A Rare Variant of Left Atrial Myxoma

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World Health Organization Pulmonary Hypertension Group 2: Pulmonary hypertension due to left heart disease in the adult—a summary statement from the Pulmonary Hypertension Council of the International Society for Heart and Lung Transplantation

Meeting abstracts from the 15th international conference on neonatal and childhood pulmonary vascular disease

The Cardio-Obstetrics Patient and the Cardiothoracic Anesthesiologist

Descriptive patterns of severe chronic pulmonary hypertension by chest radiography

The pathophysiology of primary pulmonary hypertension (PPH) involves alterations in vascular reactivity, vascular structure, and interactions of the vessel wall with circulating blood elements.1 An imbalance of vasodilator and vasoconstrictor influences is likely to be an early derangement. Progressive intimal and medial thickening, due to proliferation and migration of vascular smooth muscle cells and fibroblasts, reduces the cross-sectional area of the pulmonary microvasculature, causing fixed alterations in pulmonary resistance. Contributing to the progressive increase in pulmonary resistance is thrombosis of the small pulmonary vessels, which explains the benefit of anticoagulation in these patients. In advanced disease, “plexiform arteriopathy” of the small pulmonary vessels is observed. These lesions proliferate into the lumen, creating high-resistance, convoluted endoluminal channels. There is some controversy regarding the nature of the cells constituting these lesions, one group suggesting they are of endothelial origin, whereas more recent evidence indicates that they are myofibroblasts.2,3 See p 1493 The normal pulmonary endothelium maintains a low vascular resistance, suppresses vascular smooth muscle growth, inhibits platelet adherence and aggregation, and stems inflammation. In patients with PPH, the endothelium has lost these vasoprotective functions.1 The endothelium of the PPH patient is characterized by the increased elaboration of vasoconstrictors, mitogens, and prothrombotic and proinflammatory mediators (such as thromboxane, endothelin, plasminogen activator inhibitor, and 5-lipooxygenase). These endothelial alterations promote the pathophysiology of PPH. Furthermore, there is less influence of the countervailing factors prostacyclin and NO. Endothelium-derived NO plays a critical role in pulmonary …

Pulmonary hypertension related to thalidomide therapy in refractory multiple myeloma

Since the last World Symposium on Pulmonary Hypertension in 2008, we have witnessed numerous and exciting developments in chronic thromboembolic pulmonary hypertension (CTEPH). Emerging clinical data and advances in technology have led to reinforcing and updated guidance on diagnostic approaches to pulmonary hypertension, guidelines that we hope will lead to better recognition and more timely diagnosis of CTEPH. We have new data on treatment practices across international boundaries as well as long-term outcomes for CTEPH patients treated with or without pulmonary endarterectomy. Furthermore, we have expanded data on alternative treatment options for select CTEPH patients, including data from multiple clinical trials of medical therapy, including 1 recent pivotal trial, and compelling case series of percutaneous pulmonary angioplasty. Lastly, we have garnered more experience, and on a larger international scale, with pulmonary endarterectomy, which is the treatment of choice for operable CTEPH. This report overviews and highlights these important interval developments as deliberated among our task force of CTEPH experts and presented at the 2013 World Symposium on Pulmonary Hypertension in Nice, France.

BackgroundHereditary hemorrhagic telangiectasia (HHT) is a rare genetic disease. The prevalence of pulmonary arterial hypertension (PAH) in HHT patients is less than 1%. Severe pulmonary hypertension (PH) in pregnant woman due to HHT and reversible pulmonary hypertension in her neonate is even rarer.MethodsCases of mother and newborn with PH are presented, including their clinical manifestations, diagnosis, and treatment. Additionally, literatures were reviewed to explore the various causes of the disease.ResultsThe mother was diagnosed with HHT caused by an ACVRL1 gene variant, with her PH occurring as a complication of HHT type 2 (HHT2). The neonate was confirmed to be free of the ACVRL1 gene variant, and her PH resulted from impaired placental perfusion and adverse intrauterine environment secondary to severe maternal PH and anemia.ConclusionAttention should be paid to the progression of the disease and the comprehensive management strategies during pregnant HHT patient. Moreover, neonates born to HHT-affected mothers require evaluation for both HHT-related PH and reversible PH secondary to adverse intrauterine factors. This report aims to enhance the recognition of familial manifestations of HHT and raise awareness of the occurrence of postnatal PH in neonates of mothers with HHT, thereby facilitating early detection and timely intervention.

This paper provides a critical exploration of the ‘journey metaphor’ promoted in much business discourse on sustainability - in corporate reports and advertisements, and in commentators’ reports in the political and professional business literature. The portrayal of sustainability as a journey evokes images of corporate adaptation, learning, and a movement away from business-as-usual practices. The journey metaphor, however, masks the issue of towards what it is that businesses are actually, or even supposedly, moving. It is argued that business is constructing ‘sustainability’ as a journey to avoid specifying some future desirable state of affairs. We suggest that by portraying ‘sustainability’ in this way, businesses, and the related political and professional literature, have invoked a subtle and powerful, use of language that appears to seriously engage with elements of the discourse around sustainable development and sustainability. Yet at the same time, by constructing and promoting its own version of the discourse, it de-emphasises discussion of desirable future states of living, and neatly sidesteps any debate about, or need to radically change course. The paper illustrates how journeying is invoked throughout corporate reports and other forms of business communication in a process of corporate myth-making. Businesses are shown to be constructing a ‘wonderland’ discourse.

Cor Pulmonale from Concomitant Human Immunodeficiency Virus Infection and Methamphetamine Use

Going Home with a Patent Ductus Arteriosus: Is it Benign?

See related article, pages 1109–1114 Idiopathic pulmonary artery hypertension (IPH) is a rare illness with a poor prognosis. Whereas chronic intravenous prostacyclin relieves some of the symptoms of progressive dyspnea and prolongs survival, most patients ultimately require a lung transplant.1 Newer therapies such as nonintravenously administered prostacyclin derivatives,2,3,4 endothelin receptor blockers,5,6 and, to some extent, phosphodiesterase inhibitors,7 hold some promise as alternatives for intravenous prostacyclin, but current expectation is that, like prostacyclin, they will, at best, retard disease progression, serving as a bridge to transplant rather than as an alternative. The pathological features of IPH are loss of small distal precapillary pulmonary arteries, obliterative changes (plexogenic lesions) in more proximal pulmonary arteries associated with migration and proliferation of smooth muscle cells, and increased extracellular matrix deposition. There is also dysregulation of endothelial cells associated with increased proliferation.8 The mechanism underlying the evolution of these changes is unknown, so there was great interest when 2 groups independently identified a mutation in bone morphogenetic protein receptor 11 (BMP-RII) in 60% of families with IPH.9,10 A BMP-RII mutation also occurs in 20% of sporadic cases of IPH,11 but the biological connection between the mutation and the pathobiology of IPH has been relatively elusive. Recent studies using pulmonary artery smooth muscle cells from patients with IPH, including those with and without a BMP-RII mutation, showed similar abnormal proliferation in response to agents such as transforming growth factor-β (TGF-β) or BMP-2.12 In other studies, pulmonary artery smooth muscle cells were transfected with constructs encoding different mutant forms of BMP-RII expressing aberrant kinase or cytoplasmic domains, and impaired signaling was observed related to alterations in the induction of Smads and p38.13 Specifically, suppression of Smad1/5 and activation of p38 were related to smooth muscle cell proliferation. It …

Objective Investigate the clinical features, diagnosis and treatments of the scimitar syndrome, and different forms of treatment to alleviate pulmonary hypertension. Methods A retrospective analysis of clinical data of 14 children with scimitar syndrome from 2013 to 2017, including clinical symptoms and signs, chest X ray, echocardiography, chest CT and cardiac catheterization, treatment outcome and follow-up. Assess embolization of systemic pulmonary collateral and pulmonary venous drainage correction surgery, which is better for lowering pulmonary blood flow. Results 14 patients with scimitar syndrome were diagnosed from 2013 to 2017. There were 5 boys and 9 girls; 3 cases<7 kg in weight. Scimitar syndrome was suspected because of extroversion, and diagnosed by color Doppler echocardiography and 13 of them confirmed by cadiac CT scan when ascimitar vein was detected entering the inferior vena cava. 11 patiens had right lung dysplasia and 4 had horseshoe lung. Three patients had severe pulmonary arterial hypertension, 3 had moderate to severe pulmonary arterial hypertension, and 2 had moderate pulmonary arterial hypertension, the left had slight pulmonary arterial hypertension. 4 patients had pulmonary venous drainage correction surgery, after that 2 of them had systemic pulmonary collateral embolism. 6 patients systemic pulmonary collateral embolism first, then 4 of them had surgical repair, 1 case of 13 years old asymptomatic child without surgery. 1 patient with heart failure, severe pulmonary hypertension, pulmonary infection, died before surgery, while another died after surgical repair. At last 1 patient was lost for follow-up visits. Systemic pulmonary collateral embolism and pulmonary venous drainage correction surgery could all reduce blood flow of pulmonary. After systemic pulmonary collateral embolism, patients had slight pulmonary arterial hypertension just need follow-up visits. Conclusion Clinically, found children with heart of dextrocardia position, growth retardation, recurrent lung infections, unexplained right heart failure, pulmonary hypertension, should consider the possibility scimitar syndrome. Whether pulmonary vascular embolization or surgical repair, can significantly reduce pulmonary artery’s blood flow and alleviate pulmonary hypertension to protect pulmonary, even reduced the incidence of pneumonia and mortality. So we suppose ealy pulmonary hypertension in scimitar syndrome patients maybe dynamic pulmonary hypertension. Key words: Congenital heart defect; Scimitar syndrome; Pulmonary vein; Pulmonary hypertension; Systemic pulmonary collateral circulation

Cardiac Anesthesiologist and Global Capacity Building to Tackle Rheumatic Heart Disease