Origin Of Left Main Coronary Artery Research Articles

Anomalous origin of the left main coronary artery from the right pulmonary artery: a case report

Anomalous origin of the left coronary from the right pulmonary artery (AOLCARPA) is an extremely rare anomaly, which has been reported in only a few patients worldwide [1] An 8-month-old male patient weighing 5.7 kg came to our hospital with chief complaints of tachypnea while feeding and failure to thrive. ECG of the patient showed sinus tachycardia. The patient had a heart rate of 140/min and saturation of 99% on room air. Chest radiograph showed cardiomegaly. Transthoracic echocardiography showed moderate mitral regurgitation, dilated left atrium and ventricle, severe left ventricular (LV) dysfunction, global LV hypokinesia with an LV ejection fraction of 15–20%. Flow pattern in the left anterior descending artery and the left circumflex artery was reversed and the right coronary artery (RCA) was dilated. The left main coronary artery (LMCA) origin was not clear. As the diagnosis was unclear, computed tomography coronary angiography (CTCA) was done to delineate the coronary anatomy, which showed an anomalous origin of LMCA from the right pulmonary artery (RPA) (Figs. 1 and 2Figs 1, 2). Blood investigations including complete blood count, and liver and kidney function tests were normal. Preoperative medication of the patient included digoxin, spironolactone, furosemide, and milrinone infusion. The patient was posted for corrective repair of anomalous origin of the left coronary artery from the RPA.Figure 1: Computed tomography coronary angiography showing left main coronary artery (LMCA) arising from the right pulmonary artery (RPA). PROX LAD, proximal left anterior descending artery.Figure 2: (a) Midesophageal (ME) ascending aortic short-axis (SAX) view showing retrograde flow from left main coronary artery (LMCA) to right pulmonary artery (RPA). (b) Midesophageal (ME) four-chamber view showing dilated left ventricle (LV) and mitral regurgitation jet. (c) Origin of LMCA from RPA. LPA, left pulmonary artery; Asc Aorta, ascending aorta.On the day of surgery, the patient was taken to the operating room. Preoperative inotropic support of intravenous infusion of milrinone 0.5 µg/kg/min was continued. After attaching a multiparameter monitor with a five-lead ECG, noninvasive blood pressure monitor, and a pulse oximeter, general anesthesia was induced with intravenous fentanyl 2 µg/kg, midazolam 0.1 mg/kg, vecuronium 0.1 mg/kg, and endotracheal intubation was done. Femoral artery was cannulated for invasive arterial blood pressure monitoring, and central venous catheter was inserted in the right internal jugular vein. Anesthesia was maintained with isoflurane and intermittent boluses of fentanyl and vecuronium. Intraoperative ventilation was instituted with the goals of avoiding hypocarbia, hyperoxia, and alkalosis. An FiO2 of 0.35 was kept with PaO2 targets of 80–100 mm Hg to maintain pulmonary vascular resistance End-tidal carbon dioxide (EtCO2) was maintained between 40 and 42 mm Hg. Pre-bypass transesophageal echocardiography (TOE) examination was carried out with a pediatric 9T probe (Vivid I, GE; Vingmed Ultrasound, Horten, Norway). ME ascending aorta short-axis view showed the origin of LMCA from RPA (Fig. 2a). Midesophageal four-chamber view showed dilated LV and moderate mitral regurgitation (Fig. 2b). Heparin 400 IU/kg was given, and cardiopulmonary bypass was instituted. Cardiopulmonary bypass was established using aortobicaval cannulation with looping of both pulmonary artery branches. Myocardial preservation was obtained with antegrade cold blood potassium cardioplegia into the aorta and main pulmonary artery. Under low pump flows, LMCA origin was identified originating from the RPA (Fig. 2c). Medial trap door was made in the aorta and LMCA was anastomosed to the aorta (coronary translocation). Posterior annuloplasty of the mitral valve was performed through the trans-septal approach. Weaning from the cardiopulmonary bypass was uneventful with minimal inotropic support (milrinone 0.5 µg/kg/min; Epinephrine 0.04 µg/kg/min). The chest was kept open with a sterile membrane cover electively and the patient was shifted to the pediatric ICU with stable hemodynamics. AOLCARPA is an extremely rare congenital coronary anomaly. Only 10–20% of neonates survive till adulthood, and it depends on the adequacy of LV perfusion through collaterals to LCA from RCA and the proportion of coronary steal from the LCA to pulmonary artery [2] Coronary angiogram is usually done to delineate the anatomy and for the diagnosis. In this case, the origin of LMCA could not be visualized on transthoracic echocardiography (TTE) leading to inconclusive diagnosis. CTCA delineated the exact anatomy and showed its origin from RPA. Intraoperative TOE examination also revealed it as a case of AOLCARPA. Anesthetic management of patients presenting with ALOCARPA includes rapid and smooth intubation, while avoiding swings in blood pressure and heart rate. A decrease in pulmonary vascular resistance is avoided by preventing hyperventilation, hyperoxia and hypocarbia [3] The aim is to maintain a PaO2 of 80–100 mm Hg and an EtCO2 of 40–45 mm Hg. Both reduction and increase in afterload can hamper RCA perfusion and stroke volume, respectively. An optimum preload is required to ensure adequate cardiac output. CTCA and TOE examinations are useful modalities for delineating the coronary anatomy and help in planning the perioperative surgical and anesthetic management of the patient.

Abstract 15578: A Rare Case of Anomalous Left Main Coronary Artery Origin Presenting as ST Elevation Myocardial Infarction

Background: ST-segment elevation myocardial infarctions (STEMI) are uncommon presentations of coronary artery anomalies and pose challenges in the emergent setting of percutaneous coronary interventions. We describe an extremely rare case of left main coronary artery (LMCA) originating off the right coronary artery (RCA) in a patient who presents with STEMI and was found to have Medina 1,1,1 lesion at the origin of the anomalous (LMCA). Case Presentation: An 81-year old male, with a past medical history significant for aortic stenosis and hyperlipidemia, presents to the emergency room for substernal non-radiating chest pressure. His electrocardiogram showed ST segment elevations in inferior and anterior leads. He was taken emergently to the cardiac cath lab. Coronary angiogram revealed a single right coronary ostium with an anomalous LMCA to mid left anterior descending (LAD) artery via dual insertion within a myocardial bridge. There was severe 90% Medina 1,1,1 bifurcating proximal RCA stenosis and 90% proximal LMCA stenosis. The entire left coronary system was fed by the anomalous LMCA, which was inserted into the mid-LAD in a dual insertion, between which there was a myocardial bridge. Cardiothoracic surgery team was consulted, and decision was made to pursue coronary artery bypass grafting (CABG). An intra-aortic balloon pump was placed via right femoral artery and patient was taken for emergent surgery, where he underwent 3 vessel CABG (left internal mammary artery to LAD, saphenous venous graft to obtuse marginal branch and posterior descending artery). Discussion: A broad spectrum of coronary anomalies have been reported, but their incidence in STEMI is rare. We discuss a very rare anomaly in which the entire left coronary system branches off the RCA, and culprit lesion was found to be a Medina 1,1,1 lesion. We aim to add to the sparce data pool of STEMI management in patients with anomalous coronary arteries.

P424 SMALL HEART AND SINGLE CORONARY ARTERY IN A YOUNG PATIENT WITH CHRONIC FATIGUE SYNDROME: A CASE REPORT

Abstract Introduction Chronic fatigue syndrome/Myalgic encephalomyelitis (CFS/ME) is a clinically defined condition reported mostly in adults, characterized by severe and disabling fatigue limiting normal daily activities for at least 6 months accompanied by multiple unexplained symptoms including self–reported impairments in concentration and short–term memory, sleep disturbances, and musculoskeletal pain. Although rarely considered to have cardiac dysfunction, CFS/ME patients frequently have reduced stroke volume with a significant inverse relation between cardiac output and post–exertional malaise severity. We describe a case of a young man affected by CFS/ME and small heart with incidental findings of anomalous origin of the left main coronary artery (LMCA). Case Presentation A 19–year–old Caucasian male presented to our clinic complaining weakness, lack of concentration and excessive daytime sleepiness. Medical history included CFS/ME, D hypovitaminosis, inflammatory bowel disease associated with pancreatic insufficiency. Physical examination including cardiovascular auscultation was unremarkable, with normal blood pressure and heart rate. Electrocardiogram showed sinus rhythm at 75 bpm without any repolarization abnormalities. Echocardiogram revealed reduced diameters of left ventricle (LV) (end–diastolic diameter 36 mm), normal aortic root dimensions and, in a five–chamber apical view, a binary structure that seemed to cross the aorta perpendicularly to its long axis. Cardiac magnetic resonance (CMR) found significantly reduced LV stroke volume (34 ml/m2; normal values 44–68 ml/m2) and end–diastolic volume (57 ml/m2; normal values 68–103 ml/m2) together with reduced end–diastolic wall mass (51 g/m2; normal values 59–93 g/m2). Also, right ventricle (RV) volumes were reduced: stroke volume (32 ml/m2; normal values 40–72 ml/m2), end–diastolic volume (62 ml/m2; normal values 68–114 ml/m2). In addition, the exam confirmed the anomalous origin of LMCA stemming from the proximal segment of right coronary artery and following a retro–aortic course. Conclusions Not sufficient consideration was given to cardiovascular involvement in patients with CFS/ME, although many of signs and symptoms are suggestive of cardiovascular dysfunction. This case highlights that CFS/ME together with small heart is a condition possible also in young people. More studies and reports could be necessary to better define the association with cardiac congenital anomalies and CFS/ME.

770 Small heart and single coronary artery in a young patient with chronic fatigue syndrome: a case report

Abstract Aims Chronic fatigue syndrome/myalgic encephalomyelitis (CFS/ME) is a clinically defined condition reported mostly in adults, characterized by fatigue and unexplained aspecifical symptoms. Patients with CFS/ME frequently have reduced stroke volume with an inverse relation between cardiac output and post-exertional malaise severity. We describe a rare case of a young man affected by CFS/ME, small heart, and anomalous anatomy of left main coronary artery (LMCA). Methods and results A 19-year-old Caucasian male referred to our clinic complaining weakness, lack of concentration, and sleepiness. He suffered by CFS/ME, D hypovitaminosis, inflammatory bowel disease, and exocrine pancreas insufficiency. Blood tests revealed no pathological findings. Faecal exams documented intestinal dysbiosis and low pancreatic elastase. He was treated with oxygen-ozone rectal insufflations and probiotics. Physical examination was unremarkable. Electrocardiogram showed normal sinus rhythm. Echocardiogram revealed reduced diameters of the left ventricle (LV), normal aortic root dimensions and, in a five-chamber apical view, a binary structure that seemed to cross the aorta perpendicularly. Cardiac magnetic resonance (CMR) found reduced LV stroke volume (34 ml/m2) and end-diastolic volume (57 ml/m2) together with reduced end-diastolic wall mass (51 g/m2). Right ventricle volumes were reduced too. In addition, the exam confirmed the anomalous origin of LMCA stemming from the proximal segment of right coronary artery and following a retro-aortic course. Mechanism of CFS/ME remains unknown, although various factors have been implicated, including immune activation, chronic viral infection, and emotional disorders. A considerable number of patients affected by CFS has an anatomically small heart. Small heart syndrome, in fact, may contribute to the onset of CFS/ME. Previous studies hypothesized that clinical manifestations of CMS/ME were caused by reduced venous return, cardiac output, and heart mass, together with decreased arterial oxygen saturation. Single coronary artery is an uncommon congenital anatomic abnormality identified by a single coronary ostium giving rise to all arteries supplying the heart. Thus, we reported a rare case of a very young man affected by chronic fatigue syndrome and small heart, investigated not only with echocardiogram but also with CMR, not often used in this clinical setting. More, we found an anomalous origin of LMCA. From literature, it’s not reported any cases of a patient including these three rare conditions (CFS/ME, small heart, and single coronary artery). Conclusions This case highlights that CFS/ME together with small heart is a condition possible also in young people. More studies and reports could be necessary to better define the association between cardiac congenital anomalies and CFS/ME. 770 Figure

Unique family clustering of anomalous left main coronary artery origin from the right sinus of Valsalva: a case for echocardiographic screening and genetic determination.

Unique family clustering of anomalous left main coronary artery origin from the right sinus of Valsalva: a case for echocardiographic screening and genetic determination.

P1488 Anomalous origin of left coronary artery from right pulmonary artery in association with scimitar syndrome

Abstract Clinical Presentation A full-term neonate was referred to our institution because of respiratory distress. CXR was significant for right lung hypoplasia and mild cardiomegaly. ECG showed normal sinus rhythm, right atrial enlargement, and right ventricular hypertrophy with no signs of ischemia. Imaging Findings The initial echocardiogram demonstrated PAPVD with the right upper pulmonary vein draining into IVC/RA junction with flow acceleration (mean gradient= 7 mmHg), moderate ASD, small muscular VSD with left-right shunting, moderate PDA with bidirectional shunting. Forward flow was seen in the proximal part of left main coronary artery (LMCA). RV systolic pressure was supra-systemic with a qualitatively moderately reduced RV systolic function. The patient was taken to the catheterization lab where MPA angiography revealed an antegrade flow from the RPA into LMCA supplying both the anterior descending and the circumflex arteries. A selective injection within the scimitar vein showed drainage of the right lung into a vertical vein connecting with stenosis to IVC. A follow up echocardiogram to re-examine the coronary origin revealed an anomalous origin of LMCA from proximal RPA; 3 mm distal to branch pulmonary artery bifurcation with mainly antegrade low velocity flow into LMCA and LAD. (Image 1) Role of Imaging in Patient Care - Imaging of the coronary origin in patients with ALCAPA can be challenging especially if the LMCA originates from RPA. Also, the presence of pulmonary hypertension might contribute to maintain coronary perfusion and lead to misinterpretation of the antegrade flow in LMCA and its branches. - In certain situations, cardiac catheterization is essential to make the diagnosis of ALCAPA which prevented a potentially catastrophic outcome. Catheter intervention with a series of balloon dilations of the stenotic scimitar vein was successful in relieving the stenosis. Summary/Discussion Points: - Extensive review of the available literature revealed only three cases of Scimitar syndrome associated with ALCAPA. In all of these cases, the LMCA originated from the posterior sinus of MPA. Our case is the first to report ALCAPA from RPA in association with Scimitar syndrome. This presentation might have led to the initial misinterpretation of the echocardiography images. - The presence of pulmonary hypertension in our patient maintained an adequate antegrade flow across the LMCA preventing significant coronary steal and signs of myocardial ischemia. - The report highlights the challenges in making the diagnosis of ALCAPA with echocardiograms. Moreover, we discuss the role of cross-sectional and invasive imaging to rule out potential coronary arteries anomalies in patients with Scimitar syndrome, as this a rare although a very significant association that may have important implications in their outcomes. Abstract P1488 Figure. ALCAPA origin from RPA

False Negative Echocardiography in an Adolescent with Anomalous Left Main Coronary Artery Origin Presenting with Cardiac Arrest

False Negative Echocardiography in an Adolescent with Anomalous Left Main Coronary Artery Origin Presenting with Cardiac Arrest

Anomalous Origin of Left Coronary Artery from Main Pulmonary Artery (ALCAPA) Who Underwent Two Coronary System Repair with a Novel Technique

We report clinical, CT, invasive coronary angiography and intra-operative findings of a symptomatic elderly man with anomalous origin of left coronary artery from pulmonary artery (ALCAPA). ALCAPA is a rare syndrome with anomalous origin of left main coronary artery (LMCA) from main pulmonary artery (MPA). Survival into adulthood is rare and our case is probably the oldest survivor who has undergone two coronary system repairs for this anomaly. The unique features of our case include: 1) Absence of “q” wave myocardial infarction on ECG; 2) Absence of significant mitral regurgitation; 3) Demonstration of stress induced perfusion defects in nuclear imaging which as per our knowledge has been done for the first time in the literature; 4) A unique technique of closing anomalous LMCA origin by a pericardial patch, sutured on the internal aspect of MPA is the hallmark of this case.

An unusual co-anomaly: multiple coronary-cameral fistulas and left main coronary artery arising from the right sinus of Valsalva

A 70-year-old female patient was referred to the Cardiology Department for preoperative evaluation before undergoing total hysterectomy. She had no chest pain, but had experienced exertional dyspnea over the past several months. Besides age, she had no additional risk factors for coronary artery disease. Electrocardiogram showed normal sinus rhythm and nonspecific T wave changes, with a heart rate of 81 beats per minute. The physical examination was unremarkable, and exercise stress test was non-diagnostic. Coronary angiography revealed anomalous left main coronary artery (LMCA) arising from the right sinus of Valsalva (Fig. A, Video 1*) and the presence of multiple coronary-cameral fistulas (CCF) between the left ventricle and septal perforating branches of the left anterior descending (LAD) and right coronary arteries (Fig. B, Video 2; Fig. C, Video 3). According to the eye-dot method (Fig. B) and given the septal perforator branch before the LMCA bifurcation (Fig. A), its course was considered as septal, which was confirmed by computed tomography coronary angiography (CTCA) (Fig. D). Because of the benign nature of the abnormal septal course of the LMCA and clinically insignificant CCF, the patient was treated conservatively with β-blockers. She did not experience any chest pain during the hysterectomy and had an uneventful postoperative course. CCF is an uncommon clinical entity, which is defined as an anomalous communication between any epicardial coronary artery and the cardiac chambers. The functional significance and treatment of these fistulas remain unclear. As in our case, CCF originating from both coronary arteries and terminating in cardiac chambers is one of the least common forms. The anomalous origin of LMCA arising from the right sinus of Valsalva is quite rare, with an incidence of 0.1%. It has four possible courses: inter-arterial, retro-aortic, septal, and anterior. The clinical significance and management strategies in these arteries depend on the initial course of the anomalous vessel. In 1990, Serota et al. defined the ‘Dot and Eye’ method for identification of the initial course of anomalous coronary arteries in coronary angiography. In a septal course, the circumflex artery (Cx) will form an ellipse to the left of the aorta, similar to an ‘eye’, with the LMCA forming the inferior portion and the Cx forming the superior portion during 30 RAO injection. In conclusion, CTCA can visualize coronary anomalies accurately; however, a correct diagnosis can be made safely in selected cases by conventional angiography in the presence of clues. 782

Anomalous Left Main Coronary Artery Origin From the Right Sinus of Valsalva: A Novel Echocardiographic Screening Method

Anomalous origin of the left main coronary artery from the right sinus (ALMCA) is a rare entity associated with an increased risk for sudden cardiac death. Although transthoracic echocardiography often is the primary screening tool for the diagnosis, its accuracy remains undefined, resulting in the use of more expensive methods such as cardiac catheterization and magnetic resonance imaging when clinical suspicion exists. This report aims to describe a novel echocardiographic screening method for detecting ALMCA and to apply it retrospectively for patients with confirmed findings intraoperatively and/or on autopsy. All patients with a confirmed diagnosis of ALMCA who presented to our institution from 2004 to 2012 were included in the study. Eight patients had adequate images, including two patients with an initial diagnosis of normal coronary origins. A control group consisted of 35 patients with normal coronaries shown by echocardiogram. Five of these patients had normal coronary artery origins confirmed by magnetic resonance imaging (MRI). Echocardiographic images were reviewed, and the left main coronary was identified in the parasternal long-axis view between the aorta and pulmonary trunk. The angle of the left main coronary course was measured. In patients with ALMCA, the proximal course of the vessel was steeper as it coursed posteriorly. In contrast, the course was almost horizontal in patients with normal coronary origins. Based on these findings, a cutoff angle of 28° is proposed. In the parasternal long-axis view, ALMCA can be identified by its anomalous proximal course. This screening method is reliable and increases the accuracy of transthoracic echocardiograms.

Left main coronary artery origin from the right coronary sinus: rare, dangerous, and easy to miss on routine transoesophageal echocardiography

[Extract] Coronary artery anomalies are rare and may occur as an isolated defect or in conjunction with other congenital cardiac malformations. Sudden cardiac death due to myocardial ischaemia is a sinister complication.

E0702 Prevalence of anomalous coronary arteries

ObjectiveCoronary arteries with an anomalous origin and course may occasionally cause sudden death. This is the first study of the prevalence of anomalous coronary arteries in an Asian population.MethodsOver a...

Primary Stenting of an Anomalous Left Main Coronary Artery With an Interarterial Course During Cardiac Arrest

A 48-year-old woman was admitted with extensive anterior ST-elevation myocardial infarction and cardiac arrest on arrival. Emergent coronary angiography was performed simultaneously with cardiopulmonary resuscitation and multiple DC shocks for ventricular fibrillation. The right coronary artery was patent, but the origin of the left main coronary artery (LMCA) was not identified initially. After persistent efforts (2.5 hours), the LMCA origin was located at the right coronary sinus with a critical stenosis in its midportion. LMCA stenting was performed with a good angiographic result. The subsequent hospital course, which included prolonged mechanical ventilation and reversible anoxic brain injury, culminated in full hemodynamic and neurological recovery. Predischarge computed …

Transesophageal Echocardiography in Adult-Type Bland-White-Garland Syndrome

The origin of the left main coronary artery (LMCA) from the pulmonary artery (PA), known as Bland-White-Garland syndrome (BWGS), is a rare anomaly with mortality of 90% during infancy (1). If collateral circulation between right and left coronary systems ensues, BWGS patients may reach adulthood (1,2). However coronary flow directs preferentially into the lower pressure PA, away from the left ventricle (LV) creating “coronary steal,” and left-to-right shunt. Mitral regurgitation (MR) is further common in BWGS most likely due to multiple factors (3). Surgical correction of BWGS is strongly recommended, even in asymptomatic patients, because of the high risk of developing heart failure, myocardial infarction, ventricular arrhythmias, and sudden death (1,2). A 44-yr-old man presented with recent onset of fatigue and atrial fibrillation. Transthoracic echocardiography demonstrated severe MR with a posterior-directed jet, mild anterior mitral leaflet prolapse, a dilated mitral annulus, enlarged left atrium (5.8 cm), preserved global LV function, a moderately dilated right ventricle with moderate tricuspid regurgitation, and estimated right ventricle systolic pressure of 60 mm Hg. Because of the patient’s occasional chest pain, a persantine–thallium study was performed demonstrating a significant perfusion defect of the anterior LV wall. Cardiac catheterization revealed the absence of the LMCA in the left sinus of Valsalva. Contrast dye injected into the right coronary artery (RCA) filled the main branches of the LMCA with anomalous drainage through the LMCA into the PA. The patient was referred for mitral valve (MV) repair and correction of anomalous LMCA. Intraoperative transesophageal echocardiography (TEE) mostly confirmed the preoperative echocardiography findings but now demonstrated a large RCA (17 mm of diameter) that was identified at the right sinus of Valsalva. Color flow Doppler examination showed that the LMCA passed close to the left sinus of Valsalva with no connection to the aortic root. The LMCA origin was identified as arising from the PA, 18 mm distal to the pulmonic valve with retrograde flow into the PA (Figs. 1 and 2; please see video clip available at www.anesthesia-analgesia.org).Figure 1.: Preoperative multiplane transesophageal echocardiography (TEE), mid-esophageal aortic valve long axis view. The large orifice of the right coronary artery can be seen with laminar diastolic antegrade flow demonstrated by Color Doppler mapping. (LA, left atrium; MV, mitral valve; LVOT, left ventricle outflow tract, IVS, interventricular septum; AoV, aortic valve; AO, ascending aorta; RCA, right coronary artery.)Figure 2.: Preoperative multiplane transesophageal echocardiography (TEE), modified mid-esophageal aortic valve short axis view. The ascending aorta just above the aortic valve can be recognized. Color Doppler shows the left main coronary artery (LMCA) passing close to the left sinus of Valsalva however with no identified connection to the Aortic root. (LA, left atrium; RA, right atrium; IAS, interatrial septum; AO, ascending aorta.)The patient underwent uncomplicated reimplantation of the LMCA into the aortic root and MV repair. The postoperative TEE showed LMCA orifice reimplanted into the left sinus of Valsalva, and the MV annuloplasty ring with mild MR (Fig. 3).Figure 3.: Postoperative multiplane transesophageal echocardiography (TEE), mid-esophageal aortic valve short axis view. The LMCA orifice now reimplanted into the left sinus of Valsalva can be seen. (LA, left atrium; RA, right atrium; IAS, interatrial septum; LCC, left coronary cusp; RCC, right coronary cusp; NCC, noncoronary cusp; LMCA, left main coronary artery.)Identification of the coronary orifices and normal coronary flow pattern is an important part of a TEE examination. Young adults with no risk factors for coronary disease may present with MR and unrecognized BWGS. In the presence of a single enlarged RCA orifice, BWGS should be considered.