Management and clinical outcomes of patients with homozygous familial hypercholesteremia in Saudi Arabia.

We report the incidence, patient characteristic with clinical outcomes in patients with homozygous familial hypercholesterolemia (HoFH) in Saudi Arabia. This is a retrospective and prospective, single center study which included 37 patients 14 years and older enrolled and followed up between 2018-2021 for three years. 46% were females, 78% were offspring of consanguineous marriage. LDLR mutation was in 78% and LDL-C/LDLRAP in 3% of patients. Mean LDL-C at the first presentation was 14.2±3.7 mmol/L, average Dutch lipid score was 20.9±6.24. LDL apheresis was performed on 70% of patients. Most patients were on ezetimibe (92%), high-dose statins ( 84%) and  PCSK9 inhibitors (32%). 48.6% had aortic stenosis, out of which 30% had severe aortic stenosis. Ten underwent aortic valve surgery (5 mechanical valve, 3 Ross procedure, 1 aortic valve repair, 1 bioprosthetic valve) and one had transcatheter aortic valve implantation (TAVI). Coronary artery bypass surgery (CABG) was performed on 32% and percutaneous intervention (PCI) on 11% of patients. HoFH patients have complex diseases with high morbidity and mortality, and benefit from a highly specialized multidisciplinary clinic to address their clinical needs. Although there are several therapeutic agents on the horizon, early diagnosis, and treatment of HoFH remain critical to optimize patient outcomes.

Transcatheter mitral valve-in-valve implantation for failed bioprosthesis.

OBJECTIVE: This study is a report of clinical and echocardiographic outcomes of experience with transapical mitral valve-in-valve (VIV) replacement. METHODS: Eleven patients with a mean age of 63.7±13.0 years who underwent transapical mitral VIV implantation for a failed bioprosthesis at a single institution were enrolled. All of the patients were considered high-risk for surgical intervention, with a Society of Thoracic Surgery predicted risk of mortality of 14.2±17.6%, and a mean European System for Cardiac Operative Risk Evaluation (EuroSCORE II) of 10.5±6.1%. RESULTS: Transapical mitral VIV implantation was successful in all of the patients. Edwards, Sapien XT and Sapien 3 valves (Edwards Lifesciences Corp., Irvine, CA, USA) were used in 8 (73%), 2 (18%), and 1 (9%) patients, respectively. Size 26 valves were used in 6 (55%) patients while size 29 valves were used in 5 (45%) patients. All of the patients (11, 100%) had no or only trace mitral regurgitation at the end of the procedure. The mean length of hospital stay was 19±8.0 days. The survival was 100% at 14 days, and 90% at 30 days and at 4 years. One patient died as a result of multiorgan failure on day 16 of intensive care unit stay. The mean mitral valve gradient across the percutaneous valve was 2.26±1.047 mmHg, and the mean valve area was 2.20±0.14 cm2. Through the 4 years follow up, the New York Heart Association class of the 10 patients remaining improved to class II with no readmission for heart failure. All of the patients were on coumadin with a target international normalized ratio of 2-3. CONCLUSION: In high-risk patients, transapical mitral VIV implantation can be performed with a high success rate and considerable improvement in clinical status.

Isolated Reoperative Tricuspid Valve Surgery: Outcomes and Risk Assessment.

OBJECTIVE: To describe patient characteristics and post-operative outcomes, including early and late mortality, defined by death within 30 days and after 30 days post-surgery, respectively, as well as 20-year survival after isolated reoperative tricuspid surgery. METHODS: We retrospectively analyzed 169 patients who underwent isolated reoperative tricuspid valve surgery at our institution (between 1997 and 2000) and describe post-surgical outcomes including intraoperative, early and late mortality. All patients included completed 21 years of follow-up. RESULTS: The majority of our patients were females 147 (87%) with the mean age of 45.9 ± 12.9 years. The mean body mass index (BMI, kg/m2) was 27.4 ± 6.0. Previous cardiac surgeries included tricuspid valve surgeries in 169 (100%) patients, with bioprosthetic valves, mechanical valves, annual rings and tricuspid repair surgeries utilized in 37 (21.9%), 21 (12.4%), 38 (22.4%) and 73 (43.2%) patients, respectively. The indication for previous tricuspid surgery was rheumatic heart disease in 154 (91.5%) patients.The most common cause of reoperative valvular surgery was tricuspid regurgitation (TR) in 139 (82.2%), with 66% of patients having severe TR. Other reasons for reoperative surgery included tricuspid stenosis 22 (13%) and dehiscence 8 (4.7%). For the redo surgery, 125 (74%) patients underwent Tricuspid Valve Replacement (TVR), 90 (53%) of whom received bioprosthetic valves while 35 (21%) received mechanical valves. Forty-four patients (26%) underwent Tricuspid Valve Repair. Mortality within 30 days of surgery was 11.3% (20 patients) and 11.4% after 30 days, with 20 years survival being about 80%. CONCLUSIONS: Based on our experience, reoperation for failed isolated tricuspid valve replacement or repair was associated with reasonable mortality and good survival rate over long period of time.

Cardiovascular Toxicity Associated With Tyrosine Kinase Inhibitor Therapy In Chronic Myeloid Leukemia.

Treatment of Chronic myeloid leukemia (CML) typically entails a long-term course of tyrosine kinase inhibitors (TKI) therapy. This review provides a summary on the cardiotoxic effects of TKIs. Five small molecular TKIs were evaluated in our review. The cardiotoxic effects of TKIs can range from superficial edema to potentially fatal conditions such as congestive heart failure (HF) and acute coronary syndrome (ACS). With the constant introduction of newer generations of TKIs, it has been demonstrated that different TKIs have distinct cardiovascular safety profiles. Amongst which, the first-generation TKI - imatinib appears to have the safest profile, mainly causing edema along with nausea, rash and muscle cramps. Other TKIs, like the second-generation dasatinib, bosutinib,and nilotinib, have shown an increased incidence of pleural effusion and QT prolongation. Ponatinib, a third generation TKI, has shown a relatively high incidence of serious adverse effects including thrombotic vascular occlusion and heart failure, particularly in patients with a prior history of cardiovascular impairment. Therefore, it is advisable that at-risk patients taking TKIs be screened with an Electrocardiogram (ECG) and have a careful cardiovascular risk assessment before starting TKI therapy to avoid potential cardiotoxic effects such as arrhythmias, acute coronary syndrome (ACS), congestive heart failure, and pleural effusion. Keywords: tyrosine kinase inhibitor, TKI, chronic myelogenous leukemia, CML, cardiotoxicity, side effects, imatinib, dasatinib, bosutinib, nilotinib, ponatinib.

Peer-Led Surgical Safety Learning Among Medical Students Using a Novel Story-Based Approach.

Introduction Even though medical students are important for the future of the overall healthcare system, they are often overlooked as valuable participants in safeguarding patient safety. Moreover, surgical safety is a critical topic that deserves to be addressed thoroughly during medical education, as part of the broader topic of patient safety. To maximize students' engagement and to enhance their interest in patient safety and healthcare quality, it is imperative to explore and innovate stirring and interactive methods of learning. Through this paper, we present a unique narrative novel story-based, peer-led surgical safety teaching session organized by medical student representatives of Middle East's first student-led patient safety initiative and attended by medical students from various academic years. Methods A 1-hour case-based interactive learning session on surgical safety was developed, based on the World Health Organization (WHO) patient safety curriculum for medical schools. The session was peer-led at Alfaisal University College of Medicine, Riyadh and participation was voluntary. Learning objectives included surgical safety checklist, human factors, complexity of healthcare, students' critical role in safety and open disclosure. The session included a verbal "story-telling" segment, followed by a didactic segment where learning objectives were covered. Attendees were continuously engaged throughout the session with the help of verbal inquiries. Pre-test and post-test questionnaires were distributed to assess participants' knowledge, awareness and perceptions regarding surgical safety and other objectives covered in the session. Results A total of 75 students participated in the session, consisting of 57.3% females and 42.7% males. Responses to the pre-test and post-test were analyzed and compared. Most students who attended the session reported that it was of value to them - with more than 90% of students considering the session either valuable or highly valuable. After the session, more students (10.67%, p = 0.10) were correctly able to identify that the initial reaction in healthcare is often individual blame (Q.1). Additionally, more students (30.63%, p < 0.001) were able to correctly identify the implementation of the WHO surgical safety checklist as the major factor that has contributed to the reduction of errors in healthcare (Q.2). Students' responses also indicated that after attending the session a higher number (16%, p = 0.01) correctly identified that most errors linked to surgery were potentially preventable (Q.3). Conclusion Students are inherently willing to learn and engage in interactive learning. It was encouraging to see medical students show interest in this important patient safety topic, which also encourages similar future peer-learning initiatives. As reported, narrative story-based peer-assisted learning is an effective way to engage medical students in the cause of patient safety and should be utilized to further their knowledge and awareness regarding critical healthcare safety areas such as surgical safety, medication safety and infection control and instill a sense of responsibility in these future physicians.

Urgent Need for Adolescent Physical Activity Policies and Promotion: Lessons from "Jeeluna".

Physical inactivity is a growing concern in Kingdom of Saudi Arabia (KSA) and globally. Data on physical activity (PA) trends, barriers, and facilitators among adolescents in KSA are scarce. This study aims to identify PA trends amongst adolescents in KSA and associated health and lifestyle behaviors. Data from "Jeeluna", a national study in KSA involving around 12,500 adolescents, were utilized. School students were invited to participate, and a multistage sampling procedure was used. Data collection included a self-administered questionnaire, anthropometric measurements, and blood sampling. Adolescents who performed PA for at least one day per week for >30 min each day were considered to "engage in PA". Mean age of the participants was 15.8 ± 0.8 years, and 51.3% were male. Forty-four percent did not engage in PA regularly. Only 35% engaged in PA at school, while 40% were not offered PA at school. Significantly more 10-14-year old than 15-19-year-old adolescents and more males than females engaged in PA (<0.01). Mental health was better in adolescents who engaged in PA (<0.01). Adolescents who engaged in PA were more likely to eat healthy food and less likely to live a sedentary lifestyle (<0.01). It is imperative that socio-cultural and demographic factors be taken into consideration during program and policy development. This study highlights the urgent need for promoting PA among adolescents in KSA and addressing perceived barriers, while offering a treasure of information to policy and decision makers.

Outcomes of Routine Coronary Angiography in Asymptomatic Patients With End-Stage Renal Disease Prior to Kidney Transplantation.

We report the prevalence of coronary artery disease (CAD) in asymptomatic patients with end-stage kidney disease (ESKD) on hemodialysis and explore the best revascularization strategies prior to kidney transplantation. This is a retrospective single-center study, which included all patients who were candidates for kidney transplantation and underwent coronary angiography between 2003 and 2018. All included patients underwent coronary angiography without noninvasive testing and were asymptomatic cardiac-wise. Out of the 368 patients with ESRD, 45% had coronary vessel disease, 17% had 3-vessel disease, 11% had 2-vessel disease, 5.2% had significant left main artery narrowing, and 17% had single-vessel disease. Patients with 3-vessel disease had the worst survival rate at 5 and 10 years. The patients with significant 3-vessel disease or left main artery involvement underwent revascularization; 19% underwent coronary artery bypass grafting, 5% had stenting of the coronary arteries, and 4.7% were on maximal medical therapy. The patients who underwent stenting had a better survival than those on medical therapy, but the difference was not significant (P = .445). Our findings reflect a high prevalence of CAD in patients with ESKD. There is a need for further studies to evaluate benefits of cardiovascular screening in this patient population.