Intraluminal Pulmonary Vein Stenosis in Children: A "New" Lesion.

Pulmonary vein stenosis (PVS) is a rare disorder that leads to progressive narrowing of the extrapulmonary veins. PVS has been reported in both children and adults and in its worse iteration leads to pulmonary hypertension, right ventricular failure, and death. Multiple etiologies of PVS have been described in children and adults. This review will focus on intraluminal PVS in children. Intraluminal PVS has an estimated incidence ranging from 0.0017% to 0.03%. It is associated with conditions such as prematurity, bronchopulmonary dysplasia, necrotizing enterocolitis, Smith-Lemli-Opitz syndrome, and Down syndrome. Cardiac catheterization and pulmonary vein angiography are the gold standard for diagnosis and anatomic delineation. Other imaging modalities including magnetic resonance imaging, chest tomography, and transesophageal echocardiography are increasingly being used. Mortality of PVS in children is approximately 50%. Predictors of mortality include involvement of ≥3 pulmonary veins, bilateral pulmonary vein involvement, onset of PVS in infancy, elevated pulmonary artery pressure or systolic pulmonary artery-to-aortic pressure ratio, right ventricular dysfunction, restenosis after surgery, distal/upstream disease, and disease progression to previously uninvolved pulmonary veins. Treatment includes catheter-based pulmonary vein dilations with or without stenting, surgical interventions, medical therapy, and in some instances, lung transplantation. Cardiac catheterization for PVS involves a comprehensive hemodynamic and anatomic assessment of the pulmonary veins as well as therapeutic transcatheter interventions. Several surgical strategies have been used. Sutureless repair is currently most commonly used, but patch venoplasty, endarterectomy, ostial resection, and reimplantation are used in select circumstances as well. Medical therapies such as imatinib mesylate and bevacizumab are increasingly being used in an effort to suppress the myofibroblastic proliferation seen in PVS patients. Lung transplantation has been used as an alternative treatment strategy for end-stage, refractory PVS. Nonetheless, despite the different innovative approaches used, morbidity and mortality remain high. At present, the preferred treatment strategy is frequent reassessment of disease progression to guide use of catheter-based and surgical interventions in conjunction with medical therapy.

Dabigatran bleed risk with closed head injuries: are we prepared?

OBJECT: The direct thrombin inhibitor dabigatran has recently been approved in the US as an alternative to warfarin. The lack of guidelines, protocols, and an established specific antidote to reverse the anticoagulation effect of dabigatran potentially increases the rates of morbidity and mortality in patients with closed head injury (CHI). Confronted with this new problem, the authors reviewed their initial clinical experience. METHODS: The authors retrospectively reviewed all cases of adult patients (age ≥ 18 years) who sustained CHI secondary to ground-level falls and who presented to the authors' provisional regional Level I trauma center between February 2011 and May 2011. The authors divided these patients into 3 groups based on anticoagulant therapy: dabigatran, warfarin, and no anticoagulants. RESULTS: Between February 2011 and May 2011, CHIs from ground-level falls were sustained by 5 patients while on dabigatran, by 15 patients on warfarin, and by 25 patients who were not on anticoagulants. The treatment of the patients on dabigatran at the authors' institution had great diversity. Repeat CT scans obtained during reversal showed 4 of 5 patients with new or expanded hemorrhages in the dabigatran group, whereas the warfarin group had 3 of 15 (p = 0.03). The overall mortality rate for patients sustaining CHI on dabigatran was 2 (40%) of 5, whereas that of the warfarin group was 0 (0%) of 15 (p = 0.05). CONCLUSIONS: It is critical for physicians involved in the care of patients with CHI on dabigatran to be aware of an elevated mortality rate if no treatment protocol or guideline is in place. The authors will soon implement a reversal management protocol for patients with CHI on dabigatran at their institution in an attempt to improve efficacy and safety in their treatment approach.