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Idiopathic pulmonary fibrosis (IPF) is a lethal lung disease of unknown etiology. The development of pulmonary hypertension (PH) is considered the single most significant predictor of mortality in patients with chronic lung diseases. The processes that govern the progression and development of fibroproliferative and vascular lesions in IPF are not fully understood. Using human lung explant samples from patients with IPF with or without a diagnosis of PH as well as normal control tissue, we report reduced BMPR2 expression in patients with IPF or IPF PH. These changes were consistent with dampened P-SMAD 1/5/8 and elevated P-SMAD 2/3, demonstrating reduced BMPR2 signaling and elevated TGF- activity in IPF. In the bleomycin (BLM) model of lung fibrosis and PH, we also report decreased BMPR2 expression compared with control animals that correlated with vascular remodeling and PH. We show that genetic abrogation or pharmacological inhibition of interleukin-6 leads to diminished markers of fibrosis and PH consistent with elevated levels of BMPR2 and reduced levels of a collection of microRNAs (miRs) that are able to degrade BMPR2. We also demonstrate that isolated bone marrow-derived macrophages from BLM-exposed mice show reduced BMPR2 levels upon exposure with IL6 or the IL6 IL6R complex that are consistent with immunohistochemistry showing reduced BMPR2 in CD206 expressing macrophages from lung sections from IPF and IPF PH patients. In conclusion, our data suggest that depletion of BMPR2 mediated by a collection of miRs induced by IL6 and subsequent STAT3 phosphorylation as a novel mechanism participating to fibroproliferative and vascular injuries in IPF. [ABSTRACT FROM AUTHOR]

Treatment of patients with INTERMACS class I heart failure can be very challenging, and temporary long-term device support may be needed. In this article, we review the currently available temporary support devices in order to support these severely ill patients with decompensated heart failure. Strategies of using a temporary assist as a bridge to long-term device support are also discussed. [ABSTRACT FROM AUTHOR]

Idiopathic pulmonary fibrosis (IPF) is a lethal lung disease of unknown etiology. The development of pulmonary hypertension (PH) is considered the single most significant predictor of mortality in patients with chronic lung diseases. The processes that govern the progression and development of fibroproliferative and vascular lesions in IPF are not fully understood. Using human lung explant samples from patients with IPF with or without a diagnosis of PH as well as normal control tissue, we report reduced BMPR2 expression in patients with IPF or IPF+PH. These changes were consistent with dampened P-SMAD 1/5/8 and elevated P-SMAD 2/3, demonstrating reduced BMPR2 signaling and elevated TGF-β activity in IPF. In the bleomycin (BLM) model of lung fibrosis and PH, we also report decreased BMPR2 expression compared with control animals that correlated with vascular remodeling and PH. We show that genetic abrogation or pharmacological inhibition of interleukin-6 leads to diminished markers of fibrosis and PH consistent with elevated levels of BMPR2 and reduced levels of a collection of microRNAs (miRs) that are able to degrade BMPR2. We also demonstrate that isolated bone marrow-derived macrophages from BLM-exposed mice show reduced BMPR2 levels upon exposure with IL6 or the IL6+IL6R complex that are consistent with immunohistochemistry showing reduced BMPR2 in CD206 expressing macrophages from lung sections from IPF and IPF+PH patients. In conclusion, our data suggest that depletion of BMPR2 mediated by a collection of miRs induced by IL6 and subsequent STAT3 phosphorylation as a novel mechanism participating to fibroproliferative and vascular injuries in IPF., (Copyright © 2016 the American Physiological Society.)

Primary cardiac sarcomas are rare and carry a grave prognosis. Improved survival requires a complete margin negative resection of the tumor. These surgical resections are often large and complex, requiring extensive reconstructive procedures. The appropriate material for cardiac reconstruction is not known. We have used glutaraldehyde-fixed bovine pericardium in our early series but have recently employed the MatriStem(®) Surgical Matrix PSMX membrane (ACell(®), Inc.; Columbia, MD), a unique proprietary urinary bladder matrix derived from porcine urinary bladder with the potential for viability and tissue ingrowth. In our study of six patients at this institution, all six underwent successful surgical resection and repair with the MatriStem acellular porcine urinary bladder membrane (ACell). The postoperative course was uncomplicated in all patients, and they are still alive at this time. An aggressive surgical approach to cardiac tumors can possibly lead to complete resection but often requires reconstruction of the cardiac tissue with a membrane. We were able to achieve acceptable results in our cardiac reconstruction by using the ACell extracellular matrix to reconstruct the defect following tumor resection. Longer-term follow-up in these patients, including imaging studies, will be necessary to demonstrate the durability and integrity of the reconstruction.

Hemangioma of the heart presenting as a primary cardiac tumor is extremely rare, accounting for approximately 2% of all primary resected heart tumors. Only a few cases of cardiac hemangiomas have been reported to arise from the left atrial wall. In this case report we share our experience in the diagnosis and surgical resection of a large (9 × 7 cm) left atrial hemangioma and reconstruction of the heart using porcine urinary bladder membrane.

Unilateral agenesis of the pulmonary artery (UAPA) is a rare congenital anomaly. This report describes a 52-year-old female who gave a long history of chronic, recurrent, left-sided pulmonary infections related to UAPA. For many years, she was managed medically but the infection continued to recur. She eventually underwent left pneumonectomy and made a good recovery.

Treatment options for aortic arch disease in high-risk patients includes supra-aortic debranching and thoracic endovascular aortic repair (TEVAR). Acute ascending aortic dissection is a concerning complication of this approach and has been reported to occur in a retrograde fashion. We report a case of a 60-year-old gentleman who had undergone thoracic endovascular aortic repair with debranching and presented 31 months later with acute isolated ascending aortic dissection. The patient underwent successful total replacement of the ascending aorta with a 30-mm gelwave Valsalva(™) graft using cardiopulmonary bypass.