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...
Conclusions: In patients with FIP, PVR is a significant contributor of 6MWD, independently from the extent of fibrosis on HRCT. These results strengthen both the rationale to use 6MWD as endpoint in FIP and to target APH with specific therapies.Respiration
ConclusionsPatients with CS have similar post-transplant hemodynamics as patients without CS, without evidence of right ventricular dysfunction or pulmonary hypertension. Neither significant rejection nor recurrence of sarcoid in the allograft was observed in this cohort of patients with CS. Survival is similar between patients with CS and those without CS. Heart transplant is a viable strategy in selected patients with CS with excellent outcomes.RésuméIntroductionLa transplantation cardiaque orthotopique (TCO) est de plus en plus utilisée lors d’insuffisance cardiaque terminale liée &agra...
Repair of Hemi-Truncus with "Irreversible" Pulmonary Hypertension. Ann Thorac Surg. 2018 Jul 13;: Authors: Sha JM, Cao Y, Xu SS Abstract Anomalous origin of the pulmonary artery from the ascending aorta (AOPA) can lead to congestive heart failure in infancy and with advancing age many patients will develop severe pulmonary hypertension. Surgical intervention had high mortality/morbidity risk if this happens. Strategies to manage these patients seem only limited to heart-lung transplantation or lung transplantation. Here, we successfully carried out surgical intervention to in an adult patien...
Despite advances in targeted medical therapy, pulmonary arterial hypertension (PAH) remains a fatal disease because of progressive right ventricular dysfunction. For patients who are refractory to medical therapy, heart-lung and lung transplantation are important treatment options. Because of longer waiting time, surgical interventions including extracorporeal lung support and atrial septostomy can be used in PAH patients bridging to transplantation.
We examined safety and long-term outcomes of intravenous treprostinil administered via the implantable LENUS Pro ® pump in patients with severe pulmonary hypertension (PH).
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but severe complication of acute pulmonary embolism leading to right heart failure and premature mortality. Its cumulative incidence ranges from 0.1 –9.1% within the first 2 years after symptomatic acute pulmonary embolism, although probably underestimated due to the lack of systematic follow-up and screening in clinical routine.1–3
Chronic thromboembolic pulmonary hypertension (CTEPH) results from persistent pulmonary vascular obstructions, presumably due to inflammatory thrombosis. Because estimates of thrombus volume at diagnosis have no predictive value, we investigated the role of the thrombosis marker D-dimer and the inflammation marker C-reactive protein (CRP) for predicting outcomes in CTEPH.
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.
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).
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.