Intramural Course Research Articles

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 this study, we created different compression levels, i.e., proximal narrowing, in the intramural course of a representative patient model and calculated hyperemic stenosis resistance (HSR) as well as virtual fractional flow reserve (vFFR). Models also included the effect of the distal hyperemic microvascular resistance (HMR) on vFFR. Our results agreed with similar FFR studies indicating that FFR is 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.

Assessment of the intramural segment of interarterial anomalous coronary arteries originating from the opposite sinus of Valsalva on CT angiography

Abstract Background Of the coronary anomaly variants, an anomalous coronary artery originating from the opposite sinus (AAOCA) with an interarterial course poses the highest the risk of sudden cardiac death. Assessment of high risk anatomical characteristics can be done with Computed Tomography Angiography (CTA). High risk features are a slit-like ostium, acute angle take-off, proximal narrowing and an intramural course. For an intramural course no clear-cut CT parameters exist. Purpose To deduct new CTA criteria to identify an intramural course as well as the length of the intramural segment based on peroperative findings. Material and methods Twenty patients were included that received unroofing surgery of the right or left AAOCA between 2010 and 2019. All patients had a pre-operative CTA (0.5–1mm slice-thickness) performed. The presence of the intramural segment was measured peroperatively by the surgeon and used as indicator for CTA evaluation. Using multiplanar reconstructions, CTA images were rotated perpendicular to the horizontal plane of the aortic valve annulus and AAOCA to assess the distance between the aortic and AAOCA lumen and the shape of the AAOCA. This was done at every 2mm for the length of the intramural course as described by the surgeon (Figure 1). Results Analysis of 20 patients (40% male, AAORCA n=17, age at diagnosis AAOCA 45.6±10.5 years), showed a mean intramural length of 11.5±2.4mm at surgery. The median distance between the aortic and AAOCA lumen was 0.76mm (IQR 0.72–0.97mm) for the intramural segment. At the distal end of the intramural part (indicated by no. 5 in Figure 1), the mean distance was 1.20mm±0.27mm. The median ratio between the antero-posterior and transverse diameter of the AAOCA lumen at the distal end of the intramural part was 0.94 (IQR 0.88–0.99). Along the intramural part (Figure 1, no. 1–4) this ratio was 0.56mm±0.11mm, indicating a more flattened ostial shape along the course of the vessel. Conclusions Results indicate that an aortic to AAOCA lumen distance of ≤0.76mm on CTA is suggestive of an intramural course. A distance between the aorta and AAOCA of ≥1.2mm combined with an antero-posterior to transverse diameter ratio of 0.94 of the AAOCA indicates that the intramural trajectory has ended. Funding Acknowledgement Type of funding sources: None.

Comprehensive non-invasive and invasive functional assessment of anomalous coronary arteries with anatomical high-risk features

Abstract Background Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital disease associated with an increased risk of myocardial ischemia. In AAOCA, the occurrence of ischemia is based on the extent of a fixed and a dynamic component, each attributed to different anatomical high-risk features (i.e., acute take-off angle, slit-like ostium, proximal narrowing, elliptic vessel shape and intramural course). Coronary computed tomography angiography (CCTA) is the primary non-invasive imaging method to depict the presence and quantitatively assess anatomical high-risk features while invasive physiologic evaluation under maximal dobutamine-volume challenge is the gold standard to unravel the hemodynamic relevance of AAOCA. Methods We included all consecutive AAOCA patients with anatomical high-risk features from our prospective, open-label registry. The objective is to quantify anatomical high-risk features in the CCTA and to measure hemodynamic relevance using invasive fractional flow reserve with maximal dobutamine-volume challenge (gradually increasing dose of dobutamine max. 40 μg/kg per body weight/min, max. 3000 mL ringer lactate and max. 1mg atropine). Pathological invasive FFR was defined as FFR Dobutamine<0.80. Additionally, CCTA-data were processed to assess computational fluid dynamics (CT FFR) and intravascular ultrasound (IVUS) was used to determine minimal lumen area (MLA) during baseline and maximal stress conditions. Results A total of 11 patients were included between 05/19 and 11/2020. Mean age was 59±13 years (range: 40–79), 10 patients showed a right-AAOCA and one patient showed a single right coronary artery. All patients had either one (i.e., in 1 patient) or more than one anatomical-high risk features (i.e., in 10 patients). Mean invasive FFR Dobutamine was 0.88±0.07 with n=2 (18%) being pathological. Mean non-invasive CT FFR was 0.89±0.04 (FFR<0.80; n=0), mean invasive FFR Adenosine was 0.92±0.06 (FFR<0.80; n=1; 9%). There was a significant decrease in IVUS MLA between rest (7.93±2.79mm2) and under dobutamine-volume challenge (6.57±3.20mm2, p=0.008). Mean percentage of MLA reduction was 19±18%. Conclusion Our preliminary results provide evidence that relevant myocardial ischemia seems to be often absent in a middle-aged population with AAOCA and anatomical high-risk features. However, in a minority of cases hemodynamic relevance could be depicted, especially when stressing with dobutamine-volume challenge. The presence of a dynamic component in AAOCA is represented by the reduced minimal lumen area under stress conditions. Comprehensive diagnostic evaluation should be performed to prevent unnecessary guideline recommended open-heart surgery in a middle-aged population with AAOCA. Funding Acknowledgement Type of funding sources: None.

Combined endo- and epicardial pace-mapping to localize ventricular tachycardia isthmus in ischaemic and non-ischaemic cardiomyopathy.

A high-density pace-mapping can depict an abrupt transition in paced QRS morphology from a poor to excellent match, unmasking the critical component of ventricular tachycardia (VT) isthmus from the entrance to exit. We sought to assess pace-mapping at multiple sites within the endo- and epicardial scars to identify the VT isthmus in patients with ischaemic (ICM) and non-ischaemic cardiomyopathy (NICM). Colour-coded maps correlating to the percentage matches between 12-lead electrocardiograms during VT and pace-mapping [referred to as correlation score maps (CSMs)] were analysed. We studied 115 CSMs (80 endo- and 35 epicardial CSMs) in 37 patients (17 ICM, 20 NICM). The CSM with an abrupt change (AC) in pacemap score (AC-type) on the endocardium was more frequently observed in ICM than in NICM [11/39 (28%) vs. 1/41 (2%); P = 0.001]. Among 35 CSMs that were analysed by the combined endo- and epicardial mapping, 10 (29%) CSMs exhibited non-AC-type on the endocardium; however, AC-type was present on the opposite epicardium. Although 24 (69%) CSMs did not show AC-type on both the endocardium and epicardium, 16 of them had either an excellent (>90%) or poor (<0%) correlation score on either side, associated with isthmus exit or entrance, respectively. However, the remaining eight CSMs had neither excellent nor poor scores. The CSM may provide electrophysiological information to localize the endo- and epicardial VT isthmus. The absence of AC-type CSM on the endocardium, which is frequently observed in NICM, appears to indicate the sub-epicardial or intramural course of the critical isthmus.

Echocardiographic Diagnosis of Anomalous Origin of the Left Coronary Artery From Pulmonary Artery With Intramural Course: A Single-Center Study

Echocardiographic Diagnosis of Anomalous Origin of the Left Coronary Artery From Pulmonary Artery With Intramural Course: A Single-Center Study

Anomalous aortic origin of a coronary artery: learning from the past to make advances in the future.

To review anomalous aortic origin of a coronary artery (AAOCA) anatomy, prevalence, mechanism and risk of ischemia, presentation, evaluation, management, and future directions. Although most anatomic variants of AAOCA are benign, a small number are associated with increased risk of sudden death. A complete evaluation, including the use of advanced noninvasive imaging and provocative testing should be performed on nearly every patient with AAOCA. On the basis of recent studies, the ischemic risk appears to be greatest with a left anomalous coronary artery but an anomalous right coronary artery is not benign. Other risk factors include: a left anomalous coronary with an intramural course, high take-off, or slit-like orifice, and a right anomalous coronary with a longer intramural course. Exercise restriction is rarely recommended. Management primarily consists of nonoperative care, or surgical repair in those who are symptomatic or who have high-risk variants. Surgery itself continues to evolve; however, it is not benign, with a higher than expected chance of morbidity. Advances have been made over the past decade regarding management of patients with AAOCA; however, the mechanism of ischemia and ability to predict risk is still incompletely understood. Management decisions should be based on anatomy, results of investigations, and shared decision-making with patients and their families. Surgery may be recommended for those at higher risk and should be done at centers experienced in AAOCA surgery. Future research should be collaborative in order to share experiences and insights to help advance our understanding of risk and ultimately to improve patient management.

Value of echocardiography to diagnose coronary arteries patterns in transposition of great arteries

Abnormal coronary pattern may complicate coronary transfer during arterial switch operation. The objective of this study was to evaluate the accuracy of echocardiographic to assess coronary arteries anatomy in transposition of the great arteries and determine impact on outcomes. Retrospective analysis of data in neonates with transposition of the great arteries. Preoperative echocardiographic coronary artery pattern and surgical intraoperative reports were compared. Mismatch between transthoracic echocardiography and surgical intraoperative reports, and its impact on perioperative outcome were assessed. Coronary patterns were classified in 4 groups: I = normal, II = coronary with intramural course, III = CA loop. One hundred and eight neonates who underwent arterial switch operation were included: 68 ranged in group I, 7 in group II, 32 in group III and 1 in group II + III. Intraoperative complications occurred in 10 cases. Five patients died, from coronary cause in 3 ( Table 1 ). Survival rates were 96% at 1-month. Transthoracic echocardiography and surgical intraoperative reports differed in 17.6%. Death rates were 15.8% in case of inappropriate diagnosis and 2.2% if the diagnosis was appropriate ( P = 0.0108) ( Fig. 1 ). Mortality in group II increased up to 50% in case of inappropriate diagnosis versus 0% if concordance. By multivariate analysis, an inappropriate preoperative transthoracic echocardiography diagnosis of coronary pattern was the only significant risk factor for mortality ( P = 0.04). Echocardiography can assess coronary artery anatomy in neonates with transposition of the great arteries. Intramural coronary course is often misdiagnosed. Missed diagnosis of coronary artery anomaly preoperatively may impact on perioperative mortality.

Congenital Aorto-Cardiac Connections (CACC) Revisited: Introduction of a Novel Anatomic-therapeutic Classification.

Abnormal congenital aorto-cardiac communications (CACC) are a heterogeneous constellation of anomalies that provide an abnormal connection between the aorta and other cardiac chambers or structures, including the atria, ventricles, the main pulmonary artery, and the coronary sinus. The current terminology of CACC has significant errors and shortcomings including inconsistent and interchangeable use of terms of fistula and tunnel and lack of an inclusive classification with practical information on therapeutic management. The aims of this study were threefold: firstly, to perform a concise narrative review of congenital pathologic connections between the aortic root and cardiac chambers which include rupture of congenital sinus of Valsalva aneurysm, aorto-left ventricular and less commonly right ventricular tunnels, coronary cameral fistulas, and aorto-atrial communications; secondly, to investigate the differentiating features of the so-called aorta right atrial tunnel (ARAT), with and without coronary artery take-off from the tunnel, and coronary cameral fistula (CCF) by applying a differential diagnostic assistance toolbox to two groups of patients with ARAT and CCF; and lastly, to propose a practical and inclusive anatomic-therapeutic classification for CACCs. The two main cornerstones of the proposed classification are the type of the connector between the aorta and cardiac chamber (hole versus passage) and the nature of the connecting passage ( anatomic versus extra-anatomic). We classified CACCs into three types. Depending on the intramural versus extramural course of the extra-anatomic connecting passage, type 3 is further subdivided into type 3A and type 3B.

Anomalous origin of the right coronary artery with interarterial course: red flag or innocent bystander?

Abstract Funding Acknowledgements Type of funding sources: None. Background Anomalous origin of the right coronary artery (right ACAOS) with interarterial course (IAC) has been associated with increased risk of sudden cardiac death (SCD). Widespread use of coronary computed tomographic angiography (CCTA) has led to increasing recognition of this condition, even among healthy individuals. This study sought to examine the prevalence, anatomical characteristics and outcomes of right ACAOS with IAC in patients undergoing CCTA for all-indications. Methods We conducted a retrospective analysis of consecutive patients referred for CCTA at one tertiary hospital between January 2012 and December 2020. Right ACAOS patients with IAC were analyzed for cardiac symptoms (anginal chest pain, syncope, aborted SCD) and long-term outcomes were evaluated for myocardial infarction, ischemic test results, revascularization procedures and all-cause or cardiovascular (CV) mortality. CCTAs were reviewed for proposed high-risk features (ie., take-off angle, length and severity of proximal narrowing, intramural course, interarterial length) and concomitant coronary artery disease (CAD). Association between high-risk features was analyzed. Long-term outcomes were evaluated. Results Among 10,928 patients referred for CCTA during the study period, we identified 28 patients (0.3% prevalence) with right ACAOS and IAC. Mean age was 55 ± 17 years, 64% were male and 11 (39.3%) presented cardiac symptoms. During a median follow-up of 44.1 ± 31.8 months, there were no CV deaths and only 1 patient (3.65%) underwent surgical revascularization. Baseline characteristics and CCTA findings are presented in figure 1. Conclusion Right ACAOS and IAC is an uncommon finding, with an observed prevalence of 0.3%. CCTA provides excellent anatomical characterization of anomalous vessels, including suggested high-risk features. In a population of asymptomatic patients who survived this condition well into adulthood, the risk of events was very low and medical follow up might be a reasonable option.

Non-invasive And Invasive Functional Assessment Of Anomalous Coronary Arteries With Anatomical High-risk Features In A Middle-aged Population

Introduction: Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital disease associated with an increased risk of myocardial ischemia. Coronary computed tomography angiography (CCTA) is the primary non-invasive imaging method to depict the presence and quantitatively assess anatomical high-risk features (i.e., acute take-off angle, slit-like ostium, proximal narrowing, elliptic vessel shape and intramural course), while invasive physiologic evaluation under maximal dobutamine-volume challenge is the gold standard to unravel the hemodynamic relevance of AAOCA.

Algorithmic Approaches in Diagnostic Decision Support for Combined Use of Speckle-Tracking Echocardiography and Coronary Angiography in Patients with Coronary Artery Disease

Background. Improving methods of coronary artery disease (CAD) diagnosis involves determining the most informative and less expensive methods. Speckle tracking echocardiography (STE) is now widely used for early myocardial ischemia diagnosis, allowing better verification of ambivalent results of coronary angiography (CAG).&#x0D; The aim. To develop algorithmic approaches to early CAD diagnosis through the combined use of STE and CAG in order to increase accuracy and reduce the cost of diagnosis and treatment of CAD.&#x0D; Methods. We examined 568 patients (425 [74.8%] men and 143 [25.2%] women) aged 62.3±8.9 years admitted to our center for diagnosis verification and establishing indications for coronary interventions. All the patients underwent CAG directly before or after STE.&#x0D; Results. Through both methods no pathology was detected in 5.2% of men and 16.8% of women. In 2.6% of men and 4.2% of women, STE found no regional strain disorders in the absence of significant alterations in coronary arteries (CA) according to CAG. Such false-positive STE results may witness of: 1) insignificant myocardial ischemia due to congenital distal coronary branches tortuosity or intramural (I/M) CA course, as well as myocardial ischemia due to microcirculation disorders not diagnosed by CAG; 2) non-coronary myocardial dysfunction. In 1.2% of men and 4.2% of women, CAG found significant or insignificant (up to 50–70%) atherosclerotic stenotic lesions in the absence of data suggestive of myocardial ischemia by longitudinal strain “drop” during STE. Such false-negative STE results may witness of high coronary reserve due to advanced collateral circulation in viable myocardium in ischemic coronary area. Myocardial ischemia according to both coronary beds visualization through CAG, as well as according to characteristic regional strain decrease revealed by STE, was found in 91.1% men and 74.8% women, witnessing of high correlation of the results obtained through both methods. Such results show coincidence of signs of hemodynamically significant CA stenoses according to both methods, including I/M CA segments course.&#x0D; Conclusions. Combination of CAG and STE in patients with CAD allows better determination of CA lesions hemodynamic significance, including I/M CA segments course, and to use STE as a non-invasive imaging technique in CAD, including pharmacological stress test. Combined CAG and STE use with integrated analysis thereof allows identification of coronary circulatory disorders that could not be detected separately, helping to increase diagnostic accuracy and to improve cost-effectiveness of diagnosis and treatment.

Cystoscopic-guided laser ablation for treatment of ectopic ureteroceles in 2 female dogs

Two female intact Labrador Retriever dogs (6 and 3 months of age, respectively) presented with a history of urinary incontinence. In both dogs, abdominal ultrasound revealed evidence of a unilateral ectopic ureterocele. Diagnosis of ureteral ectopia was established urethrocystoscopically by visualization of the ureteral orifice in the urethra, and an intramural course was confirmed via retrograde contrast fluoroscopy. Ectopic ureteral orifices were stenotic in both dogs. Cystoscopic- and fluoroscopic-guided laser ablation of the ectopic ureter were performed with a Hol:YAG laser. Following the procedure, both dogs were fully continent without any medical treatment. Cystoscopic- guided laser ablation of ureteroceles was effective and safe in these 2 dogs. Thus, this minimally invasive technique for the treatment of ectopic ureteroceles provides an alternative to surgical intervention.

Accuracy of computed tomography angiography and structured reporting of high-risk morphology in anomalous aortic origin of coronary artery: comparison with surgery.

Morphological features including interarterial course, intramural course, high ostial location and slit-like ostium are presumed risk factors for sudden cardiac death in children with anomalous aortic origin of the coronary artery (AAOCA). To facilitate clinical risk stratification, the diagnostic accuracy of CT angiography for individual risk factors in the setting of AAOCA must be established. We assessed diagnostic accuracy of standardized CT angiography interpretation for morphological characteristics that might determine risk in children with AAOCA by comparing them to surgical findings. We created a standardized protocol for CT angiography of AAOCA and retrospectively evaluated diagnostic performance in 25 consecutive surgical patients. Relevant morphological variables in AAOCA were assessed by three independent blinded readers, with surgery as the reference standard. We used Cohen kappa coefficients and accuracies to assess agreement between readers and surgical findings, and we calculated intraclass correlation coefficients to compare length of the intramural course. CT angiography correctly identified AAOCA in all patients. For the three readers, accuracies for detecting ostial stenosis were 84%, 94% and 96%; for high ostial origin, accuracies were 76%, 78% 82%; for intramurality using the peri-coronary fat sign, accuracies were 98%, 96% and 92%; and for intramurality using oval shape of coronary artery, accuracies were 98%, 94% and 92%. The intraclass correlation coefficients (ICCs) for predicting intramural length among the three readers were 0.67, 0.75 and 0.81 using peri-coronary fat, and 0.69, 0.50 and 0.81 using oval shape, respectively. CT angiography reliably identified AAOCA in all children and detected the presence of intramurality with high accuracy.

Coronary artery bypass grafting for an anomalous origin of the right coronary artery: is it a valid surgical procedure?

The right internal thoracic artery to the right coronary artery bypass with ligation of the proximal native vessel is a simple and reliable option for the treatment of an anomalous aortic origin of the right coronary artery arising from the left sinus of Valsalva without an intramural course. Coronary artery bypass grafting is an uncomplicated option for elderly patients, those with connective tissue diseases, and those for whom combined aortic valve procedures are planned. Herein, we present four cases of this anomaly that underwent right internal thoracic artery anastomosis to the distal right coronary artery along with proximal right coronary artery ligation using a surgical clip. There was no occurrence of complications such as hypoperfusion syndrome, graft occlusion, recurrent symptoms, or late cardiac events.

Clinical Outcome of Patients with Transposition of the Great Arteries and Intramural Coronary Artery.

To review the early and intermediate outcomes of patients with transposition of the great arteries (TGA) and intramural coronary artery (IMCA) who underwent arterial switch operation (ASO) at our center. Among 450 patients with TGA who underwent an ASO between 2010 and 2018, 26 (5.8%) patients were identified with IMCA. The left coronary artery was intramural in 21 of 26 patients. We adopted coronary transfer using double coronary buttons with unroofed intramural course for all 26 patients. Early mortality for patients with IMCA was 3 of 26 (11.5%) compared with 10 of 424 (2.4%) for those without IMCA (p = 0.007). Six patients suffered major adverse events, including extracorporeal membrane oxygenation support in 3 patients, delayed sternal closure in 6 patients. The follow-up was available for all 23 survivors, with the mean follow-up period of 73.5 ± 28.7months. There was no late death and reinterventions, and all patients were asymptomatic at last follow-up. One patient exhibited moderate neopulmonary regurgitation, and 1 patient presented with distal stenosis of the right pulmonary artery. Coronary transfer using double coronary buttons with unroofed intramural course was a good option for patients with TGA and IMCA. With this technique, ASO could be performed with optimal early and intermediate outcomes.

Diagnosis and prognostic significance of anomalous origin of coronary artery from the opposite sinus of Valsalva assess by dual-source coronary computed tomography angiography.

BackgroundClinically recognized coronary anomalies in adults are infrequent and characteristically establish on autopsy. The clinical importance coronary arteries consist of those with anomalous origin of the coronary artery from the opposite sinus of Valsalva (ACAOS) with an inter-arterial course (IAC). We have endeavored to attribute variable risk based on morphological appearances of the ACAOS by dual-source coronary computed tomography angiography (DSCTA).Material and methodsACAOS patients who undergoing DSCTA over a 5-year period were identified and assess morphologic characteristics. Medical records were reviewed for major adverse cardiovascular events (MACEs).ResultsA total of 1126 patients who undergoing DSCTA were evaluated. Twenty-four patients with ACAOS with IAC were recognized with a prevalence of 2.1% of study population. Twenty patients had anomalous origin of right coronary artery from left sinus of Valsalva (RCA-LSV) and 4 patients had anomalous origin of left coronary from right sinus of Valsalva (LCA-RSV). A significant increased prevalence of MACEs in were observed in intramural, slit-like and high inter-arterial course morphology group. Median follow-up time was 13 months (IQR 3–18 months). During follow-up 2 died, 4 had PCI, 13 had myocardial infarction and 7 had surgical treatment.ConclusionsIntramural, slit-like and high type inter-arterial course morphology of ACAOS with IAC are high risk features for MACEs which can be identified by DSCTA.

Surgical management of anomalous origin of coronary artery from pulmonary artery.

BackgroundAnomalous origin of coronary artery from pulmonary artery (AOCAPA), as is evident from the name, is defined as abnormal origin of either coronary artery from the pulmonary artery. The consequences vary in most cases and these anomalies lead to severe coronary hypo-perfusion and ventricular dysfunction. The common variants of this cardiac malformation are an anomalous origin of a left coronary artery from a pulmonary artery (ALCAPA) and anomalous origin of the right coronary artery from a pulmonary artery (ARCAPA). Another rare variant is left main coronary artery atresia that resembles ALCAPA in its mode of presentation. This article presents a single surgeon experience of managing this complex subset of the coronary anomaly from April 2006 to July 2019.Material and methodsThe 105 patients, who underwent surgery for AOCAPA from April 2006 to July 2019, have been included in the study. The patients have been analysed by follow-up echocardiography and electrocardiography (ECG) at our hospital by paediatric cardiologists. Out of 105 patients of AOCAPA, 98 (93.3%) patients underwent ALCAPA repair, of which 59 (60.2%) were males and 39 (39.7%) were females. Four out of five patients, who had an anomalous origin of the left coronary artery from the right pulmonary artery (ALCARPA), had an intramural aortic course. Three patients (3%) had left main coronary artery atresia and four patients (4%) had ARCAPA. It may be mentioned that seven infants (7.14%) and one adult patient (1%) underwent concomitant mitral valve repair. All the patients with ALCAPA, left main coronary artery atresia and ARCAPA, and 1 of the patients with ALCARPA, underwent coronary relocation. In four out of five patients with ALCARPA, unroofing of intra-aortic intramural course was performed.ResultsOut of 105 patients of AOCAPA, 9 (8.5%) patients had in-hospital mortality. Five infants (5.0%) with ALCAPA and one patient (1%) with ALCARPA died in the post-operative period due to severe left ventricular dysfunction, mitral regurgitation (MR) and sepsis. One adult patient (1%) with ALCAPA, who underwent coronary relocation using in situ trap door technique and mitral valve (MV) repair, died due to massive intracranial bleeding. Two patients out of three (66.6%) with left main coronary atresia died in intensive care unit (ICU) after 3rd and 4th postoperative day, due to low cardiac output, severe ventricular dysfunction and severe MR. Patients were followed up for a median 5.9 years. Seven patients were lost to follow-up, including the sole survivor of left main coronary atresia, after a median follow-up of 4 years after surgery. Three patients underwent mitral valve replacement for progressive residual MR. There has been no late mortality.ConclusionAOCAPA is a rare congenital cardiac anomaly, which usually presents in infancy with left ventricular dysfunction and mitral valve regurgitation. Early diagnosis and surgical re-establishment of the dual coronary system has given gratifying results, with improvement in left ventricular function in survivors. Mitral valve intervention for MR was required, in both early and late phases.

High-Risk Congenital Coronary Abnormalities in Patients with Bicuspid Aortic Valve

Objective: Abnormal coronary artery origin (ACAO) from the opposite sinus with inter-arterial course of the ectopic proximal vessel is associated with the greatest potential for clinical manifestations, specifically sudden death. Data remain limited regarding the association between bicuspid aortic valve (BAV) and this potentially dangerous coronary variant reported in up to 0.6% in the general population. We investigated the frequency of this high-risk ACAO with inter-arterial course in our surgical series of BAV patients. Methods and Results: We conducted a retrospective study to identify BAV patients with ACAO and inter-arterial course who underwent elective aortic valve/root surgery between 2010 and 2019 in our tertiary center. A total of 279 consecutive patients with BAV were identified. Among these, four patients (1.4%) had ACAO with inter-arterial course. Three patients had abnormal right coronary artery arising from the theoretical opposite coronary sinus with intramural course. The fourth patient presented an abnormal left coronary artery from a single coronary artery arising from the theoretical right coronary sinus with a long inter-arterial course. Conclusion: Our results from a single-center experience support high-risk ACAOS may be more frequently diagnosed in BAV patients, especially in surgical series, justifying a careful preoperative assessment.

Aortic Sinus Pouch Technique for Dextro-Transposition of the Great Arteries With a Single or Intramural Coronary Artery

The clinical results of the arterial switch operation (ASO) for dextro-transposition of the great arteries (d-TGA) are less than ideal when the anomaly is associated with an unusual coronary artery pattern, such as a single coronary artery or intramural coronary artery course. We developed a novel technique to deal with d-TGA with a single coronary pattern, including variations in which all the coronary arteries arise from a single aortic sinus. In this procedure, the coronary cuff is trimmed from the aorta together with the adjacent noncoronary sinus wall and is formed into a tubular pouch, which is then anastomosed with the pulmonary trunk in a side-to-side fashion. This technique not only enables extension of the length of the coronary trunk due to the thickness of the pouch, but also allows it to function as a "flexible joint," absorbing the undesired external force on the implanted coronary artery, thereby preventing kinking and distortion of the transplanted coronary artery. In addition, absence of any obstructing structure in the postoperative pulmonary outflow might support a favorable clinical course without the need for re-intervention. Although potential aneurysmal degeneration of the pouch or coronary malperfusion caused by compression of the pouch by the pulmonary trunk needs to be assessed with long-term follow-up, we believe that our technique is safe, reproducible and technically less demanding than direct coronary transplantation, making it a promising alternative technique for patients with particular coronary patterns that are difficult to deal with using the existing surgical maneuvers.

Unusual coronary artery arrangement in D-transposition of the great arteries with ventricular septal defect.

A term female with prenatally diagnosed D-Transposition of the great arteries, large membranous ventricular septal defect with inlet extension, moderate secundum atrial septal defect, and large patent ductus arteriosus (Fig1) was born by scheduled caesarean section. Transthoracic echocardiogram confirmed the anatomy with both coronary arteries arising from a single sinus with separate ostia. The right coronary artery arose from right posterior facing sinus (Fig2). The left coronary artery arose anomalously from the same sinus adjacent to the right coronary artery ostium, coursing posterior to the aorta, with brief intramural and interarterial course before bifurcating into the left anterior descending and left circumflex coronary arteries (Figs3 and 4). As a result of this unique coronary pattern, she underwent unroofing of the intramural left coronary artery noted on opening the aortic root to the coronary ostium. Both coronary buttons were harvested and this large button was then divided into two buttons. The left coronary artery button was implanted with a trapdoor technique, right coronary artery button was implanted, and the remainder of the arterial switch procedure along with LeCompte maneuver was completed uneventfully, with closure of the atrial and ventricular septal defects. The post-operative course was uneventful and the patient was discharged on the seventh post-operative day. At discharge, the patient had normal biventricular systolic function, no residual intracardiac shunt, and robust antegrade flow in the reimplanted coronary arteries. The patient was growing well at the fourth month post-operative visit with normal biventricular function, patent coronaries, and outflow tracts.