UNASSIGNED: LAMA2-related muscular dystrophy (LAMA2-MD) and SELENON-related myopathy (SELENON-RM) are two rare neuromuscular diseases characterized by proximal and axial muscle weakness, scoliosis, spinal rigidity, low bone quality and respiratory impairment. Cardiac involvement has previously been described in retrospective studies and case reports, but large case series and prospective studies in unselected cohorts are lacking.
UNASSIGNED: The objective of this study is to conduct prevalence estimations, perform cardiac phenotyping, and provide recommendations for clinical care.
UNASSIGNED: In this case series including two time points, we conducted comprehensive assessments with electrocardiography (ECG) and transthoracic echocardiography (TTE). ECGs were systematically assessed for a large subset of variables. TTE included left and right ventricular ejection fraction (LVEF/RVEF) and left ventricular global longitudinal strain (GLS), the latter being a more early and sensitive marker of left ventricular dysfunction.
UNASSIGNED: 21 LAMA2-MD (M = 5; 20±14 years) and 10 SELENON-RM patients (M = 7; 18±12 years) were included. In most patients, QRS fragmentation and Q waves, markers of heterogeneous ventricular activation, were present both at baseline and at follow-up. GLS was abnormal (age specific in children, > -18% in adults) in 33% of LAMA2-MD and 43% of SELENON-RM patients at baseline. Reduced LVEF (<52% in males, <54% in females and <55% in pediatric population) was observed in three LAMA2-MD patients at baseline and in none of the SELENON-RM patients. GLS and LVEF did not change between baseline and follow-up. RVEF was normal in all patients.
UNASSIGNED: ECG abnormalities and abnormal GLS are prevalent in LAMA2-MD and SELENON-RM, yet abnormal LVEF was only seen in LAMA2-MD patients. One LAMA2-MD patient had a clinically relevant deterioration in LVEF during 1.5-year follow-up. We advise routine screening of all patients with LAMA2-MD or SELENON-RM with ECG and echocardiography at diagnosis, minimally every two years from second decade of life and if new cardiac signs arise.

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UNASSIGNED: Early biventricular dysfunction in repaired tetralogy of Fallot (TOF) children may lead to poor clinical outcomes. We aimed to assess biventricular function in TOF children before and after surgery by speckle tracking echocardiography (STE) and compare them with the controls.
UNASSIGNED: Twenty repaired TOF children and 20 normal children as controls were assessed by STE. Tricuspid annular plane systolic excursion (TAPSE), left ventricular ejection fraction (LVEF), biventricular strain, and strain rate were compared before and after surgery and between TOF children and controls.
UNASSIGNED: Postoperative LVEF (p = 0.001), strain (p = 0.001), and strain rate (p = 0.001) for left ventricle improved significantly compared to preoperative phase. However, postoperative left ventricular strain (p = 0.05) and strain rate (p = 0.01) in TOF children were significantly impaired compared to controls. Postoperative LVEF was correlated inversely with postoperative strain rate (r = -0.40, p = 0.04). Postoperative TAPSE (p = 0.001), strain (p = 0.001), and strain rate (p = 0.001) for right ventricle significantly worsened when compared with the preoperative phase. Moreover, postoperative TAPSE (p = 0.001), strain (p = 0.001), and strain rate (p = 0.01) were significantly impaired compared to controls. Postoperative right ventricular strain rate was correlated significantly with the weight of children (r = 0.48, p = 0.02), and postoperative left ventricular strain showed significant correlations with aortic clamp time (r = 0.44, p = 0.04) and with ICU stay (r = -0.46, p = 0.04).
UNASSIGNED: Despite normal LVEF, TOF children exhibit impaired left ventricular strain and strain rate after surgery. TAPSE, strain, and strain rate for the right ventricle worsen after surgical repair. STE-driven strain can be used to detect early ventricular dysfunction and the associated prognostic implications.

Takotsubo cardiomyopathy is characterized by transient regional systolic dysfunction of the left ventricle (LV) and mimics myocardial infarction. The LV displays a systolic apical ballooning appearance in this particular cardiomyopathy. This case demonstrated a patient with true stress-induced cardiomyopathy or \"broken heart,\" presenting to the emergency room with chest pain mimicking a non-ST elevation myocardial infarction.

Takotsubo cardiomyopathy (TCM) is a rare occurrence in patients with troponin-positive acute coronary syndrome (ACS). It usually manifests as transient apical ballooning of the left ventricle with concomitant occurrence of right ventricular involvement in only one-third of cases. Biventricular TCM is associated with more hemodynamic instability as compared to left sided alone. Depressed ventricular systolic function and localized ventricular dyskinesis can facilitate clot formation in ventricular cavity. We present a case of 80-year-old man who presented to the ED for evaluation of hypotension. An electrocardiogram suggested acute anterior wall myocardial infarction. He underwent emergent coronary angiography and was found to have mid to apical akinesis and basal hyperkinesis with normal left coronaries and chronic total right coronary artery occlusion with excellent collaterals from left. A transthoracic echocardiography (TTE) revealed left ventricular ejection fraction 25-30% and akinesis of left and right ventricle except in the basal region. TTE with definity showed sessile thrombus. In our patient, sepsis was the most important triggering factor given initial presentation of hypotension with leukocytosis. Broad spectrum antibiotics including vancomycin and Zosyn were started considering a combination of septic and cardiogenic shock. Repeat EKG showed resolution of ST-T segment elevation but our patient remained hemodynamically unstable even with two pressure support and, ultimately, died 72 hours after admission. Herein, we emphasize on the importance right ventricular involvement and its relation to hemodynamic instability. This case highlights the importance of anticipating hemodynamic instability and clot formation in patients with biventricular Takotsubo cardiomyopathy.

UNASSIGNED: Multisystem inflammatory syndrome associated with SARS-CoV-2 infection can lead to myocardial injury and shock in children, likely the result of a severe inflammatory state, and can mimic Kawasaki disease.
UNASSIGNED: To describe the characteristics of shock and myocardial injury in children with confirmed or suspeted COVID-19 during the SARS-CoV-2 pandemic in Spain, including clinical presentation, laboratory and imaging findings, treatment, disease course, and outcome. An extensive literature review is provided.
UNASSIGNED: Retrospective case series including all children (age 1 month-18 years) admitted to a pediatric intensive care unit in Madrid, Spain, between March 15 and April 30, 2020 with suspected or confirmed SARS-CoV-2 infection and shock.
UNASSIGNED: Twelve previously healthy patients with shock, age 5 to 14 years, were included. All required volume resuscitation and 75% required vasoactive/inotropic support. Distributive shock was present on admission in 67% (n = 8), and 4 patients (33%) showed features of cardiogenic shock. Myocardial injury was diagnosed in 67% (n = 8) and ventricular dysfunction in 33% (n = 4). The most common symptoms on presentation were fever (100%), anorexia (100%), diarrhea (75%), and vomiting (75%). Five patients showed signs of Kawasaki disease but none met the criteria for the classic form. Laboratory findings revealed lymphopenia (83%), thrombocytopenia (83%), and increased inflammatory markers (100%). Respiratory status was not significantly impacted. Chest X-ray showed bilateral alveolar infiltrates in 7 (58%) and bilateral pneumonia in 3 (25%). COVID-19 was confirmed in 11 cases (92%). All received empirical therapy against COVID-19, thromboprophylaxis and immunomodulation. Median stay in the PICU and inpatient ward was 4.5 and 10 days, respectively. No patients died.
UNASSIGNED: Multisystem inflammatory syndrome in children with COVID-19 can mimic Kawasaki disease and lead to a combination of distributive and cardiogenic shock, probably secondary to a hyperinflammatory state that remains to be precisely defined. Treatment strategies include hemodynamic support, empirical therapies against COVID-19, thromboprophylaxis, and immunomodulation.

Severe acute respiratory syndrome coronavirus 2 is the new coronavirus responsible for the coronavirus disease 2019 pandemic, characterized by acute respiratory distress syndrome and atypical pneumonia. In nonpregnant women, studies have shown that severe acute respiratory syndrome coronavirus 2 causes cardiac injury, which can result in myocardial inflammation and damage. Despite many studies investigating the extent of cardiac compromise in patients with severe coronavirus disease 2019, little is known regarding its impact on pregnant women.
This study aimed to illustrate the clinical, laboratory, radiologic findings and outcomes of pregnant patients with coronavirus disease 2019 who developed myocardial injury with ventricular dysfunction.
We retrospectively reviewed the paper records of 15 pregnant women with coronavirus disease 2019, who developed myocardial injury on a single tertiary care hospital in the Dominican Republic. Patients\' baseline characteristics, clinical picture, and laboratory and radiologic findings were presented, and maternal and fetal outcomes were analyzed.
Of 154 pregnant patients diagnosed as having coronavirus disease 2019 at our hospital during the study period, 15 (9.7%), developed myocardial injury. These patients\' mean age and gestational age were 29.87±5.83 and 32.31±3.68, respectively. Furthermore, 66.7% of patients presented with shortness of breath and 16.3% with palpitations. All patients were admitted to the intensive care unit, and 86.6% needed intubation. Patients developed myocardial injury, confirmed with highly elevated troponin (34.6 [14.4-55.5 ng/mL]), and pro-B-type natriuretic peptide concentrations (209 [184-246 pg/mL]). In addition, all patients developed left ventricular dysfunction demonstrated by an echocardiogram with a mean left ventricular ejection fraction of 37.67±6.4. Unfortunately, 2 patients who presented with palpitations died a few days after admission.
Our study showed coronavirus disease 2019 induced myocardial injury and left ventricular dysfunction in pregnant women with a 13.3% mortality rate, which was attributed to malignant arrhythmias.

Over the years, Takotsubo Cardiomyopathy (TCMP) has become increasingly apparent, now comprising a significant portion of patients presenting with suspected acute coronary syndrome. The most common presentation of TCMP is ST segment elevation on EKG, troponin elevation, and apical ballooning in the absence of significant coronary artery disease as seen via cardiac catheterization. Although this is the most common presentation, it is important to highlight the less common variants of TCMP. In this article, we present a case series of patients presenting with the different variants of TCMP, followed by a literature review.

Background: Pheochromocytoma is a catecholamine secreting tumor that, in extremely rare cases, may develop over time from a non-functional adrenal adenoma. Catecholamine excess can lead to a kind of cardiomyopathy similar to that seen in tako-tsubo syndrome (TTS). Case report: A 69 years old female with a history of type 2 diabetes, hypertension, and a non-functional right adrenal adenoma diagnosed 3 years earlier was referred to our center for further investigations. During the evaluation, she had a hypertensive crisis with chest pain, tachycardia, and diaphoresis. Suspecting an acute coronary syndrome, she underwent coronary angiography, which showed the typical features of TTS. The high 24 h-urinary metanephrines excretion and abdominal MRI findings were suggestive of pheochromocytoma. Right laparoscopic adrenalectomy was performed, with the resolution of all symptoms. Pathology findings confirmed the diagnosis of pheochromocytoma. After 12 months, the patient was still asymptomatic, with the echocardiography displaying a complete recovery of the left-ventricular function. Conclusions: The development of a pheochromocytoma from an adrenal non functional adenoma is an extremely rare event, but potentially life-threating because of the catecholamine-associated cardiovascular toxicity. In particular, TTS is a form of cardiomyopathy that has been increasingly described as associated with catecholamine-secreting tumors. The exclusion of pheochromocytoma in a patient with TTS has important therapeutic implications, since the administration of β-blockers may be extremely harmful in patients with catecholamine surge in the absence of adequate α-blockage.

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