The pathophysiological mechanisms which lead to sudden infant death syndrome (SIDS) are not completely understood. Cardiac channelopathies are a well-established causative factor with long QT syndrome (LQTS) being the most frequent one, accounting for approximately 12% of SIDS cases. The genetic substrate of the above arrhythmogenic syndrome has been thoroughly described but only specific gene mutations or polymorphisms have been identified as SIDS causative. The review will focus on the prevalence of LQTS-induced SIDS or near-SIDS cases and the mutations held responsible. A literature search was performed in PubMed and Scopus electronic databases. Search terms used were: long QT syndrome, channelopathies, QT prolongation, cardiac ion channels. The above-mentioned search terms were always combined with the term: sudden infant death syndrome. Study types considered eligible were: case–control, family pedigree analysis, case reports. The prevalence of LQTS-induced SIDS according to six broad genetic studies ranges from 3.9 to 20.6%, with an average of 12%. Since LQTS can be effectively managed, LQTS-related SIDS cases could be prevented, provided that a screening method is efficient enough to detect all the affected infants.

The delineation of bone structures is a crucial step in Chest X-ray image analysis. In the case of lung field segmentation, the main approach after the localization of bone structures is either their individual analysis or their suppression. We prove that a very fast and approximate identification of bone points that are most probably located inside the lung area can help in the segmentation of the lung fields, without the need for bone structure suppression. We introduce a deformation-tolerant region growing procedure. In a two-step approach, a sparse representation of the rib cage is guided to several support points on the lung border. We studied and dealt with the presence of other bone structures that interfere with the lung field. Our method demonstrated very robust behavior even with highly deformed lung appearances, and it achieved state-of-the-art performance in segmentations for the vast majority of evaluated CXR images. Our region growing approach based on the automatically detected rib cage points achieved an average Dice similarity score of 0.92 on the Montgomery County Chest X-ray dataset. We are confident that bone seed points can robustly mark a high-quality lung area while remaining unaffected by different lung shapes and abnormal structures.

Periostin, a secreted matricellular protein, has been implicated in cardiac extracellular matrix remodeling and fibrosis. Evidence suggest that periostin stimulates cardiomyocyte hypertrophy. The current study aims to investigate the extent of periostin expression in patients with advanced Hypertrophic Cardiomyopathy (HCM) and its correlation with fibrosis and hallmark histopathological features of the disease. Interventricular septal tissue from thirty-nine HCM patients who underwent myectomy and five controls who died from non-cardiac causes was obtained. Staining with Masson’s Trichrome and immunohistochemistry were used to localize fibrosis and periostin respectively. The extent of fibrosis and the expression of periostin were defined as the stained percentage of total tissue area using digital pathology software. Periostin expression was higher in HCM patients compared to controls (p<0.0001), positively correlated with the extent of fibrosis (r = 0.82, p<0.001), positively correlated with maximal interventricular septal thickness (Rho = 0.33, p = 0.04) and negatively correlated with LVEF (r = -0.416, p = 0.009). Periostin was approximately co-localized with fibrosis. Mean periostin expression was lower in patients with mild grade cardiomyocyte hypertrophy compared to those with moderate grade (p = 0.049) and lower in patients with mild grade replacement fibrosis compared to moderate grade (p = 0.036). In conclusion, periostin is overexpressed in advanced HCM, correlated with fibrosis and possibly related to cardiomyocyte hypertrophy.

Pulmonary artery aneurysms are a rare but often fatal clinical entity with an estimated incidence of 1 in 14,000 individuals in postmortem studies. They can be congenital or acquired. No specific guidelines regarding their optimal management, medical or surgical, currently exist and treatment is planned on a case-by-case basis since data regarding their clinical course and prognosis are limited. We present the case of a 77-year-old male patient who presented at the Emergency Department of our hospital with a complaint of exertional dyspnea and dull substernal pain over 1 week. Upon investigation, a main pulmonary artery true aneurysm measuring 61 mm on Computed Tomography was detected. The patient's history was remarkable for heavy smoking, arterial hypertension, dyslipidemia, known ascending aortic aneurysm, moderate COPD, and past tuberculosis. He was admitted to the Cardiology unit and treated as a case of decompensated heart failure with preserved ejection fraction. His symptoms improved with intravenous diuretics. A past chest MRI report, 7 years before his current event, described the main PA aneurysm measuring 51-52 mm. Regarding the main PA aneurysm, the heart team decided to follow a conservative approach with regular follow-up visits based on the patient's comorbidities, functional status, and slow growth rate of the PA aneurysm. Management of pulmonary artery aneurysms requires a heart-team approach in the context of the patient's underlying conditions and symptoms. More data are required in order to guide a treatment plan with an acceptable risk – benefit profile for each patient.

A single coronary artery (SCA) is a rare congenital anomaly with an incidence of 0.024 - 0.066% in angiographies and potential implications for adverse events depending on the course of the anomalous artery. We present a unique case of a single coronary artery arising from the right sinus of Valsalva. A 77-year-old female presented to the emergency department with an ongoing 3-hour episode of palpitations and intermittent atypical chest pain over 1 week. Her medical history included hypertension and dyslipidemia. Electrocardiography revealed atrial fibrillation with rapid ventricular response, which was successfully managed with intravenous amiodarone and the diagnostic workup ruled out life threatening thoracic pathology including cardiac ischemia. A CT coronary angiography was performed due to the moderate pretest probability of coronary artery disease. The scan identified a single coronary artery with a common origin of the left main coronary artery (LMCA) and the right coronary artery (RCA), from the right sinus of Valsalva, classified as Lipton Type RII-A which is a benign variant. This case highlights the importance of identifying CAAs, which are often incidental but may have clinical relevance depending on the anatomical course and associated risk factors. Early and accurate diagnosis through advanced imaging techniques is crucial to guide appropriate management and ensure optimal outcomes for the patient.