|Year : 2017 | Volume
| Issue : 4 | Page : 38-42
Surgical intervention for left main compression syndrome due to severe secondary pulmonary hypertension
Laichun Song, Ming Xu, Chao Tao, Lei Shi, Bo Wang, Huadong Yu, Xufa Chen, Liang Tao, Xi Su
Department of Cardiac Surgery, Asia Heart Hospital, Wuhan, PR China
|Date of Web Publication||12-Mar-2018|
Prof. Liang Tao
Department of Cardiac Surgery, Asia Heart Hospital, Wuhan
Source of Support: None, Conflict of Interest: None
A 51-year-old man presented with non-ST-elevation myocardial infarction due to extrinsic compression of the left main coronary artery (LMCA) caused by a dilated pulmonary artery (PA) with secondary pulmonary hypertension and was successfully treated with surgical intervention including pulmonary angioplasty, atrial septal defect correction, and thromboendarterectomy. Imaging modalities were extremely useful in making the diagnosis and providing follow-up of LMCA compression syndrome in this case. During the follow-up, a sufficient hemodynamic improvement was obtained, without exacerbation of the PA dilatation, resulting in the absence of compression of the LMCA.
Keywords: Congenital atrial septal defect, dilated pulmonary artery, left main coronary artery, pulmonary hypertension
|How to cite this article:|
Song L, Xu M, Tao C, Shi L, Wang B, Yu H, Chen X, Tao L, Su X. Surgical intervention for left main compression syndrome due to severe secondary pulmonary hypertension. Cardiol Plus 2017;2:38-42
|How to cite this URL:|
Song L, Xu M, Tao C, Shi L, Wang B, Yu H, Chen X, Tao L, Su X. Surgical intervention for left main compression syndrome due to severe secondary pulmonary hypertension. Cardiol Plus [serial online] 2017 [cited 2019 Apr 19];2:38-42. Available from: http://www.cardiologyplus.org/text.asp?2017/2/4/38/227171
| Introduction|| |
Pulmonary artery (PA) dilatation can occur in isolation or in association with other cardiovascular anomalies, and most cases are related to primary or secondary pulmonary hypertension (PAH). The dilated main PA may compress the adjacent structures. Extrinsic compression of the left main coronary artery (LMCA) may be less common,,, however, which can cause major complications such as myocardial ischemia and even sudden death. Now, there is no definite management for LMCA compression by PA dilatation. Revascularization with coronary artery bypass surgery or percutaneous coronary intervention may be used as bridging management before lung or heart–lung transplantation. Here, we present a case with severe PH who presented with severe angina and non-ST elevation acute myocardial infarction due to extrinsic compression of the LMCA from a dilated PA. The severe PH was induced by delayed closure of the atrial septal defect (ASD) and pulmonary embolism. The diagnosis was confirmed by multi-detector computerized tomography (MDCT) and coronary angiogram (CAG). Pulmonary angioplasty, ASD correction, and pulmonary embolectomy were successfully performed, and his unstable angina was relieved.
| Case Report|| |
A 51-year-old man was admitted to the hospital because of exercise intolerance, shortness of breath, and substernal chest pain. He had been diagnosed with congenital ASD and PAH 3 years before. Surgical or transcatheter intervention was refused at that time because of no symptoms. Ten days before the onset of substernal chest pain, an atrial flutter had been noted. During his hospital stay, he experienced an acute episode of chest pain with anterior ST-segment depression on the electrocardiogram and elevated cardiac enzymes (cTnI, 1.23 ng/ml), consistent with non-ST elevation myocardial infarction [Figure 1].
|Figure 1: Twelve-lead electrocardiogram during chest pain. ST-segment depression and T-wave inversion became pronounced during chest pain, particularly in leads V1–V6|
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On admission, a chest radiograph showed moderate cardiomegaly and a prominent dilated PA [Figure 2]. A transthoracic echocardiography evaluation revealed a large ostium secundum-type ASD (diameter, 4.3 cm × 3.6 cm) with bidirectional shunt flow, severe tricuspid valve regurgitation, significant dilatation of the right ventricle (6.7 cm) and the main PA (5.4 cm), severe PAH (systolic PA pressure, 75 mm Hg), normal left ventricle size, and decreased global systolic function (left ventricular ejection fraction 35%) [Figure 3].
|Figure 2: Posterior-anterior chest X-ray showing cardiomegaly and severely dilated pulmonary arteries|
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|Figure 3: A transthoracic echocardiography showed a large ostium secundum-type atrial septal defect (diameter, 4.3 cm × 3.6 cm)|
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Under the suspicion of LMCA compression by a giant PA aneurysm (PAA), MDCT was performed. MDCT showed important pulmonary aneurysmal dilatation including main, left, and right PA (77.9/35.8/38.9 mm) with LMCA compression [Figure 1] and [Figure 4]. Pulmonary embolism was also visualized in the left PA [Figure 2] and [Figure 4]. Moreover, LMCA had deviated inferiorly and was being compressed by the PAA [Figure 3] and [Figure 4]. Therefore, we arranged right heart catheterization (RHC) and CAG for further evaluation. RHC revealed the hemodynamic status (PA pressure, 85/37/53 mmHg; Qp: Qs: 2.57:1; total peripheral resistance [TPR] 447 dyn). CAG revealed that the LMCA was eccentrically narrowed at the ostium and open distally (99% occlusion) without atherosclerotic narrowing [Figure 5].
|Figure 4: (1 and 2) Multi-detector computerized tomography angiography image demonstrates a severe dilatation of the pulmonary artery causing extrinsic compression with inferior displacement of the left main coronary artery at the ostium; (3) multi-detector computerized tomography showed a filling defect (arrow) in the pulmonary artery implying a thrombus|
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|Figure 5: Coronary angiography shows ostial stenosis of the left main coronary artery seen right anterior oblique with cranial angulation|
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After discussion with a cardiologist, he was referred for the strategy of surgical correction. The strategy included ASD correction, resection of the PA aneurysm, and thromboendarterectomy. During the procedure, the pulmonary aneurysmal dilatation with LMCA compression was easily identified. Thromboendarterectomy was performed in main and left PA. The right PA and LMCA were probed without lesion [Figure 6]. Postoperative computed tomography showed no occlusion of LMCA and narrowing PA [Figure 7]. At 12 months after the procedure, the patient remained clinically stable with no further episodes of angina.
|Figure 6: The surgical strategy including atrial septal defect correction, resection of the pulmonary artery aneurysm, and thromboendarterectomy|
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|Figure 7: During the follow-up, multi-detector computerized tomography angiography image demonstrates the left main coronary artery was patent without compression|
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| Discussion|| |
PA dilatation can occur in isolation or in association with other anomalies, including congenital heart disease with significant left-to-right shunt (ASD, ventricular septal defect, patent ductus arteriosus), other congenital defects (e.g., tetralogy of Fallot, pulmonary valve stenosis), connective tissue disorders (Marfan syndrome), and vasculitis (Behcet's syndrome). The dilated main PA may compress the adjacent structures. LMCA compression occurs at a frequency of 5%–19% among patients with PH., The symptoms include chest angina, syncope, left ventricular dysfunction, and malignant arrhythmia. Thirty-three percent of patients with PAH presenting with angina were found to have compression of the LMCA by PA dilatation. In small prospective coronary angiography studies, 40%–50% of patients with PAH have been found to have >50% compression of the LMCA.,
Revascularization with coronary artery bypass surgery has been the traditional and standard treatment option for patients with LMCA disease. However, the patients with LMCA compression due to severe PH may have an extremely high risk of anesthesia and perioperative recovery; therefore, percutaneous intervention with stent implantation may be an alternative to coronary artery bypass graft (CABG) for treating the LMCA stenosis. Rich et al. first reported stenting of LMCA in patients with primary PAH in 2001, with excellent postinterventional results. There have been about 10 case reports of patients with LMCA compression who were treated with a percutaneous procedure.,,,,, All patients had relief of symptoms and ischemia postprocedure. Although acutely successful, the average follow-up reported in these case reports ranged from 1 to 6 months. Vaseghi et al. reported that the average follow-up in the study was 16.6 months, with the patient in the longest follow-up being angina-free and without recurrence of left ventricular ischemia for 39 months. However, the treatment after revascularization with PAH-target drugs, including bosentan, sildenafil, and epoprostenol, could not obtain a sufficient improvement in hemodynamics. The PA dilatation may exacerbate with repeat compression of the LMCA. Thus, surgical treatment including pulmonary angioplasty should be considered in patients with PAH who could tolerate the procedure.
The case presented in this article underwent successful surgical treatment with chest pain relief. The PAH in this patient is due to ASD and chronic pulmonary embolism without a sufficient improvement after the use of PAH-target drugs. Moreover, the result of RHC revealed the hemodynamic status (PA pressure, 85/37/53 mmHg; Qp: Qs: 2.57:1; TPR 447 dyn) and provided the possibility of surgical correction of ASD and pulmonary embolism. CABG may be the last choice for LMCA compression after the pulmonary angioplasty. The pulmonary angioplasty was successful with resection of the main pulmonary trunk to relieve the compression of LMCA. ASD correction eradicated the left-to-right shunt and the pulmonary thrombus in the main PA was cleared to reduce the pulmonary vascular resistance, which may increase the PAH-target drugs action and avoid the exacerbation of PAH. After the procedure, the patient recovered eventually. During the follow-up, angina disappeared and no expansion of the pulmonary artery.
| Conclusion|| |
The appropriate diagnosis and treatment are very important for extrinsic compression of the LMCA. In selected patients, surgical pulmonary trunk reduction, aortocoronary bypass, and thromboendarterectomy to reduce PA pressure could achieve good results, which could significantly improve symptoms and quality of life.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Lee MS, Oyama J, Bhatia R, Kim YH, Park SJ. Left main coronary artery compression from pulmonary artery enlargement due to pulmonary hypertension: A contemporary review and argument for percutaneous revascularization. Catheter Cardiovasc Interv 2010;76:543-50.
Shankar O, Lohiya BV. Cardiovocal syndrome – A rare presentation of primary pulmonary hypertension. Indian Heart J 2014;66:375-7.
Mesquita SM, Castro CR, Ikari NM, Oliveira SA, Lopes AA. Likelihood of left main coronary artery compression based on pulmonary trunk diameter in patients with pulmonary hypertension. Am J Med 2004;116:369-74.
Sakuma M, Demachi J, Suzuki J, Nawata J, Takahashi T, Shirato K, et al.
Proximal pulmonary artery aneurysms in patients with pulmonary artery hypertension: Complicated cases. Intern Med 2007;46:1789-93.
Decuypere V, Delcroix M, Budts W. Left main coronary artery and right pulmonary vein compression by a large pulmonary artery aneurysm. Heart 2004;90:e21.
Kothari SS, Chatterjee SS, Sharma S, Rajani M, Wasir HS. Left main coronary artery compression by dilated main pulmonary artery in atrial septal defect. Indian Heart J 1994;46:165-7.
Kajita LJ, Martinez EE, Ambrose JA, Lemos PA, Esteves A, Nogueira da Gama M, et al.
Extrinsic compression of the left main coronary artery by a dilated pulmonary artery: Clinical, angiographic, and hemodynamic determinants. Catheter Cardiovasc Interv 2001;52:49-54.
Mitsudo K, Fujino T, Matsunaga K, Doi O, Nishihara Y, Awa J, et al.
Coronary arteriographic findings in the patients with atrial septal defect and pulmonary hypertension (ASD + PH) – Compression of left main coronary artery by pulmonary trunk. Kokyu To Junkan 1989;37:649-55.
Rich S, McLaughlin VV, O'Neill W. Stenting to reverse left ventricular ischemia due to left main coronary artery compression in primary pulmonary hypertension. Chest 2001;120:1412-5.
Gómez Varela S, Montes Orbe PM, Alcíbar Villa J, Egurbide MV, Sainz I, Barrenetxea Benguría JI, et al.
Stenting in primary pulmonary hypertension with compression of the left main coronary artery. Rev Esp Cardiol 2004;57:695-8.
Dodd JD, Maree A, Palacios I, de Moor MM, Mooyaart EA, Shapiro MD, et al.
Images in cardiovascular medicine. Left main coronary artery compression syndrome: Evaluation with 64-slice cardiac multidetector computed tomography. Circulation 2007;115:e7-8.
Dubois CL, Dymarkowski S, Van Cleemput J. Compression of the left main coronary artery by the pulmonary artery in a patient with the Eisenmenger syndrome. Eur Heart J 2007;28:1945.
Lindsey JB, Brilakis ES, Banerjee S. Acute coronary syndrome due to extrinsic compression of the left main coronary artery in a patient with severe pulmonary hypertension: Successful treatment with percutaneous coronary intervention. Cardiovasc Revasc Med 2008;9:47-51.
Vaseghi M, Lee JS, Currier JW. Acute myocardial infarction secondary to left main coronary artery compression by pulmonary artery aneurysm in pulmonary arterial hypertension. J Invasive Cardiol 2007;19:E375-7.
Caldera AE, Cruz-Gonzalez I, Bezerra HG, Cury RC, Palacios IF, Cockrill BA, et al.
Endovascular therapy for left main compression syndrome. Case report and literature review. Chest 2009;135:1648-50.
Ginghina C, Popescu BA, Enache R, Ungureanu C, Deleanu D, Platon P, et al.
Pulmonary artery dilatation: An overlooked mechanism for angina pectoris. J Cardiovasc Med (Hagerstown) 2008;9:747-50.
Vaseghi M, Lee MS, Currier J, Tobis J, Shapiro S, Aboulhosn J, et al.
Percutaneous intervention of left main coronary artery compression by pulmonary artery aneurysm. Catheter Cardiovasc Interv 2010;76:352-6.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]