Endomyocardiofibrosis in the Americas Collaborative Study: The EMF-SIAC Registry.

Endomyocardiofibrosis (EMF) is a restrictive cardiomyopathy characterized by subendocardial fibrosis due to eosinophilic myocardial infiltration. EMF may commonly present with heart failure (HF) or atrial fibrillation (AF). Immunosuppression can be effective in early stages, but not in the chronic phase. Our objective was to describe the characteristics of EMF patients in the Americas. This registry is a retrospective multicenter cross-sectional study including patients ≥18 years-old with EMF diagnosed by imaging methods, according to the Mocumbi criteria. Clinical, biochemical, and imaging variables were analyzed. On the 54 patients included, 28 (52%) were male with an age of 47 years. The etiology was idiopathic in 47 (87%) patients, familial in 4 (7%), and secondary to chemotherapy in 2 (3.5%). We detected a history of HF in 41 patients (76%), AF in 19 (35%), and ischemic stroke in 8 (15%). The diagnosis was made by echocardiography in all patients, and 38% had Cardiac Resonance or Computed Tomography. Thirty-five patients (65%) presented a left ventricular ejection fraction ≥50%, 11 (21%) severe mitral regurgitation, and 18 (33%) severe tricuspid regurgitation. In 17 patients (32%) the diagnosis was confirmed by endomyocardial biopsy. Among medical therapy, 72% received beta-blockers, 63% vasodilators, 65% mineralocorticoid antagonists, 7.4% SGLT2 inhibitors, and 11% corticosteroids. Subendocardial resection was performed in 9 (16%) patients and mitral valve replacement in 11 (20%) patients. In conclusion, EMF patients had a high prevalence of HF, AF, and embolic events. The diagnosis was frequently made in an advanced stage when HF management and surgery were the only effective treatments.

Esquistossomose e o Coração - Em Nome das Doenças Tropicais Negligenciadas e Outras Doenças Infecciosas que Afetam o Coração (Projeto NET-Heart) / Schistosomiasis & Heart - On Behalf of the Neglected Tropical Diseases and other Infectious Diseases affecting the Heart (the NET-Heart Project)

Resumo Fundamento A esquistossomose é uma doença tropical negligenciada que pode levar a complicações cardiovasculares. No entanto, o envolvimento cardiovascular na esquistossomose ainda precisa ser totalmente elucidado, devido ao número limitado de casos e ausência de evidência confiável, uma vez que a doença ocorre tipicamente em locais sem infraestrutura adequada para uma coleta de dados robusta. Objetivo Esta revisão sistemática teve como objetivo avaliar as implicações cardiovasculares da esquistossomose, incluindo no diagnóstico e tratamento, e propor um algoritmo para rastrear as manifestações cardiovasculares. Métodos Foi realizada uma revisão sistemática nos bancos de dados MEDLINE/PubMed e LILACS, com busca por artigos sobre o comprometimento cardiovascular na esquistossomose. Resultados Trinta e três artigos foram considerados para esta revisão: seis artigos de revisão, uma revisão sistemática, um ensaio clínico, 14 estudos observacionais, sete relatos de casos, e quatro séries de casos. O comprometimento cardiovascular inclui um amplo espectro de condições clínicas, tais como isquemia do miocárdio, disfunção ventricular, miocardite, hipertensão arterial pulmonar, e pericardite. Conclusões As complicações cardíacas da esquistossomose podem causar incapacidade em longo prazo e morte. O monitoramento clínico, exame físico, eletrocardiograma precoce, e ecocardiograma devem ser considerados como medidas chave para detectar o envolvimento cardiovascular. Dada a ausência de um tratamento eficaz das complicações, são necessários saneamento e educação nas áreas endêmicas para a eliminação desse problema de saúde mundial.

Abstract Background Schistosomiasis is a Neglected Tropical Disease which may lead to cardiovascular (CV) complications. However, the CV involvement in schistosomiasis has yet to be fully elucidated due to the limited number of cases and lack of reliable evidence, as schistosomiasis typically occurs in locations without adequate infrastructure for robust data collection. Objective This systematic review aims to assess cardiovascular implications of schistosomiasis, including in the diagnosis and treatment, and propose an algorithm for screening of CV manifestations. Methods A systematic review was performed in the MEDLINE/PubMed and LILACS databases of articles on the CV involvement in schistosomiasis. Results Thirty-three records were considered for this review: six review articles, one systematic review, one clinical trial, 14 observational studies, seven case reports, and four cases series. CV involvement includes a wide spectrum of clinical conditions, such as myocardial ischemia, ventricular dysfunction, myocarditis, pulmonary arterial hypertension, and pericarditis. Conclusions Cardiac complications of schistosomiasis may cause long-term disability and death. Clinical monitoring, physical examination, early electrocardiogram, and echocardiogram should be considered as key measures to detect CV involvement. Due to the lack of effective treatment of complications, sanitation and education in endemic areas are necessary for the elimination of this global health problem.

Schistosomiasis & Heart - On Behalf of the Neglected Tropical Diseases and other Infectious Diseases affecting the Heart (the NET-Heart Project). / Esquistossomose e o Coração - Em Nome das Doenças Tropicais Negligenciadas e Outras Doenças Infecciosas que Afetam o Coração (Projeto NET-Heart).

BACKGROUND: Schistosomiasis is a Neglected Tropical Disease which may lead to cardiovascular (CV) complications. However, the CV involvement in schistosomiasis has yet to be fully elucidated due to the limited number of cases and lack of reliable evidence, as schistosomiasis typically occurs in locations without adequate infrastructure for robust data collection. OBJECTIVE: This systematic review aims to assess cardiovascular implications of schistosomiasis, including in the diagnosis and treatment, and propose an algorithm for screening of CV manifestations. METHODS: A systematic review was performed in the MEDLINE/PubMed and LILACS databases of articles on the CV involvement in schistosomiasis. RESULTS: Thirty-three records were considered for this review: six review articles, one systematic review, one clinical trial, 14 observational studies, seven case reports, and four cases series. CV involvement includes a wide spectrum of clinical conditions, such as myocardial ischemia, ventricular dysfunction, myocarditis, pulmonary arterial hypertension, and pericarditis. CONCLUSIONS: Cardiac complications of schistosomiasis may cause long-term disability and death. Clinical monitoring, physical examination, early electrocardiogram, and echocardiogram should be considered as key measures to detect CV involvement. Due to the lack of effective treatment of complications, sanitation and education in endemic areas are necessary for the elimination of this global health problem.

FUNDAMENTO: A esquistossomose é uma doença tropical negligenciada que pode levar a complicações cardiovasculares. No entanto, o envolvimento cardiovascular na esquistossomose ainda precisa ser totalmente elucidado, devido ao número limitado de casos e ausência de evidência confiável, uma vez que a doença ocorre tipicamente em locais sem infraestrutura adequada para uma coleta de dados robusta. OBJETIVO: Esta revisão sistemática teve como objetivo avaliar as implicações cardiovasculares da esquistossomose, incluindo no diagnóstico e tratamento, e propor um algoritmo para rastrear as manifestações cardiovasculares. MÉTODOS: Foi realizada uma revisão sistemática nos bancos de dados MEDLINE/PubMed e LILACS, com busca por artigos sobre o comprometimento cardiovascular na esquistossomose. RESULTADOS: Trinta e três artigos foram considerados para esta revisão: seis artigos de revisão, uma revisão sistemática, um ensaio clínico, 14 estudos observacionais, sete relatos de casos, e quatro séries de casos. O comprometimento cardiovascular inclui um amplo espectro de condições clínicas, tais como isquemia do miocárdio, disfunção ventricular, miocardite, hipertensão arterial pulmonar, e pericardite. CONCLUSÕES: As complicações cardíacas da esquistossomose podem causar incapacidade em longo prazo e morte. O monitoramento clínico, exame físico, eletrocardiograma precoce, e ecocardiograma devem ser considerados como medidas chave para detectar o envolvimento cardiovascular. Dada a ausência de um tratamento eficaz das complicações, são necessários saneamento e educação nas áreas endêmicas para a eliminação desse problema de saúde mundial.

Vasodilator Strain Stress Echocardiography in Suspected Coronary Microvascular Angina.

Background: In patients with Ischemia and non-obstructive coronary artery stenosis (INOCA) wall motion is rarely abnormal during stress echocardiography (SE). Our aim was to determine if patients with INOCA and reduced coronary flow velocity reserve (CVFR) have altered cardiac mechanics using two-dimensional speckle-tracking echocardiography (2DSTE) during SE. Methods: In a prospective, multicenter, international study, we recruited 135 patients with INOCA. Overall, we performed high dose (0.84 mg/kg) dipyridamole SE with combined assessment of CVFR and 2DSTE. The population was divided in patients with normal CVFR (>2, group 1, n = 95) and abnormal CVFR (≤2, group 2, n = 35). Clinical and 2DSTE parameters were compared between groups. Results: Feasibility was high for CFVR (98%) and 2DSTE (97%). A total of 130 patients (mean age 63 ± 12 years, 67 women) had complete flow and strain data. The two groups showed similar 2DSTE values at rest. At peak SE, Group 1 patients showed lower global longitudinal strain (p < 0.007), higher mechanical dispersion (p < 0.0005), lower endocardial (p < 0.001), and epicardial (p < 0.0002) layer specific strain. Conclusions: In patients with INOCA, vasodilator SE with simultaneous assessment of CFVR and strain is highly feasible. Coronary microvascular dysfunction is accompanied by an impairment of global and layer-specific deformation indices during stress.

Acute changes of left ventricular function during surgical revascularization by 3D speckle tracking.

BACKGROUND: Detecting early impact of coronary artery bypass grafting (CABG) on left ventricular (LV) function is important because such measures may contribute to meaningful improvement in clinical outcomes. We aimed to gain knowledge about acute changes of LV performance during surgical revascularization using three-dimensional speckle tracking echocardiography (3D STE). METHODS: Thirty-five patients scheduled for CABG surgery who underwent intraoperative transesophageal echocardiography (TEE) were enrolled (mean age 68.9 ± 7.3 years). TEE was performed before and after surgery, as well as before and after grafting. 3D LV ejection fraction (LVEF), tissue motion annular displacement (TMAD) of the mitral valves, 3D global longitudinal strain (GLS), global circumferential strain (GCS), twist, and torsion were quantified. Regional longitudinal strain (LS) was calculated based on coronary perfusion territories in a 16-segment LV model. RESULTS: Despite the absence of change in TMAD and 3D LVEF, 3D GLS (-18.6 ± 4.3% at baseline vs -16.0 ± 4.0% after surgery, P = .01) was significantly decreased, followed with no significant effect on GCS, twist, and torsion during surgery. 3D GLS correlated significantly with 3D LVEF (r between -0.34 and -0.51, P < .05 for all) under the whole operation. Territorial LS did not increase immediately after surgery. CONCLUSION: 3D speckle tracking imaging allows for detailed and direct evaluation of myocardial deformation, though impaired LV longitudinal function is still apparent immediately after surgery. GLS is more sensitive to an acute reduction in LV function than conventional parameters, which can be potentially useful for serial monitoring of functional recovery.

Endomyocardiofibrosis: A Systematic Review.

Endomyocardiofibrosis was described first time in Uganda as an infrequent restrictive cardiomyopathy with a poor prognosis, characterized by fibrosis of the ventricular subendocardium and severe restrictive physiology leading to difficult therapeutic management and frequently associated with hypereosinophilic syndrome. Its higher prevalence in the tropics and its relationship in some cases with hypereosinophilic endocarditis has led to the search for genetic, infectious, autoimmune and nutritional causes, but its etiology remains unclear. It is a rare cardiomyopathy, difficult to diagnose and with a nonexistent effective treatment. Imaging methods such as echocardiography and cardiac magnetic resonance are essential for the initial diagnosis, although endomyocardial biopsy establishes the definitive diagnosis. Immunosuppressive treatment is only useful in the early stages of the disease and usually ineffective if installed late when signs of heart failure are present. Surgical treatment is generally palliative.

Right ventricular free wall strain predicts functional capacity in patients with repaired Tetralogy of Fallot.

To investigate the role of right ventricular free wall strain (RVFWSL) to predict low functional capacity in repaired tetralogy of Fallot (rTOF). We prospectively enrolled 33 patients with rTOF with moderate to severe PR who underwent rest and peak exercise echocardiography on a semisupine cycloergometer. Conventional function and strain imaging parameters of both ventricles were measured. Patients performing < 7 METS were defined to have low functional capacity. Logistic regression was used to identify parameters associated with low functional capacity. Eleven patients (33.3%) had low functional capacity. These patients were shorter (height 155 ± 7 vs 163 ± 9 cm, p = 0.023), more frequently female (27.3 vs 72.7%, p = 0.024) and had history of Blalock-Taussig shunt (45.5 vs 9.1%, p = 0.027). On multivariate analysis RVFWSL was the only predictor of low functional capacity OR 1.39 (CI 95%, 1.06-1.83., p = 0.018) per % change. A RVFWSL < 17% (absolute value) had an AUC of 0.785, sensitivity of 81.8% and specificity of 77.3% to predict low functional capacity. Right ventricular free wall strain is an independent predictor of low functional capacity in repaired tetralogy of Fallot with moderate to severe PR. A value < 17% might be useful in deciding when to perform pulmonary valve replacement, when functional capacity cannot be objectively measured.

Differentiating spontaneous echo contrast, sludge, and thrombus in the left atrial appendage: Can ultrasound enhancing agents help?

The accurate identification of thrombus in the left atrial appendage with transesophageal echocardiogram (TEE) in patients with atrial fibrillation (AF) before cardioversion is essential. Most of these patients have some grade of spontaneous echo contrast (SEC). Severe SEC is often called "sludge," and its prognosis and treatment are still controversial. Current guidelines suggest the use of ultrasound enhancing agents (UEAs) when significant SEC is present. However, little is known about the utility of the UEAs in the differentiation between sludge and less severe SEC.

Right ventricular longitudinal strain predicts acute kidney injury and short-term prognosis in patients with right ventricular myocardial infarction.

Right ventricular (RV) systolic dysfunction due to acute myocardial infarction is associated with serious complications in the short-term. Acute kidney injury (AKI) is a frequent and unrecognized complication. This study aimed to assess whether RV longitudinal strain predicts AKI and short-term prognosis in patients with RV infarction. Prospective cohort of patients with RV infarction. RV function was evaluated with global and free wall right ventricular longitudinal strain (GRVLS and FWRVLS), tricuspid annular plane systolic excursion, and tricuspid S' wave. The primary endpoint was AKI defined as an increase ≥ 50% in serum creatinine and/or a decrease ≥ 25% in glomerular filtration rate during follow-up at 7 days. The secondary endpoint was death from any cause at 30 days. We included 101 patients with RV infarction (male 67%, age 66 ± 11 years). During follow-up at 7 days, 40% of patients developed AKI. At 30 days, 8% of patients died. At univariate analysis, FWRVLS was significantly associated with AKI (Hazard ratio [HR] 1.11, 95% confidence interval [CI] 1.03-1.20, p = 0.006). At multivariate analysis, only age, temporary pacemaker implant, and FWRVLS remained as independent predictors of AKI (HR 1.05, 95% CI 1.02-1.08, p = 0.002; HR 2.12, 95% CI 1.11-4.07, p = 0.023; HR 1.10, 95% CI 1.02-1.19, p = 0.018, respectively). At 30 days, patients with FWRVLS ≥ - 15.5% showed a lower survival rate than those with lower strain (84 ± 6 vs. 97 ± 2%, p = 0.021). In patients with RV infarction, FWRVLS was an independent predictor of AKI and was associated with higher mortality in the short-term.

Disminución de la fracción de expulsión del ventrículo izquierdo por estrés con adenosina como predictor de isquemia.

INTRODUCCIÓN: En estudios de medicina nuclear se ha observado que la disminución de la fracción de expulsión del ventrículo izquierdo (FEVI) se asocia con enfermedad coronaria trivascular; en resonancia magnética cardiaca (RMC) no se ha estudiado el papel que desempeña la disminución de la FEVI. OBJETIVO: Evaluar la asociación entre la disminución de la FEVI y la isquemia cardiaca en pacientes con estudios de RMC con adenosina. MÉTODO: Estudio transversal comparativo. Los criterios de inclusión fueron pacientes evaluados con RMC con adenosina entre enero de 2009 y junio de 2015. Se comparó el cambio en la FEVI en pacientes con estudio positivo versus pacientes con estudio negativo para isquemia por este método. RESULTADOS: Se incluyeron 59 pacientes: 41 del sexo masculino (70 %), edad de 59.7 ± 10.9 años; 38 % de los estudios fueron positivos para isquemia. La delta de la FEVI (FEVI postestrés - FEVI reposo) fue de -0.16 ± 5.9 versus 5.3 ± 4.7 (p<0.001) en pacientes con y sin isquemia, respectivamente. CONCLUSIÓN: Los pacientes con estudios positivos para isquemia tuvieron menor delta FEVI que los que tuvieron estudios negativos para isquemia. BACKGROUND: The decrease in left ventricular ejection fraction (LVEF) has been observed to be associated with three-vessel coronary disease in nuclear medicine studies; however, the role played by LVEF decrease has not been studied with cardiovascular magnetic resonance (CMR). OBJECTIVE: To assess the association between LVEF decrease and cardiac ischemia in patients with CMR studies with adenosine. METHOD: Cross-sectional, comparative study. Inclusion criteria were: patients assessed with CMR with adenosine between January 2009 and June 2015. LVEF change was compared between patients testing positive for ischemia versus those who tested negative. RESULTS: Fifty nine patients were included: 41 were males (70%), mean age was 59.7 ± 10.9 years; 38% of the studies tested positive for ischemia. Delta LVEF (post-stress LVEF ­ resting LVEF) was ­0.16 ± 5.9 versus 5.3 ± 4.7 (p < 0.001) in patients with and without ischemia, respectively. CONCLUSION: Patients who tested positive for ischemia had lower delta LVEF than those with negative studies for ischemia.

Right atrial pressure predicts worsening renal function in patients with acute right ventricular myocardial infarction.

BACKGROUND: Right ventricular myocardial infarction (RVMI) is associated with serious complications in the short-term. Worsening renal function (WRF) is a frequent and dangerous complication. We investigated if right atrial pressure (RAP) predicts WRF in these patients. METHODS: We prospectively studied patients with RVMI. RAP was obtained invasively at admission to coronary care unit. Blood samples were extracted from patients at baseline and every 24h for creatinine measurements for seven days. We defined WRF as an increase of 25% or 0.5mg/dl in serum creatinine during the first seven days compared to baseline creatinine. RESULTS: We included forty-five patients (age 68±10years, male 71%). WRF occurred in 51%. The best cut-off value of RAP for WRF prediction was 11mmHg. RAP ≥11mmHg was associated with WRF at univariate analysis (OR 5.5, 95% CI 1.27-24.3, p=0.023) and multivariate analysis (OR 6.1, 95% CI 1.07-35.4, p=0.042). RAP ≥11mmHg improved reclassification and discrimination after usual prediction with the Mehran score (net reclassification improvement 64.8%, p=0.030; integrated discrimination improvement 7.5%, p=0.037). CONCLUSION: In patients with RVMI, RAP ≥11mmHg predicted WRF and improved discrimination.

Coexisting bicuspid aortic and pulmonary valves diagnosed by 3D transthoracic echocardiography.

Coexisting bicuspid aortic and pulmonary valves is an extremely rare condition, and there have been few published cases. Diagnosis of bicuspid aortic valve is straightforward with 2D echocardiography; however, analysis of the morphology of the pulmonary valve is challenging. In this study, we report on a case of a 32-year-old man with bicuspid aortic and pulmonary valves diagnosed by 2D and 3D transthoracic echocardiography. The enlarged pulmonary artery without any obvious etiology led us to suspect a pulmonary valve anomaly; thus, we comprehensively evaluated it with 2D and 3D echocardiography, which confirmed the diagnosis of bicuspid pulmonary valve.