Computational Assessment of Hemodynamic Significance in Patients with Intramural Anomalous Aortic Origin of the Coronary Artery Using Virtually Derived Fractional Flow Reserve and Downstream Microvascular Resistance

Anomalous aortic origin of a coronary artery (AAOCA) is the second most common cause of sudden cardiac death in young athletes. One of the hypothesized mechanisms of ischemia in these patients is the lateral compression of the anomalous artery with an intramural or interarterial course. The presence of a narrowing in the anomalous artery will cause physiologic changes in downstream resistance that should be included for computational assessment of possible clinical ramifications. In the current study, we created different compression levels, i.e., proximal narrowing, in the intramural course of a representative patient model and calculated virtual fractional flow reserve (vFFR). Models also included the effect of the distal hyperemic microvascular resistance (HMR) on vFFR. Our results were in agreement with similar FFR studies indicating that FFR was increased with increasing HMR, and that different compression levels could have similar FFR depending on the HMR. For example, vFFR at HSR:1.0-1.3 and HMR: 2.30 mmHg/cm/s is 0.68 and close to vFFR at HSR:0.6-0.7 and HMR: 1.6 mmHg/cm/s, which is 0.7. The current findings suggest that functional assessment of anomalous coronary arteries through FFR should consider the vascular resistance distal to the narrowing in addition to the impact of a proximal narrowing and provides computational approaches for implementation of these important considerations.

Patients with varying courses of single coronary artery: case series

Background Single coronary artery (SCA) is a rare congenital anomaly where blood to the heart is supplied through a common trunk. Identifying these abnormalities is important because some variants can compromise myocardial blood flow and increase risk of sudden cardiac death. Case summary We present five patients with varying Lipton Group I and Group II SCA subtypes, corroborated on multi-imaging modalities and evaluated with comprehensive non-invasive as well as invasive testing. Their clinical presentations also vary from a spectrum of asymptomatic finding to angina equivalent. The decision for definitive surgical intervention involving unroofing of the involved vessel depends largely on symptoms and evidence of myocardial ischaemia. Discussion While SCA findings are often incidental and benign, understanding the origin, branching pattern, and course of the anomalous artery has implications in prognosis and treatment. This usually involves a combination of anatomic assessment with imaging such as coronary CTA, MRA, and/or coronary angiography as well as functional assessment with invasive testing using tools like instantaneous wave-free ratio and intravascular ultrasound both at rest and with stress. Individualized treatment plans can then be made through a multidisciplinary approach involving adult congenital heart disease specialists and congenital cardiothoracic surgeons.

Clinical course and surgical outcomes in middle-age adults with anomalous aortic origin of a coronary artery

Funding Acknowledgements Type of funding sources: None. Background Patients with anomalous aortic origin of a coronary artery (AAOCA) present with a wide range of clinical manifestations, including ischemic symptoms (chest pain or dyspnea) and sudden cardiac death (SCD). Studies have identified coronary anatomic characteristics associated with a higher risk of SCD. However, most of the published literature consists of studies in adolescents and young adults. There is a paucity of data regarding outcomes in middle-aged patients. Current guidelines reveal gaps in evidence for identification of adults are at risk for SCD, and for whom surgery is beneficial. Purpose To study the clinical course and rate of major adverse cardiac events (MACE) in middle-aged adults with AAOCA based on presenting symptoms, coronary anatomy on coronary computed tomography angiography (CCTA), stress test results, and surgical management. Methods We included all patients from January 2013 to December 2019 age > 18 at our institution who were found to have AAOCA. Patients with the following were excluded to minimize confounding factors which could cause MACE: coronary artery disease (CAD) with >50% stenosis in any coronary vessel, CAD requiring revascularization, heart failure with ejection fraction <40%, history of heart transplant, and non-AAOCA congenital heart disease. All patient charts were reviewed for demographics, coronary anatomy on CCTA, presenting symptoms, rationale for pursuing stress testing and CCTA, nature of surgical interventions, post-surgical course, and MACE (cardiovascular death, myocardial infarction, and need for coronary revascularization). All patients underwent PET as well as treadmill stress testing. Results Of 19,367 patients who underwent CCTA, 47 met inclusion criteria, with median age at diagnosis of 54 and median follow-up of 48 months. No patients suffered MACE. Twenty-five patients had AAORCA and 22 had AAOLCA (Table 1). Ten patients with AAORCA and 8 patients with AAOLCA presented with ischemic symptoms and had coronary anatomy characteristics associated with higher risk of SCD, as well as ischemia corresponding to the anomalous artery on stress testing and did not undergo surgery due to personal preference. Five symptomatic patients with stress-induced ischemia corresponding to the anomalous artery underwent surgery and all achieved symptom relief over a median follow up of 5 years. Conclusion As AAOCA is a significant cause of SCD in young adults, it is compelling to observe this adult cohort in which no patients experienced MACE, including 18 symptomatic patients with high-risk anatomy and stress-induced ischemia, as well as a Class I recommendations for surgery. The results suggest that although surgery may be beneficial for symptom relief, it does not necessarily improve mortality over an intermediate follow-up period. Future studies should examine surgical outcomes in middle-aged cohorts with larger sample sizes.

Pulmonary Sequestration Second case series from Iraq

Background: Pulmonary sequestration is a lung tissue (out of function), that received its blood supply from anomalous artery and not continuous with the tracheobronchial tree. Aim: To report a personal experience in dealing with five patients with pulmonary sequestration, due to anomalous arterial supply from the descending Thoracic Aorta ,ways of diagnosis and proper surgical management. Patients and Methods: Five patients with pulmonary sequestration, admitted, investigated and surgically managed at the department of Thoracic and Vascular surgery, in the surgical sub specialties hospital of the Medical City Teaching Complex during ten years period (2010-2019). Results : Four of our patients were male, the remaining one was a female, all managed successfully by Operative ligation of the anomalous blood supply and resection of the involved segment or lobe. Conclusion: Resection of the involved segment or lobe after ligation of the anomalous artery offers the best chance of cure.

Intralobar Pulmonary Sequestration with Anomalous Artery Arising from the Celiac Trunk

Pulmonary saequestration is a rare congenital malformation characterized by a dysplastic portion of lung parenchyma supplied by an anomalous artery originating from the aorta or its branches. The worldwide incidence of pulmonary sequestration among all congenital lung malformations in children ranges from 1.5% to 6.4%. There are two main types of pulmonary sequestration according to the localization of the malformation, i.e., intrapulmonary sequestration (dysplastic tissue located inside a lobe of the normal lung) and extrapulmonary sequestration. Our case presentation aims to make physicians aware of this rare anomaly which may be difficult to diagnose because of its oligosymptomatic course prior to first presentation. We present the case of a 10-year-old girl who suffered from a second episode of prolonged pneumonia of the left lower lobe. Contrast-enhanced-computed-tomography (CT) scan of the thoraco-abdominal segment of the aorta and its branches revealed intrapulmonary sequestration localized at the left lower lobe of the lung. The intrapulmonary sequester was perfused by a large artery arising from the celiac trunk. The girl underwent open surgery with ligation of the anomalous feeding artery and atypical pulmonary resection of the affected area of the left lower lobe. Postoperatively, the child recovered without any complications.

Combined endovascular and surgical approaches to treat intralobar pulmonary sequestration: a case report

Pulmonary sequestration is a congenital abnormality of a non-functional pulmonary mass with anomalous systemic arterial supply. Surgical resection is the gold standard treatment, but it carries a risk of life-threatening haemorrhage from accidental injury of the anomalous artery. Endovascular embolisation has been introduced as a safe alternative, but does not eliminate the possibility of symptom recurrence. We report a case of a 61-year old woman with intralobar pulmonary sequestration treated with a combination of endovascular coil embolisation and surgical resection.

Primary Percutaneous Coronary Intervention in an Anomalous Left Circumflex Artery: Search for the Hidden Culprit

Congenital coronary anomalies are rarely detected in patients undergoing diagnostic coronary angiography. They pose a challenge in delay to identify the anomalous artery as well as difficulty in engaging the anomalous vessel. The present case report was of 56-years-old male patient presented with acute onset of chest pain. On evaluation, his electrocardiogram was suggestive of ST segment elevation in inferior leads. He underwent diagnostic coronary angiogram which revealed totally occluded Left Circumflex Artery (LCx) arising anomalously from right coronary osteum. Primary Percutaneous Coronary Intervention (PCI) of LCx could not be performed with routine guide catheters necessitating the use of special multipurpose guide catheter with balloon support. The case highlights the need for the knowledge of coronary anatomy and anomalous origins of coronary arteries and the selection of appropriate guiding catheters and coronary wires.

Surgical repair of anomalous aortic origin of coronary artery in adults

Anomalous aortic origin of a coronary artery is a congenital abnormality of the origin or course of a coronary artery that arises from the aorta. The surgical treatment of this anomaly is highly variable and controversial and is achieved by implementing elaborate techniques of anatomic repair or by simple coronary artery bypass grafting. This review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Two independent reviewers determined whether studies met the inclusion criteria. Eligible papers were published in English, clinical studies describing surgical repair of anomalous aortic origin of a coronary artery including coronary artery bypass in adults, and enrolled >5 patients. In the absence of multicentre trials, 7 single-center retrospective series were included, which demonstrated comparable short- and midterm outcomes of anatomic repair and coronary artery bypass in adults with anomalous aortic origin of a coronary artery. Coronary unroofing was the preferred technique when the anomalous artery has an intramural component, but there is a risk of aortic insufficiency. Anatomic repair is technically demanding and should be carried out in experienced centers. Coronary artery bypass with internal thoracic artery or vein grafts is technically straightforward with low operative risk and comparable midterm outcomes, however, long-term outcomes are unknown. Coronary artery bypass grafting is the technique of choice for older patients, in those with concomitant coronary artery disease, as a bailout procedure for failed anatomic repair, or in centers without experience in anatomic repair for anomalous aortic origin of a coronary artery.

Partial Anomalous Left Pulmonary Artery: A Case Report and Literature Review

: Hereby, the case of an 8-month-old girl diagnosed with partial anomalous left pulmonary artery (PLPA) is presented. Echocardiography and computed tomography (CT) examination demonstrated that the anomalous artery originated from the right pulmonary artery supplying the left upper lobe. PLPA is sometimes associated with tracheobronchial anomaly and congenital cardiovascular defects. A relationship between PLPA and Kabuki syndrome has also been suggested. Echocardiography and CT angiography can be used as accurate tools for identifying an anomalous left pulmonary artery and adjacent abnormal anatomic structures.