The Clinical Manifestations of Painless Aortic Dissection

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The Clinical Manifestations of Painless Aortic Dissection: A 3-year Retrospective Analysis of a Sample from China

Background: Painless AD cases have been reported in several studies. Its atypical manifestations, especially patients with no pain, were too easy to be neglected, thereby increasing mortality. As some study showed that the mortality rate of painless AD was higher than patients who experienced pain. But the related studies on clinical manifestations and diagnosis in painless AD patients were less.

Methods: Electronic medical records of AD patients (N=679) admitted to a hospital in China between January 1, 2010 and March 31, 2013 were reviewed. In total, 61 (8.98%) painless AD cases were identified and retrospectively analyzed.

Results: The painless AD patients had significantly higher mortality (18.3% vs. 9.39%,P=0.033)than AD patients with pain; Most painless AD patients were classified as DeBakey type III (65.57%) in china. The initial symptoms of painless AD patients included chest tightness, cough, central nervous system symptoms (syncope, dizziness, headache, numbness, and weakness), gastrointestinal bleeding, and discontinuous macroscopic hematuria.

Conclusions: It was difficult to make a definitive diagnosis about painless AD. Given its high mortality, medicals should be aware of those atypical symptoms and its differential diagnoses, thereby shortening the time from symptom onset to treatment and decreasing its mortality.

INTRODUCTION

Acute dissection of the aorta can be one of the most dramatic cardiovascular diseases. Classically, aortic dissection presents as sudden, severe chest, back, or abdominal pain

that is characterized as ripping or tearing in nature1. When considering its typical manifestations (abrupt onset of severe chest and/or back pain; the pain may be sharp, ripping, tearing, knife-like, and typically different from other causes of chest pain),and facilitated by the continuous development of non-invasive examination technology, the clinical diagnosis of AD is relatively easy to make.2However, a timely diagnosis can be elusive in the event of an atypical presentation. Some atypical painless AD cases had been reported in several recent publications, these cases had no pain when onset.3-5Because of a lack of studies on the clinical manifestations of painless AD, critical care medical staffs were unaware of its features and are difficulty to give corresponding measures, thereby may increase the AD-related mortality rate. As some study showed that the mortality rate of painless AD was higher than that of the AD patients who experienced pain6.

Current studies on the initial symptoms of painless AD are relatively few. Therefore, in the present study, we aimed to perform a 3-year retrospective analysis of the clinical characteristics, initial symptoms, diagnosis and the possible mechanism of these diagnostic problems in patients with painless AD. The goal was to provide evidence of being alert and vigilant for the presentation of it, thus decreasing painless AD-related mortality.

METHODS

Patient Population

Data from patients were included in the study if the patients (1) met the diagnostic criteria of AD by exhibiting the diagnostic findings (true and false lumen or nomadic intimal flap) on diagnostic imaging; and (2) be diagnosed with painless AD because they did not complain of pain on any part of their body at the initial presentation. Patients with cerebral and/or cervical artery dissection and patients with an AD secondary to trauma were excluded from the study. In total, 679 patients with AD were admitted to our hospital from January 1, 2010 to March 31, 2013; among them, 61cases of painless AD were identified.

Data Collection

Using “aortic dissection” as a keyword, we searched all admitting and/or discharge diagnoses that included AD, from January 2010 to March 2013, in the electronic medical records and emergency medical services documentation of Tong Ji Hospital, Tong Ji Medical College, University of Huazhong Science and Technology, Wuhan, China. We exported the original medical records of all of the cases and assessed them individually. There were 61 cases of painless AD that fit the inclusion and exclusion criteria. A questionnaire was used to collect data, including information on patient demographics, medical history, admission and discharge diagnosis, initial symptoms, imaging study results, and in-hospital management and outcomes. Microsoft Office Excel 2007 (Microsoft Corp., Redmond, Washington, USA) was used to create the AD database, and clinical features and information of missed diagnoses and misdiagnoses of painless AD patients were retrospectively analyzed.

The classification of AD was based on the DeBakey classification system (Type I, Type II, Type III).The “unclear” classification consisted of patients with an unclear diagnosis of AD. Hypertension was considered when the patient had a systolic blood pressure of ≥140mmHg, and/or diastolic blood pressure ≤90mmHg, or when the patient was taking antihypertensive medication. The patients’ clinical manifestations at the onset of symptoms or during admission to the hospital were considered as the initial symptoms. Painless AD patients were defined as those whose initial symptoms did not include pain. Misdiagnosis referred to instances in which the admission diagnosis was different from painless AD, and missed diagnosis referred to instances in which there was no admission diagnosis of painless AD. The prognosis was classified as “getting better”, “critical”, and “death” in the discharge record.

Data Analysis

Data are presented as mean ± standard deviation for continuous variables, or number (percentage) for discrete variables. Statistical analyses were performed with SPSS Statistics 20.0 software (IBM Corporation, Armonk, NY, USA). The chi-squared test was used to compare discrete variables. Student’s t-test was used to analyze between-group comparisons of continuous variables. P values less than 0.05 were considered statistically significant.

RESULTS

We reviewed data from 679 AD patients admitted to our hospital from January 2010 to March 2013. These cases consisted of 618 typical AD patients who exhibited painful signs and symptoms (pain group) and 61 cases of painless AD (painless group). There was no significant difference in the incidence of hypertension between the pain and painless group (52.5%versus 69.9%, respectively; P=0.72).The painless group was significantly older (58.62±18.36 versus 52.72±11.46years, P<0.01) and had significantly higher rates of mortality (18.3% versus 9.39%, P=0.033) and misdiagnosis (32.79% versus 10.52%, P<0.01) than the pain group. Furthermore, 24 patients were along with other related medical history: heart dysfunction, coronary heart disease, pulmonary infections, and bronchitis. For the misdiagnosis patients, nine were admitted to the Department of Cardiology, and 11 were admitted to other departments.

According to the DeBakey types, 40(65.57%) painless AD patients being Type III; 4(6.56%), Type II; and 16(26.23%), Type I. 2 of the Type III patients, 1 of the Type II patients, and 7 of the Type I patients died, and the mortality rate of the Type I patients was significantly higher than that of the Type III patients (43.75% versus 5.00%, P<0.01); this was not compared in Type II patients because of the small sample size. Most of the painless AD patients were admitted to the Cardiovascular Clinical Department and the Cardiothoracic Surgery Department (32 [52.46%] and 17 [27.87%], respectively), while 12(19.67%) were admitted to other departments such as the Department of Neurology, Gastroenterology, Nephrology, and Intensive Care Unit.

The initial symptoms of the painless AD patients included chief complaints of being unaware of their symptoms(9 cases),a sensation of tightness in the chest and/or shortness of breath (29 cases), neurological symptoms(i.e., dizziness, headache, syncope, limb with sensory or motor deficits; 20 cases), gastrointestinal hemorrhage (2 cases),and gross hematuria (1case).

DISCUSSION

Clinician Vigilance of Painless Aortic Dissection

Pooled data from more than 1000 patients in 8 studies revealed that the pain of acute AD is abrupt in onset in 84% of the cases (95% confidence interval [CI], 80–89%) and that its intensity is severe in 90% of the cases (95% CI, 88–92%).7However, in the present study, the initial symptom presentation did not consist of pain in 61 out of 679 AD cases(8.98%), and this rate was higher than that reported by Park et al. (6.4%)6 and lower than that reported by Imamura et al.(17%).4 A specific proportion of atypical cases of painless AD is present among the total cases of AD. Similar to the results of Park et al.6, this study found that the mortality rate of painless AD (18.3%) was higher than that of the AD patients who experienced pain. Given this high mortality rate, the identification of atypical symptoms of painless AD and its related diagnoses, and a high degree of suspicion for its presence in diagnostic evaluations, is necessary. An early and definitive diagnosis as well as a timely and effective treatment may help reduce the mortality rate of patients with painless AD.

Clinical Manifestations of Painless Aortic Dissection

Clinical Features

Our findings are consistent with those of a study conducted by Park et al.; they found that patients with painless AD were older than those with painful AD.6 Differential from other studies, in the present study, most of the painless AD patients were classified as Type III, and the mortality rate of Type I patients was significantly higher than Type III (P<0.01).

The rate of missed diagnoses in patients with AD can reach 38% at the first assessment, and 28% of patients have missed diagnoses at their autopsy.1 In the present study, the missed diagnosis rate of AD was significantly higher in the painless group than in the pain group (P<0.01). One key reason for this finding is likely the lack of typical pain, so it is important for critical care nurses to master the manifestations of painless AD.

Initial Symptoms

The complexity of the clinical symptoms of painless AD increased the difficulty of making an early and definitive diagnosis.

  • Chest tightness and shortness of breath accompanied by palpitations, fatigue, or lower extremity edema (29/61, 47.54%) Some of the patients were also along with heart dysfunction, coronary heart disease, pulmonary infections, and bronchitis (24 cases);
  • Central nervous system (CNS)symptoms (20/61, 32.79%) The CNS symptoms that presented as initial symptoms or in combination with other symptoms of AD had been reported in a considerable number of studies in recent years.8-10The dizziness, headache, hemiplegia, aphasia, and other CNS symptoms in AD patients could result from the extension of the dissection along the innominate or cephalic artery, which cuts off the blood supply to the brain or spinal cord. Among these symptoms, syncope is well-recognized as being related to dissection.11,12 Approximately 5% of cases had syncope caused by AD, and also presented with relevant symptoms (cardiac tamponade, hemothorax, cerebral ischemia, or cerebral embolism). When dissection involving the carotid, subclavian, or iliac arteries can cut off the blood supply to the limbs, thus resulting in numbness, weakness, and other related symptoms. Henke et al.13 found that symptoms of limb ischemia and end-organ ischemia may be related to increased AD-related mortality, and that AD patients with lower limb ischemia had a higher fatality rate. Therefore, observations of limb pulses and blood pressure changes, which reflect the degree of hypoperfusion, may help detect the presence of AD.
  • Not being aware of any symptoms and diagnoses were made by physical examination (9/61, 14.75%) Although these patients did not exhibit any signs or symptoms of AD, their medical histories indicated AD risk factors (e.g., high blood pressure, coronary heart disease, and myocardial infarction). This finding should remind critical care nurses that subjects with AD risk factors should undergo medical examinations on a regular basis to ensure the early detection and treatment of AD.
  • Gastrointestinal bleeding and no history of any other disease (2/61, 3.28%) When the dissection separation involves the large branch of the aorta, corresponding symptoms of insufficient blood supply to the viscera may develop.14A hematoma formation or an intimal artery tear causing gastrointestinal bleeding or hematochezia could result from perfusion deficiency of the abdominal aorta or superior mesenteric artery.

Macroscopic hematuria (1/61, 1.64%) In the present study, one patient diagnosed with an abdominal AD—namely, the dissection crevasse tore from the aorta to the right iliac artery. Urinary tract symptoms could occur when the dissection involves the renal artery. Therefore, the critical nurses should take AD into consideration, when patients present with the above-mentioned symptoms with no clear explanation.

CONCLUSION

The initial symptoms of painless AD were complex, diverse, and not easily detected, resulting in difficult diagnosis and evaluation. Painless AD patients had higher rates of missed diagnosis and mortality than those with pain. Given the high mortality rate, it is necessary to be aware of the atypical symptoms and differential diagnoses, and to maintain a high degree of suspicion of its presence. By improving the rate of accurate and timely diagnosis of this disease, to give corresponding measures, and the time from the onset of symptoms to treatment and the mortality rate of AD can be ultimately decreased.

IMPLICATIONS FOR CARDIOTHORACIC SURGERY MEDICALS

AD is a kind of critical disease, and these patients often are admitted to cardiothoracic surgery department immediately. It is important to be knowledgeable on the subject of painless AD and to aid specialist physicians in the vigilance of this disease. In the presence of potential cases of acute AD, the patients should have their pulses checked in all extremities to identify the presence of perfusion deficits. The patient evaluation should include the relevant medical history, clinical characteristics, and an assessment of atypical symptoms at their initial presentation. This study comprehensively analyzed the clinical characteristics, initial symptoms about painless AD. Our findings may help those medicals to make a correct and timely evaluation and diagnosis, and implement corresponding treating measures.

LIMITATIONS

This study had some limitations. This was a retrospective analysis of patients in only one clinical setting. Therefore, our sample might be biased. In addition, because there were few cases of painless AD, we only explained a few of the mechanisms underlying the initial symptoms. In order for critical care nurses to understand and recognize the relevant symptoms more easily, the underlying mechanisms of painless AD should have been elucidated more thoroughly. Further studies on the initial symptoms of painless AD are needed to expand our knowledge of this disease.

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