Deep learning-based image quantification of CTPA to phenotype pulmonary arterial hypertension

Pulmonary Hypertension: From an Orphan Disease to a Public Health Problem

A New Classification of Pulmonary Hypertension

Echocardiography in Pulmonary Arterial Hypertension: An Essential Tool

The Metabolic Syndrome and Pulmonary Vascular Disease

Knowledge of molecular mechanisms underlying pulmonary arterial hypertension (PAH) continues to increase with the emerging theme that PAH is a heterogeneous disease involving multiple molecular abnormalities. Mutations in several genes have been identified in subsets of patients with PAH, and multiple signaling systems that influence vascular tone, function, and remodeling have been associated with PAH.1,2,3 In addition to mutations in BMPR2 , serotonin ( 5-HT ) and polymorphisms in its transporter ( SERT ) play a critical role in the pulmonary vascular smooth muscle hyperplasia and vascular remodeling found in PAH.4,5 Other genes and signals thought to contribute to the development of idiopathic pulmonary arterial hypertension (IPAH) include somatic mutations of BAX ,5 upregulation of Angiopoietin 1 ,6 transforming growth factor β1 polymorphisms,7 ALK1 mutation,8 SMAD8 mutation,9 and increased hyaluronic acid content associated with increased Hyaluronan Synthase 1 and decreased Hyaluronoglucosaminidase 1 gene expression.10 A recent observation also suggests that the noncanonical Wnt pathway is activated in IPAH.11 Article see p 2313 Several transient receptor potential canonical (TRPC) family …

See related article, pages 1323–1330 Serotonin (5-HT, 5-hydroxytryptamine) has long been recognized as one of the most potent naturally occurring pulmonary vasoconstrictors.1 It was first implicated in the pathogenesis of pulmonary arterial hypertension (PAH) after an outbreak of the disease in Switzerland in the 1960’s among patients taking aminorex fumarate, an appetite suppressant that inhibits serotonin uptake by platelets.2 Since that time further outbreaks of PAH have been identified in Europe and the USA associated with the use of fenfluramine-derivate anorexigens,3–5 eventually leading to their withdrawal from the world market in 1997. Although this was, at least in retrospect, a predictable tragedy, it has ironically opened avenues of research into the biology of serotonin signaling in PAH. As fenfluramine-derivatives are substrates for the serotonin transporter (5-HTT, SERT) proteins,6 this suggests that abnormal SERT expression or functional activity could play a role in the pathogenesis of PAH. There is now a body of evidence supporting this hypothesis that provides hope for the development of effective therapeutic strategies targeting specific components of this signaling pathway in patients with these diseases. Most of the serotonin produced in the body is secreted by enterochromaffin cells of the intestine into the portal circulation where it is partially metabolized by the liver. However, levels of free circulating serotonin are maintained in the low nanomolar range through energy-dependent SERT-mediated transport into platelets. This led some researchers to hypothesize that fenfluramines might cause PAH by increasing free plasma levels of serotonin. However, this hypothesis is inconsistent with the observation that chronic treatment with fenfluramine-derivatives if anything reduces plasma levels of serotonin.6 This suggests that other SERT-related effects promote PAH in susceptible patients. This is supported by the observation that patients with idiopathic PAH have increased frequency of the so called L-type polymorphism …

Drug Therapy for Pulmonary Arterial Hypertension: What's on the Menu Today?

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

25-Year-Old Woman With Increasing Abdominal Girth and Shortness of Breath

The Registry of the International Society for Heart and Lung Transplantation: Thirty-third Adult Lung and Heart–Lung Transplant Report—2016; Focus Theme: Primary Diagnostic Indications for Transplant

Pulmonary hypertension is caused by a heterogenous group of disorders with diverse pathophysiological mechanisms, with ultimate structural changes in the pulmonary vascular bed. Platelet activation plays an important role in the development of pulmonary arterial hypertension, while it is unknown whether it contributes to pathogenesis in other conditions. We aimed to investigate platelet activation in different causes of pulmonary hypertension by means of mean platelet volume measurement. A total of 67 patients with different causes of pulmonary hypertension, and 31 controls, were retrospectively reviewed. Patients with pulmonary hypertension were further grouped according to underlying disease, including pulmonary arterial hypertension, pulmonary hypertension due to left ventricular failure, and pulmonary hypertension due to chronic obstructive pulmonary disorder. All patients and controls past medical data, admission echocardiograms and complete blood counts were reviewed. Patients with pulmonary hypertension had higher mean platelet volume levels compared to healthy controls (8.77 ± 1.18 vs 7.89 ± 0.53; p < 0.001), and statistical significance was still present when pulmonary arterial hypertension patients were not included in the pulmonary hypertension group (8.59 ± 1.23 vs 7.89 ± 0.53; p < 0.001). Among patients with pulmonary hypertension, the pulmonary arterial hypertension group and the pulmonary hypertension due to left ventricular failure group had higher mean platelet volumes compared to healthy controls. Mean platelet volume did not correlate with pulmonary artery pressure. Our results indicate that mean platelet volume is not only elevated in pulmonary arterial hypertension, but also due to other causes of pulmonary hypertension.

To be EndMT or not to be, that is the question in pulmonary hypertension.

The Early Diagnosis of Pulmonary Arterial Hypertension: Can We Do Better?

<i>Circulation: Heart Failure</i> Editors’ Picks

INTRODUCTION: The majority of cases of pulmonary hypertension (PH) in pregnant women belong to clinical group 1 (pulmonary arterial hypertension (PAH)) and group 2 (pulmonary hypertension associated with left heart disease (venous)). OBJECTIVES: Identification of risk factors (RF) for adverse peripartum period (PPP) in patients with PAH and pulmonary venous hypertension. MATERIALS AND METHODS: A single-center retrospective cohort study including 242 patients with PH in groups 1 and 2 was performed. All women in the prenatal period had systolic pulmonary artery pressure (sPAP) of 35 mmHg or more, had PH of group 1 (n = 142) or PH of group 2 (n = 100). There were 5 criteria for the adverse PPP outcomes: maternal mortality, premature delivery, heart failure (HF) decompensation, deterioration of hypoxemia and ICU stay for more than 48 hours. Logistic regression and ROC analysis were used. RESULTS: In the entire cohort of 242 women, the presence of Group 1 PH was an independent RF for maternal mortality (odds ratio (OR) = 5.91, p = 0.042), HF decompensation (OR = 3.48, p = 0.008), and hypoxemia deterioration (OR = 10.12, p &lt; 0.001). sPAP was the most significant RF of the adverse course for patients with Group 1 PH: for HF decompensation, OR = 1.03, p = 0.004; for hypoxemia deterioration, OR = 1.2, p &lt; 0.001; for long ICU stay, OR = 1.1, p &lt; 0.001. For women with Group 2 PH, a significant RF in HF decompensation was left ventricular ejection fraction OR = 0.87, p = 0.046 and functional class, OR = 5.89, p = 0.026. All cases of maternal mortality (n = 4; 2.8 %) occurred in patients with Group 1 PH. CONCLUSIONS: Pregnant women with Group 1 PH are characterized by a more complicated course in the PPP compared to those with Group 2 PH.