Cardiac Sarcoidosis Presenting as Sustained Ventricular Tachycardia.

Introduction: Sarcoidosis has many possible clinical presentations since it can affect any organ, most commonly the lungs. The hallmark of the disease consists of the formation of non-necrotising granulomas. Pathogenesis is thought to rely on the interplay of genetic, environmental and epigenetic factors. This case highlights the importance of a thorough clinical history and physical examination, and the correlation with imaging findings in the diagnostic work-up of the non-ischaemic cardiomyopathy. Case description: A 57-year-old woman was admitted due to the sudden onset of malaise, dizziness, and chest discomfort. Sustained monomorphic ventricular tachycardia was evidenced and the patient rapidly evolved with haemodynamic instability; she underwent successful electrical cardioversion. The electrocardiogram afterwards showed a high-risk electrocardiographic pattern. Invasive coronary angiography excluded obstructive epicardial coronary lesions. Physical examination revealed skin lesions on the lower limbs which raised suspicion for erythema nodosum and therefore a biopsy was performed. Transthoracic echocardiography and cardiac magnetic resonance imaging revealed features consistent with an inflammatory cardiomyopathy, and an implantable cardioverter-defibrillator was placed. The histologic examination of the cutaneous lesions showed a non-necrotising granulomatous inflammatory process. Radionuclide imaging was inconclusive. The patient underwent an endomyocardial biopsy, which confirmed the diagnosis of systemic sarcoidosis with cardiac involvement. Conclusions: Systemic sarcoidosis with cardiac involvement is a challenging diagnosis. The role of imaging techniques such as transthoracic echocardiography, cardiac magnetic resonance imaging and radionuclide imaging is essential in raising suspicion and diagnosing this pathology. Endomyocardial biopsy is the 'gold standard' for its diagnosis; however, it has a low diagnostic yield. LEARNING POINTS: Systemic sarcoidosis with cardiac involvement is a challenging diagnosis as it may present in many different ways.The case presented highlights the importance of a thorough clinical history and physical examination, and the correlation with imaging findings.Imaging techniques such as transthoracic echocardiogram, cardiac magnetic resonance and radionuclide imaging are essential in raising suspicion and diagnosing cardiac sarcoidosis.

Clinical Application of Exercise Stress Echocardiography in an Outpatient Pediatric Population.

Background: Exercise stress echocardiography (ESE) is commonly employed in adults, but its applicability in pediatric populations remains to be clarified. Methods: A total of 309 consecutive children (C), with a mean age of 14.1 ± 2.6 years (range 6-17 years), underwent treadmill ESE starting in 2002. They were divided into two groups: Group I comprised 258 children, including 237 with symptoms related to exercise (such as chest pain, fatigue, lipothymia/syncope, or one aborted sudden death), 15 with electrocardiogram (ECG) abnormalities, and 6 with a positive ECG stress test showing ST changes. Group II consisted of 10 asymptomatic children whose parents requested routine screening, 11 with symptoms unrelated to exercise, 12 with a family history of sudden death, and 17 with known pathologies (including 10 with hypertrophic cardiomyopathy, 2 with aortic coarctation, and the remainder with various conditions, such as Cortriatriatum sinister, pulmonary stenosis, subaortic stenosis, bicuspid aortic valve, left ventricular hypertrophy related to arterial hypertension, and aortic switch operation). Regional wall motion abnormalities (RWMAs) and transvalvular or intraventricular (IVG) gradients were assessed using 2D and continuous-wave Doppler, respectively, in all cases. Results: The success rate was 100% (309/309). Stress-induced RWMAs were observed in two children. A significant IVG (>30 mmHg) was detected in 101 out of the 258 children (39%) in Group I, who presented with exercise-related symptoms, ECG abnormalities, or positive stress ECG. In Group I, the odds ratio (OR) of ESE reproducing the symptoms in children with IVG compared to those without IVG was 8.22 (95% CI: 4.84-13.99, p < 0.001). Conclusions: Treadmill ESE is both feasible and safe for pediatric populations. RWMAs demonstrated limited utility in our cohort of children, while IVG induced by exercise was frequently observed in symptomatic children.

The Importance of Left Ventricular Outflow Tract and Mid-Ventricular Gradients in Stress Echocardiography: A Narrative Review.

This review aims to serve as a guide for clinical practice and to appraise the current knowledge on exercise stress echocardiography in the evaluation of intraventricular obstruction in HCM, in patients with cardiac syndrome X, in athletes with symptoms related to exercise, and in patients with normal left ventricular systolic function and exercise-related unexplained tiredness. The appearance of intraventricular obstruction while exercising is considered rare, and it usually occurs in patients with hypertrophy of the left ventricle. The occurrence of intraventricular obstruction when exercising has been evidenced in patients with hypertrophic cardiomyopathy, athletes, patients with cardiac syndrome X, patients with syncope or dizziness related to exercise, and patients with dyspnea and preserved ejection fraction. The clinical significance of this observation and the exercise modality that is most likely to trigger intraventricular obstruction remains unknown. Supine exercise and lying supine after exercise are less technically demanding, but they are also less physiologically demanding than upright exercise. Importantly, in everyday life, human beings generally do not become supine after exercise, as takes place in post-exercise treadmill stress echocardiograms in most echocardiography labs. The presence of induced intraventricular obstruction might be considered when patients have exercise-related symptoms that are not understood, and to assess prognosis in hypertrophic cardiomyopathy.

Exercise-Induced Intraventricular Gradients As a Potential Cause of Sudden Cardiac Death.

A 16-year-old boy reported an episode of dizziness related to intense training six months before an episode of aborted sudden death. The screening required for competitive sports practice was normal. There were no personal or familial antecedents of sudden death or heart disease. After winning a triathlon competition, he experienced a cardiac arrest episode. He received defibrillation with the return of spontaneous circulation. A medical evaluation that included electrocardiogram (ECG) and echocardiogram had normal results. A complete study including cardiac MRI, coronary CT angiography, a genetic study for heart disease, the flecainide test, and a stress echocardiogram with ergometrine was done, and all results were normal. During a Holter ECG and exercise stress echo, isolated premature ventricular complexes were detected. During the effort treadmill stress echocardiogram, the athlete developed a significant intraventricular obstruction with an end-systolic peak, without systolic anterior movement of the mitral valve, which disappeared in the first minute of the recovery. We highlight the possible cause-effect relation between the events.

Intraventricular gradients-When we have to "exercise" our minds: A case report.

Increased intraventricular pressure gradients due to dynamic left ventricular outflow tract obstruction during exercise have long been known to cause different symptoms. Exercise stress echocardiography is fundamental in the diagnostic approach of symptoms presenting during exercise. We hypothesize on the possible pathophysiological mechanisms responsible for our patient's syncopal episodes.

Exercise stress echocardiography: Where are we now?

Exercise stress echocardiography (ESE) is a widely used diagnostic test in cardiology departments. ESE is mainly used to study patients with coronary artery disease; however, it has increasingly been used in other clinical scenarios including valve pathology, congenital heart disease, hypertrophic and dilated cardiomyopathies, athlete evaluations, diastolic function evaluation, and pulmonary circulation study. In our laboratories, we use an established methodology in which cardiac function is evaluated while exercising on a treadmill. After completing the exercise regimen, patients remain in a standing position or lie down on the left lateral decubitus, depending on the clinical questions to be answered for further evaluation. This method increases the quality and quantity of information obtained. Here, we present the various methods of exercise stress echocardiography and our experience in many clinical arenas in detail. We also present alternatives to ESE that may be used and their advantages and disadvantages. We review recent advances in ESE and future directions for this established method in the study of cardiac patients and underline the advantage of using a diagnostic tool that is radiation-free.