Follow-up Right Heart Catheterization Research Articles

Importance of gamma-glutamyl transferase elevation in patients with Fontan-associated liver disease.

In patients with Fontan-associated liver disease (FALD), gamma-glutamyl transferase (GGT) levels are often elevated, however, its clinical importance is unclear. We investigated the relationship between the clinical course of FALD and GGT levels. We enrolled 145 patients with FALD who underwent right-heart catheterization (RHC) and visited our department. Ursodeoxycholic acid (UDCA) was administered to 62 of the patients. Patients with GGT levels <50 and ≥50U/L were compared. Follow-up RHC was undertaken in 76 patients. Cases in which GGT levels decreased by ≥10% or <50U/L were defined as improved (n=33). Patients with GGT levels ≥50U/L had significantly lower levels of albumin and higher levels of alanine transaminase (ALT) but no significant differences in RHC factors. Over a 4.6-year period, 43.4% showed improvement in GGT levels. Improved cases had significantly lower total bilirubin (1.1 vs. 1.6mg/dL), AST (22 vs. 28U/L), and ALT (18 vs. 27U/L) levels than nonimproved cases (n=29, p<0.05), and the change in platelet count (-0.5 vs. -3.0×10-4/μL) was significantly lower in the latter (p=0.03). The improvement rate was significantly higher in UDCA-treated cases (55.2%) with GGT levels ≥50U/L compared to cases not treated with UDCA (18.2%, p=0.04). In cases of FALD with no improvement in GGT level, the platelet count decreased over time, suggesting progression of fibrosis. Physicians should be aware of the importance of a high GGT level in patients with FALD.

Risk stratification refinements with inclusion of haemodynamic variables at follow-up in patients with pulmonary arterial hypertension.

Haemodynamic variables are prognostic factors in pulmonary arterial hypertension (PAH). However, right heart catheterisation (RHC) is not systematically recommended to assess the risk status during follow-up. This study aimed to assess the added value of haemodynamic variables in prevalent patients to predict the risk of death or lung transplantation according to their risk status assessed by the non-invasive four-strata model as recommended by the European guidelines. We evaluated incident patients with PAH enrolled in the French pulmonary hypertension registry between 2009 and 2020 who had a first follow-up RHC. Cox regression identified, in each follow-up risk status, haemodynamic variables significantly associated with transplant-free survival. Optimal thresholds were determined by time-dependent receiver operating characteristics. Several multivariable Cox regression models were performed to identify the haemodynamic variables improving the non-invasive risk stratification model. We analysed 1240 incident patients reassessed within 1 year by RHC. None of the haemodynamic variables were significantly associated with transplant-free survival among low-risk (n=386) or high-risk (n=71) patients. Among patients at intermediate (intermediate-low, n=483 and intermediate-high, n=300) risk at first follow-up, multivariable models including either stroke volume index (SVI) or mixed venous oxygen saturation (S vO2 ) were the best. The prognostic performance of a refined six-strata risk stratification model including the non-invasive four-strata model and SVI >37 mL·m-2 and/or S vO2 >65% for patients at intermediate risk (area under the curve (AUC) 0.81; c-index 0.74) was better than that of the four-strata model (AUC 0.79, p=0.009; c-index 0.72). Cardiopulmonary haemodynamics may improve risk stratification at follow-up in patients at intermediate risk.

Prognostic value of follow-up vasoreactivity test in pulmonary arterial hypertension

BackgroundAcute vasoreactivity test with inhaled nitric oxide (NO) is performed during diagnostic right heart catheterization (RHC) to identify patients with pulmonary arterial hypertension (PAH) who respond to calcium channel blockers. Our purpose was to investigate the prognostic importance of follow-up vasoreactivity test after treatment. MethodsWe retrospectively analyzed 36 PAH patients (mean age, 47 years; 61 % treatment-naïve), who underwent diagnostic and follow-up RHC and vasoreactivity tests at our center. The primary outcome was all-cause mortality. ResultsThe median time between baseline and follow-up RHC was 9.7 months. Absolute change in mean pulmonary arterial pressure (ΔmPAP) during NO challenge was less pronounced after treatment, but there was great variability among patients. Overall cohort was dichotomized into two groups: preserved vasoreactivity (ΔmPAP ≤ −1 mmHg) and less vasoreactivity (ΔmPAP ≥0 mmHg) at follow-up RHC. Less vasoreactivity group had higher usage rate of endothelin receptor antagonists and parenteral prostacyclin analogues. During a median observation period of 6.3 years after follow-up RHC, 7 patients died, of which 6 showed less vasoreactivity at follow-up. Absolute ΔmPAP ≥0 at follow-up RHC was associated with all-cause mortality in univariable Cox regression analysis (hazard ratio, 8.728; 95 % confidence interval, 1.045–72.887; p = 0.045), whereas other hemodynamic parameters were not. Absolute ΔmPAP ≥0 at follow-up RHC was associated with all-cause mortality in multivariable Cox analysis adjusted for age and known PAH prognostic factors (HR, 12.814; 95 % CI, 1.088–150.891; p = 0.043). Kaplan-Meier survival analysis revealed a significantly worse survival of less vasoreactivity group compared to preserved vasoreactivity group (log-rank test, p = 0.016). ConclusionsFollow-up vasoreactivity test after treatment could contribute to the detection of high-risk subgroups who might need careful monitoring and referral for lung transplantation.

Long Term Safety and Outcomes Utilizing the CardioMEMS Device in Patients with Pulmonary Arterial Hypertension

<h3>Purpose</h3> Baseline and periodic hemodynamic monitoring of pulmonary artery pressures using right heart catheterization (RHC) is recommended for initiating and titrating pulmonary arterial hypertension (PAH) therapies as well as for prognostication. The CardioMEMS HF System is an ambulatory implantable hemodynamic monitor, previously only used in patients with left heart failure (HF). In this study, we describe the long-term safety and outcomes using the CardioMEMS device in PAH patients implanted as a part of an investigational pilot study. <h3>Methods</h3> 23 PAH patients between the ages of 31-80 years, were implanted with CardioMEMS device as a part of an investigational pilot study (VITA contract HHSN268201400008C) between 08/2014 - 09/2018. 3 patients were lost to follow-up. Complete follow up data was available for 20 patients who were included in this analysis. <h3>Results</h3> 85% of the PAH patients were female, with a median post-implant follow-up period of 58.5 months. The number of right heart catheterization (RHC) prior to the device implant was 4.37 per patient year, while only 1.99 RHC per patient year were done post implant. In long term follow up there were no significant device related adverse events and all devices remain functional. 8 patients did require RHC for recalibration of the device. 12 patients are still undergoing active monitoring. Of the remaining patients, 1 was enrolled in hospice, 4 patients died from progression of PAH, 1 patient died from malignancy; 1 from end-stage cirrhosis. There was a total of 13 HF hospitalizations between these 20 patients over 3 to 7 year follow-up based on the time of implant. <h3>Conclusion</h3> The CardioMEMS device has previously been reported to reduce hospitalizations in patients with left sided heart failure. Based on our study, deployment of the device was safe in PAH patients with no long-term device related complications. It reduced the number of follow-up invasive RHC by more than 50%. Larger prospective studies are warranted before widespread use of the device for monitoring PA pressures in PAH patients.

Changes in hemodynamic classification over time are common in systemic sclerosis-associated pulmonary hypertension: insights from the PHAROS cohort.

Group classification of pulmonary hypertension (PH) is based on pulmonary artery wedge pressure (PAWP) on right heart catheterization (RHC). How hemodynamics, particularly PAWP, change over time in systemic sclerosis (SSc)-PH patients is unknown. SSc-PH patients enrolled in the prospective observational PHAROS registry who had > 1 RHC (n = 120) were included in this analysis. Patients were considered to have a “PAWP class change” if they had a PAWP ≤ 15 mmHg on RHC-1 and then a PAWP > 15 on RHC-2 or had a PAWP > 15 on RHC-1 and then PAWP ≤ 15 on RHC-2. There was a median time of 1.4 years between RHC-1 and RHC-2 and 75% of patients had a PH medication added after their initial RHC. PAWP increased significantly (11 ± 5 versus 13 ± 6 mmHg, P = 0.01) between RHC-1 and RHC-2, particularly for patients who were started on PH medications. Overall, 30% of patients who had a repeat RHC experienced a PAWP class change between their initial and follow-up RHC, independent of whether a PH medication was added. Patients initially classified as World Health Organization group 2 PH were most likely to change PAWP class over time. In conclusion, PAWP values commonly change to a significant degree in SSc-PH, which highlights the challenges in using a single time-point PAWP to define clinical classification groups.

ECG Markers of Hemodynamic Improvement in Patients with Pulmonary Hypertension.

Introduction Several diagnostic tests have been recommended for risk assessment in pulmonary hypertension (PH), but the role of electrocardiography (ECG) in monitoring of PH patients has not been yet established. Therefore the aim of the study was to evaluate which ECG patterns characteristic for pulmonary hypertension can predict hemodynamic improvement in patients treated with targeted therapies. Methods Consecutive patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) were eligible to be included if they had had performed two consecutive right heart catheterization (RHC) procedures before and after starting of targeted therapies. Patients were followed up from June 2009 to July 2017. ECG patterns of right ventricular hypertrophy according to American College of Cardiology Foundation were assessed. Results We enrolled 80 patients with PAH and 11 patients with inoperable CTEPH. The follow-up RHC was performed within 12.6 ± 10.0 months after starting therapy. Based on median change of pulmonary vascular resistance, we divided our patients into two subgroups: with and without significant hemodynamic improvement. RV1, max⁡RV1,2 + max⁡SI,aVL − SV1, and PII improved along with the improvement of hemodynamic parameters including PVR. They predicted hemodynamic improvement with similarly good accuracy as shown in ROC analysis: RV1 (AUC: 0.75; 95% CI: 0.63–0.84), PII (AUC: 0.67, 95% CI: 0.56–0.77), and max⁡RV1,2 + max⁡SI,aVL − SV1 (0.73; 95% CI: 0.63–0.82). In Cox regression only change in RV1 remained significant mortality predictor (HR: 1.12, 95% CI: 1.01–1.24). Conclusion Electrocardiogram may be useful in predicting hemodynamic effects of targeted therapy in precapillary pulmonary hypertension. Decrease of RV1, max⁡RV1,2 + max⁡SI,aVL − SV1, and PII corresponds with hemodynamic improvement after treatment. Of these changes a decrease of R wave amplitude in V1 is associated with better survival.

Progression of mean pulmonary arterial pressure in idiopathic pulmonary fibrosis with mild to moderate restriction.

Elevation of mean pulmonary arterial pressure (MPAP) is associated with poor prognosis in patients with idiopathic pulmonary fibrosis (IPF), yet the progression of MPAP in patients with IPF has not been sufficiently elucidated. We evaluated serial changes in MPAP and its determinants in patients with IPF with mild to moderate restriction. We retrospectively reviewed patients with IPF who underwent initial evaluations including right heart catheterization (RHC) in our institute from May 2007 to December 2013 with follow-up RHC at least 1 year later. Patients with forced vital capacity (FVC) < 50% predicted or those with pulmonary artery wedge pressure >15 mm Hg were excluded. A total of 95 patients were included. Median follow-up time of second RHC was 1.8 years. MPAP increased significantly at follow-up (from 16.8 to 20.2 mm Hg; P < 0.001), and annual change in MPAP (ΔMPAP) was 1.8 mm Hg/year. In multiple regression analysis, the lowest oxygen saturation (SpO2 ) at 6-min walk test (6MWT) was an independent predictor of ΔMPAP. When adjusted for age, sex, baseline MPAP and FVC % predicted, ΔMPAP was a significant predictor of mortality (hazard ratio: 1.21; P = 0.001). ΔMPAP was significantly associated with desaturation in the 6MWT, and with increased mortality in patients with IPF with mild to moderate restriction.

Rapid initiation of intravenous epoprostenol infusion is the favored option in patients with advanced pulmonary arterial hypertension.

BackgroundIntravenous infusion (IVI) of epoprostenol is an effective treatment for patients with advanced pulmonary arterial hypertension (PAH). However, there is no widely accepted standard method for initiating the IVI therapy. This study evaluated the hemodynamic improvements achieved with IVI epoprostenol to determine the optimal protocol for treatment initiation.Methods and ResultsWe retrospectively analyzed 42 consecutive PAH patients who underwent IVI epoprostenol in Keio University Hospital from 2001 to 2013. The study group comprised 30 women with a mean age of 34.3 ± 1.9 years. The etiology of PAH was idiopathic or heritable PAH (I/HPAH) in 38 cases, PAH associated with connective tissue disease in 3, and Eissenmenger’s syndrome in the remaining case. We divided the patients into rapid- and slow-initiation therapy groups according to the cumulative epoprostenol dose administered during the first 180 days, and compared the hemodynamic changes between the groups. The median cumulative doses were 6142 ± 165 μg/kg and 3998 ± 132 μg/kg epoprostenol, respectively. While there were no significant differences in mean pulmonary artery pressure (mPAP), pulmonary vascular resistance (PVR), or cardiac index (CI) between the groups before the IVI epoprostenol therapy, the rapid-initiation therapy group achieved significant improvements in these hemodynamic data compared with the slow-initiation therapy group (P < 0.005) at the follow-up right-heart catheterization (RHC).ConclusionRapid initiation of IVI epoprostenol therapy achieved the optimal hemodynamic improvements in patients with severe PAH.

Abstract 18178: Cardiac Index Declines During Long-term LVAD Support

Background: It is increasingly common for patients to be supported by left ventricular assist devices (LVADs) for an extended period of time. At our institution, we have anecdotally noticed a decline in functional status during the course of prolonged LVAD therapy. Our objective was to analyze hemodynamic and echocardiographic data of patients with at least 2 years of ongoing LVAD support. Methods: We retrospectively reviewed all patients who underwent HeartMate II implantation between 2005 and 2012. Of the 131 patients reviewed, we identified 20 patients who were supported by LVADs for at least 2 years and had right heart catheterization (RHC) in both the initial postoperative period and at 2-3 year follow-up. No patients were on inotropic therapy at the time of RHC. Results: The mean times of initial and follow-up post-LVAD RHCs were 59 ± 41 days and 889 ± 160 days, respectively. Cardiac index (CI) declined by an average of 0.4 L/min/m2 over the study period (p=0.04). Other than a reduction in transpulmonary gradient, no other hemodynamic differences were observed. There was a statistically significant worsening of aortic insufficiency (AI) at follow-up. However, when dichotomized by the median change in CI (-0.4 L/min/m2), there is no difference in AI between groups (p =0.60). In fact, 2 of the 3 patients with moderate AI had an increase in CI. There were no differences in pump speed, mean pressure, aortic valve opening, or lactate dehydrogenase between initial and follow up periods (Table 1). Conclusion: In the first study to report long term hemodynamic follow up in patients supported by HeartMate II LVADs, cardiac index significantly declined over time. The decline was not the result of worsening right ventricular function or valvular regurgitation nor a difference in pump speed or blood pressure. This is a potentially worrisome finding, especially for destination therapy patients. Further studies are needed to understand what factors may contribute to this decline.

Experience with exercise right heart catheterization in the diagnosis of pulmonary hypertension: a retrospective study

&#x0D; &#x0D; &#x0D; &#x0D; Background: Data on exercise pulmonary hemodynamics in healthy people and patients with pulmonary hypertension (PH) are rare. We analyzed exercise right heart catheterization (RHC) data in a symptomatic collective referred with suspected PH to characterize the differential response by diagnostic groups, to correlate resting with exercise hemodynamics, and to evaluate safety.&#x0D; Methods: This is a retrospective single-center study reviewing data from patients in whom an exercise RHC was performed between January 2006 and January 2013. Patients with follow-up RHC under PH -therapy were excluded.&#x0D; Results: Data from 101 patients were analyzed, none of them had an adverse event. In 35% we detected a resting PH (27.8% precapillary, 6.9% postcapillary). Exercise PH (mean pulmonary arterial pressure (mPAP) &gt;30 mmHg at exercise) was found in 38.6%, whereas in 25.7% PH was excluded. We found a remarkable number of exercise PH in scleroderma patients, the majority being postcapillary. 83% of patients with mPAP-values between 20 and 24.9 mmHg at rest had exercise PH. Patients with resting PH had worse hemodynamics and were older compared with exercise PH ones.&#x0D; Conclusion: In this real-life experience in symptomatic patients undergoing exercise RHC for suspected PH, we found that exercise RHC is safe. The facts that the vast majority of patients with mPAP-values between 20 and 24.9 mmHg at rest had exercise PH and the older age of patients with resting PH may indicate that exercise PH is a precursor of resting PH. Whether earlier treatment start in patients with exercise PH would stabilize the disease should be addressed in future studies.&#x0D; &#x0D; &#x0D; &#x0D;

Long-term patient survival with idiopathic/heritable pulmonary arterial hypertension treated at a single center in Japan

AimsIdiopathic/heritable pulmonary arterial hypertension (I/HPAH) carries a poor prognosis despite the therapeutic options available. Patient survival from Western countries has been reported, but data from Asia are scarce. Main methodsWe retrospectively reviewed 56 patients with I/HPAH treated at a single referral center in Japan. Survival analyses were conducted using the Kaplan–Meier method with the log-rank test. Variables associated with survival were determined using a Cox proportional hazard model. Key findingsThere were 41 women (73%) and the mean age at the diagnosis was 32±17years. Mean survival time from the diagnosis was 14.9±0.8years (95% CI, 13.4–16.4years), with 1-, 2-, 3-, 5- and 10-year survival rates of 98, 96, 96, 96 and 78%, respectively. In patients who underwent follow-up right-heart catheterization >3months after initial catheterization, mean pulmonary arterial pressure (mPAP) was decreased significantly from 63±15 to 35±10mmHg with an improved cardiac index. Patients with high levels of brain natriuretic peptide (BNP) or low oxygen saturation at baseline showed worse survival. At follow-up, 98% of patients were on PAH-targeted drugs. WHO functional classes I and II, mPAP <42.5mmHg, cardiac index >2.5L/min/m2, BNP <52pg/mL, and 6-min walk distance >347m at follow-up were predictors of good prognosis in the univariate analysis. SignificanceThe study revealed a long-term survival of Japanese patients with I/HPAH. Hemodynamic parameters improved significantly after treatment, which might be related to high prescription rates of PAH-targeted drugs. Multicenter studies are needed to reveal the prognostic factors for I/HPAH.

Experience with exercise right heart catheterization in the diagnosis of pulmonary hypertension: a retrospective study.

BackgroundData on exercise pulmonary hemodynamics in healthy people and patients with pulmonary hypertension (PH) are rare. We analyzed exercise right heart catheterization (RHC) data in a symptomatic collective referred with suspected PH to characterize the differential response by diagnostic groups, to correlate resting with exercise hemodynamics, and to evaluate safety.MethodsThis is a retrospective single-center study reviewing data from patients in whom an exercise RHC was performed between January 2006 and January 2013. Patients with follow-up RHC under PH -therapy were excluded.ResultsData from 101 patients were analyzed, none of them had an adverse event. In 35% we detected a resting PH (27.8% precapillary, 6.9% postcapillary). Exercise PH (mean pulmonary arterial pressure (mPAP) >30 mmHg at exercise) was found in 38.6%, whereas in 25.7% PH was excluded. We found a remarkable number of exercise PH in scleroderma patients, the majority being postcapillary. 83% of patients with mPAP-values between 20 and 24.9 mmHg at rest had exercise PH. Patients with resting PH had worse hemodynamics and were older compared with exercise PH ones.ConclusionIn this real-life experience in symptomatic patients undergoing exercise RHC for suspected PH, we found that exercise RHC is safe. The facts that the vast majority of patients with mPAP-values between 20 and 24.9 mmHg at rest had exercise PH and the older age of patients with resting PH may indicate that exercise PH is a precursor of resting PH. Whether earlier treatment start in patients with exercise PH would stabilize the disease should be addressed in future studies.

Effect of PAH specific therapy on pulmonary hemodynamics and six-minute walk distance in portopulmonary hypertension: a systematic review and meta-analysis.

Background. Little is known about the effect of pulmonary arterial hypertension (PAH) specific therapy on pulmonary hemodynamics and exercise capacity in patients with portopulmonary hypertension (PoPH) because such patients are usually excluded from randomized clinical trials (RCT) of such therapy. Methods. We searched PUBMED using the terms “(Therapy/Broad (filter)) AND (portopulmonary hypertension).” We included studies that met the following criteria: ≥5 patients, AND PoPH confirmed by right heart catheterization (RHC), AND follow-up RHC data, AND/OR baseline and follow-up 6MWD available. Results. 12 studies met our inclusion criteria. None was a RCT. The baseline mPAP was 48.6 ± 4.4 mmHg, cardiac output (CO) 5.6 ± 0.9 L/min, and pulmonary vascular resistance (PVR) 668.6 ± 219.1 dynes.sec/cm5. The baseline 6MWD was 348.2 ± 35.6 meters. The use of PAH specific therapy improved mPAP by 7.54 mmHg (95% CI 10.2 to 4.9), CO by 1.77 L/min (95% CI 1.1 to 2.4), and PVR by 253 dynes.sec/cm5 (95% CI 291.4 to 214.6) (n = 135) and 6MWD by 61.8 meters (95% CI 47.5 to 76) (n = 122). Conclusions. The use of PAH specific therapy in PoPH results in significant improvement in both pulmonary hemodynamics and 6MWD.

Long-Term Pulmonary Hemodynamic Effects of Ambrisentan in Pulmonary Arterial Hypertension

The long-term effects of endothelin receptor antagonists on pulmonary arterial pressure (PAP) and pulmonary vascular resistance (PVR) in patients with pulmonary arterial hypertension (PAH) are not well studied. This post hoc analysis examined changes in pulmonary hemodynamics in a cohort of patients with PAH who underwent follow-up right heart catheterization (RHC) in a long-term ambrisentan study (ARIES-E). A retrospective review was conducted of patients who underwent RHC after >3 months of ambrisentan therapy. Changes from baseline in mean PAP, mean right atrial pressure, cardiac index, and PVR were assessed and correlations between these hemodynamic changes and exercise capacity were examined. Sixty-eight patients who received ambrisentan in the ARIES studies had ≥1 follow-up RHC while receiving ambrisentan. Fifty-eight patients were on ambrisentan alone at the time of the first RHC. Median time from initiation of ambrisentan therapy to follow-up RHC was 60 weeks (range 14 to 158). Significant improvements compared to baseline were observed for mean PAP (-7.6 mm Hg, 95% confidence interval [CI] -10.0 to -5.1), PVR (-266 dyne × s/cm(5), 95% CI -350 to -180), and cardiac index (0.4 L/min/m(2), 95% CI 0.2 to 0.6 L/min/m(2)); for patients on ambrisentan alone, changes in mean PAP and PVR were inversely correlated with change from baseline 6-minute walking distance (r = -0.41 and -0.43, respectively, p <0.001 for the 2 comparisons) at time of follow-up RHC. In conclusion, ambrisentan may provide sustained improvements in pulmonary hemodynamics in patients with PAH who receive long-term treatment and these changes correlate with improvements in exercise capacity.