Successful rescue from acute myocardial infarction in an anemic woman with anomalous origin of the left main coronary artery from the right coronary artery: a case description and literature analysis

Introduction

Anomalous origin of the left main coronary artery (LMCA) from the right coronary sinus (RCS) is a rare anatomical anomaly of the heart. Under normal circumstances, the LMCA originates from the left coronary sinus, whereas the right coronary artery (RCA) originates from the RCS. However, when the origin of the LMCA is within the RCS, this feature is referred to as an LMCA arising from the RCS. Some patients may be asymptomatic, whereas others may present with symptoms such as angina, abnormal electrocardiogram findings, abnormal cardiac ultrasound, or even sudden death (1). In this case report, a woman who experienced scar pregnancy, medical abortion, and hysteroscopic removal of retained placental tissue developed heart failure and was diagnosed with a rare congenital anomaly of her left coronary artery (LCA).

Case presentation

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient for publication of this article and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

A 34-year-old female patient had a history of caesarean section. At 73 days of gestation, she underwent medical abortion due to pregnancy at the caesarean scar site (Figure 1A), resulting in expulsion of the fetus but incomplete removal of placental tissue. She subsequently received methotrexate (MTX) chemotherapy to promote the expulsion of placental tissue, but the treatment was not successful. She underwent a follow-up for 77 days. During the monitoring period, she received weekly human chorionic gonadotropin (HCG) and ultrasound assessments and was able to access online consultations at any time. Her HCG level decreased from 144,384 mIU/mL (26/8/2023) to 181.60 mIU/mL (8/11/2023). During the follow-up, the patient experienced intermittent slight vaginal bleeding but had no vaginal tissue discharge. The ultrasound results on 26 August 2023 revealed placental tissue located in the lower segment of the anterior wall of the uterus, measuring 60 mm × 31 mm × 46 mm, with abundant blood flow signals detected (Figure 1B, US1). The ultrasound results on 8 November 2023 indicated that the size of the placental tissue measured 70 mm × 50 mm × 70 mm and was still located in the lower segment of the anterior wall of the uterus, with blood flow signals observed (Figure 1B, US2). On 8 November 2023, her white blood cell count was 6.8×10⁹/L, red blood cell count was 5.58×10¹²/L, hemoglobin concentration was 110 g/L, neutrophil percentage was 58.2%, and platelet count was 225×10⁹/L. During ultrasound-guided hysteroscopic evacuation, the patient experienced severe bleeding, and approximately 80 grams of placental tissue (Figure 1C) was removed intraoperatively. Despite receiving blood transfusions and treatment to promote uterine contraction, the patient remained anemic postoperatively (hemoglobin 66 g/L), with continued minimal vaginal bleeding. During the 24-hour post-operative monitoring period, she remained conscious, showed no dizziness or chest discomfort, experienced minimal vaginal bleeding, and had stable vital signs. However, 28 hours postoperatively, she developed severe chest pain accompanied by hypotension and altered consciousness, necessitating transfer to the intensive care unit (ICU) for further management. In the ICU, the patient received vasopressor therapy to maintain blood pressure but subsequently experienced cardiac arrest, requiring cardiopulmonary resuscitation (CPR) followed by extracorporeal membrane oxygenation (ECMO) therapy and tracheal intubation-assisted ventilation. During her ICU treatment, she also underwent continuous renal replacement therapy (CRRT). The ECMO tube was removed 7 days after her condition had stabilized. However, before the ECMO was removed, there was a rupture of the ECMO vessel (right side), and she underwent ECMO weaning along with right femoral artery repair and right femoral vein repair. To clarify the cause, pulmonary cardiac computed tomography angiography (CTA) and coronary angiography (CAG) were performed, which indicated that the LMCA arose from the RCS (Figures 2,3). Rehabilitation treatment was carried out for 1 month during ICU observation. Her mental state and diet were both good, and she could stand on her own, but her daily urine output was still less than 100 mL. CRRT therapy was continued. On 30 November 2023, she experienced another sudden drop in blood pressure, for which pressor and noninvasive respiratory therapy were ineffective. Owing to unstable hemodynamics and poor heart function, the patient was again placed on ECMO. After 6 days (6/12/2023), the ECMO device was removed, and due to severe mitral regurgitation, intra-aortic balloon counterpulsation (IABP) retention surgery was performed. After a further 5 days (11/12/2023), the IABP was removed, and due to low urinary output, ECMO and CRRT treatment were continued. During the ICU treatment, the patient underwent blood pressure maintenance, rehabilitation, and traditional Chinese medicine treatment. However, she still experienced chest tightness and discomfort after physical activity, repeated drops in blood pressure, fluctuations in mean arterial pressure between 60 and 85 mmHg, moderate mitral regurgitation, pulmonary hypertension, and left ventricular systolic dysfunction. On 6 February 2024, she underwent correction of the abnormal origin of the LCA (LCA decapitation surgery), mitral valve repair, temporary pacemaker implantation, mediastinal drainage tube implantation, and pericardial drainage tube implantation. No significant pericardial effusion was observed, and the mediastinal pericardial drainage tube was removed. The temporary pacemaker was not activated after surgery. On the 8th day (14/2/2024), the temporary pacemaker was removed, and on the 8th day after surgery, there was pleural effusion on the right side. A chest ARROW tube was placed for drainage for 2 days (16/2/2024), and 720 mL of drainage fluid was removed. After surgery, maintenance of vital signs, maintenance of the internal environment, anti-infection, antiplatelet aggregation, diuresis, and enhanced nutrition therapy were given. After 20 days of kidney function recovery (20/2/2024), the patient was transferred out of the ICU and continued with rehabilitation treatment in the general ward. Postoperative CAG indicated that the coronary arteries were patent (Figure 4, 6/8/2024). The entire procedure is shown in Figure 5.

Figure 1 Imaging and pathological description of scar pregnancy mass. (A) An intrauterine gestational sac measuring 66 mm × 21 mm is observed by MRI. Within the sac, there are mixed masses resembling the embryonic form. The placenta is located on the anterior wall of the uterus, with its lower edge covering the internal cervical os. The boundary between part of the placenta and the anterior isthmic portion is indistinct. (B) In the uterine cavity, mixed echogenicity with blood flow is observed by US (US1). The demarcation between the anterior wall and the isthmic portion of the muscular layer is indistinct, although the blood flow signal has decreased compared to before (US2). (C) The scar pregnancy mass was confirmed to be degenerated necrotic placental tissue with calcification by HE staining analysis (100×). MRI, magnetic resonance imaging; US, ultrasound; HE, hematoxylin and eosin.

Figure 2 The coronary artery shows a right-dominant pattern, with the left main stem originating from the right sinus and the right coronary artery also originating from the right sinus. IPL, intermediate posterior left; LAD, left anterior descending artery; LI, left intermediate; RCA, right coronary artery; SAR, superior anterior right.

Figure 3 The coronary artery demonstrates a right-dominant pattern, with the right coronary artery originating from the right sinus and the left coronary artery opening in the upper part of the right sinus. There was approximately 80–90% eccentric narrowing in the proximal segment of the left main stem.

Figure 4 The patient underwent surgical correction of the anomalous origin of the LCA under cardiopulmonary bypass, and six months post-operation, the coronary cardiac CTA showed normal blood flow in the coronary arteries. AR, anterior right; CTA, computed tomography angiography; LAD, left anterior descending artery; LCA, left coronary artery; PL, posterolateral branch; RCA, right coronary artery; SRP, superior right posterior.

Figure 5 Flowchart of the entire event. CPR, cardiopulmonary resuscitation; ECOG, extracorporeal membrane oxygenation; IABP, Intra-aortic balloon counterpulsation; ICU, intensive care unit; MRI, magnetic resonance imaging.

Discussion

Coronary artery anomalies encompass anomalies in the origin and distribution of coronary arteries, anomalous numbers of coronary artery branches, and coronary artery fistulas. The etiology of these anomalies remains unclear but may be related to abnormalities during embryonic heart development. The hallmark is an anomaly in the origin or development of any of the three major epicardial coronary arteries (2-4).

According to the literature, the overall detection rate of coronary artery origin anomalies is approximately 0.6–1.2%, with various anomalies of LCA origin accounting for 73.3–77.4% of these anomalies, and a left circumflex artery originating from the RCS or RCA is the most common (5). The LMCA originating from the RCS is an extremely rare and prognostically severe anomaly of coronary artery origin, with reported incidence rates ranging from 0.017% to 0.03% (6).

Approximately 40% of patients experience sudden death, with up to 38–66% of patients with coronary artery anomalies experiencing sudden death without prior symptoms (7). The incidence of sudden death during or shortly after exercise is relatively high (8). Clinically, the diagnosis rate ranges from 0.5% to 40%, with a discovery rate of 85% in 15% of autopsies. This difference suggests that not all coronary artery anomalies cause symptoms, but they may be discovered during autopsies following sudden death (9). The LMCA originating from the RCS has four courses: (I) a posterior course behind the aorta; (II) originating from the RCS anteriorly and coursing between the aorta and pulmonary artery (PA); (III) coursing through the interventricular septum; and (IV) coursing anteriorly through the right ventricular outflow tract (10). This case involves an ectopic LCA originating from the right sinus of Valsalva and running between the aorta and the pulmonary trunk left coronary sinus. It originates from the anterior aspect of the RCS and travels between the aorta and the PA. Anemia induces myocardial contraction, which may cause episodic ischemia due to vessel compression during systolic expansion of the aortic root, ultimately resulting in myocardial infarction and sudden cardiac death (SCD) (11). For imaging examinations of the anomalous origin of coronary arteries, echocardiography is noninvasive and radiation-free, aiding in detecting the coronary artery ostium and proximal course in most children, but its effectiveness in adults is limited (12,13). Coronary computed tomography angiography (CCTA) is an accurate and sensitive noninvasive alternative to conventional CAG and is now widely used in clinical practice. It can accurately provide diagnostic information regarding the origin, course, and anatomical relationship with major vessels of the coronary arteries and can obtain high-resolution three-dimensional reconstructed images. Compared with CCTA, cardiac magnetic resonance imaging (MRI) examination does not involve radiation exposure, making it more suitable for young patients, and it can assess cardiac size and function, blood flow, perfusion, myocardial viability, and postoperative complications (14). CCTA examination has been recommended by the European Society of Cardiology as the preferred diagnostic method for confirming a suspected anomalous origin of the coronary arteries, and the American Heart Association Cardiovascular Imaging Committee has classified CCTA or MRI examination as a class IIA recommendation with level B evidence for diagnosing anomalous origin of the coronary arteries (15). The lack of detection of cardiac vascular malformations in this patient’s preoperative ultrasound may be attributed to the insensitivity of ultrasound screening for cardiac vascular malformations in adults.

The treatment-related issues of the anomalous origin of the coronary artery depend on the location and characteristics of the lesion. Other anatomical variations, such as the anomalous retro-aortic course of the LCA, the free wall course of the right ventricle, and the interventricular septum course at the base of the right ventricle, are considered benign (16). However, the anomalous course of the LMCA (ALM) arising from the right sinus of Valsalva, with the coronary artery traversing between the PA and the aorta, increases the risk of malignant arrhythmias and sudden death (17). This increased risk may be due to the anomalous course of the coronary artery, resulting in compression, or the provision of a greater myocardial mass by this type of anomaly. The presence of a slit-like origin and intramural course of the proximal coronary artery from the contralateral sinus may play a significant role in coronary ischemia and malignant arrhythmias (18). Anginal symptoms and malignant arrhythmias typically occur during exercise, likely owing to myocardial ischemia, which may be caused by any or all of the aforementioned factors.

Traditionally, surgical treatment has been recommended for patients with symptoms or evidence of myocardial ischemia and ventricular arrhythmias because of their increased risk of death. Among the various surgical methods described, coronary artery reimplantation involves opening the intramural course of the main stem from within the aorta with the aim of relieving the stenosis of the intramural segment and positioning the coronary artery opening within the correct sinus of the aorta. In addition, percutaneous coronary intervention (PCI) and stent placement are potential alternative options that offer good immediate results, but long-term data are lacking (19).

This patient underwent surgical correction of the anomalous origin of the LCA under cardiopulmonary bypass, mitral valve plasty, temporary pacemaker implantation, mediastinal tube drainage, and pericardial tube drainage, and the surgery went smoothly. The procedure relieved the pressure on the patient’s LMCA and restored cardiac blood flow. The patient recovered well after surgery, with no occurrence of cardiogenic shock.

The endotracheal tube was removed 2.7 days postoperatively, and the patient was treated with digoxin for cardiac strengthening, furosemide for diuresis, aspirin for antiplatelet aggregation, and rehabilitation, after which she gradually recovered. Surgical treatment of the anomalous aortic origin of a coronary artery (AAOCA) has a good prognosis, with symptoms disappearing in 97% of patients postoperatively and a cardiac mortality rate of <1% (20).

In this case, the patient’s chest tightness disappeared after surgery, and there was no cardiogenic shock during the 2-month postoperative period. Currently, the patient is on medication to maintain cardiac function, and is being closely monitored for any subsequent symptoms.

In addition to vigorous exercise, anemia caused by bleeding is also a factor leading to angina pectoris, malignant arrhythmias, cardiogenic shock, or even sudden cardiac arrest in patients in whom the LCA originates from the RCS. This case reveals a high-risk factor for sudden death due to this coronary vascular anomaly, especially in female patients with such anomalies, who should avoid anemia due to pregnancy to prevent myocardial ischemia and even sudden cardiac arrest. Therefore, early detection of cardiovascular anomalies is crucial. However, owing to the limited detection capabilities of adult echocardiography for cardiac anomalies, nonessential tests such as CCTA, cardiac MRI, and CAG are not routinely performed. Hence, such coronary vascular anomalies are difficult to detect and may be overlooked. If sudden cardiac arrest occurs without timely rescue, this anomaly may only be discovered through autopsy. Therefore, timely surgical correction of anomalies affecting coronary perfusion is necessary to prevent the occurrence of life-threatening cardiovascular events.

Conclusions

A LMCA originating from the RCS is an extremely rare and prognostically severe coronary artery origin anomaly. Owing to the limited diagnostic ability of adult echocardiography for detecting cardiac abnormalities, the diagnostic rates of noninvasive tests such as CCTA, cardiac MRI, and CAG are low in adults. However, the incidence of sudden death associated with this AAOCA is high. In female patients, factors that may trigger sudden death, in addition to strenuous exercise, include heavy menstrual bleeding, postpartum hemorrhage, and various other causes of bleeding leading to anemia. Given the high risk of sudden death associated with the LMCA originating from the RCS, patients with clinically malignant anomalies must undergo surgical treatment to prevent catastrophic complications resulting from ischemic events.

Acknowledgments

None.

Footnote

Funding: This work was supported by Zhejiang Provincial Basic Public Welfare Research Plan Project (No. LGD21H040001), Zhejiang Medical and Health Science Project (No.2022KY442), and Zhejiang Province Medical and Health Talent Training Project (No. 2022).

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://qims.amegroups.com/article/view/10.21037/qims-24-1380/coif) and report that this work was supported by Zhejiang Provincial Basic Public Welfare Research Plan Project (No. LGD21H040001), Zhejiang Medical and Health Science Project (No.2022KY442), and Zhejiang Province Medical and Health Talent Training Project (No. 2022). The authors have no other conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient for publication of this article and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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