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Unique treatment method offers hope to child with severe pulmonary hypertension

When Danny Martinez came to Boston from Florida he was very sick. At the time the young boy was suffering from severe pulmonary hypertension, a type of high blood pressure that affects the arteries in the lungs and the right side of the heart. Because Danny’s pulmonary hypertension was so serious doctors in his home state were unsure of how to save him. Along with his parents they arranged it so he could be transferred to Boston Children’s Hospital where he could receive care from the Pulmonary Hypertension Program. As one of the leading pulmonary hypertension programs in the world, Danny’s new team included cardiologists, radiologists and pulmonologists, each with a specific skill for helping children with pulmonary hypertension. After careful examination it became evident that Danny would need a lung transplant to live. However, at the time he was too critically ill to survive until the operation. To keep him alive until the surgery the Pulmonary Hypertension team devised an innovative treatment plan that would act as a bridge to lung transplant. A new approach to treatment To support his lungs, Danny was fitted with a Quadrox membrane oxygenator, a special lung-assist device that adds oxygen to the blood in the same way a healthy pair of lungs would. This device is almost always used as part of an ECMO machine, a special therapy doctors sometimes use to support a critically ill child.  But Danny only needed lung assistance, not full ECMO, so his doc...
Source: Thrive, Children's Hospital Boston - Category: Pediatrics Authors: Tags: All posts Source Type: news

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The pathobiology of chronic thromboembolic pulmonary hypertension (CTEPH) is poorly understood. Metabolic dysregulation is prominent in idiopathic pulmonary arterial hypertension (IPAH). Using an “omics” approach, we sought to determine the metabolic fingerprint of CTEPH patients compared to IPAH and healthy controls.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Source Type: research
We hypothesized that dynamic measures of pulmonary arterial compliance (Cpa), and elastance (Epa), RV elastance (Ees), and RV-PA coupling would improve prediction of post-op mean PA pressure (PAP), Length of Stay (LOS), ICU duration (ICUd) and need for inotropes (NFI) compared to prediction based solely on pre-op PAP, CO and PVR in patients with chronic thromboembolic pulmonary hypertension (CTEPH) undergoing pulmonary thromboendaterectomy (PTE).
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Source Type: research
Exercise right heart catheterization (RHC), currently used to identify occult pulmonary hypertension (PH), may have additional applications. An elevated total pulmonary resistance (TPR), the change in mean pulmonary arterial pressure (mPAP) divided by the change in cardiac output during exercise, has been associated with worse clinical outcomes. We hypothesize that the combination of the TPR and the PCWP during exercise ( ∆TPRex) reflects pulmonary arterial disease and is a marker of outcomes.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Source Type: research
LVAD therapy in patients with pulmonary hypertension (PH) has shown improvements in pulmonary vascular resistance (PVR) but benefits of this strategy of mechanical unloading pre heart transplantation (HTx) remains uncertain. Here, we determine if patients with PH who are bridge to transplant (BTT) with an LVAD have improved outcomes after HTx.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Source Type: research
Right ventricular (RV) dysfunction in patients with pulmonary hypertension due to chronic lung disease (Group 3 PH) is not well described. We compared RV function in Group 3 and Group 1 PH patients, and investigated the correlates of RV function in Group 3 PH patients.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Source Type: research
Chronic thromboembolic pulmonary hypertension (CTEPH), is a progressive condition characterized by persistent occlusion of the pulmonary arteries by organized thrombus and a pulmonary arteriopahty. CTEPH is potentially curable with pulmonary thrombo-endarterectomy (PTE). The pathophysiological mechanisms that lead to the development of CTEPH and progressive arteriopathy has not been fully elucidated. We hypothesize that Endothelin-1(ET1), a potent endogenous vasoconstrictor and smooth muscle mitogen may contribute to the development of CTEPH.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Source Type: research
Endothelial to Mesenchymal Transition (EndMT) is a complex biological process in which endothelial cells transdifferentiate to collagen producing mesenchymal cells. The phenomenon of EndMT has been associated with development of vascular remodeling in monocrotaline rat model of pulmonary arterial hypertension (PAH). However, data on its role in pulmonary vascular remodeling in heart failure (HF) leading to pulmonary hypertension (PH) is lacking. The presence of End MT in lungs in association with pulmonary vascular remodeling could indicate its role in development of PH.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Source Type: research
Chronic thrombo-embolic pulmonary hypertension (CTEPH) results in right ventricular (RV) dysfunction, primary cause of death in CTEPH. Previous studies described a continuum from an adaptive heart with modified metabolism, angiogenesis and structure, to a maladaptive heart with RV failure. We sought to describe relationships between histological features of RV remodeling and functional and metabolic imaging of the right ventricle in CTEPH.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Source Type: research
We present a case of vasculitis-associated PH initially diagnosed as CTEPH.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Source Type: research
Right ventricular (RV) is a key driver of outcome in heart failure and pulmonary vascular disorders. Although the RV fails in response to an increased afterload, it has been shown to be affected in patients with left heart diseases in the absence of pulmonary hypertension (PH). Therefore, it is recommended to consider the RV and the pulmonary artery (PA) as a single unit, defined physiologically by RV-PA coupling. The aim of this study is to assess whether non-invasive RV-PA coupling may predict outcome in PAH and in LHD.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Source Type: research
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