ABSTRACT
Cleidocranial dysplasia (CCD) is a rare genetic disorder characterized by skeletal abnormalities, including hypoplastic or absent clavicles, delayed closure of cranial sutures, and dental anomalies. We present a case of a 72-year-old female with a history of breast cancer treated with mastectomy and radio chemotherapy with the port-a catheter still in place in the left subclavian region. She presented to the emergency room with syncope related to a complete atrioventricular (AV) block. The patient underwent temporary pacing via femoral access while awaiting definitive pacemaker implantation. The absence of the right clavicle, first observed during prepuncture fluoroscopy and later confirmed on postprocedure imaging, significantly influenced the approach to pacemaker implantation. Venography played a crucial role in visualizing the venous trajectory and guiding the puncture, ensuring successful lead placement. The procedural challenges encountered due to the patient's skeletal anomalies highlight the importance of individualized approach and careful consideration of anatomical variations in interventional cardiology procedures.
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Coronary artery ectasia (CAE) is a rare condition, affecting 3%-8% of patients with atherosclerotic coronary artery disease, and is characterized by the abnormal dilatation of the coronary arteries. While the etiology of coronary artery ectasia encompasses a myriad of acquired and genetic factors, its pathogenesis still remains a subject of investigation. The clinical manifestations are varied, ranging from asymptomatic cases to chest angina and myocardial infarction. Coronary angiography remains the gold standard for diagnosing CAE. We herein report four cases of coronary ectasia: the first involving myocardial infarction, the second associated with bicuspid aortic valve with severe aortic regurgitation, the third detected during coronary angiography for moderate left ventricular dysfunction, and the last one detected during coronary angiography for stable angina. The aims of our study are to highlight the diversity of clinical presentations as well as the challenge of management, given that there are no universal treatments or guidelines.
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Although the incidence of systemic thromboembolism in valvular heart disease has been reported to be as high as 10% to 35%, embolization to the coronary arteries is uncommon. We present a case of a patient with acute myocardial infarction caused by coronary thromboemboli associated with combined valvular heart disease and atrial fibrillation. The thromboemboli were documented in the left descending artery. Coronary interventions including thromboaspiration and percutaneous coronary balloon angioplasty were attempted.
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Renal infarction is a rare pathology, threatening the functional prognosis of the kidney. Given the lack of clinical features specific to this pathology, the practitioner may wrongly diagnose pyelonephritis or other causes of abdominal pain. Renal infarction frequently occurs in patients with thromboembolic risk factors, most often secondary to atrial fibrillation. We report the case of a 49-year-old patient, with no previous history, who presented to the emergency room with palpitations and pain in the right flank with an atrial fibrillation rhythm on the ECG, in whom abdominal CT angiography showed signs of a focal renal infarction of the right kidney. Later, the progression was towards non-traumatic macroscopic hematuria with preservation of normal renal function, which resolved spontaneously after a few days. AF was accepted in the face of significant dilatation of the left atrium, in consultation with the patient, and he was put on a beta-blocker to control the rate. Renal infarction must be considered despite its rarity to initiate adequate treatment and increase the chances of renal rescue.
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BACKGROUND: Cardiac myxomas present a diagnostic challenge due to their ability to mimic various cardiovascular and systemic conditions. Timely identification is crucial for implementing surgical intervention and averting life-threatening complications. CASE PRESENTATION: We reported the case of a 49-year-old male patient who presented sudden legs weakness and slurred speech and was admitted 10 h later in emergency department. Physical examination was significant for paraparesis and paraphasia. Cardiac and carotid auscultation was normal. CT brain revealed multiple acute ischemic strokes and MRA was suggestive of cerebral vasculitis. As pre-therapy assessment, the EKG revealed no electrical abnormalities and the chest X-ray showed signs of left atrial enlargement. Transthoracic and transesophageal echocardiography showed a left atrial mass attached to the interatrial septum, measuring 9*5*4 cm and extending into the left ventricular cavity during diastole, which suggested the diagnosis of left atrial myxoma. The patient was referred for open-heart surgery and histopathological examination confirmed the diagnosis of myxoma. The patient weaned off from cardiopulmonary bypass and the postoperative period was uneventful. CONCLUSION: We reported an interesting case with an unusual and misleading neurological presentation of a cardiac myxoma. The unpredictability of serious complications occurrence must awaken our medical flair, for an early diagnosis among a long list of differentials.
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BACKGROUND: Gaucher disease (GD) is a lysosomal storage disease that leads to the accumulation of glucocerebroside within reticuloendothelial cells, haematological, neurological, skeletal and abdominal organs. These clinical manifestations are common to all types of GD, but categorization depends on the absence of neurological involvement (type I) or its presence (type II and III). Cardiac involvement is rare and only reported in few cases, where valvular and aortic calcifications were associated with type IIIc. Other cardiac manifestations, such as constrictive pericarditis, pulmonary hypertension, myocardial infiltration, and restrictive cardiomyopathy, had also been reported. CASE PRESENTATION: We report a case of a 72-year-old patient with known type 1 GD who presented with a sudden syncope during exercise. He reported also an exercise intolerance evolving for three months. Echocardiography found concentric left ventricular hypertrophy with segmental hypokinesis, bi-atrial enlargement, and mildly reduced ejection fraction. Mitral flow was in favour of grade II diastolic dysfunction with elevated filling pressure. Cardiac magnetic resonance (CMR) showed interstitial fibrosis in the basal infero-septal wall, probably due to the myocardial infiltration of GD. Due to the lack of echocardiographic and CMR hallmarks of cardiac GD, we conducted a literature review on similar findings. CONCLUSION: This case illustrates the importance of non-invasive cardiac imaging in the diagnosis, prognosis and management of cardiac manifestations of GD.
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Pulmonary hypertension is a condition characterised by elevated pulmonary arterial pressures secondary to various aetiologies; the most common ones are left heart diseases. Similarly, an association between thyroid diseases and pulmonary hypertension has been reported in some cases, but the pathophysiological relationship has not been fully elucidated. Etiological investigation is an important step in the management of pulmonary hypertension and determines the appropriate treatment. In this report, we present a case of severe pulmonary hypertension in a 57-year-old woman, in which mixed aortic valve disease and hypothyroidism were involved.
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Primary cardiac tumors are a rarity, and sarcomas emerge as the prevailing form of primary malignant cardiac tumors across age groups, encompassing both children and adults. Within this category, angiosarcoma stands out, constituting around 31% of all primary malignant cardiac tumors. Primary cardiac angiosarcoma displays a notably aggressive nature, characterized by early systemic metastasis, and is accompanied by a generally unfavorable prognosis. We describe a case concerning a previously healthy teenage girl who displayed persistent constitutional symptoms and hemoptysis for 15 days. Subsequent investigation uncovered alveolar hemorrhage, ultimately linked to a cardiac angiosarcoma. The difficulty in this instance arose from the vague nature of the initial symptoms, posing a challenge to promptly and accurately diagnose the condition. This case highlights the aggressive nature of primary cardiac angiosarcoma. The vague initial symptoms underscore the need for early detection and optimized treatment to improve the generally unfavorable prognosis associated with this condition. Increased awareness and a multidisciplinary approach are crucial in addressing the diagnostic and therapeutic challenges posed by primary cardiac angiosarcoma.
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Mitral valve aneurysm (MVA) is characterized by a saccular outpouching of the mitral leaflet, and it represents a rare condition typically associated with aortic valve endocarditis. Three-Dimensional Transesophageal Echocardiography (3D-TEE) serves as an effective tool for detecting the presence of MVA and its potential complications. In this report, we present a case involving a young man with striking images of bicuspid aortic valve endocarditis complicated by an aortic root abscess and multiple perforated mitral valve aneurysms, diagnosed using 3D TEE. This case suggests the uncommon coexistence of Marfan like morphotype, bicuspid aortic valve, and infective endocarditis as a triple mechanism in the occurrence of MVA. It underscores the significance of early and accurate imaging diagnosis for facilitating prompt surgical intervention.
Subject(s)
Bicuspid Aortic Valve Disease , Echocardiography, Three-Dimensional , Endocarditis, Bacterial , Endocarditis , Heart Aneurysm , Marfan Syndrome , Humans , Male , Abscess/diagnostic imaging , Abscess/etiology , Aorta, Thoracic , Aortic Valve/diagnostic imaging , Aortic Valve/surgery , Bicuspid Aortic Valve Disease/complications , Echocardiography, Transesophageal/methods , Endocarditis/complications , Endocarditis/diagnostic imaging , Endocarditis, Bacterial/complications , Endocarditis, Bacterial/diagnostic imaging , Endocarditis, Bacterial/surgery , Heart Aneurysm/etiology , Heart Aneurysm/complications , Marfan Syndrome/complications , Marfan Syndrome/diagnosis , Mitral Valve/diagnostic imaging , Mitral Valve/surgeryABSTRACT
BACKGROUND: Tumor embolism is the least well-described cause of pulmonary embolism, taking into account the non-specificity of radiographic and nuclear imaging results, the necessity of anatomopathological evidence and the frequency of deep venous thrombosis in the course of solid tumors, suggesting thus thromboembolism. CASE PRESENTATION: We report a rare case of urothelial carcinoma of the urinary bladder associated with persistent pulmonary embolism despite being on different anticoagulation regimens, the patient was ultimately found to have tumor thrombus in the pulmonary trunk secondary to tumoral extension. We provide a literature review as well about the diagnosis, evaluation and prognosis and of this rare clinical entity. CONCLUSIONS: Our case highlights the importance of keeping this unusual etiology in mind, particularly when faced with pulmonary embolism occurring in the context of a solid tumor and the ineffectiveness of various anticoagulation protocols. Furthermore, it emphasizes the pivotal role of cytology in confirming diagnosis and guiding therapy.
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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.
Subject(s)
Cardiology , Myocardial Infarction , ST Elevation Myocardial Infarction , Adult , Humans , Male , Middle Aged , Aged , Female , ST Elevation Myocardial Infarction/diagnosis , ST Elevation Myocardial Infarction/epidemiology , ST Elevation Myocardial Infarction/therapy , Hospitals, University , RegistriesABSTRACT
Complex congenital heart disease and pregnancy are a challenge and delicate medical situation. We describe a first-time pregnancy of a woman living with an uncorrected d-transposition of the great arteries (TGA) with serious fetal complications required multidisciplinary assessment. Twenty-six years old primigravida referred to our cardiology center in 34 weeks of gestation with peripheral cyanosis. The patient was stable hemodynamically and the room air oxygen was at 82%. A continuous murmur systolic and diastolic at second left intercostals space was found. The transthoracic echocardiographic showed an uncorrected TGA with a large atrial defect and patent ductus arteriosus. Obstetrical ultrasonography showed severe intrauterine growth restriction. The patient was delivered by caesarean section under epidural anesthesia with good outcome. This is a case report with discussion of the maternal-fetal outcome of pregnant women with asymptomatic uncorrected TGA. Coordinated care by an informed obstetrician and cardiologist should be the aim.
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Spontaneous coronary artery dissection (SCAD) is often revealed by an acute coronary syndrome classified then as a MINOCA. The typical patient is a female patient with no or few cardiovascular risk factor. Our work aims to illustrate the effectiveness of medical treatment in patients with SCAD. Case Report: We report a case of a 56-year-old female patient who was admitted after 3 days of infarct-like thoracic pain related to an anterior extended ST-elevation myocardial infarction. The coronarography showed a SCAD of the left anterior descending coronary artery. The patient was discharged under medical treatment. Six weeks later, coronarography showed a perfused coronary artery. Discussion: Most cases of SCAD present as acute coronary syndrome. Coronary angiography remains the 'first-line' examination. The use of endocoronary imaging such as IVUS and optical coherence tomography is necessary in case of diagnostic doubt (especially in SCAD type 2 and 3). The optimal management of SCAD remains unclear. A conservative approach should be the preferred strategy. Conclusion: SCAD should be considered in any young woman presenting with suspicious chest pain with positive troponin. The diagnosis is initially angiographic and may require endocoronary imaging for greater accuracy. Conservative treatment remains the best option.
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Introduction and importance: Primary malignant pericardial tumors are an entity that is infrequently encountered and may be a cause of pericardial effusion. Primary pericardial leiomyosarcoma are even rarer, and highly aggressive tumors, with no more than 200 cases reported in the literature. In this case report, we are presenting a rare case of a primary pericardial leiomyosarcoma that was diagnosed at our institution. We discuss the available diagnostic modalities and also shed light on alternative therapies when patients are not ideal surgical candidates. Case presentation: A 27-year-old male patient was admitted with a gradually worsening dyspnea associated with a deterioration of general condition. Echocardiography examination showed a circumferentiel pericardial effusion with significant fibrin deposits and pericardial thickening. An open surgical biopsy of the pericardium was indicated which was in favor of the diagnosis of pericardial leiomyosarcoma. Unfortunately, the patient died during the procedure. Clinical discussion: Despite its rarity, primary pericardial leiomyosarcoma should be considered as a differential diagnosis in the assessment of a pericardial effusion of an unknown etiology. Cardiac magnetic resonance imaging is considered to be the reference standard technique for evaluation of a suspected pericardial tumor. Surgical biopsies provide the best odds for detection of the cell of origin, but it's fought with periprocedural risks depending on the site of the tumor. Conclusion: Primary pericardial leiomyosarcomas appear to have a poor prognosis. Surgical approach is the primary modality of treatment. Chemotherapy and radiotherapy should be offered to patients who are not ideal surgical candidates.
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Only few cases of vascular dissection and essential thrombocythemia association have been reported. To the best of our knowledge, we reported the second case of aortic dissection and essential thrombocythemia association in a 60-year-old man with positive JAK2V617F mutation who had no history of hypertension or connective tissue disorders. Through this case, we discussed the eventual existence of a causal relationship between the two conditions. We also suggested the use of hydroxyurea as a prevention treatment of thrombosis in myeloproliferative neoplasms.
Subject(s)
Aortic Dissection , Polycythemia Vera , Thrombocythemia, Essential , Aortic Dissection/complications , Aortic Dissection/diagnostic imaging , Humans , Hydroxyurea/therapeutic use , Janus Kinase 2/genetics , Male , Middle Aged , Polycythemia Vera/complications , Polycythemia Vera/genetics , Thrombocythemia, Essential/complications , Thrombocythemia, Essential/diagnosis , Thrombocythemia, Essential/drug therapyABSTRACT
Cerebrovascular events are rare but devastating events that can complicate any coronary intervention. In the vast majority of cases, they involve major cerebral arteries. We report the case of a 56-year-old woman admitted for unstable angina associated with severe left systolic dysfunction. She developed moderate cerebellar stroke while undergoing percutaneous coronary intervention, with a national institutes of health stroke scale score of 5. Immediate systemic thrombolysis was performed, but her neurological status deteriorated. A large hemorrhagic transformation was then diagnosed, and she died despite surgical intervention. Periprocedural strokes are marred with high morbidity and mortality, therefore preventionis key, as many risk factors can be controlled or mitigated. Our patient presented many of these factors; they can be procedure-related (transfemoral approach, anticoagulation) or patient-related (age, diabetes mellitus, uncontrolled hypertension, diffuse atherosclerosis).
Subject(s)
Hypertension , Percutaneous Coronary Intervention , Stroke , Cardiac Catheterization/adverse effects , Female , Humans , Hypertension/etiology , Middle Aged , Risk Factors , Stroke/etiology , Treatment OutcomeABSTRACT
Paradoxical embolism in coronary artery is a rarely diagnosed clinical entity. In the majority of reported cases; the diagnostic of this pathology is « presumptive ¼ based on certain criteria. It can be considered "proven" when the embolus is found lodged in the abnormal communication between the venous and arterial circulation; which is very rare. We herein report a case of myocardial infarction caused by a proven paradoxical coronary embolism through a patent foramen ovale. The authors highlight through this paper the contribution of echocardiography and particularly trans-esophageal echocardiography, especially if performed soon after presentation, for early diagnosis.
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Right heart thrombi can form in situ or lodge in the right cardiac chambers, originating from deep venous thrombosis. The latter carries a poor prognosis, taking into account the very high mortality rate. We herein report a case of an 83-year-old man who developed thrombus in the inferior vena cava that extended up to the right atrium, along with two distinct masses attached to the right ventricle wall.
Subject(s)
Heart Atria/diagnostic imaging , Thrombosis/diagnostic imaging , Vena Cava, Inferior/diagnostic imaging , Aged, 80 and over , Echocardiography, Transesophageal , Heart Atria/pathology , Humans , Male , Thrombosis/pathology , Vena Cava, Inferior/pathologyABSTRACT
Left atrial appendage (LAA) aneurysm is an extremely rare anomaly. So far, less than one hundred cases only have been reported worldwide. Revelation modes are dominated by complications such as arrhythmias and thromboembolic events. We herein report a pediatric case of huge congenital LAA aneurysm with an original revelation mode that has never been described before in medical literature.