title: Extracorporeal membrane oxygenation for pheochromocytoma-induced cardiogenic shock.
abstract: BACKGROUND
  Pheochromocytoma, a rare catecholamine-producing tumor, might provoke stress-induced Takotsubo-like cardiomyopathy and severe cardiogenic shock. Because venoarterial-extracorporeal membrane oxygenation (VA-ECMO) rescue of pheochromocytoma-induced refractory cardiogenic shock has rarely been reported, we reviewed our ICU patients' presentations and outcomes.
  
  
  METHODS
  All pheochromocytoma-induced refractory cardiogenic shock cases managed with VA-ECMO (January 2007-March 2015) were prospectively included and reviewed. We also performed a systematic review on this topic.
  
  
  RESULTS
  Nine patients (7 women, 2 men; 31-51 [median, 43 (IQR 36-49) years old]) were included; none had a previously known pheochromocytoma. Six of them had medical histories suggestive of the diagnosis: palpitations and headaches for several months for four, multiple endocrine neoplasia syndrome type 1 for one and recurrent Takotsubo disease for one; at hospital admission, all were hypertensive despite cardiogenic shock. Three others had an identified surgical triggering factor. All nine patients rapidly developed refractory cardiogenic shock with very severe left ventricular (LV) impairment (LV ejection-fraction range 5-20%; LV outflow-tract velocity-time integral range 3-8 cm). Seven patients' abdominal computed tomography scans showed pheochromocytoma-suggestive adrenal gland tumors (no scan during ICU stay for 2). Despite VA-ECMO implantation, three patients died of refractory multiple organ failure. For the six others, myocardial function improved and ECMO was removed 3-7 days post-implantation; α- and β-blockers were progressively introduced. Five survivors underwent pheochromocytoma excision 3 weeks-4 months post-ICU discharge, with satisfactory outcomes. One patient, whose pheochromocytoma was diagnosed 1 year after the index event, underwent uneventful surgical adrenalectomy. Systematic review retrieved 40 cases of pheochromocytoma-induced cardiogenic shock requiring mechanical support (mostly ECMO), with a mortality rate of 7%. Pheochromocytoma was removed surgically after mechanical support weaning in 31 patients and during mechanical support in 5. Four were not operated.
  
  
  CONCLUSIONS
  Pheochromocytoma is a rare but reversible cause of cardiogenic shock amenable to VA-ECMO rescue. Adrenal gland imaging should be obtained for all patients with unexplained cardiogenic shock. Lastly, it might be safer to perform adrenalectomy several weeks after the initial catastrophic presentation, once recovery of LV systolic function is complete.
authors.affiliation: Intensive Care Unit, Institute of Cardiology, Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France. guillaume.hekimian@aphp.fr.|UPMC Université Paris 06, INSERM, UMRS_1166-ICAN Institute of Cardiometabolism and Nutrition, Paris, France. guillaume.hekimian@aphp.fr.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Hekimian
authors.affiliation: Institute of Endocrinology, Pierre et Marie Curie Sorbonne Université, APHP, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.
authors.forename: Fatima
authors.initials: F
authors.lastname: Kharcha
authors.affiliation: Intensive Care Unit, Institute of Cardiology, Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France.|UPMC Université Paris 06, INSERM, UMRS_1166-ICAN Institute of Cardiometabolism and Nutrition, Paris, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Bréchot
authors.affiliation: Intensive Care Unit, Institute of Cardiology, Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France.|UPMC Université Paris 06, INSERM, UMRS_1166-ICAN Institute of Cardiometabolism and Nutrition, Paris, France.
authors.forename: Matthieu
authors.initials: M
authors.lastname: Schmidt
authors.affiliation: Institute of Endocrinology, Pierre et Marie Curie Sorbonne Université, APHP, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.
authors.forename: Cécile
authors.initials: C
authors.lastname: Ghander
authors.affiliation: Department of Cardiac and Thoracic Surgery, Pierre et Marie Curie Sorbonne Université, APHP, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Lebreton
authors.affiliation: Institute of Endocrinology, Pierre et Marie Curie Sorbonne Université, APHP, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Girerd
authors.affiliation: Department of General and Endocrine Surgery, Pierre et Marie Curie Sorbonne Université, APHP, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Tresallet
authors.affiliation: Intensive Care Unit, Institute of Cardiology, Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France.|UPMC Université Paris 06, INSERM, UMRS_1166-ICAN Institute of Cardiometabolism and Nutrition, Paris, France.
authors.forename: Jean-Louis
authors.initials: JL
authors.lastname: Trouillet
authors.affiliation: Department of Cardiac and Thoracic Surgery, Pierre et Marie Curie Sorbonne Université, APHP, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Leprince
authors.affiliation: Intensive Care Unit, Institute of Cardiology, Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France.|UPMC Université Paris 06, INSERM, UMRS_1166-ICAN Institute of Cardiometabolism and Nutrition, Paris, France.
authors.forename: Jean
authors.initials: J
authors.lastname: Chastre
authors.affiliation: Intensive Care Unit, Institute of Cardiology, Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France.|UPMC Université Paris 06, INSERM, UMRS_1166-ICAN Institute of Cardiometabolism and Nutrition, Paris, France.
authors.forename: Alain
authors.initials: A
authors.lastname: Combes
authors.affiliation: Intensive Care Unit, Institute of Cardiology, Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France.|UPMC Université Paris 06, INSERM, UMRS_1166-ICAN Institute of Cardiometabolism and Nutrition, Paris, France.
authors.forename: Charles-Edouard
authors.initials: CE
authors.lastname: Luyt