Cardiogenic Shock Induced by Coronary Steal Syndrome Through a Neovessel of Mediastinal Neuroendocrine Tumor
Clinical Case
Graphical abstract
Abstract
A woman was admitted for cardiogenic shock with a left ventricular ejection fraction of 15%. Coronary angiography showed a neovessel coursing superiorly toward a mediastinal mass. Cardiac dysfunction was assumed to be the result of coronary steal, and the neovessel was embolized, thereby allowing recovery of the left ventricular ejection fraction. (Level of Difficulty: Advanced.)
Introduction
Cancer and heart disease are 2 of the leading causes of death in the world. Advances in the field of oncology therapy have led to improved outcomes and survival in patients with cancer. The counterpart of this improvement in survival is an increase in cancer therapy–related toxicities that has led to the development and growth of the field of oncocardiology as a new medical specialty (1). Here, we report a rare case of coronary steal induced by a mediastinal tumor.
Learning Objectives
| • | The main objective is to understand the mechanism of a coronary steal syndrome. | ||||
| • | Another objective is to be able to rank and prioritize multimodality imaging for the etiologic exploration of left ventricular systolic dysfunction. | ||||
History of Presentation and Past Medical History
A 63-year-old woman was admitted in the Department of Cardiology at the University Hospital of Rangueil (Toulouse, France) for cardiogenic shock. She had a history of thymic neuroendocrine tumor with bone metastases that was diagnosed 3 years ago and managed with radionuclide therapy using beta particles. She presented with new mediastinal and boneinvolvement of the tumor few months ago; this required radiotherapy, which stabilized the tumor process. She reported slowly increasing dyspnea within the previous weeks, with significant asthenia that she attributed to the tumor process. There was no specific clinical trigger before the cardiogenic shock.
Investigations
On admission, transthoracic echocardiography showed left ventricular dilatation with severe global hypokinesia and a left ventricular ejection fraction of 15%. Coronary angiography showed a large, tortuous neovessel, originating from the first diagonal artery and coursing superiorly toward a mediastinal mass lesion (Figures 1A to 1C, Videos 1 and 2). Cardiac gated computed tomography confirmed the path of the neovessel coursing from the first diagonal artery toward the mediastinal tumor (Figure 1D). Cardiac magnetic resonance did not demonstrate myocardial late gadolinium enhancement, thereby confirming viability of the myocardium.
Coronary Angiography and Computed Tomography
(A) Cranial, (B) caudal, and (C) transverse coronary angiographic views showing several neovessels with a large tortuous vessel arising from the first diagonal artery and coursing superiorly toward the mediastinum (arrows). (D) Coronary computed tomography confirming the path of the main feeder vessel (arrow) from the first diagonal artery to the mediastinal tumor (asterisks). Also see Videos 1 and 2.
Management
The mechanism of cardiac dysfunction was assumed to be a coronary steal syndrome, and the neovessel was embolized through the first diagonal artery. First, 3 detachable coils (Microplex Hypersoft Helical Coils, Terumo Medical, Somerset, New Jersey) were embolized in the main feeder neovessel arising from the first diagonal artery through a microcatheter (Finecross, Terumo Medical). Then, because of residual flow in the main feeder neovessel, selective alcohol ablation was performed, and 2 ml of absolute alcohol was injected through an over-the-wire balloon, thus allowing the complete occlusion of the vessel (Figures 2A to 2C). The patient's case was extensively discussed at a multidisciplinary meeting. Despite the fact that the mechanism of cardiogenic shock and decrease in ejection fraction was not definite, there was a consensus to address the coronary steal.
Neovessel Coil Embolization and Alcohol Ablation
(A) Main feeder neovessel angiography (white arrow) through the microcatheter (red arrow). (B) Embolization of the main feeder neovessel with 3 coils (arrow) through the microcatheter. (C) Final result showing the coils and the complete occlusion of the main feeder neovessel from the first diagonal artery (arrow).
Follow-up
Nine months later, the patient is still alive. She reports New York Heart Association functional class II dyspnea, and transthoracic echocardiography shows a left ventricular ejection fraction of 48% (Figure 3).
Change of Left Ventricular Systolic Function Assessed by Transthoracic Echocardiography
Polar map of left ventricular longitudinal strain and change of left ventricular volumes, ejection fraction, and global longitudinal strain from the diagnosis (March) to the follow-up (December). GLS = global longitudinal strain; LVEDV = left ventricular end-diastolic volume; LVEF = left ventricular ejection fraction; LVESV = left ventricular end-systolic volume.
Discussion
Neuroendocrine tumors are rare tumors that tend to divert vascularization from surrounding organs. When mediastinal neuroendocrine tumors adhere to the surrounding myocardium, they mostly derive their blood supply from the coronary arteries (2), and this can cause a coronary steal syndrome. Coronary steal is known to play an important role in the pathogenesis of myocardial contractile impairment (3). Coil embolization through the branch of the coronary artery can be a way to treat tumor proliferation (4). To our knowledge, this is the first case showing that heart failure can be induced by this type of vasculature misappropriation and the first experience demonstrating that coil and alcohol embolization of the neovasculature through the coronary arteries can normalize left ventricular systolic function.
Conclusions
Myocardial ischemia is a classic cause of left ventricular systolic dysfunction. Among the nonatheromatous causes of myocardial ischemia, coronary steal is rare and must be treated by limiting the diversion of coronary flow to restore physiological myocardial perfusion.
1. : "The emergence of a new specialty of oncocardiology". Postgrad Med J 2017; 93: 59.
2. : "Cardiac paragangliomas". J Card Surg 2015; 30: 55.
3. : "Relation between coronary “steal” and contractile function at rest in collateral-dependent myocardium of humans with ischemic heart disease". Circulation 1999; 99: 2510.
4. : "Coil embolization of a left circumflex feeder branch in a patient with a mediastinal paraganglioma". J Am Coll Cardiol Intv 2011; 4: 1345.
Footnotes
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
