Brugada syndrome uncovered in patient with pseudohypoaldosteronism due to hyperkalaemia.

Brugada syndrome is a rare sodium channelopathy that predisposes to an increased risk of malignant arrythmias and sudden cardiac death. Previous studies have reported that metabolic disturbances can uncover a Brugada ECG pattern. Given the risk of malignant arrhythmias, it is important to correctly diagnose and treat Brugada syndrome. We report a case of Brugada syndrome uncovered by hyperkalaemia precipitated in a patient with pseudohypoaldosteronism.

Outcome of Pregnancy in Women With D-Transposition of the Great Arteries: A Systematic Review.

Background Information on maternal and fetal outcomes of pregnancy in women with D-transposition of the great arteries is limited. We conducted a systematic literature review on pregnancies in women with transposition of the great arteries after atrial and arterial switch operations to better define maternal and fetal risk. Methods and Results A systematic review was performed on studies between 2000 and 2021 that identified 676 pregnancies in 444 women with transposition of the great arteries. A total of 556 pregnancies in women with atrial switch operation were tolerated by most cases with low mortality (0.6%). Most common maternal complications, however, were arrhythmias (9%) and heart failure (8%) associated with serious morbidity in some patients. Worsening functional capacity, right ventricular function, and tricuspid regurgitation occurred in ≈20% of the cases. Rate of fetal and neonatal mortality was 1.4% and 0.8%, respectively, and rate of prematurity was 32%. A total of 120 pregnancies in women with arterial switch operation were associated with no maternal mortality, numerically lower rates of arrhythmias and heart failure (6% and 5%, respectively), significantly lower rate of prematurity (11%; P<0.001), and only 1 fetal loss. Conclusions Pregnancy is tolerated by most women with transposition of the great arteries and atrial switch operation with low mortality but important morbidity. Most common maternal complications were arrhythmias, heart failure, worsening of right ventricular function, and tricuspid regurgitation. There was also a high incidence of prematurity and increased rate of fetal loss and neonatal mortality. Outcome of pregnancy in women after arterial switch operations is more favorable, with reduced incidence of maternal complications and fetal outcomes similar to women without underlying cardiac disease.

Coronary Artery Aneurysm in Kawasaki Disease: Coronary CT Angiography through the Lens of Pathophysiology and Differential Diagnosis.

Kawasaki disease (KD) is an inflammatory autoimmune vasculitis affecting the coronary arteries of very young patients, which can result in coronary artery aneurysms (CAAs) with lifelong manifestations. Accurate identification and assessment of CAAs in the acute phase and sequentially during the chronic phase of KD is fundamental to the treatment plan for these patients. The differential diagnosis of CAA includes atherosclerosis, other vasculitic processes, connective tissue disorders, fistulas, mycotic aneurysms, and procedural sequelae. Understanding of the initial pathophysiology and evolutionary arterial changes is important to interpretation of imaging findings. There are multiple applicable imaging modalities, each with its own strengths, limitations, and role at various stages of the disease process. Coronary CT angiography is useful for evaluation of CAAs as it provides assessment of the entire coronary tree, CAA size, structure, wall, and lumen characteristics and visualization of other cardiothoracic vasculature. Knowledge of the natural history of KD, the spectrum of other conditions that can cause CAA, and the strengths and limitations of cardiovascular imaging are all important factors in imaging decisions and interpretation. Keywords: Pediatrics, Coronary Arteries, Angiography, Cardiac © RSNA, 2021.