Right Ventricle Outflow Obstruction in Biventricular Hypertrophic Cardiomyopathy in Amyloidosis

Light-chain (AL) amyloidosis is the most common type of amyloidosis; cardiac involvement is rare but has a poor prognosis. Biventricular hypertrophic cardiomyopathy is an exceptional finding in amyloidosis and its association with obstructive right ventricular gradient is even rarer. We report the case of a male patient with biventricular hypertrophy suggesting amyloidosis, with an obstructive gradient in the right ventricle

Kearns Sayre syndrome: a rare etiology of complete atrioventricular block in children (case report)

Kearns Sayre syndrome is a rare mitochondrial abnormality first described in 1958, characterized by a triad associating progressive external ophthalmoplegia, ptosis, and pigmentary retinopathy with progressive alteration of cardiac conduction, which determines the vital prognosis of this entity. Here we report the case of a 13-year-old child of consanguineous parents who consults for recurrent syncope. The clinical exam found bilateral ptosis with complete atrioventricular block on electrocardiogram. The ophthalmological exam found pigmentary retinopathy. The patient underwent successful implantation of a double chamber pacemaker within 24 hours of admission, with an uneventful postoperative course. This case report highlights the interest of systematically assessing cardiac complications in children with mitochondrial disease such as Kearns Sayre syndrome, especially since cardiac involvement is the major prognostic factor in this disease

Enigma of Huge mass filling the apical wall of the left ventricle. Is it thrombus, tumor or endomyocardial fibrosis?

Background: Cardiac masses have a wide range of etiologies with the most common being thrombi and less commonly tumors. Certain areas of the heart have specific tumor predilections. We present an interesting case in which the diagnosis of the ventricular mass was difficult given the initial symptomatology and the patient's phobia. Case summary: A 45-year-old male patient with medical history of smoking, was admitted to the emergency room complaining of severe breathlessness, However, the patient declined any chest discomfort or pain. On physical examination he was dyspneic, with vital signs revealed heart rate of 110 beats/min, blood pressure of 125/90 mmHg. Cardiovascular examination showed regular heart rhythm and normal heart sounds. Electrocardiogram revealed sinus tachycardia, chest X-ray was suggestive of cardiomegaly. Transthoracic Echocardiography demonstrated a dilated left ventricle with severely reduced ejection fraction and diffuses kinetic disturbances. The presence of a huge mass filling the apical wall of the left ventricle, hyperechogenic in appearance and very adherent to the ventricle. In front of this doubtful appearance at the echocardiography the patient was programmed for a cardiac magnetic resonance imaging which could not be carried out because of the claustrophobic state of the patient. In the meantime, the patient had a cardiac arrest due to refractory ventricular fibrillation successfully resuscitated. In front of this complication, he benefited from an automatic implantable defibrillator as a secondary prevention. Subsequently, a coronary angiography was performed in the context of the patient's smoking habits and kinetic disorders, to our surprise showed a complete occlusion of the left anterior descending artery knowing that the patient is not diabetic and he never complained of any painful symptomatology, primary coronary intervention was successfully performed to the left anterior descending artery. The patient was started on therapeutic anticoagulation and heart failure therapy. Follow-up imaging after 6months showed a significant improvement of the left ventricular systolic function and thrombus regression to apical segment without embolic events. Conclusion: The diagnosis of a ventricular mass remains difficult, the orientation can be done initially by the ground of the patient while being helped by the transtoracic echography and the multimodality imagery at the end to specify the nature of the intra-cardiac mass for an adequate and fast management. Keywords: Thrombus, ventricular mass, coronary angiography, heart failure, oral anticoagulation. DOI: 10.7176/JHMN/98-05 Publication date:March 31st 202

Management of Cardiac Involvement in NeuroMuscular Diseases: Review

Neuromuscular Diseases are a heterogeneous molecular, clinical and prognosis group. Progress has been achieved in the understanding and classification of these diseases

A complicated steroid resistant nephrotic syndrome: a large intracardiac thrombus with bilateral distal embolization

Nephrotic syndrome (NS) is a consequence of the reduced ability of the glomerulus barrier to exclude proteins of intermediate size and other macromolecules from urine. Albumin and proteins that modulate the coagulation cascade are among the substances eliminated in urine. This is responsible of thromboembolic complication. The incidence of this extra renal complication is probably underestimated because of asymptomatic thromboembolic events. We report the case of a 23 years old man followed up for an idiopathic nephrotic syndrome since childhood who presented a large intracardiac thrombus complicated by distal embolisation of his two lower limbs, successfully treated by fogartisation-embolectomy and oral anticoagulation

Use of magnetic resonance imaging in assessment of constrictive pericarditis: a Moroccan center experience

<p>Abstract</p> <p>Background</p> <p>The diagnosis of constrictive pericarditis continues to be a clinical challenge. Magnetic resonance imaging provides excellent visualization of the pericardium. The aim of our study is to clarify the contribution of this non invasive exploration in the diagnosis of constrictive pericarditis in our center.</p> <p>Methods</p> <p>we conducted a prospective study over a period of two years, since 2008, covering a series of patients (n = 11), mean age 44 ± 15 years, in whom constrictive pericarditis was suspected clinically and on transthoracic echocardiography. We studied its characteristics on magnetic resonance imaging.</p> <p>Results</p> <p>Magnetic resonance imaging confirmed the diagnosis showing pericardial thickening in all cases, measuring 8.2 +/- 2.6 mm on average, circumferential in 64%, and localized in 36%. The imaging data, particularly pericardial thickening and its topography, were confirmed by surgical exploration, and results were concordant in all cases.</p> <p>Conclusion</p> <p>Magnetic resonance imaging is a powerful tool to establish constrictive pericarditis diagnosis.</p

Data sharing: A new editorial initiative of the international committee of medical journal editors. Implications for the editors´ network

The International Committee of Medical Journal Editors (ICMJE) provides recommendations to improve the editorial standards and scientific quality of biomedical journals. These recommendations range from uniform technical requirements to more complex and elusive editorial issues including ethical aspects of the scientific process. Recently, registration of clinical trials, conflicts of interest disclosure, and new criteria for authorship -emphasizing the importance of responsibility and accountability-, have been proposed. Last year, a new editorial initiative to foster sharing of clinical trial data was launched. This review discusses this novel initiative with the aim of increasing awareness among readers, investigators, authors and editors belonging to the Editors´ Network of the European Society of Cardiolog

Data sharing: A new editorial initiative of the international committee of medical journal editors. Implications for the editors´ network

The International Committee of Medical Journal Editors (ICMJE) provides recommendations to improve the editorial standards and scientific quality of biomedical journals. These recommendations range from uniform technical requirements to more complex and elusive editorial issues including ethical aspects of the scientific process. Recently, registration of clinical trials, conflicts of interest disclosure, and new criteria for authorship -emphasizing the importance of responsibility and accountability-, have been proposed. Last year, a new editorial initiative to foster sharing of clinical trial data was launched. This review discusses this novel initiative with the aim of increasing awareness among readers, investigators, authors and editors belonging to the Editors´ Network of the European Society of Cardiolog

Case Roport: Pericardial tamponade and coexisting pulmonary embolism as first manifestation of non-advanced lung adenocarcinoma

Pericardial effusion and pulmonary embolism are relatively common complications of malignancy and are  uncommon as its initial manifestation. This report describes a case of a patient, who presented with this  association, due to an underlying pulmonary adenocarcinoma. When a major pericardial effusion is associated with pulmonary hypertension, some echocardiographic signs may redress the diagnosis. This case  emphasizes a challenge diagnostic which may be guided by high right ventricular pressure and on the other hand the importance of keeping both these conditions in mind when dealing with context of malignancy.Key words: Pericardial tamponade, pulmonary embolism, lung adenocarcinom