Severe familial coronary artery spasm in 2 siblings: About 2 cases.

Vasospastic angina is the spasm of coronary arteries causing transient myocardial ischemia. VSA is commonly managed with antispasmodic medications including calcium-channel blockers and nitrates. When vasospasm is refractory to conventional medications, unconventional treatment modalities may be used for symptomatic relief. Coronary artery spasm was observed in 2 sisters. Neither of them had significant atheromatous stenosis in the coronary arteries. The 22-year younger sister presented with rest angina in the early morning. The 32-year-old elder sister complained of rest and effort angina. Their coronary angiogram showed spontaneous spasm in the proximal segment of the left anterior descending coronary artery. The youngest one had resistant and recurrent coronary vasospasm involving different segments of the coronary tree causing myocardial infarction with total occlusion of the proximal segment in the left anterior descending coronary artery. Our patients presented with a lesser-known phenomenon called refractory VSA, where intermittent vasospasm continues despite being on a combination of 2 medications. The familial appearance of coronary artery spasm had been previously reported. Although it is not well understood, the underlying mechanism appears to involve a combination of endothelial damage and vasoactive mediators. Genetic factors such as human leucocyte antigen contribute to susceptibility to coronary spasm in some patients with VSA. Treatment for VSA is well documented; however, little data is available for refractory VSA.

Post-cardiac injury syndrome due to iatrogenic injury successfully managed medically: a case report.

BACKGROUND: Coronary artery perforation is a rare but serious complication of percutaneous coronary interventions, that may eventually lead to major and fatal events such as myocardial infarction, cardiac tamponade, and ultimately death. The risk of coronary artery perforation is more significant during complex procedures as chronic total occlusions but it can occur in other circumstances such as oversized stents and/or balloons, excessive post-dilatation, and the use of hydrophilic wires. Coronary artery perforation is often not recognized during the procedure and the diagnosis is frequently not made until later when the patient develops signs related to pericardial effusion. Thus, causing a delay in management and worsening the prognosis. CASE PRESENTATION: We report a case of a distal coronary artery perforation secondary to using a hydrophilic guide in a young male patient of 52-year-old arab, initially presented with an ST-segment elevation myocardial infarction, complicated by pericardial effusion that was treated medically with a favorable outcome. CONCLUSIONS: This work highlights that coronary artery perforation is a complication that must be anticipated in high-risk situations and its diagnosis must be made early to allow adequate management.

Mechanical prosthetic mitral valve obstruction: Pannus or thrombus? A case report.

Substitution of a defective heart valve with a prosthetic heart valve turns the native disease for prosthesis-related complications. One of the most serious and dreaded complications is prosthetic valve obstruction. It is either the result of a thrombus or pannus formation. For the evaluation of prosthetic valve obstruction, transthoracic echography and fluoroscopy provide functional information but may not provide information about the etiology of the obstruction, unlike multidetector computed tomography (MDCT) which allows a more precise etiological diagnosis to guide the therapeutic attitude. Here, we report a case of a mechanical prosthetic mitral valve obstruction in a 45-year-old patient in whom the diagnosis of pannus was retained on the basis of clinical, biological, and imaging data. The differentiation between thrombus and pannus is crucial because it conditions the therapeutic attitude. Advanced imaging specially MDCT options should be considered whenever mechanical prosthesis valve obstruction is suspected.

Mitral valvar anomalies causing subaortic stenosis: two rare case reports.

Subaortic stenosis (SubAS) is a common cause of left ventricular outflow tract obstruction. It may be focal or diffuse, leading to the development of a subaortic tunnel. Considered for a long time as a congenital anomaly, SubAS has been established lately as an acquired anomaly and secondary to a pre-existing anatomic alteration in the interventricular septum and mitral valve apparatus. It is a progressive disease that is often confused with obstructive hypertrophic cardiomyopathy, which can lead to several complications. Case presentation: In this paper, the authors report two cases of SubAS secondary to different mitral valvar anomalies. The analysis of echocardiographic data was a milestone in raising this diagnosis and identifying its mechanisms. Conclusion: This work highlights a rare situation, often underdiagnosed, in which the evolution can be marked by an important risk of recurrence even after surgical cure.

Anomalous Left Main Coronary Artery From the Right Sinus of Valsalva.

Anomalous coronary arteries are rare, mostly benign anatomic abnormalities. Anomalous origin of the left main coronary artery from the right sinus of Valsalva (LCA-RSV) is a rare variant that may lead to myocardial ischemia or sudden cardiac death. We present the case of a 49-year-old patient with a history of type 2 diabetes and smoking who presented to the emergency department with acute chest pain and was diagnosed with inferior ST-elevation myocardial infarction (STEMI). A transthoracic echocardiogram demonstrated inferolateral wall motion abnormalities of the left ventricle. The patient underwent cardiac catheterization that showed an anomalous left main coronary artery originating from the right sinus of Valsalva, alongside atherosclerotic triple-vessel disease. He was discharged home on medical management, including dual antiplatelet therapy, beta blockers, and statins, with scheduled follow-up.

Systemic sclerosis and tachycardia-bradycardia syndrome: a case report.

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.

Accelerated junctional rhythm (AJR) revealing light-chain cardiac amyloidosis: A case report with literature review.

Introduction and importance: Cardiac amyloidosis (CA) is a rare condition, characterized by fibrillary proteins infiltration in the extracellular space of the heart. Even though many types of cardiac amyloidosis exist, light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR) remain the most described forms. The diagnosis of amyloidosis represents a real challenge for clinicians, requiring both invasive and non-invasive investigations. Conduction defects and atrial arrhythmias are well known complications of cardiac amyloidosis. However, only a few studies have reported junctional rhythm a primary presentation of light chain cardiac amyloidosis (AL). An early diagnosis and proper management are crucial to improve the prognosis of this disease. Case presentation: Here, we report a rare case of a 48 year-old patient, in acutely decompensated heart failure, presenting an accelerated junctional rhythm (AJR) as initial presentation of light-chain cardiac amyloidosis. The diagnosis was made based on clinical, biological, radiological and histological findings. This case shows diagnostic difficulties and management of this rare disease.

Acute ST-elevation myocardial infarction (STEMI) in a young woman with unknown mitral stenosis and atrial fibrillation: Case report.

Introduction and importance: Chronic rheumatic heart disease is the most common cause of mitral stenosis. It remains a major public health problem. In almost half of the cases, paroxysmal or chronic atrial fibrillation occurs during the evolution of mitral stenosis, thereby exposing to an increased risk of thrombo-embolic events.Whereas the most frequent site for embolism is the cerebral circulation, any organ may be involved, especially the coronary circulation, resulting in a myocardial infarction (MI). Case presentation: Here, we report a rare case of a 50-year-old patient, with no risk factors for cardiovascular disease, presenting an acute ST-elevation myocardial infarction (STEMI) as initial presentation of unknown mitral stenosis with atrial fibrillation and strongly suggesting an embolic origin. The diagnosis was made based on the national cerebral and cardiovascular center (NCVC) criteria for the clinical diagnosis of coronary artery embolism (CE). Coronary angiography showed a distal thrombus in the right coronary artery that has been medically treated. The outcome was favorable and the patient was referred after that for mitral valve replacement.

Mitral aneurysm: a rare complication of subaortic diaphragm infective endocarditis / Aneurisma mitral: una complicación poco frecuente de la endocarditis infecciosa del diafragma subaórtico

Abstract Mitral valve aneurysm is a rare and uncommon complication of infective endocarditis leading to a weakened mitral tissue. The most feared mitral valve aneurysm's complications are: perforation and severe mitral regurgitation. Multiple mechanisms have been suggested to explain the development of mitral valve aneurysm in aortic infective endocarditis including: local extension of the infection, the mitral kissing vegetation and aortic regurgitation. We report the case of a 29-year-old man who had infective endocarditis of a native aortic valve and the sub-aortic diaphragm complicated by a perforated mitral valve aneurysm diagnosed only by transesophageal echocardiogram. The patient had no sign of heart failure. We hypothesized that all those mechanisms lead to the developing of the mitral valve aneurysm in this case, but also the presence of the diaphragm favored the spread of the infection.

Resumen Una de las complicaciones poco comunes e inusuales de la endocarditis infecciosa son los aneurismas de la válvula mitral. Las complicaciones más temidas de estos últimos son la perforación y la insuficiencia mitral severa. Diferentes mecanismos pueden explicar el desarrollo de dicha insuficiencia en el contexto de una endocarditis infecciosa, incluyendo una extensión local, una vegetación sobre la válvula mitral y una insuficiencia aórtica. Nuestro caso clínico es el de un joven de 29 años diagnosticado de endocarditis infecciosa sobre su válvula aortica nativa con un diafragma subaortico complicado con un aneurisma de la válvula mitral perforado. El paciente no presentaba signos de insuficiencia cardíaca. Hemos supuesto que todos los mecanismos que hemos evocado anteriormente han llevado al desarrollo de dicho aneurisma, junto con la presencia del diafragma, elemento en favor de la diseminación de la infección.

Myocardial infarction in a pregnant woman revealing a transitional deficit in protein S: a rare case report.

The occurrence of myocardial ischemia and myocardial infarction in pregnancy is relatively rare, the occurrence of myocardial infarction in pregnancy is associated with cardiovascular risk factors. The deficiency of coagulation regulatory systems in the occurrence of venous thrombosis is well established; however, their role in arterial thrombosis is controversial. Here, we present an interesting case of a 34-year-old of acute myocardial syndrome without persistent ST segment elevation, which revealed transient protein S deficiency. Management of acute coronary syndrome (ACS) during pregnancy may represent a unique clinical challenge. In this manuscript, we review the clinical presentation, anatomic considerations, and management strategy in our patient presenting with ACS. Objective: this case highlights the importance of multimodality approach to help to obtain a more timely diagnosis of myocardial infarction in pregnancy and the importance collaboration between obstetricians, cardiologists, pediatricians and anesthesiologists to ensure optimal care.

Corrigendum: Kounis syndrome induced by oral intake of aspirin: case report and literature review.

[This corrects the article DOI: 10.11604/pamj.2018.30.301.14948.].

[Inferior myocardial infarction: first Moroccan study of 103 cases]. / Infarctus du myocarde inférieur: première série marocaine, à propos de 103 cas.

Myocardial infarction (MI) is a major cause of cardiovascular mortality. Inferior MI accounts for 30-50% of infarctions but it is associated with a favorable prognosis compared to anterior infarct. This study aimed to study the epidemiological, clinical, electrical, echocardiographic, angiographic features of inferior MI, as well as its complications and its therapeutic approaches. Over a period of 3 years, we admitted 720 patients with STEMI, of whom 103 with inferior STEMI, reflecting a rate of 14.3%. There was a clear male predominance, with an average age of 58 years (men) and 62 years (women). Smoking was the main risk factor for cardiovascular disease (57.28% of patients were smokers). Right ventricular infarction was found in 11.65% of cases. Half of these patients had hemodynamic instability. Third-degree atrioventricular block was diagnosed in 12.6% of patients. Therapeutic approach was based on thrombolysis (7 patients) and coronary angiogram (42 patients). Inferior MI was caused by right coronary lesion in 53% of cases and circumflex artery occlusion in 47% of cases. Right coronary is responsible for right ventricular infarction in 100% of cases. Coronary angioplasty was performed in 18 patients after coronary angiogram. Eleven patients underwent transluminal coronary angioplasty in the right coronary while 2 patients underwent transluminal coronary angioplasty in the circumflex artery. Early mortality at 30 days was 1.94%. In the right ventricular infarction group mortality rate was about 17%.

[Perforated posterior mitral valve aneurysm: a rare complication of infective endocarditis: a case report]. / Perforation d'anévrysme de la valve mitrale postérieure: complication rare de l'endocardite infectieuse: à propos d'un cas.

Mitral valve aneurysm is a rare abnormality whose pathophysiology is poorly understood. It is defined as a bulge of the mitral valve leaflet toward the left atrium. Aneurysm in the posterior leaflet is exceptional. We report the case of a 26-year old man, who had been followed up for rheumatic aortic regurgitation for 4-years, hospitalized for febrile syndrome associated with episodes of left heart failure. Transthoracic echocardiographic examination (TTE) and transesophageal echocardiography (TEE) showed aortic valve vegetations with wide aneurysm of the small mitral valve associated with severe mitral valve regurgitation. The patient underwent mitral and aortic valve replacement with simple postoperative outcome. Clinical suspicion associated with suitable preoperative imaging and early surgical treatment are essential to recognize and treat this rare complication of infectious endocard.

Kounis syndrome induced by oral intake of aspirin: case report and literature review.

The Kounis-Zavras syndrome is defined as the coincidental occurrence of acute coronary events and hypersensitivity reactions following an allergic reaction including a mast-cell degranulation of vasospastic mediators. This report describes a case of Kounis-Zavras syndrome in the setting of aspirin-induced asthma also known as Samter-Beer triad combining nasal polyps, asthma, and aspirin allergy leading to vasospasm and myocardial infarction. All physicians should be aware of The Kounis syndrome and always keep that unique clinical entity in mind to recognize it promptly and direct the therapy at suppressing the allergic reaction.

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.

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

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.

Multiple mycotic aneurysms reveal Staphylococcus lugdunensis endocarditis in a young patient.

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.

Cardiac thrombosis as a manifestation of Behçet syndrome.

Herein, we report 3 cases of Behçet syndrome that were accompanied by intracardiac thrombus. The 1st patient was a 30-year-old man who presented with dyspnea; a right atrial thrombus was identified upon transthoracic echocardiography. The 2nd patient was a 52-year-old man who was admitted for dyspnea; transthoracic echocardiography revealed an echogenic mass in the right ventricle. The 3rd patient was a 23-year-old man who was hospitalized for hemoptysis; the diagnosis of pulmonary embolism was made, and right ventricular thrombosis was found.Because these patients had no hemodynamic compromise, medical management consisting of immunosuppressive and anticoagulative therapy was adopted. This treatment resulted in complete dissolution of the thrombi.

Management of Behçet disease with multiple complications.

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.