Search

Background Wolff-Parkinson-White (WPW) syndrome is a condition characterized by the persistence of an accessory pathway responsible for ventricular pre-excitation that can lead to symptomatic and potentially severe arrhythmias. Coexistence with atrial fibrillation is well known and not uncommon, exposing to potential degenerescence into ventricular fibrillation when atrial impulses are transmitted along the accessory pathway. WPW syndrome is most prevalent in younger patients and cases revealed after an advanced age have rarely been described in the literature. Case presentation Here, we report a case of atrial pre-excitation first diagnosed at the age of 72 years that required external electrical cardioversion with a favorable outcome. The diagnosis was based on clinical and electrographic findings. Conclusions WPW syndrome is a relatively rare cardiac disorder that can be a cause of sudden death, especially when combined with atrial fibrillation. Therefore, cardiologists have to consider this diagnosis in patients presenting clinical signs of arrhythmia with an electrical pattern of WPW.

COVID 19 so far is not a known cardiotropic virus, and the term “myocarditis” should be exclusively used after EMB or autopsy proven diagnosis. We report a case of 26-year-old man admitted for COVID 19 infection and symptoms leading to myocarditis. We describe the workup that led to the potential diagnosis.

Background Systemic sclerosis is a multisystemic character autoimmune disease. It is characterized by vascular dysfunction and progressive fibrosis affecting mainly the skin but also different internal organs. All heart structures are commonly affected, including the pericardium, myocardium, and conduction system. However, tachycardia–bradycardia syndrome is not common in the literature as a cardiac complication of systemic sclerosis. Case presentation We report a case of tachycardia–bradycardia syndrome in a 46-year-old Moroccan woman followed for systemic sclerosis with cutaneous, vascular, and articular manifestations. The diagnosis was based mainly on patient-reported symptoms and electrocardiogram data. A permanent pacemaker was implanted, allowing the introduction of beta-blockers with good outcomes. Conclusions This case aims to show that even minor electrocardiogram abnormalities should be monitored in this group of patients, preferably by 24-hour ambulatory electrocardiogram because they could be a good indicator of the activity and progression of cardiac fibrosis.

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.

2019 ESC Guidelines for the management of patients with supraventricular tachycardia : The Task Force for the management of patients with supraventricular tachycardia of the European Society of Cardiology (ESC): Developed in collaboration with the Association for European Paediatric and Congenital Cardiology (AEPC)

A 39 yo female patient was admitted for symptomatic complete atrioventricular Block. A family history and screening showed 5 cases of sudden death, and 4 cases of complete atrioventricular block, treated with Pacemaker implantation. Genetic screening showed a new variant in TRPM4 gene: c.512 G >A; p. Arg171Gln.

Key Clinical Message This report highlights the importance of a more cautious approach in a patient with a history of implanted cardiac leads presenting with chest pain or dyspnea, to prevent overlooking cardiac lead perforations especially in hypertrophic cardiomyopathy which seems to be not absolutely protective.

Background Hypertrophic cardiomyopathy (HCM) is a myocardial disease characterized by an unexplained left ventricular hypertrophy (LVH), usually asymmetrical and involving the interventricular septum. HCM is a relatively frequent disorder, affecting one of 500 individuals from the general population. Some patients may not have any symptoms during their whole life, whereas in other cases, HCM may lead to severe symptoms such as syncope or dyspnea, sudden death, or congestive heart failure. It remains the most prevalent cause of inexplained sudden death in athletes during exercise. HCM is a familial disease in at least 50% of cases, with mainly an autosomal dominant mode of inheritance with variable expression and incomplete penetrance. It may be also inherited in autosomal recessive pattern or X-linked manner. HCM are caused by mutations in nearly 22 different genes, >17 genes are involved in autosomal dominant HCM. The most frequently mutations fall within myosin heavy chain 7 (MYH7) and myosin binding protein C (MYBPC3), both accounting for up to 50% of HCM cases. Case presentation The present study reports the molecular results of a series of six unrelated Moroccan patients with HCM using for the first time in Morocco a next generation sequencing (NGS) customized multigene panel to investigate the two major HCM genes MYH7 and MYBPC3. Genetic testing lead to the identification of a novel MYBPC mutation (c.1049delA; p.Lys350fs) and three others previously described variants at heterozygous state. Conclusion Molecular diagnosis by NGS customized multigenes panel allowed us to set up a fast and firstline upon request cost-efficient strategy in order to screen various genes and diseases including HCM. This approach is well suited to general medical genetics laboratories dealing with almost all types of rare genetic diseases with limited funds for a molecular testing more cost-effective.

Mycotic aneurysms are rare, and depending on their location, can threaten functional prognosis. We report on a 17-year-old girl with no previous history of cardiovascular or infectious disease, referred to our Department of Cardiology with right hemiplegia and aphasia. A neurological evaluation revealed thrombosis of a mycotic cerebral aneurysm, complicated by ischemic and hemorrhagic infarction. Transthoracic echocardiography indicated huge, highly mobile mitral vegetation associated with a mitral regurgitation with a triple stream. Hemocultures isolated Staphylococcus lugdunensis. Shortly afterward, she developed bilateral tibial and pedal mycotic aneurysm. The patient received antibiotics, with minor neurological improvement initially, but she soon died because of a brain herniation. Based on our findings in this case, we discuss the features of endocarditis attributable to S. lugdunensis.

Neuromuscular Diseases are a heterogeneous molecular, clinical and prognosis group. Progress has been achieved in the understanding and classification of these diseases. Cardiac involvement in neuromuscular diseases namely conduction disorders, ventricular dilatation and dilated cardiomyopathy with its impact on prognosis, is often dissociated from the peripheral myopathy. Therefore, close surveillance is mandatory in the affected patients. In this context, preventive therapy (beta-blockers and angiotensin converting enzyme inhibitors) has been recently recommended in the most common Neuromuscular Diseases, Duchenne Muscular Dystrophy and Myotonic Dystrophy.

La maladie de Steinert ou dystrophie myotonique de type 1 (DM1) est une maladie génétique à transmission autosomique dominante caractérisée par une myotonie et une atteinte de plusieurs organes dont le céur. L'atteinte cardiaque est la plus grave des atteintes systémiques puisqu'elle conditionne le pronostic vital. Ce travail a pour but de déterminer les anomalies cardiaques rencontrées au cours de la DM1 et de mettre en exergue l'intérêt d'un examen cardiaque rigoureux et régulier, indépendamment de la sévérité de l'atteinte neuromusculaire, ainsi que l'apport des examens cardiaques complémentaires et notamment l'exploration électrophysiologique. 18 patients atteints de DM1 ont bénéficiés d'une exploration cardiaque systématique. Il s'agit de 9 hommes et de 9 femmes, d'âge moyen de 41,8 +/- 16,2 ans. 66 p.100 des patients sont symptomatiques sur le plan cardiovasculaire. Les anomalies électrocardiographiques sont dominées par un trouble de la conduction intra-ventriculaire dans 16 p.100 des cas et un BAV de 1er degré dans 16 p.100 des cas. L'Holter ECG objective une hyperexcitabilité à l'étage atrial et/ou ventriculaire dans 50p.100 des cas. L'ETT est normale chez 95 p.100 des patients. L'exploration électrophysiologique, réalisée chez 4 patients symptomatiques, a objectivé un bloc tronculaire dans un cas ayant conduit à l'implantation d'un PM double chambre. Un seul patient est décédé suite à une détresse respiratoire. Enfin, on n'a pas noté de corrélation entre l'atteinte cardiaque et neuromusculaire. Une exploration cardiaque est indispensable chez tout patient atteint de DM1, en dépit de l'absence de symptômes, et un bilan annuel minimal s'impose pour guetter un éventuel trouble rythmique et/ou conductif, fatal en l'absence de traitement adéquat.

Title : Paroxysmal extreme pain disorder (PEPD) is an autosomal dominant painful neuropathy with many,but not all, cases linked to gain-of-function mutations in SCN9A which encodes voltage-gated sodium channel Na.1.7. It is a very rare condition featured by flushing of the lower half of the body and excruciating burning pain caused by any stimulus below the waist or in the perianal region. PEPD may be associated with cardiovascular instability, especially prolonged sinus pauses, and thus has anesthetic implications. Pacemaker implantation is the alternative therapeutic option, but its indications have not been clarified yet. Background: This condition is well described in neurological literature, but to our knowledge, this is the first case report of a patient with paroxysmal extreme pain disorder with prolonged sinus pauses requiring anesthesia for an epicardial pacemaker even with the peri-operative risk of the pathology.This clinical observation can help for a better management and understanding of the cardiac risk complications of PEPD especially for an infant whose diagnostic is frequently made at the stage of complication This clinical observation can put the item on the necessity of establishing recommendations for management of cardiac complications during PEPD. Case report : We extensively searched the literature on cardiac pacing in patients with PEPD and we described a new case of a 9 month old infant who was admitted in the emergency department for an episode of malaise apnea and hemifacial cyanosis relevant to PEPD. The neurologic exploration was normal. The diagnostic was confirmed by genetic study. The 24 hours recording demonstrated long pauses of 15 seconds during the crisis justifying the implantation of epicardial pacemaker without peri-operatory complications due to the high anesthetic risk of this pathology. Conclusion : Paroxysmal extreme pain disorder is a highly distinctive sodium channelopathy with incompletely carbamazepine-sensitive bouts of pain and sympathetic nervous system dysfunction. It is most likely to be misdiagnosed as epilepsy and, particularly in infancy, as hyperekplexia and reflex anoxic seizures. Clinicians must evocate this diagnostic even any clinical suspicion given the severity of cardiac complications.

Le bloc auriculo-ventriculaire (BAV) représente une complication non négligeable de la chirurgie cardiaque. Il est responsable de séquelles lourdes et graves compromettant le pronostic de la maladie et conduisant à la mise en place d'un stimulateur cardiaque définitif. Il est primordial d'étudier et de déterminer les différents facteurs prédictifs de BAV post opératoire, son histoire naturelle, son incidence ainsi que le délai exact de la mise en place d'un pacemaker. Notre étude est une étude rétrospective descriptive à propos de 23 patients opérés pour chirurgie cardiaque sous circulation extracorporelle entre octobre 1989 et août 2010 ayant présentés des troubles conductifs auriculo-ventriculaires en post opératoire..Plusieurs facteurs de risque ont été étudiés dans notre série, liés surtout à l'atteinte directe du noeud auriculo-ventriculaire ou à l'ischémie myocardique. Le délai d'apparition du trouble conductif ainsi que le délai d'implantation on été également étudié. Plusieurs facteurs favorisants de survenue de BAV ont été identifiés liés essentiellement au type de la cardiopathie opérée avec une prédominance de la cardiopathie congénitale, d'autres facteurs ont été rapportés notamment la présence d'hypertrophie ventriculaire gauche (HVG) de troubles conductifs en préopératoire, une durée du clampage aortique et de CEC prolongée. La moitié des patients ont présenté un BAV immédiat. Le délai d'implantation par rapport à la date de la découverte du BAV varie dans notre série d'une implantation immédiate après le diagnostic positif (J0) à un délai d'implantation allant jusqu'à 57 jours. Plusieurs facteurs de risques déterminent la survenue de BAV post chirurgie cardiaque, leur connaissance est primordial ainsi que le délai exact de l'implantation du stimulateur cardiaque définitif.

Le bloc auriculo-ventriculaire (BAV) représente une complication non négligeable de la chirurgie cardiaque. Il est responsable de séquelles lourdes et graves compromettant le pronostic de la maladie et conduisant à la mise en place d'un stimulateur cardiaque définitif. Il est primordial d’étudier et de déterminer les différents facteurs prédictifs de BAV post opératoire, son histoire naturelle, son incidence ainsi que le délai exact de la mise en place d'un pacemaker. Notre étude est une étude rétrospective descriptive à propos de 23 patients opérés pour chirurgie cardiaque sous circulation extracorporelle entre octobre 1989 et août 2010 ayant présentés des troubles conductifs auriculo-ventriculaires en post opératoire. Plusieurs facteurs de risque ont été étudiés dans notre série, liés surtout à l'atteinte directe du noeud auriculo-ventriculaire ou à l'ischémie myocardique. Le délai d'apparition du trouble conductif ainsi que le délai d'implantation on été également étudié. Plusieurs facteurs favorisants de survenue de BAV ont été identifiés liés essentiellement au type de la cardiopathie opérée avec une prédominance de la cardiopathie congénitale, d'autres facteurs ont été rapportés notamment la présence d'hypertrophie ventriculaire gauche (HVG) de troubles conductifs en préopératoire, une durée du clampage aortique et de CEC prolongée. La moitié des patients ont présenté un BAV immédiat. Le délai d'implantation par rapport à la date de la découverte du BAV varie dans notre série d'une implantation immédiate après le diagnostic positif (J0) à un délai d'implantation allant jusqu’à 57 jours. Plusieurs facteurs de risques déterminent la survenue de BAV post chirurgie cardiaque, leur connaissance est primordial ainsi que le délai exact de l'implantation du stimulateur cardiaque définitif.

Objective Isolated ventricular noncompaction is a rare primary genetic cardiomyopathy characterized by persistent embryonic myocardial morphology without any other cardiac anomalies. Arrhythmias are frequently present, including both tachyarrhythmia and conduction disturbance. Our study aimed to describe the electrocardiographic findings and to correlate them with the clinical presentation and cardiac magnetic resonance imaging findings. Methods We retrospectively reviewed 24 patients diagnosed with isolated ventricular noncompaction (IVNC) by cardiac magnetic resonance imaging. Correlations were investigated between arrhythmias and the site of ventricular noncompaction, number of noncompacted segments, presence of fibrosis, and left ventricular dysfunction. Results The mean age was 42.7±13.1 years. Patients were first presented with heart failure in 41.7% and arrhythmia in 45.8%. Electrocardiogram was abnormal in 91.6% of patients; the most common anomaly was left bundle branch block (LBBB) (41.7%), followed by supraventricular arrhythmias (29.1%), repolarization abnormalities (29.1%), and ventricular tachycardia (20.8%). A normal left ventricular systolic function was frequently observed in patients who first presented with rhythm disorders than heart failure (p=0.008). There was also a delayed diagnosis of IVNC when presented with arrhythmia versus heart failure (p=0.02). We found no correlation between arrhythmias and the noncompaction site or fibrosis, except for LBBB, which was associated to left ventricle lateral wall involvement (p=0.028). No correlation between systolic dysfunction and the number of noncompacted segments, fibrosis, or arrhythmia was demonstrated. Conclusion While electrocardiographic abnormalities are frequent in isolated ventricular noncompaction, no specific patterns were identified. More large studies are needed for stratification of arrhythmic risk of this highly arrhythmogenic substrate.

Background 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. Methods 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. Results 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). Conclusion 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.

From December 1994 to May 1999, 487 patients underwent percutaneous transvenous mitral commissurotomy according to the Inoue balloon technique for tight mitral stenosis. In three cases, the Inoue balloon technique was not possible because the catheter balloon could not enter the mitral orifice and the double-balloon technique was performed instead. In two cases, a flow-guided balloon catheter met the mitral orifice and the double-balloon technique was effective. In the third case, passing the valve with a flow balloon catheter was impossible, a 0.032 Terumo straight wire crossed the valve, which was dilated with a single balloon the first time and a double balloon the second time. The mitral valve area increased in the three cases from 0.8 to 1.8 cm2, from 0.7 to 1.6 cm2, and from 0.5 to 1.5 cm2, respectively. There were no complications. (J Interven Cardiol 2001; 14:17–19)

We present the case of an 18-year-old male patient with Behçet's disease who presented with dyspnoea, fever and haemoptysis. A diagnosis of right ventricular thrombosis associated with endomyocardial fibrosis, a pulmonary artery aneurysm, and cerebral vein thrombosis was made. Considering the absence of haemodynamic compromise and the risk of recurrence after surgical treatment for cardiac thrombus, we preferred medical management, which consisted of immunosuppression and anticoagulation. During a follow-up period of 9 months we observed complete dissolution of the thrombus and dramatic improvement of the patient's clinical status.