Coronary Artery Anomalies.

Coronary artery anomalies (CAAs) are a group of congenital conditions characterized by abnormal origin or course of any of the 3 main epicardial coronary arteries. Although CAAs have been identified as a common underlying condition in young athletes with sudden cardiac death, the widespread use of invasive and noninvasive coronary imaging has led to increased recognition of CAAs among adults. CAAS are often discovered as an incidental finding during the diagnostic workup for ischemic heart disease. The clinical correlates and prognostic implication of CAAs remain poorly understood in this context, and guideline-recommended therapeutic choices are supported by a low level of scientific evidence. Several studies have examined whether assessment of CAA-related myocardial ischemia can improve risk stratification in these patients, suggesting that multimodality imaging and functional tests may be key in the management of CAAs. The aim of this review is to outline definitions, classification, and epidemiology of the most relevant CAAs, highlighting recent advances and the potential impact of multimodality evaluation, and to discuss current therapeutic opportunities.

Midterm follow up of transcatheter closure of coronary artery fistula with Nit-Occlud® patent ductus arteriosus coil.

BACKGROUND: Coronary artery fistula (CAF) is a rare congenital anomaly with a challenging scenario in children. This study reports our experience in transcatheter closure of CAF with Nit-Occlude PDA coil and midterm clinical and imaging follow-up. METHODS: Twelve children with congenital CAF between 2009 and 2019, mean age 2.05 ± 2.05 years (4 days to 7.2 years), mean weight 8.8 ± 4.83 (2.8-17 kg), who underwent transcatheter closure with PFM coil at the Namazi hospital, Shiraz, Iran, were reported. Echocardiography and electrocardiogram were done before and after the procedure (early, 3, and 6 months after), and Multi-slice computerized tomography or conventional coronary angiography was performed at least one year after closure. RESULTS: In a median follow-up of 5.5 years (range 13 months to 8 years), retrogradely closed fistula had no residual, and the fistula tract was wholly occluded, but in most anterogradely closed fistula, had a small residual, which made the fistula tract open and need additional coil closure. CONCLUSIONS: Transcatheter closure of CAF with PFM coil is feasible and effective with low mortality and morbidity, although antegrade closure with this device may be accompanied by residual shunt and need for multiple coil insertion.

Technical success and long-term outcomes after anomalous right coronary artery stenting with cardiac computed tomography angiography correlation.

INTRODUCTION: Anomalous origin of coronary arteries has been observed in about 0.35-2.10% of the population. Patients with anomalous right coronary artery (ARCA) may present with significant symptoms, arrhythmias or ACS, and at times sudden death. Traditionally, surgical correction has been the recommended treatment. However, these may be technically challenging, and bypass grafting for such anomalies has the potential for graft failure because of competitive flow. We sought to determine the intermediate and long-term outcomes of drug-eluting stent placement for patients with symptomatic ARCA. We also looked at angiographic findings suggestive of interarterial course as confirmed by subsequent computed tomography (CT) findings. METHODS: Between January 2005 and December 2012, we enrolled 11 patients for elective percutaneous coronary intervention (PCI) of ARCA in a single center, prospective, nonrandomized fashion. Patients were followed up in clinic at 1 week, 3 months, 6 months, and 1 year, and then annually or more frequently if needed. All patients underwent a cardiac CT, as well as functional stress testing when needed to assess for recurrence of disease. RESULTS: All 11 of our patients, who presented with significant symptomatic stenosis with an ARCA, were successfully treated with PCI. Mean follow-up duration was 8.5 years. The only two deaths during follow-up were related to noncardiac causes (sepsis), with a mortality rate of 18.2%. Two patients had a positive functional study and on subsequent coronary angiography, one of them had significant in-stent restenosis (target lesion revascularization of 9.1%) and one distal to the stent (target vessel revascularization 9.1%). We found the observation of a "slit-like lesion" on angiography to have a sensitivity of 100% and specificity of 86% for the diagnosis of interarterial course of the anomalous vessel seen on subsequent CT. CONCLUSIONS: Our study results suggest that PCI of ARCA is an effective and low-risk alternative to surgical correction, with good procedural success and long-term outcomes. It can provide symptomatic relief in such patients and may reduce the risk of sudden death in younger patients, without the inherent risks associated with surgical repair.

Recanalization of an atretic intramural left main coronary artery after bypass surgery in a pediatric patient with anomalous aortic origin of the left main coronary artery arising from the right sinus of Valsalva.

We report a pediatric patient with nonatherosclerotic chronic total occlusion (CTO) of the left main coronary artery (LMCA) leading to complete LMCA atresia which was successfully recanalized via retrograde techniques through a previous internal mammary bypass graft. After the CTO was treated, the artery was found to be anomalous off the right cusp with an intramural coarse and slit-like orifice. The patient's ischemic symptoms resolved after Percutaneous Coronary Intervention (PCI), and she has continued to do well.

Long-term outcomes of percutaneous closure of coronary artery fistulae in the adult: A single-center experience.

Coronary artery fistulae (CAF) are rare congenital cardiac abnormalities. With increasing age, patients with moderate to large fistulae are likely to become symptomatic and encounter complications. Percutaneous closure has been accepted as a safe and effective alternative to surgery; however, information regarding long-term outcomes in adult patients after transcatheter closure is limited. METHODS: We describe our 20-year experience with percutaneous closure of CAF in adult patients, focusing on long-term outcomes. RESULTS: From 1995 to 2015, 25 adult patients underwent 33 transcatheter interventions to close 26 CAF at our institution. Mean age was 51.4 ± 16.8 years, and 14 (56%) were female. All the patients were symptomatic with dyspnea (21/25, 84%) being the most common complaint followed by chest pain (17/25, 68%), palpitations (9/25, 36%), and heart failure (2/25, 8%). Two patients presented with acute coronary syndrome, whereas 10/25 (40%) also had inducible ischemia on stress test. These CAFs were classified as medium (12, 46.2%), large (5, 19.2%), and giant (9, 34.6%). Immediately postprocedure complete occlusion was achieved in 21 (21/26, 81%), and residual trace leak was observed in three fistulae, whereas we could not deliver coils/device in two cases. Periprocedural complications occurred in the form of coronary dissection (n = 1), myocardial infarction (n = 2), coil embolization (n = 1), transient ST elevation (n = 1); the majority of complications were observed in the early years of our experience. Patients reported marked improvement in symptoms after the intervention. During a mean follow-up of 76 ± 69 (5-214 months) (n = 22), 15 patients were investigated by either conventional (n = 6) or CT angiography (n = 8) or both (n = 1) that demonstrated thrombotic occlusion of a proximal coronary artery where a distal fistula was treated. Another patient was noted to have thrombus proximal to a vascular plug in a distal giant fistula. Three patients were lost to follow-up. None of the investigated patients had recanalization of their fistula. We did not observe remodeling of the native coronary artery that was feeding the fistula, even after successful complete closure. CONCLUSION: Percutaneous device closure of CAF is a feasible and effective therapeutic intervention in adult patients; however, patients with distal fistulas represent a significant challenge and the outcomes of these procedures are in question. All patients should have long-term angiographic follow-up.

Clinical characteristics in patients with rest angina and hypoplastic right coronary artery.

Hypoplastic coronary artery disease is a rare congenital abnormality reported to be associated with myocardial infarction and sudden cardiac death. Provoked positive spasm in the left circumflex coronary artery (LCX) with pharmacological spasm provocation tests was remarkably lower than other coronary arteries. We sometimes encountered patients with rest angina and hypoplastic right coronary artery (H-RCA). Among 5953 patients with diagnostic and follow-up coronary arteriography, we found 93 patients (1.6%) with H-RCA. During the same period, we could perform spasm provocation tests in 564 patients with rest angina including 13 patients with H-RCA and 249 patients with effort angina including 10 patients with H-RCA. Pharmacological spasm provocation tests were performed in 51 of 93 patients including 34 patients with ischemic heart disease (IHD) and 17 patients with non-IHD. Provoked spasm incidence in patients with IHD was higher than in those with non-IHD but not significant (52.9% vs. 29.4%, p = 0.1114). Provoked positive spasm in the LCX in patients with rest angina and H-RCA was significantly higher than that in those without H-RCA (69.2% vs. 23.4%, p < 0.001). Provoked spasm on both left anterior descending artery and LCX in patients with rest angina and H-RCA was also remarkably higher than in those without H-RCA (53.8% vs. 3.1%, p < 0,001). There were no clinical differences between patients with and without H-RCA rest angina. Two-vessel spasm (61.5% vs. 0%, p < 0.01) and LCX-provoked spasm (69.2% vs. o%, p < 0.01) were significantly higher in patients with H-RCA and rest angina than that in those with H-RCA and effort angina. In patients with rest angina and H-RCA, LCX-positive spasm was significantly higher and these patients may have a potential of high disease activity in the clinic as a coronary spastic angina.

It hurts to swallow! Pseudoachalasia resulting from attempted transcatheter occlusion of a giant congenital coronary artery fistula.

Congenital coronary artery fistula (CAF) is a rare anomaly that can cause heart failure and myocardial ischemia. In recent decades, transcatheter approaches to occlude CAF have emerged as minimally invasive alternatives to surgical ligation. Reported complications with transcatheter CAF occlusion include device embolization and dissection. We report the first case of attempted transcatheter occlusion of a giant CAF that resulted in severe pseudoachalasia.

Symptomatic right coronary anomaly with dynamic systolic intramural obliteration and isolated right ventricular ischemia.

A 52-year-old man was referred for an anomalous right coronary artery (RCA) originating from the left sinus of Valsalva with an intramural course (R-ACAOS-IM), accompanied by progressive angina and dyspnea. He had been initially advised to have surgical treatment. Computerized axial tomographic angiography showed he had an ectopic origin from the left sinus of a small RCA, with a course between the aorta and pulmonary artery. His negative treadmill nuclear stress test was prematurely terminated because of angina and dyspnea. At our institution, intravascular ultrasound imaging indicated an intramural course and critically severe phasically changing proximal stenosis (80%-100%). The stenosis was resolved with stent-angioplasty of the intramural segment. He had no complications and returned quickly to an asymptomatic state with unrestricted physical activity.

Trans-radial closure of coronary artery fistulae using Amplatzer vascular plug IV: A case series.

BACKGROUND: Trans-catheter closure of coronary artery fistulae (CAF) has become a successful alternative to cardiac surgery with excellent results and acceptable low rate of complications. Different techniques and devices are available to treat the extreme anatomical variability of CAF. AIM: We retrospectively describe our case series of five patients trans-radially treated using an Amplatzer vascular plug IV (AVP-IV) with telescoping catheter technique. RESULTS: Trans-radial closure of CAF using the "Child in Mother" technique with dedicated catheters to deliver the vascular plugs AVP-IV was successfully performed in all patients, in one case for a complex CAF, an hybrid step approach using coils and plugs was needed. CONCLUSIONS: Trans-radial closure of CAF using AVP-IV and a telescoping catheter appeared to be safe and feasible in our case series. A persistent closure of CAF was achieved in all patients at 2 year coronary angiography follow-up.

Coronary artery fenestration as rescue management of intramural haematoma with luminal compression.

Conservative management is the first-line treatment for spontaneous coronary artery dissection (SCAD) with stable haemodynamic status and thrombolysis in myocardial infarction three flow on angiography. However, in a few very specific patients, recurrent ischemia, or haemodynamic instability necessitates revascularization. Here, we describe a case of successful optical coherence tomography (OCT)-guided percutaneous coronary intervention (PCI) with a cutting balloon. We performed fenestration at multiple decompression sites prior to stenting in an intramural haematoma with luminal compression. Rescue management of SCAD with luminal compression is a critical issue, because SCAD is an emergent clinical condition affecting young patients with a severe prognosis. To our knowledge, there are no large published series evaluating procedural success rates and long-term follow-up of this technique.

Exercise-induced ventricular arrhythmia due to bilateral coronary to pulmonary artery fistulas.

Ischemic polymorphic ventricular ectopy was documented during exercise testing in a 65-year-old Caucasian male patient. Coronary angiogram revealed four coronary to pulmonary artery fistulas (CPAFs) originating from the right and left coronary artery, leading to myocardial ischemia due to steal phenomenon. The three dominant fistulas were coiled percutaneously, while one small fistula was left untreated. During follow-up, no significant residual ventricular arrhythmia was detected.

Ectopic Origin of the Left Anterior Descending Artery from the Proximal Right Coronary Artery: An Imaging Report.

Anomalies of the coronary arteries represent rare congenital disorders, which are characterized by a wide spectrum of clinical manifestations. Usually, they are asymptomatic, but sometimes they cause myocardial ischemia or sudden cardiac death. Here, we describe the case of a patient who suffered from angina. Coronary angiography revealed an ectopic origin of the left anterior descending coronary artery from the proximal trait of the right coronary artery and the left circumflex artery, originating from the left sinus; the whole coronary tree was free of atherosclerosis. To better define the coronary anatomy, we performed computed tomography angiography with a three-dimensional reconstruction. The patient was discharged from the clinic after 48 h under optimal medical treatment.

Dual-source computed tomography coronary artery imaging in children.

Computed tomography (CT) has a well-established diagnostic role in the assessment of coronary arteries in adults. However, its application in a pediatric setting is still limited and often impaired by several technical issues, such as high heart rates, poor patient cooperation, and radiation dose exposure. Nonetheless, CT is becoming crucial in the noninvasive approach of children affected by coronary abnormalities and congenital heart disease. In some circumstances, CT might be preferred to other noninvasive techniques such as echocardiography and MRI for its lack of acoustic window influence, shorter acquisition time, and high spatial resolution. The introduction of dual-source CT has expanded the role of CT in the evaluation of pediatric cardiovascular anatomy and pathology. Furthermore, technical advances in the optimization of low-dose protocols represent an attractive innovation. Dual-source CT can play a key role in several clinical settings in children, namely in the evaluation of children with suspected congenital coronary artery anomalies, both isolated and in association with congenital heart disease. Moreover, it can be used to assess acquired coronary artery abnormalities, as in children with Kawasaki disease and after surgical manipulation, especially in case of transposition of the great arteries treated with arterial switch operation and in case of coronary re-implantation.

Anomalous aortic origin of a coronary artery (AAOCA): A case report.

Anomalous Aortic Origin of a Coronary Artery (AAOCA) is a rare anomaly of the coronary artery with a considerable risk of sudden cardiac death due to ischaemia of the heart. Symptoms may include chest pain on exertion, breathlessness or dizziness. We encountered a case of a 46- year-old female who complained of exertional chest pain with a positive-stress test and subsequently diagnosed with AAOCA through CT angiography (CTA). She successfully underwent a coronary artery bypass graft (CABG) surgery using a saphenous vein graft with uneventful recovery. Right internal mammary artery (RIMA) was not used as it was flimsy and the flow was very poor.

Anomalous coronary artery originating from the opposite sinus of Valsalva (ACAOS), fractional flow reserve- and intravascular ultrasound-guided management in adult patients.

OBJECTIVES: To describe the use of fractional flow reserve (FFR) and intravascular ultrasound (IVUS) in the evaluation of patients with anomalous coronary arteries originating from the opposite sinus of Valsalva (ACAOS). BACKGROUND: ACAOS of the right and left coronary are rare, but may lead to symptoms and impose a risk for sudden cardiac death, depending on several anatomical features. Assessment and risk estimation is challenging in (nonathlete) adults, especially if they present without symptoms or with atypical complaints. METHODS: The team retrospectively studied 30 consecutive patients with ACAOS with interarterial course, who received IVUS- and FFR-guided treatment at our institution between October 2010 and September 2017. RESULTS: FFR was abnormal in only seven patients. IVUS showed the typical slit-like anatomy of the orifice in 23 patients. Based on FFR and/or IVUS results, in conjunction with the clinical presentation, clinical decision was made. A decision for intervention was made if at least two out of three entities were abnormal. Intervention implied unroofing of the coronary artery (n = 10) or coronary artery bypass grafting (n = 1). In all other patients a conservative strategy was followed. No adverse events occurred in the total population after a median of 37 (0-62) months of follow-up. CONCLUSIONS: Conservative treatment may be justifiable in adult patients with ACAOS in the presence of normal FFR and nonsuspicious symptoms, despite the presence of an interarterial course and/or slitlike orifice on IVUS. We recommend the use of FFR and IVUS in the standard work-up for adult patients with ACAOS and propose the use of a flowchart to aid in decision-making.

Anatomic spectrum of left coronary artery anomalies and associated mechanisms of coronary insufficiency.

This is a novel re-appraisal of an understudied and misunderstood group of important coronary anomalies. The general name of the group is "anomalous origin of the left coronary artery," but several additional details should be included in this group of anomalies and the explanation of their pathophysiology. The most lethal form in young athletes or military recruits features intramural aortic proximal course. This comprehensive review is based on a large experience at a dedicated center for coronary artery anomalies, using evolving knowledge (over 20 years) while employing prospective and disciplined programs of evaluation and treatment, according to the nature and severity of each anomaly. The most common pathogenic mechanism of coronary dysfunction relates to intramural coronary course, with or without ectopic origin, leading to variable lateral compression and stenosis inside the aortic tunica media; this compression is present at rest and worsens with exertion. We propose that such variable and dynamic stenoses can be best studied by examining their specific anatomy and clinical presentation, stress testing, and, most importantly, in vivo evaluation by intravascular ultrasonography. Such methods should be used to support individual risk evaluation and selection among treatment options.

An unusual case of coronary artery compression that did not preclude successful transcatheter pulmonary valve placement.

During transcatheter pulmonary valve placement, coronary compression observed during simultaneous right ventricular outflow tract angioplasty and coronary angiography typically contraindicates valve implantation. We present a unique patient with tetralogy of Fallot who underwent successful transcatheter Melody valve placement despite coronary compression observed during right ventricular outflow tract balloon angioplasty.

Chest pain in a young female.

Acute Coronary Syndrome (ACS) is a common diagnosis in the emergency department (ED), the most severe manifestation of which is ST elevation on electrocardiogram (ECG). ST elevation reflects obstruction of flow through the coronary arteries, most commonly due to coronary atherosclerotic plaque rupture. However, alternative causes of coronary obstruction causing ST elevation are possible. Spontaneous coronary artery dissection (SCAD) is an unusual cause of ST elevation in ED patients which providers may encounter in patients without traditional atherosclerosis risk factors. Patients presenting with SCAD as a cause of ST elevation require unique management from traditional ACS. Here we report a case of a 43 year old female presenting with chest pain and unusual ECG findings including accelerated idioventricular rhythm followed by subtle ST segment elevation and resolution of abnormalities. This case illustrates subtle clinical and ECG findings suggestive of SCAD which emergency physicians should consider when evaluating patients for ACS in the absence of traditional clinical presentations. Such considerations may prompt physicians to avoid therapy for coronary plaque rupture which is not indicated in patients with SCAD.

Presence of reduced regional left ventricular function even in the absence of left ventricular wall scar tissue in the long term after repair of an anomalous left coronary artery from the pulmonary artery.

BACKGROUND: We sought to assess left ventricular regional function in patients with and without left ventricular wall scar tissue in the long term after repair of an anomalous origin of the left coronary artery from the pulmonary artery. METHODS: A total of 20 patients aged 12.8±7.4 years were assessed 10 (0.5-17) years after the repair of an anomalous origin of the left coronary artery from the pulmonary artery; of them, 10 (50%) patients showed left ventricular wall scar tissue on current cardiac MRI. Left ventricular regional function was assessed by two-dimensional speckle-tracking echocardiography in 10 patients with scar tissue and 10 patients without scar tissue and in 10 age-matched controls. RESULTS: In patients with scar tissue, MRI-derived left ventricular ejection fraction was significantly reduced compared with that in patients without scar tissue (51 versus 61%, p<0.05), and echocardiography-derived longitudinal strain was significantly reduced in five of six left ventricular areas compared with that in healthy controls (average relative reduction, 46%; p<0.05). In patients without scar tissue, longitudinal strain was significantly reduced in two of six left ventricular areas (average relative reduction, 23%; p<0.05) and circumferential strain was reduced in one of six left ventricular areas (relative reduction, 56%; p<0.05) compared with that in healthy controls. CONCLUSIONS: Regional left ventricular function is reduced even in patients without left ventricular wall scar tissue late after successful repair of an anomalous origin of the left coronary artery from the pulmonary artery. This highlights the need for meticulous lifelong follow-up in all patients with a repaired anomalous origin of the left coronary artery from the pulmonary artery.

Anomalous Left Coronary Artery From the Right Sinus of Valsalva in a Patient Presenting with ST-Segment Elevation in Lead aVR: A Case Report.

BACKGROUND: Anomalous coronary artery origins appear in roughly 1% of coronary angiograms, and up to 15% of syncope and sudden cardiac death events can be attributed to anomalous coronaries. Patients with an anomalous coronary artery arising from the opposite sinus may initially present with syncope and electrocardiographic findings of ischemia. CASE REPORT: We describe a case in which an adolescent male presented with exercise-induced angina and syncope, and his initial electrocardiogram (ECG) showed diffuse ST-segment depression with ST-segment elevation in lead aVR. Cardiac catheterization revealed there was no coronary ostium in the left coronary cusp, and the left coronary artery had an anomalous origin from the right cusp. The patient received urgent left internal mammary artery-to-left anterior descending artery coronary bypass and a saphenous vein graft to the ramus intermedius. After he underwent 6 months of medical therapy with ß-blockade and angiotensin-receptor blockade, his left ventricular systolic function improved to low-normal level (left ventricular ejection fraction, approximately 50%). WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: ST-segment elevation in lead aVR is strongly prognostic for left main or triple-vessel coronary artery disease. However, in patients who present with syncope and few other coronary artery disease risk factors, this ECG finding should be suggestive of an ischemic event caused by an anomalous left coronary artery. Early recognition of this pattern of clinical signs and ECG findings by an emergency physician could be critical for making the correct diagnosis and risk stratifying the patient for early coronary angiography and urgent surgical revascularization.