Fibromuscular Dysplasia Implicated in Spontaneous Coronary Artery Dissection (SCAD): A Case Report of Chest Pain in Young Women.

Spontaneous coronary artery dissection (SCAD) is a well-recognized cause of acute coronary syndrome (ACS) which can lead to myocardial infarction and sudden death. Unlike typical atherosclerosis, SCAD operates through distinct pathophysiology, affecting both individuals with and without conventional cardiovascular risk factors. We present a case of a young female presented with retrosternal chest pain radiating to the left arm, mimicking ACS symptoms with mildly elevated troponin levels, and slightly reduced left ventricular ejection fraction (45%). Subsequent evaluation using coronary angiography unveiled a Type 2A SCAD. A comprehensive computed tomography angiography (CTA) of her entire body revealed findings suggestive of fibromuscular dysplasia (FMD) affecting multiple arteries in different sites. Our case entailed the successful management of a young female patient with SCAD stemming from FMD.

Benign metastasizing fumarate hydratase (FH)-deficient uterine leiomyomas: clinicopathological and molecular study with first documentation of multi-organ metastases.

Leiomyoma is the most prevalent benign tumor of the female reproductive system. Benign metastasizing leiomyoma (BML) is a rare phenomenon that presents at distant sites, typically the lungs, exhibiting histopathological features similar to the primary uterine tumor in the absence of malignancy features in both. Fumarate hydratase-deficient uterine leiomyoma (FH-d UL) is an uncommon subtype among uterine smooth muscle tumors (0.5-2%), showing distinctive histomorphology and FH inactivation. The majority of FH-d ULs are sporadic, caused by somatic FH inactivation, while a minority of cases occur in the context of the hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome caused by germline FH inactivation. Metastasizing FH-d UL has not been well documented and might be under-reported. Here, we present two cases (21- and 34-year-old females) who presented with metastasizing FH-d UL after myomectomy/hysterectomy with histologically proven multiple lung metastases in both, in addition to multi-organ involvement in one case (cervical-thoracic lymph nodes, left kidney, perihepatic region, left zygomatic bone, and soft tissues). Pathological examination confirmed FH-d leiomyomas in the primary/recurrent uterine tumors, multiple lung lesions, and a renal mass. The minimal criteria for diagnosis of leiomyosarcoma were not fulfilled. Genetic testing revealed germline pathogenic FH variants in both cases (c.1256C > T; p.Ser419Leu in Case 1 and c.425A > G; p.Gln142Arg in Case 2). These novel cases highlight a rare but possibly under-recognized presentation of FH-d BML. Our study suggests that FH-d BML cases might be enriched for the HLRCC syndrome.

Establishment of diagnostic reference levels in computed tomography in two large hospitals in Oman.

This study aimed to estimate diagnostic reference levels (DRLs) for the most frequent computed tomography (CT) imaging examinations to monitor and better control radiation doses delivered to patients. Seven CT imaging examinations: Head, Chest, Chest High Resolution (CHR), Abdomen Pelvis (AP), Chest Abdomen Pelvis (CAP), Kidneys Ureters Bladder (KUB) and Cardiac, were considered. CT dosimetric quantities and patient demographics were collected from data storage systems. Local typical values for DRLs were calculated for CTDIvol (mGy), dose length product (DLP) (mGy·cm) and effective doses (mSv) were estimated for each examination. The calculated DRLs were given as (median CTDIvol (mGy):median DLP (mGy·cm)): Head: 39:657; Chest: 13:451; CHR: 6:228; AP: 12:578; CAP: 20:807; KUB: 7:315, and Cardiac: 2:31. Estimated effective doses for Head, Chest, CHR, AP, CAP, KUB and Cardiac were 1.3, 12.7, 6.3, 12.5, 18.1, 5.8 and 0.8 mSv, respectively. The estimated DRLs will act as guidance doses to prevent systematic excess of patient doses.

Identifying Anomalies of Systemic Venous Drainage: Systemic Venous Anomalies; Atrial Morphology.

So as to produce totally anomalous systemic venous connection, all of the systemic venous tributaries, along with the coronary sinus, should be connected with the morphologically left atrium. Previous descriptions of this rare constellation of anomalous connections of the systemic venous tributaries of the heart have been compromised by the inclusion of individuals having isomeric atrial appendages. In these settings, most frequently, the totally, or almost totally, anomalous systemic venous connections are associated with a sinus venosus defect. It is the anomalous pulmonary venous connections that then create a venovenous bridge, which permits the systemic venous tributaries to drain into the morphologically left atrium, even though they may be predominantly connected to the right atrium. More rarely, it is feasible for the primary atrial septum to develop so as to leave the systemic venous sinus in direct connection with the body of the morphologically left, rather than the morphologically right, atrium. We report a series of patients potentially falling into the category of anomalous systemic venous connections. The findings show a spectrum from partially to totally anomalous connections, with some better interpreted on the basis of anomalous drainage. Included in our cases, nonetheless, is an autopsied example of totally anomalous systemic venous connection produced by an abnormal location of the primary atrial septum. We discuss the potential morphogenesis for this finding. We emphasize the distinction that needs to be made between anomalous systemic venous connections and anomalous systemic venous drainage.

The Sinus Venosus Veno-Venous Bridge: Not a septal defect.

This review provides an update on the morphology of the sinus venosus defect. It was earlier believed that a 'common wall' separated the right pulmonary veins from the superior caval vein. In the sinus venosus defects, this wall was absent. Current evidence shows that the superior rim of the oval fossa, rather than forming a second septum or representing a common wall, is an infolding between the walls of the caval veins and the right pulmonary veins. The sinus venosus defect is caused by the anomalous connection of one or more pulmonary veins to a systemic vein. However, the pulmonary vein(s) retain their left atrial connections, leading to a veno-venous bridge that allows interatrial shunting outside the oval fossa. True atrial septal defects are located within the oval fossa or in the anteo-inferior buttress, while sinus venosus defects, ostium defects and coronary sinus defects are morphologically distinct from them.

Bronchial Leiomyoma: Importance of Preoperative Biopsy for Lung Preserving Surgery.

Tracheobronchial leiomyoma is a rare tumor of the airway. They arise from the lower respiratory tract tissue of the bronchi, trachea, and lung. Symptomatology is based on the degree of endoluminal bronchial obstruction, and surgical resection is generally the mainstay of treatment. We present the case of a 33-year-old male who suffered from chronic cough and breathlessness for two years caused by large endobronchial leiomyoma diagnosed by preoperative biopsy. The tumor was surgically resected through bronchotomy and complete preservation of the lung parenchyma. We stress the importance of a definitive preoperative diagnosis of this rare tumor to employ lung preserving surgical techniques.

Bronchial Mucoepidermoid Carcinoma Misdiagnosed as Adenocarcinoma: A Word of Caution.

Tracheobronchial mucoepidermoid tumors (METs) typically occur in the head and neck region but rarely in the trachea and lung. They are salivary-type tumors that arise from the glandular component of the tracheobronchial epithelium. The most common type, mucoepidermoid carcinoma (MEC) has histological features that overlap with more aggressive lung carcinomas such as adenosquamous carcinoma. It is important to realize the histological features and limitations of a diagnostic biopsy. This case illustrates this point where an initially diagnosed lung adenocarcinoma turns out to be MEC. We report a case of a 43-year-old woman with a one-year history of recurrent episodes of cough and fever. Initial bronchial biopsy diagnosed her as having adenocarcinoma of the lung. However, her surgical biopsy confirmed it was MEC. High clinical suspicion that the diagnosis may not have been correct saved her from a potential pneumonectomy. She instead underwent bi-lobectomy sleeve resection. This case illustrates the importance of recognizing less common and less aggressive lung tumors that may appear histologically as adenosquamous carcinoma. High clinical suspicion, not only biopsy results, from clinical history, imaging and gross appearance is always needed in all cases. The use of intraoperative frozen section is mandatory. It is important to be aware that because of morphological limitations of small endobronchial biopsies, diagnosis of a more common pathology may be favored.

Two-step machine learning to diagnose and predict involvement of lungs in COVID-19 and pneumonia using CT radiomics.

OBJECTIVE: To develop a two-step machine learning (ML) based model to diagnose and predict involvement of lungs in COVID-19 and non COVID-19 pneumonia patients using CT chest radiomic features. METHODS: Three hundred CT scans (3-classes: 100 COVID-19, 100 pneumonia, and 100 healthy subjects) were enrolled in this study. Diagnostic task included 3-class classification. Severity prediction score for COVID-19 and pneumonia was considered as mild (0-25%), moderate (26-50%), and severe (>50%). Whole lungs were segmented utilizing deep learning-based segmentation. Altogether, 107 features including shape, first-order histogram, second and high order texture features were extracted. Pearson correlation coefficient (PCC≥90%) followed by different features selection algorithms were employed. ML-based supervised algorithms (Naïve Bays, Support Vector Machine, Bagging, Random Forest, K-nearest neighbors, Decision Tree and Ensemble Meta voting) were utilized. The optimal model was selected based on precision, recall and area-under-curve (AUC) by randomizing the training/validation, followed by testing using the test set. RESULTS: Nine pertinent features (2 shape, 1 first-order, and 6 second-order) were obtained after features selection for both phases. In diagnostic task, the performance of 3-class classification using Random Forest was 0.909±0.026, 0.907±0.056, 0.902±0.044, 0.939±0.031, and 0.982±0.010 for precision, recall, F1-score, accuracy, and AUC, respectively. The severity prediction task using Random Forest achieved 0.868±0.123 precision, 0.865±0.121 recall, 0.853±0.139 F1-score, 0.934±0.024 accuracy, and 0.969±0.022 AUC. CONCLUSION: The two-phase ML-based model accurately classified COVID-19 and pneumonia patients using CT radiomics, and adequately predicted severity of lungs involvement. This 2-steps model showed great potential in assessing COVID-19 CT images towards improved management of patients.

Evaluation of pulmonary blood flow in bilateral bidirectional Glenn shunts: value of 4-D flow cardiac magnetic resonance in the evaluation of pulmonary artery confluence stenosis.

Bilateral bidirectional Glenn shunts are associated with the risk of developing pulmonary artery bifurcation stenosis, resulting in variable pulmonary blood flow to either lung. This could negatively impact the subsequent stages of the single ventricle palliation pathway. This report highlights the value of 4D flow sequence from the cardiac magnetic resonance imaging in demonstrating the pulmonary blood flow characteristics following a bilateral bidirectional Glenn procedure. Mapping the blood flow pattern and its quantification to each lung provide objective insights into the possible predisposing factors for the development of pulmonary bifurcation stenosis.

COVID-2019 Pneumonia: Severity and distribution of lung changes observed on the initial chest X-ray as an indicator of final outcomes.

Objectives: This study aimed to assess the correlation between the severity of the initial chest x-ray (CXR) abnormalities in patients with a confirmed diagnosis of COVID-19 and the final outcomes. Methods: This retrospective study was conducted at the Royal Hospital, Oman between mid-March and May 2020 and included patients who had been admitted with a confirmed diagnosis of COVID-19 and had a final outcome. Serial CXRs were identified and examined for presence, extent, distribution and progression pattern of radiological abnormalities. Each lung field was divided into three zones on each CXR and a score was allocated for each zone (0 is normal and 1-4 is mild-severe). The scores for all six zones per CXR examination were summed to provide a cumulative chest radiographic score (range: 0-24). Results: A total of 64 patients were included; the majority were male (89.1%) and the mean age was 50.22 ± 14.86 years. The initial CXR was abnormal in 60 patients (93.8%). The most common finding was ground glass opacity (n = 58, 96.7%) followed by consolidation (n = 50, 83.3%). Most patients had bilateral (n = 51, 85.0%), multifocal (n = 57, 95.0%) and mixed central and peripheral (n = 36, 60.0%) lung abnormalities. The median score of initial CXR for deceased patients was significantly higher than recovered patients (17 versus 11; P = 0.009). Five CXR evolution patterns were identified: type I (initial radiograph deteriorates then improves), type II (fluctuate), type III (static), type IV (progressive deterioration) and type V (progressive improvement). Conclusion: A higher baseline CXR score is associated with higher mortality rate and poor prognosis in those with COVID-19 pneumonia.

Chest Radiography Findings of COVID-19 Patients: Differences between the Intubated and Non-intubated Groups.

OBJECTIVES: We sought to identify the chest radiography differences at presentation between two groups of hospitalized confirmed COVID-19 patients; intubated group compared to non-intubated group. METHODS: We retrospectively collected the data of confirmed hospitalized COVID-19 patients at the Royal Hospital, Muscat, Oman, from March to April 2020. Radiographic and clinical data were collected from the hospital and radiology information systems and compared between two groups based on intubation status. RESULTS: Twenty-six patients confirmed to have COVID-19 by reverse-transcriptase polymerase chain reaction test were included in the study; 15/26 were non-intubated, and 11/26 were intubated. Overall, 88.5% were males in the intubated group. Respiratory symptoms were the most common presentation (84.6%) followed by fever (76.9%), with no statistical difference between the two groups. There was a statistically significant difference in having diabetes mellitus (p = 0.020) in which 8/11 and 4/15 were recorded to have diabetes mellitus in the intubated and non-intubated groups, respectively. Other comorbidities showed no statistically significant difference. The radiographic analysis redemonstrates the peripheral lower zone distribution but no statistically significant difference among the two groups. There were no differences between the intubated and non-intubated chest radiography in laterality involvement, central and peripheral distribution, and lesions type. However, upper zones involvement was more noted in the intubated group with 10/11 (90.9%) compared to 7/15 (46.7%) in non-intubated cases (p = 0.036). There were higher numbers of zone involvement in intubated cases than non-intubated cases: 9/11 (81.8%) of intubated patients had 10-12 areas of involvement on chest radiographs compared to 3/15 (20.0%) in the non-intubated group. Half of the cases were discharged home; 3/11 from the intubated group and 10/15 from the non-intubated group. Five patients died from the intubated group (5/11) versus 3/15 from the non-intubated group. Five patients are still hospitalized (three from the intubated group and two from the non-intubated group). CONCLUSIONS: The radiographic findings among intubated and non-intubated hospitalized COVID-19 patients demonstrate differences in the number of zones involved. More upper zone involvement was noted in the intubated group. Male sex and diabetes mellitus carried a poorer prognosis and were more associated with the intubated group.

Artificial intelligence-driven assessment of radiological images for COVID-19.

Artificial Intelligence (AI) methods have significant potential for diagnosis and prognosis of COVID-19 infections. Rapid identification of COVID-19 and its severity in individual patients is expected to enable better control of the disease individually and at-large. There has been remarkable interest by the scientific community in using imaging biomarkers to improve detection and management of COVID-19. Exploratory tools such as AI-based models may help explain the complex biological mechanisms and provide better understanding of the underlying pathophysiological processes. The present review focuses on AI-based COVID-19 studies as applies to chest x-ray (CXR) and computed tomography (CT) imaging modalities, and the associated challenges. Explicit radiomics, deep learning methods, and hybrid methods that combine both deep learning and explicit radiomics have the potential to enhance the ability and usefulness of radiological images to assist clinicians in the current COVID-19 pandemic. The aims of this review are: first, to outline COVID-19 AI-analysis workflows, including acquisition of data, feature selection, segmentation methods, feature extraction, and multi-variate model development and validation as appropriate for AI-based COVID-19 studies. Secondly, existing limitations of AI-based COVID-19 analyses are discussed, highlighting potential improvements that can be made. Finally, the impact of AI and radiomics methods and the associated clinical outcomes are summarized. In this review, pipelines that include the key steps for AI-based COVID-19 signatures identification are elaborated. Sample size, non-standard imaging protocols, segmentation, availability of public COVID-19 databases, combination of imaging and clinical information and full clinical validation remain major limitations and challenges. We conclude that AI-based assessment of CXR and CT images has significant potential as a viable pathway for the diagnosis, follow-up and prognosis of COVID-19.

COVID-19 Associated Pneumonia: A review of chest radiograph and computed tomography findings.

Medical imaging, including chest radiography and computed tomography, plays a major role in the diagnosis and follow-up of patients with COVID-19 associated pneumonia. This review aims to summarise current information on this topic based on the existing literature. A search of the Google Scholar (Google LLC, Mountain View, California, USA) and MEDLINE® (National Library of Medicine, Bethesda, Maryland, USA) databases was conducted for articles published until April 2020. A total of 30 articles involving 4,002 patients were identified. The most frequently reported imaging findings were bilateral ground glass and consolidative pulmonary opacities with a predominant lower lobe and peripheral subpleural distribution.

Pulmonary Alveolar Microlithiasis: A Case Report.

Pulmonary alveolar microlithiasis (PAM) is a rare disease caused by a mutation in the SLC34A2 gene encoding the type IIb sodium phosphate cotransporter in alveolar type II cells. This results in the formation and accumulation of calcium phosphates crystals in the alveoli. Early in the disease, most patients are asymptomatic or might experience mild symptoms. However, in some patients, PAM can progress resulting in pulmonary fibrosis, cor pulmonale, and respiratory failure. We report the case of a 33-year-old Omani male who was referred to our institute with a history of fever and shortness of breath. A chest radiograph revealed bilateral dense consolidation. Chest computed tomography showed bilateral dense interlobular thickening and extensive consolidations with a lower lung predominance. Our findings were highly suggestive of PAM. The diagnosis was confirmed by bronchoalveolar lavage.

Prevalence and Spectrum of Coronary Anomalies Detected on Coronary Computed Tomography Angiography: A single centre experience in Oman.

OBJECTIVES: Coronary artery anomalies (CAAs) are uncommon congenital abnormalities with a prevalence ranging from 0.2-2%. CAAs can be asymptomatic or less commonly present with life-threatening symptoms. This study aimed to investigate the prevalence and spectrum of CAAs in patients who underwent coronary computed tomography angiography (CCTA) in Oman. METHODS: This retrospective study was conducted at the National Heart Centre, Muscat, Oman between September 2012 and August 2018. All consecutive patients who had undergone CCTA were included. RESULTS: A total of 4,445 patients were included in this study. Of these, 59 patients (1.3%) were diagnosed with CAAs with a mean age of 52.6 years (range: 12-80 years) and an equal gender distribution. Among the patients with CAAs, the majority (69.5%) had anomalous origins from the opposite or non-coronary sinus. Right coronary artery arising from the left coronary sinus was the most common type (33.9%). Fewer patients (18.6%) had left circumflex arising from the right coronary sinus (RCS). Seven patients (11.9%) had left main arising from the RCS. Other CAAs were in the dual left anterior descending artery (8.5%), high coronary artery take-off (6.8%), single coronary ostia (6.8%) and coronary artery fistula (6.8%). CONCLUSION: The prevalence of CAAs was 1.3% which is similar to the literature.

A New Variant of Dual Left Anterior Descending Artery Anomaly: Type XI.

A dual left anterior descending (LAD) artery is a rare congenital anomaly which is classified into different types based on the origin, course and termination of the short and long LAD arteries. To date, 10 variants of dual LAD artery anomalies have been described. We report a 44-year-old woman who was referred to the Department of Radiology, Royal Hospital, Muscat, Oman, in 2017. Coronary computed tomography angiography revealed a dual LAD artery anomaly in which the short and long LAD arteries shared a common ostium with the right coronary artery from the right coronary sinus. To the best of the authors' knowledge, this type of variant has not been previously reported in the literature.

Ortner's syndrome: Cardiovocal syndrome caused by aortic arch pseudoaneurysm.

72-year-old hypertensive presented with two weeks history of left sided chest pain and hoarseness. Workup demonstrated a pseudoaneurysm in the lesser curvature of the distal aortic arch opposite the origin of the left subclavian artery from a penetrating atherosclerotic ulcer. Following a left carotid-subclavian bypass, endovascular stenting of the aorta was performed excluding the pseudoaneurysm. Patient had excellent angiographic results post-stenting. Follow up at 12 weeks demonstrated complete resolution of his symptoms and good stent position with no endo-leak. Ortner's syndrome describes vocal changes caused by cardiovascular pathology. It should be included in the differential diagnosis of patients with cardiovascular risk factors presenting with hoarseness. This case demonstrates the use of endovascular stents to treat the causative pathology with resolution of symptoms. In expert hands, it represents low risk, minimally invasive therapeutic strategy with excellent early results in patients who are high risk for open procedure.

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: The Role of Multislice Computed Tomography (MSCT).

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary abnormality also known as Bland-White-Garland syndrome. The incidence of ALCAPA is about 1 in every 300,000 live births, and constitutes 0.24% and 0.46% of all congenital cardiac disease. It has a high infant mortality rate reaching up to 90% if left untreated. For many years, the diagnosis of ALCAPA was by angiography or autopsy. However, multislice computed tomography (MSCT) is a non-invasive imaging tool that allows accurate, non-invasive diagnosis of ALCAPA. Here we report a case of ALCAPA in a six-month-old girl who presented with a two-week history of cough, fever, tachypnea, and sweating during feeding. During admission, an echocardiogram was performed that revealed ALCAPA, which was confirmed using CT. We discuss the role of MSCT in its diagnosis.

Carcinoma of unknown primary presenting as large right atrial and ventricular mass secondary to disseminated tumor thrombosis.

We report a 65-year-old male patient who presented with right heart failure and a large mobile right atrial and ventricular mass on echocardiography. His computed tomography demonstrated bilateral supraclavicular/mediastinal lymphadenopathy, right atrial and ventricular mass with right pulmonary artery segmental embolism, and multiple liver hypodense lesions. His tumor markers were negative. However, fine-needle aspiration cytology of supraclavicular lymph node revealed metastatic carcinoma suggestive of squamous cell carcinoma. He was suspected to have carcinoma of unknown primary origin. This case illustrates a rare presentation of carcinoma of unknown primary origin with disseminated tumor thrombosis primarily manifesting in heart and other sites.