Ectopic Origin Research Articles

Ectopic High Origin of a Coronary Artery from the Aorta – A Possible Cause of Sudden Death

Congenital anomalies originating it the coronary arteries are of special interest for forensic specialists. The presented case is a man found dead on the Coast of Marmara Sea. The death was considered to be suspicious and an autopsy was performed. On the macroscopic autopsy, the ostium of the right coronary artery was hole-like, located in a normal position, but the ostium of the left coronary artery was pocket-shaped and located in the left wall of the ascending aorta and above the rim of the sinotubular junction. We describe an asymptomatic, but didactic case with ectopic high origin of the left coronary artery.

Successful percutaneous coronary intervention of anomalous origin right coronary arteries with 3-D RCA guide catheters: a report of three cases

Right coronary arteries with anomalous origin (ARCA) may be associated with myocardial ischemia and sudden death. Selective catheterization and percutaneous coronary intervention (PCI) of ARCA can be technically challenging due to their slit-like orifice and aortic intramural course with abrupt angulation. The 3DRCA guide catheter is a unique catheter with a helical configuration that provides optimal guide support during PCI. We describe three cases of successful PCI to right coronary arteries with anomalous origin (two from the left sinus of Valsalva, one with ectopic origin from the right sinus of Valsalva) using 3DRCA guide catheters after a variety of other catheters were tried unsuccessfully.

Secreción oculta de corticotropina y suprarrenalectomía bilateral: cuando en ocasiones es necesario matar al mensajero

Cushing's syndrome due to ectopic adrenocorticotrophic hormone (ACTH) secretion is infrequent, the most frequent cause being bronchial carcinoid; nevertheless, in a considerable proportion of cases (up to 16%), the origin of ACTH secretion is not found. Treatment of Cushing's syndrome due to ectopic ACTH secretion is surgical resection of the tumor. However, surgical excision is not always feasible, either because the tumor has not been located, or because it is unresectable; in these cases the question is when to carry out a bilateral adrenalectomy. We present a case of ACTH-dependent hypercortisolism, in which the ectopic origin of ACTH secretion was established from the results of complementary tests (suppression tests, petrosal sinus sampling, imaging); nevertheless, none of the imaging tests was able to identify the secreting tumor. Consequently, after a reasonable interval, a bilateral adrenalectomy was performed, with good clinical results.

Ectopic Origin of the Right Coronary Artery Shown on Cardiac Computed Tomography

Coronary artery anomalies can be life-threatening, with a prevalence of approximately 1%. An ectopic origin of the right coronary artery (RCA) from the left sinus of Valsalva accounts for less than 1% of all coronary artery anomalies. Although conventional angiography plays a standard role in diagnosis, recent advances in computed tomography (CT) are more accurate for three-dimensional structures. We report a CT-confirmed symptomatic case of an ectopic origin of the RCA from the left sinus of Valsalva. [ Tzu Chi Med J 2008;20(1):67–69]

Bigeminal Pulmonary Vein Ectopy Suppressed by Pulmonary Vein Isolation

A 58-year-old man with atrial fibrillation underwent pulmonary vein (PV) isolation (PVI). Bigeminal atrial premature beats persisted from the beginning of the PVI. The cardiac recordings from a basket catheter (BC) revealed the PV ectopic origin in the distal right superior PV. Successful PVI with the guidance of BC was confirmed by the appearance of concealed ectopy. Surprisingly, the PV ectopy completely disappeared immediately after the successful PVI. The findings suggest that the generation of PV trigger is sometimes dependent on left atrial input and that the underlying mechanism of the PV trigger may have been triggered activity or reentry.

Separate Origin of the Main Components of the Left Coronary Artery in Syrian Hamsters (Mesocricetus auratus)

This study describes a rare congenital coronary artery anomaly in the Syrian hamster; namely, the separate origin of the obtuse marginal and left circumflex arteries which are the main components of the left coronary artery. The hearts of nine affected animals were examined by means of a corrosion-cast technique and histology. The hamsters belonged to a laboratory inbred family with a high incidence of coronary artery anomalies and bicuspid aortic valve. The aortic valve was tricuspid in three hamsters and bicuspid in the other six hamsters. In all cases, the right coronary artery was normal, whereas the left coronary artery main trunk was absent. The present anomalous coronary artery patterns could be classified into two main entities: (i) ectopic origin of the obtuse marginal artery from the right aortic sinus or from the right coronary artery, with the left circumflex artery arising from the left side of the aortic valve; and (ii) ectopic origin of both the obtuse marginal artery from the right aortic sinus or from the right coronary artery and left circumflex artery from the dorsal aortic sinus. In all cases, the obtuse marginal artery coursed to the right side of the heart through the ventral wall of the right ventricular outflow tract. When the left circumflex artery arose from the dorsal aortic sinus, it formed an acute angle with the aortic wall. This report seems to be the first to describe the separate origin of the main components of the left coronary artery in a non-human mammalian species. In man, the congenital coronary artery and aortic valve defects reported herein may entail the risk of clinical complications. However, none of the affected hamsters showed signs of disease.

Coronary artery anomalies — a short review

Abstract Coronary artery anomalies are rare but sometimes important findings in the evaluation of the coronary artery. The majority of the anomalies are of benign prognosis, but others can be associated with cardiac symptoms and syndromes (angina, dyspnea, syncope, congestive heart failure, myocardial infarction and sudden death). The potentially serious anomalies include: ectopic coronary origin from the pulmonary artery; ectopic coronary origin from the opposite aortic sinus; and large coronary fistulae. Appropriate diagnosis is critical for recognition and management. Treatment can be performed by surgical or percutaneous approach. However, management is conservative in the majority of them. Contemporary diagnosis and clinical management of these anomalies are briefly reviewed and discussed in this article.

Ectopic origin of bronchial arteries: assessment with multidetector helical CT angiography

The purpose of this study was to determine non-invasively the frequency of ectopic bronchial arteries (BA) (i.e., bronchial arteries originating at a level of the descending aorta other than T5-T6 or from any aortic collateral vessel) on multidetector-row CT angiograms (CTA) obtained in patients with hemoptysis. Over a 5-year period (2000-2005), 251 consecutive patients with hemoptysis underwent multidetector-row CT angiography of the thorax. From this population, 37 patients were excluded because of a suboptimal CTA examination (n = 19), the presence of extensive mediastinal disease (n = 15) or severe chest deformation (n = 3) precluding any precise analysis of the bronchial arteries at CTA. Our final study group included 214 patients who underwent a thin-collimated CT angiogram (contrast agent: 300 to 350 mg/ml) on a 4- (n = 56), 16- (n = 119) and 64- (n = 39) detector-row scanner. The site of origin and distribution of bronchial arteries were analyzed on transverse CT scans, maximum intensity projections and volume-rendered images. The site of the ostium of a bronchial artery was coded as orthotopic when the artery originated from the descending aorta between the levels of the fifth and sixth thoracic vertebrae; all other bronchial arteries were considered ectopic. From the studied population, 137 (64%) patients had only orthotopic bronchial arteries, whereas 77 patients (36%) had at least one bronchial artery of ectopic origin. A total of 147 ectopic arteries were depicted, originating as common bronchial trunks (n = 23; 19%) or isolated right or left bronchial arteries (n = 101; 81%). The most frequent sites of origin of the 124 ostiums were the concavity of the aortic arch (92/124; 74%), the subclavian artery (13/124; 10.5%) and the descending aorta (10/124; 8.5%). The isolated ectopic bronchial arteries supplied the ipsilateral lung in all but three cases. Bronchial artery embolization was indicated in 26 patients. On the basis of CTA information, (1) bronchial embolization was attempted in 24 patients; it was technically successful in 21 patients (orthotopic BAs: 6 patients; orthotopic and ectopic BAs: 3 patients; ectopic BAs: 12 patients) and failed in 3 patients due to an instable catheterization of the ectopic BAs; the absence of additional bronchial arterial supply and no abnormalities of nonbronchial systemic arteries at CTA avoided additional arteriograms in these 3 patients; (2) owing to the iatrogenic risk of the embolization procedure of ectopic BAs, the surgical ligation of the abnormal vessels was the favored therapeutic option in 2 patients. This study enabled the depiction of ectopic bronchial arteries in 36% of the studied population, important anatomical information prior to therapeutic decision making.

Ectopic mammary carcinoma in a male cat

SIR, — We wish to report a case of a mammary carcinoma of presumed ectopic mammary gland origin in a male cat. A male eight-year-old cat was presented for evaluation of a tumour that had arisen in the subcutaneous tissue overlying the pectineus muscle in one of the hindlimbs. According to the

Binding of AlF-C, an Orc1-Binding Transcriptional Regulator, Enhances Replicator Activity of the Rat Aldolase B Origin

A region encompassing the rat aldolase B gene (aldB) promoter acts as a chromosomal origin of DNA replication (origin) in rat aldolase B-nonexpressing hepatoma cells. To examine replicator function of the aldB origin, we constructed recombinant mouse cell lines in which the rat aldB origin and the mutant derivatives were inserted into the same position at the mouse chromosome 8 by cre-mediated recombination. Nascent strand abundance assays revealed that the rat origin acts as a replicator at the ectopic mouse locus. Mutation of site C in the rat origin, which binds an Orc1-binding protein AlF-C in vitro, resulted in a significant reduction of the replicator activity in the mouse cells. Chromatin immunoprecipitation (ChIP) assays indicated that the reduction of replicator activity was paralleled with the reduced binding of AlF-C and Orc1, suggesting that sequence-specific binding of AlF-C to the ectopic rat origin leads to enhanced replicator activity in cooperation with Orc1. Involvement of AlF-C in replication in vivo was further examined for the aldB origin at its original rat locus and for a different rat origin identified in the present study, which contained an AlF-C-binding site. ChIP assays revealed that both replication origins bind AlF-C and Orc1. We think that the results presented here may represent one mode of origin recognition in mammalian cells.

Bronchial and Nonbronchial Systemic Arteries in Patients with Hemoptysis: Depiction on MDCT Angiography

In this pictorial essay, we show the usefulness of MDCT angiography for visualization of the bronchial and nonbronchial systemic feeder vessels responsible for hemoptysis. By providing thin-section transaxial, multiplanar reconstruction, and 3D images, CT angiography using MDCT allows comparable or better images than conventional angiography with respect to the depiction of bronchial or nonbronchial systemic arteries. CT angiography is particularly useful for visualizing the ectopic origin of bronchial arteries and nonbronchial systemic collateral arteries.

Moray mouth heart

Bicuspid aortic valve (BAV) is the most common congenital heart defect in adult patients. A 1–2% of prevalence has been reported in the general population. The association with aortic wall abnormalities (cystic medial necrosis, aortic coartaction and aortic dissection) is well known [1]. Coexisting coronary anomalies, in particular ectopic coronary origins, have also been evidenced by some authors [2]. Such associations support the hypothesis of a common embryologic defect involving the developmental process of aortic valve, aortic wall and proximal coronaries. In this case report, we describe for the first time the association between BAV and an aberrant configuration of cardiac apex [3,4] and we report a not yet described angiographic image of this anomaly.

Modular structure of the human lamin B2 replicator.

The cis-acting elements necessary for the activity of DNA replication origins in metazoan cells are still poorly understood. Here we report a thorough characterization of the DNA sequence requirements of the origin associated with the human lamin B2 gene. A 1.2-kb DNA segment, comprising the start site of DNA replication and located within a large protein-bound region, as well as a CpG island, displays origin activity when moved to different ectopic positions. Genomic footprinting analysis of both the endogenous and the ectopic origins indicates that the large protein complex is assembled in both cases around the replication start site. Replacement of this footprinted region with an unrelated sequence, maintaining the CpG island intact, abolishes origin activity and the interaction with hORC2, a subunit of the origin recognition complex. Conversely, the replacement of 17 bp within the protected region reduces the extension of the protection without affecting the interaction with hORC2. This substitution does not abolish the origin activity but makes it more sensitive to the integration site. Finally, the nearby CpG island positively affects the efficiency of initiation. This analysis reveals the modular structure of the lamin B2 origin and supports the idea that sequence elements close to the replication start site play an important role in origin activation.

Increased Hexokinase Activity, of Either Ectopic or Endogenous Origin, Protects Renal Epithelial Cells against Acute Oxidant-induced Cell Death

Glucose (Glc) metabolism protects cells against oxidant injury. By virtue of their central position in both Glc uptake and utilization, hexokinases (HKs) are ideally suited to contribute to these effects. Compatible with this hypothesis, endogenous HK activity correlates inversely with injury susceptibility in individual renal cell types. We recently reported that ectopic HK expression mimics the anti-apoptotic effects of growth factors in cultured fibroblasts, but anti-apoptotic roles for HKs have not been examined in other cell types or in a cellular injury model. We therefore evaluated HK overexpression for the ability to mitigate acute oxidant-induced cell death in an established epithelial cell culture injury model. In parallel, we examined salutary heparin-binding epidermal growth factor (EGF)-like growth factor (HB-EGF) treatment for the ability to 1) increase endogenous HK activity and 2) mimic the protective effects of ectopic HK expression. Both HK overexpression and HB-EGF increased Glc-phosphorylating capacity and metabolism, and these changes were associated with markedly reduced susceptibility to acute oxidant-induced apoptosis. The uniform Glc dependence of these effects suggests an important adaptive role for Glc metabolism, and for HK activity in particular, in the promotion of epithelial cell survival. These findings also support the contention that HKs contribute to the protective effects of growth factors.

Blood-brain barrier formation of grafted human umbilical vein endothelial cells in athymic mouse brain.

Human umbilical vein endothelial cells (HUVECs) were transplanted in athymic mouse brain and neovascularization of grafted endothelial cells was studied. HUVECs were transfected by a reporter gene pEGFPE-N1 in vitro and grafted stereotactically in unilateral striatum of adult nude mice. Histological studies in 4 weeks revealed that grafted HUVECs newly formed microvessels in brain, which were migrated and fused with host vessels. Intravenous injection of Evans blue before sacrificing animals resulted in no extravasation of dye, indicating that a blood-brain barrier (BBB) was formed by the grafted HUVECs. Immunohistochemistry demonstrated that host astrocytes extended glial feet on the grafted endothelial cells and a part of the newly formed vessels was positive with glucose transporter-1. These results indicate that endothelial cells from an ectopic origin have the potential to form a BBB after grafting in the central nervous system.

Anomalous origin of the left main coronary artery from the right aortic sinus with intramyocardial tunneling through the septum with free portion in the right ventricular cavity

Anomalous origin of the left main coronary artery from the right aortic sinus with intramyocardial tunneling through the septum with free portion in the right ventricular cavity

The one-kilobase DNA fragment upstream of the ardC actin gene of Physarum polycephalum is both a replicator and a promoter.

The 1-kb DNA fragment upstream of the ardC actin gene of Physarum polycephalum promotes the transcription of a reporter gene either in a transient-plasmid assay or as an integrated copy in an ectopic position, defining this region as the transcriptional promoter of the ardC gene (PardC). Since we mapped an origin of replication activated at the onset of S phase within this same fragment, we examined the pattern of replication of a cassette containing the PardC promoter and the hygromycin phosphotransferase gene, hph, integrated into two different chromosomal sites. In both cases, we show by two-dimensional agarose gel electrophoresis that an efficient, early activated origin coincides with the ectopic PardC fragment. One of the integration sites was a normally late-replicating region. The presence of the ectopic origin converted this late-replicating domain into an early-replicating domain in which replication forks propagate with kinetics indistinguishable from those of the native PardC replicon. This is the first demonstration that initiation sites for DNA replication in Physarum correspond to cis-acting replicator sequences. This work also confirms the close proximity of a replication origin and a promoter, with both functions being located within the 1-kb proximal region of the ardC actin gene. A more precise location of the replication origin with respect to the transcriptional promoter must await the development of a functional autonomously replicating sequence assay in Physarum.

Coronary MRA: use in assessing anomalies of coronary artery origin.

The purpose of this work was to describe the use of coronary MR angiography (MRA) in the clinical evaluation of a series of patients with anomalous origin of the coronary arteries suspected on coronary angiography. Eight patients underwent coronary MRA to further define variant coronary anatomy that was found on coronary angiography. A 2D segmented k-space gradient echo sequence was used with breath-holding. MRA images were assessed for traversal of an anomalous artery between the aorta and pulmonary artery trunks, which carries the greatest clinical significance. Of six patients with anomalous origin of the right coronary artery on angiography, two were shown by MRA to have an interarterial course of the anomalous vessel. Neither of two left coronary arteries with ectopic origin coursed between the great arteries, although one passed through the septum. Coronary MRA is a useful adjunctive technique to angiography in the evaluation of the relationship of anomalous coronary arteries to the great arteries.

Sudden death in cases with anomalous origin of the left coronary artery

Ectopic origin of the left coronary artery from the right sinus of valsalva with anterior and posterior courses are thought to be benign anomalies. Case reports: a 58-year-old woman died suddenly after a car accident without having sustained any injuries. The only abnormal finding was an ectopic origin of the left coronary artery from the right sinus with anterior free wall course. The anomaly was complicated by the absence of a left descending branch and a hypoplastic circumflex artery. A 38-year-old male died suddenly during work. Beside the ostium of the right coronary artery originated the normal calibered circumflex branch of the left coronary artery which passed behind the aorta. The left anterior descending branch had a normal origin. There was a 2 cm in diameter transmural infarct observed microscopically.

Epidemiology of congenital coronary artery anomalies: a coronary arteriography study on a central European population.

The anatomical patterns and frequency of occurrence of congenital coronary anomalies (CCA) in a Central European cohort has not yet been studied. The angiographic data of 7,694 consecutive patients undergoing coronary arteriography at the Albert Szent-Györgyi Medical University, Szeged, Hungary, from 1984 to 1994 were analyzed. CCA were found in 103 patients (1.34% incidence). Ninety-eight of them (95.2%) had anomalies of origin and distribution, and five (4.8%) had coronary artery fistulae. The incidence was the highest for the separate origin of left descending artery and left circumflex from the left sinus of Valsalva (52.42%). Anomalous origin of the left circumflex coronary artery from the right coronary was 8.7% while from the right sinus of Valsalva 18.4%. CCA, which may be associated with potentially serious events, such as ectopic coronary origin from the opposite aortic sinus (1.9%) and single coronary arteries (3.88%), were not frequent. The incidence of CCA in the Central European cohort under study was similar to that of the largest North American study. The anatomic classification presented can be useful from both clinical and surgical standpoints.