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November 30, 2021 Circulation. 2021;144:e368–e454. DOI: 10.1161/CIR.0000000000001029 e406 CLINICAL STATEMENTS AND GUIDELINES actions, low pain thresholds, hyperbody vigilance, sympathetic activation, as well as anxiety, depression, and panic disorder.6,7,9,14,23-30 It has been reported that these patients undergo extensive and repetitive cardiac testing and have low referral to cognitive-behavioral therapists, suggesting a lost opportunity for pharmacologic or cognitive-behavioral therapy.6 Recommendation-Specific Supportive Text 1. Most low-risk patients presenting to the ED or office setting with chest pain do not have life-threatening conditions. Diagnoses may include psychological entities such as somatization or noncardiac chest pain.1-13 It has been reported that in low-risk chest pain patients without evidence of cardiac disease, depression, anxiety, and gastroesophageal syndromes each exceeded CAD by almost 10-fold.7 Additionally, care of these patients often includes multiple tests, high cost, and avoidable radiation exposure (5.0 mSv).6 A low rate (<10%) of clinician inquiry, documentation, or referral has also been noted for psychological factors, even in chest pain patients with self-reported anxiety.6,7 A systematic review of therapy for patients with chest pain, no evidence of cardiac disease, and psychological disorders revealed that antidepressants and anxiolytics had mixed evidence for efficacy,10 but a Cochrane database of psychotherapy (17 RCTs) for such patients revealed a 32% reduction in chest pain frequency11 for a 3-month interval. Approaches using cognitive-behavioral methods were most effective.11 These results were limited by small study cohorts and patient heterogeneity; however, they do suggest benefit from consideration of psychogenic factors in patients who continue to seek evaluation for chest pain despite previous definitive, negative workups. 4.3.3 Evaluation of Acute Chest Pain in Patients With Sickle Cell Disease Recommendations for Evaluation of Acute Chest Pain in Patients With Sickle Cell Disease Referenced studies that support the recommendations are summarized in Online Data Supplement 26. COR LOE Recommendations 1 B-NR 1. In patients with sickle cell disease who report acute chest pain, emergency transfer by EMS to an acute care setting is recommended.1-5 1 C-LD 2. In patients with sickle cell disease who report acute chest pain, ACS should be excluded.3-5 Synopsis Acute chest syndrome is a leading cause of death for patients with sickle cell disease.1,2 Patients with sickle cell disease who are experiencing chest pain require prompt evaluation.3 Although chest pain occurs in most, other manifestations of acute chest syndrome include shortness of breath, fever, arm and leg pain, and the presence of a new density on chest radiography. Older adolescents and adults with sickle cell disease who present with chest pain and shortness of breath should be evaluated for AMI or myocardial ischemia.4 AMI occurs in patients with sickle cell disease at a relatively early age, usually without the traditional risk factors for ACS. Death from ACS in patients with sickle cell disease is significantly high in age-, sex-, and race-matched controls.5 Recommendation-Specific Supportive Text 1. In patients with sickle cell disease who experience chest pain, ACS is associated with significant morbidity and mortality rates. These patients should be transferred to an acute care setting by EMS when there is clinical suspicion of ACS. 2. The recommended diagnostic evaluation for all adults with sickle cell disease who have a clinical presentation concerning for acute chest syndrome includes an ECG, troponin test, complete blood count with white blood cell differential, reticulocyte count, anteroposterior and lateral chest radiograph, and blood and sputum cultures. 5 EVALUATION OF PATIENTS WITH STABLE CHEST PAIN 5.1 Patients With No Known CAD Presenting With Stable Chest Pain Stable chest pain is a symptom of myocardial ischemia characterized by chest pain that is provoked with stress (physical or emotional). Risk status in suspected stable ischemic heart disease (SIHD) is not well defined. Figure 11 provides a description of SIHD risk estimates.1 5.1.1. Pretest Risk Probability to Guide Need for Stress and Anatomic Tests In the evaluation of symptomatic patients with suspected CAD, use of validated scores to predict the pretest probability of obstructive CAD may be useful to identify low-risk patients for whom testing may be deferred. It is preferable to use contemporary estimates such as those published in the past 10 years, such as the pretest probability proposed by Juarez- Orozco et al.1 in preference to scores from historical patient series, which may overestimate the frequency of obstructive CAD. Alternatively, low-risk patients may be those <40 years of age or who have symptoms that have a low likelihood of representing ischemia (Section 5.1.2). When available, information on the presence and amount of CAC may be useful for enhancing the pretest probability of obstructive CAD, as shown in Figure 11.2 This information can be ob- Downloaded from http://ahajournals.org by on October 7, 2026