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Pediatr Cardiol. 2022 Dec 2. doi: 10.1007/s00246-022-03056-y. Online ahead of print.

ABSTRACT

Neonatal lupus (NLE) is a rare acquired autoimmune disorder caused by transplacental passage of maternal autoantibodies to Sjogren's Syndrome A or B (SSA-SSB) autoantigens (Vanoni et al. in Clin Rev Allerg Immunol 53:469-476, 2017) which target fetal and neonatal tissues for immune destruction. The cardiac trademark of NLE is autoimmune heart block, which accounts for more than 80% of cases of complete atrioventricular heart block (AVB) in newborns with a structurally normal heart (Martin in Cardiol Young 24: 41-46, 2014). NLE presenting with cardiac alterations not involving rhythm disturbances are described in the literature, but they are rare. Here, we report a case of a neonate with high anti-SSA antibodies who developed severe ventricular dysfunction in the absence of rhythm abnormalities, endocardial fibroelastosis, and dilated cardiomyopathy (Trucco et al. in J Am Coll Cardiol 57:715-723, https://doi.org/10.1016/j.jacc.2010.09.044 , 2011), the most common cardiac presentations of NLE. The patient developed severe multiorgan dysfunction syndrome that required prolonged critical care support but fully recovered and was discharged home. We highlight the unusual clinical features of this NLE case and the importance of timely treatment of NLE allowing complete recovery of a critically ill neonate.

PMID:36460799 | DOI:10.1007/s00246-022-03056-y

Eur J Med Genet. 2023 Jan;66(1):104673. doi: 10.1016/j.ejmg.2022.104673. Epub 2022 Nov 29.

ABSTRACT

Heritable thoracic aortic diseases (HTAD) are rare pathologies associated with thoracic aortic aneurysms and dissection, which can be syndromic or non-syndromic. They may result from genetic defects. Associated genes identified to date are classified into those encoding components of the (a) extracellular matrix (b) TGFβ pathway and (c) smooth muscle contractile mechanism. Timely diagnosis allows for prompt aortic surveillance and prophylactic surgery, hence improving life expectancy and reducing maternal complications as well as providing reassurance to family members when a diagnosis is ruled out. This document is an expert opinion reflecting strategies put forward by medical experts and patient representatives involved in the HTAD Rare Disease Working Group of VASCERN. It aims to provide a patient pathway that improves patient care by diminishing time to diagnosis, facilitating the establishment of a correct diagnosis using molecular genetics when possible, excluding the diagnosis in unaffected persons through appropriate family screening and avoiding overuse of resources. It is being recommended that patients are referred to an expert centre for further evaluation if they meet at least one of the following criteria: (1) thoracic aortic dissection (<70 years if hypertensive; all ages if non-hypertensive), (2) thoracic aortic aneurysm (all adults with Z score >3.5 or 2.5-3.5 if non-hypertensive or hypertensive and <60 years; all children with Z score >3), (3) family history of HTAD with/without a pathogenic variant in a gene linked to HTAD, (4) ectopia lentis without other obvious explanation and (5) a systemic score of >5 in adults and >3 in children. Aortic imaging primarily relies on transthoracic echocardiography with magnetic resonance imaging or computed tomography as needed. Genetic testing should be considered in those with a high suspicion of underlying genetic aortopathy. Though panels vary among centers, for patients with thoracic aortic aneurysm or dissection or systemic features these should include genes with a definitive or strong association to HTAD. Genetic cascade screening and serial aortic imaging should be considered for family screening and follow-up. In conclusion, the implementation of these strategies should help standardise the diagnostic work-up and follow-up of patients with suspected HTAD and the screening of their relatives.

PMID:36460281 | DOI:10.1016/j.ejmg.2022.104673

Braz J Cardiovasc Surg. 2022 Dec 1. doi: 10.21470/1678-9741-2022-0008. Online ahead of print.

ABSTRACT

INTRODUCTION: The heart and liver are two organs that are closely related. The Albumin-Bilirubin (ALBI) score is a developed scoring system for assessing liver function. The aims of this study were to examine the correlation between preoperative ALBI score and pulmonary artery pressure and to investigate its ability to predict heart valve surgery mortality outcomes.

METHODS: The data of 872 patients who underwent isolated and combined heart valve surgery from 2014 to 2021 were retrospectively screened. In the preoperative period, 152 patients with laboratory tests including albumin and total bilirubin were found and analyzed retrospectively. Thirteen of these patients were excluded from the study. The remaining 139 patients were included in the analysis. Baseline demographic data, echocardiography data, performance status, laboratory data, operative data, and postoperative status were collected. The optimal cutoff value of preoperative ALBI score was calculated.

RESULTS: The cutoff for ALBI scores was calculated as -2.44 to predict in-hospital mortality (sensitivity = 75.0%, specificity = 70%). Based on the cutoff value, 90 patients had a low ALBI score (≤ -2.44, 64.7%) and 49 patients had a high ALBI score (> -2.44, 35.3%). High ALBI score was associated with an increased incidence of acute kidney injury and in-hospital mortality, and a positive correlation was found between ALBI score and pulmonary artery pressure.

CONCLUSION: In patients with valvular surgery, high ALBI score was an independent prognostic factor of in-hospital mortality and acute kidney injury. It is easily measurable and a cost-effective way to predict mortality.

PMID:36459475 | DOI:10.21470/1678-9741-2022-0008

Braz J Cardiovasc Surg. 2022 Dec 1. doi: 10.21470/1678-9741-2022-0022. Online ahead of print.

ABSTRACT

INTRODUCTION: The objectives of this study were to investigate the main treatment strategies and long-term follow-up results of aortic dissection surgery after open-heart surgery (ADSOHS) and to analyze the risk factors that cause ADSOHS.

METHODS: One hundred thirty-seven patients with ADSOHS hospitalized in our hospital from January 2009 to December 2018 were selected as the research object. Long-term follow-up results, complications, mortality, and changes of cardiac function before and after operation were used to explore the value of Sun's operation.

RESULTS: The length of stay in intensive care unit of these 137 patients ranged from 9.5 to 623.75 hours (average of 76.41±97.29 hours), auxiliary ventilation time ranged from 6.0 to 259.83 hours (average of 46.16±55.59 hours), and hospital stay ranged from six to 85 days (average of 25.06±13.04 days). There were seven cases of postoperative low cardiac output, 18 cases of coma and stroke, and six cases of transient neurological dysfunction. A total of 33 patients died; 19 patients died during the perioperative period, 18 died during Sun's operation and one died during other operation; and 14 patients died during follow-up (January 2021), 12 cases of Sun's operation and two cases of other operations.

CONCLUSION: ADSOHS treatment strategy is of high application value, and the risk of neurological complications and mortality is low. The main risk factors are postoperative low cardiac output, coma, stroke, and transient neurological dysfunction. The extracorporeal circulation time is relatively long. Short- and long-term follow-up effects are good, and it is worthy of clinical promotion.

PMID:36459476 | DOI:10.21470/1678-9741-2022-0022

J Visc Surg. 2022 Nov 29:S1878-7886(22)00157-6. doi: 10.1016/j.jviscsurg.2022.11.001. Online ahead of print.

ABSTRACT

BACKGROUND: Diabetes mellitus may increase the risk of adverse perioperative outcomes and prolong hospital stay. An enhanced recovery program (ERP) reduces surgical stress and its metabolic consequences, so attenuating the impact of preoperative risk factors. We tested the hypothesis that diabetes would have only a minor impact on outcome after colorectal surgery with an ERP.

METHODS: The data for patients scheduled for colorectal surgery between 2015 and 2021, were analyzed (n=769). All the patients were managed with the same protocol. Demographic data, preoperative risk factors, postoperative complications, and length of stay were compared between patients with and without diabetes.

RESULTS: In all, 124 patients (16.1%) had diabetes, of whom 30 (24.1%) required insulin. The following preoperative risk factors for postoperative complications were significantly more frequent in the patients with diabetes: age>70 years, ASA score ≥ III, renal failure, cardiac disease, BMI>30 kg/m2, anemia, and cancer as indication for surgery. Despite more risk factors, patients with diabetes did not experience more overall postoperative complications than controls (OR (95%IC): 0.9 [0.6-1.5], p=0.85). Length of hospital stay was not significantly longer in patients with diabetes than in those without (4 [2-7] vs. 3 [2-7] days; p=0.45).

CONCLUSIONS: Despite more risk factors, patients with diabetes did not experience more complications or longer length of stay after colorectal surgery with an ERP. The multimodal, multidisciplinary approach of ERP to reducing surgical stress may thus help mitigate the reported deleterious effects of diabetes.

PMID:36460550 | DOI:10.1016/j.jviscsurg.2022.11.001

Semin Fetal Neonatal Med. 2022 Dec;27(6):101409. doi: 10.1016/j.siny.2022.101409. Epub 2022 Nov 25.

NO ABSTRACT

PMID:36456434 | DOI:10.1016/j.siny.2022.101409

Ulus Travma Acil Cerrahi Derg. 2022 Dec;28(12):1747-1753. doi: 10.14744/tjtes.2021.49383.

ABSTRACT

Mediastinal masses can compress the respiratory or cardiovascular system, especially when anteriorly located. Obtaining histological material for diagnosis poses a challenge due to the major risk of cardiorespiratory collapse following anesthetic procedure. Our case shows the utility of rescue with venovenous extracorporeal membrane oxygenation (VV-ECMO) after occurrence of such an event and demonstrates the feasibility of administering chemotherapy during VV-ECMO. A 4-year-old boy was referred to the pediatric oncology clinic of our hospital after a large mediastinal mass was observed on chest radiography ordered due to persistent cough. Computed tomography of the thorax revealed a 100×85 mm mass in the anterior mediastinum, surrounding the heart, and showed that there was compression to the trachea, bronchiole, and vascular structures. Percutaneous needle biopsy accompanied by ultrasonography was planned for diagnostic purposes. Low-dose ketamine and midazolam were administered for procedural sedation in the operating room. After the biopsy procedure, the patient developed sudden airway obstruction requiring intubation. Despite 100% oxygen support with a mechanical ventilator, pulse oximeter saturation remained below 80%. Chest X-ray revealed total collapse of the left lung, and the patient's oxygen saturation did not increase with selective left bronchial intubation. Bi-caval dual-lumen ECMO cannula was placed in the internal jugular vein and VV-ECMO was initiated, resulting in swift improvement in hypoxemia. The patients's anterior mediastinal mass shrank rapidly and left lung improved with chemotherapy. The patient remained on ECMO for a total of 9 days and was extubated 2 days after ECMO termination, followed by discharge to the pediatric oncology ward on the 20th day of pediatric intensive care unit stay. It is well known that large, anteriorly-located mediastinal masses carry a considerable risk of causing cardio-pulmonary collapse during procedures involving anesthesia. All life-saving options, including emergency ECMO, should be available before any planned invasive procedures in these patients.

PMID:36453783 | DOI:10.14744/tjtes.2021.49383

Herz. 2022 Nov 30. doi: 10.1007/s00059-022-05150-6. Online ahead of print.

ABSTRACT

The new European Society of Cardiology (ESC) guidelines on the cardiovascular assessment and management of patients undergoing non-cardiac surgery were published in August 2022. In the preparation of the new document the previous guidelines published in 2014 were completely revised and the recommendations for action were adapted or renewed. Furthermore, the guidelines have been supplemented with some new chapters. The new and revised recommendations result in significant changes for the clinical practice. This particularly applies to the preoperative risk stratification, the perioperative risk management and the detection and management approaches in cases of perioperative and postoperative complications. Cardiovascular biomarkers play a special role in both the preoperative risk stratification and the detection of postoperative complications in combination with appropriate algorithms for action. The perioperative management of antithrombotic treatment (antiplatelet therapy or oral anticoagulation) is becoming increasingly individualized depending on the risk of ischemia or bleeding of the planned surgery. Particular attention is paid to the early detection of perioperative or postoperative myocardial infarctions based on determination of high-sensitivity (hs) cardiac troponin, not least because its association with high risk of morbidity and mortality. In patients with complex cardiovascular diseases, such as severe coronary artery disease, valvular heart diseases or those with ventricular support systems, the decision in an interdisciplinary team is highly recommended.

PMID:36449042 | DOI:10.1007/s00059-022-05150-6

Int Heart J. 2022;63(6):1187-1193. doi: 10.1536/ihj.22-237.

ABSTRACT

The combination of venoarterial extracorporeal membrane oxygenation (VA-ECMO) and Impella, referred to as ECPELLA, is a powerful transient mechanical circulatory support for patients with severe cardiogenic shock (CS). During ECPELLA support, VA-ECMO loads the left ventricle (LV) and Impella unloads the LV. Therefore, evaluating the degree of LV unloading during ECPELLA may be a prerequisite to protect the injured myocardium. Here we report a patient with CS due to an inferior ST-elevation myocardial infarction in which the degree of LV unloading on ECPELLA was confirmed by direct LV pressure (LVP) measurement. After the percutaneous coronary intervention for the right coronary artery on ECPELLA, the aortic pressure became nonpulsatile and the peak systolic LVP was reduced at approximately 10 mmHg with 20 mA of the Impella motor current (MC) amplitude, which we referred to as the total LV unloading condition. We maintained the condition in the early phase of ECPELLA by monitoring the Impella MC amplitude at 20 mA and less with nonpulsatile aortic pressure. The patient was successfully weaned off VA-ECMO on day 3, and Impella was explanted on day 8. Prior to the Impella explant, the Impella MC amplitude increased more than 100 mA and the estimated pressure gradient between the aortic pressure and LVP was well matched with the directly measured LVP. In this case, the patient was successfully treated by ECPELLA with the total LV unloading condition, and we showed that the degree of LV unloading on ECPELLA can be estimated from the aortic pressure and Impella MC amplitude at given Impella flows.

PMID:36450558 | DOI:10.1536/ihj.22-237

Anaesthesiologie. 2022 Dec;71(12):967-982. doi: 10.1007/s00101-022-01230-8. Epub 2022 Nov 30.

ABSTRACT

Extracorporeal support systems for the heart and lungs are employed for cardiac, pulmonary and also cardiopulmonary failure; however, neither the pure lung support by venovenous extracorporeal membrane oxygenation (vvECMO) nor the venoarterial (va) ECMO behave in a hemodynamically inert manner with respect to the patient's own cardiovascular system. The success of ECMO treatment is decisively dependent on monitoring before and during the execution and the pathophysiological understanding of the hemodynamic changes that occur during treatment. This article explicitly elucidates these "concomitant phenomena" and discusses fundamental aspects of cardiovascular physiology and the specific interplay with ECMO treatment.

PMID:36449054 | PMC:PMC9709734 | DOI:10.1007/s00101-022-01230-8

Cochrane Database Syst Rev. 2022 Nov 30;11(11):CD013494. doi: 10.1002/14651858.CD013494.pub2.

ABSTRACT

BACKGROUND: Cardiac surgery is performed worldwide. Most types of cardiac surgery are performed using cardiopulmonary bypass (CPB). Cardiac surgery performed with CPB is associated with morbidities. CPB needs an extracorporeal circulation that replaces the heart and lungs, and performs circulation, ventilation, and oxygenation of the blood. The lower limit of mean blood pressure to maintain blood flow to vital organs increases in people with chronic hypertension. Because people undergoing cardiac surgery commonly have chronic hypertension, we hypothesised that maintaining a relatively high blood pressure improves desirable outcomes among the people undergoing cardiac surgery with CPB.

OBJECTIVES: To evaluate the benefits and harms of higher versus lower blood pressure targets during cardiac surgery with CPB.

SEARCH METHODS: We used standard, extensive Cochrane search methods. The latest search of databases was November 2021 and trials registries in January 2020.

SELECTION CRITERIA: We included randomised controlled trials (RCTs) comparing a higher blood pressure target (mean arterial pressure 65 mmHg or greater) with a lower blood pressure target (mean arterial pressure less than 65 mmHg) in adults undergoing cardiac surgery with CPB.

DATA COLLECTION AND ANALYSIS: We used standard Cochrane methods. Primary outcomes were 1. acute kidney injury, 2. cognitive deterioration, and 3. all-cause mortality. Secondary outcomes were 4. quality of life, 5. acute ischaemic stroke, 6. haemorrhagic stroke, 7. length of hospital stay, 8. renal replacement therapy, 9. delirium, 10. perioperative transfusion of blood products, and 11. perioperative myocardial infarction. We used GRADE to assess certainty of evidence.

MAIN RESULTS: We included three RCTs with 737 people compared a higher blood pressure target with a lower blood pressure target during cardiac surgery with CPB. A high blood pressure target may result in little to no difference in acute kidney injury (risk ratio (RR) 1.30, 95% confidence interval (CI) 0.81 to 2.08; I² = 72%; 2 studies, 487 participants; low-certainty evidence), cognitive deterioration (RR 0.82, 95% CI 0.45 to 1.50; I² = 0%; 2 studies, 389 participants; low-certainty evidence), and all-cause mortality (RR 1.33, 95% CI 0.30 to 5.90; I² = 49%; 3 studies, 737 participants; low-certainty evidence). No study reported haemorrhagic stroke. Although a high blood pressure target may increase the length of hospital stay slightly, we found no differences between a higher and a lower blood pressure target for the other secondary outcomes. We also identified one ongoing RCT which is comparing a higher versus a lower blood pressure target among the people who undergo cardiac surgery with CPB.

AUTHORS' CONCLUSIONS: A high blood pressure target may result in little to no difference in patient outcomes including acute kidney injury and mortality. Given the wide CIs, further studies are needed to confirm the efficacy of a higher blood pressure target among those who undergo cardiac surgery with CPB.

PMID:36448514 | PMC:PMC9709767 | DOI:10.1002/14651858.CD013494.pub2

J Interv Cardiol. 2022 Nov 7;2022:8167011. doi: 10.1155/2022/8167011. eCollection 2022.

ABSTRACT

OBJECTIVES: To compare two different forms of mechanical circulatory support (MCS) in patients with complex high-risk indicated PCI (CHIP): the Impella CP system and veno-arterial extracorporeal membrane oxygenation (VA-ECMO).

BACKGROUND: To prevent hemodynamic instability in CHIP, various MCS systems are available. However, comparable data on different forms of MCS are not at hand.

METHODS: In this multicenter observational study, we retrospectively evaluated all CHIP procedures with the support of an Impella CP or VA-ECMO, who were declined surgery by the heart team. Major adverse cardiac events (MACE), mortality at discharge, and 30-day mortality were evaluated.

RESULTS: A total of 41 patients were included, of which 27 patients were supported with Impella CP and 14 patients with VA-ECMO. Baseline characteristics were well-balanced in both groups. No significant difference in periprocedural hemodynamic instability was observed between both groups (3.7% vs. 14.3%; p = 0.22). The composite outcome of MACE showed no significant difference (30.7% vs. 21.4%; p = 0.59). Bleeding complications were higher in the Impella CP group, but showed no significant difference (22.2% vs. 7.1%; p = 0.22) and occurred more at the non-Impella access site. In-hospital mortality was 7.4% in the Impella CP group versus 14.3% in the VA-ECMO group and showed no significant difference (p = 0.48). 30-Day mortality showed no significant difference (7.4% vs. 21.4%; p = 0.09).

CONCLUSIONS: In patients with CHIP, there were no significant differences in hemodynamic instability and overall MACE between VA-ECMO or Impella CP device as mechanical circulatory support. Based on this study, the choice of either VA-ECMO or Impella CP does not alter the outcome.

PMID:36447936 | PMC:PMC9663242 | DOI:10.1155/2022/8167011

BMJ Open. 2022 Nov 29;12(11):e064861. doi: 10.1136/bmjopen-2022-064861.

ABSTRACT

INTRODUCTION: Atrial fibrillation (AF) is common in patients with rheumatic mitral valve disease (RMVD) and increase the risk of stroke and death. Bi-atrial or left atrial ablation remains controversial for treatment of AF during mitral valve surgery. The study aims to compare the efficacy and safety of bi-atrial ablation with those of left atrial ablation among patients with RMVD and persistent or long-standing persistent AF.

METHODS AND ANALYSIS: The ABLATION trial (Bi-atrial vs Left Atrial Ablation for Patients with RMVD and Non-paroxysmal AF) is a prospective, multicentre, randomised controlled study. The trial will randomly assign 320 patients with RMVD and persistent or long-standing persistent AF to bi-atrial ablation procedure or left atrial ablation procedure in a 1:1 randomisation. The primary end point is freedom from documented AF, atrial flutter or atrial tachycardia of >30 s at 12 months after surgery off antiarrhythmic drugs. Key secondary end point is the probability of freedom from permanent pacemaker implantation at 12 months after surgery. Secondary outcomes include the probability of freedom from any recurrence of atrial tachyarrhythmias with antiarrhythmic drugs, AF burden, incidence of adverse events and cardiac function documented by echocardiography at 12 months after operation.

ETHICS AND DISSEMINATION: The central ethics committee at Fuwai Hospital approved the ABLATION trial. The results of this study will be disseminated through publications in peer-reviewed journals and conference presentations.

TRIAL REGISTRATION NUMBER: NCT05021601.

PMID:36446460 | PMC:PMC9710358 | DOI:10.1136/bmjopen-2022-064861

Int J Cardiovasc Imaging. 2022 Dec;38(12):2645-2653. doi: 10.1007/s10554-022-02673-1. Epub 2022 Jul 3.

ABSTRACT

The impact of "downstream" pathophysiological cardiac consequences in aortic regurgitation patients were not well established. The aim of our study was to validate a staging system built for severe aortic stenosis in a large real-world cohort of aortic regurgitation (AR) patients, evaluating the prevalence of different stages of cardiac damage and assess its prognostic impact. Clinical, echocardiographic and outcome data of patients with moderate-severe AR who underwent transthoracic echocardiography between January/2014 and September/2019 were retrospectively analysed. Patients were classified according to the extent of cardiac damage: Stage 0 (no cardiac damage), Stage 1 (left ventricular damage), Stage 2 (mitral valve or left atrial damage), Stage 3 (tricuspid valve or pulmonary artery vasculature damage) and Stage 4 (right ventricular damage). The primary endpoint was all-cause mortality. A total of 571 patients (median age 73, 51% male) were enrolled: Stage 0 (14.0%), Stage 1 (21.5%), Stage 2 (49.2%), Stage 3 (12.3%) and Stage 4 (3.0%). Median follow-up time was 39.5 months (IQR 22.2 to 61.0). At the end of follow-up, cumulative death was significantly higher in more advanced disease stages (log-rank p < 0.001). On multivariable analysis, Stage 3-4 was associated with increased risk of all-cause mortality (HR 3.20; 95% CI 1.48-6.93; p = 0.003). Our study suggests that extra-valvular damage is common in patients with significant AR and that a staging system developed for aortic stenosis also provides prognostic information in these patients. This staging system may be helpful for clinical decision-making regarding the time of valvular intervention.

PMID:36445657 | DOI:10.1007/s10554-022-02673-1

Int J Cardiovasc Imaging. 2022 Dec;38(12):2635-2644. doi: 10.1007/s10554-022-02669-x. Epub 2022 Jul 1.

ABSTRACT

Few studies analyzed left atrial (LA) peak atrial longitudinal strain (PALS) determinants, particularly across heart failure (HF) stages. We aimed to analyze the pathophysiological and clinical PALS correlates in a large multicentric prospective study. This is a multicenter prospective observational study enrolling 745 patients with HF stages. Data included PALS and left ventricular global longitudinal strain (LV-GLS). Exclusion criteria were: valvular prosthesis; atrial fibrillation; cardiac transplantation; poor acoustic window. Median global PALS was 17% [24-32]. 29% of patients were in HF-stage 0/A, 35% in stage-B, and 36% in stage-C. Together with age, the echocardiographic determinants of PALS were LA volume and LV-GLS (overall model R2 = 0.50, p < 0.0001). LV-GLS had the strongest association with PALS at multivariable analysis (beta: -3.60 ± 0.20, p < 0.0001). Among HF stages, LV-GLS remained the most important PALS predictor (p < 0.0001) whereas age was only associated with PALS in lower HF-stage 0/A or B (R = - 0.26 p < 0.0001, R = - 0.23 p = 0.0001). LA volume increased its association to PALS moving from stage 0/A (R = - 0.11; P = 0.1) to C (R = - 0.42; P < 0.0001). PALS was the single most potent echocardiographic parameter in predicting the HF stage (AUC for B vs. 0/A 0.81, and AUC vs. 0/A for C 0.76). PALS remained independently associated with HF stages after adjusting for ejection fraction, E/e' ratio, and mitral regurgitation grade (p < 0.0001). Although influenced by LV-GLS and LA size across HF stages, PALS is incrementally and independently associated with clinical status. LA function may reflect a substantial part of the hemodynamic consequences of ventricular dysfunction.

PMID:36445656 | PMC:PMC9708811 | DOI:10.1007/s10554-022-02669-x

Arch Argent Pediatr. 2022 Dec 1:e202202568. doi: 10.5546/aap.2022-02568.eng. Online ahead of print.

ABSTRACT

Introduction. Complex congenital heart defects are the most frequent malformations and entail a significant burden of disease. The objective of this study was to determine the health status and disability of children who underwent surgery at a tertiary care hospital. Population and methods. A total of 84 patients aged 21 to 39 months who had a surgery with extracorporeal circulation during their first year of life were assessed in terms of growth, neurodevelopment, and disability. Results. In most children, growth was below the 50 th percentile in all 3 parameters, and greater involvement was observed in those with an associated genetic disorder. The frequency of disability was 55%. In the group with isolated congenital heart disease, the severity of disease and a pathological neurological examination at discharge were associated with disability (p = 0.047 and p = 0.03). Having only public health coverage was associated with less access to timely interventions (p = 0.02). Conclusions. Nearly half of the patients develop moderate-severe disability. Being aware of morbidities beyond the cardiovascular aspect and risk factors is part of the health care team's scope. Barriers in access to appropriate interventions caution health care providers of the relevance of seeking strategies to overcome them and achieve the maximum development potential of patients.

PMID:36445172 | DOI:10.5546/aap.2022-02568.eng

J Med Case Rep. 2022 Nov 29;16(1):447. doi: 10.1186/s13256-022-03644-9.

ABSTRACT

BACKGROUND: Methotrexate is an antifolate antimetabolite that inhibits the activity of dihydrofolate reductase by acting as a false substrate, which leads to defects of DNA synthesis, specifically the inhibition of purine and pyrimidine synthesis. Thus, methotrexate is a powerful agent for treating autoimmune diseases and cancer. In general, methotrexate is thought to be cardioprotective and reports of methotrexate-induced cardiomyopathy are rare. We present a case of methotrexate-induced severe cardiotoxicity diagnosed by exclusion of all other potential causes.

CASE PRESENTATION: The patient was a 54-year-old Caucasian man presenting to an outside hospital with a chief complaint of abdominal pain and bloating who reported taking methotrexate up to 20 mg per week for systemic sclerosis. After a transthoracic echocardiogram found a left ventricular ejection fraction of 10% and coronary catheterization demonstrated no significant disease, he was transferred to our hospital for advanced heart failure therapies. His condition deteriorated, and he was eventually placed on veno-arterial extracorporeal membrane oxygenation. Owing to a lack of an identifiable etiology of cardiac failure, toxicology consultation recommended 24 hours of intravenous leucovorin therapy to overcome any residual and potentially cardiotoxic methotrexate still in his system. Over the next 5 days, his cardiac function improved daily, such that on day 5 of extracorporeal membrane oxygenation, he had a left ventricular ejection fraction of 40% and was able to be decannulated. Two days later, his ejection fraction improved to 60% and normal right ventricular function. Initially, his renal function improved while on extracorporeal membrane oxygenation, but over the next week deteriorated such that he required intermittent hemodialysis until hospital discharge.

CONCLUSIONS: After a process of elimination, the most likely cause of this patient's acute decline and rapid recovery of bi-ventricular function was methotrexate toxicity. Leucovorin may have aided the reversal of methotrexate toxicity.

PMID:36443884 | PMC:PMC9707053 | DOI:10.1186/s13256-022-03644-9

Eur J Cardiothorac Surg. 2022 Dec 2;63(1):ezac539. doi: 10.1093/ejcts/ezac539.

ABSTRACT

OBJECTIVES: During mitral valve replacement, the anterior mitral leaflet is usually resected or modified. Anterior leaflet splitting seems the least disruptive modification. Reattachment of the modified leaflet to the annulus reduces the annulopapillary distance. The goal of this study was to quantify the acute effects on left ventricular function of splitting the anterior mitral leaflet and shortening the annulopapillary distance.

METHODS: In 6 adult sheep, a wire was placed around the anterior leaflet and exteriorized through the left ventricular wall to enable splitting the leaflet in the beating heart. Releasable snares to reduce annulopapillary distance were likewise positioned and exteriorized. A mechanical mitral prosthesis was inserted to prevent mitral incompetence during external manipulations of the native valve. Instantaneous changes in left ventricular function were recorded before and after shortening the annulopapillary distance, then before and after splitting the anterior leaflet.

RESULTS: After splitting the anterior leaflet, preload recruitable stroke work, stroke work, stroke volume, cardiac output, left ventricular end systolic pressure and mean pressure were significantly decreased by 26%, 23%, 12%, 9%, 15% and 11%, respectively. Shortening the annulopapillary distance was associated with significant decreases in the end systolic pressure volume relationship, preload recruitable stroke work, stroke work and left ventricular end systolic pressure by 67%, 33%, 15% and 13%, respectively. Shortening the annulopapillary distance after splitting the leaflet had no significant effect.

CONCLUSIONS: Splitting the anterior mitral leaflet acutely impaired left ventricular contractility and haemodynamics in an ovine model. Shortening the annulopapillary distance after leaflet splitting did not further impair left ventricular function.

PMID:36440952 | DOI:10.1093/ejcts/ezac539

Int J Cardiol Congenit Heart Dis. 2023 Mar;11:100428. doi: 10.1016/j.ijcchd.2022.100428. Epub 2022 Nov 21.

ABSTRACT

BACKGROUND: At the beginning of the COVID-19 pandemic, professionals in charge of particularly vulnerable populations, such as adult congenital heart disease (ACHD) patients, were confronted with difficult decision-making. We aimed to assess changes in risk stratification and outcomes of ACHD patients suffering from COVID-19 between March 2020 and April 2021.

METHODS AND RESULTS: Risk stratification among ACHD experts (before and after the first outcome data were available) was assessed by means of questionnaires. In addition, COVID-19 cases and the corresponding patient characteristics were recorded among participating centres. Predictors for the outcome of interest (complicated disease course) were assessed by means of multivariable logistic regression models calculated with cluster-robust standard errors. When assessing the importance of general and ACHD specific risk factors for a complicated disease course, their overall importance and the corresponding risk perception among ACHD experts decreased over time. Overall, 638 patients (n = 168 during the first wave and n = 470 during the subsequent waves) were included (median age 34 years, 52% women). Main independent predictors for a complicated disease course were male sex, increasing age, a BMI >25 kg/m2, having ≥2 comorbidities, suffering from a cyanotic heart disease or having suffered COVID-19 in the first wave vs. subsequent waves.

CONCLUSIONS: Apart from cyanotic heart disease, general risk factors for poor outcome in case of COVID-19 reported in the general population are equally important among ACHD patients. Risk perception among ACHD experts decreased during the course of the pandemic.

PMID:36440468 | PMC:PMC9678209 | DOI:10.1016/j.ijcchd.2022.100428

Front Cardiovasc Med. 2022 Nov 9;9:985644. doi: 10.3389/fcvm.2022.985644. eCollection 2022.

ABSTRACT

Transapical Neochord mitral valve repair has been proven to be a technically safe procedure to correct primary mitral regurgitation (MR). Recurrent MR due to ruptured artificial chords is rare. Here, we present 2 cases of recurrent severe MR due to the detached or partially ruptured artificial chords after the Neochord procedure.

PMID:36440013 | PMC:PMC9682110 | DOI:10.3389/fcvm.2022.985644