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Background: Although previous studies have demonstrated that paediatric pulmonary arterial hypertension remains distinct from that in adults, there are limited studies evaluating a direct comparison between children and adults. The aim of this head-to-head comparison study was to compare the gender, haemodynamic parameters, and prognosis between paediatric and adult pulmonary arterial hypertension. Methods and Results: We retrospectively assessed the clinical differences in 40 childhood-onset (under 20 years old) patients and 40 adult-onset patients with idiopathic and heritable pulmonary arterial hypertension who were followed up at two centres. There was no female predominance among patients with childhood-onset pulmonary arterial hypertension (child female: 42.5%, adult female: 80%). The percent of New York Heart Association functional class IV in adult-onset pulmonary arterial hypertension tended to be higher than those in childhood-onset pulmonary arterial hypertension (22.5 and 10%, respectively), although children had worse haemodynamic parameters at diagnosis (mean pulmonary artery pressure (children versus adults); median 65 mmHg versus 49 mmHg, p < 0.001). There was no significant difference in the event-free survival rate between the two groups (95% vs. 85%) during the follow-up period (median, 96 months; range, 1–120 months). Conclusions: Although paediatric pulmonary arterial hypertension patients had worse haemodynamic parameters at diagnosis than adults, children survived as long as adults with appropriate therapeutic strategies.

Mean pulmonary arterial pressure (mPAP) has not been recognized as a therapeutic target for pulmonary arterial hypertension (PAH). However, previous reports demonstrated that the survival of patients with some types of pulmonary hypertension was associated with mPAP. Therefore, we treated all subsets of PAH patients with the aim of lowering mPAP and evaluated the efficacy of the treatment algorithm.From September 2014 to August 2019, 43 consecutive patients with PAH on treatment3 months on referral were enrolled in this study. They were treated according to our treatment algorithm mainly based on World Health Organization functional class, mPAP, and percent predicted diffusing capacity of the lung for carbon monoxide. The therapeutic goal was the achievement of mPAP40 mmHg at follow-up. We evaluated clinical parameters, survival rate, and its determinants. Survival analyses were conducted using the Kaplan-Meier method and the relationship between all-cause death and selected variables was analyzed using the Cox proportional hazards model for mortality.mPAP significantly improved at the last follow-up [45 (35-55) mmHg to 24 (19-30) mmHg, p 0.05], and the therapeutic goal was achieved in 37 patients (86%). The 5-year survival rate of all patients was 90.7%. The survival rate of patients who achieved the therapeutic goal (3- and 5-year survival: 97.3%) was significantly better than that of the patients who did not achieve the therapeutic goal (3-year survival: 50.0%, p 0.05). The 5-year survival rates of the patients were the same among different treatment regimens (p = 0.985). mPAP40 mmHg at follow-up was the independent predictor of survival.Lowering mPAP resulted in favorable outcomes in all subsets of PAH patients, making it a therapeutic goal.

Balloon pulmonary angioplasty (BPA) is now a treatment option for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). However, the incidence of restenosis and long-term changes in vessel diameters in pulmonary arteries after BPA are unknown. The present study investigated the incidence of restenosis by measuring changes in vessel diameter after BPA. We reviewed 58 patients (168 lesions) with CTEPH who underwent single dilation for the target lesion (type A/B/C lesions) during BPA procedure followed by selective pulmonary angiography more than 6 months after the final BPA procedure. The outcomes of BPA were assessed in terms of pulmonary artery diameters. In a median follow-up of 1.9 (1.2-2.7) years, restenosis occurred in only one case with a type C lesion after BPA (0.6%). In type A/B lesions, the minimal lumen diameter was significantly enlarged at follow-up after BPA [3.48 (2.59-4.34) to 4.22 (3.31-4.90) mm]. In type C lesions, the minimal lumen diameter was unchanged at follow-up after BPA [3.15 (1.96-3.64) to 3.28 (2.38-4.61) mm]. The present results revealed that restenosis after BPA rarely occurs in type A/B/C lesions. Minimal lumen diameters for type A/B lesions continually increased and those for type C lesions did not decrease. Stent implantation in type A/B/C lesions would be unnecessary after BPA.

Pulmonary injury is a major complication of balloon pulmonary angioplasty (BPA) for chronic thromboembolic pulmonary hypertension (CTEPH). Lung injury after BPA can be exacerbated by a high mean pulmonary arterial pressure (PAP). Although oxygen inhalation is expected to lower mean PAP in patients with CTEPH, no relevant investigation has been conducted.Consecutive patients with CTEPH who underwent BPA were enrolled in this study. We evaluated the hemodynamics using right heart catheterization while breathing ambient air and with administration of 5 L/min oxygen for 10 min.This study included 52 consecutive patients with CTEPH, of whom 23 (44%) were treated with specific pulmonary vasodilators. Exposure to oxygen was well tolerated. Oxygen administration significantly decreased mean PAP by 3.8 ± 3.2 mmHg (p0.001) and pulmonary vascular resistance by 0.8 ± 1.8 Wood units (p0.001). Moreover, the ratio of pulmonary vascular resistance to systemic vascular resistance was significantly reduced by 13.5% (p0.001). Multivariate regression analysis identified baseline mean PAP (β = -0.427, p = 0.006) as the only significant predictor of decreased mean PAP under oxygen administration. No significant difference in oxygen effect on mean PAP was found between patients with and without vasodilators.In patients with CTEPH, 5 L/min supplemental oxygen inhalation could decrease mean PAP significantly by selective pulmonary artery dilatation, regardless of the usage of vasodilators, and thus could be helpful to maximize the safety of BPA.UMIN Clinical Trials Registry (No.: UMIN000026882); URL: https://www.umin.ac.jp/ctr/index.htm.

Lung injury is a serious complication of balloon pulmonary angioplasty (BPA) for chronic thromboembolic pulmonary hypertension (CTEPH). Selecting a smaller balloon might minimize the occurrence of lung injury; however, it could also increase the risk of restenosis.We analyzed the first target lesions in the initial BPA procedure in 34 consecutive patients with CTEPH. We measured proximal pressure with a guiding catheter and distal pressure with a pressure wire. We retrospectively investigated pressure gradients at the lesions before and immediately after the initial dilation with a smaller balloon and before the second procedure.The proximal pressure decreased, and the distal pressure increased immediately after the initial balloon dilation. The pressure gradient at the lesion diminished significantly by the second procedure (from 26.4 ± 10.7 to 18.2 ± 9.9 mm Hg, P0.001) without serious complications. The residual pressure gradient had further diminished (to 9.8 ± 6.5 mm Hg, P = 0.02) until the second procedure. The reduction in pressure gradient at the lesion from immediately after the initial balloon dilation to the second procedure was positively correlated with both the baseline mean pulmonary arterial pressure (rThe residual pressure gradient at the lesion diminished continually after dilation with a smaller balloon. This strategy could decrease pulmonary arterial pressure safely. It would be reasonable to dilate the lesions sequentially in 2 procedures.

Purpose of review Balloon pulmonary angioplasty (BPA) has been performed worldwide for patients who are ineligible for pulmonary endarterectomy (PEA). However, the technical details of BPA have not been standardized, and no international consensus regarding patient and lesion selection for BPA has been reached. Evidence for the combination of BPA with PEA or medical therapy is also lacking. This review highlights recent progress in BPA in terms of patient and lesion selection and the current procedural approach for BPA, including combination treatment. Recent findings The indications for BPA have expanded with recent reports describing the improved safety and efficacy of BPA. Because lesions are generally present in all segmental and subsegmental pulmonary arteries, it is recommended to treat all the lesions to achieve desirable hemodynamic improvement. Selective pulmonary angiography is the gold standard for lesion selection in modern BPA aimed at total revascularization. Despite the lack of randomized controlled studies, combination treatment with BPA may be well tolerated and effective. Summary BPA, alone or in combination with PEA or medical therapy, may be a treatment option for patients who are not candidates for monotreatment of PEA. However, further investigation is required to standardize patient and lesion selection for BPA.

BackgroundTreatment options for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) remain limited. Selexipag, an oral selective IP prostacyclin receptor agonist approved for pulmonary arterial hypertension, is a potential treatment option for CTEPH.MethodsIn this multicentre, randomised, double-blind, placebo-controlled study, 78 Japanese patients with inoperable CTEPH or persistent/recurrent pulmonary hypertension after pulmonary endarterectomy and/or balloon pulmonary angioplasty were randomly assigned to receive placebo or selexipag. The primary end-point was the change in pulmonary vascular resistance (PVR) from baseline to week 20. Secondary end-points were changes in other haemodynamic parameters: 6-min walk distance (6MWD), Borg dyspnoea scale score, World Health Organization (WHO) functional class, EuroQol five-dimension five-level tool and N-terminal pro-brain natriuretic peptide.ResultsThe change in PVR was −98.2±111.3 dyn·s·cm−5 and −4.6±163.6 dyn·s·cm−5 in the selexipag and placebo groups, respectively (mean difference −93.5 dyn·s·cm−5; 95% CI −156.8 to −30.3; p=0.006). The changes in cardiac index (pConclusionSelexipag significantly improved PVR and other haemodynamic variables in patients with CTEPH, although exercise capacity remained unchanged. Further large-scale investigation is necessary to prove the role of selexipag in CTEPH.

Background: Pulmonary arterial hypertension (PAH) has a poor prognosis despite the available therapeutic options. Normalization of hemodynamics would be needed to obtain the good prognosis. Aims and Objectives: We aimed to investigate the determinants in PAH patients for normalization of hemodynamics only with oral drugs. Methods: We retrospectively reviewed 190 patients with PAH treated at a single referral center. Hemodynamic parameters were investigated before and after treatments. All the patients were divided into a good control group with mean pulmonary arterial pressure (mPAP)＜30mmHg and a poor control group with mPAP≧30mmHg at follow-up. Results: Eighty-two patients were idiopathic/heritable, 65 patients were connective tissue disease, 22 patients congenital heart disease and 21 patients were portal hypertension. mPAP decreased significantly from baseline to follow-up (51±18.7 mmHg to 34.1±15.2 mmHg, p Conclusions: To achieve normalization of hemodynamics in PAH, parenteral prostanoid should be used in cases with baseline mPAP exceeding 40mmHg.

Background— Balloon pulmonary angioplasty (BPA) has become an alternative therapy for patients with chronic thromboembolic pulmonary hypertension who are ineligible for pulmonary endarterectomy. We retrospectively investigated intravascular ultrasound measurements to elucidate how the lumen enlarges immediately after BPA. Methods and Results— A total of 326 lesions in 71 patients with chronic thromboembolic pulmonary hypertension were analyzed. The cross-sectional areas (CSAs) of the external elastic membrane and lumen measured by intravascular ultrasound before and after BPA were assessed for each lesion type (type A, ring-like stenosis lesions; type B, web lesions; type C, subtotal occlusion lesions). Comparing the lesion types, the proportion of the area occupied by fibrous tissue was smallest in the type A lesions and largest in the type C lesions. The mean lesion lumen CSA was increased after BPA because of an increase in external elastic membrane CSA (from 23.2±12.9 to 27.2±14.3 mm 2 ; P 2 ; P r 2 =0.762; P Conclusions— Lumen enlargement immediately after BPA was obtained by the overall vessel expansion induced by a stretch of the arterial wall. In addition, the amount of fibrous tissue depended on each lesion type. Thus, it might be beneficial to change the balloon size to correspond with the lesion type.

Background Although vascular endothelial growth factor (VEGF) is elevated in patients with acute myocardial infarction (AMI), the clinical significance of its elevation remains unclear. The present study was designed to determine the relationship between VEGF and left ventricular dimension in patients with AMI. Methods and results Plasma VEGF levels were examined by enzyme-linked immunosorbent assay daily for one week and then weekly for four weeks in 38 patients with AMI (65.4 ± 1.7 years). Left ventriculography was performed at 14 days, 6 months, and 2 years after the onset of AMI. Plasma VEGF levels were significantly elevated and reached a peak on day 6. Peak plasma VEGF levels positively correlated with both end-diastolic and end-systolic volume indices at 14 days after the onset of AMI. When patients with AMI were divided into two groups according to plasma VEGF levels on admission, left ventricular volume indices were higher in the high VEGF group than in the low VEGF group at the subacute phase of AMI (14 days). These differences were no longer present in the chronic phase of AMI. Conclusion Plasma VEGF levels were increased in patients with AMI, and peak levels were associated with left ventricular volume indices in the subacute phase, suggesting an important role of endogenous VEGF in the left ventricular dimension in patients with AMI.

Background— Balloon pulmonary angioplasty (BPA) is an alternative therapy for patients with chronic thromboembolic pulmonary hypertension who are ineligible for standard therapy, pulmonary endarterectomy. Although there are several classifications of vascular lesions, these classifications are based on the features of the specimen removed during pulmonary endarterectomy. Because organized thrombi are not removed during balloon pulmonary angioplasty, we attempted to establish a new classification of vascular lesions based on pulmonary angiographic images. We evaluated the success and complication rate of BPA in accordance with the location and morphology of thromboembolic lesions. Methods and Results— We reviewed 500 consecutive procedures (1936 lesions) of BPA in 97 patients with chronic thromboembolic pulmonary hypertension and investigated the outcomes of BPA based on the lesion distribution and the angiographic characteristics of the thromboembolic lesions, as follows: type A, ring-like stenosis lesion; type B, web lesion; type C, subtotal lesion; type D, total occlusion lesion, and type E, tortuous lesion. The success rate was higher, and the complication rate was lower in ring-like stenosis and web lesions. The total occlusion lesions had the lowest success rate. Tortuous lesions were associated with a high complication rate and should be treated only by operators with extensive experience with BPA. Conclusions— We modified the previous angiographic classification and established a new classification for each vascular lesion. We clarified that the outcome and complication rate of the BPA are highly dependent on the lesion characteristics.

Balloon pulmonary angioplasty (BPA) has become an effective treatment for patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are ineligible for pulmonary endarterectomy. All the thromboembolic lesion types should be treated to obtain excellent outcomes in BPA. Although we have

Background: Plasma B-type natriuretic peptide (BNP) level is reported to be a strong marker of congestive heart failure (CHF). Heterogeneity of the BNP levels among individuals with CHF, however, can cause confusion in interpreting the results. The influence of mitral mechanical prostheses on BNP levels in patients presenting with CHF is not well known. Methods and Results: In the present study 214 consecutive patients with CHF diagnosed using the Framingham criteria were enrolled and divided into 2 groups with and without mitral mechanical prostheses (prosthesis group, n=31; native group, n=183). The plasma BNP levels were measured, and clinical examinations including echocardiography were performed at the same time to assess cardiac performance. There was no difference in the left ventricular ejection fractions between the 2 groups. Despite having a lower body mass index, larger prevalence ratio of atrial fibrillation and larger size of the left atrium, the prosthesis group had a significantly lower logBNP level than the native group (prosthesis group vs native group: 5.12±1.01 vs 6.21±0.92, P

SummaryA 60-year-old woman presented with acute pulmonary edema followed by cardiopulmonary arrest due to idiopathic ventricular fibrillation. Owing to immediate cardioversion, her electrocardiogram showed sinus rhythm and echocardiography did not show any wall motion abnormalities. The next day, echocardiographic re-examination was characterized by akinesis of both apical and mid segments of the left ventricle. One hour later, subsequently performed coronary angiography revealed non-occlusive coronary artery disease, but left ventriculography demonstrated only akinesis of mid-ventricular segment with hypercontractile other segments. Further echocardiographic investigation on the following day showed total resolution of left ventricular wall motion abnormalities. The diagnosis of mid-ventricular ballooning syndrome was made according to the findings of left ventriculography. In this case, the time course changes of both patient's pathophysiological condition and echocardiographic wall motion from the onset to recovery are rapid and uncommon.

Peripheral pulmonary artery stenosis (PPS) presenting in adulthood is an uncommon disease and may be misdiagnosed as idiopathic pulmonary artery hypertension or chronic thromboembolic pulmonary hypertension. Standard curative treatment has not fully established because of the limited experience of

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is known as a serious disease with poor prognosis. Pulmonary endarterectomy (PEA) is a treatment option, however, suitable indication is restricted within the proximal lesions and few hospitals routinely perform PEA. In the present situation, we

Pulmonary endarterectomy is a conventional definitive therapeutic strategy for the treatment of chronic thromboembolic pulmonary hypertension.1 Several reports recently demonstrated that balloon pulmonary angioplasty (BPA) improves clinical status and hemodynamics in patients with chronic thromboembolic pulmonary hypertension.2 Because the success of reperfusion of occluded pulmonary vessels is considered to depend on the patency of the distal vessels via the systemic collateral circulation, the assessment of regional pulmonary perfusion is important for the selection of the candidates when performing either BPA or pulmonary endarterectomy. Lung perfusion scintigraphy cannot provide information regarding lung perfusion via the systemic collateral circulation. Lung perfused blood volume computed tomography (PBV-CT) is useful for the assessment of lung perfusion in occluded areas during the pulmonary arterial phase and the systemic arterial phase.3 A 73-year-old man was referred to …

Soluble glycoprotein 130 (sgp130), a circulating form of receptor subunit for the interleukin (IL) -6 cytokine family, modulates the biological actions of its ligands as an inhibitory regulator. The role of sgpl30 in cardiovascular diseases such as acute coronary syndrome remains unknown.Plasma levels of sgp130 were examined by enzyme-linked immunosorbent assay in 33 patients with acute myocardial infarction (AMI; mean age 67 +/- 2 years, 21 males and 12 females), who were admitted to our hospital within 24 hr of onset of AMI and survived for 4 weeks.Plasma sgp130 levels were significantly higher at admission (260.5 +/- 7.3 ng/ml), and were significantly lower from day 2 to day 5 (202.4 +/- 5.1 ng/ml at day 3) as compared with normal control subjects (n = 38, 227.1 +/- 5.6 ng/ml). The lowest sgp130 levels inversely correlated with white blood cell count at admission (r = -0.42, p0.05) and with peak C-reactive protein levels (r = -0.43, p0.05). Additional in vitro study revealed that incubation of AMI plasma with exogenous IL-6 plus soluble IL-6 receptor resulted in a decrease in plasma sgp130 levels, suggesting the possible reason for reduced plasma sgp130 levels in AMI.The present study indicates that plasma sgp130 levels were modulated during the time course of AMI and inversely associated with inflammation in AMI.

A 77-year-old man with atrial tumors was admitted to our hospital. He had no history of dyspnea, palpitation, or chest pain. He had a history of hypertension and atrial fibrillation without valve disease. Transthoracic and transesophageal echocardiography showed floating ball tumors with stalks from the right atrium (28 X 31 mm), the interatrial septum (16 X 11 mm), and the left atrial appendage (14 X 8 mm). The tumors were surgically removed and histologic examination of these tumors confirmed organization thrombus. This case of multiple atrial ball thrombi illustrates the problems with differentiation of myxoma and thrombus.