O. Chioncel et al. Cardiogenic shock (CS) is a complex multifactorial clinical syndrome with extremely high mortality, developing as a continuum, and progressing from the initial insult (underlying cause) to the subsequent occurrence of organ failure and death. There is a large spectrum of CS presentations resulting from the interaction between an acute cardiac insult and a patient's underlying cardiac and overall medical condition. Phenotyping patients with CS may have clinical impact on management because classification would support initiation of appropriate therapies. CS management should consider appropriate organization of the health care services, and therapies must be given to the appropriately selected patients, in a timely manner, whilst avoiding iatrogenic harm. Although several consensus-driven algorithms have been proposed, CS management remains challenging and substantial investments in research and development have not yielded proof of efficacy and safety for most of the therapies tested, and outcome in this condition remains poor. Future studies should consider the identification of the new pathophysiological targets, and high-quality translational research should facilitate incorporation of more targeted interventions in clinical research protocols, aimed to improve individual patient outcomes. Designing outcome clinical trials in CS remains particularly challenging in this critical and very costly scenario in cardiology, but information from these trials is imperiously needed to better inform the guidelines and clinical practice. The goal of this review is to summarize the current knowledge concerning the definition, epidemiology, underlying causes, pathophysiology and management of CS based on important lessons from clinical trials and registries, with a focus on improving in-hospital management.

We report the case of a patient with a pulmonary artery (PA) aneurysm secondary to a regurgitant quadricuspid pulmonary valve, in which both lesions were successfully repaired. The patient, a 16-year old boy, was known to have had pulmonary regurgitation and progressive dilation of the PA for years. He was operated on when he developed symptoms of effort, a dilated right ventricle and a PA of 55 mm. The quadricuspid pulmonary valve was an intraoperative finding. It had a dilated annulus, two normal cusps, a third with a 'sparrow-nest' configuration and the fourth was severely hypoplastic (which explained the valve regurgitation). The valve was repaired through tricuspidisation of the quadricuspid pulmonary valve (annular plication at the level of the hypoplastic cusp, freeing of excedentary tissue from the 'sparrow-nest' cusp and its reattachment to the plicated annulus). The aneurysm was treated through a reduction pulmonary arterioplasty. Early assessment showed the minimal regurgitation of the valve and a normal diameter PA. The outcome was uneventful, with a stable correction after 44 months of follow-up. To our knowledge, this is the first repair of a quadricuspid pulmonary valve, through tricuspidisation.

Background: Cardiogenic shock (CS) is a life-threatening state of tissue hypoperfusion, associated to a very high risk of mortality, despite intensive monitoring and modern treatment modalities. Present review aims to describe the therapeutic advances in the management of CS. Areas of Uncertainty: Many uncertainties about CS management remain in clinical practice, and these relate to intensity of invasive monitoring, the type and timing of vasoactive therapies, the risk-benefit ratio of mechanical circulatory support (MCS) therapy and optimal ventilation mode. Furthermore, most of the data is coming from CS in setting of acute myocardial infarction (AMI), while for nonAMI-CS patients there are very few evidences for etiological or MCS therapies. Data Sources: The prospective multicentric acute heart failure registries that specifically presented characteristics of patients with CS, distinct to other phenotypes, were included in the present review. Relevant clinical trials investigating therapeutic strategies in post AMI-CS patients were added as source information. Several trials investigating vasoactive medications and metaanalysis providing information about benefits and risks of MCS were reviewed in the manuscript.

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