Severe pulmonary hypertension associated with hypothyroidism and mixed aortic valve disease: A case report.

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.

Rare case of cardiac angiosarcoma and alveolar hemorrhage in an adolescent patient initially suspected COVID19 infection: A case report and literature review.

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.

Primary autonomic failure: a complex case of orthostatic hypotension in a hypertensive elderly patient.

Background: Primary autonomic failure (PAF) or Bradbury Eggleston syndrome is a neurodegenerative disorder of the autonomic nervous system characterized by orthostatic hypotension. Case summary: We report the case of a 76-year-old patient with a history of hypertension, who presented with exercise-induced fatigue. He exhibited systolic hypertension and resting bradycardia in the supine position, with orthostatic hypotension without reactive tachycardia, suggesting dysautonomia. Neurological examination was unremarkable. The patient underwent cardiovascular autonomic testing, revealing evidence of beta-sympathetic deficiency associated with neurogenic orthostatic hypotension. Causes of secondary dysautonomia were excluded. The patient was diagnosed with PAF. Even if managing the combination of supine hypertension and orthostatic hypotension was challenging, significant improvements in functional and haemodynamic status were observed with a personalized management approach. Discussion: Throughout this case report, we emphasize the critical need for an evaluation of autonomic function and blood pressure's dynamics in hypertensive patients experiencing orthostatic symptoms, enabling the implementation of tailored therapeutic strategies.

Rare complications of infective endocarditis in marfan-like morphotype: diagnosis of multiple mitral valve aneurysms and aortic root abscess using three-dimensional transesophageal echocardiography.

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.

Pulmonary tumor embolism secondary to urothelial carcinoma of urinary bladder: case report and literature review.

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.

Congenitally uncorrected transposition of the great arteries in a pregnant woman 26-year-old: Description and literature review.

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.

Post-COVID myocarditis simulating acute coronary syndrome: Case report.

Acute myocarditis corresponds to an acute inflammation of the myocardium whose origin is most often viral. Several viruses can be incriminated to note the parvovirus B19, the virus herpes of the group 6 and to a lesser degree the virus of the hepatitis C (VHC). Since 2019 and with the discovery of SARS COV2 some cases of myocarditis associated with COVID have been noted, this last association is rare and is present in only 5% of cases. The diagnosis of myocarditis is sometimes difficult and can lead to confusion with acute coronary syndrome, especially in cases of ST-segment elevation on the EKG, hence the interest of magnetic resonance imaging, which has made it possible in recent years to reduce the rate of unnecessary coronary angiography, especially in the case of young subjects with no cardiovascular risk factors. Here, we report the case of a 33-year-old patient with no cardiovascular risk factors and no medical or surgical antecedents who was admitted to the emergency department for the management of acute chest pain, the patient had initially undergone an electrocardiogram which showed an ST-segment elevation in the inferior territory and in the low lateral territory with a mirror image in the high lateral territory. In view of the typical character of the pain and based on the electrical data, it was decided to carry out a coronary angiography which came back without any particularity. As part of the etiological work-up, an MRI scan was performed, which showed an appearance compatible with viral myocarditis. This case shows diagnostic difficulties and management of this disease.

Homozygous factor V leiden mutation: Rare etiology of pulmonary embolism.

Introduction and importance: Venous thromboembolic disease (VTE), which includes pulmonary embolism (PE) and deep vein thrombosis (DVT), is a major public health problem with high morbidity and mortality. The main risk factors for VTE are surgery, active cancer, immobilization, trauma or fracture, pregnancy and estrogen therapy. Genetic risk factors are also present and are dominated by the factor V Leiden mutation, which is present in 20% of VTE and in 2-5% of the general population with an annual incidence of 0.1% (Margaglione and Grandone, 2011; Ridker et al., 1995) [4,5]. This mutation can be heterozygous or homozygous, which is rarer. In this context, we report the case of a 37-year-old patient with no medical or surgical history and no notable risk factors who was admitted to the emergency room for the management of acute dyspnea at rest in connection with a bilateral proximal pulmonary embolism originating from a homozygous factor V Leiden mutation.Despite the efforts of the World Health organization, pulmonary embolism remains a major cause of morbidity and mortality in our days, and the etiological assessment is performed in a very few cases, which makes the management standardized and not specific. That is why it is important to make an etiological assessment in a systematic way especially in young subjects for an optimal management and to avoid recurrences. Case presentation: Here, we report a rare case of a 37-year-old patient, who was admitted for the management of resting dyspnea related to bilateral proximal pulmonary embolism, in whom the etiological work-up was in favor of a homozygous factor V Leiden mutation. This case shows diagnostic difficulties and management of this rare disease.

A case report of a primary pericardial leiomyosarcoma: An extremely rare cardiac neoplasm.

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.

Essential thrombocythemia and aortic dissection,causal or incidental association?

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.

Extracardiac conduit as alternative repair for scimitar syndrome.

Scimitar syndrome has been repaired by different surgical procedures including intracardiac baffle technique, reimplantation of scimitar vein (SV) to the right atrium or the left atrium (LA). However, several anatomical variations such as short venous collector coursing deeply within the lung hilum with infradiaphragmatic drainage make the repair more challenging with conventional repair techniques. We present an alternative method for repair using a tube graft to connect the SV to the LA.

Cerebellar stroke complicating coronary catheterization: a case report.

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).

Concomitant in situ and in transit right heart thrombi: a case report.

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.

Asymptomatic interventricular septal dissection and giant coronary artery aneurysms simulating cardiac cysts: Multi-modality imaging.

we report an exceptionnel case of asymptomatic interventricular septal dissection and giant coronary artery aneurysms simulating cardiac cysts with a focus in the interst of Multi-modality Imaging.

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.

Predictive factors of left atrial spontaneous echo contrast in patients with rheumatic mitral valve stenosis: a retrospective study of 159 patients.

BACKGROUND: Mitral valve stenosis is a common manifestation of chronic rheumatic heart disease. The presence of spontaneous echo contrast in the left atrium and left atrial appendage has been reported to be an independent predictor of thrombo-embolic risk in patients with mitral stenosis. The objective of this study was to retrospectively investigate various clinical and echocardiographic variables to predict the spontaneous echo contrast in these patients. METHODOLOGY: This is a bicentric retrospective study which includes 159 cases of symptomatic mitral stenosis from January 2011 to June 2012. All of the patients had transthoracic and transesophageal echocardiography. Patients who had significant mitral regurgitation (> Grade I), significant aortic valve disease, previous mitral valvulotomy and anticoagulation or antiplatelet therapy were excluded from the study. Our study population was divided into two groups based on the presence (Group I) or absence (Group II) of spontaneous echo contrast. RESULT: Left atrial spontaneous contrast was present in 34.6% of cases. Patients in this group have more frequent atrial fibrillation (P = 0.001), larger left atrial area (P = 0.027) and diameter (P=0.023), smaller mitral valve area (P = 0.025), and higher mean transmitral diastolic gradient (p = 0.003) as compared to patients without spontaneous echo contrast. There were no significant differences in the mean age (p = 0.38), duration of symptoms (p = 0.4) and left ventricular ejection fraction (p = 0.7) between patients with and without spontaneous echo contrast. On multivariate analysis, only mitral valve area and transmitral diastolic gradient (OR: 18.753, 1.21, CI [1,838-191,332], [1,064-1,376], p: 0.013, 0.004, respectively) were found to be independently associated to the presence of spontaneous echo contrast. CONCLUSION: Patients with severe rheumatic mitral stenosis in atrial fibrillation or sinus rhythm have a higher risk of developing spontaneous echo contrast. These patients might benefit from prophylactic anticoagulation. The long-term outcomes can be ascertained in a study over a longer period and with periodic follow-up.

Unusual findings in secondary hypertension: double orifice mitral associated to aortic coarctation, bicuspid aortic valve, and ventricular septal defect.

Double orifice mitral valve is a rare congenital anomaly presenting as the division of the mitral orifice into two anatomically distinct orifices, it is most often associated with other congenital heart defects such as left-sided obstructive lesions, ventricular septal defects or aortic coarctation. We report the case of a 15 year's old boy, admitted for arterial hypertension, auscultation revealed a rude aortic systolic murmur. Femoral pulses were weak. Owing to the suspicion of aortic coarctation, transthoracic echocardiography was performed, the aortic coarctation with dilation of the aorta proximal to the stenosis was confirmed and bicuspid aortic valve was found with good function. The mitral valve was dysmorphic, having two orifices; it was divided into 2 separate valve orifices by a fibrous bridge. No mitral or aortic regurgitation was documented by color Doppler flow imaging. The left ventricular ejection fraction was normal. There was a small peri membranous ventricular septal defect with left to right shunt. Owing to the severity of the aortic coarctation and taking into account the anatomy and characteristics of the patient, he was made a surgical correction of aortic coarctation with good outcome.

A giant left ventricular pseudoaneurysm in Behçet's disease: a case report.

Behçet's disease is a chronic autoimmune disease with vascular complications that are most frequently manifested as thromboembolism in veins and pseudoaneurysm in arteries. We report the case of a 13-year-old boy admitted for clinical and biological signs of rheumatic fever associated with chest pain. The clinical examination found heart sounds with a discrete systolic murmur of mitral regurgitation. The electrocardiogram showed a microvoltage with diffuse repolarisation disorder. Biologically, he had inflammatory syndrome. Transthoracic echocardiography showed circumferential pericardial effusion with anterosepto-apical hypokinesia of the left ventricle with systolic dysfunction, and a minimal mitral regurgitation. The patient was treated by corticotherapy and antibiotherapy. The outcome was marked by orogenital aphthous ulceration and decreased visual acuity related to intermediate uveitis. The retinal angiography showed a vasculitis. The late appearance of this symptom led to the right diagnosis of Behçet's syndrome. Transthoracic echocardiography showed a hypokinetic dilated cardiomyopathy left ventricular with septo apical and anterior akinesia and severe systolic dysfunction, with a defect of the inferior septal with a collar communicating the left ventricle with a giant pseudo aneurysm. Magnetic resonance imaging showed a giant pseudoaneurysm communicating with the left ventricle. The coronary computed tomography was normal. The patient had undergone surgical treatment for the pseudoaneurysm with good outcomes.

Giant left atrial myxoma mimicking severe mitral valve stenosis and severe pulmonary hypertension.

Myxoma is the most common primary tumor of the heart and can arise in any of the cardiac chambers. This paper reports A 50 -year-old woman without medical history and any cardiovascular risk factors was hospitalized for exertional dyspnea and palpitations from three months and signifiant weight loss. Transthoracic echocardiogram showed a giant left atrial myxoma mobile confined to the left atrium in systole, in diastole the tumor was seen prolapsing across the mitral valve into the left ventricle and partially obstructing it and causing severe functional mitral stenosis with a mean gradient of 21,3 mmHg. Severe pulmonary hypertension was confirmed by Doppler PAPs =137 mmHg. The patient was scheduled for cardiac surgery with good outcome.