Myxoma Involving the Pulmonary Valve - A Case Report of an Atypical Cardiac Tumor, Treated Using an Unusual Approach.

Background: Atypical myxoma has been reported in various locations in the heart, however, myxoma involving the pulmonary valve is rare. Here we present a case of pulmonic valve myxoma which was resected via a percutaneous approach. Case Report: A 66-year-old female with known metastatic adenocarcinoma of the lung, and chronic obstructive pulmonary disease presented with acute onset shortness of breath for two days. The patient experienced respiratory arrest en-route to the hospital and required intubation. Computed tomography angiography (CTA) of the chest revealed a new 1.4 × 1.6 cm intracardiac mass along the pulmonary valve. Further evaluation with cardiac magnetic resonance imaging revealed it to be a large vascular tumor on the ventricular side of the pulmonary valve, attached with a narrow stalk. Due to high surgical risk, the patient underwent transesophageal echocardiographic guided percutaneous removal of the mass. Pathology confirmed the mass to be a myxoma. Conclusion: Atypical myxoma should be considered in the differential diagnosis of valvular masses. Percutaneous resection of valvular masses may be feasible in high-risk surgical patients. LEARNING POINTS: Pulmonary valve myxoma is a rare condition and the literature on the characteristics and treatment options for pulmonary valve myxoma is limited.Our patient was treated with a minimally invasive treatment approach: removal of a tumor with intra operative transesophageal echocardiographic guidance using AngioVac and Flow Triever catheters.Percutaneous resection of valvular masses may be feasible in high surgical risk patients.

Parachute Mitral Valve and Mid-Aortic Syndrome - Unusual Associations of Alagille Syndrome.

Background: Alagille syndrome (ALGS) is a multisystem disorder involving at least three systems among the liver, heart, skeleton, face, and eyes. Common cardiac associations include pulmonary artery stenosis/atresia, atrial septal defect (ASD), ventricular septal defect (VSD) and tetralogy of fallot (ToF). Coarctation of aorta (CoA), renal and intracranial arteries are commonly involved vessels in Alagille syndrome. We present two cases with rare cardiovascular manifestations of Alagille syndrome. Case description. Case 1: A 25-year-old female with a history of Alagille syndrome presented to the cardiologist office for progressive exertional dyspnoea, orthopnoea, and palpitations. She was tachycardiac on examination and had an apical diastolic rumble. A transthoracic echocardiogram (TTE) showed a left ventricular ejection fraction (LVEF) of 60% and parachute mitral valve (PMV) with severe mitral stenosis. A transoesophageal echocardiogram (TOE) showed insertion of chordae into the anterolateral papillary muscle, severe mitral stenosis with a valve area of 0.7 cm. She was referred to a congenital heart disease specialist and underwent robotic mitral valve replacement with improvement in her symptoms. Case 2: A 27-year-old female with known Alagille syndrome and resistant hypertension presented to the cardiologist office due to progressive exertional dyspnoea for a year. She was hypertensive and had a new 2/6 systolic ejection murmur along the left upper sternal border. TTE revealed an LVEF of 60% and pulmonary artery pressure of 19 mmHg. A CoA was suspected distal to the left subclavian artery due to a peak gradient of 38 mmHg. Cardiac magnetic resonance (CMR) imaging ruled out CoA, and diffuse narrowing of the descending thoracic aorta measuring 13-14 mm in diameter was noted. The patient was referred to a congenital heart disease specialist for further management. Conclusion: PMV presenting as mitral stenosis and mid-aortic syndrome are not commonly described anomalies in association with Alagille syndrome. TTE, TOE and CMR played a key role in diagnosis and management of these patients. LEARNING POINTS: Alagille syndrome (ALGS) is a complex multisystem disorder involving the liver, heart, skeleton, face, and eyes. Cardiovascular involvement occurs in up to 95% of the patients.Common cardiac associations include pulmonary artery stenosis/atresia, atrial septal defect (ASD), ventricular septal defect (VSD) and tetralogy of fallot (ToF).A parachute mitral valve (PMV) presenting as mitral stenosis and mid-aortic syndrome is not commonly described anomalies in association with ALGS. Here, we present such rare cases.

Left Anterior Descending Coronary T-wave Inversion Pattern (Wellens' Syndrome) Associated with Myopericarditis and a Normal Left Coronary Artery.

Background: Wellens' syndrome is characterised by a history of chest pain with an abnormal electrocardiogram (EKG), demonstrating biphasic or deeply inverted T waves in leads V2-3 (may extend to involve all precordial and lateral limb leads - the type B Wellens' pattern). A Wellens' EKG pattern is considered highly specific for critical stenosis involving the ostial/proximal left anterior descending artery (LAD). However, there are no reported cases of an association of a Wellens' EKG pattern with myopericarditis. Here, we present such a rare case. Case description: A thirty-one-year-old female with known essential hypertension and psoriatic arthritis presented with a constant, central chest pain radiating to the shoulders and back. The patient's physical examination was unremarkable at presentation other than elevated blood pressure at 170/68 mmHg. An EKG at presentation demonstrated deep symmetric T-wave inversions in anterolateral leads with elevated high-sensitivity troponin, and an elevated erythrocyte sedimentation rate. The patient was referred to the cardiac catheterisation laboratory for concerns of a Wellens' EKG pattern; however, invasive angiography demonstrated only obtuse marginal branch disease - no LAD disease was noted. Cardiac magnetic resonance (CMR) imaging confirmed the diagnosis of myopericarditis and absence of myocardial infarction. The patient was medically managed and discharged home in a stable condition. Conclusion: In literature and established clinical practice, the Wellens' EKG pattern is considered highly concerning for critical ostial/proximal LAD stenosis. However, we now propose that myopericarditis may be considered in a differential diagnosis for this EKG pattern. LEARNING POINTS: Wellens' syndrome is characterised by a history of chest pain with an abnormal electrocardiogram (EKG), demonstrating biphasic or deeply inverted T waves in leads V2-3.A Wellens' EKG pattern is considered highly specific for critical stenosis involving the ostial/proximal left anterior descending artery (LAD).Association of Wellens' pattern EKG has been described in association with various other pathologies; however, its association with acute myopericarditis has not been well described.

Mitral annular disjunction; how accurate are we? A cardiovascular MRI study defining risk.

Aims: Mitral Annular Disjunction (MAD) refers to embryologic fibrous separation between mitral annular ring and basal left ventricular myocardium. Since its original description, the role of MAD in arrhythmic mitral valve prolapse (MVP) has been the subject of active research. In this study we sought to assess prognostic and imaging characteristics of MVP patients with and without underlying MAD. Methods and results: Patients with posterior or bi-leaflet MVP were retrospectively identified via a review of all patients referred to our cardiac magnetic resonance (CMR) imaging laboratory from January 2015 to May 2022. MVP patients were further stratified by underlying MAD status. A total of 100 MVP patients undergoing CMR imaging (52 MVP patients with posterior MAD) were retrospectively identified with female comprising 55 % of the cohort. MVP patients with MAD were more likely to have an abnormal basal inferolateral/ papillary muscles LGE (51 % vs 21 %, p < 0.01). Posterior MAD longitudinal disjunction gap in 'mm' was a predictor of ventricular tachycardia (VT) [1.29, p = 0.01)]. Using ROC curve analysis, a disjunction gap of ≥ 4 mm was predictive of VT (AUC-0.71, p < 0.01), and incorporation of LGE in ROC model further improved AUC to 0.78 confirmed via Akaike information criterion (p < 0.01). Conclusion: Abnormal LGE involving basal inferolateral myocardium and papillary muscles may provide etiologic substrate for arrythmia in MVP patients.

A Rare Case of Atypical Hemolytic Uremia Syndrome Triggered by Influenza Vaccination.

Atypical hemolytic uremic syndrome (aHUS) occurs in patients with defective alternative complement pathways, making them susceptible to thrombotic microangiopathy (thrombocytopenia, intravascular hemolysis, and renal failure), and is usually triggered by infectious agents. Influenza and Streptococcus pneumonia are known triggers for aHUS. However, influenza vaccination triggering aHUS is rarely reported. We present a 30-year-old male who presented with chills, abdominal discomfort, and night sweats after receiving the influenza vaccine. The patient had thrombocytopenia, elevated creatinine, blood urea nitrogen, liver enzymes, and bilirubin with schistocytes with peripheral smear. ADAMTS13 activity was normal so the patient was diagnosed with aHUS. The patient improved with eculizumab and was ultimately found to have a mutation in CD46, which made him susceptible to aHUS. This case shows patients with dysregulated alternative complement pathways may be predisposed to develop aHUS after receiving influenza vaccination.

Perinephric Hematoma Associated with Pyelonephritis Following Ureteral Stent Placement for Ureteral Obstruction Causing Hydronephrosis.

BACKGROUND Perinephric hematomas are rare, especially following ureteral stent placement. Etiologies of perinephric hematomas include post-extracorporeal shockwave lithotripsy, Wunderlich syndrome, and renal cell carcinoma, none of which occurred in our patient, who underwent stent replacement. Subcapsular renal hematoma, rather than a perinephric hematoma, can occur following double-J ureteral stent placement. Also, renal parenchymal perforation leading to perinephric hematoma development are complications of double-J ureteral stent placement. Herein, we present a case of a perinephric hematoma following a double-J ureteral stent placement for a ureteral obstruction causing hydronephrosis. CASE REPORT A 43-year-old woman with type 2 diabetes mellitus, hypertension, systemic lupus erythematosus, and recurrent nephrolithiasis presented to our hospital with left flank pain of a 1-day duration. The patient was found to have an obstructive kidney stone causing hydronephrosis. She underwent stent placement and then developed a perinephric hematoma days later. Typically, hematomas are treated conservatively and have spontaneous resolution. The patient received 2 weeks of intravenous antibiotics and 2 more weeks of oral antibiotics, and failed conservative treatment. She re-presented to our hospital 3 days after discharge. Upon the second admission, a perinephric drain was placed. The patient was given another course of antibiotics and was discharged 18 days later. CONCLUSIONS A perinephric hematoma is a rare complication after ureteral stent placement. Perinephric hematoma development can be decreased by controlling blood pressure, treating preoperative urinary tract infections, and shortening operating time during ureteroscopy. It is important to reevaluate potential causes of continued abdominal pain with laboratory testing and repeat imaging.

Brodifacoum-Laced Synthetic Marijuana Toxicity: A Fight Against Time.

BACKGROUND As marijuana is being legalized in some states in the United States, there is a growing need for physicians to be aware of potential complications related to various forms of marijuana used in the community. Historically, marijuana has been laced with potentially toxic substances to increase its efficacy, and brodifacoum is one of them. Here, we present the case of a patient with toxicity related to use of brodifacoum-laced synthetic marijuana. CASE REPORT A 30-year-old man with history of polysubstance abuse presented with 5 days of flank pain and hematuria. He reported current use of synthetic marijuana. Vital signs were unremarkable. On physical examination, he had petechiae on bilateral upper extremities. Pertinent lab findings included: leukocytosis of 14 000 K/UL, international normalized ratio (INR) 13, prothrombin time (PT) 134.6 s, activated partial thromboplastin time (aPTT) 58.3 s, and only hematuria on urinalysis. CT scans of the abdomen and pelvis were unremarkable. The initial toxicology screen was negative. Brodifacoum toxicity was suspected. The patient was managed in collaboration with poison control, and he was treated with oral vitamin K and close monitoring of INR. CONCLUSIONS Brodifacoum-laced synthetic marijuana toxicity can lead to potentially lethal complications if not recognized and treated in a timely manner. Hence, physicians should have a high index of suspicion in patients presenting with unexplained coagulation abnormalities.

Early MRI Versus CT Scan for Evaluation of Cerebrovascular Events in a Community Hospital: A Cost Minimization Analysis.

BACKGROUND: Diffusion-weighted MRI is shown to be equally effective, as a CT scan, in diagnosing ischemic and hemorrhagic strokes. Would it be cost-effective to perform an early MRI instead of a CT head? METHODS: A retrospective chart review was conducted between October 1, 2015, through October 1, 2017, for patients admitted for possible cerebrovascular accident (CVA). Inclusion criteria were age >/= 18 years and symptoms suggestive of a stroke. Exclusion criteria were pregnancy and age <18 years. We obtained information regarding patients' length of hospital stay, imaging modalities performed, and the related cost. We performed a cost analysis by calculating the total duration and cost of hospitalization, and cost for each investigation. RESULTS: The study included 828 patients who underwent CT head without contrast initially. A total of 634 (76.5%) patients got MRI brain without contrast, 261 (31.5%) had MRI brain with and without contrast, 406 (49%) had magnetic resonance angiography (MRA) head without contrast, 60 patients (7.2%) had MRA neck without contrast, 272 (32.8%) had MRA neck with and without contrast, and 1 patient (0.1%) had MRA head with and without contrast. The hospital duration for all patients was 1,797 days. The average duration per patient was 1.9364 days. The total health care cost for all patients was $25,383,983. Average per patient hospitalization cost was $25,383,983/828 = $30656.98. Average per day cost for all patients would be $25383983/1.93days = $13,152,322.8. Combined costs of all MRIs performed on all patients = $1,413,014. If MRI brain with and without contrast was considered as an initial modality, a total of $335,340 can be saved on the diagnostic imaging. Ultimately, it can also help reduce the hospitalization duration. CONCLUSION: Early MRI (in appropriately selected patients) can reduce the length of hospitalization and cut some health care costs. However, more studies are required to develop appropriate patient selection criteria.

Frequency of functional constipation in 3 different populations and its causative factors.

OBJECTIVE: To assess the frequency of constipation and its causative factors in 3 different populations, namely, the hospitalized patients admitted at Civil Hospital Karachi, their attendants and medical students. METHOD: A sample size of 200 was divided into 3 groups: hospitalized patients, their attendants and medical students. Frequency of constipation was expected to be greater in patients, so 100 subjects were taken from this population; while from the other 2 populations only 50 subjects were taken. The subjects were diagnosed as constipated on the basis of Rome III criteria for functional constipation and their dietary habits and physical activity were also assessed. RESULTS: The frequency of functional constipation was found to be 53% in hospitalized patients, 52% in their attendants and 34% in medical students. Among the constipated individuals, 50.94% of hospitalized patients, 46.15% of attendants and 29.41% of medical students took laxatives for relief of their symptoms. Constipation was most prevalent among the age group of 18 to 30 years in all the 3 populations. CONCLUSION: Constipation was fairly common in all 3 populations. Male to female ratio is almost equal. Physical inactivity and low fiber intake were prominent risk factors for constipation.