Project description:Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital anomaly with a large spectrum of anatomical variations. Selective engagement of an AAOCA can present challenges during cardiac catheterization. A comprehensive understanding of the characteristics of major AAOCA can effectively assist operators for selecting and maneuvering catheters. This review outlines the recommended catheter manipulations based on the site of ectopic coronary origin. Identifying the initial course (prepulmonic, subpulmonic, interarterial or retroaortic course) is crucial for classifying each AAOCA. Besides invasive coronary angiography, coronary computed tomography angiography is frequently utilized to enhance the diagnostic assessment. Cardiac catheterization enables the use of intracoronary imaging and physiologic tools for accurately assessing the significance of AAOCA identified as at risk, mainly the anomalies associated with an interarterial course. Intravascular ultrasound is recognized as the gold standard for analyzing AAOCA with interarterial course. Optical tomography coherence imaging can be interesting to evaluate the rare AAOCA with a subpulmonic course, which are associated with ischemic symptoms or myocardial ischemia. Invasive physiological indices using pressure wires can be employed, with the caveat that their threshold values remain uncertain. Decision-making can be challenging for patients with AAOCA. Both non-invasive and invasive imaging tools are essential to support the final choice.
Project description:Organogenesis during embryonic development occurs through the differentiation of progenitor cells. This process is extraordinarily accurate, but the mechanisms ensuring high fidelity are poorly understood. Coronary vessels of the mouse heart derive from at least two progenitor pools, the sinus venosus and endocardium. We find that the ELABELA (ELA)-APJ signaling axis is only required for sinus venosus-derived progenitors. Because they do not depend on ELA-APJ, endocardial progenitors are able to expand and compensate for faulty sinus venosus development in Apj mutants, leading to normal adult heart function. An upregulation of endocardial SOX17 accompanied compensation in Apj mutants, which was also seen in Ccbe1 knockouts, indicating that the endocardium is activated in multiple cases where sinus venosus angiogenesis is stunted. Our data demonstrate that by diversifying their responsivity to growth cues, distinct coronary progenitor pools are able to compensate for each other during coronary development, thereby providing robustness to organ development.
Project description:Chronic coronary heart disease (cCHD) is characterized by atherosclerosis, which progressively narrows the coronary artery lumen and impairs myocardial blood flow. Restoration of occluded coronary vessels with newly formed collaterals remains an ideal therapeutic approach due to the need for redirecting blood flow into the ischemic heart. In this study, we investigated the effect of an active fraction isolated from Geum joponicum (AFGJ) on angiogenesis in cCHD hearts. Our results demonstrated that AFGJ not only enhanced capillary tube formation of endothelial cells, but also promoted the growth of new coronary collaterals (at the diameter 0.021-0.21 mm) in the ischemic region of hearts in rat cCHD model. Our study also indicated that the growth of new collaterals in ischemic hearts resulted in improved functional recovery of the cCHD hearts as demonstrated by ECG and echocardiography analyses. These data suggest that AFGJ may provide a novel therapeutic method for effective treatment of cCHD.
Project description:ObjectivesAnomalous aortic origin of the right coronary artery (AAORCA) may cause ischemia and sudden death. However, the specific anatomic indications for surgery are unclear, so dobutamine-stress instantaneous wave-free ratio (iFR) is increasingly used. Meanwhile, advances in fluid-structure interaction (FSI) modeling can simulate the pulsatile hemodynamics and tissue deformation. We sought to evaluate the feasibility of simulating the resting and dobutamine-stress iFR in AAORCA using patient-specific FSI models and to visualize the mechanism of ischemia within the intramural geometry and associated lumen narrowing.MethodsWe developed 6 patient-specific FSI models of AAORCA using SimVascular software. Three-dimensional geometries were segmented from coronary computed tomography angiography. Vascular outlets were coupled to lumped-parameter networks that included dynamic compression of the coronary microvasculature and were tuned to each patient's vitals and cardiac output.ResultsAll cases were interarterial, and 5 of 6 had an intramural course. Measured iFRs ranged from 0.95 to 0.98 at rest and 0.80 to 0.95 under dobutamine stress. After we tuned the distal coronary resistances to achieve a stress flow rate triple that at rest, the simulations adequately matched the measured iFRs (r = 0.85, root-mean-square error = 0.04). The intramural lumen remained narrowed with simulated stress and resulted in lower iFRs without needing external compression from the pulmonary root.ConclusionsPatient-specific FSI modeling of AAORCA is a promising, noninvasive method to assess the iFR reduction caused by intramural geometries and inform surgical intervention. However, the models' sensitivity to distal coronary resistance suggests that quantitative stress-perfusion imaging may augment virtual and invasive iFR studies.
Project description:This is a report on a 10-year-old child with anomalous origin of left coronary artery (LCA) from pulmonary artery (ALCAPA), severe pulmonary hypertension (PH), old myocardial infarction and poor intercoronary collateralization. It discusses the echocardiographic pitfalls in this particular setting and introduces a new echocardiographic view (posterior pulmonary cusp view) for visualization of the anomalous origin of LCA from the posterior pulmonary cusp (PC) in patients with ALCAPA from the PC of the pulmonary artery. We describe three echocardiographic pitfalls that can mislead the echocardiographer and two helpful hints that guide the clinician to the correct diagnosis. The survival of this child shows that limited size of left ventricular myocardial infarction and severe mitral regurgitation in early infancy can result in a life-saving pulmonary hypertension which preserves viability and function of left ventricle despite lack of intercoronary collateral arteries. After one year follow-up, she is doing well on medical treatment.
Project description:Anomalous right coronary artery from left coronary sinus can have dynamic narrowing and kinking causing symptoms of myocardial ischemia and sudden cardiac death. Surgical repair of the anomaly is required in the symptomatic patient because of risk of ischemia or ventricular arrhythmia. Asymptomatic incidentally diagnosed low-risk patients can be closely followed up with exercise restriction as per present guideline.
Project description:BackgroundAnomalous origin of a coronary artery from the pulmonary trunk is a small group of rare congenital anomalies present in up to 1% of the population. These patients, in absence of an adequate collateral supply, may present with congestive heart failure secondary to ischaemia, arrhythmia, or sudden cardiac death in up to 90% of cases within the first months of life.Case summaryWe present four cases diagnosed in adulthood over 10 years in two high-volume centres. The first patient presented with dyspnoea and orthopnoea. The second with chest pain and episodes of non-sustained ventricular tachycardia. The third patient presented during her third pregnancy with chest pain, palpitations, and arrhythmia (non-sustained ventricular tachycardia). The fourth patient presented with sudden cardiac death.DiscussionIn all cases with anomalous origin of coronary arteries, it is recommendable to consider surgical correction to avoid the progression of ischaemia, congestive heart failure, arrhythmia, and sudden death.
Project description:Coronary Artery Fistulae (CAFs) are cardiac congenital anomalies consisting of an abnormal communication of a coronary artery with either a cardiac chamber or another cardiac vessel. In humans, these congenital anomalies can lead to complications such as myocardial hypertrophy, endocarditis, heart dilatation, and failure. Unfortunately, despite their clinical relevance, the aetiology of CAFs remains unknown. In this work, we have used two different species (mouse and avian embryos) to experimentally model CAFs morphogenesis. Both conditional Itga4 (alpha 4 integrin) epicardial deletion in mice and cryocauterisation of chick embryonic hearts disrupted epicardial development and ventricular wall growth, two essential events in coronary embryogenesis. Our results suggest that myocardial discontinuities in the embryonic ventricular wall promote the early contact of the endocardium with epicardial-derived coronary progenitors at the cardiac surface, leading to ventricular endocardial extrusion, precocious differentiation of coronary smooth muscle cells, and the formation of pouch-like aberrant coronary-like structures in direct connection with the ventricular lumen. The structure of these CAF-like anomalies was compared with histopathological data from a human CAF. Our results provide relevant information for the early diagnosis of these congenital anomalies and the molecular mechanisms that regulate their embryogenesis.