Masahiko Matsumura

Clinical implication of plasma natriuretic peptides in children with ventricular septal defect

Abstract Background : There is little information available concerning plasma concentrations of B‐type natriuretic peptide (BNP) in children with a ventricular septal defect. The aim of the present study was to determine hemodynamic factors that control plasma concentrations of BNP and the clinical implications of BNP compared with atrial natriuretic peptide (ANP) in children with ventricular septal defect.

Elevated Levels of High-Sensitivity C-Reactive Protein and Serum Amyloid-A Late After Kawasaki Disease: Association Between Inflammation and Late Coronary Sequelae in Kawasaki Disease

Background—Coronary sequelae that persist after Kawasaki disease (KD) have been associated with obstructive changes of the lesions and coronary vascular events in adolescents and young adults. However, little is known about the association between sequelae late after KD and inflammatory markers, which are potential mediators and markers for atherogenesis. Methods and Results—Cross-sectional study was performed to test the hypothesis that coronary sequelae are associated with elevated levels of inflammatory markers in patients late after KD (mean time interval after the onset, 10 years, 10 months). Levels of high-sensitivity C-reactive protein (CRP), serum amyloid-A (SAA), interleukin-6, and soluble intercellular adhesion molecule-1 were measured in the 4 groups (n=80): the referent group (n=15) and KD subgroups with normal coronary arteries from the onset (n=27); with regressed aneurysms (n=18); and with coronary artery lesions, such as persistent aneurysms, stenosis, and occlusion (n=20). CRP levels were significantly elevated in a KD subgroup with coronary artery lesions compared with the referent or other KD subgroups, as analyzed by ANOVA and ANCOVA after adjustment for a confounding factor body mass index. Levels of CRP, SAA, and interleukin-6 were positively correlated. Stepwise regression and logistic regression analyses support the association between the persistence of coronary artery lesions and the levels of CRP and SAA. Conclusions—Results demonstrate that the persistence of coronary lesions late after KD was independently associated with levels of CRP and SAA, suggesting that inflammation may be a novel functional aspect of coronary artery diseases late after KD.

Vascular smooth muscle cell phenotypes in primary pulmonary hypertension.

Primary pulmonary hypertension (PPH) is associated with specific structural alterations, including cellular intimal thickening, intimal fibrosis, and plexiform lesions. To determine the phenotypes of smooth muscle cells (SMCs) in such lesions, the authors conducted an immunohistochemical analysis of lung tissues from two patients with PPH, using two antimuscle actin antibodies, HHF35 and CGA7, and two anti-SMC myosin heavy chain markers, anti-SM1 and anti-SM2 antibodies and related antibodies. Cells that stained positive (+) with HHF35, CGA7, anti-SM1, and anti-SM2 were considered to be SMCs of a mature state. Conversely, those that stained positive with HHF35 and anti-SM1, but weakly positive (+/-) or negative (-) with CGA7 and anti-SM2, were considered to be SMCs exhibiting an immature state. Cellular intimal thickening was composed of SMCs of an immature phenotype (HHF35+, CGA7+/-, SM1+, SM2+/-), accompanied by the expression of fibronectin and the presence of macrophages; intimal fibrosis contained mature SMCs (HHF35+, CGA7+, SM1+, SM2+); and plexiform lesion consisted of proliferative endothelial cells (von Willebrand factor-positive cells, proliferating cell nuclear antigen-positive cells) and underlying immature SMCs (HHF35+, CGA7-, SM1+, SM2-) associated with fibronectin expression and macrophage infiltration. These findings suggest that smooth muscle cells with specific phenotypes may contribute to the development of specific vascular lesions in primary pulmonary hypertension.

Kawasaki disease complicated by cerebral infarction

An 8-month-old boy presented with right hemiplegia of sudden onset after 20 days of Kawasaki disease, which was not initially treated by gamma globulin. Cranial X-ray computed tomography confirmed cerebral infarction as the cause of the right hemiplegia. In subsequent weeks, he developed multiple thromboses in coronary aneurysms. He successfully underwent intracoronary thrombolysis using tissue plasminogen activator without haemorrhagic complications. Cerebral infarction as a complication of Kawasaki disease is rare, and is a difficult clinical situation to manage.

An MRI study of the myelination pattern in West syndrome

Using magnetic resonance imaging (MRI), we studied the myelination of the brains of 8 patients with West syndrome. All cases were symptomatic, 2 having severe asphyxia, 1 lissencephaly, 1 Leigh encephalopathy, 2 tuberous sclerosis, 1 multiple anomalies and 1 microcephaly. Myelination of the pons, cerebellum, thalamus, internal capsules, optic radiation, centrum semiovale and cerebral white matter was separately assessed. The 2 cases with tuberous sclerosis exhibited normal myelination patterns, although asymmetry of the cerebral white matter was noted. These cases had a moderate degree of mental retardation and persistent seizures. The other 6 cases exhibited a marked delay of myelination throughout the central nervous system except for the midbrain. These cases had severe psychomotor retardation and persistent seizures. Although the difference in the outcome may simply reflect the different etiological disorders, these results suggest that the myelination pattern is related to the psychomotor retardation but not to the severity of the seizures in West syndrome. Atrophy of the corpus callosum developed during ACTH therapy and disappeared after the therapy. It was thus suggested that the callosal atrophy caused by the ACTH therapy was reversible.

Early and late results of repair of tetralogy of Fallot with subarterial ventricular septal defect. A comparative evaluation of tetralogy with perimembranous ventricular septal defect.

Between November 1966 and December 1990, 511 pediatric patients with tetralogy of Fallot underwent corrective operation at Tenri Hospital. There were 78 patients with subarterial ventricular septal defect. Mean age at repair was 5.6 +/- 3.3 years. The method of right ventricular outflow tract reconstruction was simple infundibulectomy in 14 patients, right ventricular ventricular outflow patch in 36, and transannular patch in 28. There were 7 (9.0%) early deaths as a result of low cardiac output syndrome and acute renal failure. The pressure ratio of the right ventricle to the left ventricle was 0.62 +/- 0.18 during the early postoperative catheterization. Follow-up was achieved for 442.6 patient-years and ranged from 0.5 to 27 years, with an average of 8.5 +/- 6.7 years. There were three late deaths (2 cardiac and 1 noncardiac). Actuarial survival was 94.8% +/- 4.0% at 20 years. Catheterization during late follow-up (6.8 +/- 4.7 years after repair) was done in 53 patients and the pressure ratio of the right ventricle to the left ventricle was 0.48 +/- 0.21. Fifteen patients underwent subsequent operation because of residual lesions, including ventricular septal defect in four patients, pulmonary stenosis in nine, combined ventricular septal defect and pulmonary stenosis in one, and pulmonary regurgitation in one, with no mortality. Actuarial rate of freedom from reoperation was 71.1% +/- 8.0% at 10 years and 58.8% +/- 16.8% at 20 years. Patients with tetralogy and subarterial ventricular septal defect were more likely to have the development of residual obstruction at the level of the pulmonary valve anulus after repair than were those with tetralogy and perimembranous ventricular septal defect.

Transposition of the great arteries associated with total anomalous pulmonary venous return

A case of a 1-year-old boy with combined transposition of the great arteries and total anomalous pulmonary venous return is reported. The anomalies were successfully repaired by a new atrial partitioning technique.

Prenatally Diagnosed Female Prune Belly Syndrome Associated with Tetralogy of Fallot

Female prune belly syndrome and tetralogy of Fallot were diagnosed in obstetric ultrasound screening at 13 and 28 weeks of gestation, respectively. The baby was born at 38 weeks showing abdominal distension, hypoplasia of abdominal muscles, vaginal atresia, imperforate anus and clubfeet. Radiologic and ultrasound examinations confirmed our prenatal diagnosis and revealed other abnormalities of patent ductus arteriosus, major aortopulmonary collateral arteries, bilateral vesicoureteral reflux, rectourethral fistula and hypoplasia of the right kidney. Urination has been smooth, but cystitis was observed repeatedly once a month. The infant was discharged in good condition and has been followed in the outpatient clinic.

Myotonic dystrophy presenting as atrial flutter in childhood

A 12-year-old boy presented with tachycardia and cardiomegaly. An electrocardiogram showed atrial flutter with a ventricular rate of 170 beats/min. The echocardiogram showed left ventricular dilation, with decreased contractility. Electromyography confirmed the diagnosis of myotonic dystrophy. The atrial flutter was converted to sinus rhythm by electroversion, leading to restoration of normal cardiac function. Our case illustrates that atrial flutter can be an early sign of the cardiac complications of myotonic dystrophy in childhood.

Shunt obstruction after an air travel in an adult patient with cyanotic congenital heart disease.

was that she had to wait an extra 2 h in the airplane before landing because of unfavorable weather around the airport. She was well aware of the ‘economy class syndrome’ [2] and remembered that she had tried to drink more water than usual to avoid dehydration and had exercised in the cabin. Her laboratory data on this visit showed only mild polycythemia, a red blood cell count of 634 ! 10 4 / l and a hematocrit of 44%. She was brought to the catheterization laboratory and underwent diagnostic and interventional catheterization that revealed a discrete thrombus in the middle of the shunt ( fi g. 1 ) with a decreased pulmonary to systemic blood fl ow ratio of 0.77: 1.0. Balloon dilation of the shunt in combination with topical infusion of recombinant tissue plasminogen activator failed to open up this shunt. Therefore, she underwent successful placement of an additional modifi ed Blalock-Taussig shunt that increased her oxygen saturation to 92%. This case illustrates that air travel may result in signifi cant shunt obstruction in an adult patient with cyanotic congenital heart disease. Although we do not have the pathological specimen of the shunt, we think this is a thrombotic obstruction rather than an intimal growth because of the fast time course of shunt obstruction. In addition to air travel itself, the tropical weather in Bali might also have been involved in the formation of this thrombus. In the management of cyanotic adult patients, we need to remember that air travel may result in thrombotic complications. Increasing numbers of adult patients with cyanotic congenital heart disease have enjoyed air travel with relatively low risk. It has been shown that there is little risk of a hazardous decrease in oxygen saturation in cyanotic patients during commercial air travel [1] . Recently, however, we have treated an adult patient with cyanotic congenital heart disease who developed shunt obstruction and progression of cyanosis after air travel. A 34-year-old lady, who has tricuspid stenosis with hypoplastic right ventricle and atrial septal defect, was referred to us by a local physician because of severe cyanosis with 40% oxygen saturation measured by pulse oximeter. She had had a left aortopulmonary shunt operation on two occasions. Since the last operation 12 years ago using an 8-mm prosthetic tube, she has been on digoxin, furosemide, allopurinol, and aspirin, and her oxygen saturation ranged from 82 to 84% with stage II life activity of the New York Heart Association classifi cation. Before this visit she had traveled to Bali on a fl ight taking 6 h and spent 6 days there. After she had come back home, she started to fatigue easily and visited her local physician. There she was noted to have progression of cyanosis and a smaller continuous murmur with a grade 2/6 at the right sternal border; the murmur had been grade 3/6 on a prior visit. This air travel was her fourth air travel abroad. She traveled to Hawaii twice on fl ights taking 8 h and to Singapore once on a fl ight taking 6 h. The only unusual thing that happened on this travel Received: June 7, 2004 Accepted: June 13, 2004 Published online: January 19, 2005