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.

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.

Comparison of 30-day outcomes of coronary artery bypass grafting surgery verus hybrid coronary revascularization stratified by SYNTAX and euroSCORE.

OBJECTIVE: The optimal treatment of multivessel coronary artery disease is not well established. Hybrid coronary revascularization by combining the left internal mammary artery-left anterior descending artery graft and drug-eluting stents in non-left anterior descending artery territories might offer superior results compared with sole coronary artery bypass grafting or sole percutaneous coronary intervention. METHODS: We retrospectively analyzed the 30-day outcomes of 381 consecutive patients undergoing coronary artery bypass grafting (n = 301) vs hybrid coronary revascularization (n = 80). In a 2 × 2 matrix, the 2 groups were stratified by the Synergy Between Percutaneous Coronary Intervention With Taxus and Cardiac Surgery (SYNTAX) score (≤32 vs ≥33) and the European System for Cardiac Operative Risk Evaluation (euroSCORE) (<5 vs ≥5). The composite endpoint (death from any cause, stroke, myocardial infarction, low cardiac output syndrome) and secondary endpoints (worsening postprocedural renal function and bleeding) were determined. RESULTS: After stratification using the SYNTAX and the euroSCORE, the preoperative characteristics were similar within the 4 groups, except for the ≥33 SYNTAX/>5 euroSCORE. The hybrid coronary revascularization patients were older (77 vs 65 years, P = .001). The postoperative outcomes using combined SYNTAX and the euroSCORE stratification showed a similar rate of the composite endpoint for all groups except for patients with ≥33 SYNTAX/>5 euroSCORE (0% for the coronary artery bypass grafting group vs 33% for the hybrid coronary revascularization group, P = .001). An analysis of the secondary endpoint showed similar results across all groups, except for in the ≥33 SYNTAX/>5 euroSCORE group, in which bleeding (re-exploration for bleeding and transfusion >3 packed red blood cell units per patient) was 44% in the hybrid coronary revascularization group vs 11% in the coronary artery bypass grafting group (P = .05). CONCLUSIONS: Hybrid coronary revascularization is a safe alternative to coronary artery bypass grafting in many patients with multivessel coronary artery disease. However, in high-risk patients with complex coronary artery disease (≥33 SYNTAX/>5 euroSCORE), coronary artery bypass grafting is superior to hybrid coronary revascularization.