Giant unruptured left sinus of Valsalva aneurysm with left coronary artery compression.

Likelihood of left main coronary artery compression based on pulmonary trunk diameter in patients with pulmonary hypertension

A 5-week-old child presented with a cardiac arrest secondary to myocardial ischaemia. Echocardiography demonstrated a single coronary artery arising from the right sinus of Valsalva. The coronary artery branched into left and right arteries, with the left artery then coursing anomalously in the tissue plane between the aortic root and the subpulmonary infundibulum. Compression of the left coronary artery caused severe myocardial ischaemia that resolved following construction of a bypass graft using the left internal thoracic artery. Stenosis at the anastomosis between the graft and the coronary artery was successfully treated by coronary angioplasty 2 years later.

An extremely unusual case of myocardial infarction associated with infective endocarditis (IE) is described. A 38-year old male with a high fever was transferred to our hospital for further treatment of IE. Two-dimensional echocardiogram showed a large mycotic aneurysm of the sinus of Valsalva in contact with neighbouring structures. The patient had a rapid recovery within several days after administration of antibiotic agents. However, he then developed abrupt onset of severe precordial pain. From the echocardiogram images and biochemical evaluation he was diagnosed as having an acute subendocardial infarction. Serial echocardiograms revealed expansion of the aneurysm, extending from the myocardium of the anterolateral free wall to the lower margin of the proximal left coronary artery. The cause of acute myocardial infarction was thought to be incomplete occlusion of the coronary artery through compression by the enlarging mycotic aneurysm of the sinus of Valsalva. Urgent surgery confirmed compression of the left coronary artery by the large mycotic aneurysm as the cause of acute myocardial infarction.

Rationale:Acute myocardial infarction is usually caused by coronary atherosclerotic plaque disruption (rupture or erosion), also including other uncommon etiologies. Pulmonary epithelioid hemangioendothelioma (PEH) is a rare low to intermediate malignant vascular tumor originating from vascular endothelial cells. Here, we report a rare case of acute ST-segment elevation myocardial infarction (STEMI) due to extrinsic compression of left coronary artery from PEH.Patient concerns:A 63-year-old woman with pulmonary nodules received left pulmonary nodulectomy, and the pathological examination indicated PEH. Five months after the pulmonary nodulectomy, the patient was admitted due to progressive dyspnea.Diagnosis:Electrocardiography showed the obvious ST-segment elevation in the leads I, aVL, and V1–3, and laboratory tests revealed the elevated level of cardiac troponin T. Emergent coronary angiography and the contrast-enhanced computed tomography scan conformed STEMI due to extrinsic compression of left coronary artery from PEH.Interventions:The patient did not undergo further therapy after the pulmonary nodulectomy. During the present hospitalization, she received basic life support and nutritional support treatment.Outcomes:The patient deteriorated rapidly into multi-organ failure and eventually died.Lessons:Acute STEMI could be caused by extrinsic compression of the coronary artery from the mass effects of PEH, and active therapy and close follow-up should be considered for patients with PEH.

Surgical Treatment of Anomalous Aortic Origin of Coronary Arteries: The Reimplantation Technique and Its Modifications

Left coronary artery compression syndrome is characterized by compression of the left main coronary artery between the aorta and an enlarged main pulmonary artery. A 39-year-old woman presented with angina with dyspnea on exertion for two years. Detailed investigations revealed an atrial septal defect, valvular lesions, and severe pulmonary hypertension with left main coronary artery compression. Patch closure of the atrial septal defect, left coronary artery bypass, and valve repair was carried out. The patient recovered well and was asymptomatic on follow-up. The optimal management of such patients is yet to be clarified.

This review focuses on a rare complication of pulmonary arterial hypertension (PAH), extravasation compression of the left coronary artery (LCA) dilated by the pulmonary artery. The review described clinical manifestations and methods for diagnostics of LCA compression, and advantages of the endovascular correction of this complication in patients with pulmonary hypertension. Selection of a device to be implanted during the endovascular intervention in these patients was discussed with due account for concomitant treatment with oral anticoagulants. As an illustration of the issue under discussion, a clinical case of acute coronary syndrome in a female patient from the PAH Registry of the V. A. Almazov National Medical Research Center was provided.

Percutaneous pulmonary valve implantation (PPVI) is a non-surgical treatment for right ventricular outflow tract (RVOT) dysfunction. During PPVI, a stented valve, delivered via catheter, replaces the dysfunctional pulmonary valve. Stent oversizing allows valve anchoring within the RVOT, but overexpansion can intrude on the surrounding structures. Potentially dangerous outcomes include aortic valve insufficiency (AVI) from aortic root (AR) distortion and myocardial ischemia from coronary artery (CA) compression. Currently, risks are evaluated via balloon angioplasty/sizing before stent deployment. Patient-specific finite element (FE) analysis frameworks can improve pre-procedural risk assessment, but current methods require hundreds of hours of high-performance computation. We created a simplified method to simulate the procedure using patient-specific FE models for accurate, efficient pre-procedural PPVI (using balloon expandable valves) risk assessment. The methodology was tested by retrospectively evaluating the clinical outcome of 12 PPVI candidates. Of 12 patients (median age 14.5 years) with dysfunctional RVOT, 7 had native RVOT and 5 had RV-PA conduits. Seven patients had undergone successful RVOT stent/valve placement, three had significant AVI on balloon testing, one had left CA compression, and one had both AVI and left CA compression. A model-calculated change of more than 20% in lumen diameter of the AR or coronary arteries correctly predicted aortic valve sufficiency and/or CA compression in all the patients. Agreement between FE results and clinical outcomes is excellent. Additionally, these models run in 2-6 min on a desktop computer, demonstrating potential use of FE analysis for pre-procedural risk assessment of PPVI in a clinically relevant timeframe.

Left coronary artery compression syndrome is an uncommon entity and characterized by compression of the LMCA in-between the aorta and an enlarged main pulmonary arterial trunk. It is usually associated with a congenital cardiac defect. Cardiac 64-slice MDCT provides a non-invasive and an accurate method for assessing the degree of dynamic LMCA compression throughout the cardiac cycle, its angulation relative to the left sinus of Valsalva and depiction of pulmonary pathology, making it a valuable tool in the workup of patients suspected of left coronary artery compression.

One case of unruptured sinus of Valsalva aneurysm causing left coronary artery compression and lethal myocardial infarction in a 39-year old woman is described. In reviewing the literature, 15 previously published cases of sinus of Valsalva aneurysms disturbing coronary flow were found. It is concluded that the mechanism of coronary flow disturbance differs between aneurysms of the left and right sinuses, respectively. The rare congenital aneurysms of the left sinus carry a considerably greater risk of myocardial ischemia than those of the right sinus. The risk of this complication supports an aggressive surgical attitude towards asymptomatic sinus of Valsalva aneurysms.

(J Card Surg 2010;25:72-73)

Left Coronary Artery Occlusion After Percutaneous Pulmonary Valve Implantation

We report two cases of symptomatic cardiac ischemia caused by left coronary artery compression. One was a 16-year-old boy with history of the Ross procedure, aortic root aneurysm, and right ventricle-to-pulmonary artery conduit stenosis. The other was a 32-year-old woman with history of pulmonary atresia with ventricular septal defect repair and a giant right ventricle-to-pulmonary artery conduit aneurysm. In both cases, the left coronary artery was compressed by the previously placed valved autologous pericardial roll right ventricle-to-pulmonary artery conduit. Conduit replacement without direct coronary intervention relieved the coronary ischemia. Early diagnosis and relief of external compression avoided potentially fatal outcomes.

Left Main Coronary Artery Compression Long Term After Repair of Conotruncal Lesions: The Bow String Conduit

Expert consensus guidelines: Anomalous aortic origin of a coronary artery