Introduction

Aortic aneurysm (AA) is a condition where the diameter of aorta is dilated for more than 50% of the normal size. In AA, dilation will happen gradually, and it is usually associated with increasing age and atherosclerosis. 2, 1 AA is divided based on the position of the aneurysm. Aneurysm which is located in thoracic aorta is called as thoracic aortic aneurysm (TAA). Aneurysm which is located in the abdominal aorta is called abdominal aortic aneurysm (AAA) and TAA that expands to the abdominal aorta is called thoracoabdominal aortic aneurysm (TAAA) or extensive AA. 3, 2 TAAA is a complex aortic disease. The incidence of TAAA is low, with only 10 new cases diagnosed per 100,000 population-year. 3

Women are diagnosed with TAAA in older age compared to men and have a higher risk of rupture. 4 AA is often asymptomatic and detected accidentally when doing imaging examinations, such as ultrasound and CT scan, therefore the mortality rate of AA is high.5 Patient with chronic obstructive pulmonary disease (COPD) in the age above 75 years has a high prevalence of AA, severe lung destruction, and calcification of aortic wall which are risk factors for AA and also has 3.6 times greater risk for rupture. 8, 7, 6 We reported a rare case of TAAA and explained about COPD as a risk factor of AA development.

CASE REPORT

An 83-year-old woman came to the emergency room with complaint of weakness. She had no appetite for 2 weeks before being admitted to the hospital. The patient complained of shortness of breath and coughing since 3 days before the admission. The patient also complained of intermittent epigastric pain, nausea and vomiting since the last 1 month. Complaints of slashing pain in the chest and abdomen area were denied. The patient was known to have a history of COPD for the past 1 year, but complaints of shortness of breath often appear in the past month. History of hypertension, diabetes mellitus, and kidney disease were denied.

Vital signs showed blood pressure 100/70 mmHg, tachycardia (90 bpm), tachypnea (26 times/minute), afebrile, oxygen saturation 96% with 4 lpm O2 via nasal canula. Physical examination showed barrel chest shape with widening of the rib cage. There was an prolonged expiration accompanied by rales and wheezing throughout the chest. There was an abdominal distension and tenderness throughout the abdomen, especially in the epigastric region, normal bowel sound, and no bruit sound. Complete blood count, blood sugar, lipid profile, and kidney function test results were within the normal limit. Electrocardiography (ECG) result supported COPD Figure 1 .

Figure 1

Chest radiograph showed COPD accompanied by prominent aortic notch and widening of the left posterior mediastinum suspicious of AA with differential diagnosis of mediastinal mass Figure 2 . Echocardiography showed aortic regurgitation, mitral regurgitation, dilation of sinus of Valsalva, ST junction and proximal ascending aorta Figure 3 .

Figure 2

Figure 3

Thoracic CT scan showed AA of ascending aorta (4.4 cm in diameter), aortic arch, descending aorta (4.5 cm in diameter) to the thoracoabdominal aorta. Intraluminal thrombus appeared along the TAA. Aortic atherosclerosis appeared with descending aortic wall irregularities as high as the ninth thoracic vertebraeFigure 4 .

Figure 4

The patient received symptomatic therapy such as nebulization with salbutamol every 8 hours, intravenous methylprednisolone 62.5 mg twice daily, intravenous pantoprazole 40 mg once daily, and oral paracetamol 1,000 mg thrice daily. Patient was advised to be referred for surgical intervention to treat the TAA, however the family refused due to the potential risks during and after surgery. Therefore, the patient was given medical therapy for AA, such as oral bisoprolol 2.5 mg once daily and oral simvastatin 20 mg once daily.

DISCUSSION

In this case, an 83-year-old female patient had a history of COPD. The complaint of shortness of breath was felt more often in the last one month and accompanied by intermittent epigastric pain. The vital signs were within the normal limit. Physical examination of the chest showed a barrel chest shape, elongated expiration accompanied by rales and wheezing throughout the lung. Physical examination of the abdomen showed tenderness throughout the abdomen, especially in the epigastric region and no bruit was obtained. ECG examination is consistent with COPD. We gave initial therapy for COPD and dyspepsia to the patient However, the chest radiograph examination revealed an image of COPD accompanied by prominent aortic notch and widening of the left posterior mediastinum. Therefore, this patient was suspected of having AA with a differential diagnosis of the left posterior mediastinal mass. We further evaluated the CT scan of the chest and found ascending AA (4.4 cm in diameter), aortic arch, and descending aorta (4.5 cm in diameter) to the thoracoabdominal aorta accompanied by an intraluminal thrombus along the TAAA. Aortic atherosclerosis appeared with descending aortic wall irregularities as high as the ninth thoracic vertebrae. The echocardiography result also showed aortic dilation. AA is often diagnosed accidentally when performing imaging tests, such as ultrasound and CT scan, because it is often asymptomatic. Screening studies performed in the general population showed that the prevalence of AAA is 4.9%. 9

Aneurysm is a deterioration and damage of the aortic wall architecture due to gradual loss of elastin and smooth muscle cells as results of aging process and their progression is influenced by several risk factors. 10, 2, 1 Compared to men, TAA is more commonly diagnosed at older age in women, with mean of age is 75.9 in women vs 62.8 years in men (p=0.01). 3 Meanwhile, AAA is more common in men. In this case, we found a female patient at the age of 83 years old when TAAA was first identified, and the TAAA was originated from TAA which extended to the abdominal aorta.

In this case, the patient complained of shortness of breath which occurred more often in the last one month. It was because patient who has aneurysm tends to experience more airway obstruction. 8 Complaints of intermittent epigastric pain accompanied by nausea and vomiting with stable vital signs can be caused by compression in the area due to an enlarged aorta. However, attention must be given to the abdominal pain because it might be a sign of aortic dissection, especially if abdominal pain is accompanied by other supporting signs, such as decreased consciousness and hypotension. Although complaints of slashing pain in the chest and abdomen were denied, the presence of aortic dissection in this case can not be ruled out. Based on the result of thoracic CT scan, there was ascending aortic wall irregularities as high as ninth thoracic vertebrae, so that the presence of aortic dissection can not be removed due to the limitation of CT scan facilities where we only have CT scan with a single slice of 10 mm thick slices and can not be reformed into coronal and sagital planes.

The mechanism of AA is generally explained as follows. The medial layer of the aortic wall consists mainly of structural proteins, such as collagen and elastin, which contribute to the aortic capacitance and elasticity. Degradation of structural proteins or the presence of defect in their composition causes degeneration of the medial layer and weakening of the aortic wall. Subsequent dilation occurs due to hemodynamic force in the arterial wall and intrinsic change in the composition of the artery wall itself. According to Laplace’s law, the wall tension is proportional to the pressure multiplied by the radius of the arterial canal. Then, when the diameter of the aorta increases, the aortic wall tension will also increase, so that it becomes a vicious circle. The condition will be worsen by hypertension. 4 The patient discussed in this case had no history of hypertension.

Risk factors for AA include old age, smoking, hypertension, aortic calcification, hyperlipidemia, and COPD. In this case, the risk factors which were considered to cause AA were old age and COPD. Old age is a risk factor because individuals over 60 years old will progressively deposit calcium in the major arteries which will cause vascular calcification that led to the decreased elasticity of the aorta and other arteries, which can interfere with cardiovascular hemodynamics. 11, 7 Smoking and COPD are closely related, but both are also factors that influence the formation of atherosclerosis which will later be associated with the occurrence of aneurysm. 10 In COPD, there is an inflammation condition that causes an increase of C-reactive protein (CRP) and other systemic inflammatory markers. Increased production of proinflammatory cytokines and tissue factors by monocytes causes increased absorption of LDL by the macrophages, and directly induces expression of adhesion molecules in human endothelial cells. In addition, CRP can be directly deposited to artery walls during atherogenesis which then interacts with other inflammatory mediators to form foam cells, which are the basis of atherosclerosis plaque formation. 14, 13, 12 Atherosclerosis predilection is more common in the abdominal aorta than in the thoracic aorta. This is because the smooth muscle cells in the thoracic aorta have a better replicative ability to repair themselves at injury compared to those in the abdominal aorta. 10

Patients with COPD have a 2.6 times higher risk of AAA. 16, 15 The prevalence of AAA with aortic diameter ≥30 cm in COPD patients is 3.7%. COPD patients over 75 years old have a high prevalence of AA, severe lung damage, and calcification of the aortic wall which are the risk factors for AA. 8 Systemic inflammation and hypoxic conditions in COPD contribute to abnormal vasa vasorum circulation to AA. 7 The underlying pathophysiology of AAA and COPD is a persistent pro-inflamatory response associated with proteolysis which causes damage to the arterial wall structure19, 18, 17

The diagnosis of AA can be made based on imaging test, such as ultrasound, CT scan, or MRI. The most commonly used TAA classification is the Crawford Classification, which has been modified by Safi et al. In this classification, TAA is divided into 5 categories based on the location of AA. 20 In this case, the patient cannot be classified by using this classification due to the limitation of our facilities. We do not have a CT scan result of the sagittal and coronal plane of the thorax

When aneurysms are formed, they are naturally gradually increased in size and rupture risk. The most serious complication of AA is rupture. AAA with a diameter of 4.0-5.5 cm has an estimated 1-year rupture rate of 1%.21 When the TAAA diameter exceeds 6 cm, the rupture rate is 14% per year.22 If TAAA is left untreated, 80% will experience a rupture. 4 TAAA and TAA patients who are non-dissecting with a history of COPD had a rupture risk that increased up to 3.6-folds (p=0.04).6 Factors that were independently and significantly associated with rupture risk are female gender (OR 4.5, 95% CI 1.98-1.02), smoking (OR 2.1, 95% CI 0.95-4.67), and hypertension (OR 1.04, 95% CI 1.02-1.07).23 On CT scan of the chest, the patient had an intraluminal thrombus along the thoracoabdominal aorta. This also aggravates the condition of AA. Study investigating the effects of intraluminal thrombus with AA progression showed that increased intraluminal thrombus porosity can cause plasma proteins to diffuse into the aortic wall, thereby affecting AA growth.24 Because the patient discussed in this case is a woman with COPD history and a large diameter of AA accompanied by extensive intraluminal thrombus, she has high risk of progression and rupture. Therefore, further evaluation and appropriate treatment are required.

The definitive therapy in the management of TAAA is open surgery. In this case, the patient had an aneurysm with diameter around 4.4-4.5 cm. Patients who suffer from TAA with diameter of 4.0-4.9 cm, have an annual risk of rupture of 2%. 25 Although surgical intervention is the only treatment strategy that has been proven to reduce the rupture risk, which is effective and long lasting, this procedure has a mortality rate of >5% in several countries.26 Reported result of 30-day survival of TAAA intervention is 92-95%. Reported acute kidney failure was 2.1-9.0% and spinal cord ischemia was 1.3-15.5%. 29, 28, 27 The mortality rate from descending aortic and TAAA surgery is 8%.22 The family of the patient refused to be referred for surgery intervention due to the potential risk of complication which might occur during and after surgery.

The main goal of medical therapy in AA is to reduce shear stress by lowering blood pressure and heart contractility. In patients who are not candidates for surgery or refuse surgical treatment, blood pressure control, smoking cessation, and lipid profile optimization should be carried out. Systolic blood pressure (SBP) target is between 100 and 120 mmHg.30 Prophylaxis using beta blockers may be useful in reducing the level of aortic dilation by reducing the strength of myocardial contractions. Patients who received an ACE inhibitor were less likely to have a rupture of aneurysm compared to those who did not get an ACE inhibitor. 24 We did not give ACE inhibitor for this patient due to her blood pressure always in the lowest threshold of SBP target. The meta-analysis showed a significant decrease in AAA growth in patients who received statin therapy compared to those who did not.31 Statin can also prevent the expansion of aneurysm. 33, 32 This patient received symptomatic therapy, including intravenous methylprednisolone 62.5 mg twice daily, salbutamol inhalation every 8 hours, oral paracetamol 1 gram thrice daily. Because the patient refused to be referred for surgical intervention, we gave medical therapy for AA including bisoprolol 2.5 mg once daily, simvastatin 40 mg once daily, pantoprazole 40 mg once daily. Our goal was to reduce shear stress and rupture risk. After the main complaint of this patient was improved, the patient was discharged and provided with symptomatic drugs, AA medical therapy, and was advised for routine check.

CONCLUSION

Patient with COPD can potentially have various comorbidities, one of which is AA. AA is a very dangerous disease, because it is often asymptomatic. Whereas, based on its natural course, AA will continue to dilate so the risk of rupture increases. Today, the most effective treatment for AA is surgical intervention. However, there are several medical therapies that provide benefits, especially in preventing the occurrence of AA rupture. If the patient is known to have AA, it is recommended to conduct routine surveillance checks to see the development and rupture of AA. Hence, the appropriate interventions can be carried out and can improve the prognosis.

ACKNOWLEDGEMENT

We would like to express our special thanks of gratitude to our teacher and also the head of Internal Medicine Department at Wangaya Regional Public Hospital, DR. dr. Ketut Suryana, SpPD-KAI, FINASIM, who gave us the opportunity and support in doing a lot of research or other scientific things.

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