Assessment of electromyocardial continuity in conjoined (thoracopagus) twins.
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Biomedical subjects
Publications and source records attributed to H C Lue.
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A 4-month-old infant with cardiac hemangioendothelioma presented with thrombocytopenia, and pericardial effusion, as well as signs and symptoms of heart failure. This is the first reported case of infantile cardiac hemangioma successfully treated with steroids.
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Balloon pulmonary valvuloplasty (BPV) is a well accepted treatment for moderate and severe pulmonary stenosis. However, the efficacy of BPV in treating mild pulmonary stenosis is still unanswered. Therefore, the efficacy of BPV in treating patients with mild pulmonary stenosis was compared to that in treating patients with moderate or severe pulmonary stenosis. A total of 46 patients with pulmonary stenosis were arbitrarily divided into 3 groups; Group I consisted of 9 patients with a pressure gradient less than 40 mm Hg, Group II consisted of 5 patients with a pressure gradient ranged from 40 to 50 mm Hg, and Group III consisted of 32 patients with a gradient greater than 50 mm Hg. Following BPV, the gradient reduced significantly in all 3 groups (p less than 0.05 in each group). The efficacy of BPV in the 3 groups was evaluated and compared by two parameters; one is the percentage of gradient reduction, the other is the right ventricular pressure ratio (RV post-bpv/RV pre-bpv). The percentage of gradient reduction in Group I, II, and III were 28 +/- 20, 55 +/- 17 and 70 +/- 9% respectively. The right ventricular pressure remained 84 +/- 11, 59 +/- 16, and 46 +/- 12% of pre-valvuloplasty level in Group I, II and III respectively. If the efficacy of BPV was compared among the 3 groups. Groups III had the best efficacy and Group I had the worst.(ABSTRACT TRUNCATED AT 250 WORDS)
Junctional ectopic tachycardia (JET) in infancy is one of the serious arrhythmias which can be fatal. Typical features of JET include rapid and irregular heart beats with atrioventricular dissociation. Two cases of JET are reported: Case 1 was a 35-week-gestational age newborn who was found to have hydropsy and fetal tachycardia at the 21st week of gestational age. Antiarrhythmic agents including digoxin, propranolol and verapamil were administered to his mother to treat the fetal arrhythmia without success. JET was recognized at birth which was spontaneously converted into a sinus rhythm at 1 month of age. The maternal history revealed that two previous pregnancies ended in hydrops fetalis, and one of these was documented to have fetal tachycardia. Case 2 was a 6-month-old male infant with JET and congestive heart failure. After failure of various antiarrhythmic agents, amiodarone finally slowed down his heart rate and controlled his congestive heart failure.
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In order to examine the chronologic changes in genetic variance and heritability of anthropometric characteristics of Chinese infants in Taiwan, a total of 521 pairs of same-sexed twin neonates given birth in four major general teaching hospitals in Taipei City were studied. Based on the placental pattern and 12 red blood cell antigens, 428 MZ and 93 DZ twin pairs were identified and followed up to the age of one year. There was no significant genetic variance for all anthropometric characteristics adjusted for sex and gestational week before the age of six months. After adjusting for sex and gestational week, a significant genetic variance was observed at the age of six months, with heritability values of 0.51 (weight), 0.63 (head circumference), 0.77 (chest circumference), and 0.53 (arm circumference), as well as at one year, although with considerably lower heritability values. This implies that growth is dynamically determined by both genetic and environmental factors during infancy.
In order to examine the genetic variance and heritability of temperament among Chinese infants in Taiwan, a total of 62 pairs of same-sexed twin infants given birth in four major general teaching hospitals in Taipei City were studied. Based on placentation and 12 red blood cell antigens, 44 MZ and 18 DZ pairs were identified. Temperament was assessed at the age of six months by the Chinese edition of Carey's Temperament scale. Significant genetic variance was observed for activity level, approach or withdrawal, intensity of reaction, quality of mood, and threshold of responsiveness, with a heritability of 0.64, 0.56, 0.74, 0.39, and 0.45, respectively. There was no significant intrapair difference in temperamental characteristics between monochorionic and dichorionic MZ twins.
In order to examine the chronological changes in genetic variance and heritability of arterial systolic and diastolic blood pressure (SBP and DBP of Chinese infants in Taiwan, a total of 339 same-sexed twin neonates born in four major general teaching hospitals in Taipei City were studied. Based on placentation and 12 red blood cell antigens, 274 monozygotic (MZ) and 65 dizygotic (DZ) twin pairs were identified and followed up to the age of one year. Both SBP and DBP were measured by Doppler blood pressure monitor. Within-pair mean squares of SBP and DBP were consistently smaller in MZ than DZ twins at ages one month and over. The findings remained unchanged after the adjustment for the effects of age, sex, gestational age, placentation and physical state during blood pressure measurement. Falconer's heritability indices for adjusted SBP and DBP at ages two months and over ranged from 0.29 to 0.55 and from 0.27 to 0.45, respectively. The study indicates an important genetic influence on blood pressure during infancy.
In order to examine the genetic variance and heritability of serum total cholesterol, high-density lipoprotein cholesterol (HDL-C), low-density lipoprotein cholesterol (LDL-C) and triglycerides, a total of 349 pairs of same-sexed twin neonates born in four major general teaching hospitals in Taipei City were studied. Based on the placental pattern and 12 red blood cell antigens, 271 monozygotic (MZ) and 78 dizygotic (DZ) twin pairs were identified. There was a significant genetic variance for total cholesterol, HDL-C, LDL-C, and triglycerides both unadjusted and adjusted for sex, gestational age and placentation. The unadjusted heritability of total cholesterol, HDL-C, LDL-C, and triglycerides was 0.59, 0.30, 0.25 and 0.75, respectively; while the corresponding adjusted heritability was 0.74, 0.38, 0.31, and 0.49, respectively. Intrapair variance of serum lipids was not significantly different between monochorionic and dichorionic MZ twins.
The natural history of ventricular septal defect (VSD) varies with the exact location of the defect and also with the tissue changes surrounding it. Seven cases of perimembranous trabecular VSD were noted during color Doppler echocardiographic examination to have left ventricular-to-right atrial (LV-to-RA) shunts in association with aneurysmal transformation of VSD. Repeated cardiac catheterization documented the role of aneurysmal transformation in the increase of LV-to-RA shunts. A VSD located near the antero-septal commissure was diagnosed in all cases by echocardiography from the apical 5-chamber and parasternal inflow views. Echocardiographic signs of LV-to-RA shunts are high velocity backward turbulence in the RA without the presence of an elevated right ventricular systolic pressure and a two-directional turbulence through the "transformed" VSD. Corrective surgery confirmed the diagnosis in one case. Another case was complicated by an episode of viridans streptococcal endocarditis. The significance of LV-to-RA shunts in perimembranous trabecular VSD remains unknown.
The choice of a valved conduit or a valve in the right ventricular outflow tract remains a problem. We propose to reconstruct the pulmonary valve inside the patient's own pulmonary trunk by autologous pericardium in tri-semilunar fashion with concomitant formation of the sinus of Valsalva and commissure. This operation was carried out on a 7-year-old girl suffering from Fallot's tetralogy with an absent pulmonary valve. The ventricular septal defect was repaired and the pulmonary valve reconstructed as above. The postoperative cross-sectional echocardiogram, color Doppler echocardiogram and angiogram showed a pliable tri-semilunar valve across the pulmonary outflow tract without regurgitation. It is concluded that firstly, the sinus of Valsalva is indispensable for a stentless semilunar valve to be competent; secondly, reconstruction of the semilunar valve inside the pulmonary trunk by autologous pericardium is recommended for the absent pulmonary valve syndrome, but the long-term fate of this pericardial valve awaits further evaluation.
Studies published in the past 10 years suggest that group A streptococcal infections are frequent in the Orient and lead to a high incidence of rheumatic fever (RF) and rheumatic heart disease (RHD). In the present study, streptococcal infections were found to be more prevalent in Japan and Taiwan, whereas RF and RHD were more common and severe in the Philippines, Thailand, and Indonesia, particularly among the socioeconomically less privileged populations. The pattern of childhood RF varied: Carditis was the most common manifestation, occurring in 57% to 94% of the patients; polyarthritis was generally atypical and less common in the tropics; chorea minor and erythema marginatum were much more common in Japan, less common in Taiwan and rare in the tropics. RF recurrences were quite common and led to the development of new carditis, and deterioration or persistence of the pre-existing heart disease. The 5 year mortality rates differed greatly, ranging from zero to 42%. There was disappearance of the heart murmur in 16.5% to 37.5% of patients. Such apparent recovery was related to adherence to chemoprophylaxis. The major risk factors adversely affecting survival were the severity of carditis, inadequacy of medical service, non-compliance to chemoprophylaxis, RF recurrence, poor socioeconomic status, and high prevalence of group A streptococci. It is concluded that there is no uniform "Oriental-type" of natural history of RF and RHD. The natural history varies greatly among countries as is true in other parts of the world.
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