Endomyocardial Biopsy in Pediatric Myocarditis and Dilated Cardiomyopathy: A Tool in Search for a Role

Endomyocardial biopsy (EMB) is a well-known diagnostic tool for the investigation and treatment of myocardial diseases and remains the gold standard for the diagnosis of myocarditis. Due to its invasiveness, with a complication rate ranging from 1 to 15%, its role in the diagnostic work-up of pediatric heart failure is not well established. The aim of this review is to define the role of EMB as diagnostic technique in the work up of children presenting with severe left ventricular dysfunction with the support of our center experience.

Biventricular non-compaction cardiomyopathy and tricuspid hypoplasia in a novel non-POU domain-containing octamer-binding gene variant

A maternally inherited novel pathogenic non-POU domain-containing octamer-binding gene variant c.767G>T, p.R256I [NM_001145408], manifested in a male infant as dilated cardiomyopathy with severe left ventricular dysfunction and dilation, biventricular non-compaction, tricuspid hypoplasia, and hydrocephaly. To the best of our knowledge, no previous non-POU domain-containing octamer-binding gene variants with biventricular non-compaction have been associated with tricuspid valve hypoplasia. Hence, this case introduces a new pathogenic variant observed in the non-POU domain-containing octamer-binding gene and adds to the range of cardiac phenotypes identified in non-POU domain-containing octamer-binding gene variants.

Percutaneous thrombectomy and right ventricular mechanical circulatory support for pulmonary embolism in a COVID-19 patient: Case report, one-year update, and echocardiographic findings

Background We previously described percutaneous thrombectomy and right ventricular mechanical support of a COVID-19 patient with a massive pulmonary embolism. Here we present a detailed echocardiographic and clinical timeline with 1 year follow-up. Case Summary A 57-year-old female with COVID-19 went into shock from a massive pulmonary embolism. After percutaneous removal of a large thrombus burden (AngioVac system; AngioDynamics Inc, Latham, NY, USA), she became severely hypotensive, requiring CPR, and was resuscitated with an Impella RP device (Abiomed, Danvers, MA, USA). A pediatric TEE probe monitored the procedure because an adult probe would not pass (S7-3t—Philips Medical Systems, Andover, MA, USA). Post thrombectomy, surface imaging documented gradual resolution of right ventricular dysfunction, tricuspid regurgitation, and elevated pulmonary artery pressure. Her course was complicated by renal failure requiring temporary dialysis. She was discharged home on apixaban. Hypercoagulability work-up was negative. Two months later, vocal cord surgery was performed for persistent stridor. Esophagoscopy at that time was prevented by osteophyte obstruction. At 10 months, she received the Pfizer-BioNTech vaccine. At one year, the patient remains healthy on apixaban, and her echocardiogram is normal. Discussion This case illustrates the pivotal role of echocardiography in the diagnosis, percutaneous treatment, and near- and long-term follow-up and management of a patient with massive pulmonary embolism due to COVID-19 with documentation of complete recovery from severe right ventricular dysfunction and hemodynamic collapse. A pediatric TEE probe was a crucial alternative to the adult probe because of possible osteophyte obstruction.

Takotsubo Syndrome: A Review of Presentation, Diagnosis and Management

Takotsubo Syndrome (TTS) is a condition of transient left ventricular dysfunction that is typically triggered by emotional or physical stress. Since first described in Japan in 1990, it has increasingly been recognised in clinical practice, accounting for up to 2% of Acute Coronary Syndrome (ACS) presentations. In fact, the clinical presentation can be indistinguishable from a myocardial infarction. Although current evidence suggests a catecholamine induced myocardial stunning, the pathophysiological mechanisms remain unknown. Interestingly, it is more common in woman, particularly those who are post-menopausal. This review aims to summarise the current research and provide an overview of the diagnostic strategies and treatment options.

Role of Echocardiography for Assessment of Cardiac Remodeling in Congenitally Corrected Transposition of Great Arteries

Background: The purpose of this study was to assess the role of echocardiography for a comprehensive assessment of cardiac remodeling, and the relationship between indices of cardiac remodeling and cardiovascular events (defined as the composite end point of heart failure hospitalization, heart transplant, or cardiovascular death) in adults with congenitally corrected transposition of great arteries (cc-TGA). Methods: This is a retrospective study of adults with cc-TGA who underwent echocardiogram (2003–2020). Offline image analysis was performed in all patients. Chamber (atrial and ventricular) function and size were assessed by strain imaging and 2-dimensional echocardiography. Results: Of 233 patients with cc-TGA (40±15 years), 123 (55%) had at least one cardiac procedure before baseline echocardiogram. Of 233 patients, 76% and 61% had left atrial dysfunction and systemic right ventricular dysfunction, respectively; while 43% and 11% had right atrial dysfunction and left ventricular dysfunction, respectively. During a median follow-up of 8.9 years, 114 (49%) underwent additional cardiac procedures, and 66 (28%) had cardiovascular events. Left atrial reservoir strain, right ventricular global longitudinal strain, right atrial reservoir strain, left ventricular systolic pressure, and left ventricular global longitudinal strain were independently associated with cardiovascular events. Conclusions: In addition to the clinical importance of right ventricular systolic dysfunction in cc-TGA that is already well described, the current study demonstrated, for the first time, that biatrial dysfunction was common and was associated with clinical outcomes. Since there are currently no effective therapies for atrial and ventricular dysfunction in patients with cc-TGA, there is a need for research to identify novel strategies to prevent atrial and ventricular dysfunction in this population.

Prediction of subclinical left ventricular dysfunction by speckle-tracking echocardiography in patients with anti-neutrophil cytoplasmic antibody--associated vasculitis

Objectives: This study aims to evaluate left ventricular functions using speckle-tracking echocardiography (STE) in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Patients and methods: Between June 2018 and July 2019, a total of 31 AAV patients (17 males, 14 females; median age: 53 years; range, 47 to 62 years) and 21 healthy controls (11 males, 10 females; median age: 56 years; range, 46 to 60 years) were included in the study. Clinical and biochemical characteristics of all participants were recorded. All participants underwent conventional and two-dimensional STE. The receiver operating characteristic (ROC) curve analysis was performed to determine the cut-off value of serum N-terminal prohormone of brain natriuretic peptide (NT-pro-BNP) that predicted subclinical left ventricular dysfunction. The Spearman correlation analysis was used to determine the correlation between left ventricular global longitudinal strain (LV-GLS) and NT-pro-BNP. Results: The LV-GLS was lower in AAV patients (19.3% vs. 21.7%, respectively; p=0.014). NT-pro-BNP was negatively correlated with LV-GLS (p=0.005, r=0.401). Conclusion: Subclinical left ventricular dysfunction can be detected by STE in patients with AAV who have free of clinically overt cardiovascular disease. The LV-GLS is negatively correlated with serum NT-pro-BNP levels.