RNF213 p.Arg4810Lys is linked to various vascular diseases, including pulmonary arterial hypertension (PAH); however, its pathogenesis remains unclear. Here, we report the unique features of two cases of severe PAH with this variant: one is the first reported case with stenosis of the thoracic and abdominal aorta, femoral arteries, and subclavian veins. Coexistence of severe and continuous eosinophilic inflammation, which has been suspected to be implicated in the pathogenesis of PAH in previous fundamental studies, was also present in both cases. Further studies are needed to clarify the pathogenetic mechanisms in vascular lesions with this variant.

UNASSIGNED: Treatment strategy for vascular injury during balloon pulmonary angioplasty (BPA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) was uncertain.
UNASSIGNED: This study aimed to identify an optimal therapeutic strategy for vascular injury during BPA in patients with CTEPH.
UNASSIGNED: This study reviewed 207 patients with CTEPH and 956 BPA procedures between November 1, 2012 and November 30, 2015. Patients who were diagnosed with vascular injury during BPA, which was defined as angiographic signs or sudden respiratory and hemodynamic defects were included in this study. The study investigated the safety and efficacy of the hierarchically systematic treatment strategy including gelatin sponge embolization (GSE).
UNASSIGNED: More than one-half of the 79 patients and 133 procedures with vascular injury were improved by general treatment with reversal of heparin and high-flow oxygen administration. The investigators performed conventional treatment of proximal vessel occlusion using a guiding or balloon catheter in 47 procedures (35%) in which the culprit vessels could be detected under patients\' stable conditions. In 32 procedures (24%) without detected culprit lesions or improvement by conventional treatment, GSE could significantly improve patient condition. The treatment strategy obtained successful bailout in 98% of procedures with vascular injury. No patients who underwent GSE died within 30 days after the treatment. There was no significant difference in cumulative mortality rate (median follow-up: 6.6 years) between groups with or without GSE (15.6% vs 8.2%; adjusted HR: 1.47; 95% CI: 0.25-8.69; P = 0.67).
UNASSIGNED: Treatment strategy including GSE would be promising for vascular injury during BPA in patients with CTEPH.

While in labor, a 37-year-old woman developed acute dyspnea, hypoxemia, and tachycardia. Transthoracic echocardiography demonstrated severe right ventricular dilation and dysfunction, raising the suspicion of acute pulmonary embolism. The patient indeed had bilateral pulmonary embolism, necessitating percutaneous thrombectomy. Her course was complicated by another saddle pulmonary embolus, heparin-induced thrombocytopenia, and COVID-19 infection. This clinical case illustrates the importance of prompt diagnosis of acute pulmonary embolism in a peripartum female patient, the multidisciplinary approach of management, and how to approach clinical complications such as heparin-induced thrombocytopenia. Furthermore, long-term management in acute pulmonary embolism is presented.

UNASSIGNED: Although heart failure with preserved ejection fraction (HFpEF) is a serious disease, only limited options are available for its treatment. Recent studies have analyzed the effects of phosphodiesterase (PDE) inhibitors, especially PDE5 and PDE3 inhibitors, in patients with HFpEF, with mixed outcomes.
UNASSIGNED: We searched PUBMED and EMBASE databases up to August 2021. Randomized controlled trials (RCTs) and clinical trials that tested the effects of PDE inhibitors on patients with HFpEF were included as eligible studies. Indicators of left ventricular (LV) function, pulmonary arterial pressure (PAP), right ventricular (RV) function, exercise capacity, and quality of life (QOL) were used to evaluate the efficacy of PDE inhibitors in HFpEF.
UNASSIGNED: Six RCTs that reported in 7 studies were included to evaluate the efficiency of PDE inhibitors on HFpEF patients. In the pooled analysis, PDE inhibitors showed insignificant changes in the ratio of early diastolic mitral inflow to annular velocities, left atrial volume index, pulmonary artery systolic pressure (PASP), pulmonary vascular resistance (PVR), peak oxygen uptake, 6-minute walking test distance, as well as Kansas City Cardiomyopathy Questionnaire score. However, substantial improvement was observed in the tricuspid annular plane systolic excursion (TAPSE). Additionally, the regression analysis showed that PDE inhibitor administration time is a critical factor for the decrease in PASP.
UNASSIGNED: PDE inhibitors did not effectively improve LV function, PAP, exercise capacity, and QOL in patients with HFpEF. However, they improved RV function with significant difference, suggesting that PDE inhibitors might be a promising option for HFpEF patients with RV dysfunction.

UNASSIGNED: The ratio of pulmonary artery (PA) and ascending aorta (AA) diameters has recently been shown to be a useful indicator for disease severity and predictor of outcome in patients with pulmonary hypertension and heart failure. This study aimed at evaluating the applicability of this ratio for perioperative risk assessment of patients with chronic thromboembolic pulmonary hypertension undergoing pulmonary endarterectomy.
UNASSIGNED: In this retrospective cohort study on 149 patients undergoing pulmonary endarterectomy between 2013 and 2020, the preoperative PA to AA ratio was analyzed on axial computed tomography. Variables of pulmonary hemodynamic status were assessed during preoperative right heart catheterization and postoperative Swan-Ganz catheter measurements. Perioperative survival was analyzed by Kaplan-Meier method and log-rank tests.
UNASSIGNED: Preoperative computed tomography measurements showed a median AA diameter of 31 mm (range, 19-47 mm), and a median PA diameter of 36 mm (range, 25-55 mm). The calculated median PA to AA ratio was 1.13 (range, 0.79-1.80). PA to AA ratio correlated positively with PA pressure (systolic, r = 0.352 [P < .001]; diastolic, r = 0.406 [P < .001]; mean, r = 0.318 [P < .001]) and inversely with age (r = -0.484 [P < .001]). Univariable Cox regression analysis identified PA diameter (P = .008) as a preoperative parameter predictive of survival. There was a significant difference (log-rank P = .037) in 30-day survival probability for patients with lower PA to AA ratios (<1.136; survival probability, 97.4%) compared with patients with higher ratios (>1.136; survival probability, 88.9%).
UNASSIGNED: PA to AA ratio shows a correlation with other variables associated with pulmonary hypertension. In addition, patients with higher PA to AA ratios have lower survival probabilities after PEA. Further analysis of PA to AA ratio on the selection of chronic thromboembolic pulmonary hypertension for different treatment modalities-pulmonary endarterectomy, medical therapy, and or balloon pulmonary angioplasty-is warranted.

UNASSIGNED: Soluble CD40 ligand (sCD40L) is associated with some pathobiological states. However, whether sCD40L in patients with chronic thromboembolic pulmonary hypertension (CTEPH) who underwent pulmonary endarterectomy (PEA) is associated with perioperative pulmonary hemodynamics and surgical outcomes has not been elucidated. Here we aimed to investigate whether sCD40L is a useful serologic biomarker of poor surgical outcome of PEA in patients with CTEPH.
UNASSIGNED: Ninety patients with CTEPH who underwent PEA were enrolled. Independent preoperative parameters were examined, including sCD40L related to lower cardiac index (CI), higher pulmonary vascular resistance (PVR), and poor surgical outcomes after PEA, according to the multivariate logistic regression analysis. In addition, the area under the curve (AUC) value of sCD40L to predict poor surgical outcomes was compared with the AUCs of D-dimer and C-reactive protein (CRP). The generalizability of this study model was tested by a 5-fold cross-validation analysis.
UNASSIGNED: Multivariate logistic regression analysis showed that high sCD40L level was related to postoperative lower CI, higher PVR, and poor surgical outcomes independent of other preoperative parameters. The AUC value of sCD40L to predict poor surgical outcomes was higher than those of D-dimer and CRP. A sCD40L cutoff value of 1.45 ng/mL predicted poor surgical outcomes with 79.3% sensitivity and 67.3% specificity. The 5-fold cross-validation analysis showed the effectiveness of our model\'s performance.
UNASSIGNED: Preoperative sCD40L level could be a promising serologic biomarker associated with poor surgical outcomes in CTEPH. In addition to known preoperative parameters, the biomarker might have the potential to identify patients at high risk of PEA, thereby reducing the mortality rates.

UNASSIGNED: The Advanced ventricular assist device (Advanced VAD) is designed as a universal pump intended to prevent backflow in the event of pump stoppage, to maintain physiological pulse pressure, and to be used as both a left and right VAD. The purpose of this study was to evaluate the performance of the Advanced VAD as both a left and right VAD in an acute in vivo study in calves.
UNASSIGNED: The Advanced VAD was implanted through a median sternotomy in 5 healthy calves (weight, 71.4-91.2 kg) as a left VAD (n = 3) or a right VAD (n = 2). After implantation, hemodynamic parameters, including general performance and pump stoppage, were evaluated.
UNASSIGNED: The Advanced VAD was successfully implanted as a left and right VAD without cardiopulmonary bypass. The speed range of the Advanced VAD was 2500 to 3500 rpm as a left VAD and 2000 to 2500 rpm as a right VAD. Up to 4.3 L/min was achieved for both left and right VAD configurations. To demonstrate the automatic shut-off feature, the pump was stopped without clamping the outflow graft. The outflow graft was then clamped, which produced no significant changes in the arterial pressure waveform. The pulse pressures under the left VAD configuration were 38 mm Hg, 17 mm Hg, 14 mm Hg, and 16 mm Hg at baseline, 2500 rpm, 3000 rpm, and 3500 rpm, respectively.
UNASSIGNED: This acute in vivo study demonstrated the pump performance, anatomical fitting as both left VAD and right VAD, and regurgitant flow shut-off feature of the Advanced VAD.

UNASSIGNED: Arginine vasopressin (AVP) is used to treat hypotension. Because AVP increases blood pressure by increasing systemic vascular resistance, it may have an adverse effect on tissue oxygenation following the Norwood procedure.
UNASSIGNED: Retrospective analysis of continuously captured hemodynamic data of neonates receiving AVP following the Norwood procedure.
UNASSIGNED: We studied 64 neonates exposed to AVP within 7 days after the Norwood procedure. For the entire group, AVP significantly increased mean blood pressure (2.5 ± 6.3) and cerebral and renal oxygen extraction ratios (4.1% ± 9.6% and 2.0% ± 4.7%, respectively; P < .001 for all values). In the right ventricle to pulmonary artery shunt cohort, AVP significantly increased blood pressure, arterial oxygen saturation (1.4% ± 3.8%; P = .011), pulmonary to systemic perfusion ratio (0.2 ± 0.4; P = .017), and cerebral and renal oxygen extraction ratios (4.6% ± 8.7%; P = .010% and 4.7% ± 9.4%; P = .014, respectively). The Blalock-Taussig shunt cohort experienced a less significant vasopressor response and no change in arterial oxygen saturation, pulmonary to systemic perfusion ratio, or cerebral and renal oxygen extraction ratios.
UNASSIGNED: The right ventricle to pulmonary artery shunt cohort experienced a significant vasopressor response to AVP that was associated with a significant increase in pulmonary perfusion and decrease in cerebral and renal perfusion, whereas the Blalock-Taussig shunt cohort experienced a less significant vasopressor response and no change in pulmonary or systemic perfusion. The influence of AVP on tissue oxygenation following the Norwood procedure may have clinical implications that require further study.

UNASSIGNED: In single-ventricle physiology, focus on pulmonary vascular resistance neglects the resistance in the conduit supplying the pulmonary inflow.
UNASSIGNED: Conduit length and diameter, which can approximate conduit resistance, are available in the public dataset of Single Ventricle Reconstruction (SVR) trial. Conduit resistance was then calculated for SVR trial participants and the relationship with clinically important variables (death or transplant at 1 year, pulmonary artery size at second-stage palliation, pulmonary-to-systemic blood flow ratio, and supplemental oxygen requirement) was explored. To validate this calculated resistance, calculated resistance was compared with catheterization measurements at a single institution (not included in the SVR trial).
UNASSIGNED: In the institutional dataset, calculated and measured resistances had an intraclass correlation of 0.78 for modified Blalock-Taussig shunts (MBTS). Within the SVR trial, transplant-free survivors had a lower MBTS resistance (median, 8.3 Woods Units [WU]. interquartile range [IQR], 6.5-11.1 WU) than patients who died or required transplantation (median, 13.0 WU; IQR, 9.4-16.6 WU, P = .0001). When we controlled for left pulmonary artery diameter after the Norwood procedure in the SVR trial, for each unit increase in MBTS resistance, the left pulmonary artery diameter at stage II decreased (-0.006 ± 0.002 cm, P = .005). When we controlled for pulmonary vascular resistance, greater MBTS resistance was associated with a decrease in log pulmonary-to-systemic blood flow ratio (-0.04 ± 0.015, P = .0048) in the SVR trial. Patients in the SVR trial requiring supplemental oxygen on admission for stage II palliation had greater MBTS resistance (median. 11.1 WU; IQR, 6.6-16.6 WU) than patients not requiring oxygen (median 8.3, WU; IQR, 6.5-11.1 WU, P = .015).
UNASSIGNED: Conduit resistance is associated with important clinical outcomes after Norwood; however, further studies are required to guide conduit resistance optimization.

Restrictive cardiomyopathy (RCM) has a poor prognosis and limited treatment options apart from heart transplantation (HTx). We report on the first-in-human interventional atrial flow regulator (AFR) implantations in 3 children with RCM, leading to marked clinical and hemodynamic improvement. We propose the AFR as bridge to HTx or destination therapy in RCM. (Level of Difficulty: Advanced.).