Abhay Vakil

Pretransplant frailty is associated with decreased survival after lung transplantation

BACKGROUND Frailty is a condition of increased vulnerability to adverse health outcomes. Although frailty is an important prognostic factor for many conditions, the effect of frailty on mortality in lung transplantation is unknown. Our objective was to assess the association of frailty with survival after lung transplantation. METHODS We performed a retrospective cohort analysis of all adult lung transplant recipients at our institution between 2002 and 2013. Frailty was assessed using the frailty deficit index, a validated instrument that assesses cumulative deficits for up to 32 impairments and measures the proportion of deficits present (with frailty defined as >0.25). We examined the association between frailty and survival, adjusting for age, sex, and bilateral (vs single) lung transplant using Cox proportional hazard regression models. RESULTS Among 144 lung transplant patients, 102 (71%) completed self-reported questionnaires necessary to assess the frailty deficit index within 1 year before lung transplantation. Frail patients (n = 46) had an increased risk of death, with an adjusted hazard ratio (HR) of 2.24 (95% confidence interval [CI], 1.22-4.19; p = 0.0089). Frailty was not associated with an increased duration of mechanical ventilation (median, 2 vs 2 days; p = 0.26), intensive care unit length of stay (median, 7.5 vs 6 days; p = 0.36) or hospital length of stay after transplantation (median, 14 vs 10.5 days; p = 0.26). CONCLUSIONS Pre-transplant frailty was independently associated with decreased survival after lung transplantation. Pre-transplant frailty may represent an important area for intervention to improve candidate selection and lung transplant outcomes.

Malignant tracheal tumors: a review of current diagnostic and management strategies.

Purpose of review This article reviews the current literature for the purpose of developing a practical approach for the diagnosis and management of primary tracheal tumors. Recent findings Because of nonspecific symptoms, tracheal tumors remain a diagnostic challenge. Currently available management strategies are not being optimally utilized due to lack of physician awareness and knowledge. The use of newer diagnostic modalities has increased diagnostic accuracy resulting in earlier detection in recent years. This review describes currently available diagnostic modalities along with relatively newer ones such as virtual bronchoscopy, anatomic Optical Coherence Tomography, spectroscopic techniques, and endobronchial ultrasonography. We will review and discuss management strategies including surgical options, adjuvant therapies, and interventional pulmonary techniques including their role in palliation. Summary Early detection along with improved surgical and interventional pulmonology techniques has led to a decline in the death rates from tracheal cancer in recent years. However, further studies are required to define the role of chemotherapeutic agents, combination therapies, and novel techniques such as tracheal transplantation, in the management of primary tracheal tumors. More robust evidence-based studies are needed to provide evidence for clinical practice guidelines for the treatment of primary tracheal tumors.

A case of recurrent massive pulmonary embolism in Klippel-Trenaunay-Weber syndrome treated with thrombolytics.

Klippel – Trenaunay – Weber syndrome (KTWS) is a congenital condition characterized by a triad of capillary malformations of the skin, soft tissue and bone hypertrophy resulting in limb enlargement, and abnormalities of arteriovenous and lymphatic systems of the affected limb. In this case, we present a patient with KTWS receiving chronic anticoagulation that had a massive pulmonary embolism and was successfully treated with thrombolytic therapy. The purpose of this case is to educate readers about this uncommon condition and to increase awareness, recognition and timely treatment of its most common complications, namely thrombosis and pulmonary embolism.

Patent foramen ovale in idiopathic pulmonary arterial hypertension: Long-term risk and morbidity.

INTRODUCTION Little is known about the presence of patent foramen ovale in idiopathic pulmonary arterial hypertension. While there is suspected worsening of hypoxemia confounding assessment and management of pulmonary hypertension, as well as possible increased morbidity from paradoxical emboli, there may be theoretical relief of worsening right-sided pressures by the same mechanism of right-to-left shunting. METHODS Retrospective review of consecutive patients diagnosed with idiopathic pulmonary arterial hypertension (WHO Group 1) via right heart catheterization, from 1998 to 2010. All patients also underwent a four chamber transthoracic echocardiogram with agitated saline contrast for the evaluation of patent foramen ovale. Primary clinical data was collected and compared between patients with and without patent foramen ovale along with univariable and multivariable predictors of long term survival. RESULTS One hundred and fifty five patients were included in the study, 42 with patent foramen ovale (27%). Patients with patent foramen ovale were younger at pulmonary arterial hypertension diagnosis and trended towards higher right ventricular systolic pressures on echocardiography and mean pulmonary arterial pressures by right heart catheterization. Predictors of mortality included age, diffusing capacity for carbon monoxide, and severe hypoxemia. Only diffusing capacity and age were predictive of mortality after adjustment for a priori covariables. CONCLUSION Patent foramen ovale is seen in a quarter of patients with idiopathic pulmonary arterial hypertension and associated with increased prevalence of severe hypoxemia but had no effect on long term survival.

Idiopathic pulmonary hemosiderosis presenting in an adult: A case report and review of the literature

Diffuse alveolar hemorrhage (DAH) is characterized by the presence of hemoptysis, anemia, and the presence of diffuse parenchymal infiltrates on imaging studies. Idiopathic pulmonary hemosiderosis (IPH) is an uncommon cause of diffuse alveolar hemorrhage (DAH) and is classically known to present in childhood. Adult-onset IPH is extremely rare. We report the case of a 48-year-old female patient who presented with hemoptysis and acute hypoxic respiratory failure, requiring intubation and mechanical ventilation. Imaging studies showed diffuse bilateral patchy infiltrates. Bronchoalveolar lavage (BAL) confirmed the diagnosis of DAH. Extensive workup including video-assisted thoracoscopic surgical lung biopsy (VATS) failed to reveal any vasculitis, infectious, immunological or connective tissue disorder, as the underlying cause for DAH. The patient was successfully treated with high-dose steroid therapy.

Diffuse cholangiocarcinoma presenting with hepatic failure and extensive portal and mesenteric vein thrombosis

A 64-year-old previously healthy man presented with a 4-week history of vague right upper quadrant abdominal pain. Imaging studies revealed extensive portal, splenic, superior and inferior mesenteric vein thrombosis with mosaic perfusion and wedge-shaped areas of liver perfusion abnormalities. An extensive thrombophilia workup including tests for factor V Leiden, prothrombin G20210A, lupus anticoagulant, paroxysmal nocturnal haemoglobinuria, protein C and S, homocysteine and antinuclear antibody titres were all negative. Other laboratory testing revealed an elevated alkaline phosphatase (340 IU/L). Surgical exploration and catheter-directed thrombolysis were not felt to be feasible given the extensive clot burden. He was started on anticoagulation therapy. Over the next 10 days, he required intensive care unit admission due to progressive hepatic encephalopathy and fulminant liver failure. He continued to decline and eventually died of multiorgan failure. Autopsy revealed extensive, diffuse intrahepatic cholangiocarcinoma that had almost entirely replaced his normal liver parenchyma.

Varicella Pneumonia in a Human Immunodeficiency Virus-Positive Adult

CASE PRESENTATION A46-year-old human immunodeficiency virus positive male presented with a 3-day history of cough with yellowish sputum production, fatigue and progressively worsening dyspnea. He had an exanthematous rash that had started 3 weeks before the development of cough. The patient denied taking his antiretroviral medications for the last 6 months. Skin examination showed polymorphic rash with pustules, vesicles and crusted lesions (Figure 1). Chest radiography showed multiple nodular densities. Chest computed tomography revealed multiple ill-defined macronudules up to 2 cm in size. Halos of ground-glass attenuation were also seen in some nodules (Figure 2). Serologic testing showed a CD4 count of o20 cells/mcL and a viral load of 1,431,623 copies/mL. Bronchoalveolar lavage and cultures failed to reveal any source of infection. The diagnosis of varicella pneumonia was established due to the presence of characteristic skin rash, pulmonary symptoms and the immunocompromised state of the patients. Intravenous acylclovir therapy led to improvement in his clinical and radiologic findings. Follow-up

Metastatic hepatoblastoma: a rare cause of lung mass in adults

Hepatoblastoma is a pediatric malignant tumor of the liver with very few cases reported in adults. There are no case reports on isolated metastatic lung involvement with hepatoblastoma in an adult who had been previously treated for the pediatric form of this disease. We report the case of a 27-year-old asymptomatic man who presented to the hospital after a motor vehicle accident. Imaging studies revealed bilateral lung masses. He had been treated for hepatoblastoma at the age of 10 years. Histopathologic examination of the lung biopsy revealed embryonal subtype of hepatoblastoma. Further imaging studies failed to reveal the presence of any concomitant liver lesions.

A 44-Year-Old Woman With Metabolic Acidosis, High Anion Gap, and Delayed Neurologic Deterioration

A 44-year-old woman was brought to the ED from John F. Kennedy International Airport. The patient was returning with her son from a 3-month visit to Bangladesh. Her journey started with a 4-h flight from Dhaka, Bangladesh to Dubai, United Arab Emirates. She consumed 240 mL of whiskey during the flight. This was followed by a 14-h flight from Dubai to New York. According to the patients son, she did not consume any alcohol during the second flight. The patient was in her usual state of health with normal mentation throughout her journey. Upon landing, she started complaining of shortness of breath. After disembarking, she was witnessed to have seizure-like activity with involuntary passage of urine, following which she collapsed. The patient was intubated by emergency medical services in the field.