with Fatal Acute Aortic Regurgitation Due to Aortic Dilatation and Aortic Valve Perforation — A Case Report
Aortitis
Syndrome
Toru Satoh, M.D. Masao Chino, M.D. Masaki Takahashi, M.D. and Keiji Suzuki, M.D.*
ASHIKAGA and MAEBASHI, JAPAN
Abstract This is a case report of a fifteen-year-old female with aortitis syndrome complicated by acute fatal aortic regurgitation due to the aortic valve perforation, as well as to aortic dilatation, which was confirmed by the autopsy. Aortic valve perforation was first recognized as the important cause of acute aortic regurgitation in the aortitis syndrome in this report. Introduction The aortitis syndrome is responsible for many disorders resulting from nonspecific vasculitis of the aorta and its large branching arteries. Aortic regurgitation, which develops in 10% to 50 % of the cases, is one of the most important causes of death. Aortic regurgitation usually progresses chronically as a result of dilatation of the aorta and the aortic valvular ring. Aortic valvular changes have been considered as contributing to aortic regurgitation but seldom have been regarded as an important cause of it. We encountered a case of fatal acute aortic regurgitation caused by aortic valve perforation, as well as by aortic dilatation.
Case
Report This fifteen-year-old female patient was in good health except for a predisposition to fever and the absence of menarche. Five days before admission she consulted her family physician for fever and productive cough. She was admitted to our hospital because of her increasing dyspnea and debility. From the Department of Cardiology, Ashikaga Red Cross Hospital of Medical Technology, Gunma University, Maebashi, Japan
Ashikaga;
and the
*Department of Pathology, College
869
Downloaded from ang.sagepub.com at Bobst Library, New York University on May 22, 2015
870 On examination she was found to be severely ill, a little drowsy, cyanotic, and orthopneic. Her heart rate was 134 per minute and regular, blood pressure 160/80 mm Hg, and body temperature 39.6°C. The patient’s jugular veins were dilated. There was a gallop heart rhythm without murmurs. There were moist rales over both her lung fields. Peripheral arterial pulsations were all equally felt. Laboratory data revealed anemia (hemoglobin 9.9 g/dL), severe thrombocytopenia (platelet count 440,000), enzyme elevation (glutamic ovaloacetic transaminase 342U/L, glutamic pyruvic transaminase 228U/L, lactic dehydrogenase 1011U/L), and marked hypoglycemia (glucose 7 mg/dL). Blood gas analysis showed metabolic acidosis (pH 7.27, Pa02 91 Torr, PaC02 13 Torr, HC03 5.7 mEq/L). Chest x-ray revealed marked cardiac enlargement, pulmonary congestion, and a dilated ascending aorta. There was left axis deviation but no sign of left ventricular hypertrophy on the electrocardiogram. A two-dimensional echocardiogram showed dilatation of the ascending aorta (60 mm in width), flailed aortic valves, and diffuse left ventricular hypokinesis without marked chamber dilatation (end-diastolic dimension 48 mm). There were massive aortic regurgitation, on the basis of a color Doppler echocardiogram, and early mitral valve closure, on the basis of an Mmode echocardiogram (Fig. 1, arrow). These findings suggest acute aortic regurgitation caused by aortic dilatation and derangement of the valve or valve-supporting structures.
FIG. 1. an
arrow)
closure of the mitral valve (indicated the M-mode echocardiogram.
Early on
by
FIG. 2.
Autopsy findings in the aortic valve: was perforated (probe inserted).
coronary cusp
Downloaded from ang.sagepub.com at Bobst Library, New York University on May 22, 2015
The left
871
The patient died unexpectedly several hours after admission, before the emergency aortic valve replacement could be performed. At necropsy the ascending aorta and the aortic valve ring were dilated. There was calcification of the basal portion of the aorta. The aortic valves were slightly thickened. The left coronary cusp was perforated (Fig. 2, arrow). The heart was dilated (340 g) and the left ventricular wall was mildly thickened. Histologically, the aortic media revealed diffuse necrosis, granuloma consisting of mononuclear cells with multinucleated giant cells, and marked fragmentation of elastic fibers. There were lymphocytic inflammatory cells with giant cells in the thickened intima and adventitia (Fig. 3A). These changes were observed segmentally throughout the aorta down to the bifurcation of the abdominal aorta. These findings led us to diagnose the patient as having had aortitis syndrome. Microscopic examination of the aortic valves revealed fragmentation and degeneration of collagen fibers, mononuclear cell inflammation, calcific deposits, necrosis, and granuloma formation in response to the necrotic tissue (Fig. 3B). There were no histologic findings corresponding to endocarditis.
Fic. 3. A. Wall of ascending aorta: marked fragmentation of elastic fibers, diffuse necrosis, and granuloma formation in the media. There are lymphocytic inflammatory cells with giant cells throughout the wall layers. Hematoxylin and eosin stains x 40.
FIG. 3. B. Aortic valve: fragmentation and degeneration of collagen fibers, profuse mononuclear cell infiltration. Hematoxylin and eosin stains x 40.
Downloaded from ang.sagepub.com at Bobst Library, New York University on May 22, 2015
872 Discussion The clinical picture of the aortitis syndrome has become clear, beginning with the first report by the Japanese ophthalmologist Takayasu in 1908. Aortic regurgitation is reported to have been observed in 20 % to 25 % of patients with this syndrome and to be the major abnorI mality leading to heart failure, the most frequent cause of death.’ Aortic regurgitation occurs mostly as a result of chronic aortic dilatation of more than five years’ duration. This patient, however, died of acute aortic regurgitation after a short and unusual course. The lack of evidence of left ventricular hypertrophy on the electrocardiogram, the finding of early mitral valve closure on the M-mode echocardiogram, and the autopsy findings of mild left ventricular enlargement imply that the patient developed acute, not
chronic, aortic
2
regurgitation .
reports of the aortitis syndrome with severe acute aortic regurgitation, including those by Honig’ and Akikusa.’ In these cases and in our own, acute There have been several such
regurgitation developed in the early prepulseless phase, before arterial stenosis had developed. The aortic valves have been generally believed to the atrophied, shortened, or thickened but seldom severely damaged. In this particular case, however, perforation of the aortic valve contributed to the patient’s acute aortic regurgitation. The only case of aortic valve perforation in chronic aortic regurgitation due to the aortitis syndrome of which the authors are aware is the one reported by Nakamura.s The severe inflammation was believed to have extended from the aorta to the aortic valve, where many inflammatory cells were found microscopically. aortic
Conclusion This is a case of a fifteen-year-old female with fatal acute aortic regurgitation caused aortic valve perforation, which is the first such report, as well as aortic dilatation.
by
Acknowledgments We are grateful to Prof. Nakamura in the Department of Geriatric Medicine, Keio University, and Prof. Hosoda in the Department of Pathology, Keio University, for their advice. Toru Satoh, M. D.
Department of Cardiology Ashikaga Red Cross Hospital 3-2100, Honjyo, Ashikaga-shi Tochigi-ken, 326 Japan References 1. Uedo H, Sugiura M, Ito I, et al: Aortic insufficiency associated with aortitis syndrome. Jpn Heart J 8:107120, 1967. 2. Morganroth J, Perloff JK, Zeldis SM: Acute severe aortic regurgitation. Ann Int Med 87:223-232, 1977. 3. Honig HS, Waintraub AM, Homes MN, et al: Severe aortic regurgitation secondary to idiopathic aortitis. Am
J Med 63:623-633, 1977. 4. Akikusa B, Kondo Y, Muraki N: Aortic insufficiency caused by Takayasu’s arteritis without usual clinical features. Arch Pathol Lab Med 105:650-651, 1981. 5. Nakamura C, Imai K, Hakuta Y, et al: Two cases of aortic and mitral regurgitation associated with aortitis syndrome. Heart (Japanese) 17:302-308, 1985.
Downloaded from ang.sagepub.com at Bobst Library, New York University on May 22, 2015
Aortitis
Syndrome
Toru Satoh, M.D. Masao Chino, M.D. Masaki Takahashi, M.D. and Keiji Suzuki, M.D.*
ASHIKAGA and MAEBASHI, JAPAN
Abstract This is a case report of a fifteen-year-old female with aortitis syndrome complicated by acute fatal aortic regurgitation due to the aortic valve perforation, as well as to aortic dilatation, which was confirmed by the autopsy. Aortic valve perforation was first recognized as the important cause of acute aortic regurgitation in the aortitis syndrome in this report. Introduction The aortitis syndrome is responsible for many disorders resulting from nonspecific vasculitis of the aorta and its large branching arteries. Aortic regurgitation, which develops in 10% to 50 % of the cases, is one of the most important causes of death. Aortic regurgitation usually progresses chronically as a result of dilatation of the aorta and the aortic valvular ring. Aortic valvular changes have been considered as contributing to aortic regurgitation but seldom have been regarded as an important cause of it. We encountered a case of fatal acute aortic regurgitation caused by aortic valve perforation, as well as by aortic dilatation.
Case
Report This fifteen-year-old female patient was in good health except for a predisposition to fever and the absence of menarche. Five days before admission she consulted her family physician for fever and productive cough. She was admitted to our hospital because of her increasing dyspnea and debility. From the Department of Cardiology, Ashikaga Red Cross Hospital of Medical Technology, Gunma University, Maebashi, Japan
Ashikaga;
and the
*Department of Pathology, College
869
Downloaded from ang.sagepub.com at Bobst Library, New York University on May 22, 2015
870 On examination she was found to be severely ill, a little drowsy, cyanotic, and orthopneic. Her heart rate was 134 per minute and regular, blood pressure 160/80 mm Hg, and body temperature 39.6°C. The patient’s jugular veins were dilated. There was a gallop heart rhythm without murmurs. There were moist rales over both her lung fields. Peripheral arterial pulsations were all equally felt. Laboratory data revealed anemia (hemoglobin 9.9 g/dL), severe thrombocytopenia (platelet count 440,000), enzyme elevation (glutamic ovaloacetic transaminase 342U/L, glutamic pyruvic transaminase 228U/L, lactic dehydrogenase 1011U/L), and marked hypoglycemia (glucose 7 mg/dL). Blood gas analysis showed metabolic acidosis (pH 7.27, Pa02 91 Torr, PaC02 13 Torr, HC03 5.7 mEq/L). Chest x-ray revealed marked cardiac enlargement, pulmonary congestion, and a dilated ascending aorta. There was left axis deviation but no sign of left ventricular hypertrophy on the electrocardiogram. A two-dimensional echocardiogram showed dilatation of the ascending aorta (60 mm in width), flailed aortic valves, and diffuse left ventricular hypokinesis without marked chamber dilatation (end-diastolic dimension 48 mm). There were massive aortic regurgitation, on the basis of a color Doppler echocardiogram, and early mitral valve closure, on the basis of an Mmode echocardiogram (Fig. 1, arrow). These findings suggest acute aortic regurgitation caused by aortic dilatation and derangement of the valve or valve-supporting structures.
FIG. 1. an
arrow)
closure of the mitral valve (indicated the M-mode echocardiogram.
Early on
by
FIG. 2.
Autopsy findings in the aortic valve: was perforated (probe inserted).
coronary cusp
Downloaded from ang.sagepub.com at Bobst Library, New York University on May 22, 2015
The left
871
The patient died unexpectedly several hours after admission, before the emergency aortic valve replacement could be performed. At necropsy the ascending aorta and the aortic valve ring were dilated. There was calcification of the basal portion of the aorta. The aortic valves were slightly thickened. The left coronary cusp was perforated (Fig. 2, arrow). The heart was dilated (340 g) and the left ventricular wall was mildly thickened. Histologically, the aortic media revealed diffuse necrosis, granuloma consisting of mononuclear cells with multinucleated giant cells, and marked fragmentation of elastic fibers. There were lymphocytic inflammatory cells with giant cells in the thickened intima and adventitia (Fig. 3A). These changes were observed segmentally throughout the aorta down to the bifurcation of the abdominal aorta. These findings led us to diagnose the patient as having had aortitis syndrome. Microscopic examination of the aortic valves revealed fragmentation and degeneration of collagen fibers, mononuclear cell inflammation, calcific deposits, necrosis, and granuloma formation in response to the necrotic tissue (Fig. 3B). There were no histologic findings corresponding to endocarditis.
Fic. 3. A. Wall of ascending aorta: marked fragmentation of elastic fibers, diffuse necrosis, and granuloma formation in the media. There are lymphocytic inflammatory cells with giant cells throughout the wall layers. Hematoxylin and eosin stains x 40.
FIG. 3. B. Aortic valve: fragmentation and degeneration of collagen fibers, profuse mononuclear cell infiltration. Hematoxylin and eosin stains x 40.
Downloaded from ang.sagepub.com at Bobst Library, New York University on May 22, 2015
872 Discussion The clinical picture of the aortitis syndrome has become clear, beginning with the first report by the Japanese ophthalmologist Takayasu in 1908. Aortic regurgitation is reported to have been observed in 20 % to 25 % of patients with this syndrome and to be the major abnorI mality leading to heart failure, the most frequent cause of death.’ Aortic regurgitation occurs mostly as a result of chronic aortic dilatation of more than five years’ duration. This patient, however, died of acute aortic regurgitation after a short and unusual course. The lack of evidence of left ventricular hypertrophy on the electrocardiogram, the finding of early mitral valve closure on the M-mode echocardiogram, and the autopsy findings of mild left ventricular enlargement imply that the patient developed acute, not
chronic, aortic
2
regurgitation .
reports of the aortitis syndrome with severe acute aortic regurgitation, including those by Honig’ and Akikusa.’ In these cases and in our own, acute There have been several such
regurgitation developed in the early prepulseless phase, before arterial stenosis had developed. The aortic valves have been generally believed to the atrophied, shortened, or thickened but seldom severely damaged. In this particular case, however, perforation of the aortic valve contributed to the patient’s acute aortic regurgitation. The only case of aortic valve perforation in chronic aortic regurgitation due to the aortitis syndrome of which the authors are aware is the one reported by Nakamura.s The severe inflammation was believed to have extended from the aorta to the aortic valve, where many inflammatory cells were found microscopically. aortic
Conclusion This is a case of a fifteen-year-old female with fatal acute aortic regurgitation caused aortic valve perforation, which is the first such report, as well as aortic dilatation.
by
Acknowledgments We are grateful to Prof. Nakamura in the Department of Geriatric Medicine, Keio University, and Prof. Hosoda in the Department of Pathology, Keio University, for their advice. Toru Satoh, M. D.
Department of Cardiology Ashikaga Red Cross Hospital 3-2100, Honjyo, Ashikaga-shi Tochigi-ken, 326 Japan References 1. Uedo H, Sugiura M, Ito I, et al: Aortic insufficiency associated with aortitis syndrome. Jpn Heart J 8:107120, 1967. 2. Morganroth J, Perloff JK, Zeldis SM: Acute severe aortic regurgitation. Ann Int Med 87:223-232, 1977. 3. Honig HS, Waintraub AM, Homes MN, et al: Severe aortic regurgitation secondary to idiopathic aortitis. Am
J Med 63:623-633, 1977. 4. Akikusa B, Kondo Y, Muraki N: Aortic insufficiency caused by Takayasu’s arteritis without usual clinical features. Arch Pathol Lab Med 105:650-651, 1981. 5. Nakamura C, Imai K, Hakuta Y, et al: Two cases of aortic and mitral regurgitation associated with aortitis syndrome. Heart (Japanese) 17:302-308, 1985.
Downloaded from ang.sagepub.com at Bobst Library, New York University on May 22, 2015
