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

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

Insights from magnetic resonance imaging of left ventricular non-compaction in adults of North African descent

<p>Abstract</p> <p>Background</p> <p>Left ventricular non-compaction (LVNC) is a recently recognized rare disorder. Magnetic resonance imaging (MRI) may help to clarify the uncertainties related to this genetic cardiomyopathy. Despite the fact that many articles have been published concerning the use of MRI in the study of LVNC, there is a lack of data describing the disease in the North African population. The aim of our study is to clarify MRI findings of LVNC in North African patients.</p> <p>Methods</p> <p>In our retrospective cohort, twelve patients (7 male, mean age 53 ± 8 years) underwent MRI for suspected LVNC. Correlations were investigated between the number of non-compacted segments per patient and left ventricular ejection fraction (LVEF), then between the number of non-compacted segments and left ventricular end diastolic diameter. The presence or absence of late gadolinium enhancement (LGE) was qualitatively determined for each left ventricular myocardial segment.</p> <p>Results</p> <p>Non-compaction was more commonly observed at the apex, the anterior and the lateral walls, especially on their apical and mid-cavity segments. 83% of patients had impaired LVEF. There was no correlation between the number of non-compacted segments per patient and LVEF (r = -0.361; p = 0.263), nor between the number of non-compacted segments per patient and left ventricular end diastolic diameter (r = 0.280; p = 0.377). LGE was observed in 22 left ventricular segments. No association was found between the pattern of fibrosis and non-compaction distribution (OR = 2.2, CI [0.91-5.55], p = 0.076).</p> <p>Conclusion</p> <p>The distribution of LVNC in North African patients does not differ from other populations. Ventricular dysfunction is independent from the number of non-compacted segments. Myocardial fibrosis is not limited to non-compacted areas but can extend to compacted segments.</p

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

Intermittent obstruction of the left coronary artery ostia by a congenital supravalvular aortic membrane in an adult

Left main coronary artery obstruction due to a congenital supra-aortic membrane is a rare anomaly, but it can have serious consequences, including significant myocardial ischemia that may result in impaired ventricular function or even sudden death. Therefore, early diagnosis and treatment of this condition are crucial for improving patient outcomes. We present, in this report, a patient with intermittent obstruction of the left coronary arteries caused by a congenital ostial membrane diagnosed in adulthood that resulted in myocardial ischemia

Une polyradiculonévrite inflammatoire démyélinisante chronique paranéoplasique secondaire à un lymphome natural killer nasal

Nous rapportons un cas unique d´une polyradiculonévrite inflammatoire démyélinisante chronique paranéoplasique secondaire à un lymphome non hodgkinien T de type&nbsp;natural killer&nbsp;nasal

Left ventricular noncompaction—A rare form of cardiomyopathy: Revelation modes and predictors of mortality in adults through 23 cases

Objectives: To describe modes of clinical presentation and echocardiographic, angiographic, and rhythmic features, and prognostic characteristics of left ventricular noncompaction cardiomyopathy (LVNC) in North African adults, through one of the first series in Morocco. Background: LVNC is a rare congenital disorder, described for the first time by Engberding in 1984. The suspected diagnosis in thromboembolic, hemodynamic, or rhythm events requires both echocardiography and cardiovascular magnetic resonance (CMR). Its therapeutic management is not yet well codified but akin to that proposed for dilated cardiomyopathy. Patients and design: This study included a retrospective, descriptive, and analytical cohort of 23 cases of cardiomyopathy with LVNC diagnosed in the Noninvasive Explorations Laboratory at the Military Hospital of Rabat, Morocco, between January 2009 and October 2014. The echocardiographic criteria for LVNC include the absence of any coexisting cardiac anomalies. The characteristic appearance of numerous excessively prominent trabeculations and deep intertrabecular recesses and intertrabecular spaces filled by direct blood flow from the ventricular cavity, as visualized on color Doppler imaging with noncompacted/compacted ratio > 2 according to Jenni criteria. Twenty-three adults fulfilled the diagnostic criteria and were followed prospectively. Results: At diagnosis, the mean age was 47 ± 13 years with a male predominance at 65.2%. Of them, 56.5% had a left bundle branch block and 21.7% were in atrial fibrillation. Left ventricular end-diastolic diameter was 67.7 ± 6.6 mm and ejection fraction was at 27 ± 8%. Apex and/or midventricular segments of both the inferior and lateral wall were involved in more than 80% of patients with an average of 4.8 noncompacted segments. CMR was performed in 12 patients and was decisive for the diagnosis. Major complications were heart failure in 31% of patients, ventricular tachycardia in three patients, and thromboembolic events in one patient. Twenty eight point six percent of patients started a long-term anticoagulant therapy. One patient underwent implantation of a double-room pacemaker. Automated defibrillators were implanted in two patients. There were three deaths: one sudden death and two end-stage heart failure. Conclusion: LVNC should be looked for at any dilated cardiomyopathy particularly in young patients. It requires a careful echocardiographic examination and sometimes CMR to confirm the diagnosis. It is characterized by severe systolic and diastolic dysfunction that would provide poor prognosis