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Background: Lutembacher's syndrome refers to the rare combination of atrial septal defect and mitral stenosis. This condition is still underdiagnosed despite its distinct clinical, paraclinical, and therapeutic implications. Case summary: We report the case of a 60-year-old woman presenting with acute congestive heart failure. Investigations revealed the combination of low-gradient severe rheumatic mitral stenosis and ostium secundum atrial septal defect. Despite the medical team's conservative management of her condition and recommendation for surgical intervention, the patient chose to discharge herself against medical advice. She died 6 months later. Conclusion: Lutembacher's syndrome continues to be a rare condition in our practice. Once low-gradient severe mitral stenosis is diagnosed, it is essential to search for an associated atrial septal defect, especially when there is an early onset of significant right ventricular enlargement or dysfunction or when an intriguing inconsistency exists between the mitral area and the transmitral gradient.
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Atrial flutter, a common cardiac arrhythmia, is characterized by rapid and regular atrial contractions that result in a characteristic sawtooth pattern on the electrocardiogram. It emerges due to the formation of reentrant electrical circuits within the atria, giving rise to structured, sawtooth-patterned atrial waves as observed on electrocardiography. We present the case of a 52-year-old female with a medical history of ankylosing spondylitis, dyslipidemia, and a previous surgical closure of an atrial septal defect. The patient developed a rare form of atrial flutter, characterized by two distinct mechanisms: a clockwise isthmus-dependent flutter and an atypical scar-related flutter around the atriotomy scar. In order to effectively address this complex condition, a successful ablation procedure was performed to target both mechanisms. This case report offers valuable insights into the complexities surrounding the diagnosis and treatment of a complex case characterized by the coexistence of multiple mechanisms of atrial flutter within a single patient. While catheter ablation has demonstrated improved success rates for typical and atypical atrial flutters when occurring in isolation, predicting the prognosis of complex cases continues to pose challenges.
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Complex bifurcation lesions often requiring a two-stent revascularization approach mean more metal, a higher risk of major adverse cardiovascular events, and added difficulties in the case of late complications, such as in-stent restenosis and stent thrombosis. In this article, we report a case of late stent thrombosis in a 56-year-old patient who had left main (LM) and left anterior descending (LAD) left circumflex arteries with T and small protrusion technique percutaneous intervention (PCI) one year before her admission with hemodynamic compromise and no access to urgent coronary artery bypass grafting (CABG). We discuss challenging and high-risk PCI with limited resources, and the result was satisfactory with a favorable outcome. Stent thrombosis, a critical and life-threatening complication of PCI, frequently manifests with ST-elevation myocardial infarction, carrying a high mortality risk. Known risk factors for stent thrombosis include stent underexpansion, inadequate lesion preparation, complex percutaneous procedures, and early discontinuation of dual antiplatelet therapy. The choice of revascularization strategy is crucial, particularly for patients with extensive coronary artery disease, where surgery allows for a more complete revascularization. Coronary angioplasty is a pleasing and less invasive technique, but it requires proper lesion preparation, optimization of stent deployment through intracoronary imaging, post-dilation, and, most importantly, adherence and proper use of antithrombotic treatment following guidelines and medical therapy, which remains the cornerstone of managing ischemic heart disease.
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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.
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Cardiac tamponade is a life-threatening condition that requires emergency pericardiocentesis. In rare cases, pericardial drainage may be followed by "pericardial decompression syndrome" (PDS), a poorly understood but potentially fatal syndrome characterized by acute ventricular dysfunction. It may present in different clinical forms of varying severity and be managed differently depending on the clinical context. In this article, we report an atypical presentation of this syndrome, with the development of laminar tricuspid regurgitation after pericardial drainage. To our knowledge, this complication has never been reported in the medical literature. Our understanding of the pathophysiology of this condition is based entirely on case reports. And because clinical studies are difficult to perform, the best defense against PDS is early detection so that it can be recognized and treated quickly.
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Marfan syndrome (MFS) is a heritable connective tissue disorder that is caused by a mutation of the FBN1 gene. It is characterized by cardiovascular, skeletal, and ocular manifestations, with thoracic aortic aneurysms being the main cardiovascular complication. Unconventionally, MFS can present with left ventricular noncompaction (LVNC), which introduces a supplementary aspect of cardiac dysfunction. We herein report the case of a 42-year-old male with MFS who presented with congestive heart failure and cardiogenic shock. His transthoracic echocardiography revealed a giant aortic root aneurysm, causing severe aortic regurgitation and dilated cardiomyopathy, along with LVNC. This case provides a brief overview of this rare medical condition, particularly the natural history of ascending thoracic aortic aneurysm, which is considered a silent complication and the most life-threatening one, combined with LVNC that correspondingly impairs the heart.
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Electrical storm (ES) is a critical and potentially life-threatening cardiac rhythm disorder. It is characterized by the presence of three or more distinct episodes of sustained ventricular tachycardia (VT) or ventricular fibrillation (VF) that necessitate appropriate termination. ES may occur in the setting of acute myocardial infarction or following myocardial reperfusion. An urgent treatment approach is necessary for better outcomes. We represent a case of a 64-year-old patient who presented with sudden chest pain and an episode of palpitations related to non-ST elevation myocardial infarction (NSTEMI), who has undergone percutaneous coronary intervention of the stenotic epicardial artery, but subsequently experienced an ES in the absence of stent thrombosis. ES presented in the form of sustained monomorphic VT that required synchronous direct current cardioversion, anti-arrhythmic drugs, deep sedation, and endotracheal intubation with a favorable course, with the patient being discharged after 14 days hospital stay. The practitioner should be mindful of the potential occurrence of ES following myocardial revascularization and should tailor the management approach.
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The term "aortopulmonary window" (APW), often referred to as "aortopulmonary septal defect," refers to a rare congenital medical disorder where there is an improper direct link between the main pulmonary artery and the ascending aorta. It can be combined with other cardiac congenital conditions or be an isolated lesion. Herein, we report the incidental discovery of a minor, restrictive aortopulmonary septal defect in a 60-year-old male who denied having any clinical symptoms. Incidentally detected APW in adulthood is uncommon and, hence, can be readily overlooked, a fortiori, in asymptomatic patients.
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BACKGROUND: MR-MI is the first national Moroccan ST-elevation myocardial infarction (STEMI) registry. Its objectives are to assess patient management modalities and highlight the clinical and therapeutic characteristics of this pathology in all cardiology centres on a national scale. METHODS: Adult patients presenting with STEMI within 5 days of symptoms onset were enrolled over a period of 18 weeks from April to August 2018. 57 cardiology centres distributed in 22 cities in Morocco participated in the study, including 5 university hospitals, representing 70% of Moroccan centres managing STEMI patients. A case report form was sent to the investigators in both electronic and paper forms. Sociodemographic, clinical, management, revascularization, and follow-up data were collected. RESULTS: A total of 809 patients were recruited. The population was mostly male (74.8%) with an average age of 62.6 ± 11.6 years. The most common risk factors were smoking (38.3%) arterial hypertension (30.7%), and diabetes (28%). 30% of patients were admitted within the first 6 h of symptoms onset and early revascularization was performed on 49.6%. Mortality rate was 5.2% in-hospital and 3.2% at the one-month follow-up. CONCLUSION: MR-MI is the first Moroccan STEMI registry on a national scale. Relevant management delays are much longer than other countries, and less than 50% of the patients that present on time benefit from early revascularization. Efforts remain to be done on the optimal diagnosis and treatment of STEMI.
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Cardiologia , Infarto do Miocárdio , Infarto do Miocárdio com Supradesnível do Segmento ST , Adulto , Humanos , Masculino , Pessoa de Meia-Idade , Idoso , Feminino , Infarto do Miocárdio com Supradesnível do Segmento ST/diagnóstico , Infarto do Miocárdio com Supradesnível do Segmento ST/epidemiologia , Infarto do Miocárdio com Supradesnível do Segmento ST/terapia , Hospitais Universitários , Sistema de RegistrosRESUMO
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.
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Doença da Artéria Coronariana , Infarto do Miocárdio , Intervenção Coronária Percutânea , Derrame Pericárdico , Humanos , Masculino , Pessoa de Meia-Idade , Derrame Pericárdico/diagnóstico por imagem , Derrame Pericárdico/etiologia , Derrame Pericárdico/terapia , Angiografia Coronária , Doença da Artéria Coronariana/complicações , Intervenção Coronária Percutânea/efeitos adversos , Infarto do Miocárdio/complicações , Doença IatrogênicaRESUMO
Arrhythmogenic cardiomyopathy is a genetic heart muscle disease that typically affects the right ventricle. However, 2 other phenotypes affecting the left ventricle were recently discovered. Here, we report the case of an 18-year-old patient with biventricular arrhythmogenic cardiomyopathy, highlighting the challenges encountered in establishing this diagnosis. Diagnostic criteria for the left-sided phenotypic variants of arrhythmogenic cardiomyopathy were only introduced in 2020 by an international expert consensus document, known as the "Padua criteria," they are divided in 6 categories with an emphasis on morpho-functional ventricular abnormalities and structural myocardial tissue alterations to diagnose biventricular arrhythmogenic cardiomyopathy.
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Brugada syndrome is a rare inherited channelopathy associated with an increased risk of ventricular tachycardia and ventricular fibrillation, leading to syncope and sudden cardiac death. We present a case report of a young patient with an inducible type 1 Brugada pattern on an electrocardiogram (ECG), accompanied by a comprehensive literature review. The 19-year-old patient presented with dizziness and exhibited a type 2 Brugada pattern on admission ECG, which converted to a type 1 pattern following an Ajmaline test. Based on the absence of symptoms, inducible arrhythmias, or cardiac events in the patient's history, implantable cardioverter-defibrillator insertion was deemed unnecessary. Genetic testing was recommended, and screening ECGs were advised for the patient's first-degree relatives. The discussion explores the different types of Brugada patterns, their diagnostic significance, and the controversies surrounding risk stratification and management strategies. The case underscores the importance of maintaining clinical suspicion for Brugada syndrome in young patients and tailoring treatment approaches based on individual characteristics and risk factors.
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Submitral left ventricular aneurysm remains a rare condition with a varied etiology besides the congenital origin. We describe the case of a 62-year-old male patient who presented, two weeks after an inferobasal myocardial infarction (MI), with dyspnea and atypical chest pain. Transthoracic echocardiography (TTE) and cardiac computed tomography (CT) revealed a giant thin-walled submitral left ventricular aneurysm. He was managed conservatively given the high operative risk. The overall survival was five months after discharge. Despite its rarity, recognizing the causal relationship between ischemic heart disease and submitral aneurysm can prevent life-threatening complications and is therefore of major importance. In the era of advanced imaging, multimodality cardiac imaging techniques are a key element for guiding diagnostic and therapeutic strategies.
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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.
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Coronary artery ectasia (CAE) is an entity causing inappropriate dilatation of the coronary tree, that is angiographically defined, albeit arbitrarily, by the diameter of the ectatic segment being more than 1.5 times larger in comparison with an adjacent healthy reference segment. Although the causative mechanisms are poorly understood, atherosclerosis is greatly implicated in the causation of CAE. Clinical, angiographic, and therapeutic features have been puzzling clinicians. We illustrate three different angiographic subsets, co-existing with myocardial bridge/coronary slow flow and diversely presenting as asymptomatic, pauci, and frankly symptomatic with stable and acute coronary syndrome. These cases illuminate the diversity of CAE's clinical and angiographic presentations and pathologic progression, shedding light on this medical condition and its implications.
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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.
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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.
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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.
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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.