2021;34(4) – ABC CARDIOL

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Unusual Presentation of Takayasu’s Disease

Marta Fontes Oliveira et al. Inês Antunes Fátima Neves Elisabete Pinelo Raquel Faria

Unusual presentation of Takayasu’s disease

A 21-year-old woman was admitted with heart failure and chest pain. Echocardiography revealed a tricuspid aortic valve with severe regurgitation (A), a thickened and deformed right coronary cusp with a linear image (B), and severe left ventricular dilation. Angio-computed tomography showed continuous and concentric thickening of the aortic wall (C) with a penetrating ulcer and dissection of the descending aorta (D) and superior mesenteric and left renal arterial occlusion (E). Takayasu aortitis type V was assumed for which immunosuppression was commenced. Significant improvement was noted (F). After achieving remission, the patient underwent endovascular surgery of the aorta and aortic valve replacement. No episodes of relapses were seen during a 3-year follow-up period.

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Original Article

Transthoracic Echocardiography Findings of Hospitalized Patients with COVID-19: Results from the Brazilian Echocardiography Registry During the COVID-19 Pandemic (ECOVID)

Silvio Henrique Barberato et al. Rafael Borsoi Fabio Roston Hudson Laerte Machado Miranda … Pedro Patriota Maria Estefânia Bosco Otto Adenalva Lima de Souza Beck Anderson da Costa Armstrong João Marcos Bemfica Barbosa Ana Cristina Camarozano Wermellinger Letícia Braga Paciello da Silva Marcos Valério Coimbra Resende Marcelo Luiz Campos Vieira Marcelo Miglioranza Miguel Morita Fernandes da Silva

Abstract

Background

Transthoracic echocardiography (TTE) may play a crucial role in the evaluation of cardiac manifestations of coronavirus disease 2019 (COVID-19).

Objective

We aimed to report the prevalence of the main echocardiographic abnormalities of hospitalized COVID-19 patients.

Methods

We performed a prospective multicenter observational study in patients with COVID-19 who underwent TTE during hospitalization. Patients with pre-existing heart failure, coronary artery disease, or atrial fibrillation were categorized as having previous cardiovascular disease (CVD). Clinical and echocardiographic data about cardiac structure and function were collected.

Results

We evaluated 310 patients with COVID-19 (mean age, 62±16 years; 61% men; 53% with arterial hypertension; 33% with diabetes; and 23% with previous CVD). Overall, 65% of the patients required intensive care unit support. The most prevalent echocardiographic abnormalities were LV hypertrophy (29%), pulmonary hypertension (25%), left ventricular (LV) systolic dysfunction (16.5%), right ventricular (RV) systolic dysfunction (15.9%), grade II/III LV diastolic dysfunction (11%), and LV regional wall motion abnormality (11%). Pericardial effusion was uncommon (7% of cases). LV hypertrophy (25% vs. 45%, p=0.001), LV systolic dysfunction (11% vs. 36%, p<0.001), regional wall motion abnormalities (6% vs. 29%, p<0.001), grade II/III LV diastolic dysfunction (9% vs. 19%, p=0.03), and pulmonary hypertension (22% vs. 36%, p=0.019) were less common in patients without previous CVD. RV systolic dysfunction occurred at similar frequencies in patients with versus without previous CVD (13% vs. 25%, p=0.07).

Conclusions

Among patients hospitalized with COVID-19, abnormal echocardiographic findings were common, but less so among those without previous CVD. RV systolic dysfunction appeared to affect similar proportions of patients with versus without previous CVD.

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Case Report

Young Patient with Chagas Disease Presenting Initially as Acute Chest Pain and Treated for Acute Coronary Syndrome

Jakson Ferreira Neto et al. Dannyl Roosevelt de Vasconcelos Lima Pedro Vinicius Amorim de Medeiros Patriota João Helbert Costa e Silva … Antônio Marconi Leandro da Silva Anderson da Costa Armstrong

Introduction

Chagas disease, a chronic and endemic condition in 21 countries within the Americas, affects approximately 6 million people. The northeast region has always been particularly important for the disease. According to data from the Mortality Information System (Sistema de Informação sobre Mortalidade), between 1980 and 2007, Brazil registered 156,224 deaths from Chagas disease; of them, 20,472 were in the northeast region and 3,144 occurred in Pernambuco.

Classic symptoms of Chagas disease can include chest pain, commonly typical for angina. Records of Chagas patients usually relate typical acute anginal pain to superimposed coronary artery disease. However, microcirculation disease has also been suggested as a chest pain trigger. Despite the high prevalence of Chagas heart disease in inland northeastern Brazil, coronary artery disease remains the main cause of heart failure and acute coronary syndromes.

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Case Report

Left Ventricular Longitudinal Strain Echocardiogram and Handgrip: A Useful Tool for Detecting Ischemia in the Emergency Room

Ana Paula Torres Guimarães de Freitas et al. Natalia Marcusso Massoni Rodrigo Bellio de Mattos Barretto

Introduction

Acute coronary syndrome is among the greatest diagnostic challenges for emergency room physicians. Non-invasive imaging tests such as echocardiography (ECHO) are useful in the etiological diagnosis of chest pain. In cases of suspected ischemia, when electrocardiography (ECG) and myocardial necrosis markers (MNM) are inconclusive, ECHO can be performed at the time of pain or immediately after. The use of advanced techniques such as myocardial deformation analysis (strain) provides greater accuracy in identifying the coronary artery disease territory.

This report describes a case of a patient with chest pain without segmental changes on initial ECHO in the emergency room. Significant coronary artery disease (CAD) changes were evident on additional tools (handgrip, strain, and postsystolic peak) as showed on the subsequent coronary angiography.

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Original Article

Frequency and Characteristics of Responders to an Educational Intervention to Reduce Inappropriate Heart Testing: A Sub-study of the Echo WISELY Trial

João Ricardo Pinto Lopes et al. Cherry Chu Sacha Bhatia Luis Claudio Correia

Abstract

Introduction

The Echo WISELY Trial is a controlled randomized multicenter investigator-blinded study that evaluated an educational intervention based on the criteria for appropriate use of echocardiography to reduce the proportion of rarely appropriate outpatient echocardiograms performed.

Objective

To describe the prevalence and identify predictors of the responsiveness of responding physicians subjected to an educational intervention in the Echo WISELY Trial.

Methods

The intervention group physicians received a multifaceted educational program. A responding physician was defined as one who had a >2.5% reduction in the proportional mean of rarely appropriate tests requested between the first trimester (baseline) and any of the following trimesters (second to sixth). Physician characteristics (sex, time since graduation, medical specialty, and workplace) were compared to the echocardiogram ratings (appropriate, maybe appropriate, and rarely appropriate) and clinical reasons for the requested echocardiograms using the chi-square test. Statistical significance was indicated by a two-tailed p < 0.05.

Results

A total of 4,605 tests requested at the six participating hospitals in Ontario were analyzed and randomized for the intervention arm. Of the 36 included physicians, 26 (72%) were classified as responders. Of the variables analyzed, there was no significant difference in the outcomes of the responders versus non-responders to the educational intervention. The number of rarely appropriate tests requested by the responders was significantly lower than that of the non-responders (234 [8.67%] versus 261 [13.8%]; p < 0.0001).

Conclusion

The prevalence of responder physicians was high, but predictors of responsiveness to educational intervention were not identified among the analyzed variables. This may be a result of the psychological aspects and personal characteristics of the physicians, which were not included in this research.

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Original Article

Early Diastolic Dysfunction in Breast Cancer Patients Undergoing Chemotherapy

Geanne Maria Holanda de Menezes Barroso et al. Paulo Victor de Jesus Silva Júlio César Oliveira Costa Teles Vinícius Antônio Santos Aragão … Karin Yasmin Santos Fonsêca Marília Marques Aquino Ullany Maria Lima Amorim Coelho de Albuquerque Enaldo Vieira de Melo Antônio Carlos Sobral Sousa Joselina Luzia Menezes Oliveira

Abstract

Background

Chemotherapy for breast cancer is associated with serious cardiovascular complications such as heart failure. The left ventricular ejection fraction is the main parameter used to assess systolic function in these patients. However, the occurrence of diastolic dysfunction may precede that of systolic dysfunction.

Objectives

To evaluate left ventricle diastolic and systolic functions in women with breast cancer undergoing chemotherapy using anthracyclines.

Methods

This observational, longitudinal, analytical, and prospective study included 62 women with breast cancer aged 21–75 years old who underwent echocardiography at baseline and after three months of treatment. Diastolic function parameters were evaluated, and the patients were classified as diastolic dysfunction type 1, 2, or 3. Systolic dysfunction was defined as a left ventricular ejection fraction ≤ 53%.

Results

After three months of treatment, 35 patients (56.4%) had type 1 diastolic dysfunction, while one (1.6%) had type 2. Diastolic dysfunction was identified in 26 patients at baseline and developed in 10 patients during treatment. Diastolic function parameters, E wave velocity, and E/A ratio decreased significantly (p < 0.05) with chemotherapy; however, the others showed no significant variations. Only three patients had systolic dysfunction, but there was a greater reduction in left ventricular ejection fraction in the group that developed diastolic dysfunction during treatment versus the group with diastolic dysfunction at baseline (p = 0.04).

Conclusion

Diastolic dysfunction occurs early in women with breast cancer undergoing chemotherapy. Its onset during the course of treatment is associated with a significantly reduced left ventricular ejection fraction.

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Incidental Diagnosis of Carotid Body Paraganglioma

Silvio Henrique Barberato

Paragangliomas are rare benign neuroendocrine tumors of extra-adrenal location, but a small proportion may have a clinical malignant behavior and metastasize. In the neck, they usually affect the carotid body and are inactive in the secretion of catecholamines. These tumors are often asymptomatic but may present as a pulsatile cervical mass or cause compression in adjacent structures. Surgical excision is the only potentially curative treatment since histological evaluations may be limited to provide a definitive diagnosis of malignancy. Late recurrence can occur. Here we present the case of a 56-year-old asymptomatic woman who underwent carotid ultrasound to screen for atherosclerotic disease in whom a vascularized well-defined hypoechoic mass was incidentally noted in the carotid bifurcation (). Contrast computed tomography confirmed findings compatible with paraganglioma (). The patient underwent surgery and the diagnosis was confirmed.

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Editorial

My approach to the diagnosis of congenital coronary anomalies by transthoracic echocardiography

Vitor Coimbra Guerra

Why do we need to assess the anatomy of the coronary arteries?

Until a few decades ago, coronary artery anatomy assessment by echocardiogram (ECHO) was considered a difficult secondary task even if analyzed by other cardiovascular imaging modalities, such as angiography by cardiac catheterization. Important changes in this approach in recent years can be explained by three important aspects:1) The origin and initial course of coronary artery anomalies can be a substrate for ischemia and even sudden death in young adults;^,2) Transthoracic echocardiography (TTE) is a non-invasive radiation-free low-cost imaging test in the frontline of cardiac assessments;3) The anatomical and functional assessment of coronary arteries is part of a transthoracic test used to diagnose congenital heart diseases.^,

Which congenital coronary artery anomalies are relevant and should be identified?

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Prenatal Diagnosis of Circular Shunt: A Rare Complication of Ebstein’s Anomaly

Marcia Ferreira Alves Barberato et al. Silvio Henrique Barberato

Circular shunt is a condition in which blood from a cardiac chamber is diverted through the heart to return to the original chamber without reaching the pulmonary capillary bed. A 27-year-old pregnant woman, G1, P0, A0, 25 gestational weeks, was referred for fetal echocardiography due to an increased cardiac area on morphological obstetric ultrasound in the second trimester. She had no comorbidities, and early pregnancy ultrasound findings were normal.

A 25-week fetal echocardiogram showed cardiomegaly, attachment of the tricuspid valve septal leaflet to the interventricular septum, and significant tricuspid insufficiency compatible with Ebstein’s anomaly (). A small pericardial effusion was seen and Huhta cardiovascular score was 6. An adequate pulmonary artery diameter (Z-score 1.9) was noted () with no anterograde flow, only retrograde flow coming from the ductus arteriosus characteristic of a circular shunt. At 29 gestational weeks, fetal hydrops was observed, with zero umbilical artery diastolic flow and a Huhta score of 4 (). Fetal death occurred at 30 gestational weeks.

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Case Report

Importance of Imaging Methods for Diagnosing Constrictive Pericarditis Versus Restrictive Cardiomyopathy

Débora Pereira Galvêas Negri et al. Patrick Ventorim Costa Mateus Oliveira Potratz Carina Massariol Belinassi Fernando Luiz Torres Gomes

Introduction

Constrictive pericarditis (CP) is a challenging differential diagnosis of chest pain in a cardiac emergency. This is the case report of a patient who presented with a seven-day history of chest pain for whom the possibility of calcified pericarditis was suggested due to changes on chest X-ray. After referral to a specialized cardiology service, transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR) showed that the case was compatible with CP, which required surgical management. From chest X-ray to CMR, all imaging tests proved crucial for confirming the suspected diagnosis and defining the treatment.

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Incidental Finding of Retroaortic Renal Vein ‘Posterior Nutcracker Phenomenon’ Associated with Thrombosis

Milton Sérgio Bohatch Júnior et al. Amanda Fernandes Vidal da Silva Carlos Diego Ribeiro Centellas Maurício Serra Ribeiro Edwaldo Edner Joviliano

A 56-year-old woman was admitted to the emergency room with acute obstructive abdomen. She denied comorbidities or previous surgeries. Abdominal computed tomography showed cecal volvulus and an incidental finding of chronic recanalized left renal vein thrombosis with a retroaortic trajectory (posterior “nutcracker” phenomenon). She underwent exploratory laparotomy with a right colectomy and transverse ileum anastomosis. After a gradually progressed diet, she was discharged from hospital ten days after surgery. In outpatient follow-up, she denied symptoms of abdominal or flank pain, and laboratory tests showed no signs of proteinuria or hematuria. Conservative treatment and periodic follow-up were chosen considering the incidental finding of nutcracker phenomenon.

A retroaortic left renal vein is a rare anatomical variation that is asymptomatic in most cases and called posterior nutcracker phenomenon. In symptomatic cases, the term posterior nutcracker syndrome is used.^, Its exact prevalence is unknown, but it is more commonly diagnosed in female patients between the third and fourth decades of life. This compressive disorder can result in venous hypertension with renal vein dilation and ureteral and renal pelvis varicose veins and present as macroscopic and microscopic hematuria, flank pain, and proteinuria.^, Conservative treatment is used for asymptomatic or oligosymptomatic patients as described in here.

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Case Report

Rare Incidental Finding on Coronary Artery CT Angiography: Left Main Coronary Artery Atresia

Maria Eduarda Menezes de Siqueira et al. Mayra Isabel Dias Ana Claudia Vincezi Raudan Uski Luciano Figueiredo de Aguiar Filho

Introduction

Congenital left main coronary artery atresia (CLMCAA) is a coronary anomaly in which the left coronary ostium or trunk is absent. The anterior descending (AD) and circumflex (CX) arteries are usually connected and proximally end blindly. Left chamber perfusion is provided by the right coronary (RC) collaterals. It is the rarest coronary anomaly, with fewer than 70 cases described in the literature. The clinical CLMCAA spectrum ranges from asymptomatic cases (due to an efficient collateral system) to symptoms such as angina, tachyarrhythmias, syncope, and sudden death.^, Surgical revascularization is recommended in symptomatic patients; however, preventive surgical treatment is controversial in asymptomatic patients without inducible myocardial ischemia.^–

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Case Report

Coronary–Cavitary Fistula of the Circumflex Coronary Artery to the Left Atrium as a Probable Etiology of Heart Failure: A Case Report

Roberto Carlos Alvarez Coello et al. Vitor Coutinho Andrade Santiago Andrés Castro Vintimilla Vagner Madrini Júnior José Felix Alvarez Ramirez

Abstract

Coronary artery fistulas have a low incidence and are often diagnosed by echocardiography or coronary computed tomography angiography, although coronary angiography is the gold standard. They commonly originate in the right coronary artery, with drainage to low-pressure chambers being the most frequent finding. Treatment can be expectant, surgical, or percutaneous. This report describes a case of a rare presentation of heart failure due to a fistula of the circumflex coronary artery with drainage into the left atrium.

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Case Report

Third Recurrence of Cardiac Myxoma in a Carney Complex Patient

David Alejandro Salazar Jaya et al. Kevin Rafael de Paula Morales Félix Ramires Paulo Sampaio Gutierrez … Ricardo Ribeiro Dias Vagner Madrini Junior

Introduction

Primary cardiac tumors are an uncommon entity with an incidence of 0.3–0.7%. Most primary cardiac neoplasms are benign, with cardiac myxoma (CM) being the most prevalent type.^– Patients with CM may present symptoms due to embolic phenomena or intracardiac blood flow obstruction for which the treatment is surgical resection.^– Postoperative tumor recurrence occurs in up to 3% of cases; however, when myxomas are part of an underlying genetic syndrome, the recurrence rate can reach 22%. The main syndrome associated with this recurrence is the Carney complex (CNC). First described in 1985 by J. Aidan Carney, CNC is defined as an extremely rare autosomal dominant genetic syndrome with main clinical characteristics including skin pigmentation, heart myxomas, and endocrine neoplasms.

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Original Article

Analysis of the maintenance process and useful life of echocardiography equipment: An experience in a teaching hospital

Gabriel Peres Mazzoni et al. André Timóteo Sapalo André Schmidt Benedito Carlos Maciel Minna Moreira Dias Romano

Abstract

Introduction

Echocardiography is a diagnostic tool that is increasingly used in clinical practice in different medical scenarios; however, the preventive (PM) or corrective maintenance (CM) care and processes for this equipment are still poorly standardized. To describe the maintenance process currently implemented for echocardiographic equipment (ECHO) in a medium-sized laboratory in a tertiary-level public teaching hospital. The description of the process includes damage characterization and MP and MC implementation.

Methods

This was a descriptive and exploratory single-center observational study. Data on the maintenance process of echocardiographic equipment were obtained from electronic files from the hospital’s equipment management system between 2003 and 2018.

Results

Together with the description of the equipment maintenance process, the ten most common types of malfunctions were identified, including software (23.8%), parts (23.1%), and power outage and accessory damage (13.8%). The implementation of the PM process significantly decreased the maintenance costs (USD 44,472.10 vs USD 25,807.59, p = 0.029). Even after the MP, the CM costs related to transesophageal echocardiography equipment (TEE) (USD 7,789.17) were higher than those with other equipment modalities (USD 3,184.37 for transthoracic echocardiography equipment (TTE) and USD 1,813.00 for stress testing).

Conclusion

The maintenance process for ECHO equipment was described. ECHO equipment damage has high costs, especially in special modalities such as TEE. PM significantly reduced maintenance costs.

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Editorial

My Approach to Assessing Right Ventricular Function After Tetralogy of Fallot Repair

Laura Mercer-Rosa

Qualitative assessment

On the apical four-chamber view, I examine the motion of the tricuspid valve toward the apex (measured as the tricuspid annular plane systolic excursion [TAPSE] as detailed below) and the motion of the RV wall toward the septum versus data of prior studies. Likewise, parasternal long- and short-axis views are important. In those with favorable acoustic windows, RV wall shortening can be assessed using M-mode ( ). It is worth noting that the qualitative assessment of RV function should not be the sole parameter.^,

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Original Article

Non-Compacted Cardiomyopathy in Children and Adolescents: From the Challenge of Echocardiographic Diagnosis to Clinical Follow-Up

Ana Flávia Malheiros Torbey et al. Ana Catarina Durán Bustamante Aurea Lucia Grippa de Souza Carmen Zampirole Brandão … Luan Rodrigues Abdallah Yves Pacheco Dias March e Souza Evandro Tinoco Mesquita

Abstract

Background

Non-compacted cardiomyopathy (NCCM) is characterized by hypertrabeculations and deep recesses in the left ventricle, with a heterogeneous clinical presentation, ranging from asymptomatic patients to those with heart failure (HF), thromboembolic events and arrhythmias with risk of sudden death. As it is rare and does not have well-defined diagnostic criteria, its natural history in pediatrics is poorly understood. This study describes the clinical presentation and clinical course of patients with NCCM.

Methodology

Observational, longitudinal, prospective study of pediatric patients seen at a pediatric cardiology referral center from metropolitan region II in the state of Rio de Janeiro, with NCCM phenotype on echocardiogram (ECHO) during a 2-year follow-up, from the ChARisMa registry.

Results

6 patients aged 4 to 14, with NCCM, were analyzed. Mean age 7.5 years (SD: 3.93), 3 males (50%). The patients presented HF (n=2), cardiac murmur (n=1), cardiac arrhythmia (n=1), were asymptomatic (n=1) or were under investigation for a genetic syndrome (n=1). Phenotypes on ECHO: NCCM/dilated cardiomyopathy (n=1) and NCCM/restrictive cardiomyopathy (n=1), isolated phenotype of NCCM (n=4). Cardiac magnetic resonance imaging was performed and confirmed the diagnosis (n=4). The outcomes observed were thromboembolism, indication for heart transplantation, and sustained ventricular tachycardia.

Conclusions

This case series provides relevant data for pediatric NCCM as it shows its heterogeneous clinical presentation and potentially fatal complications. More prospective studies are needed for an accurate diagnosis and to allow its clinical course, therapeutic response and prognosis to be better known.

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