The diagnosis and assessment of an adult with anomalous origin of the left coronary artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery (the Bland-White-Garland syndrome) is a rare congenital cardiac defect which may present for the first time in adulthood. The recognition of this disorder, which produces widespread left ventricular ischaemia and the risk of sudden death, myocardial infarction and congestive heart failure, is important as the prognosis can be improved by surgical intervention. Thallium-201 scintigraphy has a useful role in demonstrating the widespread nature of the ischaemia of the left ventricle, whilst the diagnosis is confirmed at cardiac catheterisation.     

Sudden infant death with anomalous origin of the left coronary artery from the pulmonary artery

Autopsy of a 2-month-old boy revealed an anomalous origin of the left coronary artery from the pulmonary trunk. The endomyocardial fibrosis associated with myocardial ischemia was noted in the left ventricular wall of the enlarged heart. Death was attributed to silent heart failure due to the anomalous left coronary artery. Significant clinical symptoms are generally observed in patients with such anomalies, however, they might cause sudden death in childhood.     

Physiologic assessment of coronary artery fistula

Coronary artery fistula is an uncommon clinical entity. The most common coronary artery fistula is from the right coronary artery to the right side of the heart, and it is less frequent to the pulmonary artery. The effect of a coronary artery fistula may be physiologically significant because of the steal phenomenon resulting in coronary ischemia. Based on published reports, it is recommended that patients with congenital coronary artery fistulas be considered candidates for elective surgical correction to prevent complications including development of congestive heart failure, angina, subacute bacterial endocarditis, myocardial infarction, and coronary aneurysm formation with rupture or embolization. A patient is presented in whom treadmill-exercise thallium imaging was effective in determining the degree of coronary steal from a coronary artery fistula, leading to successful corrective surgery.     

Coronary artery to pulmonary artery collaterals in nonspecific aortoarteritis involving the pulmonary arteries

We report a patient with nonspecific aortoarteritis (Takayasu's disease) in whom occlusion of the right upper lobe pulmonary artery was associated with collateral flow from the left circumflex coronary artery. Coronary to pulmonary artery collaterals are rare in this disease but have important clinical implication because of their ability to produce coronary steal and myocardial ischemia. Awareness of these pathways is essential for their detection in patients with angina-like symptoms and for differentiation of myocardial ischemia due to direct coronary artery involvement by this disease.     

负荷心肌灌注显像与双源CT冠脉成像诊断冠心病的对比研究

目的:通过对心肌灌注显像(MPI)与心脏双源CT(DSCT)检查结果进行对比分析,探讨两者对冠心病的临床诊断价值.方法:对38例拟诊为冠心病的患者行MPI及心脏DSCT检查;分别对MPI图像及DSCT图像进行分析处理,其中29例经冠状动脉造影(CAG)证实为冠心病.结果:DSCT显示有29例冠脉狭窄程度＞50％,其中大...     

Use of radionuclide measurements of left ventricular function for prognosis in patients with coronary artery disease

The major clinical challenge today in the management of patients with stable coronary artery disease is identification of those patients in whom myocardial revascularization would improve or prolong life. Despite widespread use over the past decade, indications for coronary artery bypass grafting remain controversial. A definite need exists for objective measures of the magnitude of myocardial ischemia before operation and for simple assessment of the hemodynamic effects of operation. The close link between myocardial ischemia and dysfunction suggests that measurement of left ventricular function during exercise can be used to assess myocardial ischemia in individual patients. In large patient populations with coronary artery disease (CAD), a relationship has been documented between the anatomic extent of disease and the magnitude of functional alteration. However, individual variation occurs with some patients with single-vessel stenosis demonstrating greater functional impairment than other patients with involvement of three vessels. The hypothesis that patients with the greatest magnitude of exercise-induced left ventricular dysfunction would profit most from surgery was examined in 857 patients studied by radionuclide angiocardiography and coronary arteriography. These patients were followed for survival and pain relief for up to 4 years after institution of medical or surgical therapy. Patients who demonstrated the greatest amount of exercise-induced left ventricular dysfunction had the most favorable outcome to myocardial revascularization by operation as judged by survival and relief. Successful myocardial revascularization commonly caused no change in resting left ventricular function. However, most patients who underwent myocardial revascularization demonstrated a reversal of left ventricular dysfunction during exercise. Therefore, radionuclide angiocardiography during rest and exercise provides useful assessment of patients before and after coronary artery bypass grafting.     

Isotopic findings in anomalous origin of the left coronary artery from the pulmonary artery: report of an adult case

Anomalous origin of the left coronary artery from the main pulmonary trunk results in myocardial ischemia or infarction, and may be a cause of death in the first months of life. Some patients, however, develop satisfactory coronary collateral circulation and remain asymptomatic into adulthood. In these patients, myocardial perfusion and left ventricular function are not well understood. We report the case of a 17-yr-old female patient, suffering from anomalous origin of the left coronary artery from the main pulmonary trunk, who underwent reimplantation of the left coronary artery to the aorta. The preoperative permanent 201Tl defect of the left antero-lateral ventricular wall and the abnormal regional wall motion induced by stress exercise testing were fully reversed after the operation.     

Anomalous origin of the right coronary artery from the pulmonary artery: An autopsied sudden death case with severe atherosclerotic disease of the left coronary artery

Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare anomaly. It may contribute to myocardial ischemia or sudden death, although the lesion is usually asymptomatic. We report a sudden death case of a 58-year-old man with ARCAPA coexisting with severe atherosclerotic coronary artery disease. He had been healthy until he complained of chest pain, several days before death, despite the discovery of heart murmur in childhood and suspicion of valvular heart disease. The autopsy revealed not only typical findings of the right coronary anomaly with well-developed collateral circulations but also severe atherosclerotic lesions of the left coronary artery, and ischemic change of the myocardium in the left and right coronary arterial perfusion territory. In addition to the “coronary steal” phenomenon primarily caused by ARCAPA, the reduced flow of both coronary arteries and further increase of “coronary steal” due to atherosclerotic obstructive coronary disease might have contributed to the patient’s death.     

Dobutamine stress myocardial perfusion imaging in coronary artery disease.

The accurate noninvasive diagnosis and functional evaluation of coronary artery disease is an important step in selecting the appropriate management strategy. Dobutamine stress myocardial perfusion imaging is an alternative to exercise in patients with limited exercise capacity. In many centers, the test is performed on patients who have a contraindication for vasodilator stress testing. Recent studies have shown hyperemia induced by the standard dobutamine-atropine stress test is not less than hyperemia induced by dipyridamole. The feasibility of the test is 90% and is often higher in patients without beta-blocker therapy. The safety of the test has been well studied and was also demonstrated in specific patients groups, such as patients with left ventricular dysfunction, the elderly, and heart transplant recipients. The diagnostic accuracy has been demonstrated in patients with and without myocardial infarction and in specific groups such as those with hypertension, left ventricular hypertrophy, and heart transplant recipients and after revascularization. The technique has a high sensitivity for prediction of functional recovery in patients with myocardial dysfunction referred for revascularization. The presence and severity of myocardial perfusion abnormalities assessed by this method are powerful predictors of cardiac events, incremental to clinical data. This article describes the methodology, safety, feasibility, diagnostic accuracy, and prognostic value of dobutamine stress myocardial perfusion imaging in patients with known or suspected coronary artery disease, with additional considerations for the application of the test in specific patient groups.     

联合冠状动脉旁路移植术和其他心脏手术

目的　回顾分析联合冠状动脉旁路移植术和其他心脏手术的临床效果。方法　 134例患者中 ,75 .4 %例有Ⅲ～Ⅳ级心绞痛 (CCSS) ,5 1.2 %有个至少 1次心肌梗死史。冠状动脉造影示梗阻性病变在左主干 (LM) 34例次、左前降支 (LAD) 130例次、对角支 91例次、回旋支 84例次、右冠状动脉 80例次。伴有冠状动脉弥漫性病变 4 1例。左室射血分数 (LVEF) 18%～ 6 9% ,4 5 %～ 30 % 5 0例 ,<30 % 13例 ;伴左心室壁瘤 5 6例 ,同时伴瓣膜功能不全 4 2例。全部患者均在体外循环下行冠状动脉旁路移植术 ,对 36例伴有冠状动脉弥漫性病变者同期激光心肌打孔 (TMLR) ,同期左心室壁瘤切除三明治式缝合11例 ,巨大室壁瘤心内补片左室成形 4 5例 ;同期心脏瓣膜手术 4 2例 (其中 4例患者同时心脏室壁瘤切除心内补片成型 )。结果　人均旁路 2 .4 6支 ,6例患者需IABP辅助 11～ 5 4h ;2次开胸止血 6例 ;并发室上速和房颤 2 8例 ,室性心律失常 10例 ,2例电转复 ,余均药物控制 ;全组手术死亡 3例 ,死亡原因分别为严重低心排综合征 (2例 )和多脏器功能衰竭。 131例患者手术后心绞痛等症状均缓解 ,心脏功能明显改善。随访 4～ 6 0个月 (平均 12 .7个月 ) ,随访率 89% ,6 5例已恢复全日工作。结论　与单纯冠状动脉旁路多植术相比 ,联合冠     

Accuracy of 99mTc-tetrofosmin myocardial tomography in the evaluation of coronary artery disease

BACKGROUND: Technetium 99m-labeled myocardial agents have been proposed as an alternative to thallium 201. The aim of this study was to assess retrospectively the accuracy of exercise myocardial 99mTc-tetrofosmin scintigraphy with tomographic imaging (SPECT) in a large group population in the evaluation of coronary artery disease. Furthermore we evaluated the relation between the severity of scintigraphic impaired myocardial perfusion and the angiographic coronary artery stenoses in patients without myocardial infarction and with stenosis localized exclusively in the proximal segment of the 3 main coronary arteries. METHODS AND RESULTS: The study group consisted of 235 consecutive patients, 204 (87%) of whom were men, with a mean age of 57+/-10 years, and with suspected or known coronary artery disease, who underwent 99mTc-tetrofosmin SPECT and coronary angiography. Furthermore, 61 patients in a low-likelihood group for coronary artery disease were also studied. Significant disease was defined by > or = 50% luminal coronary artery stenosis in > or = 1 native coronary artery or major branch or in a saphenous vein graft or arterial mammary graft. The overall sensitivity was 95%, specificity was 76%, and predictive accuracy was 95%. The normalcy rate for the low-likelihood group was 93%. Sensitivity was 71% for the left anterior descending artery, 61% for the left circumflex artery, and 73% for the right coronary artery. Specificity was 94% for the left anterior descending artery, 96% for the left circumflex artery, and 91% for the right coronary artery. Predictive accuracy was 79% for the left anterior descending artery, 78% for the left circumflex artery, and 81 % for the right coronary artery. In patients without myocardial infarction linear regression analysis between scintigraphy and angiography showed a significant correlation in patients with severe proximal coronary artery stenosis (r = 0.53, P < .002), but not in those with moderate proximal stenosis (r = 0.31, P = NS). CONCLUSIONS: This study shows that 99mTc-tetrofosmin SPECT is accurate in the detection of coronary artery disease. The relation of the severity of scintigraphic impaired myocardial perfusion and angiographic coronary artery stenosis, however, may differ significantly in patients with proximal stenosis of different severity.     

Specific perfusion pattern in stress 201Tl myocardial scintigraphy of left main coronary artery disease

The usefulness of stress 201Tl myocardial scintigraphy for identifying left main coronary artery disease was evaluated with data from 23 patients with 50% or more narrowing of the left main coronary artery and 56 patients with 75% or more narrowing of the major coronary arteries but without left main coronary artery involvement (no left main coronary artery disease). Quantitative evaluation of stress perfusion scintigrams in all five patients with narrowing of the left main coronary artery of 90% or more showed a characteristic perfusion pattern (left main pattern) of extensive homogeneous defect over the whole anterolateral segment and simultaneous defects in all radii of the high anteroseptal and high posterolateral segments. On the other hand, such a perfusion pattern was noted in only 1 of 18 patients with less than 90% stenosis of the left main coronary artery and in only 1 of 56 patients with no left main coronary artery disease.     

冠状动脉瘘的双源CT诊断

目的:探讨双源CT(DSCT)冠状动脉成像对冠状动脉瘘的诊断价值.方法:对1000例患者行DSCT冠状动脉增强扫描,分析其断层图像,结合最大密度投影(MIP)、曲面重组(CPR)、多平面重组(MPR)和容积再现(VR)等方法观察冠状动脉的走行及心内外机构.结果:11例患者有冠状动脉瘘,其中右冠状动脉主干-肺动脉瘘5例,...     

Specific perfusion pattern in stress 201Tl myocardial scintigraphy of left main coronary artery disease

The usefulness of stress ²⁰¹Tl myocardial scintigraphy for identifying left main coronary artery disease was evaluated with data from 23 patients with 50% or more narrowing of the left main coronary artery and 56 patients with 75% or more narrowing of the major coronary arteries but without left main coronary artery involvement (no left main coronary artery disease). Quantitiative evaluation of stress perfusion scintigrams in all five patients with narrowing of the left main coronary artery of 99% or more showed a characteristic perfusion pattern (left main pattern) of extensive homogeneous defect over the whole anterolateral segment and simultaneous defects in all radii of the high anteroseptal and high posterolateral segments. On the other hand, such a perfusion pattern was noted in only 1 of 18 patients with less than 90% stenosis of the left main coronary artery and in only 1 of 56 patients with no left main coronary artery disease     

先天性心脏病冠状动脉瘘的介入治疗

目的：评价经皮穿刺栓塞术治疗冠状动脉瘘的疗效。材料与方法：１９９５年６月至１９９６年８月，对４名冠状动脉瘘患儿实施了经皮穿刺冠状动脉瘘栓塞术，采用８ｍｍ、１０ｍｍ、１２ｍｍ及１５ｍｍ直径（Ｃｏｏｋ公司产品）弹簧栓子，手术前、后进行左、右心导管检查及冠状动脉造影，以进一步诊断和评价治疗效果。结果：４例操作技术全部成功，通过手术前后心导管检查及选择性冠状动脉造影，证明栓塞效果可靠，术后心电图无异常，其中３例随诊半年，１例随诊２个月，效果良好。结论：经皮穿刺栓塞术是治疗冠状动脉瘘的有效方法。     

Diagnosis of coronary artery disease with dobutamine-stress MRI

Dobutamine-stress cardiovascular magnetic resonance (CMR) is a new diagnostic tool for the non-invasive detection of coronary artery disease. Technological advances in CMR have evolved this technique to an adequate alternative to the standard cardiac stress tests. Its high reproducibility and excellent image quality of the anatomical features of the left ventricle and left ventricular function at rest and during stress make it an ideal technique for the comprehensive evaluation of patients with suspected coronary artery disease. Besides its ability to detect myocardial ischemia, CMR has proved to be diagnostic for myocardial viability as well. A recent technical refinement in CMR using myocardial tagging has improved the diagnostic accuracy for myocardial ischemia even further. Dobutamine-stress CMR is used to identify wall motion abnormalities of the left ventricle in patients with proven or suspected coronary artery disease [1-4]. Dobutamine-stress CMR has emerged as a highly accurate and safe diagnostic modality [1-4]. Recently, the use of high-dose dobutamine CMR in combination with the myocardial tagging technique has been reported, with excellent diagnostic results. The use of this new technique and the clinical applications are discussed.     

Role of nuclear cardiology for determining management of patients with stable coronary artery disease

Both radionuclide angiography and myocardial perfusion imaging provide important insights that determine the management of patients with stable coronary artery disease. Both nuclear cardiology procedures have clearly demonstrated use in the noninvasvie identification of severe (left main or three-vessel) coronary artery disease and the noninvasive assessment of prognosis and thereby determine which patients should be sent to coronary angiography. Both radionuclide angiography and myocardial perfusion imaging provide prognostic information that is independent of resting left ventricular function and coronary anatomy and thereby influence the decision regarding which patients should be sent to coronary revascularization. This review considers the evidence supporting the uses of these nuclear cardiology procedures and provides suggestions regarding their cost-effective application.     

Anomalous origin of left coronary artery from pulmonary artery (ALCAPA): A case report

Anomalous origin of left coronary artery from pulmonary artery (ALCAPA) also known as Bland-White-Garland Syndrome is a rare anomaly of coronary arteries comprising of 0.25%-0.5% of all congenital heart defects with a prevalence of 1 in every 300,000 live births. Its clinical significance lies in the possibility of resultant coronary steal phenomenon with a left-to-right shunt causing aberrant left ventricular perfusion which may ultimately lead to myocardial ischemia and infarction in children having the abnormality. ALCAPA may manifest as an isolated defect but in 5% of cases it may be associated with other cardiac anomalies such as atrial septal defect, ventricular septal defect, and aortic coarctation. We present a case of 7 years female with ALCAPA with collaterals between RCA and LCA and additional findings of juxtaposition of left atrial appendage. Juxtaposition of atrial appendage is associated with some major congenital heart diseases, transposition of great vessels being the common one. In our case, however, juxtaposition of left atrial appendage is associated with ALCAPA. Surgery is the definite treatment modality for ALCAPA available till date. Early diagnosis of ALCAPA with the help of multislice CT angiography is always good for the patient to prevent the possible grave consequences.     

Use of the internal thoracic artery in patients with stenosis of the left main branch of the coronary artery

Internal thoracic artery (ITA) is the graft of choice in myocardial revascularization. However, superiority of the ITA graft in patients (pts) with left main coronary artery disease is still a matter of debate. PATIENTS: In the period from November 1986 through February 1999, ITA graft was used for myocardial revascularization in 2860 pts. Stenosis of the left main stem was present in 229 patients (8.0%); there were 39 women (17.0%) and 26 diabetics (11.4%). Severely depressed left ventricular function was present in 32 pts (14.0%), while the average age was 56.4 +/- 7.2 years. RESULTS: Operative mortality was 1.3% and postoperative morbidity was 5.2%. Average postoperative hospital stay was 7.9 days (6-29). There were no differences in analyzed parameters between patients who received ITA graft and similar group of 240 patients who received vein grafts only, during the same period of time. All 16 patients operated on in the period 1986-1992 (6-12 years follow-up) are alive. CONCLUSION: It is safe to use ITA graft for myocardial revascularization in patients with left main coronary artery stenosis. Early operative results are favorable and these patients should not be denied the benefit of the ITA graft, since long-term results are proved to be good as well.     

左冠状动脉瘘的影像诊断与介入治疗

目的 :探讨左冠状动脉瘘的影像诊断和介入治疗价值。方法 :2 7例均行X线平片和彩色多普勒检查 ;行升主动脉造影者 4例 ,选择性冠状动脉造影者 2 3例 ;3例行介入治疗 ,16例于体外循环下行冠状动脉瘘修复术。结果 :5例X线平片和 15例彩色多普勒检查基本上能提示诊断 ,血管造影均能明确诊断 ,血管造影显示左冠状动脉瘘通过左前降支和左回旋支形成较粗大的瘘口者 16例 (5 9.3% ) ,左冠状动脉细小分支瘘者 11例 (40 .7% ) ,其中发生于左冠状动脉前降支的细小分支者 6例、发生于左回旋支的细小分支者 4例、同时源于左前降支和左回旋支的细小分支者 1例。在这 2 7例中 ,瘘入右心室 13例 (48.2 % ) ,瘘入肺动脉者 7例 (2 5 .9% ) ,瘘入左心室 5例 (18.3% ) ,瘘入左房者 2例 (7.4 % )。 3例患者行介入治疗 ,经 6个月随访观察 ,未见异常改变。结论 :选择性冠状动脉造影是诊断冠状动脉瘘的最佳方法 ,在治疗方面目前除手术治疗外 ,介入治疗亦是一种较好的手段