Ectopic Origin Research Articles

A Case Report of Aberrant Origin of the Right Coronary Artery from thePulmonary Artery in an Adult Male: The Role of Multi-modality Imaging

Background: The normal anatomy of the coronary arteries consists of the right coronary artery and the left main coronary artery, which originate from the right and left sinuses of Valsalva in the aortic root separately. In general, congenital anomalies of the coronary artery are uncommon in adults. The ectopic origin of a coronary artery from the pulmonary trunk is part of a group of infrequent coronary artery anomalies. The Anomalous origin of the Right Coronary Artery from the Pulmonary Artery (ARCAPA) is thought to be an extremely rare congenital coronary artery anomaly. Most of our knowledge and experience about this congenital coronary malformation is derived from published case reports and series. Case Presentation: We have, herein, reported the case of a 68-year-old male who was discovered to have ARCAPA after he presented to our center with chest pain. The patient underwent multiple invasive and non-invasive diagnostic imaging modalities, including an echocardiogram, a coronary angiogram, a multislice-gated coronary computed tomographic angiogram, and stress perfusion cardiovascular magnetic resonance imaging. The clinical decision was to continue with conservative non-surgical management along with close follow-up. Conclusion: The ARCAPA anomaly is considered to be one of the rare congenital coronary artery variances that can be discovered in late adulthood as a patient may remain asymptomatic for many decades.

Ectopic right coronary artery arising from the pulmonary artery: a rare congenital malformation.

An ectopic origin of the right coronary artery from the pulmonary artery is an extremely rare congenital malformation. This case report aimed to review our experience in the diagnosis and treatment of coronary artery anomalies. We report a case of ectopic origin of the right coronary artery from the pulmonary artery. We analyzed the taxonomy and clinical implications of the ectopic origin of the coronary artery. The findings of this case may increase patients' and clinicians' awareness of this anomaly.

Findings at repeat ablation after complex left atrial pulsed field ablation for longstanding persistent atrial fibrillation

Abstract Background There is a limited knowledge on atrial arrhythmias recurring after complex left atrial (LA) pulsed field ablation (PFA) for longstanding persistent atrial fibrillation (LSPAF). Purpose To present recurrent atrial arrhythmias and their sources found at repeat ablation following PFA for LSPAF. Methods and results Of 214 patients with LSPAF (age 65±8, 53 females, continuous AF for 30±27 months) with complex LA PFA (Farapulse) in 10/2021-10/2023, 19 patients (64±8 years, 5 females) underwent repeat ablation for recurrent AF or arial tachycardia (AT) in 20 procedures. First PFA procedure included pulmonary vein isolation (PVI), roof/posterior wall, and mitral isthmus/coronary sinus/Ligament of Marshal region (LOM) ablation. At repeat ablation, 3D activation and entrainment mapping and ablation identified the AF/AT sources; ATs sources were classified as macroreentry (MRAT) (perimitral, roof-dependent, typical flutter) or localized (LOCAT) in case of smaller reentry or ectopic origin. Results The clinical recurrent arrhythmias included paroxysmal AF (n=1), persistent AF (n=2), paroxysmal AT (n=1) or persistent AT (n=16). At the onset of repeat ablation, persistent AF/AT was present (n=15) or had to be induced (n=5). AF was finally present during the procedure in 8 patients, and was terminated by ablation directly into SR (n=3) or into intermediate AT (n=3) (conversion sites included LA ridge/LOM region 2x, RA septum 1x, RA low lateral 2x, CTI 1x), or required DCC (n=2). Primary recurrent or from AF converted AT was present in 15 patients; only MRAT in 6 patients, only LOCAT in 3 patients, and both AT types in 6 (patients). Of MRAT (N. ATs = 16), there were 12 perimitral ATs, 0 roof-dependent AT, and 2 typical flutters. Of LOCATs, sources were located at LA anterior wall 4x, LA septum 2x, LA roof 1x, LA ridge/LOM 1x, persistent left vena cava 1x, and RA – para SA node 1x. Recurrent AF/AT sources were targeted by radiofrequency or PFA in 16 and 4 cases, respectively, all spontaneous and induced AF/AT were finally terminated into SR in 16 cases, subsequent noninducibility by atrial pacing up to 300 bpn was achieved in 13 cases, and recurrent AF/AT occurred in 3 patients. Conclusion At repeat ablation PFA (Farapulse) for LSPAF, recurrent AT dominated over AF, MRAT slightly prevailed over LOCAT, mostly constituted by perimitral AT, while roof-dependent AT recurrence was reduced to zero. A majority of AF/AT sources were located outside prior PFA lesions at the LA anterior wall, septum and the right atrium.

Pulsed field ablation for complex resistant atrial tachycardia recurring after radiofrequency ablation of persistent atrial fibrillation

Abstract Background There is a limited knowledge on immediate and long-term efficacy of pulsed field ablation (PFA) for residual atrial tachycardia (AT) recurring after persistent atrial fibrillation ablation (AF). Purpose To present immediate and medium-term outcome of PFA (Farapulse) for complex AT recurring after ≥1 radiofrequency ablation (RFA) of primary (longstanding) persistent AF. Methods In 5/2022-10/2023, 81 patients (64±8 years, 19 females) underwent PFA for complex AT in 83 PFA procedures after prior RFA procedure(s); in first (n=21), second (n=33), third (n=23), fourth (n=5), or fifth (n=1) repeat ablation procedure, respectively. Mapping/ablation strategy was directed by the CS activation/entrainment to: 1) no 3D mapping and direct PFA in left atrium (n=67); 2) PFA directly after 3D LA mapping (n=9); 3) PFA following RFA after 3D LA mapping (n=7). AT sources were classified as macroreentry (MRAT) (perimitral, roof-dependent, typical flutter) or localized (LOCAT) in case of smaller reentry or ectopic origin. Results At the procedure onset, persistent AT was present in 58 patients, in the remaining cases AT (n=21) or AF (n=4) were induced. AF eventually occurred at some time of procedure in another 10 patients and was terminated by ablation at LA ridge/CS/LOM region 8x, LA posterior wall 3x, LUPV 1x, RUPV 1x, and RA low lateral wall 1x. Only MRAT, only LOCAT, or both AT types were found at 34 (41%), 35 (42%), and 14 (17%) procedures, respectively. A total of 125 ATs was identified (1, 2, and 3 ATs in 52, 20, and 11 patients). MRAT (n=60) included 32 perimitral ATs, 20 roof-dependent ATs, and 8 typical flutters. LOCAT (n=65) included RUPV source (n=1), LA extraPV sources (n=59), and RA sources (n=5). SR was restored with a 5±7 PFA applications at the site of AF source, total of PFA deliveries was 71±27, and procedure and fluoroscopy times were and 89±37 and 9±5 minutes, respectively. In the 7 patients with initial ineffective 6, 6, 11, 5, 18, 10, and 2 minutes of RFA, the AT was converted into SR by subsequent 1, 4, 1, 13, 4, 24, and 20 PFA applications. All ATs were terminated and noninducibility was achieved in 82 (99%) and 74 (89%) procedures, respectively. In 52 patients with >3 month follow-up, 46 (88%) patients remain in stable SR (41 / 79%) off AAD); 3 and 3 patients have paroxysmal or persistent AT. Conclusion PFA with catheter Farapulse can affect complex/epicardial AT sources resistant to RFA in prior or present ablation procedure; however, there is a certain discrepancy between high efficacy in AT terminating and medium-term clinical outcome.

Estimation of ventricular ectopic beat origin with electrocardiographic imaging

Abstract Background Precise identification of premature ventricular contractions (PVC) origins poses a significant challenge in electroanatomic mapping. Electrocardiographic Imaging (ECGI) allows instantaneous and global activation time mapping, providing a fast way to locate the origin of ectopic contractions. However, the standard methodology to estimate local activation times (LAT) in invasive electrograms is not directly transferable to ECGI signals. Objective In this study, a new methodology to calculate LAT maps with ECGI is presented and compared to the standard method in both, mathematical simulations and patients. Method We conducted computer simulations of five distinct PVCs to generate ECGI signals. Two techniques were applied to estimate LATs: the conventional -dV/dt method, which identifies the steepest negative deflection, and our novel Bayesian approach. The error in the location of the origin of the electrical activation and the similarity of the LAT maps were calculated with respect to the reference computer model. Additionally, we tested both algorithms in four patients with PVCs originated in different locations (three in the output tract of the right ventricle and one in the ventricular base, close the coronary sinus). Results The results indicated a substantial improvement in identifying the earliest activation site using our Bayesian algorithm, with a reduced localization error (1.5 ± 2.5 cm to 0.6 ± 1.5 cm). The root mean squared error (RMSE) was also significantly lower when comparing our Bayesian LAT maps to the reference LATs obtained in the simulations (19.0 ± 5.3 vs. 10.8 ± 4.5 ms). Patient data revealed smoother and more physiologically consistent propagations in the Bayesian maps, aligning with the PVC origins identified via electroanatomical navigation systems. Conclusion This study demonstrates that ECGI signals, when processed with advanced Bayesian techniques, significantly enhance LAT estimation. The proposed methodology improves the localization accuracy of ectopic beats and has practical implications for optimizing PVC treatment protocols.

Abstract P391: Case Review of Congenital Ectopic Coronary Artery Disease

Coronary artery anomalies (CAAs), rare congenital conditions affecting approximately 1% of adults, are associated with adverse cardiac events, including sudden cardiac death. This article reviews three recent clinical cases involving CAAs, delving into their origins, clinical presentations, diagnostics, treatments, and outcomes. CAAs involve irregularities in coronary artery origin, distribution, and termination. Notably, the left coronary artery arising from the right sinus (0.15%) and the right coronary artery from the left sinus (0.92%) are common variations. Other anomalies include the left circumflex artery (LCX) originating from the right coronary sinus and the left main coronary artery or Left anterior descending artery (LAD) emerging from opposite sinuses, leading to diverse clinical presentations. Case 1 features a 70-year-old female with hypertension presenting with exertional angina. Surgical revascularization was performed after coronary angiography revealed an ectopic LAD. In Case 2, a 72-year-old male with hypertension presented with chest pain and NSTEMI. Coronary angiography showed an ectopic LCX and significant stenosis in LAD and RCA, treated with PCI. Case 3, involving a 49-year-old female with hypertension and syncope, was managed medically as angiography revealed an anomalous RCA origin without significant obstructive disease. Treatment approaches for CAAs are patient-specific due to limited guidelines. Case 1 underwent coronary artery bypass surgery, Case 2 received PCI, and Case 3 was treated medically. Diagnostic methods encompass stress testing, coronary angiography, and coronary CT angiography (CCTA). CCTA is particularly valuable for distinguishing benign and clinically significant ectopic origins. For intramural courses, coronary unroofing is the preferred surgical technique, while absent intramural courses may require coronary translocation or reimplantation.Percutaneous coronary intervention (PCI) primarily addresses downstream atherosclerotic lesions without directly addressing the CAA. Treatment choice depends on patient-specific risk factors and pathology. In summary, CAAs pose challenges in interventional cardiology, warranting early and accurate diagnosis to prevent adverse events. A personalized approach, considering patient characteristics, is crucial due to the lack of robust guidelines. Advanced imaging technologies like CCTA and MRA aid in assessing CAAs' clinical significance and guiding treatment decisions. Further research is required to establish optimal treatment strategies, including investigating CAAs' prevalence in specific regions such as Arkansas.Top of Form Bottom of Form

Coexistence of Ehlers–Danlos Syndrome with Coronary–Pulmonary Arterial Fistula and Other Multiple Coronary Artery Anomalies

This case report presents a 34-year-old male with Ehlers–Danlos syndrome, type 2 diabetes mellitus, aortic valve regurgitation, and aortic bulb aneurysm. Following spine surgery for thoracic–lumbar stabilization, the patient underwent assessment for aortic bulb aneurysm and aortic valve replacement surgeries. Five months post spinal surgery, a coronary computed tomography angiography was performed. The coronary computed tomography angiography revealed unique findings, including the absence of the left main coronary artery, right coronary artery dominance, ectopic origin of the left circumflex artery from the right sinus of the valsalva, a coronary–pulmonary arterial fistula originating from the right sinus of the valsalva, and an additional right pulmonary vein. The patient was qualified for surgical treatment for an aortic bulb aneurysm, was informed about the high surgical risk, and is awaiting surgery. This case underscores the rarity of Ehlers–Danlos syndrome coexisting with multiple coronary artery anomalies. The presence of a coronary–pulmonary arterial fistula further emphasizes the need for specialized patient monitoring when Ehlers–Danlos syndrome and coronary anomalies converge.

THU033 High Prevalence Of Venous Thrombotic Events In Cushing’s Syndrome: Data From ERCUSYN

Abstract Disclosure: K. Isand: None. R.A. Feelders: None. T.C. Brue: None. M. Toth: None. T. Deutschbein: None. M. Reincke: None. M. Krsek: None. A. Santos: None. F. Demtroder: None. O. Chabre: None. C.J. Strasburger: None. J.A. Wass: None. S.M. Webb: None. A. Aulinas Maso: None. V. Volke: None. E. Valassi: None. For all ERCUSYN investigators Background: Patients with Cushing’s syndrome (CS) have increased risk of developing venous thromboembolic complications. The UK audit and meta-analysis of literature on Cushing’s disease (CD) over 50-years in Stoke-on-Trent by Clayton et al concluded that overall mortality in CD is double that of the general population. The risk of a thrombotic event (TE) (both arterial and venous) in patients with CS has been reported to be up to 10 times higher than that of the general population. When it comes to the cause of death, atherosclerotic diseases and venous thromboembolism (VTE) account for 43.4% of the deaths in patients with endogenous CS. Aim: To evaluate the prevalence of VTE in patients included in The European Registry on Cushing’s syndrome (ERCUSYN) and to study the details of each event to determine if there is any common line in patients who have been diagnosed with VTE. Methods: A retrospective observational cohort study; data extraction from the registry was taken on the 07.02.2022. Results: There were 95 VTE events among 2173 CS patients (prevalence of 4.4 %). Among the 57 centres included in the database (from 26 European countries), events were reported in 28 (from 16 countries). Patients were aged between 14-75 years, median age 47 years; 63(66%) were female. The 95 VTE events were observed in 70 (74%) pituitary-dependent, 12 (13%) adrenal-dependent, 10 (11%) ectopic and 3(3%) CS due to other causes. Comparing aetiologies, ectopic origin accounted for 10.5% of VTE patients and only 5% of those without VTE. Mean ± SD body mass index (BMI) reported in 68/95 patients was 28.8 ± 10.7 kg/m2, and more than three quarters (52; 76%) had a BMI above 25 kg/m2, being either overweight or obese; 21 (22%) patients were smoking. The most frequent comorbidity was hypertension (65 cases, 68%), followed by diabetes mellitus (40 cases, 42%) and osteoporosis (23, 24%); 7 (7%) had a cancer; 7 (7%) had active hypercortisolism (defined by elevated levels of midnight serum cortisol, urinary cortisol or cortisol day curve), 15 (16%) were in partial remission, 46 (48%) were cured and for 7 (7%) the status was unknown. Sixty-two (65%) had had 1 surgery, 22 (23%) had had two surgeries and 1 in the VTE group had 5 operations. Comparing groups, the VTE group had more surgeries than the no-VTE group. Mean operation rate per patient was 1.42 for the VTE group and 1.2 for no-VTE event group (p=0.0068). Seven (7%) patients died during the follow-up time (for different reasons). Conclusion: We report a high VTE rate in CS patients within ERCUSYN. Of note, to our knowledge this is the largest registry-based study to date looking at VTE in CS patients. Importantly, more than half of the VTE events were diagnosed in patients with normocortisolaemia. Presentation: Thursday, June 15, 2023

Acinar cell carcinoma of gastric ectopic pancreas origin: a case report and literature review

BackgroundPrimary pancreatic-type acinar cell carcinoma of the stomach is extremely rare, often misdiagnosed, and of unclear origin.Case presentationWe report the case of a primary pure pancreatic-type acinar cell carcinoma of the stomach in a 58-year-old woman. This is the first reported case to exhibit residual ectopic pancreatic tissue adjacent to the tumor serving as evidence for the origin of the carcinoma. Furthermore, we summarized the clinicopathological features of pancreatic-type acinar cell carcinoma of the stomach in order to further understand this solid tumor.ConclusionsPrimary pancreatic-type acinar cell carcinoma of the stomach is rare. Data on tumors of this histological type are still relatively scarce, and more in-depth research is needed to elucidate their molecular biological characteristics and prognosis.

Relevance of inferior petrosal sinus sampling in the diagnosis of Cushing's syndrome: a case report.

Non-specific signs and symptoms of Cushing's syndrome (CS) can pose a diagnostic challenge. We report the case of a man referred to the service of endocrinology for suspected CS. Hypercortisolism was confirmed on CS screening tests, whereas diagnostic tests confirmed the presence of adrenocorticotropin (ACTH) -dependent CS. The corticotropin-releasing hormone stimulation (CRH) test was performed to determine whether CS hadan endogenous or ectopic origin. Since the CRH and the magnetic resonance imaging (MRI) test were negative, IPSS was performed and suggested that CS was originated in the pituitary glands. Transsphenoidal pituitary resection was carried out. Histopathological analysis confirmed the cortitotrope origin of the tumor. The etiological study and differential diagnosis of CS are complex processes that involve a variety of biochemical and imaging tests. It is important that a sequence of biochemical screening and diagnostic tests is performed, along with studies for establishing the location of the lesion, to determine whether CS has an adrenal, pituitary or ectopic origin. Despite its good diagnostic performance, the results of biochemical tests may not be conclusive, especially in ACTH-dependent CS. In the case reported, the inconclusive results obtained in the CRH test rendered an invasive procedure (IPSS) necessary, which ultimately confirmed diagnosis.

Tumores torácicos

There are multiple, heterogeneous types of thoracic and mediastinal cancers. Thoracic tumors have a broad classification based on anatomical-pathological criteria with a progressive integration of immunohistochemical aspects and characteristics related to tumor genetics which are important in regard to possible systemic treatments for these tumors. They are classified as parenchymal tumors, mesenchymal tumors, thoracic wall (osteocartilaginous, soft tissue, hematological, and metastatic) tumors, lymphohistiocytic tumors, and primary tumors of ectopic origin. Imaging techniques and clinical suspicion should help us in choosing a diagnostic technique. The diagnosis and staging should be performed in the same procedure in the least aggressive manner possible so as to propose a treatment as soon as possible. Mediastinal lesions are classified according to the mediastinal area they affect. Their symptoms depend on systemic involvement, associated paraneoplastic syndromes, and invasion and/or compression of nearby structures. Imaging techniques are fundamental for a suspected diagnosis, with later cytological/histological diagnostic confirmation. Treatment will depend on the lesion. Surgery is common in benign or limited lesions and chemotherapy and/or radiotherapy in advanced or more aggressive lesions.

An unusual congenital coronary anomaly: A circumflex coronary artery arising in ``shotgun`` with the right coronary artery in anterior ST – elevation myocardial infarction

Objective: Coronary anomalies do not generate symptoms, it is incidental findings when performing a coronary angiography in a patient with ischemic heart disease or valvular heart disease. The anomalous origin of the circumflex (LCX) of the right coronary artery (RCA) is one of the common anomalies. We describe a case of CX orifice arising from right coronary sinus in a ``shotgun`` manner with orifice of RCA and successful percutaneous coronary intervention (PCI). Case Report: In this case, the anomalous incidental finding was the arising of the CX artery in “shotgun” shape with the RCA (separate ostium) in a clinic scenario of anterior ST-segment elevation myocardial infarction wherein the patient received fibrinolytic therapy with alteplase, presenting reperfusion criteria (&qt;50% ST-segment resolution at 60 minutes and relief angina) and then underwent to successful PCI. Conclusion: The ectopic origin of the CX is a well-recognized variant, which is considered the most common coronary anomaly. Although this anomaly is classified as benign and asymptomatic, a few cases of sudden death, myocardial infarction, and angina pectoris in the absence of atherosclerotic lesion have been reported. We shared successful PCI in such coronary anomaly in a patients with ST elevation anterior myocardial infarction. Such coronary anomalies should be considered as well in patients with STEMI undergoing PCI.

Inferior Vena Cava as a Trigger for Paroxysmal Atrial Fibrillation: Incidence, Characteristics, and Implications.

Identifying nonpulmonary vein triggers during atrial fibrillation (AF) ablation is of great importance. Currently, there are limited data on AF triggered by the inferior vena cava (IVC). This study was performed to investigate the incidence, characteristics, and implications of IVC triggers for AF. A total of 661 patients who underwent initial paroxysmal AF ablation were included. After pulmonary vein isolation, ectopic beats that triggered AF were further studied. Activation mapping and angiography were performed to confirm the location of ectopic origin. Electrocardiographic analysis of the ectopic P-wave (P'-wave) was performed. Six patients (0.91%) with AF triggered by the IVC were confirmed. The mean distance from the earliest activation site to the IVC ostium was 6.8 ± 2.5mm (5.2 to 11.2mm). Furthermore, the arrhythmogenic foci within the IVCwere all located at the apical hemisphere of the IVC (3 at the septal side and 3 at the anterior side). A total of 2.3±0.5 applications of radiofrequency energy were delivered to eliminate IVC triggers. The mean duration of the P'wave was 91.2 ± 11.2 milliseconds (81 to 108 milliseconds), which was narrower than that of the sinus P-wave (115.2±19.3 milliseconds [87 to 139 milliseconds]; P = 0.002). Moreover, the configuration of all P' waves in the inferior leads was negative. During a mean follow-up period of 25.5 ± 7.3months, all 6 patients remained arrhythmia free without antiarrhythmic drugs. IVC trigger, a rare but latent source of paroxysmal AF, could be identified and safely eliminated by focal radiofrequency ablation. Ectopic beats originating from the IVC presented with narrow P'-wave duration and negative P'waves in all inferior leads.

Escherichia coli cell factories with altered chromosomal replication scenarios exhibit accelerated growth and rapid biomass production

BackgroundGenerally, bacteria have a circular genome with a single replication origin for each replicon, whereas archaea and eukaryotes can have multiple replication origins in a single chromosome. In Escherichia coli, bidirectional DNA replication is initiated at the origin of replication (oriC) and arrested by the 10 termination sites (terA–J).ResultsWe constructed E. coli derivatives with additional or ectopic replication origins, which demonstrate the relationship between DNA replication and cell physiology. The cultures of E. coli derivatives with multiple replication origins contained an increased fraction of replicating chromosomes and the cells varied in size. Without the original oriC, E. coli derivatives with double ectopic replication origins manifested impaired growth irrespective of growth conditions and enhanced cell size, and exhibited excessive and asynchronous replication initiation. The generation time of an E. coli strain with three replication origins decreased in a minimal medium supplemented with glucose as the sole carbon source. As well as cell growth, the introduction of additional replication origins promoted increased biomass production.ConclusionsBalanced cell growth and physiological stability of E. coli under rapid growth condition are affected by changes in the position and number of replication origins. Additionally, we show that, for the first time to our knowledge, the introduction of replication initiation sites to the chromosome promotes cell growth and increases protein production.

Surgical treatment of limbal dermoid: Reports of cases

Ocular dermoid occur in an unusual and sporadic manner, being considered a congenital alteration, characterized by the presence of tissue of ectopic origin in ocular structures. They can develop in several places, including eyelids, conjunctiva, cornea and nictitating membrane. The objective was to report the cases of two dogs referred to the specialized service of veterinary ophthalmology, presenting tearing and ocular secretion secondary to the dermoid in the temporal limbus region, with extension to the cornea or conjunctiva. The dermoid were surgically treated by resection through conjunctivectomy combined with superficial lamellar keratectomy and corneal polishing with a diamond burr. Surgical excision of dermoid was curative.

Acromegaly caused by a GHRH-producing pancreatic neuroendocrine tumor: a rare manifestation of MEN1 syndrome.

SummaryMultiple endocrine neoplasia type 1 NM_001370259.2(MEN1):c.466G>C(p.Gly156Arg) is characterized by tumors of various endocrine organs. We report on a rare, growth hormone-releasing hormone (GHRH)-releasing pancreatic tumor in a MEN1 patient with a long-term follow-up after surgery. A 22-year-old male with MEN1 syndrome, primary hyperparathyroidism and an acromegalic habitus was observed to have a pancreatic tumor on abdominal CT scanning, growth hormone (GH) and insulin-like growth factor 1 (IGF1) were elevated and plasma GHRH was exceptionally high. GHRH and GH were measured before the treatment and were followed during the study. During octreotide treatment, IGF1 normalized and the GH curve was near normal. After surgical treatment of primary hyperparathyroidism, a pancreatic tail tumor was enucleated. The tumor cells were positive for GHRH antibody staining. After the operation, acromegaly was cured as judged by laboratory tests. No reactivation of acromegaly has been seen during a 20-year follow-up. In conclusion, an ectopic GHRH-producing, pancreatic endocrine neoplasia may represent a rare manifestation of MEN1 syndrome.Learning pointsClinical suspicion is in a key position in detecting acromegaly.Remember genetic disorders with young individuals having primary hyperparathyroidism.Consider multiple endocrine neoplasia type 1 syndrome when a person has several endocrine neoplasia.Acromegaly may be of ectopic origin with patients showing no abnormalities in radiological imaging of the pituitary gland.

Prenatal ultrasound diagnosis and management of fetal aortopulmonary septal defects: a case series

BackgroundTo investigate the prenatal ultrasound diagnosis and management of patients with aortopulmonary septal defects (APSDs).MethodsA total of 8 fetuses with APSDs who underwent fetal echocardiography at our hospital from January 2015 to January 2019 were retrospectively included in this study.ResultsAmong the 8 fetuses, there were 4 cases of type I APSD, 3 cases were type II, and 1 case was type III. Among the 8 cases, there were 2 cases of simple APSD. There were echocardiographic characteristics that were common to all 3 types of APSD. This included defects between the ascending aorta and the trunk of the pulmonary artery in the short-axis section of the aorta, and in the three vessels and the three-vessel trachea section. Furthermore, the “V”-shaped structure confluence point of all APSD cases was positioned more forward than normal in the three-vessel trachea section. Type I APSD can be better characterized by the cross-section of the double outflow tract of the aorta and the pulmonary artery, which is close to the aortic valve and pulmonary valve. Type II APSD can be clearly diagnosed by the short-axis view of the aorta. Since the defect between the aorta and the pulmonary artery is distant from the aortic valve and pulmonary valve, the defect does not involve the bifurcation of the pulmonary artery and may be associated with an ectopic origin of the right pulmonary artery. Type III APSD is similar to a permanent arterial trunk, and the space between the ascending aorta and the trunk of the pulmonary artery is completely missing. Color and pulse Doppler showed shunt flow in the defects.ConclusionsAPSD can be diagnosed and classified by fetal echocardiography. This, together with the presence or absence of fetal intracardiac and extracardiac deformities, can provide valuable prenatal information to pregnant women and their families, which may facilitate timely diagnosis and timely surgical treatment after birth.

Variations of bronchial artery origin in 600 patients: Systematic analysis with multidetector computed tomography and digital subtraction angiography.

To identify and evaluate the spectrum and prevalence of variations in bronchial artery (BA) origin by multidetector computed tomography (MDCT) and digital subtraction angiography (DSA) in a large population with hemoptysis.From July 2008 to June 2015, data from 600 individuals with hemoptysis who underwent MDCT and DSA were retrospectively analyzed. The pattern of BA origin was investigated and classified according to distribution.A total of 1674 BAs were evaluated, 866 were right BA and 808 were left BA. Most BAs originated from the upper descending thoracic aorta, classified as orthotopic origin (n = 1464, 87.5%). Among ectopic origin BAs (n = 210, 12.5%), concavity of the aortic arch was the most common (n = 107). The most common distribution pattern was a single artery in each side (n = 262). According to our classification, Type I was most common (n = 457), including BAs originating in orthotopic fashion from the descending thoracic aorta. Type II (n = 2) was defined as BAs originating from the aortic arch or ascending aorta. Type III (not found) was defined as BAs originating from subclavian arteries, common carotid arteries, and their branch vessels. Type IV (n = 92) was Type I and II combined, Type V (n = 41) was Type I and III combined, Type VI (not found) was Type II and III combined, and Type VII (n = 8) was Type I, II, and III combined.Variations of BA origin could be systematically described in detail.

Inferior petrosal sinus sampling and stimulation with CRH: 15 years of experience in a tertiary hospital

BackgroundInferior petrosal sinus sampling (IPSS) is indicated in the diagnosis of adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome (CS), especially when the results of the initial diagnostic tests are discordant. ObjectiveTo describe the patients who underwent this invasive functional test in a tertiary hospital. MethodsThis was an observational study of a retrospective cohort of patients with ACTH-dependent CS and IPSS between 2004 and 2019. We determined their epidemiological, hormonal, radiological and functional characteristics, and evaluated their diagnostic capacity and optimal cut-off points to differentiate between Cushing's disease (CD) and ectopic Cushing's syndrome (ECS). Results23 patients were evaluated, of which 65.2% were women with the average age of 42 (36–62) years. ACTH secretion of pituitary origin was evident in 82.6% of the patients and of ectopic origin in 17.4%. Plasma cortisol, urinary free cortisol, and ACTH levels were higher in patients with ECS. Regarding IPSS, the baseline central/peripheral ACTH gradient detected 89.5% of patients with CD and after stimulation with CRH, 100%. The optimal cut-off points in the diagnosis of CD were 2.06 at baseline and 2.49 after CRH stimulation. ConclusionIPSS with CRH stimulation is a test with a high diagnostic accuracy for correctly classifying patients with CD and ECS. The cut-off points of the gradients may be different from the classic ones. Therefore, we recommend that each center perform its own evaluation.

Some Anatomical Variations of the Coronary Circulation among Sri Lankan Adults

The coronary circulation has been the centre of focus of many anatomists, pathologists, cardiologists, cardiothoracic surgeons, physiologists and even the general public especially since the introduction of coronary angiography during the 1960's. Though a large number of data regarding the coronary circulation of the Western populations are available, research about coronary circulation of the Sri Lankan population is comparatively rare. This study comprises of data from 150 autopsy specimens of adult Sri Lankans died due to non-cardiac causes. 99.3% had a tricuspid aortic valve while only in 0.7% the aortic valve was bi-cuspid. The incidence of the ectopic origin of the left and the right coronary artery was equal (1.3%), and the posterior sinus was devoid of coronary ostia. A 55.3% of the study population had a single coronary artery ostium for each main coronary artery. The corresponding figure for the left coronary artery was 91.3%. Six percent (06%) of the right coronary ostia were positioned significantly above the sinus while the corresponding figure for the left coronary ostia was higher (17.5%). The average left ventricular wall thickness for males and females were 13mm and 12mm respectively. Significant muscular bridging (in relation to at-least one coronary artery) was found in 19.3% of the study population. The study concludes that variations in the cardiac anatomy are commoner than expected. As per Kitzman chart, cardiac dimensions of the Sri Lankans are somewhat smaller compared to the dimensions of the Western populations.