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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CONCLUSIONS: PH-NF1 is characterized by a female predominance, a low DLCO and severe functional and hemodynamic impairment. Despite a potential benefit of PAH treatment, prognosis remains poor, and double-lung transplantation is an option for eligible patients. PMID: 32437637 [PubMed - as supplied by publisher]
Source: American Journal of Respiratory and Critical Care Medicine - Category: Respiratory Medicine Authors: Tags: Am J Respir Crit Care Med Source Type: research
The age of patients with pulmonary arterial hypertension (PAH) has increased, with registries now reporting mean ages of 50-65 years old. Limited data exist on age-related differences in hemodynamic and functional assessments in PAH.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Source Type: research
Introduction Sarcoidosis-associated pulmonary hypertension (SAPH) is associated with reduced survival in single-centre studies. The international Registry for SAPH (ReSAPH) with long-term follow-up was established to enrich our knowledge of this complication of sarcoidosis. This analysis aims to elucidate factors associated with reduced transplant-free survival in SAPH patients. Methods ReSAPH contains prospectively collected outcomes of SAPH patients since the time of registry enrolment. Information analysed includes right heart catheterisation data, pulmonary function testing, chest radiography, Scadding stage and 6-min...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary vascular disease Original Articles: Pulmonary hypertension Source Type: research
Conclusion PAH due to TBX4 mutations may occur with or without skeletal abnormalities across a broad age range from birth to late adulthood. PAH is usually severe and associated with bronchial and parenchymal abnormalities.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Genetics, Pulmonary vascular disease Original Articles: Pulmonary hypertension and genetics Source Type: research
An 11-year-old girl presented with pulmonary hypertension (PH), World Health Organization (WHO) functional class (FC) III associated with sickle cell disease (SCD) and history of a congenital diaphragmatic hernia (CDH) repair after birth. Her past medical history was significant for multiple episodes of pneumonia and pain crises. At age 7 years, her mean pulmonary artery pressure (mPAP) was 32 mmHg and pulmonary vascular resistance index (PVRi) was 3.6 Wood units x m2 by right heart cardiac catheterization (RHC) at another institute.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: Case Anecdotes, Comments and Opinions Source Type: research
Background: Screening and early diagnosis of pulmonary hypertension (PH) is critical for managing progression and preventing associated mortality, there are no tools for this purpose. We developed and validated an artificial intelligence (AI) algorithm for predicting PH using electrocardiography (ECG).Methods: This historical cohort study included data from consecutive patients from two hospitals. The patients in one hospital were divided into derivation (56,670 ECGs from 24,202 patients) and internal validation (3,174 ECGs from 3,174 patients) dataset, while the patients in the other hospital were included in only an exte...
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: Original Clinical Science Source Type: research
Pulmonary endarterectomy (PEA) is a curative surgical option for patients with chronic thromboembolic pulmonary hypertension (CTEPH), a disease resulting from chronic pulmonary thromboembolism. The role of sarcopenia (i.e. low skeletal muscle mass) has been shown to be associated with adverse outcomes in surgical populations, but its significance with PEA remains unknown. We sought to evaluate the association of sarcopenia with measures of CTEPH severity and post-operative hospital outcomes.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (57) Source Type: research
Pulmonary endarterectomy (PEA) is curative for suitable chronic thromboembolic pulmonary hypertension (CTEPH) patients. Careful anticoagulation prevents postoperative bleeding and thromboembolic recurrence. Heparinization is often guided by aPTT but it can be influenced by Factor VIII - a coagulation factor elevated in CTEPH. Anti-Xa-guided anticoagulation is less affected by other factors. We investigated the correlation between aPTT and Anti-Xa after PEA and the impact of coagulation factors.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (56) Source Type: research
Pulmonary artery sarcoma (PAS) is an exceedingly rare disease with historically poor survival. PAS is often mistaken for chronic thromboembolic pulmonary hypertension (CTEPH) owing to similarities in clinical presentation. Controversy exists regarding need to definitively differentiate PAS from CTEPH prior to surgical therapy. Furthermore lung resection would seem inferior palliation to tumor endarterectomy in a predominantly bilateral disease dominated by hemodynamic compromise.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (55) Source Type: research
The REVEAL risk score (RRS) predicts 1-year survival in patients with pulmonary arterial hypertension; it also improved with riociguat in patients with inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) in the Phase III CHEST-1 study, and predicted survival and clinical worsening-free survival (CWFS) in the long-term extension CHEST-2. RRS 2.0, an updated RRS, was developed based on modified point values, cut-offs, and variables. This post hoc analysis aimed to validate RRS 2.0 in the CHEST database (a population not derived from REVEAL), as done previously, and assess if the tool disc...
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (54) Source Type: research
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