Pediatric coronary cameral fistula in a structurally normal heart: a case report and review of the literature.

INTRODUCTION: Coronary cameral fistulas (CCFs) are rare congenital anomalies characterized by abnormal connections between a coronary artery and one of the cardiac chambers. These abnormal connections can lead to significant clinical implications, including heart failure and myocardial ischemia, necessitating timely diagnosis and intervention. CASE PRESENTATION: A 5-year-old Iranian boy was brought to the emergency room at Heart Hospital Center complaining of chest pain and dyspnea on exertion. He had experienced similar episodes over the past 4 months, which had not been evaluated. Physical examination revealed stable vital signs and no remarkable findings. Transthoracic echocardiography demonstrated a dilated left coronary artery with a large aneurysm and a small orifice to the right ventricular body. The left ventricular ejection fraction was 55%. Cardiac computed tomography angiography confirmed the diagnosis. Cardiac angiography showed a dilated left coronary artery and a coronary cameral fistula to the right ventricular . The aneurysm was successfully occluded using two Amplatzer™ devices. CONCLUSION: This case underscores the critical role of multimodal imaging in diagnosing and managing coronary cameral fistulae. Early detection and appropriate intervention are paramount in preventing the progression of symptoms and potential complications such as heart failure and myocardial ischemia. The successful closure with Amplatzer™ devices highlights the efficacy of minimally invasive techniques in treating complex cardiovascular anomalies. Regular follow-up and careful monitoring are essential to ensure long-term success and to manage any potential recurrences. CLINICAL KEY MESSAGE: Timely identification and management of coronary cameral fistulae are crucial to prevent complications. Advances in imaging techniques and minimally invasive treatments, such as transcatheter closure, offer effective solutions. A multidisciplinary approach and regular follow-up are essential for comprehensive care and successful long-term management.

Spontaneous coronary artery dissection: a case series illustrating current challenges in diagnosis and treatment.

Spontaneous coronary artery dissection (SCAD) has been increasingly recognized in recent years as an important cause of acute coronary syndrome, myocardial infarction, and sudden death, especially among young women. However, due to the lack of clinical trials on SCAD, evidence-based guidance on management is currently lacking. Presented are four case studies that illustrate the recent insights and challenges in SCAD diagnosis and treatment.

Spontaneous coronary artery dissection (SCAD) is a condition where the layers of a coronary artery tear without any apparent cause. In recent years, it has become more recognized as an important cause of chest pain, heart attacks, and sudden death, especially in younger women. However, because of the lack of clinical trials focused on SCAD, there is insufficient evidence-based guidance on managing the condition. This article shares four cases to show the current knowledge and challenges in diagnosing and treating SCAD.

Case report: Woven-like coronary arteries in a patient with polycythemia vera.

BACKGROUND: Woven coronary artery (WCA) is a rare and underdiagnosed congenital anomaly that involves multiple thin and tortuous epicardial arterial conduits reassembling distally into a single lumen. Recanalized thrombus may present as woven-like coronary arteries, appearing similar to WCA on angiographic images. CASE PRESENTATION: A 58-year-old female patient with intermittent chest pain for 5 years and polycythaemia vera (PV) for 8 years. The left anterior descending artery was presented like WCA on coronary angiography and finally confirmed as recanalized thrombus by optical coherence tomography(OCT), which might have been caused by PV. Given the patient's high thrombotic risk of PV and thrombotic changes in the left circumflex artery (LCX), we ultimately chose a conservative treatment without stenting. CONCLUSIONS: OCT would be needed for the diagnosis and differential diagnosis of woven-like coronary arteries. And physicians should take an appropriate treatment in a personalized way in patients with PV.

Successful percutaneous coronary intervention in a congenital single right coronary artery with acute myocardial infarction: A case report and literature review.

RATIONALE: Single coronary artery (SCA) is a rare coronary artery malformation. SCA combined with atherosclerotic plaques can cause severe and widespread myocardial ischemia and infarction, leading to hemodynamic instability and even sudden death. PATIENT CONCERNS: A 48-year-old Chinese man was admitted for treatment of persistent chest tightness and panic for 5 hours. The patient was a lorry driver with high work intensity and mental stress, with body mass index of 33.78, history of smoking and alcohol consumption, but no history of hypertension and diabetes. DIAGNOSES: Admission examination showed Troponin was 183.083 µg/L and CK-MB value was >300 µg/L. The patient was diagnosed with a congenital single right coronary artery (RCA) with acute myocardial infarction (AMI) by coronary angiography (CAG). Due to atherosclerotic plaques rupture, a complete occlusion of the proximal RCA with thrombolysis in myocardial infarction grade 0 of distal blood flow were found. INTERVENTIONS AND OUTCOMES: The patient was treated with thrombus aspiration and thrombolytic therapy by percutaneous coronary intervention under the support of intra-aortic balloon pump. Postoperative the chest tightness and panic were relieved, and CAG revealed that the proximal thrombus of the RCA was reduced, and distal blood flow was restored to thrombolysis in myocardial infarction grade 3. After 2 weeks of intensive antithrombotic and lipid-regulating drug therapy, the patient was successfully discharged. Follow-up for 6 months, the patient was able to live and work normally without experiencing chest tightness and chest pain. Computed tomography angiography (CTA) confirmed a congenital single RCA with patent lumen and no severe stenosis. LESSONS: The congenital single RCA is very rare, and it is fatal in conjunction with acute coronary syndrome. Early detection and appropriate treatment is critical for AMI patient with single RCA. CAG is the gold standard for diagnosis of single RCA, and CTA is a necessary to describe the anatomical course of abnormal coronary arteries.

Case report of spontaneous coronary artery dissection in a 50­year­old man.

BACKGROUND: Spontaneous Coronary Artery Dissection (SCAD) is an acute coronary event of uncertain origin. SCAD occurs when the coronary artery wall dissects non-traumatically and non-atherosclerotically, leading to the formation of an intramural hematoma or intimal tear, ultimately compressing and restricting the true lumen, or even occluding it. The management of SCAD remains controversial despite modern imaging techniques. In addition to supportive drug therapy, percutaneous coronary intervention (PCI) is another option that can be used as an effective treatment modality. CASE PRESENTATION: We describe A 50-year-old male with SCAD presented to the hospital emergency department complaining of chest pain. Coronary angiography incidentally showed spiral dissection from the proximal to distal right coronary artery (RCA). Three overlapping coroflex stents were deployed from the distal to the proximal RCA. CONCLUSIONS: To stabilize the coronary artery in Acute Coronary Syndrome (ACS) patients due to SCAD, prompt interventions such as stenting and angioplasty are needed. However, it is necessary to pay attention to the clinical condition of patients and quick diagnosis for the recovery of patients and reduction of complications.

Percutaneous transcatheter embolization in large tortuous coronary artery fistula patient: a case report.

Coronary artery fistula is a rare anomaly involving the coronary artery and a heart chamber or vessel. Percutaneous intervention has been shown to be effective and safe in fistulas that are small and nontortuous; however, it is not an absolute contraindication in fistulas that are large and tortuous. We report a delayed diagnosis of a single, large-diameter, tortuous coronary artery fistula that manifested as myocardial ischaemia due to the steal phenomenon in a 49 year old male. The undesirable connection was successfully obliterated by percutaneous embolisation, followed by an improvement in symptoms and daily activities. Steal phenomenon is the fundamental mechanism of myocardial ischaemia in coronary artery fistula, as confirmed by improvement in symptoms and coronary artery perfusion following occlusion of the fistula. Percutaneous catheterization is safe and effective for coronary artery fistula closure, and the occlusion site should be precise to achieve complete occlusion and prevent complications.

An unusual cause of cardiac arrest in a young infant.

BACKGROUND: Anomalous aortic origin of a coronary artery from the inappropriate sinus of Valsalva (AAOCA) is a rare congenital heart lesion. It is uncommon for patients with AAOCA to present with severe symptoms at a very young age. CASE PRESENTATION: We describe a very rare but critical presentation in a young infant with AAOCA that requires surgical repair and pacemaker placement. A three-month-old infant was referred because of syncope. Cardiac arrest occurred shortly after admission. The electrocardiogram indicated a complete atrioventricular block and a transvenous temporary pacemaker was implanted. A further coronary computed tomographic angiography (CTA) showed the anomalous origin of the right coronary artery from the left sinus of Valsalva. Coronary artery unroofing was performed due to an interarterial course with the intramural component, and a permanent epicardial pacemaker was implanted. The postoperative recovery was uneventful, and this patient was thriving and asymptomatic at the nine-month follow-up. However, the electrocardiogram still indicated a complete pacing rhythm. CONCLUSIONS: By timely diagnosis and treatment, this patient is successfully rescued. Although rare, AAOCA may be fatal even in infants.

[Angioplasty of an anomalous coronary artery : A complex procedure ?] / Angioplastie d'une anomalie coronaire congénitale : un acte compliqué ?

Coronary artery anomalies (ANOCOR) are congenital anomalies with various anatomical forms. Percutaneous treatment can be offered in certain situations, most often to address associated atherosclerotic disease or, more rarely, to correct a congenital stenosis. Due to the frequent difficulties of catheterization, percutaneous coronary interventions for ANOCOR are recognized as complex procedures. A thorough anatomical understanding facilitates the identification of the connection site and the initial ectopic course of an ANOCOR during coronary angiography. Selecting an appropriate catheter is a crucial step in the procedure. There is a higher prevalence of atherosclerotic disease along retroaortic courses compared to other ectopic courses. When treating atherosclerotic stenosis downstream of an ectopic course, techniques typically used for complex coronary procedures can be helpful. While angioplasty for congenital stenosis is technically feasible, its role in management algorithms remains to be defined. Currently, this type of percutaneous treatment may be offered to right ANOCOR with interarterial course in adults over 35 years old and with ischemic symptoms or myocardial ischemia.

The comprehensive care of the post-SCAD patient.

Spontaneous coronary artery dissection (SCAD) is the nonatherosclerotic, nontraumatic dissection of an epicardial coronary artery which is predominantly caused by intramural hematoma formation or intimal disruption. SCAD is an increasingly recognized cause of acute coronary syndrome (ACS) in young women and its true prevalence may be underestimated due to its underdiagnosis. The pathogenesis of SCAD is multifaceted and influenced by gender-specific factors, hormonal fluctuations, genetics, arteriopathies, and physical and emotional stressors. Although the pathogenesis of SCAD is multifaceted, current guidance on the care of the post-SCAD patient is limited. Moreover, it is necessary to address each of the different components contributing to the pathogenesis of SCAD in order to improve outcomes and quality of life in this patient population. This literature review aims to consolidate the current knowledge on the medical management, rehabilitation, reproductive and mental health care, and comorbidities that affect SCAD survivors.

Implantable Cardioverter-Defibrillator Placement in Spontaneous Coronary Artery Dissection: A Case Report.

BACKGROUND Spontaneous coronary artery dissection can present with acute coronary syndrome, ventricular arrhythmias, or sudden cardiac death. Implantable cardioverter-defibrillator placement in patients with spontaneous coronary artery dissection is controversial. The purpose of publishing this case is to inform physicians of potential benefits of implantable cardioverter-defibrillator implantation in patients with spontaneous coronary artery dissection. CASE REPORT A 55-year-old woman presented with chest pain, with an electrocardiogram revealing anterior ST-elevation myocardial infarction and troponin peak of 53.8 ng/mL. Coronary angiography revealed mid-left anterior descending artery occlusion, with appearance of spontaneous coronary artery dissection that was not amenable to revascularization. The decision was made to treat medically. In recovery, the patient experienced ventricular fibrillation arrest. The patient was defibrillated once with achievement of return of spontaneous circulation. An Impella CP was placed to stabilize the patient. After the patient was stabilized, an implantable cardioverter-defibrillator was placed. CONCLUSIONS Data on potential benefits of implantable cardioverter-defibrillator placement in patients with spontaneous coronary artery dissection are limited. Most patients with spontaneous coronary artery dissection recover normal coronary architecture; however, there are no guidelines for implantable cardioverter-defibrillator placement in patients with spontaneous coronary artery dissection. Patients with spontaneous coronary artery dissection with high-risk features may benefit from implantable cardioverter-defibrillator for secondary prevention of ventricular arrhythmia and sudden cardiac death, as shown with this case.

Clinical Presentation and Therapy of Coronary Artery Anomalies.

There are two major coronary arteries that arise normally directly above the aortic valve in the sinus. The left main coronary artery (LCA or LMCA) arises from the left coronary sinus and divides shortly after its origin into the left anterior descending and the circumflex coronary arteries (LCX). Branches of the left anterior descending (LAD) coronary artery include the left conus, septal, and diagonal arteries. Branches of the circumflex coronary artery may include the sinus node artery, Kugel's artery, marginal arteries, and the left atrial circumflex artery (Fig. 47.1). The LAD follows the interventricular septum to the apex, the LCX turns posterior, follows the atrioventricular groove between the left atrium and ventricle to the coronary sinus. Branches of the right coronary artery (RCX) include the conal branch, the sinus node artery, an atrial branch, the right ventricular muscle branches (including the acute marginal branch), the posterior descending coronary artery, the atrioventricular node artery, and septal branches (Fig. 47.2). The RCX follows the atrioventricular groove between the right atrium and ventricle. The "dominant coronary artery" is the one giving rise to the posterior descending coronary artery. It originates from the right coronary artery in 80% of people.

Outcomes of Transcatheter Closure of Congenital Left Circumflex Coronary Artery Fistula.

BACKGROUND: Congenital left circumflex coronary artery fistula (LCX-CAF) is a relatively rare type of coronary artery fistula (CAF); little is known about the outcomes of transcatheter closure (TCC) of LCX-CAF. METHODS AND RESULTS: All consecutive patients admitted to Fuwai Hospital and scheduled for TCC of LCX-CAF between January 2012 and December 2022 were reviewed retrospectively. Of the 25 consecutive patients (mean [±SD] age 34±20 years; 48% male) admitted and scheduled for TCC of congenital LCX-CAF, the procedure was feasible in 22 (77.3%). The mean (±SD) diameter of the fistulas was 6.99±2.04 mm; 21 (84%) patients had a large fistula (i.e., diameter >2-fold greater than non-feeding coronary artery). Occluders were deployed via a transarterial approach and arteriovenous loop in 6 (27.3%) and 16 (72.7%) patients, respectively. No procedural complications were recorded. Although the procedural success rates are similar for single LCX-CAF and left anterior descending CAF (81.25% vs. 92.86%; P=0.602), the mean time from initial angiography to first occluder deployment is significantly longer for LCX-CAF (83.06±36.07 vs. 36.00±9.49 min; P<0.001). The mean (±SD) follow-up time was 62.2±45.5 months. The incidence of myocardial infarction and recanalization of the fistula was 4.5% (1/22) and 9.1% (2/22), respectively. CONCLUSIONS: TCC of LCX-CAF is a feasible and effective alternative to surgical repair, with comparable outcomes in selected patients. Optimal medical therapy to prevent post-closure myocardial infarction requires further investigation.

Spontaneous coronary artery dissection: A review of medical management approaches.

Spontaneous coronary artery dissection (SCAD) is an underdiagnosed cause of acute coronary syndrome (ACS) that usually presents in young female patients. Risk factors include female sex, physical and emotional stressors, and fibromuscular dysplasia, and diagnosis is usually made by coronary angiography aided by intravascular ultrasound (IVUS) or optical coherence tomography (OCT). While conservative treatment is usually preferred over percutaneous coronary intervention or surgery, medical management of SCAD has been under debate. This comprehensive review aims to summarize findings from recent studies exploring various medical treatment approaches for the management of SCAD. Antiplatelet therapy with aspirin is generally safe and beneficial for SCAD patients, with dual antiplatelet (DAPT) being recommended for patients undergoing PCI. In the absence of intervention, DAPT may be given for a short period followed by a longer single-antiplatelet (SAPT) therapy with aspirin. Beta-blockers appear to be safe and effective for SCAD patients. On the other hand, fibrinolytics, anticoagulants, and glycoprotein IIa/IIIb inhibitors are contraindicated. Cardiovascular medications such as renin-angiotensin-aldosterone system (RAAS) inhibitors, mineralocorticoid receptor antagonists, and statins are not recommended in the absence of left ventricular dysfunction. Hormonal therapy is contraindicated for patients who develop SCAD during pregnancy and future pregnancy is discouraged in that patient population.

Diagnosis and Management of Congenital Coronary Artery Fistulas in Adults.

PURPOSE OF REVIEW: This review describes the presentation, diagnosis, and management of congenital coronary artery fistulas (CAFs) in adults. RECENT FINDINGS: CAFs are classified as coronary-cameral or coronary arteriovenous fistulas. Fistulous connections at the distal coronary bed are more likely to be aneurysmal with higher risk of thrombosis and myocardial infarction (MI). Medium-to-large or symptomatic CAFs can manifest as ischemia, heart failure, and arrhythmias. CAF closure is recommended when there are attributable symptoms or evidence of adverse coronary remodeling. Closure is usually achievable using transcatheter techniques, though large fistulas may require surgical ligation with bypass. Given their anatomic complexity, cardiac CT with multiplanar 3-D reconstruction can enhance procedural planning of CAF closure. Antiplatelet and anticoagulation are essential therapies in CAF management. CAFs are rare cardiac anomalies with variable presentations and complex anatomy. CAF management strategies include indefinite medical therapy, percutaneous or surgical CAF closure, and lifelong patient surveillance.

Clinical Outcomes of Cardiogenic Shock Due to Spontaneous Coronary Artery Dissection Versus Cardiogenic Shock Due to Coronary Artery Disease.

Spontaneous coronary artery dissection (SCAD) can be treated conservatively. However, some SCAD patients can develop cardiogenic shock (CS). We evaluated the outcomes of SCAD-related CS using data from a national population-based cohort study from January 1, 2016, to December 30, 2019. In our study of 32,640 patients with SCAD, about 10.6% of patients presented with CS. We found that SCAD patients with CS had higher mortality and greater complications including use of mechanical circulatory devices, arrhythmias, respiratory support, and acute heart failure compared to those without CS. When comparing CS due to SCAD with that due to coronary artery disease, we found that although mortality rates were similar, those with CS due to SCAD were associated with higher risk of use of mechanical circulatory support, major bleeding, blood transfusion, and respiratory failure.

Spontaneous Coronary Artery Dissection in Clinical Practice: Pathophysiology and Therapeutic Approaches.

Spontaneous coronary artery dissection (SCAD) is a cause of myocardial infarction without obstructive coronary artery disease (MINOCA). It is determined by a coronary artery wall layers separation, which occurs regardless of traumatic or iatrogenic injuries. Even if it is often a missed diagnosis, its incidence is growing along with the improvement of intracoronary imaging techniques that allow for better detection. The main angiographical classification distinguishes three different forms, with slightly different prognoses at long-term follow up. SCAD is a recurrent condition, severely hampering the life quality of affected patients. The predominantly young age of patients with SCAD and the high prevalence of females among them have made the topic increasingly important, especially regarding therapeutic strategies. According to the data, the most recommended treatment is conservative, based on the use of antiplatelet agents and supportive anti-ischemic therapy. However, there are conflicting opinions concerning the need for dual antiplatelet therapy and its duration. In the case of invasive treatment, the choice between percutaneous coronary intervention and coronary artery bypass graft depends on the patient's clinical stability and the interested vessel. The purpose of the current review is to revise the pathophysiological mechanisms underlying SCAD and the current knowledge of its treatment.

Spontaneous Coronary Artery Dissection (SCAD) from an Interventionalist Perspective.

PURPOSE OF REVIEW: Spontaneous coronary artery dissection (SCAD) is an increasingly recognized cause of acute coronary syndrome (ACS), particularly among women < 50 years of age. Here, we aim to review the pathogenesis of SCAD, discuss SCAD as an initial manifestation of systemic arterial disease, and highlight invasive strategies as well as unique challenges in the care of women with SCAD. RECENT FINDINGS: A paradigm shift has occurred in the care of SCAD patients in the past decade as recommendations for conservative management have become widespread. Invasive interventions are reserved for patients with hemodynamic compromise or active ischemia due to increased periprocedural complications and failure rates. Certain patient populations have been identified for larger territory infarcts and proximal disease including patients with known connective tissue disease, premenopausal women, and patients with pregnancy-associated SCAD (P-SCAD). Current recommended management of SCAD is conservative. Despite a growing awareness of SCAD and its known association with systemic arteriopathies in women, evidence-based data remains scarce. Future studies focused on identifying genetic factors, optimal medical therapy after SCAD, and techniques to minimize interventional complications are needed.

Twenty-five-year trends in incidence, angiographic appearance, and management of spontaneous coronary artery dissection.

BACKGROUND: Spontaneous coronary artery dissection (SCAD) has been described as an infrequent cause of acute coronary syndrome (ACS). Knowledge about the disease is still limited and SCAD might still be underdiagnosed. OBJECTIVES: Trends in incidence, presentation, angiographic appearance, management, and outcomes of SCAD over 25 years were analyzed. METHODS: Patients with SCAD between 1997 and 2021 at the University Hospital Zurich, Switzerland, were included. Incidences were assessed as total numbers and proportions of ACS cases. Clinical data were collected from medical records and angiographic findings were reviewed. Major adverse cardiac events (MACE) were defined as the composite of all-cause death, cardiac arrest, SCAD recurrence or progression, other myocardial infarction, and stroke. RESULTS: One hundred fifty-six SCAD cases were included in this study. The incidence increased significantly in total (p < 0.001) and relative to ACS cases (p < 0.001). This was based on an increase of shorter lesions (p = 0.004), SCAD type 2 (p < 0.001), and lesions in side branches (p = 0.014), whereas lesions in the left main coronary artery and proximal segments were decreasing (p-values 0.029 and < 0.001, respectively). There was an increase in conservative therapy (p < 0.001). The rate of MACE (24%) was stable, however, there was a reduced proportion of patients with a need for intensive care treatment (p = 0.017). CONCLUSIONS: SCAD represents an important entity of ACS that still might be underappreciated. The increasing incidence of SCAD is likely based on better awareness and familiarity with the disease. A lower need for intensive care treatment suggests positive effects of the increasing implementation of conservative management.

Diagnosis and Management of Congenital Coronary Artery Fistulas in Infants and Children.

PURPOSE OF REVIEW: Coronary artery fistulas (CAFs) are rare coronary anomalies that most often occur as congenital malformations in children. Although most children with CAFs are asymptomatic at the time of diagnosis, some present with symptoms of congestive heart failure in the setting of large left-to-right shunts. Others may develop additional complications including coronary artery ectasia and coronary thrombosis. Surgical and transcatheter closure techniques have been previously described. This review presents the classifications of CAFs in children and the short and long-term outcomes of CAF closure in children in the reported literature. We also summarize previously-reported angiographic findings and post-treatment remodeling characteristics in pediatric patients. RECENT FINDINGS: With advancements in cross-sectional imaging technologies, anatomic delineation of CAFs via these modalities has become crucial in procedural planning. Recent reports of surgical and transcatheter closure of CAFs in children have reported good procedural success and low rates of short-term morbidity and mortality. Distal-type CAFs have elevated risk for long-term sequelae post-closure compared to proximal-type CAFs. A recent report of a multi-institutional cohort also describes post-closure remodeling classifications which may predict long-term outcomes in these patients as well as guide individualized anticoagulation management. Invasive closure of significant CAFs via surgical or transcatheter techniques is feasible and safe in most children with good short and intermediate-term outcomes. However, close clinical and imaging follow-up is required to monitor for late complications even after successful closure. Antiplatelet and anticoagulation regimens remain important aspects of post-closure management, but the necessary intensity and duration of such therapy remains unknown.