Aberrant left coronary artery from the pulmonary artery with patent ductus arteriosus - a case report and review of the literature.

BACKGROUND: Aberrant left coronary artery from pulmonary artery (ALCAPA) is a very rare congenital heart defect. Its coexistence with patent ductus arteriosus (PDA) is extremely rare. The high pressures created by the left-to-right shunt in the pulmonary arteries can delay symptoms and create a real challenge in diagnosing ALCAPA. Missing this diagnosis can have severe results, including extensive ischemia and sudden death. CASE PRESENTATION: We present a case of an infant born with a large PDA. Initially treated conservatively, however, due to congestive heart failure and lack of weight gain, she underwent surgical ligation of the PDA at the age of four and a half months. Following surgery, she developed pulmonary edema. Echocardiography revealed decreased ventricular function. ECG revealed ST elevations on lateral leads, and serum troponin was significantly increased. The patient underwent cardiac magnetic resonance imaging (MRI), which revealed signs of wall ischemia and decreased function of the left ventricle (LV) with unclear coronary anatomy. Diagnostic catheterization revealed an ALCAPA. She underwent surgical intervention, and the left coronary artery was re-implanted in the aortic sinus. Follow-up revealed slow improvement of cardiac function. DISCUSSION AND LITERATURE REVIEW: The coexistence of PDA and ALCAPA is a very rare occurrence. We found at least 10 reported cases in the literature. Delayed diagnosis might be detrimental. The prognosis of these patients is variable. CONCLUSION: An unusual post-surgical course following PDA repair requires a high index of suspicion and appropriate evaluation for ALCAPA, preferably with angiography.

Myocardial Infarction in Young Adults: Diagnosis Begins Through Inspection.

Spontaneous coronary artery dissection (SCAD) is an atypical cause of myocardial infarction, predominantly seen in women. Among various predisposing factors, genetic vasculopathies such as connective tissue diseases significantly contribute to SCAD. This report discusses a 36-year-old male diagnosed with vascular type Ehlers-Danlos syndrome following an anterior myocardial infarction and explores relevant literature.

Spontaneous Coronary Artery Dissection in a Male Patient With Fibromuscular Dysplasia.

A male patient presented with cardiac arrest attributed to anterior ST-segment elevation myocardial infarction from type 1 spontaneous coronary artery dissection. Subsequent imaging confirmed fibromuscular dysplasia in noncoronary arterial segments. The patient was started on guideline-directed medical therapy and referred to cardiac rehabilitation, showing substantial improvements in clinical status. With greater awareness and advancements in imaging, spontaneous coronary artery dissection has been more frequently recognized, and although as many as 81% to 92% of all cases occur in female patients, it can be seen among men, as well. Adjunctive imaging for arteriopathies may help establish the diagnosis for equivocal causes of acute coronary syndrome in women and men.

Arterial switch operation in a child with commissural malalignment and unusual coronary anatomy.

The arterial switch operation has evolved to become the treatment of choice for transposition of the great arteries and is one of the greatest success stories in congenital heart surgery. The most crucial step of the operation is the coronary artery translocation; therefore, it is of paramount importance for surgeons to know every single detail about the morphology and spatial relationships of the coronary arteries and the roots of the great vessels. However, sometimes the surgeon may face unfavourable scenarios such as major commissural malalignment and anomalous coronary artery patterns and need to be prepared to carry out a successful coronary artery translocation. Herein, we demonstrate that the trapdoor technique is useful for transferring coronary arteries in a neonate with major commissural malalignment and unusual coronary anatomy during the arterial switch operation.

ST-segment elevation myocardial infarction (STEMI) caused by spontaneous coronary artery dissection (SCAD) in a patient with von Willebrand disease.

A postpartum woman in her early 40s, with a history of pre-eclampsia and von Willebrand disease (VWD), presented to the emergency room with chest pain suggestive of an acute coronary syndrome. Initial workup revealed an evolving anterior wall ST-segment elevation myocardial infarction on ECG and elevated cardiac biomarkers, confirming myocardial damage. Point-of-care ultrasound showed apical hypokinesis and coronary angiography revealed a distal dissection of the left anterior descending coronary artery. There was TIMI 3 flow and no evidence of plaque rupture. No percutaneous coronary intervention was performed and the patient was managed conservatively.Fibromuscular dysplasia was ruled out on screening CT angiography. Dual antiplatelet therapy was initiated for an amended course of 3 months given the history of VWD. Our patient had an uncomplicated course in the hospital with a downward trend in their cardiac biomarkers, resolving anterior ST elevation on serial ECGs, and no bleeding complications.

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.

Spontaneous Coronary Artery Dissection in a Healthy Man With Non-ST Elevation Myocardial Infarction.

This case report discusses a diagnosis of spontaneous coronary artery dissection in an otherwise healthy man with non­ST elevation myocardial infarction.

A report of fifty cases with incidental diagnosis of anomalous origin of the right coronary artery from the left sinus of Valsalva.

INTRODUCTION: Anomalous origin of the right coronary artery from the left sinus of Valsalva (R-ACAOS) is a relatively rare condition that can potentially lead to devastating outcomes. The current study aims to investigate the cardiac-related disorders among patients with incidental R-ACAOS diagnosis through computed tomography angiography (CTA). METHODS: The current cross-sectional study has been conducted on 50 patients diagnosed with R-ACAOS who underwent CTA. Based on CTA, the patients' were categorized as R-ACAOS with interarterial course and non-interarterial course. The demographic and medical characteristics, any history of cardiac intervention and New York Heart Association (NYHA) Functional Classification at the time of diagnosis were recruited. Patients were revisited to assess cardiac-associated variables, including symptoms, the presence of heart failure and current NYHA function class. RESULTS: The variables including the history of cardiac intervention (P-value<0.001), the presence of heart failure (P-value = 0.010) and NYHA function class at the time of diagnosis (P-value = 0.006) were remarkably higher among those with interarterial course of R-ACAOS; while, the other variables including chest pain at rest (P-value = 0.55) or on exertion (P-value = 0.12), current NYHA function class, current cardiac-associated symptoms except for dyspnea at rest (P-value = 0.012), mortality and coronary calium score did not differ (P-value>0.05). coronary interventions led to significantly improved NYHA function class (P-value<0.05). CONCLUSION: Based on the findings of the current study, R-ACAOS with interarterial course leads to significantly higher rates of atherosclerotic-related symptoms and events compared with the other types of RCA anomalies. Moreover, coronary interventions led to significantly improved NYHA functional class regardless of R-ACAOS category.

Spontaneous coronary artery dissection outcomes among pregnant vs. non-pregnant women.

AIMS: Spontaneous coronary artery dissection (SCAD) has become increasingly recognized. It accounts for <1-4% of acute coronary syndrome presentations. Overall, however, it makes up over 40% of pregnancy-associated myocardial infarction. Furthermore, pregnancy-associated spontaneous coronary artery dissection (P-SCAD) is described to have a greater degree of clinical manifestations, including left ventricular dysfunction, shock, and left main or multivessel involvement. The findings are disconcerting, though many studies evaluating P-SCAD are based on case series data or are single centre studies. METHODS AND RESULTS: The aim of this study was to evaluate a larger national dataset to evaluate the outcomes of SCAD and specifically P-SCAD in an attempt to better characterize the severity and clinical nature of this condition. To conduct this study, we analysed the National Readmission Database from January 2016 to December 2020. Propensity matching was done using the Greedy 1:1 method. Multivariate logistics and time-to-event Cox regression analysis models were built by including all confounders significantly associated with the outcome on univariable analysis with a cut-off P-value of 0.2. In multivariate regression analysis, P-SCAD patients had a non-propensity matched odds ratio (OR) of 0.21 (0.3-1.54, P = 0.123) of dying and a propensity matched OR of 0.11 (0.02-0.61, P = 0.012) of dying. Thirty-day readmission rate for P-SCAD was 15.8% (n = 93) and for non-pregnant spontaneous coronary artery dissection (NP-SCAD) was 11.2% (n = 2286); non-propensity matched OR for readmission for PSCAD patients was 1.68 (1.24-2.29, P = 0.001) and propensity matched OR was 3.39 (1.93-5.97, P < 0.001). CONCLUSION: Among hospitalized patient, P-SCAD was associated with similar clinical outcomes and reduced incidence of death when compared with NP-SCAD, though had higher rates of 30-day readmission. Larger-scale observational data will be needed to ascertain the true incidence of cardiovascular complications as it relates to P-SCAD.

Coronary Artery Tortuosity and Spontaneous Coronary Artery Dissection: Association With Echocardiography and Global Longitudinal Strain, Fibromuscular Dysplasia, and Outcomes.

BACKGROUND: The etiology and significance of coronary artery tortuosity (TCA) among patients with spontaneous coronary artery dissection (SCAD) are unknown. The aim of this prospective imaging cohort study was to report echocardiographic findings and evaluate whether TCA correlates with cardiac anatomy and function among patients with SCAD. Comorbidities including fibromuscular dysplasia (FMD) and outcomes were also assessed. METHODS: TCA was determined on coronary angiography performed during the diagnosis of SCAD, and cardiac structure and function were evaluated using prospective comprehensive echocardiography. RESULTS: Among 116 patients with SCAD, the mean age at echocardiography was 50.8 ± 8.8 years, a median of 10.9 months after SCAD. Sixty-two patients (53.4%) had FMD, 41 (35.3%) had histories of hypertension, and 17 (14.8%) were hypertensive during echocardiography. Most patients (n = 78 [69%]) had normal left ventricular geometry with normal median ejection fraction (61%; interquartile range, 56% to 64%) and normal global longitudinal strain (-22.2%; interquartile range, -24.0% to -19.9%). Fifteen patients (13.4%) had diastolic dysfunction that was associated with hypertension at the time of echocardiography. Patients with TCA (n = 96 [82.8%]) were older (mean age, 52.1 ± 8.0 vs 44.7 ± 9.9 years; P < .001) with a higher prevalence of FMD (59.4% vs 25%, P = .007) but a similar prevalence of hypertension (35% vs 35%, P > .99) compared with patients without TCA. Across the age range (31.5 to 66.9 years), each decade of age was associated with an approximately 0.89-unit increase in coronary tortuosity score (P < .0001). Echocardiographic parameters were not significantly different between the two groups. Median follow-up duration was 4.4 years (95% CI, 3.8 to 5.2 years). The Kaplan-Meier 3-year SCAD recurrence rate was 9.4% (95% CI, 3.7% to 14.8%). There were no deaths. CONCLUSIONS: The majority of patients with SCAD had normal or near normal echocardiographic results, including global longitudinal strain, with no differences according to TCA. However, patients with SCAD with TCA were older, with a higher prevalence of FMD.

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.

Sinoatrial nodal artery fistula to bronchial arteries originating from the right coronary artery in the setting of chronic bronchiectasis and coronary artery disease.

We present a 67-year-old male with past medical history of hyperlipidemia, hypertension, and emphysema, and who was a former smoker, with dyspnea on exertion and chest pain.

Surgical Management of Anomalous Right Coronary Artery in the Adult: Technique and Case Series.

BACKGROUND: The management of adult patients with anomalous aortic origin of the right coronary artery (ARCA) from the left aortic sinus poses important challenges. The presence of symptoms or documented ischaemia, the anatomical characteristics of the ostium, and the course of the coronary determine decision-making. METHODS: A retrospective review was performed of all cases of surgical management of ARCA at a single centre. The primary endpoints were mortality and myocardial infarction at 30 days. Secondary endpoints included recurrence of symptoms, freedom from re-intervention, and mortality during long-term follow-up. RESULTS: From October 2019 to August 2023, 15 adult patients underwent surgery for ARCA; 13 patients were included in this study (mean age 53.9±11.1 years; 10 female). A slit-like orifice, a long intramural segment, and an interarterial course were found in all patients. Twelve (12) patients (92.3%) were symptomatic: nine with angina, combined with dyspnoea on exertion in seven. One (1) patient had history of pre-syncope. One (1) patient presented with out-of-hospital cardiac arrest. All patients underwent formal unroofing of the orifice and intramural portion of the ARCA; five patients had a concomitant procedure. No 30-day mortality nor myocardial infarction was recorded. At a mean follow-up of 20.1±12.8 months, all patients were alive. One (1) patient (7.6%) developed recurrent dyspnoea; investigations showed no ischaemia. No repeated interventions were required. CONCLUSIONS: Surgical unroofing of anomalous coronary artery in the adult is safe and effective; correction of both the slit-like orifice and intramural portion of the anomaly provides a durable result in patients with ARCA.

Anomalous Aortic Origin of a Coronary Artery.

BACKGROUND: Although anomalous aortic origin of a coronary artery (AAOCA) is associated with risk of sudden cardiac arrest (SCA), there is a spectrum of disease, with the appropriate management for many remaining unclear. Increasing data warrant review for an updated perspective on management. METHODS: A panel of congenital cardiac surgeons, cardiologists, and imaging practitioners reviewed the current literature related to AAOCA and its management. Survey of relevant publications from 2010 to the present in PubMed was performed. RESULTS: The prevalence of AAOCA is 0.4% to 0.8%. Anomalous left coronary artery is 3 to 8 times less common than anomalous right coronary, but carries a much higher risk of SCA. Nevertheless, anomalous right coronary is not completely benign; 10% demonstrate ischemia, and it remains an important cause of SCA. Decision-making regarding which patients should be recommended for surgical intervention includes determining anatomic features associated with ischemia, evidence of ischemia on provocative testing, and concerning cardiovascular symptoms. Ischemia testing continues to prove challenging with low sensitivity and specificity, but the utility of new modalities is an active area of research. Surgical interventions focus on creating an unobstructed path for blood flow and choosing the appropriate surgical technique given the anatomy to accomplish this. Nontrivial morbidity has been reported with surgery, including new-onset ischemia. CONCLUSIONS: A proportion of patients with AAOCA demonstrate features and ischemia that warrant surgical intervention. Continued work remains to improve the ability to detect inducible ischemia, to risk stratify these patients, and to provide guidance in terms of which patients warrant surgical intervention.

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.

[Diagnosis and surgical treatment of high-risk anomalous aortic origin of coronary artery].

Objective: To analyze the diagnosis and surgical treatment of high-risk anomalous aortic origin of coronary artery (AAOCA). Methods: This is a retrospective case series study. From January 2016 to July 2023, 24 cases of high-risk AAOCA underwent surgical treatment in Department of Cardiac Surgery, Guangdong Provincial People's Hospital. There were 18 males and 6 females, operatively aged (M (IQR)) 13 (26) years (range: 0.3 to 57.0 years). They were confirmed by cardiac ultrasound and cardiac CT, all of which had anomalous coronary running between the aorta and the pulmonary artery. There were 15 cases of the right coronary artery from the left aortic sinus of Valsalva, 6 cases of left coronary artery from the right aortic sinus of Valsalva, 3 cases of the sigle coronary artery. Only 3 patients had no obvious related symptoms (2 cases were complicated with a positive exercise stress test and 1 case with other intracardiac malformations), 21 cases had a history of chest tightness, chest pain, or syncope after exercise. Three patients suffered syncope after exercise and underwent cardiopulmonary resuscitation (2 cases were treated with an extracorporeal membrane oxygenerator (ECMO)). The gap from the first symptom to the diagnosis was 4.0 (11.5) months (range: 0.2 to 84.0 months). The detection rate of coronary artery abnormalities suggested by the first cardiac ultrasound was only 37.5% (9/24). Seven patients were complicated with other cardiac diseases (4 cases with congenital heart defects, 2 cases with coronary atherosclerotic heart disease, 1 case with mitral valve disease). Results: All 24 patients underwent surgical treatment (23 cases underwent abnormal coronary artery unroofing, 1 case underwent coronary artery bypass grafting), and 5 patients underwent other intracardiac malformation correction at the same time. There were no death or surgery related complications in the hospital for 30 days after the operation. A patient with preoperative extracorporeal cardiopulmonary resuscitation was continuously assisted by ECMO after emergency AAOCA correction and had complications such as limb ischemia necrosis and renal dysfunction after the operation. During the follow-up of 2.2 (3.3) years (range: 1 month to 7.2 years), one patient who previously underwent percutaneous transluminal coronary angioplasty with a stent implant experienced significant postoperative symptomatic relief, and the other discharged patients had no related symptoms. Conclusions: The accurate rate of initial diagnosis for high-risk AAOCA is still low, but the risk of cardiovascular accidents is high. For sports-related chest pain and other symptoms, more attention should be paid to the detection of AAOCA, especially for adolescents. Exercise stress testing can be helpful in evaluating the cardiovascular risk of asymptomatic AAOCA. Instant surgical treatment can achieve satisfactory curative effects.