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Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA) typically presents in infancy; however, there are cases of patients who survive the infant period and present later in life. We aimed to characterize patients with late ALCAPA diagnoses and to assess perioperative and functional outcomes. A retrospective chart review of patients who underwent ALCAPA repair between 1996 and 2020 at Boston Children's Hospital was performed. This cohort was divided into early ALCAPA (< 1 year) and late ALCAPA (≥ 1 year) groups. Perioperative data were collected. Longitudinal functional assessments were made by echocardiography, exercise stress test, and cardiac magnetic resonance imaging. The median age of the late ALCAPA group was 7.6 years with 25% (6/24) of patients over 18 years. The late ALCAPA group was more likely to present as an incidental finding (63%) and required less preoperative intervention compared to the early group. On preoperative echocardiogram, the late ALCAPA group had less moderate or severe mitral regurgitation (16.7% vs 62%, p < 0.001) or left ventricular dysfunction (16.7% vs 89%, p < 0.001) compared to the early group. Reoperation was uncommon, and both groups demonstrated almost complete resolution of mitral regurgitation and left ventricular dysfunction over time. There are important differences between late and early ALCAPA subtypes. Revascularization results in excellent outcomes in both early and late groups, but long-term surveillance of ALCAPA patients is warranted as they may have functional deficits after repair. [ABSTRACT FROM AUTHOR]

Copyright of Innere Medizin (2731-7080) is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Case report: A six-year-old child who had undergone cardiac surgery five years ago presented to us with severe mitral valve regurgitation. During her current surgery, the coronary artery was injured during the attempt to release extensive epicardial adhesion, resulting in very poor contractility that prompted a delay in the intended valve repair. The injured coronary vessels were successfully repaired, yet low cardiac output syndrome persisted during perioperative care, necessitating further investigation of the coronary problem. It was later discovered that the patient had anomalous left coronary artery from pulmonary artery (ALCAPA) syndrome. Discussion: The presence of severe mitral regurgitation, pulmonary hypertension, and anatomical factors may have contributed to the delayed presentation and diagnosis in this case. The severity of mitral regurgitation and the most likely underlying mechanism indicates a low possibility of recovery following coronary repair alone, warranting the need for concomitant mitral surgery. Coronary and mitral repair were performed in this patient, resulting in a favorable outcome. Conclusions: The management of ALCAPA presents unique challenges, especially in cases with delayed diagnosis. Proper diagnosis and tailored surgical approaches are crucial for achieving favorable outcomes in patients with ALCAPA. [ABSTRACT FROM AUTHOR]

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA syndrome) is a rare congenital coronary abnormality carrying a poor prognosis. We present a clinical case series of ALCAPA-related anterior mitral valve leaflet prolapse and discuss its mechanism as a key to suspicion of ALCAPA syndrome. [ABSTRACT FROM AUTHOR]

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease, presenting either in isolation or with other cardiac anomalies. The most common presentation is congestive heart failure in infancy, though some may remain asymptomatic till adulthood. A 5-month-old infant with congestive heart failure was diagnosed to have ALCAPA with severe Left ventricular (LV) dysfunction. Unlike the more common variants where the left coronary artery (LCA) arose from the facing sinuses, the LCA arose from the left anterior nonfacing sinus of the pulmonary artery at a considerable distance from the left aortic sinus. This made direct reimplantation of LCA to the aorta challenging. LCA was reimplanted to the aorta using an extrapulmonary baffle made of an autologous pulmonary flap. The postoperative period was uneventful. The LV contractility improved during the follow-up. Early diagnosis and intervention with a novel surgical technique prevented irreversible cardiac complications and mortality. [ABSTRACT FROM AUTHOR]

Background: Anomalous left coronary artery originating from the pulmonary artery (ALCAPA), is a unique congenital anomaly, comprising only 0.24–0.46% of all congenital cardiac anomalies. Late presentations, ranging from asymptomatic cases to sudden cardiac arrest, are exceptionally rare. This unique case highlights the complexity of managing ALCAPA in adulthood and underscores the necessity of a comprehensive surgical approach addressing both coronary and valvular issues. Presentation: A 34-year-old female patient, who had been diagnosed with ALPACA in her early childhood, suffered sudden cardiac arrest at work, followed by out-of-hospital resuscitation. The patient had been followed-up regularly until adolescence, however, she had refused surgery. In the diagnostic work-up following successful resuscitation severe mitral valve regurgitation was additionally revealed. Subsequently, the patient underwent surgery, involving coronary artery bypass grafting (CABG), using bilateral internal mammary arteries, and mitral valve repair, with an excellent postsurgical result. At 16-month follow-up, the patient was asymptomatic and quality of life had significantly improved. Conclusion: This rare case initially presented as silent myocardial ischemia, resulting in reduced left ventricular function and secondary mitral incompetence. Surgical treatment of ALCAPA in adults poses greater challenges and a higher risk than in children. CABG procedure offers an excellent prognostic therapeutic strategy, since this procedure is a routine in adult cardiac surgery. [ABSTRACT FROM AUTHOR]

Objective: To demonstrate the capabilities of modern imaging modalities in diagnosis of Bland-White-Garland (BWG) syndrome (anomalous origin of the left coronary artery [AOLCA] from the pulmonary trunk) and surgical planning in a 7-year-old child.Materials and methods: We report a case of 7-year-old patient S. with a newly diagnosed complex congenital heart defect: AOLCA from the pulmonary trunk. To establish a diagnosis and assess the spatial anatomy of great vessels and the coronary bed, the patient underwent transthoracic echocardiography (TTE), coronary computed tomographic angiography (CCTA), and conventional coronary angiography. Cardiac magnetic resonance imaging (MRI) with delayed contrast enhancement was used to assess the morphological and functional parameters of the heart and fibrotic changes in the myocardium of the left ventricle (LV).Results: During TTE, a congenital heart defect was suspected: a coronary artery anomaly with LV systolic dysfunction and coronaryright ventricular fistulas. Preoperative CCTA detected AOLCA from the pulmonary trunk or BWG syndrome, whereas coronary-right ventricular fistulas were not confirmed. Based on the cardiac MRI findings, we diagnosed diffuse LV myocardial fibrosis (increased extracellular volume, 35%-37% in all segments) with areas of delayed intramyocardial accumulation, areas of focal fibrosis without signs of myocardial edema. The CCTA findings were confirmed by conventional coronary angiography and intraoperatively.Discussion: The technical capabilities of modern minimally invasive cardiac imaging modalities make it possible to determine all existing surgical risks at the preoperative stage and further prognosis in patients undergoing reimplantation for coronary artery anomalies.Conclusions: The algorithm for management of patients with suspected BWG syndrome should be determined based on the capabilities and safety of each cardiac imaging modality. Detailed and accurate assessment of coronary anatomy is a key criterion for choosing a diagnostic tool at the preoperative stage in patients undergoing reimplantation of coronary arteries. Preoperative cardiac MRI with assessment of severity of fibrotic changes in the myocardium should be used to plan management of patients with BWG syndrome and further control dynamics of morphological and functional parameters of the heart after reimplantation of coronary arteries.

Bland-White-Garland syndrome otherwise known as anomalous left coronary artery from the pulmonary artery is classified as a rare congenital heart defect which can be fatal, if unrecognized and untreated. In most cases, the disease occurs in the neonatal age, while a small percentage of patients remain asymptomatic or sparsely symptomatic for many years, making early diagnosis and treatment difficult and delayed. In the following paper, we present a case of a 39-year-old Caucasian woman with no past medical history or systematic cardiac treatment who was admitted to the cardiology clinic with escalating symptoms of heart failure. Laboratory tests revealed an increase in NT-proBNP without elevation of the myocardial necrosis markers. Echocardiography showed generalized wall hypokinesis, left ventricular enlargement with features of hypertrophy, and additional flow in the pulmonary trunk. The patient was referred for further imaging examination to make a definite diagnosis, with a suspicion of patent ductus arteriosus. [ABSTRACT FROM AUTHOR]

Symmetrical peripheral gangrene is an unusual complication of extracorporeal membrane oxygenation (ECMO) which can arise due to low cardiac output state, high inotropic support, inadequate anticoagulation, disseminated intravascular coagulation, and sepsis. In symmetrical peripheral gangrene, there is no evidence of large vessel occlusion or vasculitis. It can be due to the absence of pulsatile flow in the peripheral arteries that result in stasis and microthrombi formation which causes occlusion of small arterioles. A 10-month-old male child who underwent anomalous left coronary artery from pulmonary artery repair and required central venoarterial ECMO support post-surgery due to severe myocardial dysfunction developed symmetrical peripheral ischemia with gangrenous changes in the right-hand fingers while on extracorporeal membrane oxygenation (ECMO). The limb ischemic changes improved to normal after the child convalesced from heart failure. We describe these changes and the management here. [ABSTRACT FROM AUTHOR]

Bland White Garland Syndrome is a very rare congenital heart defect in which the left coronary artery arises abnormally from the pulmonary artery. We present an extremely rare case of Bland White Garland Syndrome with an aneurysm of the left coronary artery in a 14-year-old Afghan girl. The patient was asymptomatic throughout her life except for one attack of exertional chest discomfort. The diagnoses of these anomalies were established by electrocardiography, echocardiography, coronary angiography, and computed tomography of the chest. During her hospital stay and on discharge, she had a stable condition and was referred for surgical management in an advanced setting abroad. Anomalous origin of the left coronary artery from the pulmonary artery may coexist with an aneurysm of the left coronary artery and not exhibit symptoms until adolescence. [ABSTRACT FROM AUTHOR]

A 18-months-old male child diagnosed with anomalous origin of left coronary artery from the left posterior pulmonary sinus underwent the modification of the "trapdoor" technique, wherein the anomalous left coronary artery was detached from the pulmonary arterial sinus, and combined aortic and pulmonary arterial flaps were used to augment its length. The postoperative recovery was uneventful. [ABSTRACT FROM AUTHOR]

ALCAPA syndrome, or anomalous left coronary artery from the pulmonary artery, is a rare condition that can lead to sudden cardiac death if left untreated before the age of one. This article presents a case study of a 41-year-old woman who experienced an out-of-hospital cardiac arrest and was diagnosed with ALCAPA. The patient underwent emergency coronary angiography and coronary computed tomography angiography, which confirmed the presence of ALCAPA. The patient was then referred to a surgical center for treatment. [Extracted from the article]

Objective: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), also known as Bland- White-Garland syndrome, is a rare congenital condition which can manifest as various cardiac symptoms. Case presentation: A 64-year-old male patient presented with functional class deterioration and typical angina to hospital. He was stratified by an ischemia-induced study and underwent coronary angiography that found to have ALCAPA. Conclusions: This is a rare case of ALCAPA due to the patient's age. Survival to adulthood is possible due to collateral circulation, though the cost is the presence of heart failure, and wherein due to the high surgical risk, in this case optimal medical therapy was decided. [ABSTRACT FROM AUTHOR]

A rare clinical case of malformation of the coronary arteries is considered – anomalous left coronary artery from the pulmonary artery (Bland-WhiteGarland syndrome) in a 61-year-old patient. Detailed visualization in the form of angiograms and multispiral-CT, including those after surgery, is presented.

The colorful term "coronary steal" arose in 1967 to parallel "subclavian steal" coined in an anonymous 1961 editorial. In both instances, the word "steal" described flow reversal in the setting of an interconnected but abnormal vascular network—in one case a left subclavian stenosis proximal to the origin of the vertebral artery and in the other case a coronary fistula. Over time, the term has morphed to include a larger set of pathophysiology without explicit flow reversal but rather with a decrease in stress flow due to other mechanisms. This review aims to shed light on this phenomenon from a clinical and a pathophysiological perspective, detailing the anatomical and physiological conditions that allow so-called steal to appear and offering treatment options for six distinct scenarios. [ABSTRACT FROM AUTHOR]

The research area covers the border zone between the Central and Outer Carpathians. The purpose of this research was the interpretation of this zone based on a gravitational survey. This survey was integrated with the results of surface mapping, a deep seismic survey, and deep drillings. Three major tectonic units are located in this area: the Outer (Flysch) Carpathians, the Pieniny Klippen Belt (PKB), and the Central Carpathians. All three units contain a significant amount of flysch sequences. The lowering of the Bouguer anomaly value from north to south reflects the dip of the crystalline European Plate; in turn, the renewed increase in value correlates very well with the emergence of the crystalline ALCAPA Plate. The range of variability of the Bouguer anomaly value largely masks smaller anomalies in amplitude originating from smaller geological structures. Only three anomalies with significant horizontal extent and greater amplitudes are visible: two are clearly correlated with the Orava-Nowy Targ Basin and the third anomaly is likely connected with the thicker pile of the Outer Carpathian flysch. To separate the boundaries of geological or tectonic structures (lineaments), a horizontal derivative (THDR) and an analytical signal (ASA) were used. Both methods allowed us to confirm existing geological and tectonic boundaries (lineaments) and to identify new ones. [ABSTRACT FROM AUTHOR]

Copyright of Ümraniye Pediatri Dergisi is the property of KARE Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Introduction: ALCAPA - ‘Anomalous Left Coronary Artery from the Pulmonary Artery’, is a rare congenital cardiovascular anomaly characterized by the ‘left coronary artery’ originating from the ‘pulmonary artery’ in place of its normal origin, the aorta. Failing to treat this critical condition might result in myocardial ischemia, left ventricular dysfunction, and the occurrence of sudden cardiac death. Clinical findings: Exertional chest pain and fatigue for 6 months. Interventions, and outcomes: The diagnosis was established with the help of transoesophageal echocardiography and a coronary angiogram. Surgical repair by Left Coronary Artery reimplantation was done with a successful outcome. Conclusion: ALCAPA may rarely be present in adulthood. The prognosis in these cases is significantly better than in patients presenting in early childhood. [ABSTRACT FROM AUTHOR]

Congenital coronary artery anomalies are rare and represent 0.24-0.46% of all congenital heart defects. The most common is an anomaly in which the left coronary artery arises from the pulmonary artery (ALCAPA), also known as a Bland-White-Garland syndrome. Nonspecific symptoms typically appear at 2-3 months of age. We present the case of a 10-week-old boy admitted to the hospital due to cough and dyspnea, with suspicion of a respiratory tract infection with no clinical improvement after standard therapy. The chest X-ray revealed cardiomegaly. The electrocardiogram showed signs of anterolateral infarction. Echocardiography revealed signs of dilatated cardiomyopathy and color Doppler was of great value in establishing a final diagnosis. After confirming the diagnosis of ALCAPA in computed tomography angiography, the patient was operated on, with a good outcome. [ABSTRACT FROM AUTHOR]

The article focuses on a rare case of Bland–White–Garland (BWG) syndrome in a 58-year-old patient who survived without surgical intervention. Topics include the clinical presentation of BWG syndrome, diagnostic imaging findings such as echocardiography and CT angiography, and the importance of collateral circulation in preventing myocardial ischemia and prolonging life in this condition.

Key Clinical Message ALCAPA should be considered in the differential diagnosis of myocarditis, and contrast‐enhanced CT or catheterization should be considered even if coronary artery abnormalities are not detected on echocardiography.

Objective: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), also known as Bland-White-Garland syndrome, is a rare congenital condition which can manifest as various cardiac symptoms. Case presentation: A 64-year-old male patient presented with functional class deterioration and typical angina to hospital. He was stratified by an ischemia-induced study and underwent coronary angiography that found to have ALCAPA. Conclusions: This is a rare case of ALCAPA due to the patient´s age. Survival to adulthood is possible due to collateral circulation, though the cost is the presence of heart failure, and wherein due to the high surgical risk, in this case optimal medical therapy was decided.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a reversible cause of left ventricular (LV) dysfunction in infants. The LV function is expected to improve serially and return to normal by 1 year after surgical repair. The pattern of improvement in LV function has not been serially analyzed after ALCAPA repair. We report our preliminary experience with serial assessment of LV function in infants undergoing ALCAPA repair utilizing speckle tracking echocardiography. [ABSTRACT FROM AUTHOR]

Anomalous left coronary artery origin from pulmonary artery causes heart failure in infancy from ischemia and secondary mitral regurgitation. Rich intramyocardial collateralization may permit survival to adult age, where coronaries become tortuous and aneurysmally dilated. Surgery in adults involves left coronary ligation and providing a bypass graft to the left system, unlike coronary translocation adopted in infants. Unfavorable coronary remodeling in operated adults may lead to late coronary thrombotic occlusions. Two adults with markedly dilated tortuous coronary arteries showed variable remodeling after corrective intervention that impacted outcomes on follow-up. We stress the need for lifelong angiographic surveillance in older patients. [ABSTRACT FROM AUTHOR]

Weakley, J, Munteanu, G, Cowley, N, Johnston, R, Morrison, M, Gardiner, C, Pérez-Castilla, A, and García-Ramos, A. The criterion validity and between-day reliability of the Perch for measuring barbell velocity during commonly used resistance training exercises. J Strength Cond Res 37(4): 787–792, 2023—This study aimed to assess the criterion validity and between-day reliability (accounting for technological and biological variability) of mean and peak concentric velocity from the Perch measurement system. On 2 testing occasions, 16 subjects completed repetitions at 20, 40, 60, 80, 90, and 100% of 1-repetition maximum in the free-weight barbell back squat and bench press. To assess criterion validity, values from the Perch and a 3-dimensional motion capture system (criterion) were compared. Technological variability was assessed by determining whether the differences between the Perch and criterion for each load were comparable for both testing sessions, whereas between-day reliability with both technological and biological variability was calculated from Perch values across days. Generalized estimating equations were used to calculate R 2 and root mean square error, whereas Bland-Altman plots assessed magnitude of difference between measures. To support monitoring of athletes over time, standard error of measurement and minimum detectable changes (MDC) were calculated. There was excellent agreement between the Perch and criterion device, with mean velocity in both exercises demonstrating a mean bias ranging from −0.01 to 0.01 m·s−1. For peak velocity, Perch underestimated velocity compared with the criterion ranging from −0.08 to −0.12 m·s−1 for the back squat and −0.01 to −0.02 m·s−1 for the bench press. Technological variability between-days were all less than the MDC. These findings demonstrate that the Perch provides valid and reliable mean and peak concentric velocity outputs across a range of velocities. Therefore, practitioners can confidently implement this device for the monitoring and prescription of resistance training. [ABSTRACT FROM AUTHOR]

The article "An unusual association: chronic coronary syndrome and Bland-White-Garland syndrome" describes the case of a 53-year-old woman with type 2 diabetes, systemic arterial hypertension, and mixed dyslipidemia. The patient presented symptoms of angina and dyspnea when making physical efforts due to an anomaly in the origin of the left coronary artery, known as Bland-White-Garland syndrome. A successful surgical correction was performed, and the patient fully recovered. The article highlights the importance of timely diagnosis and management of this rare congenital disease. [Extracted from the article]

The present study is the first meta-analysis comparing long-term outcomes in patients undergoing correction for anomalous left coronary artery (ALCAPA) regarding concomitant mitral valve surgery. A systematic literature review was conducted to identify all relevant studies with comparative data on mitral valve surgery performed during surgery for ALCAPA correction. Predefined primary end points included mortality and mitral valve (re)operation. Echocardiographic outcomes were assessed when available (including severity of mitral regurgitation). Fifty-three relevant retrospective studies with comparative data for ALCAPA patients with concomitant mitral valve surgery were identified from the existing literature. The study population included 3,851 patients, of whom 55% were girls. The mean age at surgery was 6 months [0–99 months]. The mean follow-up after surgery was 22.7 years [6.3–37.2]. Among patients with available echocardiographic data at the time of ALCAPA correction, 283 had no mitral regurgitation (13%), 642 had mild mitral regurgitation (30%), 756 had moderate mitral regurgitation (36%), and 437 had severe mitral regurgitation (21%). A subgroup of patients who underwent mitral valve surgery (yMVS; n = 753; 19.5% of the total population) was identified. A total of 228 patients died during follow-up (5.9%), with no differences among patients who underwent vs those who did not undergo mitral valve concomitant surgery at baseline (p = ns). Among the 1,536 patients with available follow-up echocardiographic data, 519 had no mitral regurgitation (34%), 663 had mild mitral regurgitation (43%), 268 had moderate mitral regurgitation (17%), and 86 had severe mitral regurgitation (6%). Overall, during follow-up, 91 patients underwent mitral valve surgery (first MVS for 20 pts and reintervention for 71 pts, representing a 10% reintervention rate). In regression analysis, we found no association between the mean age at first surgery and the need for reoperation (p = 0.458). Mitral valve surgery is performed in about 20% of patients undergoing ALCAPA correction. Indications for MVS vary among centers, although the severity of MV regurgitation was the most common criterion (more than 50% of the yMVS group). Of yMVS patients, approximately 10% underwent a second MVS during follow-up, regardless of age at first surgery. Overall, long-term mortality in these patients is over 5%, with no evident impact related to concomitant MV surgery. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Anomalous left coronary artery from the pulmonary artery (ALCAPA) is frequently associated with significant mitral regurgitation (MR). We aim to identify surgical outcomes in patients with or without concomitant mitral intervention. METHODS All patients with ALCAPA who presented with >mild degree of MR at our institution between January 2008 and June 2020 were included in the retrospective study. MR recovery was defined as ≤mild MR at the last follow-up. RESULTS The study cohort included 101 patients. The median age at repair was 7.6 months. The concomitant mitral intervention was performed in 66 patients (65%). MR grade significantly improved at the last follow-up. The cumulative incidence of MR recovery 3 years after ALCAPA repair was 34% [95% confidence interval (CI), 19–50%) in patients with mitral intervention, compared to 59% (95% CI, 41–73%) in patients without mitral intervention (P  = 0.050). MR grade on postoperative day 1 was the predictor for MR recovery in patients with mitral intervention (hazard ratio, 0.080; 95% CI, 0.018–0.366; P  = 0.001), whereas preoperative mitral annulus diameter z -score was the predictor in patients without mitral intervention (hazard ratio, 0.480; 95% CI, 0.232–0.993; P  = 0.048). Freedom from mitral reoperation in patients with mitral intervention was 94% and 88% at 3 and 5 years after surgery, while freedom from mitral reoperation in patients without mitral intervention was 100% at both timepoints (P  = 0.177). CONCLUSIONS Despite significant MR improvement after ALCAPA repair, MR grade may not always return to normal regardless of the initial mitral management strategy, and reoperation for persistent MR is not rare. [ABSTRACT FROM AUTHOR]

The ADNP-gene-related neurodevelopmental disorder Helsmoortel–Van der Aa syndrome is a rare syndromic-intellectual disability—an autism spectrum disorder first described by Helsmoortel and Van der Aa in 2014. Recently, a large cohort including 78 patients and their detailed phenotypes were presented by Van Dijck et al., 2019, who reported developmental delay, speech delay and autism spectrum disorder as nearly constant findings with or without variable cardiological, gastroenterological, urogenital, endocrine and neurological manifestations. Among cardiac malformations, atrial septal defect, patent ductus arteriosus, patent foramen ovale and mitral valve prolapse were the most common findings, but other unspecified defects, such as mild pulmonary valve stenosis, were also described. We present two patients with pathogenic ADNP variants and unusual cardiothoracic manifestations—Bland–White–Garland syndrome, pectus carinatum superiorly along the costochondral junctions and pectus excavatum inferiorly in one patient, and Kawasaki syndrome with pericardiac effusion, coronary artery dilatation and aneurysm in the other—who were successfully treated with intravenous immunoglobulin, corticosteroid and aspirin. Both patients had ectodermal and/or skeletal features overlapping those seen in RASopathies, supporting the observations of Alkhunaizi et al. 2018. on the clinical overlap between Helsmoortel–Van der Aa syndrome and Noonan syndrome. We observed a morphological overlap with the Noonan-like disorder with anagen hair in our patients. [ABSTRACT FROM AUTHOR]

The paleostress analysis of the southeast part of the Ukrainian Outer Carpathians (UOC) was carried out with the separation and stress inversion TR method (TRM). The analysis includes site and bulk stress tensors through which the late-orogenic processes of the frontal part of UOC can be better described. In particular, three bulk compressive stress regimes dated to Miocene describe a compressional deformation that changes progressively from real to hybrid. The first stress regime is a pure compression and changes progressively to transpression, while the trend of the greatest principal stress axis ( σ 1 ) constantly remains ENE-WSW (065°), perpendicular to the main trend of the UOC. This progressive deformation spans the Late Burdigalian to Sarmatian period and reflects the driving stresses during the Alcapa and Tisza-Dacia mega-units drifting and docking with the Eastern European Platform. During the transpressional stress regime, the thrust displacements waned, and transfer (strike-slip) faults accommodated the deformation. The third stress regime is also a hybrid compression, i.e. transpression, but the greatest principal stress axis ( σ 1 ) trends NNE-SSW (029°). It reflects the post-docking deformation giving rise to mesoscale NW-WNW thrusts and transfer (strike-slip) faults. However, these thrusts and strike-slip faults have not modified the NNW-SSE rectilinear trend of the UOC. This hybrid compression outlasted the Late Miocene (Latest Sarmatian–Early Maeotian), and it is related to the separation and left-lateral movement of the Tisza-Dacia plate from the Alcapa plate. In addition, two extensional stress regimes with the least principal stresses ( σ 3 ) trending NE-SW and NW-SE have been defined. We interpret them to represent subsidiary stresses in the Carpathians' uppermost part due to orogen collapse both normal and parallel to the orogen after the Late Miocene. [ABSTRACT FROM AUTHOR]

Background: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly leading to progressive left ventricular dysfunction and mitral regurgitation. We conducted this study to investigate various measures to optimize the outcomes of surgical correction for ALCAPA.Materials And Methods: This was a single-centre, retrospective, observational study including consecutive patients operated for ALCAPA. The main outcomes evaluated were in-hospital mortality, duration of mechanical ventilation, and duration of intensive care unit (ICU) stay. Independent sample t- test and Fisher's exact test were used for the analysis of continuous and categorical variables respectively.Results: 31 patients underwent surgical correction for ALCAPA during the study duration. The median age was 7.3 months with a range of 21 days to 25 months. All patients underwent coronary re-implantation with the coronary button transfer technique. There was no in-hospital mortality, the mean duration of mechanical ventilation and ICU stay was 117.6 hours and 10.7 days respectively. Age at admission, development of acute kidney injury after surgery, lactate levels at 12- and 24-hours post-surgery, and heart rate at ICU admission and 12-hours post-surgery were significantly associated with mechanical ventilation duration longer than 48 hours. Use of a combination of levosimendan and milrinone and elective intermittent nasal continuous positive airway pressure ventilation after extubation in all patients with severe left ventricular dysfunction were helpful in preventing low cardiac output and need for reintubation post-surgery respectively.Conclusion: Surgical correction for ALCAPA by coronary re-implantation has an excellent short-term outcome. Optimal postoperative management is of utmost importance for achieving the best results. [ABSTRACT FROM AUTHOR]

Abstract Background Evidence for peritoneal dialysis catheter (PDC) usage in pediatric patients undergoing surgery for deteriorating cardiac dysfunction is lacking. This investigation explored factors associated with PDC usage and its effectiveness in children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Methods Eighty-four children undergoing left coronary artery transfer were retrospectively recruited. The primary endpoint was the postoperative ratio of the general ward/[intensive care unit (ICU)] length of stay. Univariable and multivariable analyses were fitted to assess factors related most strongly to PDC and the ratio of general ward/ICU length of stay. Results Of the 84 patients, 17 (20.2%) underwent postoperative PDC placement. Patients with extreme cardiac dysfunction [left ventricular ejection fraction (LVEF) ≤25%] were much more likely to require a PDC (OR, 9.88; 95% CI, 2.13–45.76; P = 0.003). Moreover, univariate analysis indicated that concomitant mitral repair significantly decreased the likelihood of PDC placement (OR, 0.25; 95% CI, 0.07–0.85; P = 0.026). In those with cardiac dysfunction (LVEF ≤50%), PDC use was associated with a reduced ratio of ward/ICU length of stay (B, − 1.62; 95% CI, − 2.77– -0.46; P = 0.008), as was age ≤ 12 months (B, − 1.57; 95% CI, − 2.88– -0.26; P = 0.02). At the 1-year follow-up, cardiac improvement was significantly greater in patients with PDC usage than in those without it (P

Background Anomalous coronary artery from the pulmonary artery is a rare congenital disorder with high mortality rates in infancy. Adult cases can present with life-threatening arrhythmias and sudden cardiac death. Case summary We report three cases of adults with anomalous pulmonary origin of each of the main coronary branches. The first patient with an anomalous left coronary artery from the pulmonary artery presented with an out-of-hospital cardiac arrest. The second patient with an anomalous circumflex artery from the pulmonary artery was evaluated for a bicuspid aortic valve and a suspected coronary fistula but was otherwise asymptomatic. The third patient with an anomalous right coronary artery from the pulmonary artery presented with anginal symptoms. In all cases, the diagnosis was made by cardiac computed tomography or coronary angiography. Cardiac magnetic resonance imaging was performed in all patients to guide clinical decision making on surgical or non-invasive management. All patients underwent surgical repair. In two patients, a dual coronary artery system was restored by aortic reimplantation of the anomalous coronary artery. In one patient, aortic reimplantation was unsuccessful due to poor vessel quality and the anomalous coronary artery was ligated. Clinical follow-up during 1.8–9.7 years did not show any cardiovascular complications and all patients are currently alive and asymptomatic. Discussion Anomalous coronary artery from the pulmonary artery can have various clinical presentations in adulthood. Cardiac magnetic resonance imaging is a useful modality to guide selection of patients who might have symptomatic or prognostic benefit from surgical repair. [ABSTRACT FROM AUTHOR]

The incidence of coronary artery anomalies (CAAs) is 0.2–1.2% of the population. Its paradox of being a rare entity with presentation ranging from sudden cardiac death, congestive heart failure, myocardial infarction to being clinically silent, asserts a challenge to its treating physician. Among the various major categories of CAA, we describe four different types of these anomalies in our retrospective evaluation over 2 years. They include – coronary cameral fistula with coronary aneurysm, congenital atresia of left main, anomalous aortic origin of left anterior descending (LAD) and circumflex artery (LCx) with malignant LAD course, anomalous origin of left coronary artery from pulmonary artery (ALCAPA). Although the child with ALCAPA succumbed despite every possible and available timely efforts, other patients had good postoperative recovery and a brief hospital stay. [ABSTRACT FROM AUTHOR]

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a very rare and life-threatening congenital anomaly. In the past ALCAPA was diagnosed by angiography or autopsy; however, the development of computed tomography cardiac angiography (CTCA) has allowed noninvasive evaluation of the coronary anatomy by direct visualization of the origin of the left coronary artery (LCA) from the pulmonary artery and is now considered the preferred modality in the diagnosis of ALCAPA.1 We report a case of adult type ALCAPA who presented with history of exertional chest pain and elevated blood pressure with no significant symptoms prior to presentation. Diagnosis was confirmed by CTCA. [ABSTRACT FROM AUTHOR]

Anomalous left coronary artery from pulmonary artery (ALCAPA), also known as Bland-White-Garland syndrome, is a rare cardiac disease. This condition may present with complications such as myocardial infarction, left ventricular dilatation, mitral regurgitation, and left heart failure in children. We report a case of a four-year-old boy who presented with shortness of breath, palpitations, and recurrent upper respiratory tract infections. He was diagnosed with mitral regurgitation. During the surgery, left coronary artery (LCA) was not present in its anatomical position and ALCAPA was identified. One should keep in mind the possibility of ALCAPA in presentation of mitral regurgitation in children despite not being reported in echocardiography.

The article describes the case of a 9-month-old girl who was diagnosed with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). The patient with a history of grunting and poor weight gain is evaluated for dehydration and respiratory distress. Topics covered include the results of the examination and imaging studies, the differential diagnosis and the symptoms presenting in infancy. It also discusses the management of ALCAPA and the outcome of the treatment.

A 5-month-old girl, weighing 5.3 kg, diagnosed ALCAPA underwent emergency surgery. The left coronary artery (LCA) originated from the posterior pulmonary artery (PA), and the left main trunk (LMT) was very short (1.5 mm), with moderate level of mitral valve regurgitation (MR). The distance from the origin to the pulmonary valve (Pv) was also short. A free extension conduit was created using adjacent sinus Valsalva flaps and implanted in the ascending aorta to avoid distortion of the coronary artery and the Pv. [ABSTRACT FROM AUTHOR]

BACKGROUND Currently there is no reliability data available for the isometric soleus strength test (ISST), commonly used as a monitoring tool in elite football settings. Isometric strength testing for other muscle groups, most notably the hamstrings, is utilized to identify injury risk and readiness to train/play. To profile athletes efficiently, performance practitioners require optimal measures that are reliable. The aim of this study was to investigate the test-retest reliability of the isometric strength test of the soleus and propose a standardized protocol for its use within an elite male football population. STUDY DESIGN Test-retest reliability single cohort study. METHODS Thirty elite male footballers (age = 22.8±5.0 years, height = 180.0±0.08 cm, weight = 70.57±4.0 kg) performed the ISST, through three maximum 3-second hold efforts with one minute rest between repetitions and 48 hours between tests, in each test. The test was performed mid-competitive season. All data bilaterally were checked for normality using the Shapiro-Wilk test before a Pearson's Correlations and Bland-Altman's analyses were performed. RESULTS Test-retest reliability demonstrated high reliability for ISST bilaterally (Right: r = 0.89; Left: r = 0.79, p<0.05). The standard error of measurement (SEM) (%) was 9.09 - 12.47% and minimal detectable change (MDC) was 25.19 - 34.56 (N) for Peak Force (PF) measures of the ISST. Bilateral levels of agreement were found to be +/- 2 standard deviations (SD) of the interval of agreement bilaterally for ISST (Levels of agreement (LOA): Right: Upper 352.49 - Lower -494.76; Left: Upper 523.82 - Lower -591.30. Bilaterally no significant difference was detected between values (Right: p=0.09, CI: -153.21-10.95; Left: p=0.52, CI: -139.81-72.33). CONCLUSION The results of this study demonstrate high reliability for the ISST. The ISST displays a high test-retest reliability for assessing PF characteristics of the soleus in elite male academy footballers. This test may be beneficial for performance practitioners for profiling soleus function of athletes. [ABSTRACT FROM AUTHOR]

Introduction: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare cardiovascular disease presented with an incidence of 1 per 300000 live births. Case Presentation: In this article, we present four cases of ALCAPA in infancy. Two infants presented with respiratory distress and two with a heart murmur. Their coronary arteries were derived from their pulmonary arteries. Two of them died, one after surgical procedure and one before surgical intervention. Of remained cases, one of them had a good surgical outcome, and another was missed in the follow-up. A brief review was done on case reports of ALCAPA in children. Eighteen articles were found, including 201 pediatric cases. Conclusions: The vast majority of affected children will die within the first year of life if ALCAPA is not diagnosed and treated appropriately.

Anomalous left coronary artery from pulmonary artery (ALCAPA), also known as Bland-White-Garland syndrome, is a rare cardiac disease. This condition may present with complications such as myocardial infarction, left ventricular dilatation, mitral regurgitation, and left heart failure in children. We report a case of a four-year-old boy who presented with shortness of breath, palpitations, and recurrent upper respiratory tract infections. He was diagnosed with mitral regurgitation. During the surgery, left coronary artery (LCA) was not present in its anatomical position and ALCAPA was identified. One should keep in mind the possibility of ALCAPA in presentation of mitral regurgitation in children despite not being reported in echocardiography. [ABSTRACT FROM AUTHOR]

Background Surgical correction of an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) has been associated with excellent survival during recent years. The purpose of this study was to evaluate the effectiveness of reimplantation of the coronary artery and to investigate the recovery of postoperative cardiac and mitral valve (MV) function. Methods From 2005 to 2015, 80 patients who had ALCAPA received surgical correction. Among them, 49 were infants. The median patient age was 7.8 months. Operative strategies included reimplantation of the coronary artery in 71 patients, the Takeuchi procedure in another 7 patients, and coronary artery ligation in the remaining 2 patients. Results There were 11 hospital deaths and 2 late deaths. Six patients required intraoperative or postoperative mechanical circulatory support. A significant improvement in the ejection fraction (EF) and shortening fraction (SF) was present in all surviving patients at discharge, at a 3-month follow-up and at a 1-year follow-up. MV function improved gradually after surgical repair with no late secondary intervention. Conclusions The repair of ALCAPA can be accomplished by establishment of a dual-coronary system, which offers an acceptable mortality rate and will rarely require a second surgery. Left ventricular (LV) recovery is a progressive process, especially for infants with impaired LV function. Concomitant MV annuloplasty is safe and reliable and can be performed as necessary in patients with moderate or severe mitral valve regurgitation. [ABSTRACT FROM AUTHOR]

This is a validation study. Tracking limb movement with body worn sensors allows clinicians to measure limb dynamics to guide treatment for patients with movement disorders. The current gold standard, 3-dimensional optical motion capture, is costly, time-consuming, requires specific training, and is conducted in specialized laboratories. The purpose of our study was to a compare consumer-grade inertial sensor to a laboratory-grade sensor to provide additional methods for capturing limb dynamics. The participants wore an Apple Watch and a laboratory-grade Xsens sensor on each wrist during 3 conditions: walk, fast-walk, and run. Acceleration data were collected simultaneously on each device per wrist for all conditions. Intraclass correlation coefficients and Bland-Altman plots were calculated to measure intra- / inter device reliability, evaluate bias, and limits of agreement. Intradevice ICCs showed good reliability during walk and fast-walk (0.79-0.87) and excellent reliability during run (0.94-0.97) conditions. Inter-device ICCs yielded moderate reliability during walk (0.52 ± 0.22) and excellent reliability in fast-walk and run (0.93 ± 0.02, 1.00 ± 0.01) conditions. Bland-Altman plots showed small biases with 90% or more of the data contained within the limits of agreement. Our study demonstrates reliability and agreement between the two devices, suggesting that both can reliably capture upper extremity motion data during gait trials. Our findings support further study of consumer-grade motion trackers to measure arm activity for clinical use. These devices are inexpensive, user-friendly, and allow for data collection outside of the laboratory. [ABSTRACT FROM AUTHOR]

Disease summary: The Bland-White-Garland syndrome (BWGS) is an extremely rare congenital cardiac abnormality [28]. This syndrome was first mentioned in 1886, but the full anatomy and clinical symptoms were first described by Bland, White and Garland in 1933 [5, 6]. Within this syndrome, the left coronary artery (LCA) has an unexpected anomalous origin from the pulmonary artery instead of the aorta. Blood flow in the coronary system follows the direction of pressure gradient and takes the path of lowest resistance [14,15]. In patients with BWGS, blood flows from the normal origin of the (often enlarged and dilatated) right coronary artery (RCA) through myocardial coronary collateral vessels to the LCA and low-pressure system of the pulmonary artery system [15,25]. After the drop of pulmonary vascular resistance in the first months of life, the majority of the blood drains to the pulmonary arteries, causing a "coronary steal" phenomenon, a left-to-right shunt and ultimately leads to an abnormal left ventricular perfusion [35]. This may lead to myocardial ischaemia and infarction, left ventricular dysfunction, mitral insufficiency and regurgitation, congestive heart failure and finally to sudden cardiac death [35,47]. BWGS occurs between 0.26 % and 0.46 % of patients with congenital heart disease and appears once in 300,000 births [3,22]. It is usually seen as an isolated cardiac abnormality, but in about 5 % of cases, it appears in coexistence with other cardiac abnormalities like patent ductus arteriosus (PDA), atrial septal defect (ASD), ventricular septal defect (VSD) with or without mitral stenosis, tetralogy of Fallot, pulmonary stenosis, coarctation of the aorta or transposition of the great vessels [1,4,8,10,15,34,35,43,45,48]. Symptoms usually become clinically apparent shortly after the neonatal period [15]. Vascular resistance is equal in right (pulmonary artery) and left (aorta) system during foetal period. This condition allows the myocardium supplied by the anomalous artery to remain well perfused. Promotive at this stage of development, there is an identical oxygen content in the pulmonary artery in comparison to the aorta. For this reason, this coronary anomaly is well tolerated in foetal and early neonatal life [4,35,38]. These conditions lead to an antegrade flow in anomalous LCA as well as in normal RCA [35]. In consequence, coronary collateral growth is not especially promoted before birth [38]. When pulmonary arterial pressure decreases physiologically after birth, the antegrade LCA flow diminishes and reverses. Moreover, the antegrade LCA is filled with deoxygenated mixed venous blood [4]. Subsequent, this coronary steal phenomenon leads to ventricular (anterolateral) myocardial ischaemia and in further consequence to mitral valve papillary muscle dysfunction [15,35]. Clinical presentation of BWGS may vary but primarily goes along with symptoms of heart failure like angina-like symptoms, severe dyspnoea, tachypnoea, cyanosis, wheezing, pallor, dizziness, profuse sweating, signs of decreased peripheral perfusion, syncope and pulmonary oedema or malignant arrhythmias [13,15,17,40,47]. In children, a failure to thrive, a smaller stature, diaphoresis while drinking and feeding, reduced exercise tolerance or poor weight gain may occur [15,51]. Two types of BWGS can be differed [25,35]. The infant type applies to patients without collateral vessels. Regarding differential diagnosis in this age, dilatated cardiomyopathy of differing genesis should be considered [35]. In contrast, the adult type occurs in patients with well-established collateral vessels and possible coronary ostial stenosis. These collaterals between RCA and LCA were formed during childhood due to ischaemic stimulation. They are essential and the only way to survive beyond infancy without surgery. Despite formation of these collaterals, adult patients develop a chronic left ventricular subendocardial ischaemia over the years, with high risk of lethal ventricular arrhythmias [35]. Asymptomatic patients with BWGS presenting in adulthood are rare [21,24,28,29,44,50,51]. They must have a well-developed coronary collateral circulation with retrograde perfusion of the left ventricle from the RCA [28,38]. An ostial stenosis of the LCA or persistence of elevated pulmonary resistance might limit the steal and increasing myocardial perfusion pressure. At least the degree of collaterals determines the moment of medical consultation due to beginning cardiac decompensation [15]. Nevertheless, adult BWGS patients without performed surgical correction are at high risk for sudden death. Typical is a precipitation by exercise at an average age of 35 years in untreated patients with BWGS [25,38,50]. The definitive treatment option for BWGS is cardiac surgery [49,51]. Untreated, 90 % of patients with BWGS die within the first year of life [35,47]. In case of early diagnosis and prompt surgical intervention, patients with BWGS may achieve adulthood. This applies also for asymptomatic patients [25]. There is one case report of an 88-year-old woman with BWGS, but, due to severe cardiac impairment, this expectation of life remains a rarity in this disease [42]. Supportive and conservative measures consist of typical pharmacotherapy of congestive heart failure (e.g., diuretics, ß-blockers and in acute failure: inotropes) [12,18,44]. [ABSTRACT FROM AUTHOR]

Background: Evidence for peritoneal dialysis catheter (PDC) usage in pediatric patients undergoing surgery for deteriorating cardiac dysfunction is lacking. This investigation explored factors associated with PDC usage and its effectiveness in children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA).Methods: Eighty-four children undergoing left coronary artery transfer were retrospectively recruited. The primary endpoint was the postoperative ratio of the general ward/[intensive care unit (ICU)] length of stay. Univariable and multivariable analyses were fitted to assess factors related most strongly to PDC and the ratio of general ward/ICU length of stay.Results: Of the 84 patients, 17 (20.2%) underwent postoperative PDC placement. Patients with extreme cardiac dysfunction [left ventricular ejection fraction (LVEF) ≤25%] were much more likely to require a PDC (OR, 9.88; 95% CI, 2.13-45.76; P = 0.003). Moreover, univariate analysis indicated that concomitant mitral repair significantly decreased the likelihood of PDC placement (OR, 0.25; 95% CI, 0.07-0.85; P = 0.026). In those with cardiac dysfunction (LVEF ≤50%), PDC use was associated with a reduced ratio of ward/ICU length of stay (B, - 1.62; 95% CI, - 2.77- -0.46; P = 0.008), as was age ≤ 12 months (B, - 1.57; 95% CI, - 2.88- -0.26; P = 0.02). At the 1-year follow-up, cardiac improvement was significantly greater in patients with PDC usage than in those without it (P <  0.001), and the number of mitral recoveries was comparable between the groups (64.2% vs. 53.3%, P = 0.434).Conclusion: In cohorts with ALCAPA, PDC placement following surgery may be necessary for patients with extreme cardiac compromise, while concomitant mitral repair can probably reduce their usage rate. PDC is beneficial in conferring an improvement in cardiac and mitral performance. Importantly, after patients are transferred from the ICU, recovery efficiency in the general ward can be enhanced by PDC placement, and hospital discharge can therefore be achieved early, especially for patients younger than 12 months or with LVEF ≤50%. [ABSTRACT FROM AUTHOR]

Anomalous total coronary artery from the pulmonary artery is an extremely rare congenital coronary artery malformation. Only a few cases have been reported over the years, but with no comprehensive medical imaging data. We present the imaging findings of this case with transthoracic echocardiography, trans-esophageal echocardiography, computed tomography angiography (CTA) and cardiac catheterization. [ABSTRACT FROM AUTHOR]