Two cases of cardiomyopathy associated with phaeochromocytoma successfully managed with veno-arterial extracorporeal membrane oxygenation (V-A ECMO).

Summary: Phaeochromocytoma, a rare neuroendocrine tumour of chromaffin cell origin, is characterised by catecholamine excess. Clinical presentation ranges from asymptomatic disease to life-threatening multiorgan dysfunction. Catecholamine-induced cardiomyopathy is a dreaded complication with high lethality. While there is lack of evidence-based guidelines for use of veno-arterial extracorporeal membrane oxygenation (V-A ECMO) in the management of this condition, limited to case reports and small case series, V-A ECMO has been reported as 'bridge to recovery' therapy, providing circulatory support in the initial period of stabilisation prior to surgery. We report on two patients presenting with catecholamine-induced cardiomyopathy and circulatory collapse who were successfully treated with V-A ECMO for 5 and 6 days, respectively, providing initial haemodynamic support. After stabilisation and introduction of alpha-blockade, both cases had favourable outcomes, with successful laparoscopic adrenalectomies on days 62 and 83 of admission, respectively. Our case reports provide further support for the use of V-A ECMO in the treatment of such gravely ill patients. Learning points: Phaeochromocytoma should be considered in the diagnosis of patients presenting with acute cardiomyopathy. Management of catecholamine-induced cardiomyopathy is complex and requires multidisciplinary specialist input. Pre-operative management of phaeochromocytoma involves alpha-blockade; however, haemodynamic instability in the setting of cardiogenic shock can preclude alpha-blockade use. Veno-arterial extracorporeal membrane oxygenation is a life-saving intervention which may be considered in cases of acute catecholamine-induced cardiomyopathy and cardiogenic shock in order to provide the required haemodynamic support in the initial phase of treatment, enabling the administration of traditional pharmacological agents, including alpha-blockade.

Long-Term Health-Related Quality of Life After Venovenous Extracorporeal Membrane Oxygenation.

This study examined the long-term health-related quality of life in adult patients treated with venovenous extracorporeal membrane oxygenation (V-V ECMO) for severe acute respiratory failure in Ireland. A retrospective, cross-sectional survey was conducted to elicit self-reported quality of life in V-V ECMO survivors who were discharged from the intensive care unit for ≥6 months. Twenty-nine patients with respiratory failure were treated with V-V ECMO from 2009 to 2013. Of the 19 (66%) patients who survived to hospital discharge, 13 participated in the study. The mean age was 44 ± 11 years, and seven were male. At a median follow-up of 36 (14-39) months, study participants reported decreased indices of physical health compared with age- and sex-matched general population in Ireland while their mental health was similar to age- and sex-matched general population in Ireland. Fifty-four percent of participants had symptoms of anxiety, 15 percent had symptoms of depression, while 23 percent of participants were at risk of posttraumatic stress disorder. Sixty-seven percent of previously employed participants had returned to work. This study highlights the protracted nature of physical and psychologic recovery in patients surviving up to three years after V-V ECMO for severe acute respiratory failure.

Fluid Balance and Recovery of Native Lung Function in Adult Patients Supported by Venovenous Extracorporeal Membrane Oxygenation and Continuous Renal Replacement Therapy.

Fluid overload is associated with increased mortality in adult patients with acute respiratory distress syndrome. In patients requiring venovenous extracorporeal membrane oxygenation (VV-ECMO), the effects of fluid removal on survival and lung recovery remain undefined. We assessed the impact of early fluid removal in adult patients supported by VV-ECMO and concomitant continuous renal replacement therapy, in an 18-bed tertiary intensive care unit between 2010 and 2015. Twenty-four patients met inclusion criteria, of these 15 (63%) survived to hospital discharge. In our patient group, a more negative cumulative daily fluid balance was strongly associated with improved pulmonary compliance (2.72 ml/cmH2O per 1 L negative fluid balance; 95% confidence interval [CI]: 1.61-3.83; P < 0.001). In addition, a more negative mean daily fluid balance was associated with improved pulmonary compliance (4.37 ml/cmH2O per 1 L negative fluid balance; 95% CI: 2.62-6.13; P < 0.001). Survivors were younger and had lower mean daily fluid balance (-0.33 L [95% CI: -1.22 to -0.06] vs. -0.07 L [95% CI: -0.76 to 0.06]; P = 0.438) and lower cumulative fluid balance up to day 14 (-4.60 L [95% CI: -8.40 to -1.45] vs. -1.00 L [95% CI: -4.60 to 0.90]; P = 0.325), although the fluid balance effect alone did not reach statistical significance.

Extracorporeal membrane oxygenation in an HIV-positive man with severe acute respiratory distress syndrome secondary to pneumocystis and cytomegalovirus pneumonia.

The management of critically ill human immunodeficiency virus (HIV)-positive patients is challenging; however, intensive care unit-related mortality has declined significantly in recent years. There are 10 case reports in the literature of extracorporeal membrane oxygenation (ECMO) use in HIV-positive patients, of whom seven survived to hospital discharge. We describe a 33-year-old Brazilian man who presented with Pneumocystis jirovecii pneumonia and severe hypoxic respiratory failure. He developed refractory acute respiratory distress syndrome (ARDS) and was commenced on veno-venous ECMO. He was successfully decannulated following 21 days of ECMO and survived to hospital discharge. Despite poor evidence surrounding the use of ECMO in immunocompromised patients, it is evident that ECMO could represent an important rescue therapy in HIV-positive patients with refractory ARDS.

Nosocomial infections in a cohort of extracorporeal life support patients.

OBJECTIVES: To examine nosocomial infections in a cohort of patients receiving extracorporeal life support (ECLS) at our institution and to identify the types of infections, impact of prophylaxis, and any apparent risk factors for infection. METHODS: In a retrospective cohort study, we examined the records of all patients who received ECLS at our institution between August 2009 and March 2011. A prospective, daily, multidisciplinary assessment of all microbiological issues in these patients was carried out, including assessment of microbiological culture positivity and clinical evidence of infection. The results of these assessments were analysed in relation to HELICS (Hospital in Europe Link for Infection Control through Surveillance) and CDC (Centers for Disease Control and Prevention) diagnostic criteria. The use of antimicrobials in these patients was also assessed, as well as the overall bloodstream infection rate in ICU patients. RESULTS: Seventeen patients received ECLS during the study period, with a total of 445 ECLS days. Of these patients, 13 received respiratory (venovenous) ECLS and four received cardiac (venoarterial) ECLS. There were 17 infections in the cohort: 11 ventilator-associated pneumonias; four bloodstream infections (likely all catheter related, yielding a rate of 9.0 infections/1000 ECLS days); one skin and soft tissue infection; and one urinary tract infection. The bloodstream infection rate in the ICU population as a whole was 9.30/1000 bed-days in 2009 and 7.21/1000 bed-days in 2010. Resistant organisms were identified in 3/17 infections: one methicillin-resistant Staphylococcus aureus, one multidrug-resistant strain of Pseudomonas and one extended-spectrum Β-lactamase-producing Escherichia coli. The median time to acquiring nosocomial infection was 25 days (interquartile range, 13-33 days). The first four ECLS patients received antibacterial (vancomycin) and antifungal (caspofungin) prophylaxis for the duration of ECLS, whereas the later cohort of 13 did not. In patients who received prophylactic antimicrobials, the defined daily dose (DDD) per 100 ECLS days was 49.54 for vancomycin and 49.63 for meropenem. In patients who did not receive prophylaxis, the corresponding DDDs were 25.31 and 37.73, respectively. In ICU patients overall, the DDD per 100 bed-days over the same time period was 13.60 for vancomycin and 19.75 for meropenem. There were 21/445 ECLS days on which antimicrobials were not used. CONCLUSION: Although ECLS patients are at high risk of acquiring nosocomial infections, the infection rate in our cohort was low. The bloodstream infection rate compared favourably with previously published rates, and was comparable with the bloodstream infection rate among ICU patients as a whole over the same time period. Increased duration of ECLS in this cohort may correlate with an increased rate of infection, consistent with data from other ECLS centres. Antimicrobial use in ECLS patients was high relative to overall use in ICU patients. Larger studies are warranted to evaluate the diagnosis, treatment and overall approach to managing nosocomial infection in ECLS patients.