Nonprogression of coronary artery atherosclerosis in homozygous familial hypercholesterolemia after 31 months of repetitive plasma exchange

The effects of plasma exchange performed every two weeks for 31 months in combination with diet and drug therapy were studied in a patient with receptor-defective homozygous familial hypercholesterolemia. Coronary angiography performed three years prior to commencing plasma exchange was compared to angiography 31 months after starting the program. Comparison of the angiograms taken six years apart showed no progression of coronary atheroma in the main left coronary artery in which a 30% narrowing was originally seen. An internal mammary artery-coronary artery bypass remained widely patent and showed no development of atherosclerosis. Plasma cholesterol levels were reduced 46% by plasma exchange, diet and drug compared to diet and drug alone. Achilles tendon xanthoma diminished significantly. It appears that plasma exchange combined with diet and drug therapy, while not producing regression of existing atheromatous lesions, does retard or prevent further progression.     

Left main coronary angioplasty in a 10-year-old boy with homozygous familial hypercholesterolemia

Homozygous familial hypercholesterolemia is a rare cause of premature coronary artery disease. A young boy with this disorder who underwent successful coronary angioplasty for left main stem stenosis is presented. © 1993 Wiley-Liss, Inc.     

Konno Ross procedure,coronary artery bypass graft and mitral valve replacement in a 12-year-old girl with homozygous familial hypercholesterolemia

BackgroundFamilial hypercholesterolemia (FH) is a genetic disorder caused by a mutation of the gene for the low density lipoprotein receptor. This mutation can lead to elevated plasma cholesterol levels and subsequently to premature coronary artery disease. Management of patients with FH is complicated and surgery is accompanied by high risk, even in skillful hands.Case presentationA 12-year-old female patient was referred to our department in January 2013 with chest pain and dyspnea. Her history showed that he had documented evidence of homozygous HF (HFH) since 2 years of age and that she underwent a Ross–Konno procedure for valvular aortic stenosis, 3 years ago. Electrocardiography showed ST depression in the inferolateral derivations and ST elevation in aVr. The echocardiography showed LV systolic dysfunction and important mitral regurgitation. Coronary angiography demonstrated stenosis in the distal part of the left main and severe three vessel coronary artery disease. The patient presented critical acute myocardial ischemia immediately after coronary angiography. She was referred for surgery. The left anterior descending artery was bypassed using saphenous vein and both right coronary artery and marginal artery using sequential saphenous vein. The mitral valve was replaced with mechanic prosthesis. The postoperative course was uneventful. She was prescribed atorvastatin accompanied by cholestyramine and diet modulation.ConclusionHFH patients are at increased risk of developing coronary artery disease and also sudden death unless the condition is recognized and treated promptly. Surgery remains the most effective means of prolonging the life of these patients.     

Percutaneous left main coronary artery stent for acute myocardial ischemia after repaired ALCAPA

Percutaneous coronary artery stent angioplasty is rare in the pediatric population but can be a life‐saving by rapidly reestablishing flow to an obstructed coronary artery. It is a technically challenging and high‐risk procedure in infants and further limited by the need for future surgical intervention. We report of an infant with anomalous left coronary artery from the pulmonary artery who underwent acutely successful surgical reimplantation of the left coronary artery onto the ascending aorta. One month later, she developed acute myocardial ischemia and emergent catheterization diagnosed near‐total occlusion of the left coronary artery. We implanted a 2.5 mm coronary stent in the left main coronary artery with reestablishment of flow. The patient's left ventricular systolic function recovered within 4 weeks and repeat angiography 3 months later showed complete normalization of the entire left coronary artery system. The patient weighed 3 kg and was < 6 weeks of age at the time of stent implantation which to our knowledge is the smallest and youngest reported patient to undergo coronary stent angioplasty. This case supports the feasibility of this procedure in infants as a temporizing solution to hemodynamic instability from myocardial ischemia due to coronary artery stenosis. The left ventricular systolic function remained normal at 7 months after stent placement and the patient was clinically well from a cardiac perspective. © 2014 Wiley Periodicals, Inc.     

Coronary stenting for failed angioplasty in acute myocardial infarction

Intracoronary stents were implanted in 15 patients after unsuccessful PTCA in the setting of acute myocardial infarction (AMI). The stented vessel was the left anterior descending (LAD) in 11 patients, the right coronary artery (RCA) in 3 patients, and a venous bypass graft to the LAD in a single patient. A total of 16 stents were implanted (15 Palmaz-Schatz, Johnson and Johnson; and 1 Wiktor, Medtronic). Follow-up: 1 patient died 10 days after stent implantation as a result of renal failure and cardiogenic shock. Subacute thrombosis occurred in 2 patients, 5 and 15 days after stent implantation; both underwent successful emergency coronary artery bypass grafting (CABG). The remaining 12 patients were free from major ischemic events (death, AMI, and further revascularization) after a mean follow-up of 18.7 ± 4.1 months. We conclude that the long-term results of intracoronary stenting in AMI after failed PTCA are favourable. © 1996 Wiley-Liss, Inc.     

Percutaneous coronary intervention for acute myocardial infarction due to unprotected left main coronary artery occlusion

Acute myocardial infarction (AMI) due to unprotected left main coronary artery (ULMCA) occlusion is an uncommon clinical entity, but often leads to severe clinical deterioration, with devastating sequalae including fatal arrhythmias, abrupt and severe circulatory failure, and sudden cardiac death. Recent guidelines have promoted treatment with percutaneous coronary intervention (PCI) as a class IIa recommendation alongside coronary artery bypass grafting (CABG), but the data are still unclear regarding optimal revascularization strategy for patients with ST‐segment elevation myocardial infarction (STEMI) and non‐STEMI (NSTEMI) with ULMCA culprit. PCI has the advantages of offering rapid reperfusion to critically ill patients, often those with prohibitive risk for surgical revascularization, with acceptable short‐ and long‐term outcomes. Recent studies demonstrate that PCI of the ULMCA is a viable alternative to CABG for appropriate patient populations, including those with ULMCA occlusion and those in cardiogenic shock, Thrombolysis In Myocardial Infarction (TIMI) flow grade 3, and significant comorbidities. A randomized trial comparing PCI with CABG is needed to clarify the ideal revascularization strategy, though the clinical picture of these critically ill patients may preclude such studies. © 2014 Wiley Periodicals, Inc.     

补救性经皮冠状动脉腔内成形术治疗急性心肌梗塞

目的探讨补救性经皮冠状动脉腔内成形术（ＰＴＣＡ）在治疗急性心肌梗塞（ＡＭＩ）中的作用。方法对溶栓治疗失败的３６例患者进行补救性ＰＴＣＡ治疗。患者心功能Ｋｉｌｐ分级：Ⅲ级和Ⅳ级４例，Ⅱ级和Ⅰ级３２例。冠状动脉造影显示梗塞相关动脉：前降支１７例，右冠状动脉１４例，回旋支４例，中间动脉１例。ＰＴＣＡ前ＴＩＭＩⅠ级和Ⅰ～Ⅱ级血流各２例，余３２例均为ＴＩＭＩ０级。３６例均进行ＰＴＣＡ治疗，其中１３例患者置入了支架。结果术中除３例失败外，３１例患者病变血管血流达到ＴＩＭＩⅢ级，２例ＴＩＭＩⅡⅢ级，残余狭窄≤５０％，成功率为９１．７％。院内并发症：１例在ＰＴＣＡ成功后当天因顽固性休克和心室纤颤死亡；１例于第３天死于心脏破裂，住院病死率为５．６％。１４例患者在术后１～２个月内复查冠状动脉造影，２例发生再狭窄。结论ＡＭＩ患者在溶栓治疗失败后，在有条件的医院可施行补救性ＰＴＣＡ治疗，成功率高，对改善患者的近期和远期预后可能有利     

Results of serial coronary angiography in patients with homozygous familial hypercholesterolaemia

We studied the progression of coronary angiographic findingsin a series of 10 patients with homozygous familial hypercholesterolaemia.All patients had medical treatment; six had additional surgicaltreatment. They were followed over a period of 2–10 years(mean follow-up five years). Three types of evolution were observed:(a) normal coronary angiography with no development of any lesionover a period of eight years in two young patients; (b) regressionof proximal coronary stenoses in three patients, and (c) progressionof coronary angiographic status in five patients. A differentevolution of proximal and distal segment lesions was observed;while more than two-thirds of proximal segments stenoses improvedand no proximal lesion increased with a 45% decrease in plasmacholesterol level, no improvement was obtained at the distallevel. Distal lesions tended to increase and new lesions appearedin spite of a major plasma cholesterol level reduction. Whenearly treatment results in a cholesterol plasma level decreaseof more than 45% and a level of about 4 g l^–1 over a verylong period of time, the coronary prognosis may be good.     

Comparative trial of stent-like balloon angioplasty versus coronary stenting for acute myocardial infarction.

Primary PTCA has been shown to be superior to any thrombolytic regimen and offers higher reperfusion rates and better coronary flow grades. Its limitations include recurrent ischemia (10%-15%), infarct-related artery reocclusion (5%-10%), angiographic restenosis (35%-50%), and need to perform repeat PTCA or CABG at 6-month follow-up (20%). Thus, the current role of coronary stenting for acute myocardial infarction (AMI) is very promising. From December 1995 through January 1997, 335 patients underwent primary angioplasty during the first 12 hr from symptom onset at our institution. We performed a retrospective study comparing the in-hospital and 6-month follow-up outcome of 61 patients who underwent coronary stenting (stent group) against 61 patients with optimal (residual lesion stenosis < 30%) balloon-only primary angioplasty (stent-like group). Patients were routinely treated with aspirin, and ticlopidine was given only to the stent group. In-hospital major adverse cardiac events (MACE) rate was 11.5% without statistical differences between the groups. Cardiac death rate was similar in both groups (4.9 vs. 6.6%; P = 1.0) and only two (3.3%) patients from the stent group and none from the PTCA group had nonfatal myocardial reinfarction. At 6-month follow-up, the rate of recurrent angina was higher in the stent-like group (30.9 vs. 7.1%; P < 0.001). Multivariate analysis showed that only stenting of the infarct-related artery was a borderline independent predictor for MACE (OR = 0; 95% CI = 0-1; P = 0.057). Primary stenting for AMI reduces the rate of recurrent angina or symptoms and MACE at 6-month follow-up.     

Predictors of short term clinical and angiographic outcome after coronary angioplasty for acute myocardial infarction

Coronary angioplasty is an effective method to achieve myocardial reperfusion in acute myocardial infarction (AMI). We reviewed our experience in 132 patients (pts) who underwent percutaneous transluminal coronary angioplasty (PTCA) of a totally occluded infarct-related artery (IRA) within 24 h after the onset of symptoms (mean delay 10±7 h), in order to identify the predictors of primary success and of major complications. PTCA was successfully performed in 113 patients (86%). Failure without complications occurred in 12 patients (8.4%); untoward events (death and emergency CABG) occurred in seven patients (5.3%). Pts in the failure group were more likely to have cardiogenic shock (53 vs. 8.8%, P<.0005), longer time to reperfusion (15±6 vs. 9±6 h, P<.0005), lower ejection fraction (EF) (42±16 vs. 54±12%, P<.0005), multivessel disease (74 vs. 43%, P<.03), and a smaller IRA diameter (2.8±0.6 vs. 3.1± 0.6 mm, P<.03). Sex, age, previous bypass surgery, previous thrombolytic treatment, IRA, and infarct location were similar in both groups. Absence of cardiogenic shock (P<.0001), decreasing time to reperfusion (P<.005) and increasing EF (P<.02) were independent predictors of successful PTCA. Presence of cardiogenic shock (P<.0001) and decreasing EF (<.05) were independent predictors of untoward events. Repeat angiography was performed 24 h after the procedure in the success group. Angiographic deterioration (stenosis ? 50% and/or TIMI flow grade ? 1) was present in 18 pts (16%), among whose 5 pts (4.4%) had re-occlusion of the IRA. Pts with early angiographic deterioration were more likely to have a lower IRA diameter (2.8±0.5 vs. 3.1±0.6 mm, P<.02). Conclusion: Emergency PTCA is an effective method for establishing reperfusion in AMI. Pts with high-risk baseline characteristics show the highest rate of untoward events, but are the most likely to benefit from aggressive reperfusion therapy. © 1995 Wiley-Liss, inc.     

冠脉造影中发生急性缺血事件的左主干病变的分析

目的分析冠状动脉造影中出现急性心肌缺血事件的左主干病变患者的临床和冠脉病变特点。方法121例患者经冠脉造影证实左主干管径狭窄≥50%。根据术中是否出现急性心肌缺血事件(心绞痛,心肌梗死,血压明显下降,严重心律失常如心动过缓、Ⅱ度或Ⅲ度以上房室传导阻滞、室性心动过速、室颤等)分为事件组(36例)和无事件组(85例)。结果事件组吸烟者比例(69.4%)高于无事件组(49.4%,P<0.05),心绞痛危险分层高,程度重,住院期间出现心血管并发症者明显多于无事件组(44.4%vs10.6%,P<0.005),死亡率高(19.4%vs1.7%,P<0.005)。事件组左主干开口病变多见,右冠脉向左冠脉形成的侧支循环少(11.1%vs28.2%,P<0.05),左主干狭窄程度高于无事件组(秩和检验,P<0.05)。结论冠脉造影中出现心肌缺血并发症的左主干病变患者是一组高危人群,住院并发症多,死亡率高,左主干狭窄程度重,侧支循环少,应及早行冠状动脉血管移植术(CABG)。     

Primary percutaneous coronary intervention for acute myocardial infarction caused by unprotected left main stem thrombosis

Background: There is a paucity of data on outcomes in patients undergoing percutaneous coronary intervention (PCI) for acute myocardial infarction (AMI) caused by left main stem (LMS) thrombosis. Objectives: We sought to determine (i) the clinical features, (ii) correlates of early mortality, and (iii) long‐term outcomes in contemporary patients undergoing primary PCI for unprotected LMS thrombosis. Methods: From 1,115 consecutive primary PCI cases at two tertiary referral centers between January 2004 and September 2007, 28 cases (2.5%) with unprotected LMS culprit lesions were identified. Data were obtained from review of institutional databases, folder audit, telephone survey of patients, and independent review of angiograms. Results: The mean age of patients was 68 ± 14 years. Males comprised 76%, and 21% had diabetes. Significant morbidity was noted at presentation: shock in 18 (62%), pulmonary oedema in 15 (52%), and cardiac arrest in 10 (35%) patients, respectively. Lesion location was ostial in 7 (25%), body in 8 (29%), and distal in 13 (46%) patients, respectively. Angiographic success was achieved in 24 patients (83%). Stents were deployed in 27 patients (96%); drug‐eluting stents in 11 patients (39%). No patient required in‐hospital CABG. Cumulative in‐hospital mortality was 36%. Univariate predictors of in‐hospital mortality included shock, preceding cardiac arrest, and angiographic failure (all P < 0.05). At a mean follow‐up of 26 ± 12 months in hospital survivors, there were two TVR (elective CABGs), one death, and no reinfarctions. Conclusion: We report a lower than previously reported in‐hospital mortality of 36% in contemporary patients undergoing primary PCI for unprotected LMS thrombosis. Long‐term outcomes in hospital survivors appear favorable. © 2009 Wiley‐Liss, Inc.     

Bail-Out stenting for left main coronary artery occlusion following diagnostic angiography

We report a case of acute left main coronary artery occlusion treated with stenting of the left main artery. The patient had a severe ostial left main stenosis and after diagnostic coronary angiography developed a total occlusion of the left main artery with life threatening hemodynamic consequences. Stenting of the unprotected left main coronary artery was successfully performed as a salvage procedure.     

Survival rate after acute myocardial infarction in patients treated with percutaneous coronary intervention within the left main coronary artery according to time of admission

The relationship regarding time of percutaneous coronary intervention (PCI) and clinical outcomes in patients with acute myocardial infarction (AMI) treated within the left main coronary artery (LMCA) is less investigated compared to the overall group of patients with AMI.Therefore, we aimed to assess the relationship between time of PCI (day- vs night-time) and overall mortality rate in patients treated due to AMI within the LMCA.This cross-sectional study included 443,805 AMI patients hospitalized between 2006 and 2018 enrolled in the Polish Registry of Acute Coronary Syndromes. We extracted 5,404 patients treated within the LMCA. The number of patients were treated during daytime hours (7:00 am–10:59 pm) was 2809 while 473 patients underwent treatment during night-time hours (11:00 pm–6:59 am). Differences in cardiac mortality rates between night- and day-hours among patients treated with PCI during the follow-up period were assessed via the Kaplan–Meier method.The 30-day (20.3% vs 14.9%, P = .003) and 12-month (31.7% vs 26.2%, P = .001) overall mortality rates were significantly greater among patients treated during night-time, which was confirmed by comparison using Kaplan–Maier survival curves (P = .001). The time of PCI was not found among predictors of survival in multiple regression analysis (hazard ratio: 1.22; 95% confidence interval: 0.96-1.55, P = .099).Patients treated during night-time in comparison to the day-time are related to higher in-hospital, 30-day and 12-month mortality. This is probably largely a consequence that the night-time, in comparison to the day-time, of treatment of patients with AMI with PCI within the LMCA is and indicator of higher comorbidity and clinical acuity of patients undergoing therapy. Therefore, the night-time was not found to be an independent predictor of greater mortality rate during the 12-months follow-up period.     

Percutaneous coronary intervention with rotational atherectomy for severely calcified unprotected left main: immediate and two-years follow-up results

Introduction : According to current practice guidelines, coronary bypass surgery is the standard care option for unprotected left main (ULM) stenosis. However, a group of high surgical risk patients who are not eligible for coronary bypass surgery could benefit from percutaneous coronary intervention (PCI). Severe calcification hampers PCI in this setting, and rotational atherectomy (RA) could be of great help. Methods : We retrospectively analyzed a cohort of 40 patients with severely calcified ULM stenosis, not eligible for coronary artery bypass grafting, who underwent RA in our center. Results : Forty patients (mean age, 73 ± 8 years; 26 males) with ULM stenosis were treated with RA. Twenty‐seven (67%) had distal stenosis. The mean number of treated vessels was 2.38 ± 0.74, with a mean lesion length of 30 ± 18 mm. Abciximab was used in 12 (30%) cases and an intra‐aortic balloon pump in three cases. One patient died during the procedure and another two patients died in the first 24 hr after the procedure. The major events registered after the procedure included one significant branch loss, and two minor and one major case of bleeding (the latter requiring transfusion) at the puncture point. After a median of 24.7 (IQR 19.6–34.3) months of follow‐up, 12 cardiac deaths were recorded. Survival free of cardiac death was 71 ± 7% and clinical guided target vessel revascularization 19.3 ± 7% at 2 years. Conclusion : In a group of high surgical risk patients, RA on severely calcified left main stenosis is feasible and, in spite of high mortality rates, could pose the only possible effective treatment. © 2012 Wiley Periodicals, Inc.     

左主干急性闭塞的急诊PCI分析

目的探讨左主干病变导致的急性心肌梗死与心源性休克的关系以及急诊经皮冠状动脉介入对其的疗效。方法1999年9月至2005年6月连续就诊于中国医科大学附属盛京医院心内科并且行急诊冠脉造影的急性心肌梗死患者752例,发现因左主干急性闭塞导致血管床灌注评分(TIMI)血流在2级或2级以下者16例(2.12%),所有患者行急诊经皮冠状动脉介入(PCI)治疗,观察住院期间病死率。结果16例患者中,9例(56.25%)住院期间死亡,7例(43.75%)出院。死亡组再灌注成功率42.86%,生存组为100%,两组间比较差异有显著性意义(P=0.029)。未成功再灌注的5例均由无复流引起,其中3例可见肉眼血栓,5例患者均死亡。16例患者中有12例发生心源性休克,发生率为75.00%,死亡组发生率100%,生存组42.85%,两组间比较差异有显著性意义(P=0.019)。心源性休克患者住院病死率为75.00%高于无休克者,死亡风险是无休克者的4倍[比值比(OR)=4.0,95%可信区间1.50～10.66,P=0.019]。结论左主干闭塞导致的急性心肌梗死患者出现心源性休克的发生率较高,且即使成功地施行了PCI治疗,也有较高的病死率。不过,急诊PCI在临床实践仍然是有效可行的治疗手段,可以有效地挽救部分生命。     

Stenting of a left main coronary artery compressed by a dilated main pulmonary artery

Left main coronary artery (LMCA) disease caused by external compression by a dilated main pulmonary artery (MPA) is an uncommon clinical entity but is one of the reversible causes of chest pain in patients with pulmonary hypertension. Traditionally, treatment of LMCA disease involves coronary artery bypass graft surgery. However, for LMCA compression by a dilated MPA, coronary angioplasty with stenting has recently been reported to have good outcomes and might be more suitable in some patients with high risk associated with surgery. Herein, we describe a 54‐year‐old man with pulmonary arterial hypertension and external compression of the LMCA by the dilated main pulmonary artery that was treated with angiographic and intravascular ultrasound‐guided coronary angioplasty and stenting. Also we briefly review current literatures about LMCA compression by a dilated MPA. © 2013 Wiley Periodicals, Inc.     

左主干急性闭塞的急诊PCI分析

目的探讨左主干病变导致的急性心肌梗死与心源性休克的关系以及急诊经皮冠状动脉介入对其的疗效。方法1999年9月至2005年6月连续就诊于中国医科大学附属盛京医院心内科并且行急诊冠脉造影的急性心肌梗死患者752例，发现因左主干急性闭塞导致血管床灌注评分（TIMI）血流在2级或2级以下者16例（2．12％），所有患者行急诊经皮冠状动脉介入（PCI）治疗，观察住院期间病死率。结果16例患者中，9例（56.25％）住院期间死亡，7例（43．75％）出院。死亡组再灌注成功率42．86％。生存组为100％，两组间比较差异有显著性意义（P=0．029）。未成功再灌注的5例均由无复流引起，其中3例可见肉眼血栓，5例患者均死亡。16例患者中有12例发生心源性休克，发生率为75．00％，死亡组发生率100％，生存组42．85％，两组间比较差异有显著性意义（P=0．019）。心源性休克患者住院病死率为75．00％高于无休克者，死亡风险是无休克者的4倍[比值比（OR）=4．0，95％可信区间1．50—10．66，P=0．019]。结论左主干闭塞导致的急性心肌梗死患者出现心源性休克的发生率较高，且即使成功地施行了PCI治疗，也有较高的病死率。不过，急诊PCI在临床实践仍然是有效可行的治疗手段，可以有效地挽救部分生命。