scholarly journals Familial Hypercholesterolaemia With Premature Coronary Artery Disease: A Case Report

2020 ◽  
Vol 1 (3-4) ◽  
pp. 150-153
Author(s):  
Chandramukhi Sunehra ◽  
Krishnaswamy Raghu

A young, 18-year-old lady presented with history of chest pain on exertion typical of angina. General examination revealed multiple tendon xanthomas. Systemic examination was unremarkable. Electrocardiogram showed segment (ST) depression in inferior and lateral leads. Echocardiogram revealed normal left ventricular systolic function and no left ventricular regional wall motion abnormalities. Diastolic flow turbulence was noted in the left main coronary artery and proximal left anterior descending artery on color Doppler interrogation across the coronary arteries. Lipid profile showed unusually high total cholesterol and low-density lipoprotein cholesterol. Subsequent evaluation with coronary angiogram revealed triple vessel coronary artery disease. The patient underwent coronary artery bypass surgery and is on antiplatelet and lipid-lowering drug therapy.

2009 ◽  
Vol 16 (02) ◽  
pp. 192-197
Author(s):  
FIDA MUHAMMAD ◽  
Nadeem Hayat Mallick, ◽  
ABDUL REHMAN ABID ◽  
AJAZ AHMAD ◽  
Shahid Imran

Objectives: This study was designed to evaluate the pattern of clinical presentation, risk factors and angiographic findingsin young males presenting with acute myocardial infarction (AMI).Materials and methodsThis cross-sectional descriptive study wasconducted at the Cardiology Department, Punjab Institute of Cardiology, Lahore from May 2005 till February 2006. After fulfilling the inclusioncriteria 200 male patients <40 years with coronary artery disease (CAD) were studied. Results: Mean age of the study population was31.5±9.2 years with an age range of 31 to 40 years. Most common risk factor was smoking present in 60% patients. Family history ofischemic heart disease (IHD) was present in 44.5% patients, hyperlipidemia in 35.5% patients, hypertension in 25.5% and diabetes mellitusin 17.5% of patients.Common mode of clinical presentation was AMI 42.5% patients. Left anterior descending (LAD) was diseased in 73.5%,followed by Left Circumflex (LCx) 51% and Right Coronary Artery (RCA) in 39% patients. Left Main Stem (LMS) disease occurred in 9.5%patients. Good left ventricular (LV) systolic function was observed in 38%, moderate LV systolic function in 34% and poor LV systolic functionin 14.5% patients. Conclusion: Patients with premature coronary artery disease have unheralded acute onset of symptoms. Smoking isthe most common risk factor. Young patients have single vessel CAD with frequent involvement of LAD and commonly have good leftventricular systolic function.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
A Lauziere ◽  
D Brisson ◽  
S Bedard ◽  
E Khoury ◽  
G Tremblay ◽  
...  

Abstract Background Familial hypercholesterolemia (FH) is an autosomal dominant trait associated with high risk of premature coronary artery disease (CAD). The worldwide prevalence of FH is estimated at 1:250 to 1:500. In certain populations, including French Canadians (FC), the prevalence is significantly higher however. From 1995 to 1998, FH contributed to 9.6% of angiographically proven CAD in a FC founder population, the burden being the highest in men aged <50 years (20.6%). In the past 2 decades, powerful statins, ezetimibe and other low-density lipoprotein-Cholesterol (LDL-C) modulators, such as PCSK9 inhibitors, have been progressively introduced and several FH diagnosis scoring systems or guidelines have been developed and disseminated in order to facilitate FH recognition and management. The impact of these measures on the FH burden is however not documented. Purpose To compare the burden of FH twenty years apart in FC patients hospitalized for CAD. Methods Lipid profiles, cardiovascular risk factors and FH status of 1,132 FC patients who were hospitalized for a CAD event and who consecutively attended the cardiovascular disease clinic in 2017 and 2018 were compared to those of 2,506 who consecutively presented angiographically proven CAD two decades ago. FH status was based on Simon Broome and FH Canada definitions. In 1998, all consenting CAD patients were also molecularly screened for the most prevalent FH causing mutations in FC. Comparisons between groups were performed using Chi-square and independent samples Student's t-test. Results Most patients in both cohorts were males (74.5% vs 73.9% in 1998 vs. 2018, respectively). At admission, mean LDL-C (± SD) was 3.99±1.67 in 1998 vs. 2.22±1.06 in 2018 (p<0.001). The proportion of patients who were treated with a statin or another lipid lowering agent was 32.9% in 1998 compared to 67.6% in 2018 (p<0.001) and the drug regimen was also significantly different. In 1998, 24.6% of patients had LDL-C >5.0 mmol/L at admission compared to 4.2% in 2018. Definite FH was diagnosed in 9.6% of patients in the 1998 cohort compared to 4.7% in the 2018 cohort (p<0.001). FH patients hospitalized for CAD were significantly older in 2018 than in 1998 (56.3±11.3 vs. 49.2±10.9 in men, p=0.001; 61.1±11.8 vs. 53.2±12.3 in women, p=0.02). In the same period, the relative burden of diabetes and other lipid disorders, including high-density lipoprotein dysmetabolism significantly increased (p<0.001). Conclusions Over a period of 20 years, in a founder population with a high prevalence of FH, the contribution of FH to hospitalizations for CAD decreased by 2-fold and affected patients now tend to be hospitalized at an older age than 2 decades ago. This suggests that early diagnosis and more effective management of FH in the last 2 decades have contributed to significantly decrease its burden. Acknowledgement/Funding ECOGENE-21, Amgen, Sanofi


2021 ◽  
Vol 9 (08) ◽  
pp. 487-491
Author(s):  
D. Massimbo ◽  
S. Nikiema ◽  
S. Ahchouch ◽  
I. Asfalou ◽  
A. Benyass

Introduction: The risk factors for aortic stenosis have been shown to be similar to those for atherosclerosis. Thus, coronary disease is often found simultaneously in patients with aortic stenosis. Our work aims to determine the frequency of coronary disease in a Moroccan population with aortic stenosis while recalling the causes and the prognostic and therapeutic impacts of this association. Materials and Methods: This is a retrospective study of 148 patients hospitalized at the cardiology center of the military hospital of Rabat over a period of 24 months, during which we analyzed clinical, electrocardiographic, echocardiographic and coronarographic data of the patients in order to evaluate the coronary involvement during aortic stenosis. Results: The mean age of the population was 65 [57, 74] years, the sex ratio was 1.21. Smoking reported in 38.5% of patients was the main modifiable cardiovascular risk factor, followed by hypertension in 35.8% of patients. Dyspnea on exertion was the most frequent reason for consultation at 81%, 64% of which were at least NYHA functional class III, followed by angina, which represented 33% of the series. The aortic stenosis was tight in the majority (mean SAo: 0.8 cm²) and the left ventricular ejection fraction was preserved overall. Coronary artery disease was associated with aortic stenosis in 24% of cases, with predominantly monotruncal involvement (53%) followed by tritruncal involvement (30%). 21.6% of these patients underwent coronary artery bypass grafting concomitantly with surgical replacement of the aortic valve. Conclusion: The incidence of coronary artery disease associated with aortic stenosis is variable according to age. It is higher in European series because of aging. In our relatively younger population, it is lower but not negligible.


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Joost Besseling ◽  
Gerard K Hovingh ◽  
John J Kastelein ◽  
Barbara A Hutten

Introduction: Heterozygous familial hypercholesterolemia (heFH) is characterized by high levels of low-density lipoprotein cholesterol (LDL-C) and increased risk for premature coronary artery disease (CAD) and death. Reduction of CAD and mortality by statins has not been properly quantified in heFH. The aim of the current study is to determine the effect of statins on CAD and mortality in heFH. Methods: All adult heFH patients identified by the Dutch FH screening program between 1994 and 2014 and registered in the PHARMO Database Network were eligible. Of these patients we obtained hospital, pharmacy (in- and outpatient), and mortality records in the period between 1995 and 2015. The effect of statins (time-varying) on CAD and all-cause mortality was determined using a Cox proportional hazard model, while correcting for the use of other lipid-lowering therapy, thrombocyte aggregation inhibitors, antihypertensive and antidiabetic medication (all time-varying). Furthermore, we used inverse probability for treatment weighting (IPTW) to account for differences between statin-treated and untreated patients regarding history of CAD before follow-up, age at start of follow-up and age of screening, as well as body mass index, LDL-C and triglycerides. Results: Of the 25,479 identified heFH patients, 11,021 gave informed consent to obtain their medical records, of whom 2,447 could be retrieved. We excluded 766 patients younger than 18. The remaining 1,681 heFH patients comprised our study population and these had very similar characteristics as compared to the 23,798 excluded FH patients, e.g. mean (SD) LDL-C levels were 214 (74) vs. 203 (77) mg/dL. Among 1,151 statin users, there were 133 CAD events and 15 deaths during 10,115 statin treated person-years, compared to 17 CAD events and 9 deaths during 4,965 person-years in 530 never statin users (combined rate: 14.6 vs. 5.2, respectively, p<0.001). After applying IPTW to account for indication bias and correcting for use of other medications, the hazard ratio of statin use for CAD and all-cause mortality was 0.61 (0.40 - 0.93). Conclusions: In heFH patients, statins lower the risk for CAD and mortality by 39%.


2020 ◽  
Vol 35 (1) ◽  
pp. 20-27
Author(s):  
Sanjay Kumar Raha ◽  
Md Sorower Hossain ◽  
Smriti Kana Biswas ◽  
Salahuddin Rahaman ◽  
Manzil Ahmad ◽  
...  

Introduction: Left ventricular dysfunction is an important predictor of in-hospital mortality. Due to the theoretical and practical advantages to avoid the harmful effects of cardiopulmonary bypass (CPB), many cardiac surgeons are using Off-pump Coronary Artery Bypass (OPCAB)as an effective alternative to conventional CABG (CCAB) even in patients with reduced left ventricular (LV)ejection fraction. Objectives: This study performed in the National Institute of Cardiovascular Diseases (NICVD) evaluated the early outcomes of OPCAB in terms of mortality and major post-operative morbidities and compared them with that of CCAB in patients with multivessel coronary artery diseases and reduced left ventricular (LV) function. Methods: Total 120 patients with multivessel coronary artery disease with reduced left ventricular ejection fraction (d”50%)were allocated into two groups: a) 60 patients who underwent OPCAB and b) another 60 patients who underwent conventional CABG between January 2013 and December 2015. Pre-operative, peroperative and early post-operative variables were recorded, compiled and compared. Results: All risk factors and co-morbidities were homogenously distributed between the two groups. Majority of the patients had triple vessel disease. Nearly three-quarter (73.3%) of patients in OPCAB group and 80% in CCAB group received 3 grafts (p=0.470). The mean total operative time (268.5 ± 33.5vs. 296.3 ± 34.8minutes, p < 0.001), intubation times(8.6±0.3 vs. 12.3±0.5 hours, p<0.001), blood losses (377.8378 ± 45 ml vs. 602 ± 60 ml, p < 0.001); requirements for blood and blood products (689.7±21.1 vs. 1199.3±34.5ml, p < 0.0010),intensive care unit stays (31.7±0.9 hours versus 41.6±1.5 hours; p<0.001) and hospital stays (8.2 ± 0.2days vs.10.3 ± 0.3days, p < 0.001)were all significantly lower in the OPCAB group. Conclusion: OPCAB is a safe and effective operative revascularization procedure for patients with multivessel coronary artery disease and left ventricular dysfunction and is associated with reduced morbidity. However, a larger and omized trial with long-term followup may show the real benefits of OPCAB. Bangladesh Heart Journal 2020; 35(1) : 20-27


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