Adult-onset idiopathic peripheral pulmonary artery stenosis.

BACKGROUND: Peripheral pulmonary artery stenosis (PPS) refers to stenosis of the pulmonary artery from the trunk to the peripheral arteries. Although paediatric PPS is well described, the clinical characteristics of adult-onset idiopathic PPS have not been established. Our objectives in this study were to characterise the disease profile of adult-onset PPS. METHODS: We collected data in Japanese centres. This cohort included patients who underwent pulmonary angiography (PAG) and excluded patients with chronic thromboembolic pulmonary hypertension or Takayasu arteritis. Patient backgrounds, right heart catheterisation (RHC) findings, imaging findings and treatment profiles were collected. RESULTS: 44 patients (median (interquartile range) age 39 (29-57) years; 29 females (65.9%)) with PPS were enrolled from 20 centres. In PAG, stenosis of segmental and peripheral pulmonary arteries was observed in 41 (93.2%) and 36 patients (81.8%), respectively. 35 patients (79.5%) received medications approved for pulmonary arterial hypertension (PAH) and 22 patients (50.0%) received combination therapy. 25 patients (56.8%) underwent transcatheter pulmonary angioplasty. RHC data showed improvements in both mean pulmonary arterial pressure (44 versus 40 mmHg; p<0.001) and pulmonary vascular resistance (760 versus 514 dyn·s·cm-5; p<0.001) from baseline to final follow-up. The 3-, 5- and 10-year survival rates of patients with PPS were 97.5% (95% CI 83.5-99.6%), 89.0% (95% CI 68.9-96.4%) and 67.0% (95% CI 41.4-83.3%), respectively. CONCLUSIONS: In this study, patients with adult-onset idiopathic PPS presented with segmental and peripheral pulmonary artery stenosis. Although patients had severe pulmonary hypertension at baseline, they showed a favourable treatment response to PAH drugs combined with transcatheter pulmonary angioplasty.

Pyothorax and Constrictive Pericarditis after Chemoradiotherapy for Esophageal Cancer: A Case Report.

A 75-year-old man underwent chemoradiotherapy for advanced esophageal cancer. After nine years, he was hospitalized for left pyothorax. Consequently, the patient underwent drainage and window opening surgery. He experienced cardiopulmonary arrest but was resuscitated. Based on cardiac catheterization data, the patient was diagnosed with constrictive pericarditis. Unfortunately, extracorporeal circulation did not improve his condition, and he ultimately died. An autopsy revealed adhesion between the pericardium and pleura, especially the pericardium in contact with the left thoracic cavity, which was markedly thickened. This suggests that constrictive pericarditis, a latent complication of chemoradiotherapy, is aggravated by pyothorax.

Drug-coated balloons for the treatment of stent edge restenosis.

BACKGROUND: Drug-coated balloon (DCB) is a device for treating patients with in-stent restenosis; however, there are scant data on its efficacy for stent edge restenosis (SER). This study aimed to investigate the clinical outcomes of DCB use for treating SER compared with new-generation drug-eluting stent (DES) implantation. METHOD: From December 2013 to January 2019, patients who underwent DES implantation or DCB for SER were enrolled. Clinical outcomes were analyzed, and propensity score with matching was conducted. The primary outcome was target-vessel revascularization (TVR). The secondary outcomes were the incidence of all-cause mortality, major adverse cardiovascular events (MACE), and target lesion revascularization (TLR). RESULT: A total of 291 patients with SER were included: 160 were treated with DCB, and 131 with new-generation DES. DCB treatment for SER treatment was associated with a lower risk of TVR than DES [hazard ratio, 0.549; 95% confidence interval (CI), 0.339-0.891] at a median follow-up of 1080 days (interquartile range; 729-1080 days). Propensity score matching (PSM) was performed to adjust for baseline clinical and lesion characteristics. After PSM, no significant difference in the risk of TVR was observed (hazard ratio, 0.965; 95% CI, 0.523-1.781). Similarly, the risk for all-cause death (hazard ratio, 0.507; 95% CI, 0.093-2.770), MACE (hazard ratio: 0.812; 95% CI, 0.451-1.462), and TLR (hazard ratio: 0.962; 95% CI, 0.505-1.833) were comparable between the two groups. CONCLUSION: DCB treatment efficacy for SER was similar to that of new-generation DES after PSM. DCB is a significant alternative to obtain comparable results with new-generation DES for the treatment of SER.

Acute pulmonary thromboembolism after messenger RNA vaccination against coronavirus disease 2019: A case report.

Vaccine-induced immune thrombotic thrombocytopenia (VITT) is defined as thrombosis after inoculation of adenovirus vector vaccines against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). VITT rarely occurs with messenger RNA vaccines, and the use of heparin for VITT is also controversial. A 74-year-old female patient with no risk factors for thrombosis was brought to our hospital after loss of consciousness. Nine days before admission, she had received the third vaccine against SARS-CoV-2 (mRNA1273, Moderna). Immediately after transport, cardiopulmonary arrest occurred, prompting extracorporeal membrane oxygenation (ECMO). Pulmonary angiography showed translucent images of both pulmonary arteries, resulting in the diagnosis of acute pulmonary thromboembolism. Unfractionated heparin was administered, but D-dimer subsequently became negative. Pulmonary thrombosis remained in large volume, indicating that heparin was ineffective. Treatment was shifted to anticoagulant therapy using argatroban, which increased D-dimer level and improved respiratory status. The patient was successfully weaned from ECMO and ventilator. Anti-platelet factor 4 antibody examined after treatment initiation showed negative results; however, VITT was considered as an underlying condition because of the time of onset after vaccination, the ineffectiveness of heparin, and the absence of other causes of thrombosis. In case heparin is not effective, argatroban can be an alternative therapy against thrombosis. Learning objective: During the coronavirus disease 2019 pandemic, treatment with vaccine against severe acute respiratory syndrome coronavirus 2 has been widely performed. Vaccine-induced immune thrombotic thrombocytopenia is the most common thrombosis after adenovirus vector vaccines. However, thrombosis can also occur after messenger RNA vaccination. Though commonly used for thrombosis, heparin may be ineffective. Non-heparin anticoagulants should be considered.

Oozing-type rupture caused by right ventricular intramural hematoma after right ventricular infarction.

An 81-year-old man was admitted to the hospital because of decreased level of consciousness. He had bradycardia (27 beats/min). Electrocardiography showed ST-segment elevation in leads II, III, and aVF and ST-segment depression in leads aVL, V1. Transthoracic echocardiography (TTE) visualized reduced motion of the left ventricular (LV) inferior wall and right ventricular (RV) free wall. Coronary angiography revealed occlusion of the right coronary artery. A primary percutaneous coronary intervention was successfully performed with temporary pacemaker backup. On the third day, the sinus rhythm recovered, and the temporary pacemaker was removed. On the fifth day, a sudden cardiac arrest occurred. Extracorporeal cardiopulmonary resuscitation was performed. TTE showed a high-echoic effusion around the right ventricle, indicating a hematoma. The drainage was ineffective. He died on the eighth day. An autopsy showed the infarcted lesion and an intramural hematoma in the RV. However, no definite perforation of the myocardium was detected. The hematoma extended to the epicardium surface, indicative of oozing-type RV rupture induced by RV infarction. The oozing-type rupture induced by RV infarction might develop asymptomatically without influence on the vital signs of the patient. Frequent echocardiographic evaluation is essential in cases of RV infarction taking care of silent oozing-type rupture. Learning objective: Inferior left ventricular infarction sometimes complicates right ventricular (RV) infarction. The typical manifestations of RV infarction include low blood pressure, low cardiac output, and elevated right atrium pressure. Although the frequency is low, fatal complications of oozing-type RV rupture might progress asymptomatically. Frequent echocardiographic screening is necessary to detect them.

Long-term outcomes of intermediate coronary stenosis in patients undergoing hemodialysis after deferred revascularization based on fractional flow reserve.

OBJECTIVES: This study aimed to assess the long-term outcomes of patients undergoing hemodialysis (HD) after deferred revascularization based on fractional flow reserve (FFR). BACKGROUND: FFR is a practical technique for assessing the functional severity of intermediate coronary stenosis. Prior research has revealed a satisfactory outcome in patients after the deferral of percutaneous coronary intervention for coronary lesions based on FFR measurement. However, little research has been conducted focusing on patients undergoing HD. METHODS: The retrospective study comprised 225 consecutive patients with FFR assessment and deferred revascularization between January 2016 and December 2019. Based on a deferral cutoff FFR value of >0.80, we assessed the differences in all-cause death, major adverse cardiac events (MACEs), and target vessel failure (TVF) between the HD (n = 69) and non-HD groups (n = 156) during a mean ± standard deviation routine follow-up of 32.2 ± 13.4 months. RESULTS: Although the HD group had significantly higher rates of diabetes mellitus than the non-HD group (53.6% vs. 37.2%, p = 0.021), there were no significant differences in sex, left ventricular ejection fraction, or other risk factors between the groups, nor with respect to stenosis diameter or mean FFR. The HD group had a significantly higher incidence of TVF than the non-HD group (34.8% vs. 14.1%, p < 0.001), as well as a significantly higher risk of all-cause death and MACEs. CONCLUSIONS: The study revealed that deferred revascularization in coronary lesions with an FFR value of >0.80 in patients undergoing HD was associated with poor outcomes. Therefore, it is important to carefully monitor patients with intermediate coronary stenosis undergoing HD.

Peripheral Venous Pressure-Assisted Exercise Stress Echocardiography in the Evaluation of Pulmonary Hypertension During Exercise in Patients With Suspected Heart Failure With Preserved Ejection Fraction.

BACKGROUND: Identification of elevated pulmonary artery (PA) pressures during exercise may provide diagnostic, prognostic, and therapeutic implications in heart failure with preserved ejection fraction. Although widely performed, exercise stress echocardiography may underestimate true PA pressures due to the difficulty in estimating right atrial pressure (RAP) during exercise. We hypothesized that peripheral venous pressure (PVP) could allow for reliable estimation of RAP, and thus PA pressures during exercise stress echocardiography. METHODS: In protocol 1, we investigated the accuracy of PVP compared with simultaneously measured RAP at rest and during exercise right heart catheterization in 19 subjects. In protocol 2, we examined whether the addition of PVP to Doppler exercise echocardiography (tricuspid regurgitant velocity) would increase the ability to identify exercise-induced pulmonary hypertension compared with inferior vena cava-based RAP estimation in 60 patients with dyspnea. RESULTS: In protocol 1, PVP was strongly correlated with simultaneously measured RAP at rest and during exercise (r=0.77 and 0.90), with little overestimation of invasively measured RAP (bias 3.4 mm Hg at rest and 1.7 mm Hg during exercise). In protocol 2, PVP increased dramatically during exercise echocardiography (14±5 mm Hg) while an increase in inferior vena cava-based RAP was modest (6±4 mm Hg). Exercise PA pressures calculated from PVP and tricuspid regurgitant velocity were significantly higher than those estimated from inferior vena cava and the use of PVP increased the proportion of patients with exercise-induced pulmonary hypertension from 40% to 68%. CONCLUSIONS: PVP may prevent underestimation of PA pressures during exercise echocardiography and could be a preferred approach to identify exercise-induced pulmonary hypertension in patients with suspected heart failure with preserved ejection fraction.

Selexipag for the treatment of chronic thromboembolic pulmonary hypertension.

BACKGROUND: Treatment 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. METHODS: In 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. RESULTS: The 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 (p<0.001) and Borg dyspnoea scale score (p=0.036) were also significantly improved over placebo. 6MWD and WHO functional class were not significantly improved. The common adverse events in the selexipag group corresponded to those generally observed following administration of a prostacyclin analogue. CONCLUSION: Selexipag 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.

Acute myocarditis associated with COVID-19 vaccination: A case report.

Recently, new vaccine platforms-including mRNA vaccines for coronavirus disease 2019 (COVID-19) have been given emergency use authorization in Japan. Here, we present a rare case of myocarditis following a COVID-19 vaccine. In this case, myocarditis was confirmed by cardiac magnetic resonance imaging, endomyocardial biopsy, and troponin levels. The degree of myocardial inflammation in the endomyocardial biopsy samples was mild and the patient's clinical course was not severe. Although the pathology of myocarditis in this case was mild, further investigation would be needed. .

Utility of E/e' Ratio During Low-Level Exercise to Diagnose Heart Failure With Preserved Ejection Fraction.

BACKGROUND: E/e' ratio during exercise is the key parameter in identifying elevated pulmonary capillary wedge pressure (PCWP), and thus heart failure with preserved ejection fraction (HFpEF). However, its diagnostic value is limited when mitral inflow or tissue velocities are fused during elevated heart rate. OBJECTIVES: The authors hypothesized that E/e' ratio during low-level (20 W) exercise (E/e'20W) can help diagnose HFpEF. METHODS: Ergometric exercise stress echocardiography was performed in 215 dyspneic patients with an EF ≥50%. The authors determined the feasibility of E/e' ratio at each stage (frequency of patients who had measurable E/e' without E-A fusion among 215 participants) and examined whether E/e'20W could predict normal E/e' ratio during peak exercise (E/e'peak ≤15). The authors also evaluated whether E/e'20W could predict normal PCWP during exercise (PCWP <25 mm Hg) in a subset of participants (n = 45) who underwent exercise right heart catheterization. RESULTS: The feasibility of the E/e' ratio decreased from 100% at rest to 96.3% during 20-W exercise and 74.9% during peak exercise caused by E-A fusion. In patients with E/e'peak >15, there was an increase in E/e' ratio from rest to 20-W exercise (11.2 ± 2.1 to 16.3 ± 3.5; P < 0.0001), but it did not change significantly from 20-W exercise to peak exercise (P = 0.12). E/e'20W predicted E/e'peak ≤15 (AUC: 0.91; P < 0.0001) with the cutoff value of ≤12.4 showing high specificity (94%) and positive predictive value (98%). During 20-W exercise, 93% of the HFpEF patients developed PCWP ≥25 mm Hg. E/e'20W predicted normal PCWP during exercise (AUC: 0.77; P = 0.01) with the cutoff value of ≤12.4 showing high specificity (83%). CONCLUSIONS: E/e' ratio during low-level exercise is highly feasible and predicts normal E/e' ratio or PCWP during peak exercise with high specificity. These data suggest that E/e'20W could be used as an alternative to the peak exercise value to rule out HFpEF in patients with dyspnea.

A case of a middle-aged patient with a ventricular septal defect complicated by severe pulmonary hypertension-stepwise surgical repair with pulmonary vasodilators.

We report a case of ventricular septal defect (VSD) in which we attempted to treat pulmonary arterial hypertension (PAH) with the goal of VSD closure in an adult with suspected Eisenmenger syndrome in childhood. Four years previously (age 41 years), she was referred to our department due to repeated hemoptysis requiring further treatment of PAH. We started combination therapy with several pulmonary vasodilators. Two years later, her pulmonary vascular resistance (PVR) was improved but still not at the level where VSD closure was possible. To control the increased PA flow resulting from intensive PAH treatment and to reduce the risk of hemoptysis, we performed pulmonary artery banding (PAB). As the risk of hemoptysis decreased, a prostacyclin analog was introduced, and the dose was increased. More than 1 year after PAB, active vasoactivity testing became positive, suggesting that the pulmonary vascular lesion was now "reversible". We performed VSD closure and atrial septal defect creation even though her PVR was still high. After the operation, her exercise capacity was remarkably improved. We suggest that stepwise surgical repair with pulmonary vasodilators is an important treatment option for select patients with VSD with severe PAH. .

Adaptive Servo-ventilation Therapy Results in the Prevention of Arrhythmias in Patients with Heart Failure Due to Ischemic Heart Disease.

Objective Whether or not adaptive servo-ventilation (ASV) is effective in preventing arrhythmias in patients with heart failure (HF) due to ischemic heart disease (IHD) is unclear. This study estimated the effects of ASV therapy on arrhythmias in patients with HF due to IHD. Methods One hundred and forty-one consecutive hospitalized patients with HF due to IHD (mean age: 74.9±11.9 years old) were retrospectively assessed in this study. Of the 141 patients, 75 were treated with ASV (ASV group), and 66 were treated without ASV (Non-ASV group). We estimated the incidence of arrhythmias, including paroxysmal atrial fibrillation (PAF) and ventricular tachycardia (VT), during one-year follow-up in both groups using multivariable logistic regression models. Results Men accounted for 55.3% of the study population. There were no significant differences in the baseline clinical characteristic data between the ASV and Non-ASV groups with respect to age, sex, heart rate, risk factors, oral medication, or laboratory data, including the estimated glomerular filtration rate (eGFR), brain natriuretic peptide, and left ventricular ejection fraction. ASV therapy was associated with a reduced incidence of arrhythmia after adjusting for demographic and cardiovascular disease risk factors (odds ratio, 0.27; 95% confidence interval, 0.11 to 0.63; p<0.01; compared to the Non-ASV group). In addition, at the 1-year follow-up, an improvement (increase) in the eGFR was found in the ASV group but not in the Non-ASV group. Conclusion ASV therapy was able to prevent arrhythmias, including PAF and VT, with short-term improvements in the renal function in patients with HF due to IHD.