NASCI case of the month: a case of anomalous right coronary artery arising from the pulmonary artery.

Coronary artery anomalies are rare but potentially fatal abnormalities with occasional striking imaging findings radiologists should recognize.

[Anomalous origin of right coronary artery from pulmonary artery associated to left main coronary stenosis]. / Anomalie de connexion de l'artère coronaire droite au niveau de l'artère pulmonaire associée à une sténose du tronc commun gauche.

We describe herein a case of a 16 years-old female patient referred to our department for further exploration of a chest pain. Color Doppler echocardiography revealed an abnormal flow at the level of the pulmonary artery. Exercise testing was abnormal leading to further imaging including computed cardiac tomography followed by coronary angiography which showed anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) associated with mid shaft left main stenosis. We discuss this uncommon association and therapeutic options.

Anomalous Arising of Right Coronary Artery from the Pulmonary Artery.

Coronary artery anomalies are seen in less than 1% of the general population and in 1.6% of cardiac catheterization cases. The anomalous origin of the coronary artery from the pulmonary artery is one of four groups of coronary artery origin anomalies. The incidence of anomalous origin of the right coronary artery from the pulmonary artery is 1 in 500,000 and was first described in 1882 by John Brook. This case report reports on a 67-year-old man with a diagnosis of asymptomatic anomalous origin of the right coronary artery from the pulmonary artery. The patient underwent surgery of the aortic valve because of valve stenosis. A concomitant surgical procedure included repositioning of the right coronary artery origin to the aortic root sinus. The patient was discharged on the 12th postoperative day, in good condition. Anomalous origin of the right coronary artery from the pulmonary artery is commonly asymptomatic, and surgery is required only if myocardial ischemia is presented.

Very late incidental diagnosis of anomalous right coronary artery from the pulmonary artery.

A 59-year-old Asian woman with paroxysmal supraventricular tachycardia (PSVT), diabetes, and dyslipidemia was hospitalized with palpitations and chest discomfort. Her electrocardiogram showed short RP tachycardia with a heart rate of 167 bpm. She received intravenous adenosine with successful restoration of sinus rhythm and resolution of her symptoms.

Anomalous right coronary artery from the main pulmonary artery (ARCAPA): Incidental finding in an asymptomatic septuagenarian.

Anomalous right coronary artery (RCA) from the main pulmonary artery (ARCAPA) is a rare finding. Clinical presentations range from asymptomatic to sudden death. We present the case of ARCAPA in a septuagenarian initially suspected on a screening chest computed tomography (CT) and later confirmed on cardiac CT. A summary of important points related to this entity is also discussed.

Anomalous origin of the right coronary artery from pulmonary artery in an adult presenting with rapid atrial fibrillation: a case report.

Background: An anomalous origin of the right coronary artery from pulmonary artery (ARCAPA) is a very rare coronary anomaly. Case summary: A 56-year-old man, presenting haemodynamically unstable due to very rapid atrial fibrillation, was found to have ARCAPA by coronary computed tomography angiography. He had normal left ventricular ejection fraction and without reversible ischaemia on an adenosine stress rubidium positron emission tomography. He was treated solely with betablockers and has been well since. Discussion: Numerous case reports on ARCAPA have previously been published, but no previous reports have found rapid atrial fibrillation to be the primary symptom of presentation. Current evidence level concerning the treatment is low; nevertheless, surgical intervention should always be considered to prevent sudden cardiac death. The patient in this case was offered surgical intervention but declined.

Cardiac imaging findings in anomalous origin of the coronary arteries from the pulmonary artery; narrative review of the literature.

INTRODUCTION: Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare coronary artery malformation with an incidence of 0.002% in patients undergoing coronary angiography. It can lead to an increased risk of myocardial infarction (MI) and sudden cardiac death, even in asymptomatic patients. METHODS: We conducted a review of published cases of ARCAPA using PubMed and Scopus databases and included patients over 18 years old with adequate echocardiographic data. RESULTS: We evaluated 28 patients with ARCAPA with a mean age of 42.8 from 1979 to 2021. Patients were diagnosed mostly by angiography and echocardiography, the most performed treatment was reimplantation (15, 53.6%) and the main echocardiographic findings were dilated coronary arteries (9, 32.1%), coronary collaterals (8, 28.6%), and retrograde flow from right coronary arteries to main pulmonary trunk (7, 25%). CONCLUSION: Although ARCAPA is rare and not as deadly as the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) still there is a chance of serious outcomes, therefore surgical treatment should be performed upon diagnosis. Angiography is the gold standard for diagnosis, but echocardiography can be a convenient, non-invasive, and most reliable method as the primary step whenever ARCAPA is suspected.

Challenging Diagnosis of Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery.

Anomalous origin of the right coronary artery (RCA) from the pulmonary artery, ARCAPA, is an extremely rare congenital heart disease. Only 200 cases were reported from 1885 to the present. Patients diagnosed with ARCAPA can be either asymptomatic or can experience symptoms, such as heart murmur, dyspnea, or angina, shortly after birth or around 40-60 years of life. Usually, those with isolated ARCAPA are diagnosed later in life compared to those who associate other structural cardiac defects. We report two cases of anomalous origin of the right coronary artery at the level of the pulmonary artery trunk (ARCAPA) that were diagnosed by invasive coronary angiography. Although asymptomatic, general recommendations suggest an early corrective intervention to prevent complications such as myocardial ischemia and cardiac dysfunction, which can lead to sudden cardiac death.

Anesthesia Consideration for a Patient With Incidentally Diagnosed Anomalous Origin of Right Coronary Artery Originating From Pulmonary Trunk (ARCAPA): A Case Study.

Anomalous origin of the right coronary artery originating from the pulmonary trunk (ARCAPA) is a rare congenital coronary anomaly that is usually diagnosed incidentally. Although usually asymptomatic, ARCAPA can lead to myocardial ischemia of the left ventricular wall and/or sudden cardiac arrest. Here, we report the case of a 48-year-old female who presented for recurrent malignant pleural effusion, who was scheduled for a bronchoscopy, thoracoscopic evaluation of left pleural effusion, multiple excisional biopsies of the left chest wall and costophrenic parietal pleural nodules, and insertion of tunneled PleurX™ catheter (Becton, Dickinson and Company, Franklin Lakes, New Jersey, United States). ARCAPA was discovered incidentally in this patient during the preoperative evaluation. The patient was asymptomatic and echocardiogram findings were within normal limits. No additional intervention was required, and the patient was managed satisfactorily with general anesthesia.

Cardiac Arrest in a 33-year-old Marathon Runner with Anomalous Right Coronary Artery Originating from the Pulmonary Artery.

A 33-year-old marathon runner presented with anomalous right coronary artery originating from the pulmonary artery after being admitted for cardiac arrest. Surgical re-implantation of the right coronary artery to the aortic root to re-establish right coronary ostial circulation was successful. The patient resumed exercise and required no further medical therapy.

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

BACKGROUND: Anomalous 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 METHODS: The 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. RESULTS: Out 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. CONCLUSION: AOCAPA 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.

Aortopulmonary window with anomalous origin of the right coronary artery from the pulmonary trunk.

Anomalous origin of coronary artery originating from the pulmonary artery in conjunction with the aorticopulmonary window (APW) is a rare but a significant anomaly in the era of congenital cardiac diseases. The occurrence of anomalous origin of the right coronary artery from the pulmonary artery among the associated anomalies is less than 5%. The severity of the clinical condition of these patients depends on the degree of left-right shunt and compromise of the pulmonary blood flow. We report surgical management of a case of a 45-day-old infant with APW, ventricular septal defect, and anomalous origin of coronary artery originated from the pulmonary artery.

Surgical treatment for anomalous origin of the right coronary artery from the pulmonary artery: a case report with five-year follow-up.

BACKGROUND: Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital heart disease affecting about 0.002% of the population. Knowledge of ARCAPA is almost collected from case reports. The aim of this study was to provide a rare case to better understand this rare congenital coronary anomaly. CASE PRESENTATION: We report a rare case of an 18-year-old male who was initially referred because of heart murmur. Dilated and tortuous coronary arteries were detected by echocardiography and congenital coronary anomaly was suggested. Further coronary CT angiography confirmed the diagnosis of ARCAPA. Although dual coronary system provides favorable long-term outcome, bypass surgery was considered technically difficult due to the huge mismatch of caliber between the right coronary artery and graft vessels. Eventually, simple right coronary artery ligation was performed. The patient was followed up for about 5 years without evidence of atherosclerosis or myocardial ischemia. CONCLUSIONS: ARCAPA presents as a rare congenital heart disease with variable clinical manifestations. Surgical treatment is highly recommended to re-establish dual coronary system and prevent further complications. To our best knowledge, only about 200 cases of ARCAPA has been reported.

Anomalous origin of coronary arteries from pulmonary artery in adults: a case series.

BACKGROUND: Anomalous origin of a coronary artery from the pulmonary trunk is a small group of rare congenital anomalies present in up to 1% of the population. These patients, in absence of an adequate collateral supply, may present with congestive heart failure secondary to ischaemia, arrhythmia, or sudden cardiac death in up to 90% of cases within the first months of life. CASE SUMMARY: We present four cases diagnosed in adulthood over 10 years in two high-volume centres. The first patient presented with dyspnoea and orthopnoea. The second with chest pain and episodes of non-sustained ventricular tachycardia. The third patient presented during her third pregnancy with chest pain, palpitations, and arrhythmia (non-sustained ventricular tachycardia). The fourth patient presented with sudden cardiac death. DISCUSSION: In all cases with anomalous origin of coronary arteries, it is recommendable to consider surgical correction to avoid the progression of ischaemia, congestive heart failure, arrhythmia, and sudden death.

Anomalous Origin of a Right Coronary Artery from the Pulmonary Artery in a Middle-Aged Woman.

A 53-year-old female patient presented for evaluation of a murmur. The examination revealed a 2/6 systolic ejection murmur in the left upper sternal border. Transthoracic echocardiography with color Doppler showed increased blood flow around the apex of the right ventricle. Further imaging revealed the right coronary artery emerging from the pulmonary artery. (Level of Difficulty: Beginner.).

Anomalies of coronary artery origin: Evaluation on multidetector CT angiography.

Anomalies of origin of coronary arteries are an uncommon occurrence and found in approximately 1-2% of the general population. While a large proportion of these anomalies are clinically silent, a few might be hemodynamically significant and may even result in sudden cardiac death. Comprehensive knowledge of the normal as well as variant anatomies of the coronary artery origin and familiarity with imaging appearances and clinical significance of these anomalies is imperative for precise diagnosis and subsequent planning of treatment, whenever required. Multidetector computed tomography angiography, on account of its non-invasiveness, faster scan times and multiplanar reconstruction capabilities, is increasingly being utilized for characterization of coronary artery origin anomalies and their three-dimensional spatial relations. It shows a superior rate of detection of these anomalies compared to conventional angiography, providing more accurate delineation of the ostium as well as course. With the advent of newer generation CT scanners and use of advanced dose reduction techniques, images can be obtained rapidly having excellent spatial resolution and with minimal radiation dose. In this review article, we present the multidetector CT angiography imaging findings of the spectrum of anomalous coronary artery origin, using a third-generation dual-source CT scanner.

Oiigen anomalo de la arteria coronaiia derecha de la arteiia pulmonar en un lactante con soplo cardiaco: A proposito de un caso / Anomalous origin of the right coronary artery from the pulmonary artery in an infant with a heart murmur: Case report

El origen anómalo de la arteria coronaria derecha de la arteria pulmonar es una entidad rara con una incidencia del 0,002 % y potencialmente grave, que puede causar muerte súbita. El diagnóstico frecuentemente es incidental, secundario a evaluaciones por soplos cardíacos a diferencia del origen anómalo de la arteria coronaria izquierda de la arteria pulmonar, reconocido por presentar isquemia miocárdica e insuficiencia cardíaca. Se reporta el caso de una niña de 6 meses evaluada por un soplo y derivada por sospecha de fístula coronaria. La evaluación mediante ecocardiograma doppler color, cateterismo cardíaco y angiotomografía mostró la presencia de una comunicación interauricular ostium secundum pequeña y origen anómalo de la arteria coronaria derecha de la arteria pulmonar. A los 11 meses, se realizó una cirugía de reimplante de la arteria coronaria derecha en la arteria aorta y el cierre de la comunicación interauricular.

The anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare entity that has an incidence of 0.002 % and can potentially cause sudden death. Unlike to the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), usually presented with myocardial ischemia and heart failure; the diagnosis of ARCAPA is often incidental during evaluation of a heart murmur. We report a case of a 6 months old female patient referred to us with a cardiac murmur and a suspicion of coronary fistula. For a proper diagnostic assessment an echocardiogram, a computed tomography angiography and a cardiac catheterization were requested. A small ostium secundum-type atrial septal defect (ASD) and an ARCAPA were revealed. At 11 months old, the patient was scheduled for corrective cardiovascular surgery. The aim of this report is to describe a low incidence and potentially fatal disease.

Anomalous origin of the right coronary artery from the pulmonary artery in an infant with a heart murmur: Case report. / Oiigen anomalo de la arteria coronaiia derecha de la arteiia pulmonar en un lactante con soplo cardiaco: A proposito de un caso.

The anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare entity that has an incidence of 0.002 % and can potentially cause sudden death. Unlike to the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), usually presented with myocardial ischemia and heart failure; the diagnosis of ARCAPA is often incidental during evaluation of a heart murmur. We report a case of a 6 months old female patient referred to us with a cardiac murmur and a suspicion of coronary fistula. For a proper diagnostic assessment an echocardiogram, a computed tomography angiography and a cardiac catheterization were requested. A small ostium secundum-type atrial septal defect (ASD) and an ARCAPA were revealed. At 11 months old, the patient was scheduled for corrective cardiovascular surgery. The aim of this report is to describe a low incidence and potentially fatal disease.

El origen anómalo de la arteria coronaria derecha de la arteria pulmonar es una entidad rara con una incidencia del 0,002 % y potencialmente grave, que puede causar muerte súbita. El diagnóstico frecuentemente es incidental, secundario a evaluaciones por soplos cardíacos a diferencia del origen anómalo de la arteria coronaria izquierda de la arteria pulmonar, reconocido por presentar isquemia miocárdica e insuficiencia cardíaca. Se reporta el caso de una niña de 6 meses evaluada por un soplo y derivada por sospecha de fístula coronaria. La evaluación mediante ecocardiograma doppler color, cateterismo cardíaco y angiotomografía mostró la presencia de una comunicación interauricular ostium secundum pequeña y origen anómalo de la arteria coronaria derecha de la arteria pulmonar. A los 11 meses, se realizó una cirugía de reimplante de la arteria coronaria derecha en la arteria aorta y el cierre de la comunicación interauricular.