Evaluation of Right Ventricular Function on Cardiac Magnetic Resonance Imaging and Correlation With Hemodynamics in Patients With Chronic Thromboembolic Pulmonary Hypertension.

Background: Balloon pulmonary angioplasty (BPA) is an alternative therapy for chronic thromboembolic pulmonary hypertension (CTEPH). Right heart catheterization (RHC) is essential to evaluate the efficacy of BPA. Cardiac magnetic resonance imaging (CMR) is also now used to assess the structure and function of the right heart non-invasively. The aim of this study was to correlate improvement in CMR with that on RHC, and compared with improvement in other non-invasive findings after BPA. Methods and Results: Forty-two patients underwent BPA between July 2012 and March 2015, and CMR, electrocardiography (ECG), and echocardiography were performed at the same time before and 6 months after BPA. Median pulmonary vascular resistance (PVR) was improved from 5.7 Wood units (IQR, 3.1-7.9 Wood units) to 2.7 Wood units (IQR, 1.6-3.9 Wood units; P<0.001). Changes in PVR were correlated with the changes in 5 CMR, 9 ECG, and 5 echocardiography parameters. On logistic analysis to identify the indicators of improving PH (i.e., PVR <3 Wood units), 4 CMR parameters were independently correlated with PVR change, one of which was median septal inversion ratio (SIR; 0.59; IQR, 0.54-0.63 to 0.54; IQR, 0.50-0.58, P<0.0001). SIR was the best predictor of PH (OR, 1.27; P<0.05). Conclusions: CMR can be used to estimate hemodynamic changes after BPA, and SIR is useful to predict alleviation of PH.

Endovascular treatment for chronic pulmonary hypertension: a focus on angioplasty for chronic thromboembolic pulmonary hypertension.

INTRODUCTION: Percutaneous transluminal pulmonary angioplasty (PTPA) was introduced for the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) in the late 20(th) century, and first attempts in collective patients were made in 2001 with beneficial effects but a moderate amount of complications. It was refined around 2010, and has been recently established as an effective and safe treatment. AREAS COVERED: The indication was originally inoperable CTEPH with peripheral lesions, but has now widened to symptomatic or hypoxic patients. The lesion is typically a meshwork-like structure of organized thrombi and is sometimes not seen as a stenosis angiographically, necessitating other means of investigation such as measurement of distal pressure. The technique to treat lesions is the same as for coronary angioplasty except in several ways. Expert commentary: The effects of PTPA are comparable to those of surgical endarterectomy, and the complications of reperfusion pulmonary edema and vascular injury are now controlled by several strategies and based on experience.

Additional percutaneous transluminal pulmonary angioplasty for residual or recurrent pulmonary hypertension after pulmonary endarterectomy.

BACKGROUND: Pulmonary endarterectomy (PEA) has been the most effective therapy for chronic thromboembolic pulmonary hypertension (CTEPH). However, residual or recurrent pulmonary hypertension often persists after PEA. Recently, catheter-based angioplasty, called percutaneous transluminal pulmonary angioplasty (PTPA) or balloon pulmonary angioplasty, has been developed as a promising strategy for CTEPH. Therefore, the usefulness of PTPA for residual or recurrent pulmonary hypertension after PEA was investigated. METHODS: Thirty-nine patients underwent PEA from January 2000, and a total of 423 consecutive PTPA sessions in 110 patients were performed from January 2009 to May 2014. Of them, 9 patients (23.0% of 39 patients undergoing PEA and 8.2% of 110 patients undergoing PTPA) had undergone previous PEA and additional PTPA. RESULTS: In these 9 patients, pulmonary vascular resistance (PVR) was 15.6 (7.8-18.9) wood units at baseline, and significantly improved after PEA [5.6 (3.5-6.5) wood units] (p<0.05). However, PVR gradually deteriorated before PTPA [8.1 (6.1-12.3) wood units] compared to after PEA, suggesting that these 9 patients had residual or recurrent pulmonary hypertension after PEA. PTPA was performed at 4.1 (2.7-7.9) years after PEA. Follow-up catheterization at 1.9 (1.3-3.3) years after PTPA revealed significant improvement of PVR [4.2 (2.8-4.8) wood units] (p<0.05). CONCLUSIONS: A hybrid approach combining PEA and additional PTPA may be reasonable for patients with both proximal and very distal lesions not easily approachable by PEA. PTPA could be a promising alternative therapeutic strategy for residual or recurrent pulmonary hypertension after PEA.

Safety and efficacy of percutaneous transluminal pulmonary angioplasty in elderly patients.

BACKGROUND: Percutaneous transluminal pulmonary angioplasty (PTPA) is a recently developed catheter-based therapy for chronic thromboembolic pulmonary hypertension (CTEPH). The aim of the present study was to investigate the safety and efficacy of PTPA in elderly patients with CTEPH. METHODS: In all, 257 PTPA sessions in 70 patients (median age 63 years) were analyzed. Patients were divided into two groups according to age: (i) a younger group (<65 years; n=39); and (ii) an elderly group (≥65 years; n=31). RESULTS: Hemodynamic improvements were comparable between the younger and elderly groups (63.1% vs. 68.2% decrease in pulmonary vascular resistance, respectively; P>0.05). The median length of stay in the intensive care unit after each session (1.0 vs. 1.0 days) and in hospital per session (9.2 vs. 9.4 days) was similar between the two groups (P>0.05 for all). The prevalence of reperfusion pulmonary edema (23.4% vs. 26.3% across all sessions) and other complications, such as contrast dye-induced nephropathy (0% vs. 2.0%), infection (0% vs. 0%), and neurological complications (0% vs. 1.0%), was comparable in the younger vs. elderly groups (P>0.05 for all). One-year all-cause mortality was similar in the younger and elderly groups (0% vs. 3.2%, respectively; P>0.05). CONCLUSIONS: PTPA can be performed safely and effectively, even in elderly patients, and could be considered as an alternative therapeutic strategy for elderly patients who are too fragile for pulmonary endarterectomy (PEA) or who are treated in institutions without highly experienced PEA surgeons.

Pressure-wire-guided percutaneous transluminal pulmonary angioplasty: a breakthrough in catheter-interventional therapy for chronic thromboembolic pulmonary hypertension.

OBJECTIVES: This study sought to prove the safety and effectiveness of pressure-wire-guided percutaneous transluminal pulmonary angioplasty (PTPA). BACKGROUND: PTPA has been demonstrated to be effective for treatment of chronic thromboembolic pulmonary hypertension. However, a major and occasionally fatal complication after PTPA is reperfusion pulmonary edema. To avoid this, we developed the PEPSI (Pulmonary Edema Predictive Scoring Index). The pressure wire has been used to detect insufficiency of flow in a vessel. METHODS: We included 350 consecutive PTPA sessions in 103 patients with chronic thromboembolic pulmonary hypertension from January 1, 2009 to December 31, 2013. During these 5 years, 140 PTPA sessions were performed without guidance, 65 with guidance of PEPSI alone, and 145 with both PEPSI and pressure-wire guidance. Each PTPA session was finished after achieving PEPSI scores of <35.4 with PEPSI guidance and each target lesion achieving distal mean pulmonary arterial pressure <35 mm Hg with pressure-wire guidance. RESULTS: The occurrence of clinically critical reperfusion pulmonary edema and vessel injuries were lowest in the group using the guidance of both pressure wire and PEPSI (0% and 6.9%, respectively). Furthermore, the group guided by pressure wire and PEPSI accomplished the same hemodynamic improvements with fewer numbers of target lesions treated and sessions performed. CONCLUSIONS: The combined approach using pressure wire and PEPSI produced more efficient clinical results and greatly reduced reperfusion pulmonary edema and vessel complications. This is further evidence that PTPA is an alternative strategy for treating chronic thromboembolic pulmonary hypertension.

Pulmonary edema predictive scoring index (PEPSI), a new index to predict risk of reperfusion pulmonary edema and improvement of hemodynamics in percutaneous transluminal pulmonary angioplasty.

OBJECTIVES: This study sought to identify useful predictors for hemodynamic improvement and risk of reperfusion pulmonary edema (RPE), a major complication of this procedure. BACKGROUND: Percutaneous transluminal pulmonary angioplasty (PTPA) has been reported to be effective for the treatment of chronic thromboembolic pulmonary hypertension (CTEPH). PTPA has not been widespread because RPE has not been well predicted. METHODS: We included 140 consecutive procedures in 54 patients with CTEPH. The flow appearance of the target vessels was graded into 4 groups (Pulmonary Flow Grade), and we proposed PEPSI (Pulmonary Edema Predictive Scoring Index) = (sum total change of Pulmonary Flow Grade scores) × (baseline pulmonary vascular resistance). Correlations between occurrence of RPE and 11 variables, including hemodynamic parameters, number of target vessels, and PEPSI, were analyzed. RESULTS: Hemodynamic parameters significantly improved after median observation period of 6.4 months, and the sum total changes in Pulmonary Flow Grade scores were significantly correlated with the improvement in hemodynamics. Multivariate analysis revealed that PEPSI was the strongest factor correlated with the occurrence of RPE (p < 0.0001). Receiver-operating characteristic curve analysis demonstrated PEPSI to be a useful marker of the risk of RPE (cutoff value 35.4, negative predictive value 92.3%). CONCLUSIONS: Pulmonary Flow Grade score is useful in determining therapeutic efficacy, and PEPSI is highly supportive to reduce the risk of RPE after PTPA. Using these 2 indexes, PTPA could become a safe and common therapeutic strategy for CTEPH.

Percutaneous transluminal pulmonary angioplasty for the treatment of chronic thromboembolic pulmonary hypertension.

BACKGROUND: Chronic thromboembolic pulmonary hypertension leads to pulmonary hypertension and right-sided heart failure. The purpose of this study was to investigate the efficacy of percutaneous transluminal pulmonary angioplasty (PTPA) for the treatment of chronic thromboembolic pulmonary hypertension. METHODS AND RESULTS: Twenty-nine patients with chronic thromboembolic pulmonary hypertension underwent PTPA. One patient had a wiring perforation as a complication of PTPA and died 2 days after the procedure. In the remaining 28 patients, PTPA did not produce immediate hemodynamic improvement at the time of the procedure. However, after follow-up (6.0 ± 6.9 months), New York Heart Association functional classifications and levels of plasma B-type natriuretic peptide significantly improved (both P<0.01). Hemodynamic parameters also significantly improved (mean pulmonary arterial pressure, 45.3 ± 9.8 versus 31.8 ± 10.0 mm Hg; cardiac output, 3.6 ± 1.2 versus 4.6 ± 1.7 L/min, baseline versus follow-up, respectively; both P<0.01). Twenty-seven of 51 procedures in total (53%), and 19 of 28 first procedures (68%), had reperfusion pulmonary edema as the chief complication. Patients with severe clinical signs and/or severe hemodynamics at baseline had a high risk of reperfusion pulmonary edema. CONCLUSIONS: PTPA improved subjective symptoms and objective variables, including pulmonary hemodynamics. PTPA may be a promising therapeutic strategy for the treatment of chronic thromboembolic pulmonary hypertension. CLINICAL TRIAL REGISTRATION: URL: http://www.umin.ac.jp. Unique identifier: UMIN000001572.

Left ventricular dysfunction due to diffuse multiple vessel coronary artery spasm can be concealed in dilated cardiomyopathy.

AIMS: Many patients with idiopathic dilated cardiomyopathy (DCM) have been diagnosed on the basis of the exclusion of significant coronary stenosis and the presence of left ventricular (LV) dysfunction. In the present study, we investigated the possibility that coronary multispasm is one of the mechanisms leading to diffuse idiopathic DCM-like LV dysfunction. METHODS AND RESULTS: Forty-two patients with severely depressed LV function but without significant coronary stenosis were enrolled (baseline LV ejection fraction, 33 ± 11%). An acetylcholine (ACh) provocation test was performed at the time of coronary angiography. In patients with a positive ACh provocation test (n = 20), coronary angiography revealed multivessel diffuse coronary spasm with marked electrocardiogram changes. In patients with a negative ACh provocation test (n = 22), significant findings compatible with idiopathic DCM were more frequently observed on magnetic resonance imaging (MRI) or in LV biopsies compared with the ACh-positive group (MRI, 73% vs. 12%; and LV biopsy, 71% vs. 0%, respectively; P < 0.01). In the ACh-positive group, LV function significantly improved after the administration of calcium channel blockers (LV ejection fraction, 34 ± 12% vs. 54 ± 10%; and brain natriuretic peptide, 803 ± 482 pg/mL vs. 69 ± 84 pg/mL, at baseline and 1 year, respectively; P < 0.01). CONCLUSIONS: Our results raise the possibility that patients with LV dysfunction due to repeated coronary multispasm are being diagnosed as idiopathic DCM, and that calcium channel blockers may prove to be a promising therapeutic strategy in those patients.