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We report a case of temporary Berlin Heart EXCOR® explantation in a pediatric patient with idiopathic dilated cardiomyopathy who suffered an uncontrollable inflow cannulation site infection while on bridge-to-transplantation. Despite failure to thrive and catheter-related infections, once free of the device, the patient was cured of infection using systemic antibiotics and surgical debridement. The patient underwent EXCOR® reimplantation after four months, and is awaiting heart transplantation in stable condition. A life-threatening ventricular assist device-related infection may require device explantation under conditions that may not fulfill conventional explantation criteria despite risks. Temporary explantation can be an effective strategy if isolated systolic dysfunction is managed carefully.
RESUMEN
Objectives: Septal myectomy confers survival benefits on patients with hypertrophic cardiomyopathy. However, its role in the treatment of severe aortic stenosis (sAS) with left ventricular outflow tract obstruction (LVOTO) remains under investigation. Another challenging question in the era of transcatheter aortic valve replacement is who would benefit more from traditional surgical aortic valve replacement (SAVR) with myectomy. Therefore, this study aimed to investigate myectomy cases at our hospital in Japan. Methods: A total of 740 patients who underwent SAVR for sAS between 2012 and 2019 were identified. The demographics and baseline echocardiographic findings were retrospectively compared between patients who underwent concomitant myectomy and those who did not. The myectomy group was further assessed for factors predisposing to LVOTO, operative details, echocardiographic changes, and prognosis. The resected septa were histopathologically analyzed. Results: The myectomy group mostly comprised elderly females with a small hypercontractile heart. Myectomy with SAVR led to statistically significant improvements in concentric left ventricular hypertrophy and LVOTO parameters. Survival was comparable with that reported in previous reports, even in the elderly subset (≥ 75 years). The septa showed mild fibrosis. Conclusions: Myectomy can be safely performed with SAVR for sAS with LVOTO, even in the elderly, and it effectively improves LVOTO. Special attention should be paid to elderly females with relatively more severe AS and a small yet extra-hypertrophic and extra-hypercontractile heart. Such patients warrant comprehensive assessment of LVOTO, and despite its invasiveness, SAVR may be potentially more beneficial by allowing direct observation of LVOTO and ancillary myectomy.
RESUMEN
Rheb (Ras homolog enriched in brain) is a GTPase conserved from yeast to human and belongs to a unique family within the Ras superfamily of GTPases. Rheb plays critical roles in the activation of mTOR, a serine/threonine kinase that is involved in the activation of protein synthesis and growth. mTOR forms two distinct complexes, mTORC1 and mTORC2. While mTORC1 is implicated in the regulation of cell growth, proliferation, and cell size in response to amino acids and growth factors, mTORC2 is involved in actin organization. However, the mechanism of activation is not fully understood. Therefore, studies to elucidate the Rheb-mTOR signaling pathway are of great importance. Here we describe methods to characterize this pathway and to evaluate constitutive active mutants of Rheb and mTOR that we recently identified. Constitutive activity of the mutants can be demonstrated by the phosphorylation of ribosomal protein S6 kinase 1 (S6K1) and eIF4E-binding protein 1 (4E-BP1) both in vivo and in vitro after starving cells for amino acids and growth factors. In addition, formation and activity of mTORC1 and mTORC2 can be measured by immunoprecipitating these complexes and carrying out in vitro kinase assays. We also describe a protocol for rapamycin treatment, which directly inhibits mTOR and can be used to investigate the mTOR signaling pathway in cell growth, cell size, etc.