Intravenous Digital Subtraction Aortography in the Preoperative and Postoperative Evaluation of Marfan's Aortic Disease*

Transcription

1 Intravenous Digital Subtraction Aortography in the Preoperative and Postoperative Evaluation of Marfan's Aortic Disease* Robert Detrano, M.D., Ph.D.; DouglasS. Moodie, M.D., F.C.C.P.; Carl C. Gill, M.D., F.C.C.P.; Darlene Markovich, R.N.; and Conrad Simpfendorfer, M.D. Seventeen patients with the skeletal stigmata of Marfan's syndrome or the physical and radiographic &ndings of Marfan's aortic disease (or both) were studied using intravenous digital subtraction aortography. Digital subtraction aortography revealed a dilated aortic root in 16 of the 17 patients. One of the 17 had mildly dilated aortic sinuses with a normal ascending aorta. All of the four children ( S16 years of age) had aortic diameters which were greater than the 95th percentile for their body surface area. The average aortic root diameter in the adult patients was 6.8 em (range, 4-11 e m Three ~ of the 17 had aortic dissection, two of which were detected by digital subtraction aortography and two by aortic root injection. Four of 13 adults and three children had pulmonary arterial diameters which exceeded 4 em. Six patients underwent ascending aorta and valvular replacement and were studied with digital subtraction aortography after surgery. All preoperative and postoperative studies were of adequate diagnostic quality. Aortic root diameters calculated from intravenous digital subtraction angiograms correlated wed with those derived from echocardiography (...- 9= 2 We 0~ conclude that intravenous digital subtraction aortography is effective in diagnosing and following M ~ fan's aortic disease both before and after surgery. M arfan's syndrome, an autosomal dominant inherited condition characterized by arachnodactyly, hyperextensible joints, subluxated lenses, prolapsing mitral valve, and aortic root dilatation, has a natural history with a fatal outcome usually due to aortic dissection, aortic rupture, or congestive heart failure secondary to regurgitant left-sided valves. u Accurate noninvasive evaluation ofmarfan's aortic disease therefore is of some importance. Echocardiography:l- 4 has been used successfully in the evaluation of this disorder. Intravenous digital subtraction aortography, a relatively new angiographic technique, has been applied successfully5 6 to the assessment of various diseases of the aorta. We describe herein our experience with the use of intravenous digital subtraction angiography in the preoperative and postoperative evaluation of Marfans aortic disease. Study Group MATERIALS AND METHODS There were 17 patients referred for digital subtraction aortography between May 1981 and June In all patients, Marfans aortic disease was suspected based on the findings of a typical body habitus, an early diastolic murmur, or family history. There were 13 male and four female patients. The mean age was 27 years (range, 7 to 69 years). *From the Department of Cardiology, Cleveland Clinic Foundation, Cleveland. Manuscript received October 26; revision accepted January 25. Reprint requests: Dr: Moodie, 9500 Euclid Avenue, Cleveland44106 Imaging Equipment and Technique Commercially available angiographic and digital subtraction aortographic units were used with previously described techniques. 5 In ten of the patients, the contrast medium was injected into a peripheral arm vein. In the remaining seven, the superior vena cava was used. The usual bolus of contrast material (Renografin-76) was 0.5 ml/kg of body weight per injection at a How rate of 15 mvsec. Images were made in the radiographic single-mask mode. Angiographic M668Uremenu A 1-cm grid was radiographically imaged at various distances of between 7.0 to 20 em from the image intensifier. For each study in each projection, the distance from the ascending aorta to the image intensifier was estimated. Since the distance between the x-ray tube and the image intensifier was kept constant, the proper grid could be matched to each study, and the angiographic aortic and pulmonary root diameters were thereby measured. The projection yielding the maximum diameter was used for the measurements reported in 'Illble 1. The upper limits of normal aortic and pulmonic root diameters derived by Snider et al 7 were used, rather than the anglographic normals, which are generaljy lower. 1 The fonner were chosen because the aortic and pulmonary roots from the digital studies were measured just above the valves at a level comparable to the parasternal short-axis plane from the echocardiogram. Clinical Information RESUI.:I'S Thble 1 indicates the presenting symptoms and important physical findings in our patients. Noteworthy is the fact that most of the patients did not have physical signs of aortic insufficiency, and six of the patients (35 percent) were asymptomatic. Six of the patients underwent surgical correction of their ascending aortas and aortic valves. All six received composite CHEST I 88 I 2 I AUGUST,

2 Clinical, Echocardiographic, Digital Subtraction Aortographic, and Cineangiographic DGttJ Table 1-Marfrani Aorlic ~ : Cardiac Examination Patient Aortic Sex, Mitral Regurgi- Conventional Age (yr) Symptoms Prolapse tation Aortography 1, F, 7 None Yes No Not done 2, F, 10 Dyspnea Yes No Not done 3, M, 13 None Yes No Not done 4, M, 15 None Yes No Not done 5, M,17 Fatigue Yes No Not done 6, M,18 Chest pain Yes Yes Done 7, M, 23 Dyspnea No Yes Done 8, M, 23 None Yes Yes Not done 9, M, 26 Dyspnea Yes No Not done 10, M, 32 Chest pain Yes Yes Done 11, F, 33 Palpitations Yes Yes Not done 12, M, 33 None Yes No Not done 13, M, 37 None No No Not done 14, M, 38 Chest pain; No Yes Done dyspnea 15, F, 40 Chest pain No No Done 16, M, 60 Chest pain No No Done 17, M, 69 Dyspnea; No Yes Done &tigue Pulmonary Aortic Surgery Aortic Root Diameter Arterial and Post- Dis sec- Diameter operative DSA tion Echocardiography DSA bydsa, em Not done No Not done No Not done No Not done No Not done No Done No t 7.1 Done No Inadequate Done No Inadequate Not done No Done No Not done No Not done No Not done No Inadequate Done Yest Not done Yes Not done Yes Done No Inadequate DSA, Digital subtraction angiography. t1wo preoperative DSA studies (second value used in averaging). *Dissection missed by DSA. Dissection missed by aortography. valve conduit grafts. Five of the six prosthetic valves were Bjork-Shiley disk valves. The remaining prosthetic valve was a Carpentier-Edwards bioprosthesis. Digital Subtraction Aortographic Results All 18 preoperative and all six postoperative digital subtraction aortographic studies done on the 17 patients were of a quality adequate to diagnose aortic root dilatation and to measure the aortic root diameters. The average aortic diameter fur the adult subjects (> 16 years), which was measured approximately 2 em distal to the aortic valve, was 6.8 em (range, 4.0 to 11.0 em). All fuur children had aortic root diameters exceeding the 95th percentile fur body sur&ce area. The correlation between echocardiographic aortic root diameter and that derived from the digital aortograms was adequate (r = 0. 92) (Fig 1). Interestingly, fuur adults and three children had pulmonary arterial diameters exceeding 4 em in at least one projection. One 26-year-old man had an aortic diameter which was at the upper limits of normal (4 em), but his aortic sinuses were mildly dilated (Fig 2). An 18-year-old man's initial study done in November 1981 (Fig 3A) revealed an aortic root diameter of 6 em. In April 1982, this young man returned fur a repeat study bcause of increasing dyspnea. The repeat study (Fig 3B) in a slightly different projection clearly shows an increase in the aortic diameter. This patient's ascending aorta and valve were replaced with a com- posite valve conduit, and his postoperative study is shown in Figure 3C. Four aortic dissections were angiographically detected in three of our patients. In one patient, dissection was detected by both intravenous digital subtraction aortography and conventional aortic root cineangiography. In a second patient, dissection was detected by digital subtraction aortography alone. Despite the fact that this patient had two obvious dissections (Fig 4), a direct aortic root injection with inadequate contrast failed to reveal either dissection. E a: w 1- w :e :$ c c 2: ~ ~ 12 II n = 13 r2 = 0.92 y = X 3'--..._.... ~ a L-L J II 12 ECHO DIAMETER (em) FIGURE 1. Scatter diagram and regression line for aortic root diameters by echocardiography (ECHO) and intravenous digital subtraction aortography (IVDSA).

3 FIGURE 2. Aortogram of 26-year-old man with mild dilatation of sinuses of Valsalva. In a third patient with ascending aortic dissection diagnosed by aortic root injection, digital subtraction aortography (Fig 5) failed to reveal this lesion. DISCUSSION Intravenous aortography was first described by Robb and Steinberg'~ in It was largely abandoned in the 1960s with the advent of ultrasonography and direct intra-arterial contrast angiography. Venous aortography was reintroduced when it was discovered that subtraction prints made by subtracting a mask image before the arrival of contrast in the aorta from the contrast images greatly enhanced the visualization of aortic pathologic findings. With the advent of digital cardiovascular imaging in the late 1970s, greatly improved mask-subtracted images became possible 10 Moodie et al5 applied the technique of digital subtraction aortography to the study of congenital abnormalities of the aorta. In the present investigation, digital subtraction aortography was successfully applied to 17 patients with Marfan's aortic disease. Echocardiography, although less invasive, was not sufficiently accurate in many of the patients in this investigation to fully define their aortic pathologic abnormalities. Although aortic root dilatation is usually successfully diagnosed with echocardiography, marfanoid aneurysm of the distal arch, which was present in one of our patients, and aortic dissections, which were present in three of our patients, are often missed and indeed were missed by echocardiography in the subjects of this investigation. Furthermore, Marfan's disease generally involves the FIGURE 3. A (top), Aortic root of 18-year-old man with mild symptoms. Arrows indicate dilated aortic root. B (center), Same patient, six months later with increased symptoms. C (bottom), Same patient, postoperative study. CHEST I 88 I 2 I AUGUST,

4 FIGURE 5. Digital subtraction aortogram in patient who had aortic dissection diagnosed by direct aortography. Poor contrast effect from peripheral injection does not reveal dissection in this study. pulmonary artery, 11 as well as the aorta. Echocardiography is often unsuccessful in a full assessment of the pulmonary artery. The finding of pulmonary arterial dilatation (Fig 6) ofbetween 4 and 7.1 em in seven of our patients suggests a rather high incidence of significant involvement of this vessel. Digital subtraction angiography can be used to follow the progression of this pulmonary arterial disease after aortic surgery has decreased the probability of a cardiac catastrophe from aortic rupture, dissection, or insufficiency. Three patients in this investigation had magnetic resonance imaging of the aorta. One of these whose digital subtraction aortogram is illustrated in Figure 4 also had computerized tomographic imaging of the aorta with contrast enhancement. Neither magnetic resonance imaging nor computerized tomography added additional useful information not obtained from the intravenous aortogram. In fact, magnetic resonance imaging did not correctly identify either aortic dissection in the subject whose digital aortogram is illustrated in Figure 4. Direct contrast aortography is, of course, the gold standard against which all noninvasive techniques must be judged. In this investigation, there was one aortic dissection which was incorrectly overlooked by digital subtraction angiographic study and correctly diagnosed by direct aortography. The failure of intravenous digital subtraction aortography to demonstrate this dissection was partially due to inadequate contrast from a peripheral intravenous injection. 12 We therefore recommend that right atrial or vena caval injections be used in these patients. There was also another dissection missed by aortography because of FIGURE 4. A(top), Ascending aorta dissection in 40-year-old woman with Marfans disease. B (center), Abdominal aortic dissection in same patient. 'Ihle aortic lumen is seen to be filling left renal and mesenteric arteries. C(bottom), Seconds later, false lumen fills iliac arteries. 252 Intravenous Digital Subtraction Aortography In Malfan's (Detr8no et ej)

5 intravenous digital subtraction aortogram is sufficient to obviate the need for cardiac catheterization. ACKNOWLEDGMENT: We thank Ms. Paula LaManna fur her secretarial assistance. FIGURE 6. Pulmonary arterial dilatation in seven-yearo{)ld girl with Marfans disease. inadequate contrast and correctly detected by digital subtraction angiography. Both dissections were confinned in the operating room. Since the completion of this investigation, we have successfully perfunned ascending aorta and valvular replacement on two of our patients with Marfans aortic disease who underwent an intravenous digital subtraction angiographic study of the aorta before surgery, but not direct contrast aortography. Although contrast aortography may at times be necessary to assess the severity of aortic insufficiency, such assessment sometimes is unnecessary and can usually be done with Doppler echocardiography. 13 We are confident that in selected cases, when strong suspicion of other cardiac pathologic abnormalities does not exist and when the severity of aortic insufficiency is not an important question, an adequate REFERENCES 1 Roberts WC, Honig HS. The spectrum of cardiovascular disease in the Marfan syndrome: a clinico-rnorphologic study of 18 necropsy patients and comparison to 151 previously reported necropsy patients. Am Heart J 1982; 104: Murdoch JL, Walker BA, Halpern BL, Kuzma JW, McKusick VA. Life expectancy and causes of death in the Marfan Syndrome. N Engl J Med 1972; 286: Corne PC, Fortuin NJ, White RI Jr, McKusick VA. Echocardiographic assessment of cardiovascular abnormalities in the Marfan syndrome. Am J Med 1983; 74: Spangler RD, Nora JJ, Lortscher RH, Wolfe RR, Okin JT. Echocardiography in Marfan's syndrome. Chest 1976; 69: Moodie DS, Yiannikas J, Gill CC, Buonocore E, Pavlicek W. Intravenous digital subtraction angiography in the evaluation of congenital abnormalities of the aorta and aortic arch. Am Heart J 1982; 104: Bismuth V, Lacombe P, Hermant C, Schournan E, Frija G. Angiographie nurnerique. Presse Med 1984; 13: Snider AR, Enderlein MA, Teitel DF, Juster RP. TwtHiirnensional echocardiographic determination of aortic and pulmonary artery sizes from infancy to adulthood in normal subjects. Am J Cardiol 1984; 53: Sievers HH, Onnasch DGW, Lange PE, Bernhard A, Heintzen PH. Dimensions of the great arteries, semilunar valve roots, and right ventricular outhow tract during growth: normative angiocardiographic data. Pediatr Cardiol1983; 4: Robb GP, Steinberg I. A practical method of visualization of the chambers of the heart, the pulmonary circulation, and great vessels in men. J Clin Invest 1938; 17: Morris ADP, Evans AF, Carty AT. Venous aortography: a forgotten technique. Clin Radio! 1979; 30: Bowden DH, Favara BE, Donahoe JL. Marfans syndrome. Am Heart J 1965; 69: Saddekni S, So TA, Sniderman KW, Srur M, Bodner LS, Kneeland JB, et al. Optimal injection technique fur intravenous digital subtraction angiography. Radiology 1984; 150: Venot C, Arneur A, Gourtchiglovian C, Lessano A, Abithol G, Kalrnanson D. Calculation of pulsed Doppler left ventricular outhow tract regurgitant index fur grading the severity of aortic regurgitation. Am Heart J 1984; 108: CHEST I 88 I 2 I AUGUST,