A 33-year-old male with severe hereditary pulmonary arterial hypertension had a confirmed diagnosis of occlusive venopathy and microvasculopathy. He remained stable for three and a half years on oral sildenafil, 75 mg t.i.d. (six-minute walked distance of 375 m vs 105 m at baseline), but required addition of bosentan (125 mg b.i.d.), subsequently. Despite the fatal outcome at five years post-diagnosis, the observations suggest a potential usefulness of vasodilators as a bridge for lung transplant in selected cases with significant venous/capillary involvement. The occurrence of veno-occlusive and capillary lesions in the familial form of pulmonary arterial hypertension reinforces the difficulties with the current classification of the disease.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1590/s0066-782x2011000900019 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Coil embolization of anomalous systemic artery to the left lower lobe in an asymptomatic adult: a case report.
J Cardiothorac Surg
January 2025
Department of Radiology, China-Japan Union Hospital of Jilin University, 126 Xiantai Street, Changchun, 130000, Jilin, China.
Background: Anomalous systemic artery to the left lower lobe (ASALLL) is a rare congenital anomaly. The primary symptoms include hemoptysis and lung infection, though some patients may remain asymptomatic. Currently, there is no consensus on the indications for treatment or the optimal choice of therapy for this condition.
View Article and Find Full Text PDFSuccessful thoracoscopic-assisted resection of a functional paraganglioma in the mediastinum with extracorporeal circulation: a case report.
J Cardiothorac Surg
January 2025
Department of Thoracic Surgery, The Second Xiangya Hospital of Central South University, Changsha, Hunan, P. R. China.
Background: Paragangliomas are rare neoplasms arising from extra-adrenal chromaffin cells, with mediastinal paragangliomas representing an exceptionally rare subset. This report details the surgical management of a complex mediastinal paraganglioma case, presenting with refractory hypertension and invasion of critical surrounding structures. A comprehensive review of the current literature is included to underscore existing cases, enhance clinical awareness, and share our insights and experience in the diagnosis and treatment of this challenging condition.
View Article and Find Full Text PDFThe first case of esophageal mediastinal fistula after immunotherapy for non-small cell lung cancer: case report and literature review.
J Cardiothorac Surg
January 2025
Department of Traditional Chinese Medicine, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
Background: Malignant esophageal mediastinal fistula is a severe complication that occurs in both the advanced stages of esophageal cancer and after radiotherapy for esophageal cancer. Esophageal mediastinal fistula is very susceptible to complications such as mediastinitis and mediastinal abscess, resulting in a significantly elevated mortality rate for patients. We reported a rare case of esophageal mediastinal fistula after immunotherapy for non-small cell lung cancer (NSCLC).
View Article and Find Full Text PDFAnomalous left pulmonary artery: case reports exploring anatomic variants.
J Cardiothorac Surg
January 2025
Department of Pediatric Cardiothoracic Surgery, Akron Children's Hospital, Akron, OH, USA.
Background: Pulmonary artery sling (PAS) is a rare congenital anomaly where the left pulmonary artery (LPA) branches from the right pulmonary artery, compressing the trachea and esophagus and frequently leading to respiratory distress in infants. Surgical intervention, such as LPA reimplantation or translocation, is crucial to relieve airway compression and restore normal pulmonary function.
Case Presentation: This report highlights varied LPA anatomies, including a unique case of an anomalous LPA without true sling formation but causing tracheal compression, alongside two typical PAS cases.
Massive pulmonary thromboembolism in a pediatric patient with eosinophilic granulomatosis with polyangiitis: a case-based review emphasizing management.
Pediatr Rheumatol Online J
January 2025
Department of Pediatric Rheumatology, Faculty of Medicine, Gazi University, Ankara, Besevler, 06500, Turkey.
Background: Pediatric patients with Eosinophilic Granulomatosis with Polyangiitis (EGPA) are at an increased risk of arterial and venous thromboembolism (AVTE). Although the exact mechanisms underlying AVTE remain unclear, eosinophils play a pivotal role in AVTE.
Main Body: Current guidelines lack evidence-based recommendations, particularly concerning anticoagulant and antiplatelet treatments for this condition.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!