Pediatr Cardiol 11:153-155, 1990
Pediatric Cardiology 9 Springer-VerlagNew York Inc. 1990
Case Reports Mitral R e g u r g i t a t i o n w i t h G r o s s D e f o r m i t y o f a Mitral L e a f l e t D u e to K a w a s a k i D i s e a s e Hideshi Tomita, 1 Yohko Sawada, ~ Yoshihito Higashidate, 1 Shunzo C h i b a , l Seiichi Ito, 2 and Ryoichi Kogasaka 2 ~Department of Pediatrics, Sapporo Medical College, Sapporo; and 2Department of Pediatrics, Sunagawa City Hospital, Sunagawa City, Japan
SUMMARY. A 4-year-old girl had two episodes of Kawasaki disease which resulted in severe mitral regurgitation. She had not had any coronary arterial lesion during the acute phase of the illness, nor any myocardial ischemia. Severe destruction of the mitral leaflet was documented by two-dimensional echocardiography. It is suggested that this lesion was due to severe carditis and valvulitis which developed during the acute phase of the illness. KEY WORDS: Kawasaki disease - - Mitrai regurgitation - - Vaivulitis
Case Report A 4-year-old girl had the first onset of Kawasaki disease (KD), displaying the six typical signs and symptoms, at 3 months of age. She was first admitted to Sunagawa City Hospital with a fever which persisted for 19 days. Two-dimensional echocardiography (2DE) revealed neither coronary arterial nor valvar lesions. She was treated with aspirin (30 mg/kg/day) and dipyridamol (4 mg/kg/day), and discharged 1 month later without any new signs or symptoms. She was readmitted to Sunagawa City Hospital at 5 months of age because of recurrent signs and symptoms. Chest roentgenogram and electrocardiogram (ECG) on admission suggested pancarditis, marked cardiomegaly on the chest X-ray [cardiothoracic ratio (CTR), 60%), abnormal Q in leads III and aVF, ST depression in leads III-VI, and flat T wave in the anterior chest leads (Fig. 1). She was again treated with aspirin (50 mg/ kg/day), dipyridamol (4 mg/kg/day), and digoxin (0.01 mg/kg/ day). The CTR increased to 66% after 10 days of hospitalization and hepatosplenomegaly appeared. At day 21 of hospitalization, a harsh pansystolic murmur (3/6) was audible and signs of congestive heart failure were greatly exaggerated. There was no coronary involvement, but the anterior and posterior leaflets of the mitral valve were thickened on 2DE when recorded on the day 24 of hospitalization. Also documented were a marked anterocentral mitral valve prolapse and a slight pericardial effusion. We diagnosed the apical heart murmur to be due to severe mitral
Address offprint requests to." Dr. Hideshi Tomita, Department of Pediatrics, Sapporo Medical College, South 1 West 16 Chuo-Ku, Sapporo, Japan.
regurgitation. She went into cardiogenic shock with hypotension on day 34 of hospitalization and was treated with continuous venous infusion of dopamine (5 ~g/kg/min). Signs and symptoms of congestive heart failure improved by day 36 of hospitalization. There was no elevation of serum glutamic-oxaloacetic transaminase, glutamic-pyruvic transaminase, lactic dehydrogenase, and creatinine phosphokinase during the acute phase. She is at present well-controlled on digoxin and diuretics. She was admitted to Sapporo Medical College, Department of Pediatrics, for further evaluation of mitral regurgitation. Her growth and development were good (weight 14 kg, height 92.3 cm), but she had slight tachydyspnea and she was in symptomatic state class 2 (NYHA). A grade 3 pansystoilc murmur and a low-pitched grade 2/6 middiastolic murmur were audible at the apex. Routine laboratory examination, however, revealed no specific abnormality. Cardiomegaly (CTR 64%) and mild pulmonary venous congestion were seen on the chest X-ray. Electrocardiogram documented marked left ventricular hypertrophy and a mitral P. The 2DE showed a marked dilatation of the left ventricle (left ventricular end-diastolic dimension 42 mm) and atrium, a slightly flattened interventricular septum, and a severely thickened and prolapsed anterior mitral leaflet (Fig. 2). No abnormalities, however, were observed in the coronary arteries and left ventricular wall motion (ejection fraction 0.73). Grade 4/4 mitral regurgitation was detected by real-time color flow-mapping. Cardiac catheterization data demonstrated a slightly elevated pulmonary capillary wedge pressure (mean = 10 mmHg) and mild pulmonary hypertension (41/14, mean = 26 mmHg). Her mitral regurgitation was diagnosed as grade 3 by left ventriculography (Fig. 3). There was no coronary involvement. Left ventricular end-diastolic volume index was 130.5 ml/m 2, ejection fraction was 0.64, and the regurgitant index was 0.76.
154
Pediatric Cardiology Vol. I I, No. 3, 1990
Discussion
Fig. 1. The ECG in the acute phase of the second episode of Kawasaki disease.
Mitral regurgitation complicated by KD is classified into two groups; one is mitral regurgitation, mainly caused by valvulitis; and the other is secondary to the papillary muscle dysfunction. It is assumed that in the former the mitral regurgitation is already noticed in the acute stage, and in the latter it also appears in the acute stage secondary to myocarditis, or in the convalescence stage secondary to myocardial hypoxia resulting from coronary artery obstruction or myocardial infarction. Some histopathological studies of KD have revealed various degrees of valvulitis found 12-25 days after the onset of illness. However, valvulitis with verruca, calcification, or deformity is quite rare. Recently, Uemura et al. [2] reported on the first case of KD in which the patient died of mitral regurgitation" due to endocarditis with valvulitis; and Imakita et al. [1] gave histopathological details of valvar changes complicated by KD. However, the long-term prognosis for severely deformed valvar disease has not yet been fully elucidated. Our patient had severe mitral regurgitation secondary to recurrent KD without coronary arterial lesions. Kawasaki disease was first noted at 3 months and recurred again at 5 months of age. Mitral regurgitation was first noticed at the second episode of KD with the signs and symptoms of pancarditis. Three years later an invasive examination documented mild pulmonary hypertension, but mitral regurgitation per se has not been progressive since that time. Although mitral regurgitation in the acute stage secondary to valvulitis or myocarditis
Fig. 2. Two-dimensional echocardiographic picture of the deformed mitral valve: (A), systole (B), diastole. Fig. 3. Left ventriculography. Grade 3/4 mitral regurgitation.
Tomita et al.: Mitral Regurgitation in Kawasaki Disease
has been thought to have a relatively good prognosis, this case demonstrates that valvulitis secondary to KD can cause a gross deformity o f a mitral leaflet and severe mitral regurgitation. However, these changes are assumed not to be progressive beyond the period of the acute stage. This deformity cannot be differentiated from that of rheumatic or other acquired valvar diseases by 2DE alone: careful history-taking is mandatory.
155
References 1. Imakita M, Sasaki Y, Misugi K, Miyazawa Y, Hyodo Y (1984) Kawasaki disease complicated with mitral insufficiency: Autopsy findings with special reference to valvular lesion. Acta Pathol Jpn 34:605-616 2. Uemura S, Negoro S, Minami Y, Nakagawa K, Uehara T, Miyashiro E, Koike M, Ono T (1982) An autopsy case of MCLS complicated with the mitral insufficiency due to endocarditis [in Japanese]. JJPS 86:38-43
Pediatric Cardiology 9 Springer-VerlagNew York Inc. 1990
Case Reports Mitral R e g u r g i t a t i o n w i t h G r o s s D e f o r m i t y o f a Mitral L e a f l e t D u e to K a w a s a k i D i s e a s e Hideshi Tomita, 1 Yohko Sawada, ~ Yoshihito Higashidate, 1 Shunzo C h i b a , l Seiichi Ito, 2 and Ryoichi Kogasaka 2 ~Department of Pediatrics, Sapporo Medical College, Sapporo; and 2Department of Pediatrics, Sunagawa City Hospital, Sunagawa City, Japan
SUMMARY. A 4-year-old girl had two episodes of Kawasaki disease which resulted in severe mitral regurgitation. She had not had any coronary arterial lesion during the acute phase of the illness, nor any myocardial ischemia. Severe destruction of the mitral leaflet was documented by two-dimensional echocardiography. It is suggested that this lesion was due to severe carditis and valvulitis which developed during the acute phase of the illness. KEY WORDS: Kawasaki disease - - Mitrai regurgitation - - Vaivulitis
Case Report A 4-year-old girl had the first onset of Kawasaki disease (KD), displaying the six typical signs and symptoms, at 3 months of age. She was first admitted to Sunagawa City Hospital with a fever which persisted for 19 days. Two-dimensional echocardiography (2DE) revealed neither coronary arterial nor valvar lesions. She was treated with aspirin (30 mg/kg/day) and dipyridamol (4 mg/kg/day), and discharged 1 month later without any new signs or symptoms. She was readmitted to Sunagawa City Hospital at 5 months of age because of recurrent signs and symptoms. Chest roentgenogram and electrocardiogram (ECG) on admission suggested pancarditis, marked cardiomegaly on the chest X-ray [cardiothoracic ratio (CTR), 60%), abnormal Q in leads III and aVF, ST depression in leads III-VI, and flat T wave in the anterior chest leads (Fig. 1). She was again treated with aspirin (50 mg/ kg/day), dipyridamol (4 mg/kg/day), and digoxin (0.01 mg/kg/ day). The CTR increased to 66% after 10 days of hospitalization and hepatosplenomegaly appeared. At day 21 of hospitalization, a harsh pansystolic murmur (3/6) was audible and signs of congestive heart failure were greatly exaggerated. There was no coronary involvement, but the anterior and posterior leaflets of the mitral valve were thickened on 2DE when recorded on the day 24 of hospitalization. Also documented were a marked anterocentral mitral valve prolapse and a slight pericardial effusion. We diagnosed the apical heart murmur to be due to severe mitral
Address offprint requests to." Dr. Hideshi Tomita, Department of Pediatrics, Sapporo Medical College, South 1 West 16 Chuo-Ku, Sapporo, Japan.
regurgitation. She went into cardiogenic shock with hypotension on day 34 of hospitalization and was treated with continuous venous infusion of dopamine (5 ~g/kg/min). Signs and symptoms of congestive heart failure improved by day 36 of hospitalization. There was no elevation of serum glutamic-oxaloacetic transaminase, glutamic-pyruvic transaminase, lactic dehydrogenase, and creatinine phosphokinase during the acute phase. She is at present well-controlled on digoxin and diuretics. She was admitted to Sapporo Medical College, Department of Pediatrics, for further evaluation of mitral regurgitation. Her growth and development were good (weight 14 kg, height 92.3 cm), but she had slight tachydyspnea and she was in symptomatic state class 2 (NYHA). A grade 3 pansystoilc murmur and a low-pitched grade 2/6 middiastolic murmur were audible at the apex. Routine laboratory examination, however, revealed no specific abnormality. Cardiomegaly (CTR 64%) and mild pulmonary venous congestion were seen on the chest X-ray. Electrocardiogram documented marked left ventricular hypertrophy and a mitral P. The 2DE showed a marked dilatation of the left ventricle (left ventricular end-diastolic dimension 42 mm) and atrium, a slightly flattened interventricular septum, and a severely thickened and prolapsed anterior mitral leaflet (Fig. 2). No abnormalities, however, were observed in the coronary arteries and left ventricular wall motion (ejection fraction 0.73). Grade 4/4 mitral regurgitation was detected by real-time color flow-mapping. Cardiac catheterization data demonstrated a slightly elevated pulmonary capillary wedge pressure (mean = 10 mmHg) and mild pulmonary hypertension (41/14, mean = 26 mmHg). Her mitral regurgitation was diagnosed as grade 3 by left ventriculography (Fig. 3). There was no coronary involvement. Left ventricular end-diastolic volume index was 130.5 ml/m 2, ejection fraction was 0.64, and the regurgitant index was 0.76.
154
Pediatric Cardiology Vol. I I, No. 3, 1990
Discussion
Fig. 1. The ECG in the acute phase of the second episode of Kawasaki disease.
Mitral regurgitation complicated by KD is classified into two groups; one is mitral regurgitation, mainly caused by valvulitis; and the other is secondary to the papillary muscle dysfunction. It is assumed that in the former the mitral regurgitation is already noticed in the acute stage, and in the latter it also appears in the acute stage secondary to myocarditis, or in the convalescence stage secondary to myocardial hypoxia resulting from coronary artery obstruction or myocardial infarction. Some histopathological studies of KD have revealed various degrees of valvulitis found 12-25 days after the onset of illness. However, valvulitis with verruca, calcification, or deformity is quite rare. Recently, Uemura et al. [2] reported on the first case of KD in which the patient died of mitral regurgitation" due to endocarditis with valvulitis; and Imakita et al. [1] gave histopathological details of valvar changes complicated by KD. However, the long-term prognosis for severely deformed valvar disease has not yet been fully elucidated. Our patient had severe mitral regurgitation secondary to recurrent KD without coronary arterial lesions. Kawasaki disease was first noted at 3 months and recurred again at 5 months of age. Mitral regurgitation was first noticed at the second episode of KD with the signs and symptoms of pancarditis. Three years later an invasive examination documented mild pulmonary hypertension, but mitral regurgitation per se has not been progressive since that time. Although mitral regurgitation in the acute stage secondary to valvulitis or myocarditis
Fig. 2. Two-dimensional echocardiographic picture of the deformed mitral valve: (A), systole (B), diastole. Fig. 3. Left ventriculography. Grade 3/4 mitral regurgitation.
Tomita et al.: Mitral Regurgitation in Kawasaki Disease
has been thought to have a relatively good prognosis, this case demonstrates that valvulitis secondary to KD can cause a gross deformity o f a mitral leaflet and severe mitral regurgitation. However, these changes are assumed not to be progressive beyond the period of the acute stage. This deformity cannot be differentiated from that of rheumatic or other acquired valvar diseases by 2DE alone: careful history-taking is mandatory.
155
References 1. Imakita M, Sasaki Y, Misugi K, Miyazawa Y, Hyodo Y (1984) Kawasaki disease complicated with mitral insufficiency: Autopsy findings with special reference to valvular lesion. Acta Pathol Jpn 34:605-616 2. Uemura S, Negoro S, Minami Y, Nakagawa K, Uehara T, Miyashiro E, Koike M, Ono T (1982) An autopsy case of MCLS complicated with the mitral insufficiency due to endocarditis [in Japanese]. JJPS 86:38-43
