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Biomedical subjects

Sheng-Ling Jan

Publications and source records attributed to Sheng-Ling Jan.

At least 19 recordsLinked to original sources

Initial clinical experience of real-time three-dimensional echocardiography in neonates with isolated congenital ductus arteriosus aneurysm.

BACKGROUND: Congenital ductus arteriosus aneurysm (DAA) was considered rare but potentially fatal abnormality, often followed by surgical intervention after careful evaluation. This prospective study used real-time three-dimensional echocardiography (RT3DE) to assist in evaluation of neonatal DAA. METHODS: A total of 1390 full-term neonates were enrolled in this study between 2002 and 2003. They received two-dimensional echocardiographic (2DE) screening and periodic follow-up. RT3DE was performed selectively for newborns with DAA. RESULTS: DAA were detected in 116 (8.34%) newborns using 2DE. Maximum diameter of the DAAs ranged from 6.8 to 14.0 mm (8.2+/-1.1 mm). None of the cases were symptomatic or had complications related to DAA. There were no significant differences in sex and gestational age between the newborns with and without DAA. Neonates with DAA had a higher birth body weight and a higher incidence of large-for-gestational-age (P<0.05). RT3DE provided instant, consistent and reliable 3D images of DAA and its related structures and allowed for more rapid examination times and reduction of baby wait times. CONCLUSIONS: Congenital DAA is as common as has been previously reported. RT3DE is useful in assisting evaluation of DAA. Preferred images of DAA were typically visualized in the high parasternal short-axis view before the third day of life. Routine use of RT3DE is suggested to enhance assessment of neonates with DAA detected by 2DE.

Ductus Arteriosus↗

A rare vascular ring caused by an aberrant right subclavian artery concomitant with the common carotid trunk: a report of two cases.

In this report, we describe two rare cases with aortic branching anomalies. One was a 7-year-old girl who had an incomplete vascular ring caused by the presence of left aortic arch and aberrant, retroesophageal right subclavian artery associated with the common carotid trunk. The other was a 6-year-old boy who had a right aortic arch and mirror image branches of the former case. Both were asymptomatic and discovered incidentally. Three-dimensional surface rendering magnetic resonance angiography was used to confirm the diagnosis. Surgical intervention was not performed in either case because of lack of vascular ring-related symptoms. The incidence, clinical symptoms, pathogenesis and management of this rare vascular ring are discussed below.

Aortic Arch Syndromes↗

Congenital chylothorax associated with isolated congenital hypoplastic superior caval vein: a case report.

UNLABELLED: Congenital absence, or hypoplasia, of the superior vena cava (SVC) with situs solitus is quite uncommon. Most cases have been found incidentally and concomitant with left persistent SVC or other cardiac disorders. Congenital chylothorax may be associated with cardiac anomalies, various syndromes, thoracic ductal or venous thrombosis, birth trauma or local compression of the thoracic duct, but it is rarely caused directly by congenital SVC anomalies. CONCLUSION: We report a rare case of congenital chylothorax associated with isolated congenital hypoplastic SVC and underdeveloped collateral circulation.

Abnormalities, Multiple↗

Prediction of ductus arteriosus closure by neonatal screening echocardiography.

AIMS: We reported the incidence, clinical manifestations and outcome of patent ductus arteriosus (PDA) in full-term infants by screening echocardiography. METHODS AND RESULTS: Total 1230 infants received echocardiographic screening and periodic follow-up. On the third day of life, there were 109 infants with isolated ductus arteriosus aneurysm (DAA), those with persistent patency of the ductus arteriosus (DA) in 26. The DA closed within 48 h in 828 infants, and persisted beyond 48 h in 180. The incidence of PDA in full-term infants was 0.91, 0.83 and 0.66% at 1, 4 and 6 months follow-up, and 0.82, 0.73 and 0.64% if infants with DAA were excluded. Infants with PDA had lower birth body weight (BBW) than those with closed DA (p = 0.02). The DA width on the third day of life ranged from 0.23 to 0.43 cm (0.32+/-0.08 cm) and 0.08 to 0.44 cm (0.16+/-0.06 cm) in infants with or without PDA, respectively, which was significantly different (p < 0.05). The sensitivity, specificity, predictive positive and negative values were 87.5, 92.5, 36.8 and 99% respectively for the cuff-off point at 0.25 cm of DA width. CONCLUSIONS: Risk factors in infants with PDA included lower BBW and larger measured DA width at the third day of life. We predicted the arterial duct would close if DA width <0.25 cm, and suggested follow up earlier if >0.25 cm on the third day of life.

Cardiac Surgical Procedures↗

Prediction of ductus arteriosus closure by neonatal screening echocardiography.

AIMS: We reported the incidence, clinical manifestations and outcome of patent ductus arteriosus (PDA) in full-term infants by screening echocardiography. METHODS AND RESULTS: Total 1230 infants received echocardiographic screening and periodic follow-up. On the third day of life, there were 109 infants with isolated ductus arteriosus aneurysm (DAA), those with persistent patency of the ductus arteriosus (DA) in 26. The DA closed within 48 h in 828 infants, and persisted beyond 48 h in 180. The incidence of PDA in full-term infants was 0.91, 0.83 and 0.66% at 1, 4 and 6 months follow-up, and 0.82, 0.73 and 0.64% if infants with DAA were excluded. Infants with PDA had lower birth body weight (BBW) than those with closed DA (p = 0.02). The DA width on the third day of life ranged from 0.23 to 0.43 cm (0.32 +/- 0.08 cm) and 0.08 to 0.44 cm (0.16 +/- 0.06 cm) in infants with or without PDA, respectively, which was significantly different (p < 0.05). The sensitivity, specificity, predictive positive and negative values were 87.5, 92.5, 36.8 and 99% respectively for the cuff-off point at 0.25 cm of DA width. CONCLUSIONS: Risk factors in infants with PDA included lower BBW and larger measured DA width at the third day of life. We predicted the arterial duct would close if DA width <0.25 cm, and suggested follow up earlier if >0.25 cm on the third day of life.

Aneurysm↗

Pulmonary edema of enterovirus 71 encephalomyelitis is associated with left ventricular failure: implications for treatment.

Epidemics of enterovirus 71 infections caused the rapid death of many children in Malaysia in 1997 and in Taiwan in 1998. Pulmonary edema occurred in most of the fatal cases and was considered to be neurogenic. The role of the heart was rarely investigated before. Between January 1998-January 2001, 34 consecutive patients who were admitted to the intensive care unit due to enterovirus infection were studied prospectively. Patients were divided into two groups: group I with pulmonary edema, and group II without pulmonary edema. Comparisons were made between the two groups based upon demographic, neurological, and cardiovascular manifestations. Group I consisted of 11 patients (5 boys, 6 girls; mean age, 22.8 months), and group II of 23 patients (12 boys, 11 girls; mean age, 28.8 months). There were no significant differences between the two groups in comparing sex, age, body weight, neurological severity, intracranial pressure, cell count, protein and glucose levels in cerebral spinal fluid, and blood pressure. All group I patients had left ventricular dysfunction, and their ejection fractions were significantly lower than those of patients in group II (37 +/- 11% vs. 75 +/- 6%, P < 0.001). Group I heart rates were higher than those of group II (175 +/- 24 vs. 137 +/- 25, P < 0.001). In group I, 9 patients who received conventional treatment died, and the only two survivors received left ventricular assist devices. In conclusion, the pulmonary edema of fulminant enterovirus 71 infection is associated with left ventricular failure. Left ventricular function is the major determinant of outcome. Early recognition of heart failure and aggressive cardiac intervention are life-saving. Pediatr Pulmonol. 2003; 35:263-268.

Child, Preschool↗

Influence of ductal size on the results of transcatheter closure of patent ductus arteriosus with coils.

To assess the influence of ductal size on the results of transcatheter closure of patent ductus arteriosus (PDA) with coils, 154 consecutive patients were studied prospectively. Ductal size was defined as the narrowest diameter of ductus measured on aortography. All patients were divided into 5 groups according to ductal size: < 1 mm, 1-1.9 mm, 2-2.9 mm, 3-3.9 mm, and > or = 4 mm. The occlusion of PDA with coils was performed through a transarterial approach. The results were evaluated by angiography at 10 minutes and by color Doppler echocardiography at 1 day, 2 days, 1 week, 1 month, 3 months, 6 months, and 12 months after the procedure. The immediate occlusion rates for ductal sizes < 1 mm, 1-1.9 mm, 2-2.9 mm, 3-3.9 mm, and > or = 4 mm were 89.7%. 75.4%, 51.4%, 30.8%, and 40%, respectively; whereas the occlusion rates at 12-months follow-up were 100%, 98.5%, 97.3%, 69.2%, and 80%, respectively. There were no significant differences in occlusion rate at 12-months follow-up among the groups with ductal sizes < 3 mm or among the groups with ductal sizes > or = 3 mm. The occlusion rate for ductal size < 3 mm at each follow-up time was significantly higher than that for ductal size > or = 3 mm (10 minutes: 71.8% vs 34.8%. P = 0.001; 12-months: 98.5% vs 73.9%, P < 0.001). The occlusion rate of residual shuntings at 12-months follow-up for ductal size < 3 mm was also significantly higher than that for ductal size > or = 3 mm (94.6% vs 60%, P = 0.007). The results of the present study demonstrate that ductal size < 3 mm had a higher occlusion rate than that for a size > or = 3 mm. PDA with a size > or = 3 mm may need other treatment strategies or other devices to achieve better results.

Cardiac Catheterization↗

Verapamil sensitive idiopathic ventricular tachycardia in an infant.

Idiopathic ventricular tachycardia is rare, especially in infants. We report here on an 8 month-old female infant who presented with tachycardia with a heart rate of 186 beats/ min. An electrocardiogram showed a right bundle branch block pattern, a QRS duration of 80 msec, a superior QRS axis, atrioventricular dissociation, and occasional fusion and capture beats. Suspected ventricular tachycardia was treated with lidocaine, propranolol and amiodarone, but in vain. The tachycardia was terminated and well controlled with the use of verapamil. According to an electrocardiogram and her clinical response, verapamil-sensitive idiopathic ventricular tachycardia was diagnosed with the arrhythmic origin in the left posterior fascicle.

Amiodarone↗

Left ventricular dysfunction in children with fulminant enterovirus 71 infection: an evaluation of the clinical course.

We describe 2 children with typical hand, foot, and mouth disease due to enterovirus 71 infection, 1 of whom died. Both cases were complicated by acute fulminant shock syndrome; the patients had remarkable acute left ventricular dysfunction. The clinical experience indicates that the rapid death associated with fulminant enterovirus rhombencephalitis is the result of rapid cardiogenic shock rather than neurogenic pulmonary edema.

Child, Preschool↗

Isolated neonatal ductus arteriosus aneurysm.

OBJECTIVE: A prospective study was performed to evaluate the incidence, clinical manifestations and outcome of ductus arteriosus aneurysm (DAA) in full-term neonates. BACKGROUND: Ductus arteriosus aneurysm has been considered to be a rare congenital lesion and a potentially fatal abnormality. METHODS: A total of 548 full-term neonates received echocardiographic screening. RESULTS: There were 48 (8.8%) patients (28 boys and 20 girls) with DAA detected by echocardiography. The maximal diameter of the DAA ranged from 6.5 to 11.2 mm (8.2 +/- 1.2 mm). All cases were asymptomatic. There were no significant differences in gender, gestational age, maternal age or Apgar score between the newborns with or without DAA. Newborns with DAA had a higher birth body weight, higher incidence of maternal gestational diabetes mellitus and more mothers with blood group A, compared with newborns without DAA (p < 0.05). Follow-up echocardiograms showed spontaneous closure of the ductus arteriosus in all patients except those without DAA. The DAA became progressively smaller after ductal closure in 33 patients (70.2%) and completely disappeared by 7 to 35 days of life. The other 14 patients (29.8%) with DAA had echocardiographic evidence of progressive formation of thrombi between the third and tenth day of life. The DAA and thrombi spontaneously disappeared in all patients by one month after birth. CONCLUSIONS: There is a higher incidence of DAA with a good outcome in our series compared with previous reports. We speculate that the presence of DAA may be a normal variant of the ductal bump and part of a normal process of spontaneous ductal closure in full-term neonates.

Aneurysm↗

Discordance between dipyridamole stress Tc-99m sestamibi SPECT and coronary angiography in patients with Kawasaki disease.

BACKGROUND: Kawasaki disease is an acute vasculitis syndrome of unknown etiology that mainly affects the coronary arteries. The purpose of this study was to assess the agreement between dipyridamole stress technetium 99m sestamibi single photon emission computed tomography (SPECT) and coronary angiography in these patients. METHODS AND RESULTS: Forty-one consecutive patients (29 boys and 12 girls) who underwent coronary angiography were studied prospectively. Their ages at onset of the disease ranged from 2 months to 4.8 years (mean +/- SD, 1.9 +/- 1.3 years). Their ages at the time of the study ranged from 8 months to 15.3 years (6.2 +/- 4.4 years). The duration between symptom onset and the study ranged from 2 months to 12 years (4.3 +/- 4.0 years). All patients underwent dipyridamole stress Tc-99m sestamibi SPECT within 1 month of their angiographic studies. They were divided into 3 groups according to coronary angiography findings. Group A consisted of 2 patients (1 boy and 1 girl, aged 10.3 and 1.9 years, respectively) with coronary stenoses who also had aneurysms. Group B consisted of 10 patients (8 boys and 2 girls, aged 0.7-15.3 years [mean, 3.8 years]) with coronary aneurysms. Group C consisted of 29 patients (20 boys and 9 girls, aged 1.2-13.8 years [mean, 7 years]) with normal coronary angiograms. Two patients in group A (100%), 3 of 10 patients in group B (30%), and 19 of 29 patients in group C (65.5%) had myocardial perfusion defects. There was poor agreement between Tc-99m sestamibi SPECT and coronary angiography for detecting coronary stenoses (kappa = 0.07; P =.222) and aneurysms (kappa = -0.184; P =.158). CONCLUSION: Significant discordance exists between Tc-99m sestamibi SPECT and coronary angiography in patients with Kawasaki disease.

Adolescent↗

Transcatheter implantation of intravascular stents for postoperative residual stenosis of peripheral pulmonary artery stenosis.

This is a prospective study of transcatheter implantation of 11 intravascular stents in 7 patients with status/post (S/P) surgical correction of major cardiovascular lesions. The safety and efficacy of balloon-expandable stents for treatment of peripheral pulmonary artery stenosis (PPAS) is evaluated and analyzed. Although the transcatheter implantation of intravascular stents has been reported as a possible treatment for stenotic peripheral pulmonary arteries, the results of intermediate follow-up studies on patients with S/P surgical correction for residual PPAS need to be evaluated. From June 1998 to December 2001, a total of 15 patients with PPAS having S/P surgery for major cardiovascular lesions were enrolled in this study. Eight of them had redo surgery after complete evaluation and the other 7 patients who might be at higher risk of mortality or morbidity from redo surgery, underwent transcatheter implantation of stents to dilate significant PPAS. Tetralogy of Fallot, S/P total correction, was done in 6 and transposition of great vessels, S/P Jatene operation, was done in 1. There were 10 stents (P 308 Palmaz stent x8 and Intrastent x2) implantation for 10 sites of the stenotic PPAS in these 7 patients, who were aged from 3.6 to 17.3 (10.1 +/- 5.6) years and had body weights ranging from 17 to 72.5 (37.1 +/- 23.0) kg. The narrowest diameter of the stenotic peripheral pulmonary arteries and pressure gradients across the stenosis were measured before and after implantation of stents. A follow-up catheterization and pulmonary angiography was performed 1 year later to evaluate the intermediate efficacy of stents implantation. All the stenotic peripheral pulmonary arteries of these 7 patients had a significant reduction of pressure gradients immediately after the procedure. The narrowest mean diameter of pulmonary arteries increased from 6.7 +/- 3.4 to 11.3 +/- 3.0 mm (p < 0.001), and the mean pressure gradient dropped from 31 +/- 9.9 to 11.4 +/- 4.6 mm Hg (p < 0.001). The follow-up catheterization 1 year later revealed a persistent effect in all but 1 patient. Only a young male presented with a recurrent stenosis with a pressure gradient of > or = 20 mm Hg, which was relieved by redilation with implantation of another stent. There was no immediate or intermediate complication. Transcatheter stent implantation for treatment of a significant residual PPAS after surgical correction of complicated congenital heart disease is a safe and effective procedure. Since children are growing with age, a long-term follow-up study to evaluate the effects and possible problems of stent implantation is mandatory.

Adolescent↗

Management of acute decompensation of neonatal maple syrup urine disease with continuous arteriovenous haemofiltration: report of one case.

A 22-day-old female infant was referred to our hospital due to unusual urine odor suspecting inborn error of metabolism. Physical examination revealed a stuporous and hypotonic infant with poor reflexes. Intravenous thiamine and high glucose along with appropriate protein were given under the suspicion of maple syrup urine disease (MSUD), which was confirmed by blood and cerebrospinal fluid amino acid assays, and urinary organic acid assay. Progressive neurological deterioration was observed despite the non-invasive treatment. So, we performed pump assisted continuous arteriovenous haemofiltration (CAVHF). Dramatic improvement in neurological function was observed hours after initiation of CAVHF, along with decrease in the level of isoleucine, leucine and valine. In our experience, CAVHF is a well-tolerated procedure for managing the acute phase of neonatal MSUD. Further study on indications, duration of treatment, and preventing complications is needed.

Acute Disease↗

Peritoneal dialysis in children with acute renal failure after open heart surgery.

Acute renal failure is a frequently reported complication after open heart surgery in children and carries a high mortality. Conservative treatment often makes post-operative care difficult and ambiguous. Peritoneal dialysis (PD) or hemofiltration is another therapeutic choice. Hemofiltration has some limitations in pediatric patients. Many aspects of PD are currently under discussion. We retrospectively reviewed 184 children who underwent open heart surgery with cardiopulmonary bypass. Four patients fulfilled the criteria of acute renal failure. PD was performed after conservative treatment failed. All patients successfully survived through the post-operative period. No electrolyte imbalance, fluid overload or acute hemodynamic changes was observed after the initiation of PD. In our limited experience, PD is a safe and effective method for managing patients with acute renal failure after open heart surgery.

Acute Kidney Injury↗

Vascular ring due to double aortic arch with atretic left arch and left ligamentum arteriosum: report of one case.

A 1-day-old female infant presented with vomiting immediately after feeding and shortness of breath after birth. Esophagography revealed external compression of the esophagus. Echocardiography showed a right aortic arch with mirror image branching of brachiocephalic vessels without intracardiac anomalies. Left ventriculography confirmed the echocardiography findings and revealed a tenting of the proximal part of the left subclavian artery and a blind pouch of Kommerell diverticulum at the descending aorta. The patient underwent surgery, which revealed a complete vascular ring compressing the trachea and the esophagus. The ring was formed by a right aortic arch, atretic left arch and left ligamentum arteriosum. Following surgical division of the ligamentum arteriosum and the atretic left arch, the symptoms subsequently improved.

Aorta, Thoracic↗

Balloon valvuloplasty for critical aortic stenosis in two infants.

Critical aortic valve stenosis (AS) is a difficult clinical condition. Besides supportive medical care, relief of the stenosis is most important to the patients' survival. We present two cases using transcatheter balloon valvuloplasty in managing critical AS in young infants. Case 1 was a 2 month-old female infant. She presented with cardiopulmonary failure. Aortic annulus was 8 mm and the aortic valve was dilated with a 6 mm and then an 8 mm diameter balloon. The pressure gradient was reduced from 83 to 13 mmHg. Case 2 was a 7 day-old male infant. Aortic annulus was 6.7 mm and the aortic valve was dilated with a 6 mm diameter balloon. The pressure gradient was reduced from 87 to 44 mmHg. Both patients tolerated the procedure well. No major complications developed except that a transient weak femoral pulse was noted in case 1. In our limited experience, balloon valvuloplasty is a safe and effective therapeutic choice for managing critical AS in young infants. The advantages include no need of bypass or general anesthesia, shortened hospital course, reduced exposure to blood product, and no scar.

Aortic Valve Stenosis↗