Diabetes Mellitus Associates with Increased Right Ventricular Afterload and Remodeling in Pulmonary Arterial Hypertension.

BACKGROUND: Diabetes mellitus is associated with left ventricular hypertrophy and dysfunction. Parallel studies have also reported associations between diabetes mellitus and right ventricular dysfunction and reduced survival in patients with pulmonary arterial hypertension. However, the impact of diabetes mellitus on the pulmonary vasculature has not been well characterized. We hypothesized that diabetes mellitus and hyperglycemia could specifically influence right ventricular afterload and remodeling in patients with Group I pulmonary arterial hypertension, providing a link to their known susceptibility to right ventricular dysfunction. METHODS: Using an adjusted model for age, sex, pulmonary vascular resistance, and medication use, associations of fasting blood glucose, glycated hemoglobin, and the presence of diabetes mellitus were evaluated with markers of disease severity in 162 patients with pulmonary arterial hypertension. RESULTS: A surrogate measure of increased pulmonary artery stiffness, elevated pulmonary arterial elastance (P = .012), along with reduced log(pulmonary artery capacitance) (P = .006) were significantly associated with the presence of diabetes mellitus in patients with pulmonary arterial hypertension in a fully adjusted model. Similar associations between pulmonary arterial elastance and capacitance were noted with both fasting blood glucose and glycated hemoglobin. Furthermore, right ventricular wall thickness on echocardiography was greater in pulmonary arterial hypertension patients with diabetes, supporting the link between right ventricular remodeling and diabetes. CONCLUSION: Cumulatively, these data demonstrate that an increase in right ventricular afterload, beyond pulmonary vascular resistance alone, may influence right ventricular remodeling and provide a mechanistic link between the susceptibility to right ventricular dysfunction in patients with both diabetes mellitus and pulmonary arterial hypertension.

The deepithelialized skin flap for closure of large myelomeningoceles: a common plastic surgery technique for a novel neurosurgery application.

PURPOSE: The study aims to demonstrate proof of principle of a common plastic surgery technique of deepithelialization for repair of large myelomeningoceles (MMCs). Whereas repair typically consists of a watertight dural closure followed by a muscle or fasciocutaeneous layer, excess skin, however, need not be discarded and can be deepithelialized to augment or, in selective cases, replace the muscle repair. METHODS: The study was performed through a retrospective chart review of two patients. RESULTS: Step-by-step figures illustrate the technique. Two patients each born with large MMCs measuring 10 × 11 cm and 6 × 9 cm, respectively, were reviewed. Excess skin was deepithelialized to create an additional layer of vascularized tissue over the muscle repair. There were no post-operative complications at 3-month follow-up. CONCLUSIONS: In large MMCs with excess skin, the use of a deepithelialized skin flap can potentially be an alternative or adjunct to a muscle flap to buttress the dural repair.

Obstructive sleep apnea in familial partial lipodystrophy type 2 with atypical skin findings and vascular disease.

INTRODUCTION: A number of metabolic conditions have been associated with Obstructive Sleep Apnea Syndrome (OSAS). Familial partial lipodystrophy type 2 (FPLD2), one of the few rare genetic disorders affecting total body fat redistribution, is one of those conditions. CASE REPORT: In this case report the authors detail OSAS associated with FPLD2 with atypical dermatological and systemic manifestations.