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Objectives: Pulmonary arterial hypertension associated with systemic sclerosis (PAH-SSc) sometimes accompanies pulmonary veno-occlusive disease (PVOD). We aimed to reveal the relationship between clinical signs of PVOD and severity of pulmonary vasculopathy in SSc., Methods: This study included 52 consecutive SSc patients who had pulmonary haemodynamic abnormalities [mean pulmonary arterial pressure (mPAP) >20 mmHg, pulmonary vascular resistance >2 WU or pulmonary artery wedge pressure (PAWP) >15 mmHg]. A chest CT scan was evaluated in all patients. Patients were divided into two groups, the 0-1 group and the 2-3 group, according to the number of chest CT signs for PVOD, including mediastinal lymph node enlargement, thickened interlobular septal wall and ground glass opacity. Pulmonary haemodynamics, echocardiography and MRI-based cardiac function, pulmonary function and serum biomarkers were compared between the two groups., Results: Mediastinal lymph node enlargement, thickened interlobular septal wall and ground glass opacity were observed in 11 (21%), 32 (62%) and 11 (21%) patients, respectively. The 2-3 group (n = 15) had higher mPAP (P = 0.02) but lower diffusing capacity of carbon monoxide (DLCO)/alveolar volume (P = 0.02) compared with the 0-1 group (n = 37). Other parameters, including PAWP, cardiac output, left ventricular ejection fraction, left atrial diameter, forced vital capacity, brain natriuretic peptide and Krebs von den Lunge-6 were not different between the two groups., Conclusions: The CT signs for PVOD had a positive correlation with mPAP but a negative correlation with DLCO in SSc patients, indicating that PAH-SSc may reflect a spectrum of pulmonary vascular disease that ranges from the pulmonary artery to the vein., (© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Introduction: Fibrosing mediastinitis (FM) is a rare disease characterized by excessive proliferation of fibrous tissue in the mediastinum and can cause bronchial stenosis, superior vena cava obstruction, pulmonary artery and vein stenosis, etc. CASE PRESENTATION: An aging patient with intermittent chest tightness and shortness of breath was diagnosed with FM associated pulmonary hypertension (FM-PH) by echocardiography and enhanced CT of the chest, and CT pulmonary artery (PA)/ pulmonary vein (PV) imaging revealed PA and PV stenosis. Selective angiography revealed complete occlusion of the right upper PV, and we performed endovascular intervention of the total occluded PV. After failure of the antegrade approach, the angiogram revealed well-developed collaterals of the occluded RSPV-V2b, so we chose to proceed via the retrograde approach. We successfully opened the occluded right upper PV and implanted a stent., Conclusions: This report may provide new management ideas for the interventional treatment of PV occlusion., (© 2024. The Author(s).)

Competing Interests: Conflict of interest: None declared.

Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis are rare types of histopathological substrates within the spectrum of pulmonary arterial hypertension (PAH) with a very poor prognosis. They are characterized by a widespread fibroproliferative process of the small caliber veins and/or capillaries with sparing of the larger veins, resulting in a pre-capillary pulmonary hypertension phenotype. Clinical presentation is unspecific and similar to other PAH etiologies. Definitive diagnosis is obtained through histological analysis, although lung biopsy is not advised due to a higher risk of complications. However, some additional findings may allow a presumptive clinical diagnosis of PVOD, particularly a history of smoking, chemotherapy drug use, exposure to organic solvents (particularly trichloroethylene), low diffusing capacity for carbon monoxide (DLCO), exercise induced desaturation, and evidence of venous congestion without left heart disease on imaging, manifested by a classical triad of ground glass opacities, septal lines, and lymphadenopathies. Lung transplant is the only effective treatment, and patients should be referred at the time of diagnosis due to the rapid progression of the disease and associated poor prognosis. We present a case of a 58-year-old man with PAH with features of venous/capillary involvement in which clinical suspicion, prompt diagnosis, and early referral for lung transplantation were determinant factors for the successful outcome.

Background: Supracardiac total anomalous pulmonary venous connection is the most common subtype of total anomalous pulmonary venous connection. We aimed to describe the morphological spectrum of supracardiac total anomalous pulmonary venous connection and to identify risk factors for death and postoperative pulmonary venous obstruction., Methods: From February 2009 to June 2019, 241 patients diagnosed with supracardiac-Ia (left-sided vertical vein, n = 185) or supracardiac-Ib (right-sided connection directly to superior vena cava, n = 56) total anomalous pulmonary venous connection underwent initial surgical repair at our institute. Cases with functionally univentricular circulations or atrial isomerism were excluded. Patients' postoperative survival was described by Kaplan-Meier curves. Cox proportional hazards models and competing risk regression models were used to identify clinical risk factors for death and postoperative pulmonary venous obstruction., Results: There were 8 early deaths and 4 late deaths. The overall survivals at 30 days, 1 year, and 10 years were 97.1%, 94.8%, and 94.8%, respectively, in the supracardiac-Ia group (2.7%, 5/185) (hazard ratio, 4.8; P = .003). Five patients required reoperation for pulmonary venous obstruction, including 2 patients who required reintervention for superior vena cava syndromes (all in the supracardiac-Ib group). One patient required superior vena cava balloon dilation for superior vena cava syndromes. Multivariable analysis showed that the supracardiac-Ib group (12.5%, 7/56) had a significantly higher mortality rate than the supracardiac-Ia group (adjusted hazard ratio, 8.5, P = .008). Surgical weight less than 2.5 kg (adjusted hazard ratio, 10.8, P = .023), longer duration of cardiopulmonary bypass (adjusted hazard ratio, 1.15 per 10 minutes, P = .012), and supracardiac-Ib subtype (adjusted hazard ratio, 4.7, P = .037) were independent risk factors associated with death. The supracardiac-Ib subtype (adjusted hazard ratio, 4.8, P = .003) was an incremental risk factor associated with postoperative pulmonary venous obstruction., Conclusions: Morphological features of supracardiac total anomalous pulmonary venous connection, especially the supracardiac-Ib subtype, were risk factors associated with postoperative pulmonary venous obstruction and survival. Patients with unique anatomic subtypes might require more individualized surgical planning., (Copyright © 2023. Published by Elsevier Inc.)

We herein report a case of pulmonary veno-occlusive disease (PVOD) induced by allo-hematopoietic stem cell transplantation (HSCT) in a 48-year-old man who was diagnosed with acute myeloid leukemia. Five months after transplantation, he developed dyspnea and was diagnosed with pulmonary hypertension based on right heart catheterization. Although he received treatment with pulmonary vasodilators, diuretics, and corticosteroids, his pulmonary artery pressure did not decrease, and his pulmonary edema worsened. Based on the clinical course, hypoxemia, diffusion impairment, and computed tomography findings, the patient was diagnosed with HSCT-related PVOD. Critical attention should be paid to dyspnea after HSCT for the early diagnosis of PVOD.

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease, with which some patients suffer from postoperative pulmonary venous obstruction (PPVO), requiring particular follow-up strategies and treatments. PPVO prediction has important clinical significance, while building a PPVO prediction model is challenging due to limited data and class imbalance distribution. Inspired by the anatomical evidence of PPVO, which is related to the structure of the left atrium (LA) and pulmonary vein (PV), we design an effective multi-task network for PPVO classification. The proposed method incorporates clinical priors and merits of the segmentation-based network into the classification task. The features learned from segmenting LA and PV are concatenated into the PPVO classification branch to constrain the learning of discriminative features. Anatomical-guided attention is applied in the aggregation of these features to restrict them focusing on TAPVC-related regions. To deal with the imbalance classification problem of PPVO, a novel classification loss derived by masked class activation map (MCAM) is designed to improve the classification performance. Computed tomography angiography (CTA) images of 146 patients diagnosed with supracardiac TAPVC in Shanghai Children's Medical Center and Guangdong Provincial People's Hospital were enrolled in this work. The comprehensive experiments demonstrate the effectiveness and generalization of our proposed method. The automatic PPVO prediction model shows the potential application in helping clinicians develop follow-up strategies, thereby improving the survival rate of TAPVC patients., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Background: In the stratification of potential causes of PH, current guidelines recommend performing V/Q lung scintigraphy to screen for CTEPH. The recognition of CTEPH is based on the identification of lung segments or sub-segments without perfusion but preserved ventilation. The presence of mismatched perfusion defects has also been described in a small proportion of idiopathic pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis (PVOD/PCH). Dual-energy CT lung perfusion changes have not been specifically investigated in these two entities., Purpose: To compare dual-energy CT (DECT) perfusion characteristics in PAH and PVOD/PCH, with specific interest in PE-type perfusion defects., Materials and Methods: Sixty-three patients with idiopathic or heritable PAH (group A; n = 51) and PVOD/PCH (group B; n = 12) were investigated with DECT angiography with reconstruction of morphologic and perfusion images., Results: The number of patients with abnormal perfusion did not differ between group A (35/51; 68.6%) and group B (6/12; 50%) (p = 0.31) nor did the mean number of segments with abnormal perfusion per patient (group A: 17.9 ± 4.9; group B: 18.3 ± 4.1; p = 0.91). The most frequent finding was the presence of patchy defects in group A (15/35; 42.9%) and a variable association of perfusion abnormalities in group B (4/6; 66.7%). The median percentage of segments with PE-type defects per patient was significantly higher in group B than in group A (p = 0.041). Two types of PE-type defects were depicted in 8 patients (group A: 5/51; 9.8%; group B: 3/12; 25%), superimposed on PH-related lung abnormalities (7/8) or normal lung (1/8). The iodine concentration was significantly lower in patients with abnormal perfusion (p < 0.001) but did not differ between groups., Conclusion: Perfusion abnormalities did not differ between the two groups at the exception of a higher median percentage of segments with PE-type defects in patients with PVOD/PCH., Key Points: • Patchy perfusion defect was the most frequent pattern in PAH. • A variable association of perfusion abnormalities was seen in PVOD/PCH. • Lobular and PE-type perfusion defects larger than a sub-segment were depicted in both PAH and PVOD/PCH patients., (© 2022. The Author(s), under exclusive licence to European Society of Radiology.)

Background: Previous studies have demonstrated increased early mortality and pulmonary vein reintervention for patients with total anomalous pulmonary venous connection (TAPVC) and heterotaxy syndrome (HTX+) compared with patients with TAPVC without heterotaxy syndrome (HTX-). We aimed to evaluate the longitudinal risk of pulmonary vein reintervention and mortality in HTX + patients., Methods: A retrospective review was performed to identify longitudinal interventions in patients with TAPVC seen at a single center from 1995 to 2019. The mean cumulative interventions were described for all patients using the Nelson-Aalen estimator. Survival with TAPVC was described using Kaplan-Meier estimates., Results: A total of 336 patients were identified with TAPVC, of whom 118 (35%) had heterotaxy syndrome. Functional single ventricles were identified in 106 of these 118 HTX + patients (90%) and in 14 of 218 HTX- patients (6%) (P < .001). Obstructed TAPVC (OBS+) was present in 49 of 118 HTX + patients (42%) and in 87 of 218 HTX- patients (40%) (P = .89). The median duration of follow-up was 6.5 years. Five-year survival was 69% for HTX+/OBS + patients, 72% for HTX+/OBS- patients, 86% for HTX-/OBS + patients, and 95% for HTX-/OBS- patients (P < .0001, log-rank test). The mean number of pulmonary vein interventions at the median follow-up time was greater in the HTX+/OBS + patients compared with HTX+/OBS- patients (mean, 2.0 vs 1.1; P = .030), HTX-/OBS + patients (mean, 1.3; P = .033), and HTX-/OBS- patients (mean, 1.3; P = .029)., Conclusions: Among the 4 cohorts, HTX+ was associated with a higher rate of mortality, and HTX+/OBS+ was associated with a greater number of pulmonary vein interventions. This may be due in part to the high prevalence of single ventricle physiology in the HTX + cohort., (Copyright © 2021. Published by Elsevier Inc.)

Pulmonary veno-occlusive disease (PVOD) is a rare type of pulmonary hypertension characterized by capillary damage or arterial pulmonary hypertension. Early lung transplantation is the only effective treatment for PVOD because of the lack of specificity in its clinical manifestations and its rapid progression and poor prognosis. A 28-year-old woman presented with exertional dyspnoea. A chest computed tomography scan revealed diffuse centrilobular ground glass opacities in both lungs, a ratio of the transverse diameter of the main pulmonary trunk to the ascending aorta of >1, and enlargement of the right ventricle and right atrium. A right atrial floating catheter test showed right ventricular pressure of 82/0/4 mmHg, mean pulmonary artery pressure of 83/34/53 mmHg, and pulmonary artery wedge pressure of 15/8/12 mmHg. A mutation was found in the eukaryotic translation initiation factor 2 alpha kinase 4 ( EIF2AK4 ) gene. Thus, the patient was diagnosed with PVOD and subsequently given standard bosentan treatment (62.5 mg twice a day). However, after 6 months of follow-up, there was no significant improvement in the pulmonary artery pressure or activity tolerance (6-minute walking test). Therefore, cardiopulmonary transplantation was performed. Early diagnosis and timely treatment of PVOD may improve the patient's prognosis.

Objectives: The aim of this study was to describe management of recurrent pulmonary vein stenosis (PVS) and determine if stenting is superior to balloon angioplasty (BA) in preventing subsequent restenosis., Background: PVS is a serious complication of atrial fibrillation ablation. BA and stenting are effective therapies; however, restenosis frequently occurs. Here we report management of recurrent stenosis., Methods: This was a prospective observational study performed from 2000 to 2014., Results: One hundred and thirteen patients with severe PVS underwent intervention in 88 veins treated with BA and 81 treated with stenting. Forty-two patients experienced restenosis. Restenosis was more common in veins treated with BA (RRR 53% [95% CI 32-70%, p = .008]). A second intervention was performed in 41 patients. In the 34 vessels treated with initial BA, 24 were treated for restenosis with a stent and 10 were treated with a second BA. The recurrence rate was 46% in those treated with BA followed by stenting and 50% in those treated with two BA procedures. In the 22 veins treated with initial stenting, 9 were treated with another stent and 13 were treated with BA. The recurrence rate was 44% in those treated with a second stent and 46% for those treated with a stent followed by BA. The risk of a third stenosis was the same among all groups (Analysis of variance [ANOVA] p = .99). Limited sample size precluded analysis of outcome by stent size., Conclusions: Restenosis occurred in 44% of patients overall. Management is challenging; stenting does not appear to be superior to BA., (© 2019 Wiley Periodicals, Inc.)

Objectives: Report long-term outcomes of percutaneous intervention in patients with pulmonary vein stenosis (PVS) after pulmonary vein isolation (PVI) from a single center over 16 years., Background: Outcome reports of percutaneous intervention for PVS resulting from PVI are limited., Methods: Retrospective review of all patients with PVS after PVI who underwent percutaneous intervention at the Cleveland Clinic Foundation between January 2000 and December 2016., Results: A total of 205 patients underwent cardiac catheterization for PVS during the study period. Completely occluded veins which could not be recanalized occurred in six patients. Of the remaining 199 patients, 27 (14%) were lost to follow-up, leaving 172 patients with 276 veins for analysis. Balloon angioplasty was performed in 62 veins and stent implantation in 250 (primary in 214, to treat postdilation restenosis in 36). Re-intervention occurred in 45/62 (73%) balloon-dilated veins and 45/250 (18%) stented veins. Freedom from re-intervention at 1 and 5 years was 90 and 73% following stenting versus 40 and 23% following balloon dilation (p < .001, Hazard ratio (HR) = 5.7). Veins with stent diameter ≥7 mm (n = 231) had greater freedom from re-intervention (95% at 1 year, 79% at 5 years) than veins with stents <7 mm (43% at 1 year, 9% at 5 years), p < .001. There was clear symptomatic improvement after intervention and no procedural mortality., Conclusions: Stent implantation at ≥7 mm for PVS after PVI is associated with low rates of re-intervention, in contrast to balloon dilation and stenting with small conventional stents., (© 2019 Wiley Periodicals, Inc.)

Fibrosing mediastinitis is a rare, often debilitating and potentially lethal disease characterized by an exuberant fibroinflammatory response within the mediastinum. Patients typically present with insidious symptoms related to compression of adjacent structures including the esophagus, heart, airways, and cardiac vessels. Fibrosing mediastinitis is most often triggered by Histoplasmosis infection; however, antifungal and anti-inflammatory therapies are largely ineffective. While structural interventions aimed at alleviating obstruction can provide significant palliation, surgical interventions are challenging with high mortality and clinical experience with percutaneous interventions is limited. Here, we will review the presentation, natural history, and treatment of fibrosing mediastinitis, placing particular emphasis on catheter-based therapies., (© 2019 Wiley Periodicals, Inc.)

Introduction: Thermal injury during radiofrequency ablation (RFA) of atrial fibrillation (AF) can lead to pulmonary vein stenosis (PVS). The aim of the present study was to analyze the natural course of RFA-induced PVS with regard to the grade of stenosis, clinical symptoms, and mortality during long-term follow-up., Methods and Results: All patients with follow-up imaging for radiofrequency-induced untreated PVS were retrospectively assessed. From 2004 to 2017, the total rate of PVS following AF ablation in our center was 0.78% (87 of 11 103). Thirty-eight patients with a total of 54 untreated PVS underwent follow-up including imaging scan. The mean degree of stenosis at the time of diagnosis was 57% ± 27% vs 45% ± 35% (P = .05) after a mean follow-up of 43 ± 31 months. There was a shift in severity of the PVS: 18 of 54 (33%) vs 16 of 54 (30%) severe PVS, 19 of 54 (35%) vs 10 of 54 (18%) moderate PVS, and 17 of 54 (32%) vs 28 of 54 (52%) mild PVS (P = .0001). The mean symptom score decreased significantly during follow-up (1.8 ± 1.0 vs 0.4 ± 0.5, P = .0001). Each of the four patients with progression of PVS underwent another pulmonary vein isolation for AF recurrence following pulmonary vein reconduction during follow-up period., Conclusion: This study showed a spontaneous reduction in stenosis grade and symptoms of PVS over a 3.5-year follow-up. Consequently, routine follow-up imaging of PVS seems not to be necessary. However, additional RF energy delivery to stenotic pulmonary veins should be avoided if possible. In case of conduction recovery, the ablation line should be done wide-antrally and follow-up imaging of PVS is recommended., (© 2019 Wiley Periodicals, Inc.)

Pulmonary veno-occlusive disease (PVOD) is a rare subtype of pulmonary arterial hypertension (PAH) characterised by preferential remodelling of the pulmonary venules. Differentiation from other subtypes of PAH is essential as the management can differ significantly; for example, initiation of vasodilator therapy may cause fatal pulmonary oedema in a patient with PVOD misdiagnosed with idiopathic PAH. PVOD also carries a substantially worse prognosis. Lung biopsy is required for definitive diagnosis, but this is hazardous, and ideally, should be avoided in pulmonary hypertension. Computed tomography (CT) may suggest the diagnosis, directing the patient towards specialist review. Potential distinguishing CT features between PVOD and other subtypes of PAH include interlobular septal thickening, mediastinal lymphadenopathy, and centrilobular ground-glass opacities. No evidence-based medical therapy exists for PVOD at present and lung transplantation remains the definitive treatment for eligible patients. Therefore, early radiological identification of this challenging diagnosis facilitates timely referral for transplant., (Crown Copyright © 2019. Published by Elsevier Ltd. All rights reserved.)

A percutaneous transcatheter balloon dilation of a pulmonary venous pathway obstruction was successfully performed in a 40-year-old patient after a Mustard procedure. During the procedure, real-time three-dimensional trans-oesophageal echocardiography demonstrated the morphology of the obstruction. Our case highlights the usefulness of real-time three-dimensional trans-oesophageal echocardiography as a guide for transcatheter intervention in the increasing number of adults with CHD.

Pulmonary vein stenosis (PVS) is a rare disorder that leads to progressive narrowing of the extrapulmonary veins. PVS has been reported in both children and adults and in its worse iteration leads to pulmonary hypertension, right ventricular failure, and death. Multiple etiologies of PVS have been described in children and adults. This review will focus on intraluminal PVS in children. Intraluminal PVS has an estimated incidence ranging from 0.0017% to 0.03%. It is associated with conditions such as prematurity, bronchopulmonary dysplasia, necrotizing enterocolitis, Smith-Lemli-Opitz syndrome, and Down syndrome. Cardiac catheterization and pulmonary vein angiography are the gold standard for diagnosis and anatomic delineation. Other imaging modalities including magnetic resonance imaging, chest tomography, and transesophageal echocardiography are increasingly being used. Mortality of PVS in children is approximately 50%. Predictors of mortality include involvement of ≥3 pulmonary veins, bilateral pulmonary vein involvement, onset of PVS in infancy, elevated pulmonary artery pressure or systolic pulmonary artery-to-aortic pressure ratio, right ventricular dysfunction, restenosis after surgery, distal/upstream disease, and disease progression to previously uninvolved pulmonary veins. Treatment includes catheter-based pulmonary vein dilations with or without stenting, surgical interventions, medical therapy, and in some instances, lung transplantation. Cardiac catheterization for PVS involves a comprehensive hemodynamic and anatomic assessment of the pulmonary veins as well as therapeutic transcatheter interventions. Several surgical strategies have been used. Sutureless repair is currently most commonly used, but patch venoplasty, endarterectomy, ostial resection, and reimplantation are used in select circumstances as well. Medical therapies such as imatinib mesylate and bevacizumab are increasingly being used in an effort to suppress the myofibroblastic proliferation seen in PVS patients. Lung transplantation has been used as an alternative treatment strategy for end-stage, refractory PVS. Nonetheless, despite the different innovative approaches used, morbidity and mortality remain high. At present, the preferred treatment strategy is frequent reassessment of disease progression to guide use of catheter-based and surgical interventions in conjunction with medical therapy.

Background: In children, idiopathic and heritable pulmonary arterial hypertension present echocardiographic and heart catheterization findings similar to findings in pulmonary veno-occlusive disease., Objective: To provide a systematic analysis of CT angiography anomalies in children with idiopathic or heritable pulmonary arterial hypertension, or pulmonary veno-occlusive disease. We also sought to identify correlations between CT findings and patients' baseline characteristics., Materials and Methods: We retrospectively analyzed CT features of children with idiopathic and heritable pulmonary arterial hypertension or pulmonary veno-occlusive disease and 30 age-matched controls between 2008 and 2014. We compared CT findings and patient characteristics, including gene mutation type, and disease outcome until 2017., Results: The pulmonary arterial hypertension group included idiopathic (n=15) and heritable pulmonary arterial hypertension (n=11) and pulmonary veno-occlusive disease (n=4). Median age was 6.5 years. Children with pulmonary arterial hypertension showed enlargement of pulmonary artery and right cardiac chambers. A threshold for the ratio between the pulmonary artery and the ascending aorta of ≥1.2 had a sensitivity of 90% and a specificity of 100% for pulmonary arterial hypertension. All children with pulmonary veno-occlusive disease had thickened interlobular septa, centrilobular ground-glass opacities, and lymphadenopathy. In children with idiopathic and heritable pulmonary arterial hypertension, presence of intrapulmonary neovessels and enlargement of the right atrium were correlated with higher mean pulmonary artery pressure (P=0.011) and pulmonary vascular resistance (P=0.038), respectively. Mediastinal lymphadenopathy was associated with disease worsening within the first 2 years of follow-up (P=0.024)., Conclusion: CT angiography could contribute to early diagnosis and prediction of severity in children with pulmonary arterial hypertension.

Objective: The efficacy of using a sutureless approach in order to surgically manage postoperative pulmonary vein stenosis following total anomalous pulmonary venous drainage (TAPVD) has been reported, though outcomes of primary treatment of supracardiac TAPVD remain unclear. We retrospectively reviewed our cardiac center experience, and compared the differences in mid-term outcomes for those patients that received conventional surgery and those that underwent sutureless technique for the primary repair of supracardiac TAPVD., Methods: A total of 43 patients (median age, 199 days; range, 35 days to 1572 days) with supracardiac TAPVD underwent surgical treatment at our cardiac center from 2014 to 2018 were studied retrospectively. Primary sutureless repair was conducted in 20 cases (46.5%). The pulmonary vein scores, left ventricular ejection fraction (LVEF), baseline of the included patients, postoperative, and outcomes data were analyzed between the two groups., Results: The pulmonary vein scores, indicating the stenosis degree, of two groups were 0.1 ± 0.3 and 0.1 ± 0.3, left ventricular ejection fraction (LVEF) (%) were separately 66.2 ± 12.1 and 67.1 ± 13.6. The average cardiopulmonary bypass time of sutureless techniques group was much longer than conventional group (96.2 ± 32.6 min vs 75.6 ± 28.2 min, P < 0.05), but there was no difference in aortic cross-clamp time between the two groups. Followed up from 0.1 to 4 years, 3 cases died overall, with 1 (5.0%) individual dying from postoperative pulmonary venous obstruction (PVO) in sutureless group, and 2 (8.6%) dying in the conventional group respectively for postoperative infection and post-PVO. There were no differences in the length of stay in the ICU, grades of PVS after surgery, LVEF and reoperation rate between the two groups., Conclusions: The mortality, post-PVO, follow up results of supracadiac TAPVD showed no differences between sutureless and conventional techniques. Post-PVO supposed to be the main reason for postoperative mortality.

Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension (PH). The prognosis of PVOD patients remains poor, since no effective medical therapy is yet available. Imatinib is a tyrosine kinase inhibitor specific for platelet-derived growth factor receptor and is expected as a treatment option for pulmonary arterial hypertension (PAH). Recently, it has been reported that imatinib improved functional capacity of a patient with PVOD. We here report a patient with suspected PVOD who has been successfully treated with imatinib and is alive for 6 years after diagnosis. A 57-year-old woman was admitted to a hospital for severe dyspnea. Echocardiography suggested the presence of PH, because tricuspid regurgitation pressure gradient was elevated. The patient was then transferred to our hospital by an ambulance ahead of schedule due to fever and worsening dyspnea. Because the patient had no left heart disease, we diagnosed that she had PAH associated with severe right heart failure. We immediately started treatment with nitric oxide (NO) for her severe hypoxia; however, it caused pulmonary edema. We suspected PVOD from CT characteristics and pulmonary edema after PAH-targeted vasodilator therapy, and then started oral imatinib treatment. In response to imatinib, her pulmonary edema gradually improved. Since then, the patient has been alive for 6 years with imatinib and pulmonary vasodilators. At present, lung transplantation is the only effective therapy for PVOD with limited availability. We therefore propose that imatinib may be a treatment option for PVOD and a bridge to lung transplantation.

Background: Thermal injury during radiofrequency ablation (RFA) of atrial fibrillation (AF) can lead to pulmonary vein stenosis (PVS). It is currently unclear if routine screening for PVS by imaging (echocardiography, computed tomography) is clinically meaningful and if there is a correlation between PVS and the electroanatomical mapping system (EAMS) used for the ablation procedure. It was therefore investigated in the current single center experience., Methods: All patients from January 2004 to December 2016 with the diagnosis of PVS after interventional ablation of AF by radiofrequency were retrospectively analyzed. From 2004 to 2007, transesophageal echocardiography was routinely performed as screening for RFA-acquired PVS (group A). Since 2008, diagnostics were only initiated in cases of clinical symptoms suggestive for PVS (group B)., Results: The overall PVS rate after interventional RFA for AF of the documented institution is 0.72% (70/9754). The incidence was not influenced by screening: group A had a 0.74% PVS rate and group B a 0.72% rate (NS). Referred to as the EAMS, there were significant differences: 20/4229 (0.5%) using CARTO®, 48/4510 (1.1%) using EnSite®, 1/853 (0.1%) using MediGuide®, and 1/162 (0.6%) using Rhythmia®. Since 2009, no significant difference between technologies was found., Conclusions: The present analysis of 9754 procedures revealed 70 cases of PVS. The incidence of PVS is not related to screening but to the application of different EAMS. Possible explanations are technological backgrounds (magnetic vs. electrical), learning curves, operator experience, and work-flow differences. Furthermore, incorporation of new technologies seems to be associated with higher incidences of PVS before workflows are optimized.

Aims: Results of catheter based interventional treatment for pulmonary vein stenosis (PVS) following radiofrequency ablation (RFA) for atrial fibrillation remain suboptimal. Surgical repair may represent an alternative therapy, though long-term results have not been thoroughly investigated., Methods and Results: We retrospectively assessed all patients in our centre undergoing surgical repair for radiofrequency-induced PVS. Data regarding surgical technique, clinical outcome, and rate of pulmonary vein (PV) restenosis were collected and analysed. Between 2004 and 2016, the rate for PVS resulting from RFA for atrial fibrillation in our institution was 0.79% (76/9633). During this period, five male patients with multiple PVS (3 ± 1) underwent surgical repair of a total of 13 symptomatic PVS. Surgery was performed in a standard setting under cardiopulmonary bypass. Stenotic veins were incised longitudinally followed by a patch augmentation plasty using either bovine pericard (n = 7) or polytetrafluoroethylene (PTFE) patches (n = 5). Localization of incision was on the anterior side of the PV only (n = 8) or on both the anterior and posterior sides (n = 4). In one PVS lesion, mechanical dilatation was sufficient. Long-term follow-up after 60 ± 69 months revealed an average restenosis rate of 38%. Restenosis was defined as narrowing >70%. All patients reported clinical improvement of symptoms at follow-up., Conclusion: Even in the era of wide circumferential lesions, PVS still occurs. While surgical PV patch plasty represents a valuable treatment option, restenosis remains an issue during follow-up. Nevertheless, surgical repair achieves highly acceptable long-term results for RFA-acquired PVS. Hence, it should be routinely discussed as a therapeutic option in cases with multiple PVS.

Background: Histologic features of pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) have been described in dogs but without a thorough clinical description., Objectives: To report the clinical features, diagnostics, treatment, and outcome of dogs with histologic evidence of PVOD and PCH., Animals: Fifteen pet dogs meeting histopathologic criteria of PVOD (occlusive remodeling of small-sized to medium-sized pulmonary veins) or PCH (alveolar capillary proliferation and congestion), or both., Methods: Medical records of dogs with PVOD and PCH identified based on histopathologic features between 2003 and 2017 were retrospectively reviewed., Results: Fifteen dogs met inclusion criteria of a histologic diagnosis of PVOD or PCH or both. Dogs were older (median 11 years) with no apparent breed or sex predisposition. Dogs presented with acute clinical signs (median 3 days), usually respiratory distress. Thoracic radiography (available in 10 dogs) revealed right cardiomegaly and patchy or diffuse interstitial to alveolar patterns, with 9 dogs having a normal left cardiac silhouette. In 5 dogs tested, pulmonary arterial hypertension (PAH) was documented. In all 3 dogs, thoracic computed tomography scans showed pulmonary arterial enlargement and perivascular diffuse nodular ground-glass opacities. Ten of 15 dogs died within 1 day; median survival was 3 days., Conclusions and Clinical Importance: In dogs with PAH, the inability to document left-sided congestive heart failure and failure to identify another cause of signs of respiratory disease should increase suspicion for PVOD and PCH. With increased awareness of PVOD and PCH by clinicians and pathologists, dogs with compatible clinicopathologic features should be evaluated for these pulmonary vascular disorders., (© 2018 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals, Inc. on behalf of the American College of Veterinary Internal Medicine.)

Purpose: Pulmonary venography is routinely used to confirm pulmonary vein (PV) occlusion during cryoballoon ablation. However, this technique is significantly limited by the risks associated with contrast media, such as renal injury and contrast allergy. We hypothesized that PV occlusion can be predicted by elevation of the balloon catheter tip pressure, avoiding the need for contrast media., Methods: Forty-eight consecutive patients with paroxysmal atrial fibrillation who underwent PV isolation with the cryoballoon technique were enrolled. The balloon catheter tip pressure was measured in each PV before and after balloon inflation., Results: We analyzed 200 applications of cryoballoon ablation in 185 PVs (excluding 3 common PVs and 1 extremely small right inferior PV) of 48 patients (age, 70 ± 11 years; male, n = 28; mean left atrial diameter, 38 ± 6 mm). Compared with patients with unsuccessful occlusion, patients with successful occlusion demonstrated a larger change in pressure after balloon inflation (6 ± 8 vs. 2 ± 4 mmHg, P < 0.001), a lower minimum temperature (- 49 ± 6 vs. - 40 ± 8 °C, P < 0.001), and a higher PV isolation rate (97 vs. 64%, P < 0.001). The best cutoff value of a change in pressure for predicting PV occlusion was 4.5 mmHg, with a sensitivity of 67%, specificity of 83%, and predictive accuracy of 72%., Conclusion: Pressure monitoring is helpful to confirm PV occlusion during cryoballoon ablation.

The objective of this study is to evaluate neonatal outcomes of total anomalous pulmonary venous return (TAPVR) and identify fetal echocardiography findings associated with preoperative pulmonary venous obstruction (PPVO). This retrospective study evaluated TAPVR cases from 2005 to 2014 for preoperative and postoperative outcomes based on prenatal diagnosis, PPVO, and heterotaxy syndrome. Fetal pulmonary and vertical vein Dopplers were analyzed as predictors of PPVO. Of 137 TAPVR cases, 12% were prenatally diagnosed; 60% had PPVO, and 21% had heterotaxy. Of the prenatally diagnosed patients, 63% also had heterotaxy. TAPVR repair was performed in 135 cases and survival to discharge was 82% (112/137). Heterotaxy was the only independent predictor of mortality on multiple regression analysis [OR 5.5 (CI 1.3-16.7), p = 0.02]. PPVO was associated with preoperative acidosis, need for inhaled nitric oxide, and more emergent surgery, but not postoperative mortality. Fetal vertical vein Doppler peak velocity > 0.74 m/s mmHg predicted PPVO (93% sensitivity; 83% specificity) while pulmonary vein Doppler did not. TAPVR has severe neonatal morbidity and mortality with low prenatal diagnosis rates in the absence of heterotaxy. Patients with obstructed TAPVR had greater preoperative morbidity, but only heterotaxy was independently associated with increased postoperative mortality. Vertical vein velocity helped prenatally identify those at risk of PPVO.

Background: Pulmonary vein (PV) stenosis remains a feared complication of atrial fibrillation ablation. Little is known about outcomes in patients with severe PV stenosis, especially about repeat ablations., Methods: In 10 368 patients undergoing atrial fibrillation ablation (2000-2015), computed tomography scans were obtained 3 to 6 months after ablation. The clinical outcomes in severe PV stenosis were determined., Results: Severe PV stenosis was diagnosed in 52 patients (0.5%). This involved mostly the left superior PV (51% of severely stenosed veins). Percutaneous interventions were performed in 43 patients, and complications occurred in 5: 3 PV ruptures, 1 stroke, and 1 phrenic injury. Over a median follow-up of 25 months, 41 (79%) patients remained arrhythmia free. Repeat ablation was performed in 15 patients (7 from the main series and 8 from prior ablation at other institutions); of whom 10 had PV stents in place. Conduction recovery was noted in all but 2 of the stenosed or stented PVs, and areas with recovery were targeted with antral ablation. Lasso entrapment within stents occurred in 2 patients but eventually freed without complications. After redo ablation, preplanned stenting was performed in 3 patients and computed tomographic scans showed progression of concomitant stenoses in 1 patient (moderate to severe). No procedure-related deaths occurred., Conclusions: The incidence of severe PV stenosis is low but remains associated with significant morbidity. In patients with recurrent arrhythmia, conduction recovery at the stenosed or stented veins is common. Care must be taken to ablate antrally to avoid stenosis progression. In patients with prior PV stents, we suggest to avoid using Lasso., (© 2018 American Heart Association, Inc.)

Case Presentation: A 77-year-old woman presented to the hospital with symptoms of progressive shortness of breath with associated right-sided pleuritic pain. The patient had begun noting dyspnea on exertion, limiting her ability to go on hikes over the few days prior to admission. Her medical history is significant for carcinoid tumor status postresection in 2012 without recurrence. She has no history of thromboembolism or clotting disorders, and she has no history of smoking or drug abuse. Current medications include amlodipine, celecoxib, hydrochlorothiazide, and rosuvastatin., (Copyright © 2017 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)

Objective: To improve the diagnosis and treatment of the pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangioma (PCH). Methods: The clinical features, radiological findings, laboratory testing and treatment in 8 cases of PVOD/PCH which was diagnosed from 2013 to 2017 were described. Results: PVOD/PCH was rare. The clinical symptoms were easily confused with IPAH, but the decrease of hypoxemia, clubbing, D(L)CO were more obvious, and the imaging features of HRCT were helpful for PVOD/PCH diagnosis. Combined with gene testing, it was helpful to diagnose PVOD/PCH and avoid the risk of surgical biopsy. Conclusion: PVOD and PCH are rare type of pulmonary vascular diseases. According to clinical manifestations, physical examination, pulmonary function test results, HRCT imaging, CPET and gene detection results, PVOD or PCH can be diagnosed.

Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension caused by alteration of pulmonary veins. Many clinical and hemodynamic similarities to idiopathic pulmonary arterial hypertension (IPAH) may cause diagnostic and therapeutic difficulties. This case report is about a patient with PVOD, whose first symptoms of the disease occurred after infectious mononucleosis. Patient was administered with prostacycline (PGI2) mimetic (Treprostinil), what made qualification process and lung transplantation possible. Despite more and more knowledge about causes, ethiopathogenesis and changes in pulmonary veins on molecular level, lung transplantation is the only successful therapeutic option for patients suffering from PVOD.

Objectives: SSc-pulmonary arterial hypertension (SSc-PAH) is associated with worse response to therapy and survival when compared with idiopathic PAH. It is suggested that the vasculopathy in SSc may involve postcapillary pulmonary venules resulting in pulmonary veno-occlusive disease (PVOD). This may underlie the lower gas transfer and worse outcome on therapy. We sought to test whether CT signs of PVOD (CTS-PVOD) were frequent in SSc-PAH and whether they were associated with pulmonary oedema on therapy and worse survival., Methods: CT thorax of 66 SSc patients with precapillary pulmonary hypertension (PH) were blindly scored by two radiologists for CTS-PVOD (⩽1 or ⩾ 2). Case note and radiograph review determined the presence of pulmonary oedema on therapy., Results: Fifty-nine patients (89%) had ⩽1 CTS-PVOD and only 7 (11%) had ⩾2 CTS-PVOD. Pulmonary oedema on therapy was relatively common in those with ⩾2 CTS-PVOD. On univariate analysis ⩾2 CTS-PVOD were associated with a trend towards worse survival., Conclusion: CTS-PVOD were less frequent in this SSc-PAH cohort than in previous reports but the presence of at least two of these signs is associated with pulmonary oedema on therapy and a trend towards worse survival on univariate analysis., (© The Author 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com)

Pulmonary veno-occlusive disease is a rare subcategory of pulmonary arterial hypertension (WHO Group 1). The disease is poorly understood and difficult to diagnose; it has no definitive cure to date. These patients present with nonspecific symptoms, including dyspnea, exercise intolerance, and weakness. Chest x-rays sometimes differ from idiopathic pulmonary arterial hypertension and may demonstrate alveolar infiltrates and pleural effusions. High resolution computed tomography scans reveal ground glass opacities, interlobular septal thickening, and lymphadenopathy. Echocardiography can estimate the level of pulmonary artery pressures; right heart catheterization is needed for complete hemodynamic characterization of these patients. Lung biopsies demonstrate remodeling of the venules and small veins with intimal and adventitial fibrosis. This can result in total venous occlusion and subsequent recanalization. Similar changes occur in the small arteries and arterioles but are less pronounced than the venous changes. There is no effective medical therapy for these patients, and treatment with the pulmonary arterial hypertension specific medications often causes acute deterioration with pulmonary edema. The recent discovery of the biallelic mutations of the EIF2AK4 gene as an etiology for heritable form of pulmonary veno-occlusive disease increases our understanding of the disease pathogenesis and potentially identifies a future approach to treatment. Without definitive treatment, the prognosis is very poor, and the life expectancy of these patients is much shorter than patients with pulmonary arterial hypertension. These patients need early referral to transplantation centers., (Published by Elsevier Ltd.)

Total anomalous pulmonary venous drainage (TAPVD) is an uncommon cardiac defect in children. The mixed subset accounts for 5%-10% of the TAPVD and is variable in its anatomy. The outcomes associated with this subset of patients are rarely reported. A retrospective review of all patients with mixed TAPVD undergoing repair at a single institution (1984-2014) was conducted. A descriptive analysis was performed. Twenty-four patients underwent repair for mixed TAPVD (6 univentricular physiology, 18 biventricular physiology). The mixed TAPVD anatomy included 8 patients in group I (2 + 2 veins), 11 patients in group II (3 + 1 veins), and 5 patients in group III (atypical). Preoperative pulmonary venous obstruction occurred in 8 patients (33%). The median age at repair was 2.2 months (range 2 days to 3 years) and median weight was 4.2 kg (range 1.9 to 12.5 kg). Operative mortality was 13% (3 of 24), 33.3% (2 of 6) for patients with univentricular physiology, and 5.6% (1 of 18) for patients with biventricular physiology. There have been no operative deaths in the biventricular group since 1997 (n = 11). Survival at 30-days was 83% ± 15% (95% confidence interval: 27%-97%) and 94% ± 5% (95% confidence interval: 67%-99%) for the univentricular and biventricular groups, respectively. Reoperation for recurrent pulmonary venous obstruction was required in 2 patients (8.3%) where the sutureless technique was used. The average follow-up after surgery was 9.3 ± 6.4 years (5 months to 21 years), and all surviving patients were asymptomatic. Mixed TAPVD can be repaired with good results in children, particularly in those undergoing biventricular repair., (Crown Copyright © 2017. Published by Elsevier Inc. All rights reserved.)

Total anomalous pulmonary venous connection refers to a spectrum of cardiac anomalies where the pulmonary veins fail to return to the left atrium and the pulmonary venous blood returns through a systemic vein or directly to the right atrium. There is a wide anatomical variety of venous connections and degrees of pulmonary venous obstruction that affect the presentation, surgical repair, and outcomes. In this review, we explore the preoperative physiology, echocardiographic diagnosis, and approach to postoperative complications.

Thoracic aneurysms can potentially cause substantial compression of adjacent structures, creating substantial symptoms. We present a case of a 56-year-old woman with fatigue and dyspnea for 6 months. We discuss her initial endovascular treatment, which was insufficient to improve symptoms, and further surgical intervention was needed to solve the issue., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Stroke is a clinically important problem. A left atrial thrombus is known as a cause of ischaemic stroke. A pulmonary vein thrombus (PVT) is thought to be rare; however, PVT is common in elderly patients. Additionally, images of PVT with transoesophageal echocardiography (TEE) following treatment have not been well contrasted with that from either 64 or 80-slice multidetector CT (80-MDCT). The images of such changes depicted by TEE remain unknown. An 87-year-old man with hypertension was examined by 80-MDCT and TEE to check the cardiac thrombus. Although 80-MDCT did not depict the thrombus, TEE depicted the thrombus in the left atrium and right lower pulmonary vein (RLPV) clearly. After 6 months of rivaroxaban treatment, the thrombus in the RLPV decreased slightly and the thrombus in the left atrium became small and lucent, as estimated with TEE. Rivaroxaban decreased the size of the thrombus and changed the quality of the thrombus., Competing Interests: Conflicts of Interest: None declared., (2017 BMJ Publishing Group Ltd.)

A 58-year-old woman was referred to our hospital with a chief complaint of exertional dyspnea. Bronchoscopy failed to establish a diagnosis, and the patient subsequently died suddenly due to respiratory insufficiency because of advanced pulmonary hypertension (PH). The pathological diagnosis at autopsy was pulmonary veno-occlusive disease (PVOD). PVOD is difficult to diagnose antemortem and has a poor prognosis. Lung transplantation is the only curative treatment for PVOD.