A Tale of 2 Aneurysms: Cardiogenic Shock Secondary to Vascular Behcet's Syndrome.
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ABSTRACT:
SUBMITTER: Doumouras BS
PROVIDER: S-EPMC8642724 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Background Cardiogenic shock (CS) is a heterogeneous syndrome with varied presentations and outcomes. We used a machine learning approach to test the hypothesis that patients with CS have distinct phenotypes at presentation, which are associated with unique clinical profiles and in-hospital mortality. Methods and Results We analyzed data from 1959 patients with CS from 2 international cohorts: CSWG (Cardiogenic Shock Working Group Registry) (myocardial infarction [CSWG-MI; n=410] and acute-on-chronic heart failure [CSWG-HF; n=480]) and the DRR (Danish Retroshock MI Registry) (n=1069). Clusters of patients with CS were identified in CSWG-MI using the consensus k means algorithm and subsequently validated in CSWG-HF and DRR. Patients in each phenotype were further categorized by their Society of Cardiovascular Angiography and Interventions staging. The machine learning algorithms revealed 3 distinct clusters in CS: "non-congested (I)", "cardiorenal (II)," and "cardiometabolic (III)" shock. Among the 3 cohorts (CSWG-MI versus DDR versus CSWG-HF), in-hospital mortality was 21% versus 28% versus 10%, 45% versus 40% versus 32%, and 55% versus 56% versus 52% for clusters I, II, and III, respectively. The "cardiometabolic shock" cluster had the highest risk of developing stage D or E shock as well as in-hospital mortality among the phenotypes, regardless of cause. Despite baseline differences, each cluster showed reproducible demographic, metabolic, and hemodynamic profiles across the 3 cohorts. Conclusions Using machine learning, we identified and validated 3 distinct CS phenotypes, with specific and reproducible associations with mortality. These phenotypes may allow for targeted patient enrollment in clinical trials and foster development of tailored treatment strategies in subsets of patients with CS.
| S-EPMC8483502 | biostudies-literature
Project description:BackgroundH syndrome (HS) is a rare autoinflammatory disease caused by a mutation in the solute carrier family 29, member 3 (SCL29A3) gene. It has a variable clinical presentation and little phenotype-genotype correlation. The pathognomonic sign of HS is cutaneous hyperpigmentation located mainly in the inner thighs and often accompanied by other systemic manifestations. Improvement after tocilizumab treatment has been reported in a few patients with HS. We report the first patient with HS who presented cardiogenic shock, multiorgan infiltration, and digital ischemia.Case presentation8-year-old boy born to consanguineous parents of Moroccan origin who was admitted to the intensive care unit during the Coronavirus Disease-2019 (COVID-19) pandemic with tachypnoea, tachycardia, and oliguria. Echocardiography showed dilated cardiomyopathy and severe systolic dysfunction compatible with cardiogenic shock. Additionally, he presented with multiple organ dysfunction syndrome. SARS-CoV-2 polymerase chain reaction (PCR) and antibody detection by chromatographic immunoassay were negative. A previously ordered gene panel for pre-existing sensorineural hearing loss showed a pathological mutation in the SCL29A3 gene compatible with H syndrome. Computed tomography scan revealed extensive alveolar infiltrates in the lungs and multiple poor defined hypodense lesions in liver, spleen, and kidneys; adenopathy; and cardiomegaly with left ventricle subendocardial nodules. Invasive mechanical ventilation, broad antibiotic and antifungal coverage showed no significant response. Therefore, Tocilizumab as compassionate use together with pulsed intravenous methylprednisolone was initiated. Improvement was impressive leading to normalization of inflammation markers, liver and kidney function, and stabilising heart function. Two weeks later, he was discharged and has been clinically well since then on two weekly administration of Tocilizumab.ConclusionsWe report the most severe disease course produced by HS described so far in the literature. Our patient's manifestations included uncommon, new complications such as acute heart failure with severe systolic dysfunction, multi-organ cell infiltrate, and digital ischemia. Most of the clinical symptoms of our patient could have been explained by SARS-CoV-2, demonstrating the importance of a detailed differential diagnosis to ensure optimal treatment. Although the mechanism of autoinflammation of HS remains uncertain, the good response of our patient to Tocilizumab makes a case for the important role of IL-6 in this syndrome and for considering Tocilizumab as a first-line treatment, at least in severely affected patients.
| S-EPMC8243620 | biostudies-literature
Project description:Background Extra Corporeal Life Support (ECLS) is an evolving therapy in therapy-resistant cardiogenic shock (CS). Vascular cannulation in emergency situations can be accomplished through puncture of the femoral vessels by specialised teams. Since lower limb ischemia constitutes one of the major complications following cannulation, a distal perfusion cannula (DPC) has emerged as standard of care. We here aimed to analyse the impact of the DPC on limb perfusion and 6-month survival rate. Methods In a retrospective study from January 2012 to December 2018, 98 patients with cardiogenic shock and peripheral (v-a) ECLS implantation with documented limb perfusion status were identified and analysed. Demographic data, laboratory parameters, cause of CS, comorbidities, limb perfusion complications and complication management were analysed. Results 53 patients (54%) received ECLS therapy in referral centers by our mobile ECLS team, while in 45 patients (46%) the cannulation occured in our center. 71 patients (72%) received a DPC (group A) at the time of ECLS implantation, whereas 27 (28%) (group B) did not or received later (14 patients owing to limb ischemia). 44 patients (45%) developed limb ischemia as a complication of ECLS therapy (31% in group A and 81% in group B- p < 0.001). The 6-month survival rate was 28% in our study cohort (30% in group A and 22% in group B- p = 0.469). Conclusion Lower limb ischemia remains a serious complication after peripheral ECLS cannulation in CS, especially when a DPC is absent. Standardised DPC implementation may reduce the rate of severe limb complications in peripheral ECLS.
| S-EPMC10236458 | biostudies-literature
Project description:Despite the rapidly evolving evidence base in modern cardiology, progress in the area of cardiogenic shock remains slow, with short-term mortality still reaching 40-50%, relatively unchanged in recent years. Despite advances with an increase in the number of clinical trials taking place in this admittedly difficult-to-study area, the evidence base on which we make day-to-day decisions in clinical practice remains relatively sparse. With only definitive evidence for early revascularisation and the relative ineffectiveness of intra-aortic balloon pumping, most aspects of patient management are based on expert consensus, rather than randomised controlled trials. This updated 2020 review will outline the management of CS mainly after acute myocardial infarction with major focus on state-of-the-art treatment based on randomised clinical trials or matched comparisons if available.
| S-EPMC9724885 | biostudies-literature
Project description: Not available
| S-EPMC6058765 | biostudies-literature
Project description:The incidence of cardiogenic shock is rising, patient complexity is increasing and patient survival has plateaued. Mirroring organisational innovations of elite military units, our multidisciplinary medical specialists at the INOVA Heart and Vascular Institute aim to combine the adaptability, agility and cohesion of small teams across our large healthcare system. We advocate for widespread adoption of our 'combat' methodology focused on: increased disease awareness, early multidisciplinary shock team activation, group decision-making, rapid initiation of mechanical circulatory support (as appropriate), haemodynamic-guided management, strict protocol adherence, complete data capture and regular after action reviews, with a goal of ending preventable death from cardiogenic shock.
| S-EPMC5980650 | biostudies-other
Project description:AimsThe optimal vascular access site for percutaneous coronary interventions (PCIs) in patients with acute myocardial infarction (AMI) complicated by cardiogenic shock (CS) remains uncertain. While observational data favour transradial access (TRA) due to lower complication rates and mortality, transfemoral access (TFA) PCI offers advantages such as shorter access and procedure times, along with quicker escalation to mechanical circulatory support (MCS). In this study, we aimed to investigate factors associated with a transfemoral approach and compare mortality rates between TRA and TFA in AMI-CS patients undergoing PCI.Methods and resultsData from a nationwide registry of AMI-CS patients undergoing PCI (2017-2021) were analysed. We compared patient demographics, procedural details, and outcomes between TRA and TFA groups. Logistic regression identified access site factors and radial-to-femoral crossover predictors. Propensity score-matched (PSM) analysis examined the impact of access site on mortality. Of the 1562 patients, 45% underwent TRA PCI, with an increasing trend over time. Transfemoral access patients were more often female, had a history of coronary artery bypass grafting, lower blood pressure, higher resuscitation and intubation rates, and elevated lactate levels. After PSM, 30-day mortality was lower in TRA (33% vs. 46%, P < 0.001). Predictors for crossover included left coronary artery interventions, multivessel PCI, and MCS initiation.ConclusionSignificant differences exist between TRA and TFA PCI in AMI-CS. Transfemoral access was more common in patients with worse haemodynamics and was associated with higher 30-day mortality compared with TRA. This mortality difference persisted in the PSM analysis.
| S-EPMC11350431 | biostudies-literature
Project description:There is limited data on the in-hospital outcomes of cardiogenic shock (CS) secondary to takotsubo syndrome (TS). We aimed to assess the incidence, predictors, and outcomes of CS in hospitalized patients with TS. All patients with TS were identified from the National Inpatient Sample database from September 2006 to December 2017. The cohort was divided into those with versus without CS and logistic regression analysis was used to identify predictors of CS and mortality in patients admitted with TS. A total of 260,144 patients with TS were included in our study, of whom 14,703 (6%) were diagnosed with CS. In-hospital mortality in patients with CS was approximately six-fold higher compared with those without CS (23% vs 4%, p <0.01). TS patients with CS had a higher incidence of malignant arrhythmias like ventricular tachycardia or ventricular fibrillation (15.0% vs 4%, p <0.01) and non-shockable cardiac arrests (12% vs 2%, p <0.01). Independent predictors of CS were male gender, Asian and Hispanic ethnicity, increased burden of co-morbidities including congestive heart failure, chronic pulmonary disease, and chronic diabetes. Independent predictors of mortality were male gender, advanced age, history of congestive heart failure, chronic renal failure, and chronic liver disease. In conclusion, CS occurs in approximately 6% of patients admitted with TS, in-hospital mortality in TS patients with CS was approximately six-fold higher compared with those without CS (23% vs 4%, p <0.01), male gender and increased burden of co-morbidities at baseline were independent predictors of CS and mortality.
| S-EPMC8375056 | biostudies-literature
Project description:Reverse takotsubo cardiomyopathy (rTCM) is characterized by basal ballooning and accounts for approximately 1% of all TCM. To our knowledge, there have been no reports describing rTCM complicated by acute, severe, transient mitral regurgitation (MR). A 75-year-old woman with a medical history of hypertension, dyslipidemia, and anxiety presented to the hospital with 2 days of substernal chest pain, dyspnea, and nausea. Initial troponin was 0.203 ng/mL, and electrocardiography showed sinus tachycardia at 121 bpm, with inferior and anterolateral ST segment depressions. Transthoracic echocardiogram (TTE) found an ejection fraction of 30%, apical hyperkinesis, severe hypokinesis of the basal to mid segments of the left ventricle (LV), and a severe central MR jet. Cardiac angiography demonstrated non-obstructive coronary artery disease, and elevated left ventricular end diastolic pressures. Left ventriculography showed a hyperdynamic apex and severe basal hypokinesis. The patient was treated medically, clinical status improved, and was discharged on day 3. TTE four weeks later, showed an ejection fraction of 60-65%, mild MR, and normal LV function. rTCM is the rarest variant of TCM. Basal and mid-myocardial stunning can cause severe secondary MR leading to acute congestive heart failure, mimicking acute coronary syndrome with acute MR. rTCM with rapidly reversible severe MR has not previously been described. <Learning objective: Mitral regurgitation secondary to reverse takotsubo cardiomyopathy can mimic coronary flow obstruction syndromes and when recognized early, can improve with amelioration of cardiomyopathy. Acute severe mitral regurgitation in reverse takotsubo cardiomyopathy is likely secondary to basilar dilatation. Reverse takotsubo cardiomyopathy represents approximately 1% of all takotsubo cardiomyopathy cases.>.
| S-EPMC8642629 | biostudies-literature
Project description:Background Cardiogenic shock from most causes has unfavorable prognosis. Hypertrophic cardiomyopathy (HCM) can uncommonly present with apical ballooning and shock in association with sudden development of severe and unrelenting left ventricular (LV) outflow obstruction. Typical HCM phenotypic features of mild septal thickening, outflow gradients, and distinctive mitral abnormalities differentiate these patients from others with Takotsubo syndrome, who have normal mitral valves and no outflow obstruction. Methods and Results We analyzed 8 patients from our 4 HCM centers with obstructive HCM and abrupt presentation of cardiogenic shock with LV ballooning, and 6 cases reported in literature. Of 14 patients, 10 (71%) were women, aged 66±9 years, presenting with acute symptoms: LV ballooning; depressed ejection fraction (25±5%); refractory systemic hypotension; marked LV outflow tract obstruction (peak gradient, 94±28 mm Hg); and elevated troponin, but absence of atherosclerotic coronary disease. Shock was managed with intravenous administration of phenylephrine (n=6), norepinephrine (n=6), β-blocker (n=7), and vasopressin (n=1). Mechanical circulatory support was required in 8, including intra-aortic balloon pump (n=4), venoarterial extracorporeal membrane oxygenation (n=3), and Impella and Tandem Heart in 1 each. In refractory shock, urgent relief of obstruction by myectomy was performed in 5, and alcohol ablation in 1. All patients survived their critical illness, with full recovery of systolic function. Conclusions When cardiogenic shock and LV ballooning occur in obstructive HCM, they are marked by distinctive anatomic and physiologic features. Relief of obstruction with targeted pharmacotherapy, mechanical circulatory support, and myectomy, when necessary for refractory shock, may lead to survival and normalization of systolic function.
| S-EPMC8751867 | biostudies-literature