Aneurysm of the Aorta in Children*

Transcription

1 Aneurysm of the Aorta in Children* Frederick T. Fricker, M.D.; Sang C. Park, M.D.; William H. Neches, M.D.; 00 Robert A.!lfathews, M.D.; and David B. Lerlwrg, M.D., F.C.C.P. Seven children with aortic aneurysm ranging in age from 4 to 15 years were seen in a period of seven years. The diagnosis was confirmed by angiography and/ or surgery in each case. In three patients, the aortic aneurysm was associated with coarctation of the aorta. One had a saccular aneurysm proximal to the coarctation, and another developed a mycotic aneurysm distal to the coarctation in association with staphylococcal endarteritis. The third patient developed a diffuse aneurysm of the aortic arch 15 years following coarctectomy. The remaining four patients had aortic aneurysms of the ascending aorta. One had a mycotic aneurysm fol- lowing aortic valvulotomy, another had Madan's syndrome, a third had a sinus of Valsalva aneurysm, and the fourth a traumatic tear of the aorta with pseudoaneurysm formation. If an aortic aneurysm develops in association with aortic valvular disease or coarctation of the aorta, a precipitating factor such as systemic hypertension, trauma, or infection is usually identified. Long-term follow-up is urged in patients with coarctation of the aorta in view of the possibility that an aneurysm of the aorta may occur even after surgical resection of a coarctation. neurysm of the aorta in childhood is rare and has A been described in association with aortic valvular stenosis, 1 3 coarctation of the aorta, 4 abnormality of a sinus of Valsalva, or congenital connective tissue disorders. 5 7 Aneurysms caused by bacterial infection ( mycotic aneurysm ), granulomatous disease, or trauma are often associated with underlying congenital abnormalities of the aorta This report describes the clinical features, course, and management of seven children with aortic aneurysms who were seen at the Children's Hospital of Pittsburgh over a seven-year period (Table 1). Case 1 CASE REPORTS This five-year-old boy had been followed-up since two years of age with a diagnosis of aortic valvular stenosis. Cardiac catheterization at 4Jf years of age demonstrated severe valvular aortic stenosis with a peak systolic gradient across the aortic valve of 170 mm Hg. An aortic valvulotomy was subsequently performed with an uneventful postoperative course. Nine months following operation, the patient presented with anemia and a two-week history of intermittent fever. Chest roentgenogram demonstrated a large anterior mediastinal mass which was subsequently demonstrated by angiography to be a aneurysm of the ascending aorta (Fig 1 ). Blood cultures grew Herellea vaginicola. At operation, a large From the Division of Pediatric Cardiology of Children's Hospital of Pittsburgh, and the Department of Pediatrics and Surgery of the University of Pittsburgh School of Medicine. 0 0 Teaching scholar of the American Heart Association. Manuscript received February 16; revision accepted April 16. Reprint requests: Dr. Fricker, Cardiology Division, Children's Hospital of Pittsburgh, Pittsburgh CHEST, 76:3, SEPTEMBER, 1979 amount of necrotic material was seen floating free within the aneurysm, and an abscess was visualized in the wall of the aorta above the right coronary sinus. The ascending aorta was resected from just above the aortic valve to the innominate artery and replaced with a Dacron graft. There did not appear to be any involvement of the coronary ostia or the aortic valve. The postoperative course was complicated by disseminated intravascular coagulopathy, low output syndrome, and the patient subsequently died. Case 2 This boy was admitted at 12 years of age because of spiking fever and petechiae on the lower extremities. Femoral pulses were decreased. Blood pressure was 150/100 mm Hg in both arms and 80 mm Hg by palpation in the legs. The spleen was palpable 4 em below the left costal margin. Blood cultures were positive for Staphylococcus aureus (coagulase positive). After an adequate course of antibiotic therapy, he underwent cardiac catheterization, and angiography demonstrated a coarctation of the aorta in the usual position and a large saccular aneurysm in the anterior wall of the descending aorta just distal to the coarctation (Fig 2). Following surgical resection of the coarctation and aneurysm, systemic hypertension persisted. Repeat aortography and selective abdominal arteriography demonstrated adequate repair of the coarctation, an arterial-venous malformation in the spleen, and multiple saccular aneurysms of the hepatic and superior mesenteric arteries. The hepatic and mesenteric artery aneurysms were resected, and a splenectomy was performed. The postoperative course was further complicated by persistent systemic hypertension and subarachnoid hemorrhage which resulted in a left hemiparesis. In the four years since the operation, the patient has improved neurologically, and his hypertension is under control with antihypertensive medication. Case 3 This patient was followed-up from three years of age with ANEURYSM OF THE AORTA IN CHILDREN 305

2 Case No. Age Sex Associated Disease 4 M ASV 2 12 M COA,BAV 3 5 F COA,BAV Table 1-Aortic Aneury m in Childhood* Locations Ascending aorta Distal to COA Proximal to COA Mode of Presentation CHF-Sepsis bacterial endocarditis Acute bacterial endocarditis COA Surgery Replacement of ascending aorta with Dacron graft Resection Resection 4 15 F Marfan's syndrome Ascending aorta Resection valve Died,MR replacement 5 4 M None Ascending aorta Mediastinal mass Resection patch aorta 6 4 M 7 15 F COA, PDA Noncoronary cusp Ascending aortic arch to site of previous COA Repair of aortic valve Outcome Abbreviations used arc as follows:, aortic regurgitation; ASV, aortic valvular stenosis; BAY, bicuspid aortic valve; CHF, congestive heart failure; COA, coarctation of the aorta; CVA, cerebrovascular accident; MR, mitral regurgitation; and PDA, patent ductus arteriosus. the diagnosis of coarctation of the aorta and a bicuspid aortic valve. Mild systemic hypertension was noted with blood pressure of 130/85. Cardiac catheterization demonstrated a coarctation of the aorta in the usual location and 3 x 4 em saccular aneurysm in the descending aorta just proximal to the coarctation. At the age of four years, she had a coarctectomy and resection of the aneurysm. Repeat cardiac catheterization demonstrated no residual coarctation or aneurysm. Case 4 This patient with Marfan's syndrome presented at seven years of age with both aortic and mitral regurgitation. Severe None Died Alive CVA aortic regurgitation and progressive dilatation of the aortic root eventually led to operation. The ascending aorta was replaced with a Teflon graft from the aortic valve to the innominate artery. The high take off of the coronary arteries prevented any attempt at narrowing the aortic valve with a McGovern prosthetic valve. Eight days after operation she suddenly developed severe aortic regurgitation and died. Autopsy demonstrated detachment of the prosthetic valve from the aortic annulus. Case 5 This four-year-old boy had a febrile illness associated with FIGURE L Levophase of pulmonary arteriogram demonstrating filling of an aneurysm of ascending aorta (case 1). 306 FRICKER ET AL FIGURE 2. An aneurysm distal to site of coarctation is visualized from descending aorta ( case 2).

3 FIGURE 3. Progressi\"e enlargement of mediastinal mass that proved to be pseudoaneurysm of ascending aorta (case 5). abdominal pain six weeks prior to admission at which time chest roentgenogram was normal (Fig 3). Because of recurrent symptoms, a repeat chest roentgenogram was obtained six weeks later and demonstrated a right anterior mediastinal mass. Exploratory thoracotomy was perfom1ed because of the suspicion of a mediastinal tumor, and a large, nonpulsating mass was found over the ascending aorta. Its vascular nature was confirmed by needle aspiration. Multiple blood cultures were then done and were negative. Subsequent cardiac catheterization and angiography demonstrated a large saccular pseudoaneurysm that communicated with the ascending aorta ( Fig 4). At operation, the false aneurysm of the aneurysm of the ascending aorta was approximately twice the size of the heart. After cross clamping the ascending aorta, the aneurysm was entered and decompressed. A 9 x 17 mm defect was identified in the wall of the aorta just above the commissure between the noncoronary and right coronary leaflets of the aortic valve. The fibrous pseudoaneurysm was resected, and the aortic wall defect was repaired using a Dacron patch. Histologically, the wall of the aneurysm consisted of fibrous granulation tissue and fibrin clots. No elastic media could be demonstrated. Even in retrospect, no history of chest trauma or deceleration injury could be elicited. Case 6 This four-year-old child was referred for evaluation of a murmur. Examination demonstrated a moderate degree of aortic insufficiency evidenced by increased peripheral pulses and typical descrescendo diastolic murmur. Chest roentgenogram and ECG were normal. Cardiac catheterization and angiography demonstrated an aneurysmal dilatation of the noncoronary sinus that bulged into the left atrium (Fig 5). At operation, a biscuspid aortic valve was found. The noncoronary leaflet was detached from the aortic annulus and resulted in aortic regurgitation. An opening to the aneurysm was seen below the sinus ridge of the noncoronary leaflet. Repair was accomplished with Teflon buttress sutures that passed through the opening of the aneurysm to the unsuspended edge of the aortic leaflet. Postoperatively, the child has done well and has minimal residual aortic insufficiency. Case 7 sufficiency and massive dilatation of the ascending aorta and arch up to the area of the previous coarctectomy. This patient has remained asymptomatic, and surgery has not been performed. DISCUSSION Although aneurysms of the aorta are rare in childhood, this report has described seven patients with various types. Six were associated with congenital abnormalities of the aorta, and one case was presumed to be traumatic (case 5). The circumstances that brought these patients to our attention include a murmur of aortic regurgitation, mediastinal mass seen on chest x-ray film, and an acute febrile illness. This patient had a coarctectomy and ligation of a patent ductus arteriosus at two months of age. At age 14 years, during routine follow-up, a murmur of aortic insufficiency was noted, and markedly dilated aorta was noted on chest roentgenogram. Cardiac catheterization demonstrated mild aortic in- CHEST, 76: 3,SEPTEMBER, 1979 FIGURE 4. Levophase of a pulmonary arteriogram demonstrating contrast filling false aneurysm from a defect (arrow) in the ascending aorta ( case 5 ). ANEURYSM OF THE AORTA IN CHILDREN "307

4 FIGURE 5. Aneurysm of noncoronary sinus of Valsalva (case 6). In contrast to the common adult presentation, none of our patients developed dissection of the aorta. Atherosclerosis and trauma are the most common causes of aortic aneurysm in adults although the association with aortic valvular stenosis was first reported in adults by McKusick and Bahnson. 1 Coarctation of the aorta and aortic valvular stenosis have been reported to be the most common congenital cardiac abnormalities associated with aortic aneurysm and when both coexisted, aneurysms were more likely to develop. Hypertension, infection (mycotic aneurysm or aortitis), and underlying abnormalities of the aortic wall (cystic medionecrosis) share in the pathogenesis of aortic aneurysms in both pediatric and adult patients. 3 &- 9 The association with coarctation of the aorta and aortic valvular stenosis is found in the pediatric literature. 1 4 Bacterial endocarditis and endoaortitis presumably caused two aortic aneurysms seen in this report. The aortic intima is generally resistant to infection but damage to it as a result of trauma, cystic medionecrosis, or atherosclerosis may predispose to bacterial invasion. The resulting mycotic aneurysms are difficult to treat and invariably require surgical resection. Traumatic aortic aneurysms have been almost exclusively reported in adults and account for approximately 10 percent. However, there are no reports of traumatic aneurysm in the pediatric age group. Case 5 was found to have a pseudoaneurysm of the ascending aorta which was most likely due to trauma despite the absence of trauma historically. Aortic tears from blunt trauma most commonly occur in the descending aorta just distal to the left 308 FRICKER ET AL subclavian artery near the ligamentum arteriosum with the ascending aorta being the second most common point of traumatic rupture. 11 A small number of patients with aortic rupture develop chronic traumatic aneurysm. 12 If the patient survives for a few weeks, a fibrous-walled false aneurysm forms which may rupture and should be resected promptly.ll,t2 A number of cardiac abnormalities are now known to be associated with Marfan's syndrome, particularly some involving the aorta Dilatation or aneurysm of the ascending aorta with dissection or rupture, aortic insufficiency secondary to aortic or annular dilatation, and aneurysm of the sinus of Valsalva have all been reported. 6 Finally, the finding of an unruptured sinus of Valsalva aneurysm in one child in this series emphasized the need to evaluate aortic regurgitation at this age by cardiac catheterization and angiocardiography. We would also emphasize the necessity of longterm follow-up in patients with coarctation of the aorta in view of the possibility of aortic aneurysm formation even after surgical resection of the coarctation. In summary, aortic aneurysms in children have been usually associated with an underlying congenital abnormality, such as aortic valvular stenosis, coarctation of the aorta, and Marfan's syndrome. The coexistence of aortic valve stenosis and coarctation in the same patient may predispose him to an even greater risk of developing an aortic aneurysm. Associated factors such as hypertension, infection, trauma, and cystic medionecrosis share in the patho-

5 genesis of the aneurysm. A final point that deserves emphasis is that a mediastinal mass that is potentially vascular should have angiography prior to surgical exploration. REFERENCES 1 McKusick VA, Logue RB, Bahnson HT: Association of aortic valvular disease and cystic medial necrosis of the ascending aorta. Circulation 16: 188, Chen S-C, Barner HG, Fagan LF, et al: Aortic aneurysm in childhood: Report of six instances. J Pediatr 89:231, Becker RM, Poirier NL, Collins GF, et al: Cystic medial necrosis and dissecting aneurysm of the aorta in a child with congenital aortic stenosis. J Thorac Cardiovasc Surg 68:108, Edwards JE: Aneurysms of the thoracic aorta complicating coarctation. Circulation 58:195, Sakakibara S, Konna S: Congenital aneurysm of the sinus of Valsalva: Anatomy and classification. Am Heart J 63:405, McKusick VA : The Marfan syndrome. In: Heritable Disorders of Connective Tissue, 48th ed. St. Louis, The C V Mosby Co, Bahnson HT, Nelson : Cystic medial necrosis as a cause of localized aortic aneurysms amenable to surgical treatment. Ann Surg 144:519, Bennett DE, Cherry JK: Bacterial infection of aorta aneurysms. Am J Surg 113:321, Wilson AC, Simpson WL, Richardson JP, et al : Mycotic aneurysms of the aortic root. Aust NZJ Surg 42:113, Hegerer G : Ruptures and aneurysms of the thoracic aorta after blunt chest trauma. J Cardiovasc Surg 12:115, Bennett DE, Cherry JK : The natural history of traumatic aneurysms of the aorta. Surgery 61 :516, Fleming AW, Green DC: Traumatic aneurysms of the thoracic aorta. Ann Thorac Surg 18:91, Bowers D: Primary abnormalities of the mitral valve in Marfan's syndrome. Br Heart J 31:676, Crosby IK, Ashcraft WC, Reed WA: Surgery of proximal aorta in Marfan's syndrome. J Thorac Cardiovasc Surg 66:75, 1973 ANEURYSM OF THE AORTA IN CHILDREN 309