A case report of systemic lupus erythematosus with severe pulmonary hypertension presenting as large pericardial effusion with early signs of cardiac tamponade: a diagnostic and therapeutic challenge.

Electrocardiogram Findings Associated With Malignant Pericardial Effusion and Cardiac Tamponade.

Isolated Left-Sided Heart Tamponade on Echocardiography in Severe Pulmonary Hypertension and Right Heart Failure

The Cardio-Obstetrics Patient and the Cardiothoracic Anesthesiologist

CARDIAC TAMPONADE WITHOUT CHARACTERISTIC ECHOCARDIOGRAPHIC FEATURES DUE TO HIGH RIGHT HEART PRESSURE FROM PULMONARY HYPERTENSION

Pulmonary arterial hypertension is associated with a decreased antioxidant capacity. However, neither the contribution of reactive oxygen species to pulmonary vasoconstrictor sensitivity, nor the therapeutic efficacy of antioxidant strategies in this setting are known. We hypothesized that reactive oxygen species play a central role in mediating both vasoconstrictor and arterial remodeling components of severe pulmonary arterial hypertension. We examined the effect of the chemical antioxidant, TEMPOL, on right ventricular systolic pressure, vascular remodeling, and enhanced vasoconstrictor reactivity in both chronic hypoxia and hypoxia/SU5416 rat models of pulmonary hypertension. SU5416 is a vascular endothelial growth factor receptor antagonist and the combination of chronic hypoxia/SU5416 produces a model of severe pulmonary arterial hypertension with vascular plexiform lesions/fibrosis that is not present with chronic hypoxia alone. The major findings from this study are: 1) compared to hypoxia alone, hypoxia/SU5416 exposure caused more severe pulmonary hypertension, right ventricular hypertrophy, adventitial lesion formation, and greater vasoconstrictor sensitivity through a superoxide and Rho kinase-dependent Ca2+ sensitization mechanism. 2) Chronic hypoxia increased medial muscularization and superoxide levels, however there was no effect of SU5416 to augment these responses. 3) Treatment with TEMPOL decreased right ventricular systolic pressure in both hypoxia and hypoxia/SU5416 groups. 4) This effect of TEMPOL was associated with normalization of vasoconstrictor responses, but not arterial remodeling. Rather, medial hypertrophy and adventitial fibrotic lesion formation were more pronounced following chronic TEMPOL treatment in hypoxia/SU5416 rats. Our findings support a major role for reactive oxygen species in mediating enhanced vasoconstrictor reactivity and pulmonary hypertension in both chronic hypoxia and hypoxia/SU5416 rat models, despite a paradoxical effect of antioxidant therapy to exacerbate arterial remodeling in animals with severe pulmonary arterial hypertension in the hypoxia/SU5416 model.

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.

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

Cardiac tamponade in systemic sclerosis is rare. We report four cases of SSc with hemodynamically significant pericardial effusions associated with pulmonary arterial hypertension, three of whom died, two following pericardiocentesis. Of 26 SSc cases reported in the literature with large pericardial effusions, seven were associated with PAH. Including our series, the mortality rate is 55%.The potential contributory role of PAH in the development of pericardial effusion and the management implications are explored. In SSc patients with hemodynamically significant pericardial effusions and severe pulmonary hypertension, initial stabilization of pulmonary artery pressure and right heart function with vasoactive therapy and then cautious pericardial drainage should be considered.

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

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

Hypoxia causes pulmonary vasoconstriction. Regional hypoxic vasoconstriction improves the matching of perfusion to alveolar ventilation. Global hypoxic vasoconstriction increases right ventricular afterload. The hypoxic pulmonary pressor response is universal in mammals and in birds, but with considerable interspecies and interindividual variability. Chronic hypoxia induces pulmonary hypertension in proportion to initial vasoconstriction. Prolonged hypoxic exposure is also associated with an increase in red blood cell mass, which aggravates pulmonary hypertension by an increase in blood viscosity. Hypoxic pulmonary hypertension in humans is usually mild to moderate, but pulmonary vascular pressure-flow relationships are steep, which corresponds to a substantial afterload on the right ventricle during exercise. A partial recovery of 10-25% of the hypoxia-induced decrease in maximal oxygen uptake has been reported with intake-specific pulmonary vasodilating interventions. Hypoxia has been reported to decrease myocardial fibre contractility in vitro. However, the acutely hypoxic right ventricle remains able to preserve the coupling of its contractility to increased afterload in intact animals. Echocardiographic studies of the right ventricle in healthy hypoxic human subjects show altered diastolic function, but systolic function that is preserved or even increased acutely and slightly depressed chronically. These findings are more pronounced in patients with chronic mountain sickness. Their clinical significance remains incompletely understood. Almost no imaging studies of right ventricular function have been reported in a minority of subjects who develop severe pulmonary hypertension and clinical right ventricular failure in hypoxia. No imaging studies of right ventricular function during hypoxic exercise in normal subjects are yet available. Thus, while it is plausible that the right ventricle limits exercise capacity in hypoxia, this still needs to be firmly established.

40-Year-Old Woman With Breathlessness and Fatigue

Perioperative implications of pericardial effusions and cardiac tamponade

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 …

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 …