%dCSBF N=25 1ooj Y =3.24X+24.9

5 10 15 AGE

20-- r = 0.44 l p<o.o5 0

0 ..-...... - .-------___-.---..------------ “La --________ _ _______ 0

0 -2o-. . : . 0 0

-40.. : . t .

.

1 ,.,,I, , , , , ,

5 10 15 AGE

FIGURE 2. Percent changes in coronary sblus biood flow (% A CSGF) and vascular resistance (% A CR) dwing atrial pac- ing. C/osed circled are mmnal chikken; open circles are sub- jects in this study.

these 8 patients, the percent change of coronary sinus bloodflow was 1.9 f 17.3%, and that of coronary vascu- lar resistance was -9.6 f 18.2%. In 4 of these 8patients, coronary sinus blood flow actually decreased during atria1 pacing. In 4 patients with ST-segment depression on stress tests, the change in coronary sinus blood flow was 10 f 16%, and that in coronary vascular resistance was -10 f 1470. Myocardial biopsy of the right ventricle was performed in these 8 patients; interstitial fibrosis was observed in 3, and mural thickening of the small coronary arteries in 1.

These results suggest that in the case of increased myocardial oxygen demand, these patients have a re- duced coronary flow reserve due to reduced dilatory ca- pacity of the small coronary arteries that are not visual- ized by coronary angiography. This reduced coronary reserve may be an important factor in the detection of positive findings on exercise stress tests in patients with Kawasaki disease without angiographic evidence of coro- nary stenosis. Furthermore, this study suggests that it may also be necessary to pay careful attention to the small coronary arteries in the evaluation of the coronary sequelae of Kawasaki disease.

1. Hamaoka K, Itoi T, Nakagawa M, Kamiya Y, Sawada T. Coronary sinus cannulation via the femoral vein. Pediatr Cardiol 1989;10:191-192. 2. Hamaoka K, Onouchi Z, Kamiya Y. Coronary sinus blood flow and coronary haemodynamic function in children: measurement by the continuous thermodilu- tion method with coronarv sinus cannulation via the femoral vein. Br Heart J 1991;65:171-173. - 3. Hamaoka K, Onouchi Z, Sakata K, Fukumochi H, Shiraishi I, Hayano T, Kamiya Y, Hayashi S. Characteristics of coronary hemodynamics in children. Jpn Circ J 1990;54:935-936.

Adult Coronary Artery Disease Secondary to Kawasaki Disease in Childhood Sugao Ishiwata, MD, Katsuo Fuse, MD, Shinichiro Nishiyama, MD, Shigemoto Nakanishi, MD, Yasunori Watanabe, MD, and Akira Seki, MD

K awasaki disease is a febrile disease of unknown etiol- ogy characterized by mucocutaneous involvement.

and occurring in infants and young children, which was first reported by Kawasaki in 1967.’ Coronary artery lesions, which develop as complications, are an important factor in determining the prognosis of this disease. Kawa- saki disease manifests itself at a comparatively early stage, and coronary aneurysms or myocardial disorders develop in about 20% of patients, with 50% of the aneu- rysms regressing spontaneously over a l- to 2-year period. Thus, ischemic heart disease is ultimately detected in <3% of all patients.2-4 Because follow-up times have been short, the long-term prognosis in these cases of residual cardiac lesions, especially coronary artery disease in the acute stage, is unclear. In recent years, there have been sporadic reports of cases of sequelae of Kawasaki disease

From the Department of Internal Medicine (1) and Surgery (2), Car- diovascular Center, Toranomon Hospital 2-2-2, Toranomon, Minatc- ku Tokyo, 105 Japan. Manuscript received September 16,199l; revised manuscript received and accepted October 28,199l.

in adults, especially young adults with ischemic heart disease.sw7 The aim of the present study was to clarify the clinical course and diagnostic imaging findings of pa- tients with residual lesions as sequelae of Kawasaki dis- ease.

This study consisted of 5 patients who recently un- derwent cineangiography for evaluation of coronary ar- tery lesions believed to be the sequelae of Kawasaki disease. The patients (2 men and 3 women) ranged in age from 17 to 36 years (Table I). All patients had long asymptomatic courses, and clinical myocardial ischemia manifested itself only after they had become adults. Pa- tients 1 and 2 had a clear history of Kawasaki disease. Age at onset of Kawasaki disease ranged from 18 months to 11 years old. The interval between the onset of Kawasaki disease and the manifestation of myocardial ischemic symptoms ranged from I1 to 28 years. The course of all 5 patients was asymptomatic for a period of >-IO years. In patients 3, 4 and 5, there was no clear history of Kawasaki disease, but Kawasaki disease was

692 THE AMERICAN JOURNAL OF CARDIOLOGY VOLUME 69 MARCH 1, 1992

strongly suspected based on the unique morphology and distribution of the coronary artery aneurysms. Old asymptomatic myocardial infarcts were observed in 4 patients, including 3 with anterior wall infarcts and 1 with an inferior wall infarct. One patient had arrhythmia and reduced left ventricularfunction. All patients under- went coronary artery bypass grafting.

All 5 patients displayed typical coronary angio- graphicfindings (Table I). Oval calcified coronary aneu- rysms were associated with stenosis or obstruction in every case. Patients 1 to 3 exhibited the proximal type of aneurysm, whereas patients 4 and 5 had several diffuse rosary-like aneurysms, ranging from the proximal to distal regions, with the same changes also being seen in both internal thoracic arteries. Marked collateral devel- opment was observed in all patients and this was also considered a characteristic finding.

The diagnosis of Kawasaki disease is based on symp- toms, and there are no test methods that provide a de% nite diagnosis. Thus, when the sequelae of Kawasaki dis- ease manifest themselves in adults, a long period has passed since the onset, no records remain of the patient’s course during infancy, and the memory of the patient or patient’s family is often not very clear. However, absence of positive history of Kawasaki disease in childhood does not exclude the diagnosis; angiographic appearance is highly suggestive of the diagnosis. Kawasaki disease pri- marily involves medium- to small-size muscular type arteries.* In many cases of congenital aneurysm, the le- sion is unilateral and its morphology is also slightly differ- ent from the aneurysm of Kawasaki disease.9 The disease that must be differentiated first is infantile periarteritis nodosa; however, this has recently come to be considered

TABLE I Clinical and Angiographic Findings in Five Patients with Sequelae of Kawasaki Disease

Pt. Age (yr) No. & sex

Age at Onset of

Kawasaki Disease (yr) Symptoms

Clinical Findings

Complications

1 30M 1.5 AP Apical MI 2 17F 6 AP

3 35M Unknown DOE Anterior MI 4 24F Unknown AP Anterior MI

DOE Arrhythmia Poor LV function

5 36F Unknown AP Inferior Ml

Aneurysm with Location of Aneurysm

Obstruction Calcification Coronary Other Collaterals

Angiographic Findings

1 + + - + 2 RCA + + + 3 LAD + + - + 4 RCA + +(D) ITA i-

5 RCA

+ LCX

+(D) ITA +

AP = angina pectoris; D = diffuse rosary-like; DOE = dyspnea on exertion; ITA = internal thoracic artery; LAD = left anterior descending artery; LCX = left circumflex artery; LV = left ventricular; MI = myocardial infarction: RCA = right coronary artery - = absent; + = present.

an acute inflammatory disease similar to Kawasaki dis- ease. lo,’ l

In all cases, no definite coronary risk factors were detected, and subsequently, there was a long-term asymptomatic course. Ischemic heart disease manifested itself when these patients became adults. Their coronary artery lesions were characterized by aneurysms associ- ated with calcification, stenosis on either side of the aneu- rysm, and the development of good collateral routes. The type of angina pectoris always took the form of advanced vascular lesions of a collateral source with occluded recip- ient branch; i.e., a jeopardized collateral. In many caseis, the right coronary artery was already obstructed in the early stage and stenosis on either side of the aneurysm of the left coronary artery as a collateral source had pro- gressed (Figure 1).

The aneurysms seen in Kawasaki disease have the same characteristics as those seen in diffuse panvasculitis; however, there is no fibrinoid necrosis or recurrence.12 Results of multiple studies based on serial angiography have shown that 50% of previously documented coronary artery aneurysms in Kawasaki disease regress spontane- ously in 1 to 2 years. In the case of many large aneurysms with almost no regression in the early stage, however,

FlGURE 1. Case 2. Selective left coronary angiogram in early phase (fop) and late phase @offo@ showing mild dilatation and severe stenosis in the proximal portion of the left anterior descending artery (fop white arrow). Right coronary artery is filled by good collateral vessels from the circumflex artery through the atrial branch. A gourd-shaped calcified aneurysm was detected in the proximal portion of right coronary artery (boffom white arrow).

BRIEF REPORTS 693

reactive intimal proliferation associated with the aggre- gation and organization of blood clots occurs within the aneurysm. Specific hemodynamic vessel wall-shearing stress is present at the inlet and outlet parts of the aneu- rysm, and this is said to damage endothelial cells and promote stenosis. l 3~14 However, the mechanism by which this stenosis progresses over a long period of time is still not well understood and requires further study.

We have no data concerning the long-term prognosis for children with Kawasaki disease who do not have aneu- rysms or whose aneurysms have regressed. A normal angiogram does not necessarily mean complete recovery by coronary arteries. Arterial damage may persist and there is a possibility of the risk of early arteriosclerosis as a sequela of vasculitis because of the characteristic fea- tures of panarteritis observed in this disease.15

Coronary artery bypass graft surgery was performed in all patients, and the l- to 4-year postoperative courses have been favorable. Arterial grafts, such as from the internal thoracic artery or gastroepiploic artery, which can be expected to retain good patency for a long period of time are currently recommended. However, in cases in which aneurysms are present throughout the entire length of the trunk of the coronary arteries, such as in patients 4 and 5, aneurysms may also be present in the internal thoracic artery, and when this vessel is planed to be used for grafting, it must be thoroughly evaluated to exclude presence of aneurysms preoperatively.

We believe that Kawasaki disease gives rise to long- term complications, such as aneurysms that progress to cause angina pectoris or myocardial infarction. A num- ber of the infants with Kawasaki disease currently being examined in the department of internal medicine may go

on to develop such complications in the future. Thus, long-term follow-up, including regular exercise tests and coronary arteriography, is considered important.

1. Kawasaki T, Kosaki F, Okawa S, Shigematsu I, Yanagawa H. A new infantile mucocutaneous lympho node syndrome (MLNS) prevailing in Japan. Pednxtrics 1914;54:21 l-216. 2. Kato H, Ichinose E, Yoshioka F, Takeshi T, Matunaga S, Suzuki K, Rikitake N. Fate of coronary aneurysms in Kawasaki disease. Serial coronary angiography and long-term follow up study. Am J Cardiol 1982;49:1758-1766. 3. Kamiya T, Suzuki J. Cardiovascular angiographic findings in Kawasaki dis- ease. Jpn J Pediatric Med 1985;17:765-770. 4. Nakano H, Ueda K, Saito A, Nojima K. Repeated angiograms in coronary arterial aneurysms in Kawasaki disease. Am J Cardiol 1985;56:846-851. 5. Flugelman YM, Hashin Y, Bassan MM, Lear R, Gotman SM. Acute myocar- dial infarction 14 years after an acute episode of Kawasaki disease. Am J Cardiol 1983;52:427-428. 6. Ohyagi Y, Hirose K, Tsujimoto S, Doyama K, Watanabe Y, Nakai T. Kawasa- ki disease complicated by acute myocardial infarction 9 years after onset. Am Heart J 1985;110:670-672. 7. Ishiwata S, Nishiyama S, Nakanishi S, Seki A, Watanabe Y, Konishi T, Fuse K. Coronary artery disease and internal mammary artery aneurysms in a young woman: possible sequelae of Kawasaki disease. Am Hear? J 1990;120:213-217, 8. Fujiwara H, Hamashima Y. Pathology of the heart in Kawasaki disease. Pediatrics 1978;61:1Of-107. 9. Wong CK, Cheng CH, Lau CP, Leung WH. Asymptomatic congenital core- nary artery aneurysm in adulthood. Ear Heart J 1989;10:947-949. 10. Landing BM, Larson E. Are infantile periarteritis nodosa with coronary artery involvement and fatal mucocutaneous lymph node syndrome the same? Comparison of 20 patients from Hawaii and Japan. Pediatrics 1977;59:651-662, 11. Tanaka N, Sekimoto K, Naoe S. Kawasaki disease. Relationship with infan- tile periarteritis nodosa. Arch Pathol Lab Med 1976;100:81-86. 12. Fujiwara H, Fujiwara T, Kao TC, Ohshio G, Hamashima Y. Pathology of Kawasaki disease in the healed stage. Relationship between typical and atypical cases of Kawasaki disease. Acta Pathol Jpn 1986;36:857-867. 13. Young DF, Tsai FY. Flow characteristics in models of arterial stenoses-steady flow. J Biomechnnics 1973;6:395-410. 14. Kim BM, Corcoran WH. Experimental measurements of turbulence spectra distal to stenosis. J Biomechanics 1974;7:335-342. 15. Kato H, Inoue 0, Akagi T. Kawasaki disease, cardiac problem and manage- ment. Pediatr Rev 1988;9:209-217.

Left Ventricular Function in Patients with Atrial Fibrillation Before and After Cardioversion Mahbubul Alam, MD, PhD, and Curt Thorstrand, MD, PhD

A trial fibrillation (AF) is a clinically important ar- rhythmia with important therapeutic and prognostic

implications. The disorganized electrical and mechanical activity causes loss of active atria1 contraction. This in turn results in insufficient atria1 emptying and impaired ventricular filling. l AF may be converted to sinus rhythm by means of direct-current countershock. Hemodynamic studies before and after cardioversion of AF to normal sinus rhythm report conflicting results regarding im- provement in hemodynamic function.2-4 Some studies suggested that normal atria1 contraction may not occur immediately after successful cardioversion of AF to sinus rhythm.lT3 The purpose of this study was to evaluate, using echocardiography and Doppler, the mechanical ac- tivity of the left atrium and its effect on left ventricular (LV) function after electrocardioversion of AF to sinus rhythm.

From the Department of Medicine I, Cardiology Section, Karolinska Institute at South Hospital (SMersjukhuset), 11883 Stockholm, Swe- den. Manuscript received July 16, 1991; revised manuscript received and accepted November 4,199l.

The study was begun with 19 patients with electro- cardiographic evidence of AF and without history of coronary artery disease or cardiomyopathy. The study was designedfor 1 -month follow-up. Seven patients were excluded: 5 owing to reversion to AF before the final echocardiographic examination, and 2 owing to unsuc- cessful cardioversion. Left atria1 dimension was not used as an exclusionary criterion. Thus, 12 patients (aged 57 f 9 years) were converted to normal sinus rhythm by means of direct-current cardioversion. Demographic data on the patients are listed in Table I. Digoxin was withdrawn 2 days before cardioversion. Patients were receiving anticoagulation therapy (warfarin sodium) for 12 weeks, with prothrombin time being within the thera- peutic range. Inpatients receivingsotalol medication, the drug was continued at the time of cardioversion.

Patients were examined before, and 4 hours, 2 weeks and 1 month after cardioversion. All patients underwent complete M-mode and 2-dimensional echocardiogra- phy, including pulsed-wave Doppler. An Aloka SSD- 870 echocardiographic machine with a 2.5 MHz trans-

694 THE AMERICAN JOURNAL OF CARDIOLOGY VOLUME 69 MARCH 1, 1992