European Heart Journal (1990) 11, 572-576
Aortic regurgitation secondary to Behcet's disease. A case report and review of the literature T. CHIKAMORI, Y. L. Dor, Y. YONEZAWA, J. TAKATA, M. KAWAMURA, AND T. OZAWA
Section of Cardiology, Department of Medicine & Geriatrics, Kochi Medical School, Okohcho, Nankoku, Kochi, Japan
KEY WORDS: Behcet's disease, aortic regurgitation, echocardiography. Cardiac complications, especially aortic regurgitation, are very rare in Behcet 's disease. This report describes a patient with a complicated abnormality of the aortic valve apparatus and severe aortic regurgitation secondary to Behcet's disease. In the literature,flailprolapsed valve, perforated valves and rupturedpseudoaneurysms of the sinus of Valsalva have been reported. Echocardiography may be most useful to detect these anatomically complex abnormalities and therefore to elucidate the cause of aortic regurgitation in Behcet's disease. Introduction Behcet's disease, a chronic relapsing inflammatory process of unknown etiology, involves multiple organs, especially the oro-cutaneo-oculo-genital systems. The prognosis of the disease, however, depends not upon the involvement of these four systems but upon the complications in other systems such as the central nervous system, the gastrointestinal tract and the vascular system'1 -H Although cardiac involvement is very rare in Behcet's disease, it may play an important role in the prognosis of the disease13"2'1. The purpose of this report is to describe a rare aortic abnormality in a patient with severe aortic regurgitation secondary to Behcet's disease who was treated surgically. In addition, reports of cases of aortic regurgitation in Behcet's disease from the English and Japanese literature are reviewed. Case report A 54-year-old woman was admitted because of increasing exertional dyspnoea. The patient had been well until the age of 30 years when a generalized skin rash first appeared. Recurrent oral and genital aphthous ulcers were noticed 10 years later. Four years before admission, she was brought to another hospital because of syncope. Electrocardiography revealed complete atrio-ventricular block and a Submitted for publication on 4 January 1989, and in revised form 18 October 1989. Address for reprints: Yoshinori I_. Doi MD, Section of Cardiology, Kochi Medical School, Okohcho, Nankoku, Kochi, 783, Japan.
permanent pacemaker was implanted. Echocardiographic examination at that time demonstrated abnormal aortic architecture which resembled a parachute in the left ventricular outflow tract during diastole. Diagnosis of flail aortic valve associated with Behcet's disease of incomplete form was made. She was well on steroid therapy for the next 3 years, but began to complain of exertional dyspnoea 1-5 years before current admission. Despite treatment with digitalis and diuretics, she was unable to make any improvement and was referred to our institution. On examination, the patient appeared chronically ill. Abnormal findings included a grade 3/6 systolic ejection murmur and a grade 3/6 diastolic blowing murmur at the left lower sternal border and a single aphthous ulcer in the mouth. An electrocardiogram showed a pacemaker rhythm. A chest X-ray showed a cardiothoracic ratio of 66% without pulmonary congestion. Echocardiographic examination disclosed an echo-free space of the aortic root near the non-coronary cusp, in addition to the previously recognized parachute-like abnormal aortic valve (Fig. la,b). Compared with the previous study (end-diastolic dimension, 51 mm; end-systolic dimension, 35 mm; ejection fraction 68%), the left ventricle showed marked dilatation (end-diastolic dimension, 75 mm; end-systolic dimension, 58 mm; ejection fraction, 54%). Colourmapped Doppler echocardiographic study demonstrated a diastolic mosaic regurgitant signal within the left ventricle, which was directed from the echofree space of the aortic root towards the interventricular septum (Fig. lc). Because of increasing
0195-668X '90/060572 + 05 S03.000 Downloaded from https://academic.oup.com/eurheartj/article-abstract/11/6/572/440961 by guest on 08 January 2018
© 1990 The European Society of Cardiology
Behget 's disease
5 73
(a
(c)
Figure 1 Echocardiographic examination at the admission, (a) Parastemal long-axis view: parachutelike abnormal echo in the left ventricular outflow tract ( ^ , and echo-free space in the posterior aortic root (*). (b) Parastemal short-axis view: round abnormal echo ( ^ ) . (c) Colour Doppler echo: mosaic regurgitant signal from the echo-free space towards [he interventricular septum.
heart size, breathlessness unresponsive to medical therapy and deterioration of left ventricular function, she was sent to surgery for aortic valve replacement. Operative findings
The aortic valve showed a fibrous appearance with fenestration of the non-coronary cusp. A pseudoaneurysm of the sinus of Valsalva, with a barrel-shaped entrance near the aortic ring of the non-coronary cusp, was found to extend to the interventricular septum (Fig. 2). The main cause of aortic regurgitation was thought to be the fenestration of the non-coronary cusp. Aortic valve replacement and patch closure of the pseudoaneurysm were undertaken, and the post-operative course was uneventful. Histological examination of the aortic valve showed non-specific inflammatory process with lymphocytic infiltration in the perivascular area and no bacterial growth was yielded from the specimen.
Discussion There has been an increasing recognition that autoimmune disorders could be a cause of anatomic abnormalities of the aortic valve apparatus'221. Ankylosing spondylitis, Reiter's syndrome, psoriatic arthritis and Takayasu's aortitis are all well known possible causes of aortic regurgitation'23"251. Behcet's disease, however, is not a commonly recognized cause of aortic regurgitation probably because of the rarity of the disease itself. To date, there have been reports of 20 cases of aortic regurgitation secondary to Behcet's disease in the English and Japanese literature (Table I)13"2". The mean age of the patients was 43-8 years (range: 26-58 years) with a male to female ratio of two to one. The duration of symptoms before the clinical manifestation of aortic regurgitation varies considerably. Confirmation of the diagnosis of aortic regurgitation was made echocardiographically in four patients, by cardiac catheterization in thirteen patients, and after autopsy in one patient. In the remaining three patients the diagnosis was made
Downloaded from https://academic.oup.com/eurheartj/article-abstract/11/6/572/440961 by guest on 08 January 2018
5 74 T. Chikamori et al.
Lt. oppendoge Blind soc
Figure 2 Operative findings: (a) The fenestration in the non-coronary cusp, (b) A schema around the aortic valve. In addition to the fenestration in the non-coronary cusp, a pseudoaneurysm of the sinus of Valsalva, with a barrel-shaped entrance near the aortic ring of the non-coronary cusp, extends to the interventricular septum. L.C.C. = left coronary cusp; R.C.C. =rightcoronary cusp; N.C.C. = non-coronary cusp; IVS - interventricular septum; PA = pulmonary artery; MV = mitral valve; LA = left atrium.
only clinically. Anatomic findings were available for 19 patients; i.e., operative findings in 12, autopsy findings in one, and echographicfindingsin six. A flail or prolapsed aortic valve was observed in five patients; a perforated aortic valve in five; a pseudoaneurysm of the sinus of Valsalva in six, and simple aortic regurgitation in three patients. Associated mitral regurgitation was found in six
patients. Aortic valve replacement, with or without wrapping of the aorta, was performed in 12 patients. Serious operative complications were seen in four patients; false aneurysm from the cannulation site in one patient and perivalvular leakage in three patients. Permanent pacemaker implantation was needed in three patients because of advanced heart block. During the follow-up, four patients
Downloaded from https://academic.oup.com/eurheartj/article-abstract/11/6/572/440961 by guest on 08 January 2018
Downloaded from https://academic.oup.com/eurheartj/article-abstract/11/6/572/440961 by guest on 08 January 2018
incomplete incomplete incomplete incomplete incomplete
incomplete
Japanese*31 Japanese1*1 Japanese"1 Japanese161 Caucasian171
Japanese111
43 M 49M 38M 43 F 26M
49M
36F 51M 44F 53F 46F 50M
47M
31F
48F 58M 35M 40M
55M
40M
55F
1 2 3 4 5 6
7 8 9 10 11 12
13
14
15 16 17 18
19
20
21
Japanese '
incomplete
simple NA NA aneurysm of the rt. sinus of Valsalva perforated AoV r pseudoaneurysm of the sinus the Valsalva L ascending aortic aneurysm ascending aortic aneurysm severe dilatation of the ascending aorta simple aortitis, chordal rupture of the MV simple (vasculitis of vasa vasorum) nodule on the RCC — incomplete closure pseudoaneurysm of the rt. sinus of Valsalva — rupture r pseudoaneurysm of the sinus of Valsalva [ (RCC), prolapse & fenestration of the NCC flail AoV (LCC, NCC) prolapse of the RCC AR + MR prolapse( + ) r pseudoaneurysm of the sinus of Valsalva [ (LCC), fenestration of the LCC & RCC flail & fenestrated LCC r pseudoaneurysm of the sinus of Valsalva [ (NCC), fenestration of the NCC
Features of AR
perivalvular leakage — reoperation well 6 months later
AVR
sudden death
NA NA NA NA
NA
well 10 months later
NA death from perivalvular leakage NA NA NA perivalvular leakage — reoperation
sudden death NA NA death from CHF false aneurysm from cannulation site NA
Prognosis
AVR
AVR (-) (-) (—) AVR + mitral valvoplasty
AVR
VR + pacemaker implantation
AVR + wrapping of the aorta AVR + MVR + wrapping (-) AVR + MVR AVR AVR
(-)
(-) (-) (-) (-) AVR
Operation
AoV-aortic valve; AR=>aortic regurgitation; AVR = aortic valve replacement; CHF*.congestive heart failure; F = female; LCC = left coronary cusp; M -male; M R mitral regurgitation; M V — mitral valve; NA = not available; NCC = non-coronary cusp; No. = number; RCC =>rightcoronary cusp; rt. = right.
Japanese
incomplete
incomplete
Japanese1"1
12 1
complete
incomplete complete complete incomplete
Japanese1"1 Chinese1'71 Caucasian1"1 Japanese1"1
complete
Japanese1"1
Alaskan Indian1'51
NA complete incomplete incomplete incomplete incomplete
Japanese ^ Japanese1'01 Japanese1'01 Japanese1"1 Japanese1'31 Japanese1'31
1
Disease form
Age& sex
Summary of findings in 21 patients with aortic regurgitation secondary to Behcet 's disease reported in the English and Japanese literature
Race
No.
Table I
576
T. Chikamoriet al.
[9] Takahashi S, Shiota K, Kazuno H et al. An operated case of vasculo-Behcet's syndrome associated with aneurysm of the ascending aorta and aortic insufficiency. Jpn Circ J 1982; 46 (Suppl III): 742 (Abstr, in Japanese). [10] Matsumura Y, Ohtsu F, Nakata S et al. Two cases of Behcet's disease associated with aortic insufficiency. Studies on Behcet's disease (Behcet's Disease Committee of Japan). Ministry of Welfare, Japan 1981): 158-60 (in Japanese). [11] Kihara Y, Hirose K, Matsumura et al. A case of heart failure due to aortic and mitral regurgitation secondary to Behcet's disease. Jpn Circ J 1982; 46: (Suppl III): 675 (Abstr, in Japanese). [12] Ohmori F, Nagata N, Miyatake K et al. A case of Behcet's disease associated with aortic insufficiency. Jpn Circ J 1982; 46 (Suppl III): 676 (Abstr, in Japanese). [13] Sasako Y, Maeda Y, lharaK. etal. Repeat valve replacement in a case of aortic regurgitation secondary to Behcet's disease. Jpn J Cardiovasc Surg 1982; 12:107-10 (Abstr, in Japanese). [14] Nojiri T, Endo M, Koyanagi H et al. A successful aortic valve replacement and permanent pacemaker implantation in a case of aortic regurgitation and ruptured aneurysm of sinus of Valsalva into left ventricular cavity associated with Behcet's disease. Jpn Ann Thorac Surg 1983; 3: 224-32 (in Japanese). [15] Comess KA, Zibelli LR, Gordon D et al. Acute, severe, aortic regurgitation in Behcet's syndrome. Ann Int Med References 1983; 99: 639-40. [1] Snyderman R. Behcet's disease. In: Wyngaarden JB, [16] Tokunaga K, Furuta S, Machii K et al. EchocardioSmith LH, eds. Cecil textbook of medicine. Philadephia: graphic manifestations of nonrheumatic aortic regurgiWBSaunders, 1985: 1960-2. tation. J Cardiography 1985; 15: 829-46 (in Japanese). [2] Eagle KA, De Sanctis RW. Diseases of the aorta. In: [17] Sheu Lu-Li, Cui Guang-Gen, Liang Ru-Lian. Valve Braunwald E, eds. Heart disease, a textbook of cardioprolapse in Behcet's disease. Br Heart J 1985; 54: 100-2. vascular medicine. Philadephia: WB Saunders, 1988: [18] Pena JM, Garcia-Alegria J, Garcia-Fernandez F et al. 1566. Mitral and aortic regurgitation in Behcet's syndrome. [3] Shimamine T, Murakami S. A pathological study of Ann Rheum Dis 1985; 44: 637-9. aortic lesions in autopsied cases of Behcet's syndrome. Studies on Behcet's disease (Behcet's Disease Research [19] Terada N, Satoh Y, Miyahara Y et al. A case of Behcet's disease associated with aortic and mitral insufficiency. Committee of Japan, Ministry of Welfare, Japan 1973): Nihon Naika Gakkai Zasshi 1986; 75: 151 (Abstr in 29-32 (in Japanese). Japanese). [4] TanakaS,TokanoN,InoueT«a/. Pathophysiologyand prognosis of Behcet's disease associated with cardio- [20] Washio M, Irisawa T, Kobayashi M et al. A case of aortic regurgitation and aneurysm of sinus of Valsalva vascular lesions. Studies on Behcet's disease (Behcet's associated with Behcet's disease. Heart 1986; 18:179-87 Disease Research Committee of Japan, Ministry of (in Japanese). Welfare, Japan 1975): 262-9 (in Japanese). [5] Ieyumi T, Nomoto K, Kawabata T el al. A case of [21] Motaki K, Oomori F, Kawazoe K et al. Behcet's disease with aorticregurgitation— A long-term observed case intestinal Behcet's syndrome associated with aortic after valve replacement. Saishinigaku 1986; 41:2657-66 insufficiency. Nihon Naika Gakkai Zasshi 1977; 66:150 (in Japanese). (Abstr, in Japanese). [6] Satoh I, Hirosawa K, Yokota Hetal.An autopsied case [22] Qaiyumi S, Hassan ZU, Toone E. Seronegative spondyloarthropathies in lone aortic insufficiency. Arch of Behcet's disease of incomplete form associated with Intern Med 1985; 145: 822-4. cardiac lesion. Nihon Naika Gakkai Zasshi 1979; 69:96 [23] Bulldey BH, Roberts WC. Ankylosing spondylitis and (Abstr, in Japanese). aorticregurgitation.Circulation 1973; 48: 1014-27. [7] Rae SA, Vandenburg M. Aortic regurgitation and false aortic aneurysm formation in Behcet's disease. Postgrad [24] Muma WF, Roller DH, Craft J et al. Psoriatic arthritis and aortic regurgitation. J Am Med Assoc 1980; 244: MedJ 1980; 56:438-9. 364-5. [8] Yabe Y, Sanada T, Nagase H et al. A case of vasculoBehcet's syndrome associated with aneurysm of ascend- [25] Paul us HE, Pearson CM, Pitts W et al. Aortic insufing aorta and aortic regurgitation. Heart 1980; 12: ficiency in five patients with Reiter's syndrome. Am J 1330-7 (in Japanese). Med 1972; 53:464-72.
died suddenly or from heart failure, although the description of the prognosis of 16 patients was inadequate to make a proper assessment. Aortic regurgitation in patients with ankylosing spondylitis, psoriatic arthritis and Reiter's syndrome is thought to be due to dilatation of the aortic valve ring and/or thickening and shortening of the cusps'23'. The mechanism of aortic regurgitation, however, seems to be much more complicated in patients with Behcet's disease. Echocardiography may be most useful to detect these anatomically complex abnormalities and therefore to elucidate the cause of aortic regurgitation in Behcet's disease. Although the prognosis of Behcet's disease and the natural history of unoperated aortic regurgitation in this condition remain to be studied, the fact that four of the 21 patients died suddenly or from heart failure may indicate that much attention must be paid to the occurrence of para valvular leakage after valve replacement. Also the occurrence of sudden death in the reports of heart block suggest that close follow-up of this abnormality is required.
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Aortic regurgitation secondary to Behcet's disease. A case report and review of the literature T. CHIKAMORI, Y. L. Dor, Y. YONEZAWA, J. TAKATA, M. KAWAMURA, AND T. OZAWA
Section of Cardiology, Department of Medicine & Geriatrics, Kochi Medical School, Okohcho, Nankoku, Kochi, Japan
KEY WORDS: Behcet's disease, aortic regurgitation, echocardiography. Cardiac complications, especially aortic regurgitation, are very rare in Behcet 's disease. This report describes a patient with a complicated abnormality of the aortic valve apparatus and severe aortic regurgitation secondary to Behcet's disease. In the literature,flailprolapsed valve, perforated valves and rupturedpseudoaneurysms of the sinus of Valsalva have been reported. Echocardiography may be most useful to detect these anatomically complex abnormalities and therefore to elucidate the cause of aortic regurgitation in Behcet's disease. Introduction Behcet's disease, a chronic relapsing inflammatory process of unknown etiology, involves multiple organs, especially the oro-cutaneo-oculo-genital systems. The prognosis of the disease, however, depends not upon the involvement of these four systems but upon the complications in other systems such as the central nervous system, the gastrointestinal tract and the vascular system'1 -H Although cardiac involvement is very rare in Behcet's disease, it may play an important role in the prognosis of the disease13"2'1. The purpose of this report is to describe a rare aortic abnormality in a patient with severe aortic regurgitation secondary to Behcet's disease who was treated surgically. In addition, reports of cases of aortic regurgitation in Behcet's disease from the English and Japanese literature are reviewed. Case report A 54-year-old woman was admitted because of increasing exertional dyspnoea. The patient had been well until the age of 30 years when a generalized skin rash first appeared. Recurrent oral and genital aphthous ulcers were noticed 10 years later. Four years before admission, she was brought to another hospital because of syncope. Electrocardiography revealed complete atrio-ventricular block and a Submitted for publication on 4 January 1989, and in revised form 18 October 1989. Address for reprints: Yoshinori I_. Doi MD, Section of Cardiology, Kochi Medical School, Okohcho, Nankoku, Kochi, 783, Japan.
permanent pacemaker was implanted. Echocardiographic examination at that time demonstrated abnormal aortic architecture which resembled a parachute in the left ventricular outflow tract during diastole. Diagnosis of flail aortic valve associated with Behcet's disease of incomplete form was made. She was well on steroid therapy for the next 3 years, but began to complain of exertional dyspnoea 1-5 years before current admission. Despite treatment with digitalis and diuretics, she was unable to make any improvement and was referred to our institution. On examination, the patient appeared chronically ill. Abnormal findings included a grade 3/6 systolic ejection murmur and a grade 3/6 diastolic blowing murmur at the left lower sternal border and a single aphthous ulcer in the mouth. An electrocardiogram showed a pacemaker rhythm. A chest X-ray showed a cardiothoracic ratio of 66% without pulmonary congestion. Echocardiographic examination disclosed an echo-free space of the aortic root near the non-coronary cusp, in addition to the previously recognized parachute-like abnormal aortic valve (Fig. la,b). Compared with the previous study (end-diastolic dimension, 51 mm; end-systolic dimension, 35 mm; ejection fraction 68%), the left ventricle showed marked dilatation (end-diastolic dimension, 75 mm; end-systolic dimension, 58 mm; ejection fraction, 54%). Colourmapped Doppler echocardiographic study demonstrated a diastolic mosaic regurgitant signal within the left ventricle, which was directed from the echofree space of the aortic root towards the interventricular septum (Fig. lc). Because of increasing
0195-668X '90/060572 + 05 S03.000 Downloaded from https://academic.oup.com/eurheartj/article-abstract/11/6/572/440961 by guest on 08 January 2018
© 1990 The European Society of Cardiology
Behget 's disease
5 73
(a
(c)
Figure 1 Echocardiographic examination at the admission, (a) Parastemal long-axis view: parachutelike abnormal echo in the left ventricular outflow tract ( ^ , and echo-free space in the posterior aortic root (*). (b) Parastemal short-axis view: round abnormal echo ( ^ ) . (c) Colour Doppler echo: mosaic regurgitant signal from the echo-free space towards [he interventricular septum.
heart size, breathlessness unresponsive to medical therapy and deterioration of left ventricular function, she was sent to surgery for aortic valve replacement. Operative findings
The aortic valve showed a fibrous appearance with fenestration of the non-coronary cusp. A pseudoaneurysm of the sinus of Valsalva, with a barrel-shaped entrance near the aortic ring of the non-coronary cusp, was found to extend to the interventricular septum (Fig. 2). The main cause of aortic regurgitation was thought to be the fenestration of the non-coronary cusp. Aortic valve replacement and patch closure of the pseudoaneurysm were undertaken, and the post-operative course was uneventful. Histological examination of the aortic valve showed non-specific inflammatory process with lymphocytic infiltration in the perivascular area and no bacterial growth was yielded from the specimen.
Discussion There has been an increasing recognition that autoimmune disorders could be a cause of anatomic abnormalities of the aortic valve apparatus'221. Ankylosing spondylitis, Reiter's syndrome, psoriatic arthritis and Takayasu's aortitis are all well known possible causes of aortic regurgitation'23"251. Behcet's disease, however, is not a commonly recognized cause of aortic regurgitation probably because of the rarity of the disease itself. To date, there have been reports of 20 cases of aortic regurgitation secondary to Behcet's disease in the English and Japanese literature (Table I)13"2". The mean age of the patients was 43-8 years (range: 26-58 years) with a male to female ratio of two to one. The duration of symptoms before the clinical manifestation of aortic regurgitation varies considerably. Confirmation of the diagnosis of aortic regurgitation was made echocardiographically in four patients, by cardiac catheterization in thirteen patients, and after autopsy in one patient. In the remaining three patients the diagnosis was made
Downloaded from https://academic.oup.com/eurheartj/article-abstract/11/6/572/440961 by guest on 08 January 2018
5 74 T. Chikamori et al.
Lt. oppendoge Blind soc
Figure 2 Operative findings: (a) The fenestration in the non-coronary cusp, (b) A schema around the aortic valve. In addition to the fenestration in the non-coronary cusp, a pseudoaneurysm of the sinus of Valsalva, with a barrel-shaped entrance near the aortic ring of the non-coronary cusp, extends to the interventricular septum. L.C.C. = left coronary cusp; R.C.C. =rightcoronary cusp; N.C.C. = non-coronary cusp; IVS - interventricular septum; PA = pulmonary artery; MV = mitral valve; LA = left atrium.
only clinically. Anatomic findings were available for 19 patients; i.e., operative findings in 12, autopsy findings in one, and echographicfindingsin six. A flail or prolapsed aortic valve was observed in five patients; a perforated aortic valve in five; a pseudoaneurysm of the sinus of Valsalva in six, and simple aortic regurgitation in three patients. Associated mitral regurgitation was found in six
patients. Aortic valve replacement, with or without wrapping of the aorta, was performed in 12 patients. Serious operative complications were seen in four patients; false aneurysm from the cannulation site in one patient and perivalvular leakage in three patients. Permanent pacemaker implantation was needed in three patients because of advanced heart block. During the follow-up, four patients
Downloaded from https://academic.oup.com/eurheartj/article-abstract/11/6/572/440961 by guest on 08 January 2018
Downloaded from https://academic.oup.com/eurheartj/article-abstract/11/6/572/440961 by guest on 08 January 2018
incomplete incomplete incomplete incomplete incomplete
incomplete
Japanese*31 Japanese1*1 Japanese"1 Japanese161 Caucasian171
Japanese111
43 M 49M 38M 43 F 26M
49M
36F 51M 44F 53F 46F 50M
47M
31F
48F 58M 35M 40M
55M
40M
55F
1 2 3 4 5 6
7 8 9 10 11 12
13
14
15 16 17 18
19
20
21
Japanese '
incomplete
simple NA NA aneurysm of the rt. sinus of Valsalva perforated AoV r pseudoaneurysm of the sinus the Valsalva L ascending aortic aneurysm ascending aortic aneurysm severe dilatation of the ascending aorta simple aortitis, chordal rupture of the MV simple (vasculitis of vasa vasorum) nodule on the RCC — incomplete closure pseudoaneurysm of the rt. sinus of Valsalva — rupture r pseudoaneurysm of the sinus of Valsalva [ (RCC), prolapse & fenestration of the NCC flail AoV (LCC, NCC) prolapse of the RCC AR + MR prolapse( + ) r pseudoaneurysm of the sinus of Valsalva [ (LCC), fenestration of the LCC & RCC flail & fenestrated LCC r pseudoaneurysm of the sinus of Valsalva [ (NCC), fenestration of the NCC
Features of AR
perivalvular leakage — reoperation well 6 months later
AVR
sudden death
NA NA NA NA
NA
well 10 months later
NA death from perivalvular leakage NA NA NA perivalvular leakage — reoperation
sudden death NA NA death from CHF false aneurysm from cannulation site NA
Prognosis
AVR
AVR (-) (-) (—) AVR + mitral valvoplasty
AVR
VR + pacemaker implantation
AVR + wrapping of the aorta AVR + MVR + wrapping (-) AVR + MVR AVR AVR
(-)
(-) (-) (-) (-) AVR
Operation
AoV-aortic valve; AR=>aortic regurgitation; AVR = aortic valve replacement; CHF*.congestive heart failure; F = female; LCC = left coronary cusp; M -male; M R mitral regurgitation; M V — mitral valve; NA = not available; NCC = non-coronary cusp; No. = number; RCC =>rightcoronary cusp; rt. = right.
Japanese
incomplete
incomplete
Japanese1"1
12 1
complete
incomplete complete complete incomplete
Japanese1"1 Chinese1'71 Caucasian1"1 Japanese1"1
complete
Japanese1"1
Alaskan Indian1'51
NA complete incomplete incomplete incomplete incomplete
Japanese ^ Japanese1'01 Japanese1'01 Japanese1"1 Japanese1'31 Japanese1'31
1
Disease form
Age& sex
Summary of findings in 21 patients with aortic regurgitation secondary to Behcet 's disease reported in the English and Japanese literature
Race
No.
Table I
576
T. Chikamoriet al.
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died suddenly or from heart failure, although the description of the prognosis of 16 patients was inadequate to make a proper assessment. Aortic regurgitation in patients with ankylosing spondylitis, psoriatic arthritis and Reiter's syndrome is thought to be due to dilatation of the aortic valve ring and/or thickening and shortening of the cusps'23'. The mechanism of aortic regurgitation, however, seems to be much more complicated in patients with Behcet's disease. Echocardiography may be most useful to detect these anatomically complex abnormalities and therefore to elucidate the cause of aortic regurgitation in Behcet's disease. Although the prognosis of Behcet's disease and the natural history of unoperated aortic regurgitation in this condition remain to be studied, the fact that four of the 21 patients died suddenly or from heart failure may indicate that much attention must be paid to the occurrence of para valvular leakage after valve replacement. Also the occurrence of sudden death in the reports of heart block suggest that close follow-up of this abnormality is required.
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