Objective(s): Drug-induced atrial fibrillation (AF) is considered an adverse effect of chemotherapeutic drugs. AF is a crucial risk factor for stroke, heart failure, myocardial infarction, and mortality. Pulmonary veins (PVs) are considered triggers inducing AF, and the sinoatrial node (SAN) may modulate PV activity and participate in AF genesis. AF was associated with early discontinuation of trastuzumab in patients with breast cancer. However, whether trastuzumab directly modulates the electrophysiological characteristics of PV and SAN remains unclear. Materials and Methods: ECG and conventional microelectrode system were used to record rabbit heart rhythm in vivo and electrical activities in vitro from isolated SAN, PV, and SAN-PV preparations. Results: Trastuzumab reduced the beating rate in isolated PV and SAN preparations at 1, 10, and 30 μ M (particularly in isolated SAN preparations) and induced burst firings in isolated PV preparations at 10 μ Μ . In addition, trastuzumab (10 μ M) induced SAN-PV conduction block and burst firings, which were blocked by wortmannin (a PI3K inhibitor, 100 nM). Similarly, ECG recordings showed that acute intravenous administration of trastuzumab (10 mg/kg) reduced rabbit heart rates. Conclusion: Trastuzumab increased PV arrhythmogenesis through interfering with PI3K signaling, which may contribute to the genesis of AF.

Primary leiomyosarcoma of the heart is extremely rare and found in about 0.2% of all cardiac tumors. Here in a 26-year-old man with progressive dyspnea, which had started since 2 months ago, is presented. Echocardiography revealed a left atrial mass, which was suggestive for a atrial myxoma.On the surgery, a tumoral tissue in the left atrium and pulmonary veins with attachment to peripheral soft tissue, was seen and incompletely resected. Histologic examination exhibited a hypercellular,necrotic and mitotically active spindle-celled tumor with fascicular arrangement. Immunohistochemistry showed a positive reaction to SMA in tumoral cells. The patient was advised to refer for postoperative chemotherapy, which was rejected. One year later, the patient was brought to hospital with tumor recurrence. Chemotherapy was initiated for the patient immediately, but the patient was expired 3 days later.

Background: Increased right ventricle-to-left ventricle (RV/LV) ratio on computed tomography pulmonary angiography (CTPA) has been reported as a poor prognostic indicator in patients with acute pulmonary embolism (PE). It has also been reported that pulmonary vein sign (PVS) on CTPA is a rare finding of PE. Objectives: To evaluate PVS on CTPA and unenhanced magnetic resonance imaging (MRI) in patients with PE suspicion. We also aimed to investigate the relationship between the severity of PE and presence of PVS, RV/LV ratio and combination of these two on unenhanced MRI. Patients and Methods: One-hundred-twelve patients with PE suspicion who underwent CTPA and unenhanced-MRI [steady state free precession (SSFP)] within the first 48-hours constituted the study group. All CTPA images were evaluated for the presence, location and severity of PE by observer-1. Two observers (observer-2 and 3), independently evaluated unenhanced-MR images for the presence of PVS without knowing the results of CTPA. Then, these 2 observers reviewed the CTPA and MRI images together with observer-4 to reach the final consensus for the presence of PVS and measurement of RV/LV ratio. Cohen’ s Kappa analysis was used to assess the agreement between observers. Relationship between the mean PE index and imaging findings (PVS, RV/LV) were calculated. Results: Presence of PVS on CT or MRI is significantly correlated with PE index and patients with PVS had more severe PE than those without. Presence of both PVS and RV/LV ratio > 1 on MRI indicates more severe pulmonary embolism than absence of PVS or RV/LV ratio > 1. There was a very good agreement for the detection of PVS between two observers on unenhanced MRI. Conclusion: PVS on CTPA or unenhanced MRI can be used as a sign of severe PE and it may also be an indicator of right heart dysfunction.

A 41-year-old man with the complaint of exertional dyspnea and atypical chest pain was admitted to the emergency department. The patient’, s electrocardiogram showed right bundle branch block (RBBB) and T-wave inversion in leads II and III. Transesophageal echocardiography revealed normal left ventricular systolic function, mild right ventricular enlargement, and a left partial anomalous pulmonary venous connection (PAPVC) with the left pulmonary vein draining into the innominate vein via a large vertical vein (Figure 1 and Video 1). Subsequently, multislice spiral computed tomography (CT) angiography displayed a connection between the left superior pulmonary vein and the left brachiocephalic vein via the vertical vein and mild enlargement of the right atrium and ventricle (Figure 2). Coronary artery angiography also showed normal coronary arteries. Therefore, the patient was referred for surgical correction. The surgical correction was successfully performed on cardiopulmonary bypass (Figure 3). . .

Introduction: Pulmonary vein stenosis includes narrowing of one or more pulmonary veins that may have congenital or acquired causes. Congenital pulmonary vein stenosis is a rare condition and also a rare cause for pulmonary hypertension. Since identifying the etiology of pulmonary hypertension plays a crucial role in medical and surgical planning, we decided to introduce a rare case of pulmonary hypertension due to pulmonary vein stenosis.Case Presentation: A 29-year-old white female patient who was suffering from exertional dyspnea was referred to our center for further evaluation. Our findings in transthoracic and trans esophageal echocardiography showed pulmonary arterial hypertension with maximum gradient of 65 - 70 mmHg and moderate to large size secondom type oval shaped atrial septal defect (2 cm - 1.2 cm) with bidirectional shunt, in a predominantly left to right direction. Atrial septal defect rims were suitable for device closure.Due to out of proportional pulmonary hypertension with atrial septal defect, further evaluations were conducted and pulmonary vein stenosis was confirmed. In this case, the echocardiography findings were consistent with right upper pulmonary vein stenosis and justified severe pulmonary hypertension with the anomaly of atrial septal defect. Device closure of atrial septal defect and balloon angioplasty of pulmonary vein stenosis were performed for our patients. Three months later, in a follow up evaluation, it was revealed that the severity of pulmonary hypertension was declined from 65 - 70 mmHg to 40 mmHg.Conclusions: Only few cases of pulmonary hypertension secondary to congenital lpulmonary vein stenosis in infancy were reported until now, and it is very uncommon in adult patients without any prior history of surgery. Patients with pulmonary vein stenosis often present with shortness of breath and may mimic symptoms of Chronic Lung Disease or pneumonia which result in misdiagnosis and treatment. Therefore, in patients with underlying congenital heart disease and out of proportional pulmonary hypertension, other potential and rare causes for pulmonary hypertension such as pulmonary vein stenosis should be considered and evaluated. It is recommended that cardiologists do not directly attribute pulmonary hypertension to the underlying heart disease without sufficient assessment to rule out other possible causes for it.

Introduction: Lemierre’, s syndrome (LS) is a rare and serious condition that often aff, ects school-aged children, adolescents, and healthy young adults. It is defi, ned as an oropharyngeal infection with secondary septicemia, internal jugular vein thrombosis, and septic emboli. Rare cases of atypical LS in which thrombosis occurs in other locations have been reported. Case Presentation: We presented a case of an adolescent with fever, sore throat, and neck tenderness. Physical examination revealed acute pharyngitis and bilateral small cervical lymph nodes. Rapid antigen group A Streptococcus and viral serologic tests were negative. Complete hemogram and blood biochemical analysis showed leukocytosis and elevated serum C-reactive protein (CRP). Cervical computerized tomography (CT) scan revealed a heterogeneous right tonsil and small cervical lymph nodes. The chest radiograph was normal. After admission, the adolescent started complaining of dyspnea and thoracic pain and had a painful small cervical right mass. In the thoracic CT angiography (CTA), several pulmonary septic emboli were apparent throughout the pulmonary parenchyma. Cervical Doppler ultrasound showed right facial vein thrombophlebitis, later confi, rmed in a CTA. Thromboses in other locations, including the internal jugular vein, were excluded, as well as cervical abscesses. The patient was successfully treated with intravenous antibiotic therapy and anticoagulation. Discussion: To this date, no reports of LS presenting with isolated thrombosis of the facial vein have been described in pediatric patients. We highlighted the importance of early recognition of LS and its atypical variants. Pediatric international guidelines regarding its management and treatment would also greatly aff, ect the outcome of these patients.