When ulcer isn’t ulcer: A case report of a fatal case report of Stanford Type A aortic dissection presenting as epigastric pain

INTRODUCTION

Dissecting aortic aneurysm, also known as aortic dissection, is a life-threatening cardiovascular emergency resulting from a tear in the intimal layer of the aorta.^[1-8] This allows blood to enter the media, forming a false lumen that may propagate antigrade or retrograde, compromising major branches or causing rupture.^[1-8] Although relatively rare, aortic dissection carries a high mortality rate and requires prompt diagnosis and intervention.^[1,6]

Global incidence is estimated at 3–5 cases/million annually,^[1] with a European autopsy-reported prevalence of 0.2–0.8%.^[6] In Nigeria, the true incidence remains unknown due to underreporting and diagnostic limitations.^[3,8] Ascending aortic dissections commonly occur in individuals aged 50–60, while descending aortic dissections peak between 60 and 70 years. Dissections in patients under 50 are unusual.^[5]

We report the case of a 47-year-old male who presented with severe epigastric pain and was initially managed as a case of peptic ulcer disease. Imaging subsequently revealed a DeBakey Type I (Stanford Type A) dissection. This case highlights the diagnostic challenge posed by atypical presentations and reinforces the importance of imaging in early detection and management.^[2,4,6]

CASE REPORT

A 47-year-old male civil servant, presented to the Accident and Emergency Department with an acute onset of severe epigastric pain, associated dizziness, and transient loss of consciousness. His symptoms began approximately 5 h before presentation and were characterized by episodic, sharp, non-radiating pain localized to the epigastric region, with intermittent radiation to the back and flanks. The episodes were recurrent over several months and had previously responded to analgesics. Notably, the patient experienced dizziness and multiple fainting spells during intense pain episodes.

He had a history of systemic hypertension but was non-compliant with antihypertensive medications. He was not diabetic and denied alcohol or tobacco use. There was a positive family history of hypertension and cerebrovascular disease. Prior episodes had been treated empirically as peptic ulcer disease in peripheral health facilities, using analgesics and anti-ulcer therapy. He was not on any regular medication and denied use of illicit substances. He was married in a monogamous relationship with five children.

On examination, he was in painful distress but alert and oriented. He was afebrile, anicteric, not cyanosed, not pale, and without peripheral edema. Vital signs revealed a pulse rate of 85 beats/min (regular and full volume), respiratory rate of 32 cycles/min, and blood pressure of 130/80 mmHg. The apex beat was located at the 6^th left intercostal space, lateral to the midclavicular line. Abdominal examination revealed fullness with tenderness localized to the epigastrium.

An initial clinical impression of acute exacerbation of peptic ulcer disease, with acute pancreatitis as a differential, was made [Figure 1]. However, investigations yielded further insights:

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Figure 1:

Diagnostic pathway for aortic dissection (index case). EF: Ejection fraction; CT: Computed tomography.

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• Urinalysis: pH 5.0 with positive urobilinogen

• ECG: Ventricular premature complexes, left atrial enlargement, and abnormal left axis deviation

• Renal function tests: Elevated urea (21.6 mmol/L; ref: 2.1–8.2), elevated creatinine (142 µmol/L; ref: 53–115)

• Lipid profile: Markedly reduced high-density lipoprotein-cholesterol (0.6 mmol/L; ref: >1.4)

• Serological tests: Non-reactive for human immunodeficiency virus, venereal disease research laboratory, and hepatitis B surface antigen.

Imaging findings

• Chest X-ray showed cardiomegaly with an unfolded aortic arch [Figure 2a]

• Abdominal ultrasonography demonstrated a distorted aortic wall with an intimal flap and false lumen [Figure 2b]

• Limited transthoracic echocardiography revealed an intimal flap in the ascending aorta [Figure 2c]

• A diagnosis of dissecting aortic aneurysm involving both ascending and descending aorta (Stanford Type A/ DeBakey Type I) was made

• Echocardiography further revealed significant aortic regurgitation and a reduced left ventricular ejection fraction of 31.3% [Figure 2d]

• CT angiography confirmed a double-lumen aorta with a hypodense intimal flap extending from the ascending aorta to the aortic bifurcation [Figure 2e and f].

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Figure 2:

Radiologic images of our study subject (a-f): (a) Plain chest radiograph showing comparatively larger cardiac shadow with cardiothoracic ratio of 63.7%, grossly unfolded aorta, upper lobe diversion, thickening of the right transverse fissure, and subtle lamella effusion noted on the right costophrenic angle. These findings were in keeping with congestive cardiac failure. (b) Gray-scale ultrasonography of the midabdomen, seen in longitudinal view, showing abdominal aorta with echogenic intimal flap (yellow arrow). (c) Parasternal long-axis view of echocardiography showing the left ventricle, left atrium, the ascending aorta, and the echogenic intimal flap in the ascending aorta (yellow arrow). (d) The parasternal long-axis view of M-mode echocardiography shows a low ejection fraction of 31.3%. (e) Axial section of computed tomography angiography of the chest at the level of the ascending aorta showing double-lumen signs at the ascending and descending aorta, in keeping with aortic dissection involving the ascending and descending aorta. (f) A sagittal reconstructed computed tomography angiogram shows a contrast-filled hyperdense cardiac chamber, ascending aorta, descending thoracic, and abdominal aorta. The demonstrated aorta has two lumens separated by a linear hypodense intimal flap (black arrow) that extends to the entire length of the aorta. This finding is consistent with DeBakey Type 1 or Stanford Type A aortic dissection. M: M-mode, a type of echocardiography display, LA: Left Atrium, LV: Left Ventricle, Ao: Aorta.

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DISCUSSION

Aortic dissection is the most catastrophic of all aortic pathologies, with a mortality rate as high as 33% within the first 24 h and up to 50% within 48 h if untreated.^[1] In the United States, the incidence is estimated at 2–3.5 cases/100,000 individuals annually.^[1] However, the true burden of disease in Nigeria remains unknown, due to diagnostic limitations, underreporting, and frequent post-mortem identification. Only a few cases have been documented in Nigerian literature. For instance, Kumtap et al. reported two conservatively managed cases in Jos involving males aged 41 and 45 years.^[2] Similarly, Ajani et al. described a fatal case of ruptured thoracic aortic aneurysm initially misdiagnosed as peptic ulcer disease.^[3] Coincidentally, our index case – a 47-year-old male – was also initially assessed as a case of peptic ulcer disease before imaging revealed a Stanford Type A (DeBakey Type I) dissection [Table 1].

A retrospective study at Lagos University Teaching Hospital by Ogunleye et al. revealed that aortic dissections predominantly affected patients aged ≥65 years with a male predominance.^[4] Although our patient was younger, his presentation aligns with the reported male predominance and clinical ambiguity. Similarly, Ibinaiye et al. described a DeBakey Type I dissection in a 68-year-old male initially misdiagnosed as myocardial infarction.^[5] Our patient presented with epigastric pain, further illustrating the diagnostic complexity of aortic dissections.

The clinical manifestations of aortic dissection are variable and often non-specific^.[1-10] Presentations may mimic myocardial infarction, pancreatitis, or peptic ulcer disease.^[1-10] In our case, epigastric pain and syncope delayed diagnosis. Ajani et al. also reported a diagnostic delay in a fatal case of thoracic aortic aneurysm rupture,^[3] highlighting the importance of clinical suspicion in low-resource settings.

Aortic dissection results from medial degeneration, intimal injury, and high intraluminal pressure. Hypertension is the most common risk factor, implicated in over 70% of cases.^[6] It promotes elastic tissue degeneration and cystic medial necrosis, particularly in the ascending aorta. Our patient had long-standing, poorly controlled hypertension and a positive family history of hypertensive complications. Although Marfan syndrome and other connective tissue disorders are recognized risk factors, there was no clinical suspicion of such disorders in our case.

The Stanford and DeBakey classifications are widely used for clinical and surgical guidance. Stanford Type A involves the ascending aorta, while Type B spares it. DeBakey Type I involves the ascending, arch, and descending aorta; Type II is limited to the ascending aorta, and Type III involves the descending aorta distal to the left subclavian artery.^[7] Our patient was classified as DeBakey Type I/Stanford Type A.

Imaging is central to diagnosis. Although chest radiographs are non-specific, they may show cardiomegaly, widened mediastinum, or an unfolded aorta. Transthoracic echocardiography (TTE) is a rapid and accessible tool to detect intimal flaps, aortic regurgitation, or left ventricular dysfunction. However, it may miss distal dissections. In this case, TTE revealed an intimal flap and reduced ejection fraction. Computed tomography angiography (CTA), with over 99% sensitivity and specificity, confirmed a double-lumen aorta.^[8] Magnetic resonance imaging angiography, while accurate, is less practical in emergencies due to longer acquisition time and motion artifacts.

Management strategies include medical therapy, endovascular repair, or open surgery.^[1-10] Stanford Type A dissections generally require emergency surgical intervention involving replacement of the dissected segment.^[1,5,8,9] Medical therapy is appropriate in uncomplicated Stanford Type B dissections or when surgery is contraindicated.^[1,5,8,9] In this case, although surgery was indicated, it was deferred due to financial constraints. The patient subsequently experienced cardiac arrest before intervention could be performed.

CONCLUSION

Aortic dissection remains a life-threatening condition with diverse and often misleading presentations. This case underscores the diagnostic challenge posed by atypical symptoms such as epigastric pain, especially in younger patients with uncontrolled hypertension. Early recognition, high clinical suspicion, and prompt imaging are critical to improving outcomes. In low-resource settings, delayed diagnosis and limited access to surgical care significantly contribute to mortality. This report highlights the need for enhanced clinician awareness, improved diagnostic infrastructure, and policies that ensure timely intervention. Greater vigilance can transform fatal misdiagnoses into life-saving opportunities for patients with acute aortic syndromes.