Coronary Artery Anomalies Treatment & Management

Updated: Dec 30, 2019
  • Author: Louis I Bezold, MD; Chief Editor: Stuart Berger, MD  more...
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Treatment

Medical Care

An infant with symptoms of coronary artery ischemia or injury requires intensive care management and therapies. Direct initial efforts at reducing myocardial oxygen demands, possibly including oxygen administration, correction of acid-base status, endotracheal intubation, and paralysis to reduce work of breathing.

Additional beta-agonist inotropy may be used in a life-saving circumstance but is likely to result in increased oxygen use and may precipitate malignant ischemic dysrhythmias. For mechanical support, left ventricular assist devices (LVADs) or extracorporeal membrane oxygenation (ECMO) have been used effectively as bridges to surgery.

Phosphodiesterase inhibitors can be used to enhance inotropy and obtain peripheral vasodilation that reduces afterload. In association with anomalous coronary artery arising from the pulmonary artery, milrinone may precipitate a reduction in pulmonary vascular resistance.

Loop diuretics may be used to relieve symptoms of congestive heart failure (CHF).

Beta-adrenergic blockers may be used in selected cases of coronary abnormalities to reduce myocardial oxygen consumption and reduce predisposition to ischemia. A recent study concluded that current data does not support or discourage the use of beta-blockers in children with CHF. [32]

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Surgical Care

Surgical management of coronary abnormalities varies and often requires the combined expertise of both adult and congenital cardiac surgical specialists.

In addition to all the varied techniques of coronary arterial bypass, anomalous origin of coronary artery from pulmonary artery may require Takeuchi tunnel repair techniques or the Jatene button relocation technique. Long-term complications are common after the Takeuchi repair and include pulmonary stenosis, baffle leaks, mitral regurgitation, and/or left ventricular dysfunction. [33]

Addressing coronary ostial obstruction or intramural coronary courses often requires unroofing (as is shown in the image below), unique resection, and patching strategies.

Operative repair of anomalous left coronary artery Operative repair of anomalous left coronary artery (LCA) from the right sinus of Valsalva. The slitlike anomalous origin of the left coronary artery from the right aortic sinus of Valsalva is demonstrated, as is the intramural course of the coronary artery. (B) The intramural course of the artery is unroofed, placing the functional ostium in the left sinus. (C) Tacking sutures are used to secure the intima of the new coronary ostium and to reinforce the adjacent commissure of the aortic valve. (Reproduced from Jaquiss RD, Tweddell JS, Litwin SB: Surgical therapy for sudden cardiac death in children. Pediatr Clin North Am 2004 Oct; 51(5): 1389-400).

Although controversy exists regarding the diagnosis and treatment of anomalous aortic origin of a coronary artery, a study of 50 patients who underwent surgical repair demonstrated no operative mortality and good medium-term (median follow-up, 5.7 y) results (47/50 free of cardiac symptoms, and no patient with sudden death). [34]

In cases of coronary fistula, cardiac catheterization with coronary embolization using coils and devices has been an effective therapy in many instances. [35]

Farouk et al published a discussion of operative technique and review of the literature on anomalous left coronary artery arising from the right pulmonary artery. [36]

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Consultations

Consultations often are required for optimal management of varied coronary artery anomalies in children. These may include a colleague experienced in the techniques of selective coronary arteriography and intervention in adults, nuclear medicine radiographers knowledgeable in quantification of myocardial injury and recovery potential, and both pediatric and adult cardiovascular surgeons to facilitate optimal surgical repair.

Transfer

Transfer patients to a facility with specialists experienced in the techniques of selective coronary arteriography and intervention, with specialists experienced in nuclear medicine, with radiographers knowledgeable in quantification of myocardial injury and recovery potential, and with both pediatric and adult cardiovascular surgeons to facilitate optimal surgical repair.

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Activity

Individualize recommended activity for those patients who have sustained a cardiac injury. Cautiously reintegrate patients into physical education and sports.

Recommendations may be aided by periodic stress assessments. However, even these may not absolutely ensure the prevention of potential catastrophic events.

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