Diffuse Plexus-Like Coronary Artery-Left Ventricular Fistulae Leading to Coronary Steal Syndrome: 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

High-Risk Acute Coronary Syndrome in a Patient with Coronary Subclavian Steal Syndrome Secondary to Critical Subclavian Artery Stenosis

Case Reports in Cardiology ◽

10.1155/2014/175235 ◽

2014 ◽

Vol 2014 ◽

pp. 1-5 ◽

Cited By ~ 2

Author(s):

Zaher Fanari ◽

Niksad Abraham ◽

Sumaya Hammami ◽

Wasif A. Qureshi

Keyword(s):

Coronary Artery ◽

High Risk ◽

Cardiogenic Shock ◽

Subclavian Artery ◽

Left Ventricular ◽

Subclavian Steal Syndrome ◽

Coronary Syndrome ◽

Subclavian Steal ◽

Coronary Subclavian Steal Syndrome ◽

Steal Syndrome

Patients with multivessel coronary artery disease are more likely to have extensive atherosclerosis that involves other major arteries. Critical subclavian artery (SCA) stenosis can result in coronary subclavian steal syndrome that may present as recurrent ischemia and even myocardial infarction in patients with coronary artery bypass graft (CABG). In patients with concomitant severe native coronary disease, occluded saphenous venous grafts (SVG) to other arteries, percutaneous intervention on critical subclavian artery (SCA) stenosis that will compromise the blood flow to left internal mammary graft (LIMA) and left anterior descending (LAD) artery will be a high-risk procedure and may be associated with cardiogenic shock, especially in patients with preexisting ischemic cardiomyopathy. The use of percutaneous left ventricular (LV) assist device like Impella will offer better hemodynamic support and coronary perfusion and therefore results in decreased myocardial damage, maximized residual cardiac function, and lower incidence of cardiogenic shock.

Download Full-text

Is stealing still a crime? Comment on left internal mammary artery side branch intervention in the management of coronary steal syndrome following coronary artery bypass grafting

Catheterization and Cardiovascular Interventions ◽

10.1002/ccd.29446 ◽

2021 ◽

Vol 97 (1) ◽

pp. 105-107

Author(s):

Morton J. Kern

Keyword(s):

Coronary Artery ◽

Coronary Artery Bypass Grafting ◽

Internal Mammary Artery ◽

Left Internal Mammary Artery ◽

Artery Bypass ◽

Side Branch ◽

Bypass Grafting ◽

Internal Mammary ◽

Coronary Steal ◽

Steal Syndrome

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