Ryoji Yanagisawa

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.

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.

Efficacy of 360-degree three-dimensional rotational pulmonary angiography to guide percutaneous transluminal pulmonary angioplasty

Percutaneous transluminal pulmonary angioplasty (PTPA) is performed with balloon dilatation of the target lesions in the pulmonary arteries through a catheter (Online Figure 1A-Online Figure 1C, Moving image 1, Moving image 2). We routinely perform pulmonary angiography (PAG) using a rotational digital angiography system (Artis zeego multi-axis; Siemens AG, Forchheim, Germany) before the PTPA procedure (Moving image 3). A 360-degree three-dimensional (3D) PAG image is used for identifying the target lesions and estimating their perfusion area easily and rapidly, in addition to identifying the anatomical structures of the pulmonary arteries (Figure 1A, Moving image 4). A rotational 3D PAG image can help us develop therapeutic strategies during PTPA procedures, including guide catheter and balloon size selection. Furthermore, we can visualise the pulmonary artery anatomy in synchronisation with the realtime angiographic view during the procedure, resulting in safe and easy wiring. The rotational 3D PAG image after PTPA is shown in Figure 1B and Moving image 5. Accordingly, this rotational 3D PAG image contributes to the safe and easy performance of PTPA and reduces the amount of contrast agent, leading to a reduction in radiation exposure.

Alu-mediated nonallelic homologous and nonhomologous recombination in the BMPR2 gene in heritable pulmonary arterial hypertension

Purpose:The purpose of this study was to undertake thorough genetic analysis of the bone morphogenetic protein type 2 receptor (BMPR2) gene in patients with pulmonary arterial hypertension.Methods:We conducted a systematic analysis for larger gene rearrangements together with conventional mutation analysis in 152 pulmonary arterial hypertension patients including 43 patients diagnosed as having idiopathic pulmonary arterial hypertension and 10 diagnosed as having familial pulmonary arterial hypertension.Results:Analysis of the BMPR2 gene revealed each of the four kinds of nonsense and frameshift mutations, one missense mutation, one splice-site mutation, and two types of exonic deletion. For cases in which exons 1–3 were deleted, the 5′ and 3′ break points were located in the AluY repeat sequences in the 5′ side of the adjacent NOP58 gene and in the AluY repeat sequences in intron 3, suggesting an AluY-mediated nonallelic homologous recombination as the mechanism responsible for the deletion. For the case in which exon 10 was deleted, nonhomologous recombination took place between the AluSx site in intron 9 and a unique sequence in intron 10.Conclusion:Exonic deletions of BMPR2 account for at least part of BMPR2 mutations associated with heritable pulmonary arterial hypertension in Japan, as previously reported in other populations. One of our cases was mediated via Alu-mediated nonallelic homologous recombination and another was mediated via nonhomologous recombination.Genet Med 15 12, 941–947.Genetics in Medicine (2013); 15 12, 941–947. doi:10.1038/gim.2013.41

Long-Term Outcomes After Percutaneous Transluminal Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension

Therapeutic options for patients with chronic thromboembolic pulmonary hypertension (CTEPH) have expanded significantly with the development of catheter-based interventional therapy, namely percutaneous transluminal pulmonary angioplasty (PTPA) or so-called balloon pulmonary angioplasty (BPA). Conventionally, surgical pulmonary endarterectomy has been the first-line treatment for operable CTEPH. Several teams, including our own, have developed PTPA procedures, and some important reports have been published.1–4 Extrapolating from the published papers, about one third of patients with confirmed CTEPH in Japan have been treated with PTPA over the past decade. It is important to note that according to updated guidelines for the diagnosis and treatment of pulmonary hypertension published in 2016 by the European Society of Cardiology and the European Respiratory Society,5 this catheter-based therapy has been accepted as a therapeutic strategy (Class IIb recommendation for patients with technically inoperable disease or with unfavorable risk-to-benefit ratio for surgery). Because PTPA has only recently been developed, long-term outcomes have not been reported thus far. Therefore, the purpose of the present observational study was to clarify the long-term outcomes for PTPA. The performance of PTPA and analysis of clinical data in the present study were approved by the institutional review boards of Kyorin University Hospital, and all patients …

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

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.

Intravascular imaging-guided percutaneous transluminal pulmonary angioplasty for peripheral pulmonary stenosis and pulmonary Takayasu arteritis

Intravascular imaging-guided percutaneous transluminal pulmonary angioplasty for peripheral pulmonary stenosis and pulmonary Takayasu arteritis Ryoji Yanagisawa, MD, Masaharu Kataoka, MD, Takumi Inami, MD, Keiichi Fukuda, MD, Hideaki Yoshino, MD, and Toru Satoh, MD From the Division of Cardiology, Second Department of Internal Medicine, Kyorin University School of Medicine, Tokyo, Japan; and the Department of Cardiology, Keio University School of Medicine, Tokyo, Japan

Diversity of Lesion Morphology in CTEPH Analyzed by OCT, Pressure Wire, and Angiography

Percutaneous transluminal pulmonary angioplasty (PTPA), or balloon pulmonary angioplasty, has been the focus of much attention and has rapidly been established as a promising new therapy for chronic thromboembolic pulmonary hypertension (CTEPH) in recent years [(1,2)][1]. The procedures have been

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

ABSTRACT Introduction: Percutaneous transluminal pulmonary angioplasty (PTPA) was introduced for the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) in the late 20th 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.