underwent evaluation, including pulmonary function test, hemodynamic studies with right-heart catheter, and 6MWT as routine general practice, were recruited. Correlates of 3 outcomes of 6MWT were examined to reveal significant predictors.Results. Forty-six patients consisting of 20 with idiopathic pulmonary fibrosis, 14 with collagen vascular disease associated ILD, and 12 with other idiopathic interstitial pneumonia were recruited (mean % predicted FVC:76.7±17.1%). Several physiological variables, including mean pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR), were correlated with each 6MWT outcome. Stepwise multivariate regression analyses showed that % predicted FVC and % predicted DLco were independent predictors of walk distance (r2=0.35,p=0.0002). For SpO2at the end of 6MWT, % predicted DLco and PVR were selected as independent predictors (r2=0.46,p<0.0001). For dyspnea at the end of 6MWT, % predicted DLco was only one predictor (r2=0.18,p=0.005).Conclusion. Mean PAP had little impact on 6MWT outcomes in ILD patients who were nonselectively recruited, although PVR was one of predictors of desaturation.