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A successful pregnancy outcome of homozygous familial hypercholesterolaemia patient on statin therapy

Mohd Kasim NA, Al-Khateeb A, Chua YA, Sanusi AR, Mohd Nawawi H

Malays J Pathol, 2021 Apr;43(1):87-93.
PMID: 33903311

Homozygous familial hypercholesterolaemia (HoFH) is a rare genetic disorder of lipoprotein metabolism mainly due to mutation of the low-density lipoprotein (LDL)-receptor gene (LDLR). It is a life-threatening disease that causes accelerated, multi-vessel atherosclerosis presented in early childhood. Pregnancy in HoFH may pose early coronary morbidity and mortality to both the foetus and mother. The combination of HoFH and pregnancy can be a fatal condition. While statins are very effective in lowering low-density lipoprotein cholesterol (LDL-C) levels, they are generally contraindicated during pregnancy, thus their use during pregnancy is uncommon. On the other hand, lipid apheresis (LA) has turned into an effective treatment to control cholesterol level amid pregnancy. However, the procedure is not widely available in our region. To date, there are scarcely documented case reports of HoFH in pregnancy in which the majority of them underwent LA to keep LDL-C at a low level. We report a rare case of successful pregnancy outcome of HoFH patient treated with lipid-lowering drugs including statin without LA therapy. Apart from that, we also discussed the genetic findings of the proband and all screened family members in which to the best of our knowledge, the first study using the whole-exome sequencing technique to identify the causative gene mutations for familial hypercholesterolaemia among the Malaysian population.
Fulltext A double-blind randomised controlled trial on the effect of Tocovid, a tocotrienol-rich capsule on postoperative atrial fibrillation at the National Heart Institute, Kuala Lumpur: an interim blinded analysis

Musa AF, Dillon J, Md Taib ME, Yunus AM, Sanusi AR, Nordin MN, et al.

J Cardiothorac Surg, 2021 Nov 24;16(1):340.
PMID: 34819126 DOI: 10.1186/s13019-021-01721-6

INTRODUCTION: Post-operative atrial fibrillation (POAF) is associated with poorer outcomes, increased resource utilisation, morbidity and mortality. Its pathogenesis is initiated by systemic inflammation and oxidative stress. It is hypothesised that a potent antioxidant and anti-inflammatory agent such as tocotrienol, an isomer of Vitamin E, could reduce or prevent POAF.
AIMS: The aim of this study is to determine whether a potent antioxidative and anti-inflammatory agent, Tocovid, a tocotrienol-rich capsule, could reduce the incidence of POAF and affect the mortality and morbidity as well as the duration of ICU, HDU and hospital stay.
METHODS: This study was planned as a prospective, randomised, controlled trial with parallel groups. The control group received placebo containing palm superolein while the treatment group received Tocovid capsules. We investigated the incidence of POAF, the length of hospital stay after surgery and the health-related quality of life.
RESULTS: Recruitment commenced in January 2019 but the preliminary results were unblinded as the study is still ongoing. Two-hundred and two patients have been recruited out of a target sample size of 250 as of January 2021. About 75% have completed the study and 6.4% were either lost during follow-up or withdrew; 4% of participants died. The mean age group was 61.44 ± 7.30 years with no statistical difference between the groups, with males having a preponderance for AF. The incidence of POAF was 24.36% and the mean time for developing POAF was 55.38 ± 29.9 h post-CABG. Obesity was not a predictive factor. No statistically significant difference was observed when comparing left atrial size, NYHA class, ejection fraction and the premorbid history. The mean cross-clamp time was 71 ± 34 min and the mean bypass time was 95 ± 46 min, with no difference between groups. There was a threefold increase in death among patients with POAF (p = 0.008) and an increase in the duration of ICU stay (p = 0.01), the total duration of hospital stay (p = 0.04) and reintubation (p = 0.045).
CONCLUSION: A relatively low incidence rate of POAF was noted although the study is still ongoing. It remains to be seen if our prophylactic intervention using Tocovid would effectively reduce the incidence of POAF. Clinical Registration Number: US National Library of Medicine. Clinical Trials - NCT03807037. Registered on 16th January 2019. Link: https://clinicaltrials.gov/ct2/show/NCT03807037.
Incidence and Outcomes of Postoperative Atrial Fibrillation after Coronary Artery Bypass Grafting of a Randomized Controlled Trial: A Blinded End-of-cycle Analysis

Musa AF, Dillon J, Taib MEM, Yunus AM, Sanusi AR, Nordin MN, et al.

Rev Cardiovasc Med, 2022 Apr;23(4):122.
PMID: 39076222 DOI: 10.31083/j.rcm2304122

OBJECTIVE: The objective of this study is to analyse the incidence of postoperative atrial fibrillation (POAF), demography, post-operative outcomes including morbidity and mortality, length of Cardiac Intensive Care Unit (CICU) stay, High Dependency Unit (HDU) stay, and total hospital stay in patients undergoing coronary bypass grafting (CABG) at Institut Jantung Negana (IJN).
METHODS: We conducted a prospective, randomised, controlled trial. We supplied the treatment group with Tocovid capsules and the control group with placebo containing palm superolein.
RESULTS: Since January 2019, we have recruited the target population of 250 patients. However, the result is still blinded as we are still analysing blood samples for tocotrienol levels. 89.2% of patients completed the study with a 3.6% mortality and a 7.6% attrition rate. 35.2% of the patients developed POAF, the mean time being 46.06 ± 26.96 hours post-CABG. We did not observe any statistically significant difference when we compared left atrial size, New York Heart Association (NYHA) functional class, ejection fraction and premorbid history, besides EuroSCORE II (The European System for Cardiac Operative Risk Evaluation II) status except for older age group, right atrial size, and pleural effusion. There was also no difference in bypass time, cross clamp time or number of anastomoses. However, we noted a significant difference in death (p = 0.01) and renal failure requiring dialysis (p = 0.007) among patients with POAF; those patients also had a longer CICU stay (p = 0.005), HDU stay (p = 0.02), and total hospital stay (p = 0.001).
CONCLUSIONS: POAF is associated with a higher incidence of renal failure and death while it increases CICU, HDU, and total hospital stay. It remains to be seen whether Tocovid reduces POAF and its associated sequelae.
CLINICAL TRIAL REGISTRATION: NCT03807037 (Registered on 16 January 2019).
Fulltext Diagnostic performance of various familial hypercholesterolaemia diagnostic criteria compared to Dutch lipid clinic criteria in an Asian population

Abdul-Razak S, Rahmat R, Mohd Kasim A, Rahman TA, Muid S, Nasir NM, et al.

BMC Cardiovasc Disord, 2017 Oct 16;17(1):264.
PMID: 29037163 DOI: 10.1186/s12872-017-0694-z

BACKGROUND: Familial hypercholesterolaemia (FH) is a genetic disorder with a high risk of developing premature coronary artery disease that should be diagnosed as early as possible. Several clinical diagnostic criteria for FH are available, with the Dutch Lipid Clinic Criteria (DLCC) being widely used. Information regarding diagnostic performances of the other criteria against the DLCC is scarce. We aimed to examine the diagnostic performance of the Simon-Broom (SB) Register criteria, the US Make Early Diagnosis to Prevent Early Deaths (US MEDPED) and the Japanese FH Management Criteria (JFHMC) compared to the DLCC.
METHODS: Seven hundered fifty five individuals from specialist clinics and community health screenings with LDL-c level ≥ 4.0 mmol/L were selected and diagnosed as FH using the DLCC, the SB Register criteria, the US MEDPED and the JFHMC. The sensitivity, specificity, efficiency, positive and negative predictive values of individuals screened with the SB register criteria, US MEDPED and JFHMC were assessed against the DLCC.
RESULTS: We found the SB register criteria identified more individuals with FH compared to the US MEDPED and the JFHMC (212 vs. 105 vs. 195; p