Hirohiko Shiraishi

Prenatal MRI in a fetus with a giant neck hemangioma: a case report

We report a fetus with a giant neck hemangioma which was examined by MRI in utero. The initial diagnosis was made by ultrasonography. The sonolucent aspect of the mass, together with the presence of pulsating Doppler flow signals, was highly suggestive of a fetal hemangioma. In late pregnancy, fetal MRI revealed the location, size and characteristics of the neck tumor. Following prenatal corticosteroid treatment and premature delivery of the pregnancy due to fetal cardiac failure, the newborn received angiography and coil embolization of the tumor vessels. Despite vigorous treatments, the newborn died 12 h after birth. Evaluation of a fetal neck hemangioma by MRI is recommended late in pregnancy for precise information on the tumor and adjacent organs since the image is valuable for planning optimal perinatal treatment. Copyright

Pulsed Doppler echocardiographic evaluation of neonatal circulatory changes.

Pulsed Doppler echocardiograms were obtained from 42 normal fullterm neonates at less than 12 hours (20 subjects), 4 days (20 subjects), and 33 days (12 subjects). The acceleration time of the flow velocity and ventricular systolic time intervals were measured on recordings obtained at the right and left ventricular outflow tract, and the patency of the ductus arteriosus was evaluated by the flow at the pulmonary end of the ductus. The flow velocity pattern of the right ventricular outflow tract changed from a triangular shape with a peak velocity in early systole in the younger age groups to a dome-like contour with a peak velocity in mid-systole; thus the ratio of mean acceleration time to right ventricular ejection time increased with age. The flow velocity pattern of the left ventricular outflow tract was triangular in all age groups, and the ratio of mean acceleration time to left ventricular ejection time showed no significant change with age. The right ventricular pre-ejection period shortened and the right ventricular ejection time lengthened with age; thus the ratio of mean right ventricular pre-ejection period to right ventricular ejection time decreased with age. The left ventricular systolic time intervals showed no significant change with age. The ductus arteriosus was patent in all subjects who were less than 12 hours old but was closed in the older neonates. Pulsed Doppler echocardiography is a valuable method of evaluating pulmonary vascular bed in the early neonatal period.

Inhalation of nebulized nitroglycerin in dogs with experimental pulmonary hypertension induced by U46619

Abstract Background: Pulmonary hypertension (PH) causes mortality in some congenital and acquired heart and lung diseases. However, inhalation of NO gas requires complicated and expensive instruments and elaborate preparations to avoid toxic gas administration. We tested the effectiveness and safety of inhaled nebulized nitroglycerin (Neb‐NTG) in dogs with experimental PH.

Hypertrophic cardiomyopathy in congenital myotonic dystrophy

Involvement of the cardiac conduction system is a common clinical feature in myotonic dystrophy, whereas the association of primary myocardial abnormalities has rarely been reported. A patient with a severe form of congenital myotonic dystrophy who developed fatal left ventricular hypertrophy at 3 months of age and died at 2 years of age is reported. Serial ultrasonographic studies revealed progressive left ventricular hypertrophy accompanied by outflow obstruction of the left ventricle. Southern analysis for the myotonin kinase gene revealed a 5.8 kb expansion of CTG repeats in addition to a fragment of normal length. The degree of expansion was much greater than those of other reported patients with congenital myotonic dystrophy. These findings suggest that left ventricular hypertrophy represents an extreme level of myocardial damage in myotonic dystrophy and that this damage may be related to the larger size of the CTG repeats.

Cerebral and Abdominal Arterial Hemodynamics in Preterm Infants with Patent Ductus Arteriosus

Using Doppler echocardiography we evaluated the effect of ductal shunt flow on the cerebral and abdominal arterial blood flow in 25 preterm infants. Eligible for inclusion in this study were healthy preterm newborn infants. They were divided into two groups based on their gestational age: group A, 33‐36 weeks (15 infants) and group B, 28–32 weeks (10 infants). Two‐dimensional Doppler echocardiograms were obtained in each infant during the first 8 hours of life and repeated every 6–12 hours until no ductal shunt flow could be detected. Flow in the ductus arteriosus, the basilar artery and the coeliac artery were examined. Closure of the ductus arteriosus occurred significantly later(p< 0.05) in group B than in group A. Pulsatility indices of flow in the basilar and coeliac arteries were high when the ductus was patent, decreasing to a fixed level with closure. This study suggests that a shunt of the patent ductus arteriosus (PDA) adversely influences the cerebral and abdominal blood flow in preterm infants.

Coronary aneurysms in Kawasaki disease: Follow-up observation by two-dimensional echocardiography

SummaryThe development and regression of the coronary aneurysms in Kawasaki disease was studied with serial two-dimensional echocardiographic (2D echo) examinations. The diameter of the aneurysms at the proximal portions of the left coronary artery was measured on the 2D echo images in ten patients with Kawasaki disease, in whom left coronary aneurysms were found at the acute stage of the illness, and followed by 2D echo for longer than eight months. It was found that coronary aneurysms usually developed during the second week of the illness, reached maximal size at 3–8 weeks, and regressed gradually thereafter. Small aneurysms disappeared in several months, and those of intermediate size regressed in one to two years. Large aneurysms may remain for many years. Mural thrombi within the aneurysms were detected with 2D echo in three patients. They decreased in echodensity and eventually disappeared echographically.

Grown-up Kawasaki disease patients who have giant coronary aneurysms

BackgroundMany Kawasaki disease (KD) patients have reached adulthood in Japan. The current status of adult patients who have giant coronary aneurysms with KD is not well understood.MethodsMedical records of 48 KD patients (33 males and 15 females) with giant coronary aneurysms (maximum coronary artery internal diameter >8 mm) aged 20 years or over were retrospectively reviewed.ResultsThe age of the patients at the diagnosis of KD ranged from 0.3 to 12.8 years (median 2.9 years) and the age of the patients in this review ranged from 20.0 to 33.1 years (median 25.2 years). During the follow-up period, the maximum coronary artery internal diameter ranged from 8.2 to 30.0 mm (median 10 mm). Giant coronary aneurysms progressed to coronary artery stenosis and/or complete occlusion in 34 (74%) of 46 patients. Coronary artery bypass graft surgery was performed in 9 (19%) of 48 patients. Myocardial infarction occurred in 14 (31%) of 45 patients. Other complications or problems occurred in 5 patients with angina pectoris, low left ventricular ejection fraction, ventricular tachycardia, hemorrhagic cerebral infarction, or thyroid carcinoma respectively. In the patients followed up, 4 dropped out. In addition, 1 patient succeeded in pregnancy and delivered a baby.ConclusionsClose attention should be paid to ventricular tachycardia in adult KD patients with giant coronary aneurysms, especially for those who have low left ventricular ejection fraction. To reduce the number of dropped out patients, it is important that the patients should be referred to a new doctor when they change their place of residence.

Increased serum granulocyte colony-stimulating factor correlates with coronary artery dilatation in Kawasaki disease.

Abstract. In acute-phase Kawasaki disease, neutrophils cause injury to the coronary arterial endothelium through the production of elastase. Previous research has demonstrated the modulation of neutrophil function and kinetics, such as development and maturation, by granulocyte colony-stimulating factor (G-CSF). To examine the correlation between G-CSF and cardiac complications in Kawasaki disease, functional activity of serum G-CSF and cytokines was measured by enzyme-linked immunosorbent assay in 30 patients with acute-phase Kawasaki disease aged 2 months to 5 years. The mean serum G-CSF was higher in the 1st week of Kawasaki disease than during weeks 2 to 4, and G-CSF was significantly higher in patients with coronary artery dilatation (CAL) than in those without. There was no significant difference in the activity of other cytokines studied or white blood cell counts between the patients with CAL. Conclusion: granulocyte colony-stimulating factor is correlated with coronary artery dilatation in acute-phase Kawasaki disease and increased neutrophil function may contribute to the pathogenesis of coronary arterial endothelial injury in these patients.

Criss-cross heart evaluated by colour Doppler echocardiography and magnetic resonance imaging

Two-dimensional colour Doppler echocardiography was performed on a 1-month-old male infant with criss-cross heart, double outlet right ventricle, ventricular septum defect and pulmonary stenosis. Complex structural abnormalities were suspected after two-dimensional echocardiography (2-D echo) and confirmed by colour Doppler and magnetic resonance imaging (MRI). We stress that the blood streams in the ventricular inflow tracts revealed by colour Doppler and the spatial relationships of the cardiac segments disclosed by MRI are essential to make an accurate non-invasive diagnosis of this complex malformation.

Ventricular Tachycardia in a Neonate with Prenatally Diagnosed Cardiac Tumors: A Case with Tuberous Sclerosis

We report a patient with prenatally diagnosed tuberous sclerosis. Fetal ultrasonography demostrated multiple cardiac tumors and arrhythmia. After birth, because of frequent supraventricular extrasystoles, the infant was admitted to the neonatal intensive care unit. Findings on 24-hour ambulatory electrocardiogram (ECG) showed frequent supraventricular tachycardia and ventricular tachycardia with four beats as the longest run. At the age of 12 days, he developed cardiopulmonary arrest after crying out. A monitored ECG showed ventricular tachycardia. Twenty minutes after onset, a 12-lead ECG showed ventricular fibrillation, which returned to normal sinus rhythm with repeated DC cardioversion. Oral antiarrhythmic therapy with carteolol hydrochloride was effective. The patient showed no further symptoms after oral medication was initiated and the tumors regressed spontaneously.