Acute aortic dissection (AD) with a tear in ascending aorta (AA) is recognized to masquerade occasionally as another condition, and therefore the proper diagnosis is never made or made too late. During a recent 3-year period (2009 to 2011) at Baylor University Medical Center at Dallas, 30 patients with acute AD with tear in AA were diagnosed: 23 (77%) were diagnosed clinically and underwent proper urgent operative therapy; the remaining 7 (23%) with AD with tear in AA were not diagnosed until operation (for another condition) or necropsy or retrospectively by review of antemortem computed tomographic scan after death. The number of patients with AD from tear in AA whose cardiac condition was not diagnosed clinically and who died in the hospital but did not have an autopsy is unclear. Because the autopsy rate in most United States hospitals, including Baylor University Medical Center, is now <5%, many cases of AD are probably not diagnosed and thus its frequency is being underestimated. In conclusion, ≥7 of 30 patients (23%) with acute AD with a tear in AA were not diagnosed until necropsy or operation (for another condition) or retrospectively by computed tomography during a 3-year period at a large tertiary medical center showing that this condition continues to be underdiagnosed, and when not diagnosed correctly is usually rapidly fatal. Because the autopsy rate today is so low, the percentage not diagnosed clinically is probably >23%.
It is well known that acute aortic dissection (AD) can masquerade as another condition or that the correct diagnosis can be delayed. In a recent 36-month period at Baylor University Medical Center (BUMC) at Dallas, 30 patients with acute AD with tear in ascending aorta (AA) were diagnosed: 23 of them went immediately to the operating room where proper operative therapy was carried out; 4 others underwent operative therapy for another condition (coronary bypass in 3 and closure of atrial septal defect in 1) and the presence of AD with tear in the AA was not recognized until operation or necropsy; and 3 others died suddenly in the hospital (hemopericardium) without operation and the AD was a surprise finding at autopsy. A review of those 30 cases is the purpose of this report to call attention again to the frequency of underdiagnosis of this condition.
Methods
The surgical pathology files of BUMC were searched for patients having resection of the AA for acute AD with a tear in the AA over a 3-year period (2009 to 2011), and the necropsy files of BUMC were searched during the same 3-year period for patients having fatal AD with a tear in AA. The aortas in all patients having operative resection for AD with a tear in AA were examined initially by W.C.R. who provided the report (10 of the 23 operative cases during this 3-year period were included in a study of 69 patients with AD studied during a 16-year period ). The hearts and aortas in the patients initially found to have AD with a tear in AA at autopsy were studied initially by J.M.G. and W.C.R. The photographs of the heart and aorta were taken by J.M.K.
The clinical records including admission history and physical, discharge summary, echocardiographic reports, cardiac catheterization reports, computed tomographic (CT) and coronary angiographic studies, operative records, and autopsy records were sought from the patients’ medical charts at BUMC.
Results
Three groups of patients were analyzed. The largest group consisted of 23 patients who were diagnosed clinically as having AD with tear in AA and they rapidly underwent proper operative replacement of the AA ( Table 1 ).
Patient Number | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 | 11 | 12 | 13 ⁎ | 14 | 15 | 16 | 17 | 18 † | 19 | 20 | 21 | 22 | 23 |
Age (years) | 34 | 35 | 38 | 42 | 45 | 47 | 52 | 52 | 53 | 55 | 59 | 64 | 68 | 70 | 70 | 72 | 72 | 73 | 74 | 74 | 74 | 75 | 82 |
Gender | M | F | M | M | F | F | M | F | M | M | M | M | F | M | M | M | M | M | M | F | F | F | M |
On admission | |||||||||||||||||||||||
Chest pain | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | 0 | 0 | + | + | 0 | 0 | + |
Back pain | 0 | + | + | 0 | + | + | 0 | 0 | 0 | 0 | + | 0 | 0 | 0 | 0 | 0 | + | 0 | 0 | 0 | 0 | + | 0 |
Neck pain | 0 | 0 | + | 0 | 0 | + | + | 0 | 0 | 0 | 0 | + | 0 | 0 | 0 | 0 | 0 | + | 0 | 0 | 0 | 0 | 0 |
Extremity | |||||||||||||||||||||||
Weakness | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | + (L) | 0 | 0 | 0 | 0 | 0 | 0 | 0 | + (L) | 0 | + (L) |
Numbness | 0 | 0 | + (A, L) | + (L) | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | + (A) | 0 | + (L) | + (A, L) | 0 | 0 | 0 | 0 | + (L) | 0 |
Hypertension ‡ | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + |
Highest BP (mm Hg) | |||||||||||||||||||||||
Systolic | 200 | 170 | 130 | 130 | 135 | 210 | 80 | 110 | 205 | 110 | 110 | 130 | 175 | 185 | 105 | 200 | 120 | 125 | 95 | 135 | 150 | 135 | 115 |
Diastolic | 110 | 80 | 50 | 65 | 65 | 100 | 50 | 80 | 100 | 60 | 55 | 70 | 80 | 105 | 55 | 60 | 55 | 55 | 55 | 85 | 60 | 60 | 70 |
Troponin (ng/ml) | <0.03 | 0.06 | — | 0.06 | — | <0.02 | <0.02 | — | 0.09 | <0.03 | — | <0.05 | 0.16 | <0.02 | 2.19 | 0.09 | <0.02 | <0.02 | 0.04 | <0.02 | <0.02 | <0.02 | — |
Precordial murmur | 0 | 0 | 0 | 0 | + (1/6) | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | + (3/6) | + | 0 | 0 | 0 | 0 | + (3/6) | — |
BMI (kg/m 2 ) | 30.0 | 27.4 | 24.7 | 24.8 | 21.5 | 44.0 | 26.6 | 22.0 | 29.1 | 24.4 | 41.4 | 23.6 | 23.4 | 23.5 | 28.2 | 34.9 | 27.1 | 37.8 | 31.1 | 44.7 | 26.6 | 21.0 | 31.2 |
Computed tomography | + | + | + | + | + | + | + | + | + | + | + | + | + | + | 0 | + | + | + | + | + | + | + | + |
Echocardiogram | 0 | 0 | 0 | 0 | + | 0 | 0 | 0 | + | 0 | 0 | 0 | 0 | + | + | 0 | 0 | 0 | + | 0 | 0 | 0 | 0 |
Chest radiograph | + | + | 0 | + | + | + | + | 0 | + | + | 0 | 0 | 0 | + | + | 0 | + | 0 | + | + | + | 0 | 0 |
Interval (hours) | |||||||||||||||||||||||
Symptom onset to admission | 1 | 1 | — | 1 | 13 | 3 | 1 | 1 | 340 | 1 | 1 | 1 | 2 | 340 | 1 | 1 | 3 | 1 | 1 | 1 | 1 | 1 | — |
Admission to operation | 3 | 6 | 5 | 7 | 5 | 16 | 3 | 5 | 15 | 6 | 8 | 7 | 5 | 42 | 15 | 2 | 3 | 6 | 17 | 13 | 3 | 10 | — |
Hemopericardium | 0 | + | 0 | 0 | 0 | 0 | + | + | 0 | 0 | 0 | + | 0 | 0 | + | 0 | + | 0 | + | 0 | 0 | 0 | + |
AMEFL (0–4+) | 1 | 0 | 3 | 0 | 0 | 0 | 4 | 0 | 0 | 0 | 0 | 1 | 0 | 0 | 0 | 0 | 0 | 2 | 0 | 0 | 1 | 1 | 3 |
Portion of aorta dissected | |||||||||||||||||||||||
Ascending | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + |
Arch | + | + | + | + | + | + | + | + | + | + | + | + | 0 | + | + | + | + | + | + | 0 | + | + | 0 |
Descending | + | 0 | + | + | + | + | + | + | + | + | + | 0 | 0 | 0 | + | + | + | + | + | 0 | + | + | 0 |
Abdominal | + | 0 | + | + | + | + | + | + | + | + | + | 0 | 0 | 0 | 0 | + | + | 0 | 0 | 0 | + | 0 | 0 |
⁎ Patient had coronary artery bypass grafting (×3) 7 days before presentation.
† Patient had abdominal aortic aneurysm resection 5 years before presentation.
The second largest group consisted of 4 patients (numbers 1 to 4) in whom the diagnosis of AD with tear in AA was not suspected clinically but all 4 underwent a cardiac operation for another condition (coronary artery bypass grafting in 3 and closure of a secundum atrial septal defect [ASD] in 1) and AD was diagnosed at operation in 2 (patients 2 and 3) and at necropsy in the other 2 (patients 1 and 4; Table 2 ). Review of the clinical and morphologic features of the latter 2 cases strongly suggested that the AD was indeed present before operation but masqueraded as a coronary event or ASD, respectively. The patient with the ASD presented initially with acute dyspnea, echocardiogram disclosed the ASD, and her symptoms were attributed to the ASD rather than the AD. The initial operative incision was a right thoracotomy. The AA was noted to be quite large after median sternotomy. Details of cases 1, 2, and 4 are presented in the legends to Figures 1 to 4 . Details of case 3 are presented in the footnotes of Table 2 .
Patient Number | 1 ⁎ | 2 ⁎ | 3 ⁎ § | 4 † | 5 ‡ | 6 ‡ | 7 ‡ |
---|---|---|---|---|---|---|---|
Age (years) | 57 | 74 | 82 | 52 | 54 | 75 | 76 |
Gender | man | woman | woman | woman | man | woman | woman |
On admission | |||||||
Chest pain | + | + | 0 | + | + | + | 0 |
Back pain | 0 | 0 | 0 | 0 | 0 | + | 0 |
Neck pain | 0 | 0 | 0 | 0 | 0 | + | 0 |
Extremity | |||||||
Weakness | 0 | + (legs) | 0 | 0 | + | 0 | 0 |
Numbness | + (arm) | 0 | 0 | 0 | 0 | + (legs) | 0 |
Systemic hypertension μ | + | + | + | + | + | + | + |
Highest blood pressure (mm Hg) | 130/85 | 120/70 | 190/85 | 150/90 | 135/60 | 130/50 | 255/110 |
Troponin (ng/ml) | 0.07 | <0.03 | 0.10 | — | <0.02 | 0.20 | <0.02 |
Precordial murmur | 0 | 0 | 0 | + | 0 | + | 0 |
Body mass index (kg/m 2 ) | 23.7 | 37.0 | 17.9 | 17.4 | 36.7 | 23.3 | 16.1 |
Bruce stress test | + | 0 | 0 | 0 | + | 0 | 0 |
Computed tomogram | 0 | 0 | 0 | 0 | 0 | + | + |
Echocardiogram | 0 | 0 | 0 | + | 0 | 0 | 0 |
Chest radiograph | + | + | 0 | 0 | + | + | + |
Coronary angiogram | + | + | + | 0 | 0 | 0 | 0 |
Interval | |||||||
Symptom onset to admission (hours) | 12 | 1 | 1 | — | 1 | 1 | 72 |
Admission to operation | 1 day | 2 days | 2 hours | 1 day | — | — | — |
Operation to death (days) | 12 | 12 | alive | 10 | — | — | — |
Admission to death | 13 days | 14 days | alive | 11 days | 66 hours | 10 hours | 37 hours |
Heart weight (g) | 415 | 580 | — | 340 | 595 | 380 | 310 |
Left ventricular wall | |||||||
Necrosis | + | 0 | — | + | 0 | 0 | 0 |
Fibrosis | 0 | 0 | — | 0 | 0 | 0 | 0 |
Left ventricular cavity dilated | 0 | 0 | — | + | + | 0 | 0 |
Hemopericardium (ml) | + (300) | 0 | 0 | 0 | + (750) | + (600) | + (600) |
Aortic medial elastic fiber loss (0–4+) | 0 | — | 2 | 4 | 0 | 0 | 1 |
Portion of aorta dissected | |||||||
Ascending | + | + | + | + | + | + | + |
Arch | + | + | 0 | + | + | + | + |
Descending thoracic | + | + | 0 | + | + | + | + |
Abdominal | — ¶ | + | 0 | — ¶ | — ¶ | + | — ¶ |
Illustrated in figure(s) | 1 and 2 | 3 | — | 4 | 5 | 6 | 7 |