Coronary Artery Abnormalities Research Articles

Intravenous immunoglobulin for the treatment of Kawasaki disease.

Intravenous immunoglobulin for the treatment of Kawasaki disease.

Cardiac MRI with late gadolinium enhancement shows cardiac involvement 3-6 months after severe acute COVID-19 similar to or worse than PIMS.

Coronavirus disease 2019 (COVID-19) in children is rarely severe. However, severe courses occur, especially in the presence of risk factors. A minority of children develop pediatric inflammatory multisystem syndrome (PIMS) with substantial morbidity. While the importance of cardiac involvement after PIMS is well established, its role after severe acute COVID-19 remains unclear. We aim to compare cardiac sequelae of children after severe acute COVID-19 using cardiac MRI and compare them with patients after PIMS. For this prospective cohort study, we recruited patients with acute COVID or PIMS in a single center. Clinical follow-up, lab work, ECG, and echocardiography were done within 2 days after disease onset and 3-6 months after discharge. At the last visit 3-6 months later, cardiac MRI (CMR) with late gadolinium enhancement (LGE) was performed to evaluate cardiac sequelae and compare both groups. Data were obtained from n = 14 patients with PIMS and n = 7 patients with severe acute COVID-19. At the start of the respective disease, left ventricular (LV) ejection fraction was reduced in seven patients with PIMS but none in the acute COVID-19 group. Transient mitral valve insufficiency was present in 38% of patients, of whom PIMS accounted for 7/8 cases. Eight patients (38%) with PIMS presented coronary artery abnormalities, with normalization in 7/8 patients. A significant decrease in LV mass index 3-6 months after disease onset was observed in both groups. MRI follow-up revealed non-ischemic myocardial pattern of LGE in 12/21 patients- in all (6/6) after severe acute COVID-19 and in less than half (6/14) after PIMS. Normal body weight-adjusted stroke volumes and end-diastolic volumes were found in 20/21 patients. We show that children suffering from severe acute COVID-19 have a similar, or worse, cardiac risk profile as patients with PIMS. Both patient groups should therefore receive close pediatric cardiac follow-up examinations. Cardiac MRI is the technique of choice, as most patients presented with delayed LGE as a sign of persistent cardiac injury despite normalization of laboratory and echocardiographic findings.

Clinical profile and outcome of multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 infection: a single-center observational study from South India

BackgroundMultisystem inflammatory syndrome in children (MIS-C) is a post-infectious sequelae of acute COVID-19 infection affecting children. This study was done over a period of 12 months from December 2020 to November 2021 to describe the clinical presentation, laboratory abnormalities, and outcome of children with MIS-C.MethodsSeventy-eight children below 12 years of age who satisfied the WHO diagnostic criteria for MIS-C were included in the study. Clinical parameters were recorded at admission. Relevant laboratory investigations, radiological studies, and outcome were documented.ResultsThe most commonly affected age group was 6–12 years with a female predominance. COVID RTPCR was negative in all patients. Most cases presented 2–6 weeks after the onset of acute COVID-19 infection. Lethargy, poor feeding, vomiting, abdominal pain, loose stools, cough, and cold are common symptoms of MIS-C syndrome in children and the common signs were rash, conjunctival congestion, hypotension, tachycardia, tachypnea, and hypoxemia. Gastrointestinal system was the commonly affected followed by the hepatic, renal, and cardiovascular systems. Coronary artery abnormalities were seen in 20% of cases. IVIg was the mainstay of therapy used in 95% of patients. Mortality was 1.3%. Cases responded well to IVIg and steroids.ConclusionOverall, the short-term outcome was favorable with low mortality in our study cohort. One-fifth of children had coronary artery abnormalities during acute phase underscoring the need for long-term follow-up.

Atypical Kawasaki disease presenting with macroscopic hematuria in an infant: a case report

BackgroundKawasaki disease is an acute febrile condition in children. It affects mainly children under 5 years old, and is known to cause coronary artery abnormalities if treatment is delayed. The diagnosis rests mainly on clinical criteria. However, it is also known that some infants do not have diagnostic criteria sufficient enough for the diagnosis of Kawasaki disease. Further, children may rarely present with unusual features, and this entity is recognized as “Atypical Kawasaki disease.”Case presentationWe present the case of a 9-month-old Tamil boy who presented with sterile gross hematuria in association with prolonged fever, lymphadenopathy, and generalized maculopapular rash. He had high inflammatory markers and echocardiogram disclosed left coronary artery dilatation. The diagnosis of incomplete Kawasaki disease was confirmed based on clinical grounds supported by investigations and exclusion of differential diagnosis. The child showed a good response to intravenous immunoglobulin and aspirin.ConclusionKawasaki disease is one of the important differential diagnoses of protracted fever of unknown origin in very young children. Since delayed treatment is associated with a high risk of complications, atypical Kawasaki disease needs to be suspected in children presenting with unusual features such as macroscopic hematuria that occurs in association with unexplained prolonged fever.

Right Coronary Artery Origin from Left Sinus of Valsalva with an Inter-arterial Course: Case Report and Review of Literature

Introduction: Overally the anomaly of the coronary arteries are rare. Its incidence according to angiographic, autopsy and echocardiographic findings, are about 1%, 0.29% and less than 0.1% respectively. Usually these are the incidental finding during angiography without clinical presentation. These anomalies may be accompanied by myocardial ischemia, arrhythmia, syncope and sudden death. Case Presentation: We present a 14-year-old Iranian boy who experienced chest pain and shortness of breath after engaging in physical activity. The right coronary artery (RCA) had an intramural and inter-arterial course and was proven to have originated from the left sinus of Valsalva by computed tomographic coronary angiography (CTCA). The RCA began adjunct to the ostium of the left main coronary artery and coursed intramural at first and then continued between the aorta and pulmonary artery. The patient underwent corrective surgery. The RCA was cut from origin and reanastomosd to the right coronary sinus after preparing the sinus. The patient recovered well and he was discharged with the ability to perform sports activity. One year Follow up of the patient was uneventful. Conclusions: Even though single ostium coronary artery abnormalities are uncommon, they have become more frequently identified in recent years using non-invasive techniques such transthoracic echocardiography, which is then followed by CT angiography or conventional angiography. These patients are susceptible to sudden death, so attention to symptoms of the patients and comprehensive approach to them can be lifesaving.

Prednisolone versus cyclosporine as initial treatment for Kawasaki disease.

Several studies have demonstrated the efficacy of prednisolone and cyclosporine as initial combination treatments for the prevention of coronary artery abnormalities (CAA) in patients with Kawasaki disease. However, whether prednisolone or cyclosporine results in superior clinical outcomes is unknown. Thus, this study aimed to compare the outcomes of these two treatments. Using the Japanese Diagnosis Procedure Combination database, we identified patients with Kawasaki disease who had received prednisolone or cyclosporine in addition to initial intravenous immunoglobulin treatment between April 2014 and March 2021. The primary outcome was the proportion of CAA; secondary outcomes included intravenous immunoglobulin resistance, medical costs, and length of hospital stay. Propensity score matching was conducted to compare outcomes between the two groups. We identified 6288 patients with Kawasaki disease who had received prednisolone (n = 6147) or cyclosporine (n = 141) as an initial treatment in combination with intravenous immunoglobulin. Four-to-one propensity score-matched analysis demonstrated no significant difference in the proportion of CAA (0.7% vs. 2.8%; p = 0.098), intravenous immunoglobulin resistance, or medical costs between the treatment groups. The length of hospital stay was significantly longer in the prednisolone group (14 vs. 11 days, p < 0.001). Prednisolone and cyclosporine used in the initial combination treatment for Kawasaki disease showed similar clinical outcomes regarding the risk of CAA, intravenous immunoglobulin resistance, and medical costs, whereas the length of hospital stay was longer in the prednisolone group than in the cyclosporine group.

Prevalence and Pattern of Congenital Coronary Artery Anomalies in Patients Undergoing Coronary Angiography at a Tertiary Care Hospital

Objective: This research aimed to characterize the clinical presentation, frequency, and distribution of CAAs in people experiencing angiography. In this study, we reviewed the presence of CAAs in 4072 patients. Study design: Cross-section study Study Setting: Current research was conducted at Department of interventional cardiology, MTI- Hayat Abad Medical Complex, Peshawar from Ist July 2021 to 30th June 2022. Methods: The current research analyzed data from 4072 angiograms performed. The medical record of the patients was used to recruit the CAAs patient’s profile. Angelini's categorization was used to categorise the coronary anomalies and two interventional cardiologists reviewed each coronary angiography separately, and in the event of a discrepancy, a senior cardiologist was contacted to achieve a consensus. GE 520 and GE 2100 were used for the coronary angiogram. Operators have the option of using either a radial or femoral incision. Results: Finding of the CAAs was found in 103 cases of CAAs (a prevalence of 2.5%). According to the data, the average age of the patients was 59.64±13.73. Of them, 89 (2.19%) individuals had anomalous coronary origin and/or course, 9 (0.2%) had intrinsic coronary arterial system abnormalities, 3 (0.07%) had anomalous coronary termination, and 2 (0.04%) had a single coronary artery. Overall, 36 individuals (0.44%) were found to have an aberrant right coronary artery (RCA), with the RCA emerging from the left coronary sinus; this was followed by 54 patients (1.3%) who lacked a left main trunk and instead had a distinct origin for the LAD and LCX. Practical implication: Our understanding of CAAs has considerably improved from the development of novel diagnostic techniques, although the majority of the data available today comes from case reports and small case series. Further research is needed to fill the knowledge gap regarding the clinical effects of CAAs and their prognosis because epidemiological data are taken from studies conducted in other countries. Conclusion: Overall, 2.5 percent of research participants had congenital coronary abnormalities. The highest prevalence was found in anomalies origin and course. This study's prevalence of CAAs was comparable to that of other research, although the distribution of abnormalities was a little bit different. Keywords: Congenital Coronary Artery Anomalies, Coronary Angiography, Prevalence, Pattern

246. Cardiac involvement in children with multisystem inflammatory syndrome (MIS-C) following infection with different SARS-CoV-2 variants

Abstract Background MIS-C is a hyper inflammatory condition following SARS-CoV-2 infection. Although COVID-19 infection rates and severity have varied based on circulating SARS-CoV-2 variants, it is unclear if cardiac involvement in MIS-C varies following infection with different SARS-CoV-2 variants. The objective of this study is to describe the severity of cardiac involvement in children with MIS-C following three different waves of SARS-CoV-2 infections. Methods Children hospitalized with a diagnosis of MIS-C were enrolled in a prospective observational study. Demographic, clinical, laboratory (troponin I and B-type natriuretic peptide (BNP)), electrocardiogram (EKG) and echocardiogram (ECHO) data for children diagnosed between 4/20 and 12/21 and followed at 1- and 6-months was analyzed. The cohort was divided into 3 groups to represent cases that followed infection with the Wuhan (4/20-10/20, group 1), Alpha (B.1.1.7, 11/20-7/21, group 2) and Delta (B.1. 617.2, 8/21-12/21, group 3) variants. Cardiac involvement during hospitalization and follow-up was compared between the groups. Results The cohort includes 131 children with MIS-C (32, 61 and 38 in groups 1, 2 and 3, respectively) with a median age of 10 years. Two-thirds were male (66.4%) and 49.6% were Black. Elevated BNP and troponin I levels were seen in 82% and 52.7% of children at initial diagnosis. A third of the cohort had at least one abnormal EKG finding. The proportion of children with abnormal laboratory and EKG findings was not different between the groups. Decreased left ventricular function on ECHO was seen in 25% (33/131) of the cohort with similar distribution among the three groups (p = 0.79). Trivial-small pericardial effusions were detected in 22% (29/131). Coronary artery abnormalities were detected in 11.45% (15/131), a majority in group 1 (25%; 8/32). At 1- and 6-monthfollow-up visits, BNP and Troponin I were normal in all children. At the 6-month follow-up visit, EKG was normal in all and ECHO was normal in 37/41 children with trivial to mild valvular regurgitation in four children. Conclusion In this single center prospective study, while a significant proportion of children with MIS-C had evidence of cardiac involvement at diagnosis, most resolved on follow-up demonstrating good outcomes. Disclosures Claudette Poole, MD, Therapy Brands: Stocks/Bonds Scott H. James, MD, Bayer: Advisor/Consultant|Pantheon Biosciences: Grant/Research Support Suresh Boppana, MD, Merck, Inc: Advisor/Consultant|Merck, Inc: Grant/Research Support|Pfizer: Grant/Research Support.

1027 CONGENITAL CORONARY ARTERY ABNORMALITIES: THE DIAGNOSTIC AND PROGNOSTIC VALUE OF CARDIAC COMPUTED TOMOGRAPHY

Abstract Introduction To the best of our knowledge, there are no previous univocal data on how CCT can accurately describe morphological variants and subtypes of congenital coronary artery abnormalities. Taking into consideration the extensive application of CCT in clinical practice in the last decades, it is not uncommon to have a congenital coronary artery anomaly detected at CCT. Thus, the study aim is to evaluate the diagnostic and prognostic impact of specific cardiac CT parameters in subjects with a diagnosis of congenital coronary artery anomalies. Material and Methods This is an intermediate evaluation of a prospective clinical registry on a population of subjects who underwent a cardiac computed tomography imaging evaluation in the period between January 2007 and October 2015 and were diagnosed to have a congenital coronary abnormality. For the present preliminary analysis only coronary anomalies of origin have been considered and 92 patients have been included. Follow-up have been collected either through a structured telephonic interview or through the evaluation of clinical records of subsequent hospitalization or ambulatory visits. Results The population enrolled in the present study has an average age of 63,0 ± 12,8 years (range 34 to 85 years), with a male prevalence of 69,6%. The left main artery is missing in 46 individuals (50%), with separate origin of LAD and LCX. Only two duplications (2.2%), one for the left coronary artery and one for the right coronary artery, have been discovered. Six participants (6.5%) had a non-coronary artery origin abnormalities and pulmonary artery was the site of origin in the majority of cases. 51 subjects (55,4%) have an anomalous origin of the coronary artery from a different coronary sinus and 50 subjects had also an anomalous course with the retro-aortic being the most common (30,4%). 11 participants (12,0%) displayed also an intramural segment and 16 (17,4%) had abnormal ostial morphology. The high take-off of the vessel was uncommon (3,3%). Age appears to be the only relevant characteristic; indeed, it displays a significant correlation both to MACE (OR 95% CI 1.03, 1.01-1.07; p=0.0349) and to all-cause of death (OR 95% CI 1.14, 1.02-1.27; p=0.0151). None of the traditional cardiovascular risk factors were found to be significantly linked with adverse outcomes in this study sample of coronary anomalies. Conclusions The result of this intermediate evaluation is that cardiac CT can be successfully used to define the anatomy and features of CAA. However, it demonstrated that in middle-aged patients, the tomographic finding of an abnormality of coronary origin might not have a meaningful, strong negative prognostic value in terms of major cardiovascular events and all-cause of death. This means that probably, in the majority of the cases, once the diagnosis is performed later on in life, no further systematic assessment is needed but a personalized approach should be suggested.

Abstract 9501: Single-Cell Transcriptomic Analysis and Patient-Specific iPSCs Reveal Dysfunctional Coronary Arterial Endothelial Cells in Hypoplastic Left Heart Syndrome

Introduction: Hypoplastic left heart syndrome (HLHS) is a severe form of single ventricle congenital heart disease characterized by the underdeveloped left ventricle. Early serial postmortem examinations revealed a high rate of coronary artery abnormalities in HLHS fetal hearts (e.g., thickened wall and kinking arteries). However, the intrinsic defect in HLHS coronary vessels and its genetic basis remain unclear. Methods: We profiled human fetal heart with an underdeveloped left ventricle (ULV) and induced pluripotent stem cells derived endothelial cells (iPSC-ECs) from HLHS patients at single-cell resolution. CD144 + / NPR3 - vascular ECs were selected and classified as venous, arterial, and late arterial populations. To study the arterial EC phenotypes, we generated iPSC-arterial ECs (AECs, CDH5 + CXCR4 + NT5E -/low ) derived from 3 HLHS patients and 3 age-matched controls, and evaluated their functionalities including cell cycle regulation, angiogenesis, and inflammatory response. Results: Revealed by single cell RNA-seq and subsequent gene ontology analysis, ULV late arterial EC population showed significant defects in EC development, proliferation, angiogenesis, and Notch signaling compared to the control. Consistently, HLHS iPSCs exhibited impaired AEC differentiation judged by the reduced CXCR4 + NT5E -/low AEC progenitors. Mature HLHS iPSC-AECs showed reduced angiogenesis and enhanced G0/G1 cell cycle arrest with downregulated cell cycle-related genes (e.g., Ki67, CCND1/2 ). Healthy human aortic smooth muscle cells exhibited abnormal proliferation and synthetic phenotypes when co-cultured with HLHS iPSC-AECs. Additionally, NOTCH pathway genes (e.g., DLL4, HEY1, GJA5 ) were suppressed in both ULV AECs and HLHS iPSC-AECs. HLHS de novo variant KMT2D directly regulated the transcription of NOTCH targeted genes involved in arterial development and proliferation via H3K4me2. Intriguingly, the treatment of NOTCH ligands (Jag1, Dll1) significantly improved the proliferation of HLHS AECs. Conclusions: Our study revealed that HLHS coronary AECs were dysfunctional in angiogenesis, proliferation, and EC-SMC interaction. KMT2D-NOTCH signaling may contribute to the impaired development and proliferation of HLHS AECs.

Microvascular Dysfunction in Hypertrophic Cardiomyopathy.

Myocardial ischemia is an established pathophysiological feature of hypertrophic cardiomyopathy (HCM) that impacts various clinical features, including heart failure (HF) and sudden cardiac death (SCD). The major determinant of myocardial ischemia in HCM is coronary microvascular dysfunction (CMD) in the absence of epicardial coronary artery abnormalities. Despite the impossibility to directly visualize microcirculation in vivo, a multimodality approach can allow a detailed assessment of microvascular dysfunction and ischemia. Accordingly, the non-invasive assessment of CMD using transthoracic Doppler echocardiography, positron emission tomography, and cardiac magnetic resonance should now be considered mandatory in any HCM patient. Noteworthy, a complete diagnostic work-up for myocardial ischemia plays a major role in the approach of the patients with HCM and their risk stratification. Chronic and recurrent episodes of ischemia can contribute to fibrosis, culminating in LV remodeling and HF. Ischemia can potentially constitute an arrhythmic substrate and might prove to have an added value in risk stratification for SCD. Accordingly, strategies for the early diagnosis of CMD should now be considered an important challenge for the scientific community.

High-Dose or Low-Dose Aspirin Application in the Initial Phase of Kawasaki Disease: A Meta-Analysis and Systematic Review

The initial dose of aspirin for Kawasaki disease (KD) is controversial to a great extent. In order to compare the efficacy of high-dose and low-dose aspirin in the treatment of KD, this study included articles containing information on the treatment of KD with aspirin before August 2021, which were collected from public databases. The results of different studies were summarized and weighted by an inverse variance model, and heterogeneity was assessed using the Q-test and I2. A meta-analysis of 12258 patients from nine retrospective studies was conducted. In general, no significant differences between high-dose and low-dose groups were found in the incidence of coronary artery abnormality (CAA) (RR = 1.154; 95% CI = 1.027–1.316) and the incidence of intravenous immunoglobulin (RR = 0.926; 95% CI = 0.594–1.441). But high-dose aspirin might be linked to the shortened duration of fever (RR = −0.134; 95% CI = −0.203–0.064) as well as hospitalized stay (RR = −1.263; 95% CI = −1.392–1.122). Conclusion. For the treatment of the acute phase of KD, low-dose aspirin plus intravenous immunoglobulin could be as effective as high-dose aspirin in preventing CAA. Nonetheless, high-dose aspirin might be related to reducing fever time and hospitalization time.

Long-Term Cardiovascular Outcomes of Multisystem Inflammatory Syndrome in Children Associated with COVID-19 Using an Institution Based Algorithm.

Cardiovascular involvement is a major cause of inpatient and intensive care unit morbidity related to Multisystem inflammatory syndrome in children (MIS-C). The objective of this study was to identify long-term cardiovascular manifestations of MIS-C. We included 80 consecutive patients admitted to the intensive care unit with MIS-C who were evaluated for a year in our follow-up clinic using an institution protocol. The outcome measures were cardiac biomarkers (troponin and BNP), electrocardiogram changes, echocardiographic findings cardiovascular magnetic resonance (CMR) and graded-exercise stress test (GXT) findings. The cohort included patients aged between 6 months and 17 years (median 9 years; 48.8% females). At the peak of the disease 81.3% had abnormal BNP and 58.8% had troponin leak which reduced to 33.8% and 18.8% respectively at discharge with complete normalization by 6 weeks post-discharge. At admission 33.8% had systolic dysfunction, which improved to 11.3% at discharge with complete resolution by 2 weeks. Coronary artery abnormalities were seen in 17.5% during the illness with complete resolution by 2 weeks post discharge except one (1.9%) with persistent giant aneurysm at 1 year-follow up. CMR was performed at 6 months in 23 patient and demonstrated 4 patients with persistent late gadolinium enhancement (17.4%). Normal exercise capacity with no ectopy was seen in the 31 qualifying patients that underwent a GXT. There is significant heterogeneity in the cardiovascular manifestations of MIS-C. Although majority of the cardiovascular manifestations resolve within 6 weeks, diastolic dysfunction, CAA and myocardial scar may persist in a small subset of patients warranting a structured long-term follow-up strategy.

Association of Kawasaki Disease Shock Syndrome with Giant Coronary Artery Dilatation – Presentation of Two Cases and A Literature Review

Introduction Kawasaki disease is an acute, self-limited vasculitis of childhood characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develop in approximately 15 to 25% of untreated children with the disease and may lead to myocardial infarction, sudden death, or ischemic heart disease. Despite an overlap of clinical features with toxic shock syndrome, children with Kawasaki disease generally do not develop chock.&#x0D; Cases Here we report two old children who presented with a toxic shock-like illness, and were diagnosed with Kawasaki disease when coronary artery abnormalities were found on transthoracic echocardiography, recently reported as a “Kawasaki disease shock syndrome”. Relevant articles about KD and shock were collected and demographic data, clinical presentation, laboratory variables, echocardiogram findings, treatment and special features were analyzed when available. &#x0D; Conclusion Pediatrics cardiologist should be aware of this potentially severe form of manifestation of the Kawasaki disease.&#x0D; Key words Kawasaki disease, Kawasaki disease shock syndrome, coronary artery aneurysm, intravenous immunoglobulin,

Coronary artery aneurysms caused by Kawasaki disease in an adult: A case report and literature review

BACKGROUNDKawasaki disease (KD) is a self-limiting febrile illness and an acute vasculitis with an unknown origin. It predominantly affects children aged < 5 years. KD is the common cause of acquired heart disease in children. We here report a case of KD in an asymptomatic young female patient diagnosed with multiple coronary aneurysms with calcification. CASE SUMMARYA 29-year-old female patient admitted to Hangzhou First People's Hospital with coronary artery abnormality identified for 1 wk. The patient was asymptomatic; however, chest computed tomography occasionally revealed strip-like dense shadows in the coronal sulcus. After coronary angiography and Doppler echocardiography, the final diagnosis was coronary artery aneurysms (CAAs) caused by KD. Although the patient was asymptomatic with no history of KD in childhood, the definitive diagnosis was CAAs caused by KD. The patient was administered anticoagulant, and surgical treatment was recommended.CONCLUSIONKD potentially causes CAAs in 25% of untreated cases, primarily occurring in the proximal portions of the coronary arteries.

Cardiac resynchronization therapy: association between genetically determinated dilated cardiomyopathy and dissinchronopathy

Abstract Background Patients with non-ischemic dilated cardiomyopathy (DCM) and left bundle branch block (LBBB) often undergoes cardiac resynchronization therapy (CRT). Despite CRT only a small group of patients restore normal left ventricular geometry and function. Many predictors of response to CRT have been described, but there are still insufficient data on the role of genetic mutations in sarcomere, cytoskeletal or desmosomal genes. Purpose To asses the role of genetic background as predictor of response after CRT in patient DCM and LBBB. Methods We retrospectively analyzed DCM patients with successful CRT implantation and available genetic testing consecutively included in the CRT-Registry of our Institution. DCM was defined by the presence of left ventricular or biventricular dilatation and systolic dysfunction in the absence of abnormal loading conditions or coronary artery disease sufficient to cause global systolic impairment. Changes in main echocardiographic parameters: left ventricular ejection fraction (LVEF), LV End Systolic and End Diastolic Volume (LVESV and LVEDV), where compared between basal and 24–48 months of follow up. LV remodeling was defined by relative changes (Δ) in LV End Systolic Volume (LVESV) [(follow-up LVESV − PRE LVESV) / PRE LVESV × 100)]. Patients were considered super-responders (SR) if LVEF at 24–48 months was&amp;gt;50%. Genetic testing was done by Next Generation DNA and all variants were validated with bidirectional Sanger sequencing. Variants of uncertain significance (VUS) were excluded from the analysis. Patients being carrier of pathogenic (P) or likely pathogenic (LP) variants were considered affected by genetic DCM (GEN+ DCM). Patients non carrier of P/LP variants and with no evidence of familial disease were considered affected by non-genetically determined DCM (GEN− DCM). Results 73 patients met the inclusion criteria, 23 patients were GEN+ DCM, the remaining 50 patients had negative genetic test and no family history of DCM. Baseline characteristics were similar between groups. GEN− group was characterized by a greater improvement in LVEF (39±12 vs 29±12, p=0,006) and a greater relative reduction in LVESV (ΔLVESV 43% vs 18%, p=0,007). GEN− patients had a 33 ml higher mean LVESV reduction at 24–48 months after CRT, indipendently of their respective baseline values. 20 patients (27% of the total population) suffered of heart failure related death, heart transplantation or left ventricular assistance device implantation during follow up and the prevalence was significantly higher in GEN+ group (43% vs 20%, p=0,037). Coherently, patients in the GEN+ group showed a lower prevalence of super responders (4%, vs 24%, p=0,02) after CRT. Conclusions Non-genetically determined DCM DCM is associated with a better clinical and instrumental response to CRT and a higher probability of being super responders. Furthermore, our data support the hypothesis about dyssyncronopathy as a specific clinical entity inside DCM diseases. Funding Acknowledgement Type of funding sources: None.

The Value of Exercise Systolic Blood Pressure as an Indicator for Coronary Artery Disease in Normotensive Men

Abstract Aim: We investigated if rates of systolic blood pressure to workloads during exercise can independently predict coronary artery disease. Methods: 78 normotensive men with normal blood pressure who had exercise ECG stress test and a coronary angiography within 30 days were enrolled in the study. Basic features of all the patients were also compared. The rates of systolic blood pressure to workloads at the first stage, peak exercise, second, fourth and sixth minute of recovery were compared in normotensive men with both normal and abnormal coronary arteries. Statistical analysis: t test and stepwise regression was used. Results: The rates of systolic blood pressure to workloads were statistically importantly higher in men with normal blood pressure which had coronary artery disease compared to those with normal coronary arteries and normal blood pressure. Conclusions: In men with normal blood pressure high levels of systolic blood pressure to workload rates at the first stage to the end of exercise test can independently predict the presence of coronary artery disease, even in non-conclusive stress tests or when ECG doesn’t show any change during exercise.

Differences in Sensitivity Between the Japanese and Z Score Criteria for Detecting Coronary Artery Abnormalities Resulting from Kawasaki Disease.

No studies have assessed differences between the Japanese and Z score criteria in the echocardiographic detection sensitivity of coronary artery (CA) abnormalities using large-scale data containing samples from multiple facilities engaged in daily clinical practices of Kawasaki disease (KD). We analyzed data from the 25th Japanese nationwide KD survey, which identified 30,415 patients from 1357 hospitals throughout Japan during 2017-2018. Hospitals were classified according to their use of Z score criteria. We assessed differences in hospital and patient background factors and compared the prevalence of CA abnormalities among groups using the Z score criteria. Multivariable logistic regression analyses were performed to evaluate differences in the detection sensitivity for CA abnormalities. The Z score criteria were more likely to be utilized in larger hospitals with more pediatricians and cardiologists. Even after controlling for potential confounders, detection sensitivities by the Z score criteria were significantly higher than by the Japanese criteria in patients with CA dilatations (adjusted odds ratio (95% confidence interval) 1.77 (1.56-2.01)) and aneurysms (1.62 (1.17-2.24)). No significant difference was found in patients with giant CA aneurysms. Compared with the Japanese criteria, the Z score criteria were significantly more sensitive for detecting patients with CA dilatations regardless of age, and for those with CA aneurysms only in patients aged ≤ 1year. Our results indicate that differences in the detection sensitivity for CA abnormalities between the Z score and the Japanese criteria were dependent on the CA size and patient age.

Occlusion of the os of the left coronary artery by dysplastic aortic valve tissue presenting as progressive mitral insufficiency and cardiac arrest.

Mitral regurgitation in the neonatal period is relatively rare. It can be secondary to a congenital malformation of the valve apparatus or mitral valve dysfunction and deformation secondary to myocardial dysfunction or volume load of the left ventricle. Less commonly, it can be due to coronary artery abnormalities leading to mitral valve papillary muscle ischaemia and subsequent dysfunction. Such coronary artery abnormalities include anomalous left coronary artery from pulmonary artery, left main coronary artery atresia, or a thromboembolic phenomenon. In this study, we describe a newborn with a dysplastic aortic valve causing obstruction of the os of the left coronary artery leading to progressive mitral insufficiency.

Long-term vascular dysfunction in Kawasaki disease: systematic review and meta-analyses.

Long-term physiological dysfunction in coronary/systemic vasculature may persist in individuals with Kawasaki disease even in the absence of coronary artery abnormalities. We perform a systematic review and meta-analyses of studies assessing long-term vascular function in Kawasaki disease. PubMed, Embase, and Web of Science databases were searched for relevant literature published till May 2021. Patients with Kawasaki disease were included as cases and healthy age/sex-matched individuals as controls. Newcastle Ottawa Scale was used to assess the study quality. Outcome measures were differences in markers of vascular function 1 year after diagnosis of Kawasaki disease. Data were analysed using Review Manager software. Comprehensive meta-analysis software was used for meta-regression. To assess the certainty of evidence, GRADE Profiler software was utilised. Of 2280 citations, 49 case-control studies (comprising 2714 cases and 2118 controls) were included for data synthesis. Decreased flow-mediated dilatation [3.83, 95%CI 0.94-6.72] and increased pulse-wave velocity [39.34 cm/sec, 95%CI 20.86-57.83], arterial stiffness [0.35, 95%CI 0.11-0.59], and common carotid artery intima-media thickness were noted in patients with Kawasaki disease. No significant difference was observed for nitroglycerine-mediated dilatation and endothelial peripheral artery tonometry (endo-PAT). Significant inter-study heterogeneity was observed for flow-mediated dilatation, arterial stiffness, carotid artery intima-media thickness, and endo-PAT. The GRADE evidence was of 'very low quality' for all outcome measures except 'moderate quality' for pulse-wave velocity. Evidence suggests the presence of long-term endothelial dysfunction in patients with Kawasaki disease even in the absence of coronary artery abnormalities. Avoidance of development of other cardiovascular risk factors seems prudent in patients with Kawasaki disease.