Poor Subpleural Perfusion Predicts Failure After Balloon Pulmonary Angioplasty for Nonoperable Chronic Thromboembolic Pulmonary Hypertension.

BACKGROUND: Poor subpleural perfusion (PSP) in the capillary phase of pulmonary angiography predicts worse outcomes following pulmonary endarterectomy in operable chronic thromboembolic pulmonary hypertension (CTEPH). Balloon pulmonary angioplasty (BPA) has emerged as a treatment for nonoperable CTEPH. The goal of the present article was to assess the association between PSP and BPA failure. METHODS: Subpleural perfusion was classified as poor (defined as subpleural spaces either not perfused or minimally perfused in all segments) or normal. We retrospectively reviewed PSP and hemodynamic variables of 101 consecutive patients who underwent BPA from February 2014 to August 2016. The total cross-sectional area of bronchial arteries was also measured by using CT scanning. Patients were categorized according to hemodynamic results after the last BPA: a failure group (defined as mean pulmonary arterial pressure > 30 mm Hg and a decrease in pulmonary vascular resistance < 30% [n = 15]) or a success group (n = 86). RESULTS: Although baseline hemodynamic variables were similar between the two groups, PSP was observed in 46.7% of patients in the failure group vs 13.9% in the success group (P = .003). Multivariate analysis revealed that PSP was the only predictor of BPA failure (OR, 4.02 [95% CI, 1.17-13.89]; P = .028). Patients with PSP exhibited poorly developed bronchial arteries compared with patients with normal perfusion (7.0 [5.8-9.6] mm2 vs 8.7 [6.9-11.3] mm2; P = .032). CONCLUSIONS: PSP in the capillary phase, suggesting the presence of small vessel disease with diffuse distal thrombosis, is a predictor of BPA failure. PSP was also associated with less developed bronchial arteries, which suggests a key role of bronchial-pulmonary anastomoses in maintaining the pulmonary capillary bed open downstream of the pulmonary arterial obstruction. PSP affected approximately 15% of patients with nonoperable CTEPH who underwent BPA.

Acquired von Willebrand syndrome in a case of polycythemia vera resulting in recurrent and massive bleeding events in the pleural and abdominal cavity.

A 52-year-old woman was admitted to the hospital three times in a span of 5 years in hypovolemic shock because of spontaneous and massive bleeding in the pleural and abdominal cavity. Blood tests revealed a high number of blood cells, and bone marrow smears showed trilineage myeloproliferation. Serum erythropoietin level was decreased. Analysis revealed a V617F mutation in the JAK2 protein. Her activated partial thromboplastin time was slightly prolonged, the ratio between von Willebrand factor (vWF) propeptide and vWF antigen was in the normal range, but the ratio between vWF and ristocetin cofactor was decreased dramatically. Further investigation revealed the absence of large and intermediate vWF-multimers. She was diagnosed with polycythemia vera with acquired von Willebrand syndrome. The bleeding was stopped using a transfusion of freshly thawed plasma and cryoprecipitate.

Congenital aberrant vessel as a source of massive hemothorax in blunt trauma.

A congenital aberrant vessel in the pleural cavity has rarely been described in the literature. It has been known to be a source of bleeding in spontaneous hemopneumothorax. As a result of the lack of pleural adhesions around the vessel, a congenital aberrant vessel is prone to deceleration injury. This is a case of massive hemothorax caused by a ruptured aberrant congenital vessel in a blunt trauma victim who sustained a two meter fall.