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:Highlights•Echocardiography is used to diagnose hypertrophic obstructive cardiomyopathy (HOCM) and Takotsubo cardiomyopathy (TCM).•Hypotension in a patient with TCM should be evaluated for left ventricular outflow tract obstruction (LVOTO).•Management of TCM is challenging in patients with HOCM with severe LVOTO.•Hypotension in LVOTO may paradoxically worsen with standard intravenous inotropes.•Fluid resuscitation, beta-blockers, alpha agonists, and intra-aortic balloon pump are the treatment options in LVOTO.
| S-EPMC6058765 | biostudies-other
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: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
Project description:BackgroundWith the high prevalence of COVID-19 infections worldwide, the multisystem inflammatory syndrome in adults (MIS-A) is becoming an increasingly recognized entity. This syndrome presents in patients several weeks after infection with COVID-19 and is associated with thrombosis, elevated inflammatory markers, hemodynamic compromise and cardiac dysfunction. Treatment is often with steroids and intravenous immunoglobulin (IVIg). The pathologic basis of myocardial injury in MIS-A, however, is not well characterized. In our case report, we obtained endomyocardial biopsy that revealed a pattern of myocardial injury similar to that found in COVID-19 cardiac specimens.Case presentationA 26-year-old male presented with fevers, chills, headache, nausea, vomiting, and diarrhea 5 weeks after his COVID-19 infection. His SARS-CoV-2 PCR was negative and IgG was positive, consistent with prior infection. He was found to be in cardiogenic shock with biventricular failure, requiring inotropes and diuretics. Given concern for acute fulminant myocarditis, an endomyocardial biopsy (EMB) was performed, showing an inflammatory infiltrate consisting predominantly of interstitial macrophages with scant T lymphocytes. The histologic pattern was similar to that of cardiac specimens from COVID-19 patients, helping rule out myocarditis as the prevailing diagnosis. His case was complicated by persistent hypoxemia, and a computed tomography scan revealed pulmonary emboli. He received IVIg, steroids, and anticoagulation with rapid recovery of biventricular function.ConclusionsMIS-A should be considered as the diagnosis in patients presenting several weeks after COVID-19 infection with severe inflammation and multi-organ involvement. In our case, EMB facilitated identification of MIS-A and guided therapy. The patient's biventricular function recovered with IVIg and steroids.
| S-EPMC8555861 | biostudies-literature
Project description:BackgroundSystemic capillary leak syndrome (SCLS) is a potentially fatal disorder characterized by relapses of hypovolemic shock episodes.Case summaryWe present a case of a 58-year-old man who presented to the Emergency Department with a history of recurrent episodes of syncope in the last hours. A few days before medical contact the patient complained of sore throat, fever, and flu-like symptoms. He was initially admitted with a diagnosis of suspected myopericarditis. Forty-eight hours later, the haemodynamic status suddenly deteriorated to a mixed cardiogenic and shock; an endomyocardial biopsy showed localized inflammatory infiltrates and areas of necrosis of cardiomyocytes with positive viral search for parvovirus B19 (PVB19), therefore the patient was treated with methylprednisolone pulses. Based on the concurrent presence of the typical triad of hypotension, hypoalbuminaemia, and haemoconcentration we suspected systemic leak capillary syndrome potentially triggered by the PVB19 infection with acute myocarditis. The clinical conditions further deteriorated with rhabdomyolysis and acute kidney injury: we started continuous veno-venous haemofiltration adding a cytokines adsorber. In the following hours, we observed a significant clinical improvement. The patient was discharged 1 month later and 5 months after discharge he experienced a new attack of SCLS, this time without myocardial involvement and with prompt symptoms resolution.ConclusionSystemic capillary leak syndrome is a potentially fatal disorder: early recognition of this entity and prompt initiation of supportive therapy are warranted, therefore, it is paramount that an emergency physician thinks of SCLS in patients with signs of cardiogenic shock and the classical triad of hypotension, hypoalbuminia, and haemoconcentration.
| S-EPMC9258032 | 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:Early use of mechanical circulatory support, e.g. veno-arterial extracorporeal membrane oxygenation (ECMO) or left ventricular unloading by microaxial pump in refractory cardiogenic shock is recommended in current guidelines. Development of acquired von Willebrand Syndrome (AVWS) in patients with left ventricular assist devices (LVADs) and ECMO has been reported. There is an increasing number of patients treated with the Impella® CP microaxial pump for left ventricular unloading. However, the prevalence of AVWS in these high risk patients is unknown and needs to be determined. We therefore screened 21 patients (68 ± 11years) treated with Impella® (17 for cardiogenic shock, 4 for protected PCI) for the presence of AVWS by determining von Willebrand factor multimers, VWF collagen binding capacity and VWF antigen. During the time course of Impella® support, 20/21 patients (95%) developed AVWS (mean duration of support: 135 ± 114 hours, mean time from device implantation to first diagnosis of AVWS: 10.6 ± 10.8 hours). Our data indicate that AVWS is a common phenomenon during left ventricular unloading via microaxial pump support. Thus, AVWS has to be considered as contributing factor for potential bleeding complications in this high risk patient population, especially in the context of dual antiplatelet therapy.
| S-EPMC5555672 | biostudies-literature