Multiple coronary artery-left ventricular fistulae: a pattern of anomalous coronary microvascularization

AbstractIn the past, coronary arterial anomalies have been difficult to diagnose by non-invasive methods. Identification of coronary arterial origins is now a routine part of the standard paediatric echocardiogram. Anomalous origin of a coronary artery from the pulmonary trunk is an extremely important diagnosis to make. Many echocardiographic features are not directly related to the visualisation of the coronary arterial origin. Left ventricular dilation and abnormal ventricular performance are common, along with mitral regurgitation and evidence of collateralisation of the flow from the coronary artery that has an aortic origin. In some cases, the anomalous coronary artery can be seen to arise directly from the pulmonary trunk. Congenital atresia of the main stem of the left coronary artery has a similar echocardiographic presentation, except that its aortic origin is not determined. Anomalous aortic origin of the coronary artery has important implications, as the first presenting symptom can be sudden death. With meticulous attention to the origins of the coronary arteries, echocardiographic diagnosis can also be achieved. In contrast to the anomalous origin of a coronary artery from the pulmonary trunk, ventricular performance is usually normal. Whenever there is doubt as to the definition of the origin of the coronary arteries and, indeed, when there is serious clinical concern that a coronary artery has an anomalous origin, other testing, such as cine-computed tomography, magnetic resonance imaging, or cardiac catheterisation may be indicated for confirmation or to provide greater anatomic detail.

Download Full-text

Abstract 15789: Cardiac MRI With Adenosine Identifies Coronary Stenosis, Perfusion Defects, and Delayed Enhancement in Pediatric Patients With Anomalous Aortic Origin of a Coronary Artery Before and After Surgical Repair

Circulation ◽

10.1161/circ.130.suppl_2.15789 ◽

2014 ◽

Vol 130 (suppl_2) ◽

Author(s):

Julie A Brothers ◽

Timothy S Kim ◽

Mark A Fogel ◽

Kevin K Whitehead ◽

Stephen A Paridon ◽

...

Keyword(s):

Coronary Artery ◽

Cardiac Mri ◽

Surgical Repair ◽

Statistical Significance ◽

Left Ventricular ◽

Lv Function ◽

Inferior Wall ◽

Before And After ◽

Perfusion Defects ◽

Anomalous Coronary

Background: Anomalous aortic origin of a coronary artery (AAOCA) with an interarterial course is associated with sudden cardiac death in children. Objectives: Using cardiac MRI with adenosine, we evaluated coronary ostial stenosis, proximal coronary size, and left ventricular (LV) function in children with AAOCA. Methods: We prospectively enrolled children 5-18 years old with interarterial AAOCA. MRIs were reviewed for coronary artery origin, proximal course, dimensions, and cardiac function. Surgery consisted of the modified unroofing procedure. We used descriptive statistics and paired t-tests to evaluate for statistical significance. Results: Between 2/2009 and 5/2014, 24 subjects with AAOCA underwent 29 MRIs. The majority were male (N=19, 79%) with anomalous right coronary artery (AAORCA, N=20, 83%). Mean age was 12.8 years at time of initial MRI. MRI was performed an average of 7 months post-operatively in 8 subjects who underwent surgery. In all, the proximal anomalous coronary arose tangential to the aorta with an elliptical, slit-like ostium. The anomalous coronary measured smaller proximally (0.20 mm) compared to distally (0.31 mm, P=< 0.0001), and after surgical repair, the post-operative origin was significantly larger (0.36 vs. 0.21 mm, P=0.02). Other abnormalities at initial MRI included fixed inferior wall (N=1) and reversible subendocardial septal/inferior wall (N=1) perfusion defects. Post-operatively, the neo-ostium was round in 6 (see Figure), but in 2, the orifice remained elliptical. One patient had a new small mid-myocardial scar and one had dyskinetic septal wall motion. LV function was normal both before and after surgery (mean ejection fraction =68.1% vs. 67.5%, P=0.85). Conclusions: Cardiac MRI with adenosine is an important tool for the evaluation of anomalous anatomy, myocardial function, and ischemia/injury and should be considered for the initial and, when applicable, post-operative assessment of children with AAOCA.

Download Full-text

447 Left ventricular non-compaction with coronary artery anomaly: incidental diagnosis after ST-elevation myocardial infarction

European Heart Journal Supplements ◽

10.1093/eurheartj/suab142.045 ◽

2021 ◽

Vol 23 (Supplement_G) ◽

Author(s):

Francesca Romana Prandi ◽

Federica Illuminato ◽

Chiara Galluccio ◽

Marialucia Milite ◽

Massimiliano Macrini ◽

...

Keyword(s):

Myocardial Infarction ◽

Coronary Artery ◽

Family History ◽

Dilated Cardiomyopathy ◽

Coronary Atherosclerosis ◽

Lateral Wall ◽

Left Ventricular ◽

Anomalous Coronary Artery ◽

Incidental Diagnosis ◽

Anomalous Coronary

Abstract Left ventricular noncompaction (LVNC) is a rare congenital cardiomyopathy thought to be caused by arrest of normal endomyocardial morphogenesis. A 33-year-old male, smoker, dyslipidemic not on medical therapy, with family history for sudden cardiac death (SCD) presented to the ER for chest pain radiated to the left arm, unrelated to exertion. The physical examination was within normal limits. Blood tests showed increased cardiac enzymes levels (Troponin I hs 49 308.7 ng/l). The EKG showed STE in the anterior leads and diffuse ventricular repolarization anomalies, suggestive of anterior STEMI. The patient underwent coronary catheterization, with evidence of anomalous origin of the Cx from the RCA and critical stenosis on the proximal LAD, treated with PPCI and implantation of a DES with good angiographic result. The patient was transferred to the Cardiac Intensive Care Unit. TTE showed moderate LV dilatation, severe LV dysfunction (EF 30%) with apical, septal and anterior wall akinesis, and lateral wall hypertrabecularization with multiple prominent trabeculations and deep intertrabecular recesses communicating with the cavity, suggestive for LVNC. Cardiac MRI documented dilated LV with EF 34%, anterior and antero-septal wall akinesis (associated with increased T1 mapping values and areas of LGE after contrast injection, compatible with ischaemic outcomes), infero-lateral wall hypokinesia and LV free wall marked hypertrabecularization with a ratio of not compacted(T)/compacted(M) myocardium of 5 (Petersen criteria for LVNC diagnosis: T/M > 2.3 in telediastolic long-axis view). The patient was discharged in stable clinical conditions in DAPT(Cardioaspirin and Ticagrelor). At two months cardiologic follow-up the patient was asymptomatic and TTE confirmed a dilated LV with severely depressed EF (30%). In consideration of the post-ischaemic dilated cardiomyopathy with severely depressed EF and of the family history of SCD (father deceased at 54-years-old), the patient was admitted in our Cardiology Unit and he underwent subcutaneous ICD (sICD) implantation. He was discharged in stable conditions with remote home monitoring transmitter. The association between LVNC and anomalous coronary artery origin is rare. LVNC is sometimes associated with coronary artery disease, but only rare cases of acute myocardial infarction have been described in literature, with exceptionally rare cases of LVNC incidental diagnosis after STEMI. To the best of our knowledge, this is the first case of LVNC associated with anomalous coronary artery origin and STEMI reported in literature. Some authors hypothesized that a single gene responsible for both myocardial development and coronary endothelium could be involved in the pathogenesis of LVNC and at the same time predispose to coronary atherosclerosis. Further studies are necessary in order to assess the possible pathophysiological mechanisms that correlate LVNC and coronary atherosclerosis. According to European guidelines, LVNC in the absence of additional risk factors is not an indication for primary ICD implantation, and for arrhythmic risk stratification it’s recommended to follow the criteria used for non-ischaemic dilated cardiomyopathy. The anomalous origin of the Cx from the RCA has an incidence of 0.37%, and it is generally not linked to an increased risk of SCD. Involvement of RV in LVNC cannot be excluded even when RV appears normal on CMR, and if involved there is higher risk of perforation by the lead. sICD overcomes disadvantages of transvenous ICD in patients without a need for pacing therapy. In literature use of sICD is reported only in 14 patients with LVNC, mainly children and young adults.

Download Full-text

ANOMALOUS ORIGIN OF THE LEFT CORONARY ARTERY FROM THE PULMONARY ARTERY (ADULT TYPE)

PEDIATRICS ◽

10.1542/peds.29.2.274 ◽

1962 ◽

Vol 29 (2) ◽

pp. 274-282

Author(s):

M. H. Agustsson ◽

B. M. Gasul ◽

R. Lundquist

Keyword(s):

Coronary Artery ◽

Pulmonary Artery ◽

Left Coronary Artery ◽

Clinical Findings ◽

Left Ventricular ◽

Anomalous Origin ◽

White Female ◽

Adult Type ◽

Anomalous Coronary ◽

Anomalous Left Coronary

The case of a 7-year-old white female with an anomalous left coronary artery from the pulmonary artery is presented. The clinical findings of an asymptomatic patient with a continuous murmur at the left lower strenal border, with left ventricular hypertrophy by roentgenography and electrocardiography raised the suspicion of an aortic fistula, which was then investigated by retrograde aortography. The aortogram demonstrated the anomalous origin of the left coronary artery with retrograde flow into the pulmonary artery. The age and circumstances of death in previously reported cases indicate that the so-called adult type is not a benign disease. The diagnosis of an anomalous coronary artery from the pulmonary artery is at any age an indication for consideration of surgical intervention.

Download Full-text

Left Ventricular and Mitral Valve Function Long After Repair of Left Anomalous Coronary Artery From the Pulmonary Artery

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135111431844 ◽

2012 ◽

Vol 3 (3) ◽

pp. 321-327

Author(s):

Stanimir G. Georgiev ◽

Stojan D. Lazarov ◽

Ivajlo D. Mitev ◽

Alexandra Z. Latcheva ◽

Georgi A. Christov ◽

...

Keyword(s):

Coronary Artery ◽

Mitral Valve ◽

Pulmonary Artery ◽

Left Ventricular ◽

Anomalous Coronary Artery ◽

Valve Function ◽

Anomalous Coronary

Download Full-text

Anomalous coronary artery origin from the pulmonary artery: correlation between surgical timing and left ventricular function recovery

The Annals of Thoracic Surgery ◽

10.1016/s0003-4975(03)00344-8 ◽

2003 ◽

Vol 76 (2) ◽

pp. 581-588 ◽

Cited By ~ 45

Author(s):

Guido Michielon ◽

Duccio Di Carlo ◽

Gianluca Brancaccio ◽

Paolo Guccione ◽

Ennio Mazzera ◽

...

Keyword(s):

Coronary Artery ◽

Pulmonary Artery ◽

Left Ventricular Function ◽

Ventricular Function ◽

Left Ventricular ◽

Anomalous Coronary Artery ◽

Surgical Timing ◽

Function Recovery ◽

Anomalous Coronary

Download Full-text

Surgery and critical care for anomalous coronary artery from the pulmonary artery

Cardiology in the Young ◽

10.1017/s1047951110001071 ◽

2010 ◽

Vol 20 (S3) ◽

pp. 35-43 ◽

Cited By ~ 3

Author(s):

Timothy A. Fehrenbacher ◽

Michael E. Mitchell ◽

Nancy S. Ghanayem ◽

James S. Tweddell

Keyword(s):

Coronary Artery ◽

Pulmonary Artery ◽

Left Coronary Artery ◽

Ventricular Dysfunction ◽

Left Ventricular ◽

Arterial System ◽

Anomalous Origin ◽

Anomalous Coronary Artery ◽

Young Infants ◽

Anomalous Coronary

AbstractAnomalous origin of the left coronary artery from the pulmonary artery is a rare congenital cardiac malformation that accounts for 0.25–0.50% of children with congenital cardiac disease and can cause myocardial dysfunction in young infants. In any infant presenting with ventricular dysfunction, the diagnosis of anomalous origin of the left coronary artery from the pulmonary artery must be suspected and the origin of the coronary arteries must be confirmed. The diagnosis of anomalous origin of the left coronary artery from the pulmonary artery is an indication for surgical repair. A two-coronary arterial system is the goal and is almost always achievable. The goal of surgical therapy is the creation of a two-coronary arterial system, which appears to provide better long-term survival and protection from left ventricular dysfunction and mitral valvar regurgitation than does simple ligation of the anomalous coronary artery. Direct reimplantation of the anomalous coronary artery is the procedure of choice. It is straightforward and borrows from well-practised techniques commonly used in other procedures such as the arterial switch operation. For the rare patient in whom direct reimplantation is not possible, strategies to lengthen the anomalous coronary artery, or baffle it within the pulmonary root, are available. Mitral valvar regurgitation is common at presentation, but following the establishment of a two-coronary arterial system and satisfactory myocardial perfusion, regurgitation of the mitral valve resolves in the vast majority. Therefore, mitral valvuloplasty at the time of initial surgery for anomalous origin of the left coronary artery from the pulmonary artery is not indicated. Post-operative care requires careful manipulation of inotropic support and reduction of afterload. Mechanical support, with either extracorporeal membrane oxygenation or left ventricular assist device, should be available for use if necessary.

Download Full-text

234 Echocardiographic visualization of retroaortic anomalous coronary artery

European Heart Journal Supplements ◽

10.1093/eurheartj/suab132.002 ◽

2021 ◽

Vol 23 (Supplement_G) ◽

Author(s):

Noemi Bruno ◽

Ilaria Ferrari ◽

Francesco Pelliccia ◽

Carlo Gaudio ◽

Luca Monzo

Keyword(s):

Coronary Artery ◽

Troponin T ◽

Left Ventricular ◽

Tubular Structure ◽

Left Ventricular Ejection ◽

Systemic Hypertension ◽

Anomalous Coronary Artery ◽

Ventricular Ejection Fraction ◽

Increased Risk ◽

Anomalous Coronary

Abstract A 65-year-old female was admitted to our hospital for sudden onset of typical chest pain at rest lasting few minutes. Her medical background included systemic hypertension, type 2 diabetes, dyslipidaemia, and mild obesity. Upon arrival in the emergency room, the electrocardiogram didn’t reveal signs of acute myocardial ischaemia and serial cardiac troponin T measurements were persistently negative. A transthoracic echocardiogram (TTE) was performed, showing mild ventricular hypertrophy, no regional wall motion abnormalities, and a preserved left ventricular ejection fraction. A highly echogenic tubular structure, located slightly on the atrial side of the atrioventricular groove was noted in multiple apical views. Its tubular shape was suggestive of a vascular structure, but its location was atypical for a normal vessel; indeed its persistence in more than an echocardiographic plane excluded an artefact. According to patient’s clinical history and her high cardiovascular risk profile she was referred for coronary angiography, demonstrating no critical stenosis but an anomalous aortic origin of a coronary artery (AAOCA) from the inappropriate sinus of Valsalva: the left main coronary artery (LMCA) arose from the right coronary cusp and then took a caudal posterior loop running posterior to the aortic root. In light of these findings we could associate the tubular structure seen at TTE to the retroaortic course of LMCA, a finding recently described as retroaortic anomalous coronary (RAC) sign. Among AAOCA, the retroaortic course of the LMCA is an uncommon diagnosis in adults, and its association with a single coronary origin is extremely rare. Although it has been usually considered a benign clinical entity, it is associated with an increased risk in morbidity and mortality during valve surgery. The presence of RAC sign at TTE was demonstrated to be highly suggestive of an anomalous coronary artery (specificity 93.9%) and strongly associated with retroaortic LMCA course at computed tomography angiography. 234 Figure B

Download Full-text