Cardiogenic Shock in Obstructive Hypertrophic Cardiomyopathy Plus Apical Ballooning: Management With VA-ECMO and Myectomy.
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ABSTRACT:
SUBMITTER: Caniato F
PROVIDER: S-EPMC8311032 | biostudies-literature |
REPOSITORIES: biostudies-literature
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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:Apical akinesis and dilation in the absence of obstructive coronary artery disease is a typical feature of stress-induced (takotsubo) cardiomyopathy, whereas apical hypertrophy is seen in apical-variant hypertrophic cardiomyopathy. We report the cases of 2 patients who presented with takotsubo cardiomyopathy and were subsequently found to have apical-variant hypertrophic cardiomyopathy, after the apical ballooning from the takotsubo cardiomyopathy had resolved. The first patient, a 43-year-old woman with a history of alcohol abuse, presented with shortness of breath, electrocardiographic and echocardiographic features consistent with takotsubo cardiomyopathy, and no significant coronary artery disease. An echocardiogram 2 weeks later revealed a normal left ventricular ejection fraction and newly apparent apical hypertrophy. The 2nd patient, a 70-year-old woman with pancreatitis, presented with chest pain, apical akinesis, and a left ventricular ejection fraction of 0.39, consistent with takotsubo cardiomyopathy. One month later, her left ventricular ejection fraction was normal; however, hypertrophy of the left ventricular apex was newly noted. To our knowledge, these are the first reported cases in which apical-variant hypertrophic cardiomyopathy was masked by apical ballooning from stress-induced cardiomyopathy.
| S-EPMC4004496 | biostudies-other
Project description: Not available
| S-EPMC2000940 | biostudies-literature
Project description:BackgroundVeno-arterial extracorporeal membrane oxygenation (VA-ECMO) is increasingly adopted for the treatment of cardiogenic shock (CS). However, a marker of successful weaning remains largely unknown. Our hypothesis was that successful weaning is associated with sustained microcirculatory function during ECMO flow reduction. Therefore, we sought to test the usefulness of microcirculatory imaging in the same sublingual spot, using incident dark field (IDF) imaging in assessing successful weaning from VA-ECMO and compare IDF imaging with echocardiographic parameters.MethodsWeaning was performed by decreasing the VA-ECMO flow to 50% (F50) from the baseline. The endpoint of the study was successful VA-ECMO explantation within 48 hours after weaning. The response of sublingual microcirculation to a weaning attempt (WA) was evaluated. Microcirculation was measured in one sublingual area (single spot (ss)) using CytoCam IDF imaging during WA. Total vessel density (TVDss) and perfused vessel density (PVDss) of the sublingual area were evaluated before and during 50% flow reduction (TVDssF50, PVDssF50) after a WA and compared to conventional echocardiographic parameters as indicators of the success or failure of the WA.ResultsPatients (n = 13) aged 49 ± 18 years, who received VA-ECMO for the treatment of refractory CS due to pulmonary embolism (n = 5), post cardiotomy (n = 3), acute coronary syndrome (n = 2), myocarditis (n = 2) and drug intoxication (n = 1), were included. TVDssF50 (21.9 vs 12.9 mm/mm2, p = 0.001), PVDssF50 (19.7 vs 12.4 mm/mm2, p = 0.01) and aortic velocity-time integral (VTI) at 50% flow reduction (VTIF50) were higher in patients successfully weaned vs not successfully weaned. The area under the curve (AUC) was 0.99 vs 0.93 vs 0.85 for TVDssF50 (small vessels) >12.2 mm/mm2, left ventricular ejection fraction (LVEF) >15% and aortic VTI >11 cm. Likewise, the AUC was 0.91 vs 0.93 vs 0.85 for the PVDssF50 (all vessels) >14.8 mm/mm2, LVEF >15% and aortic VTI >11 cm.ConclusionThis study identified sublingual microcirculation as a novel potential marker for identifying successful weaning from VA-ECMO. Sustained values of TVDssF50 and PVDssF50 were found to be specific and sensitive indicators of successful weaning from VA-ECMO as compared to echocardiographic parameters.
| S-EPMC5658964 | biostudies-literature
Project description:Impella and VA-ECMO are two possible therapeutic courses for the treatment of patients with cardiogenic shock (CS). The study aims to perform a systematic literature review and meta-analyses of a comprehensive set of clinical and socio-economic outcomes observed when using Impella or VA-ECMO with patients under CS. A systematic literature review was performed in Medline, and Web of Science databases on 21 February 2022. Nonoverlapping studies with adult patients supported for CS with Impella or VA-ECMO were searched. Study designs including RCTs, observational studies, and economic evaluations were considered. Data on patient characteristics, type of support, and outcomes were extracted. Additionally, meta-analyses were performed on the most relevant and recurring outcomes, and results shown using forest plots. A total of 102 studies were included, 57% on Impella, 43% on VA-ECMO. The most common outcomes investigated were mortality/survival, duration of support, and bleeding. Ischemic stroke was lower in patients treated with Impella compared to the VA-ECMO population, with statistically significant difference. Socio-economic outcomes including quality of life or resource use were not reported in any study. The study highlighted areas where further data collection is needed to clarify the value of complex, new technologies in the treatment of CS that will enable comparative assessments focusing both on the health impact on patient outcomes and on the financial burden for government budgets. Future studies need to fill the gap to comply with recent regulatory updates at the European and national levels.
| S-EPMC10142129 | biostudies-literature
Project description:BackgroundThe dynamic obstruction of the left ventricular outflow tract (LVOT) is a well-known complication in mitral annuloplasty but rarely seen in nonmitral cardiovascular surgery. The dynamic LVOT obstruction can lead to hemodynamic instability, even shock and the treatment is significantly different from the standard approach. Case Presentation. We reported a case of low cardiac output syndrome (LCOS) with severe mitral regurgitation (MR), dramatically reduced left ventricular ejection fraction (LVEF) after coronary artery bypass grafting in a 72-year-old female requiring an escalation of inotropic support, volume restriction, and mechanical support. The detailed echocardiography combined with lung ultrasound revealed a dynamic systolic anterior movement of the anterior mitral leaflet (SAM), apical ballooning, and no significant lung congestion. Intravenous fluids were given, diuretics withdrawn, inotrope discontinued, and vasopressors uptitrated. The dynamic SAM was rapidly relieved, the hemodynamics was stabilized, and the LVEF was improving. The patient was discharged in good condition without residual LVOT obstruction and trace MR.ConclusionWe strongly suggest that a detailed echocardiography should be performed in any patient who presents in shock to rule out a dynamic LVOT obstruction. Lung ultrasound should be a routine examination in addition to echocardiography. Once SAM is detected, treatment should be based on volume expansion, inotrope discontinuation, and a careful afterload increasing.
| S-EPMC7716751 | biostudies-literature
Project description: Not available
| S-EPMC6058765 | biostudies-literature
Project description:Venous-arterial extracorporeal membrane oxygenation (VA-ECMO) treatment for acute cardiogenic shock in patients who also have acute lung injury predisposes development of a serious complication called "north-south syndrome" (NSS) which causes cerebral hypoxia. NSS is poorly characterized and hemodynamic studies have focused on cerebral perfusion ignoring the heart. We hypothesized in NSS the heart would be more likely to receive hypoxemic blood than the brain due to the proximity of the coronary arteries to the aortic annulus. To test this, we conducted a computational fluid dynamics simulation of blood flow in a human supported by VA-ECMO. Simulations quantified the fraction of blood at each aortic branching vessel originating from residual native cardiac output versus VA-ECMO. As residual cardiac function was increased, simulations demonstrated myocardial hypoxia would develop prior to cerebral hypoxia. These results illustrate the conditions where NSS will develop and the relative cardiac function that will lead to organ-specific hypoxia. Illustration of the impact of north-south syndrome on organ-specific oxygen delivery. Patients on VA-ECMO have two sources of blood flow, one from the VA-ECMO circuit and one from the residual cardiac function. When there is no residual cardiac function, all organs are perfused with oxygenated blood. As myocardial recovery progresses, blood supply from the two sources will begin to mix resulting in non-homogeneous mixing and differential oxygenation based upon the anatomical site of branching vessels.
| S-EPMC7901681 | biostudies-literature
Project description:Surgical septal myectomy is the treatment of choice for patients with symptomatic hypertrophic obstructive cardiomyopathy refractory to medications. This report describes our minimally invasive approach for performing a septal myectomy via a ministernotomy that has been used at our institution for more than a decade. In particular, patient preparation, surgical technique, and clinical considerations are highlighted. Performed properly, this minimally invasive technique is a feasible and effective approach in our experience.
| S-EPMC5774662 | biostudies-literature
Project description:ObjectiveWe implemented a novel resectable myocardial model for mock myectomy using a hybrid method of three-dimensional (3D) printing and silicone molding for patients with apical hypertrophic cardiomyopathy (ApHCM).Materials and methodsFrom January 2019 through May 2020, 3D models from three patients with ApHCM were generated using the end-diastolic cardiac CT phase image. After computer-aided designing of measures to prevent structural deformation during silicone injection into molding, 3D printing was performed to reproduce anatomic details and molds for the left ventricular (LV) myocardial mass. We compared the myocardial thickness of each cardiac segment and the LV myocardial mass and cavity volumes between the myocardial model images and cardiac CT images. The surgeon performed mock surgery, and we compared the volume and weight of the resected silicone and myocardium.ResultsDuring the mock surgery, the surgeon could determine an ideal site for the incision and the optimal extent of myocardial resection. The mean differences in the measured myocardial thickness of the model (0.3, 1.0, 6.9, and 7.3 mm in the basal, midventricular, apical segments, and apex, respectively) and volume of the LV myocardial mass and chamber (36.9 mL and 14.8 mL, 2.9 mL and -9.4 mL, and 6.0 mL and -3.0 mL in basal, mid-ventricular and apical segments, respectively) were consistent with cardiac CT. The volume and weight of the resected silicone were similar to those of the resected myocardium (6 mL [6.2 g] of silicone and 5 mL [5.3 g] of the myocardium in patient 2; 12 mL [12.5 g] of silicone and 11.2 mL [11.8 g] of the myocardium in patient 3).ConclusionOur 3D model created using hybrid 3D printing and silicone molding may be useful for determining the extent of surgery and planning surgery guided by a rehearsal platform for ApHCM.
| S-EPMC8236372 | biostudies-literature