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Biomedical subjects

H C Lue

Publications and source records attributed to H C Lue.

At least 37 records · Page 2Linked to original sources
PubMed1999

Tricuspid atresia with persistent truncus arteriosus.

The association of tricuspid atresia and persistent truncus arteriosus is a very rare congenital anomaly. We report a newborn with a prenatal diagnosis of tricuspid atresia, in whom associated type II persistent truncus arteriosus was found by postnatal echocardiography. The patient had mild cyanosis and developed heart failure soon after birth. Balloon septostomy was performed to enlarge the interatrial communication. However, her condition rapidly deteriorated and she died of sepsis and heart failure at the age of 14 days.

Female↗
PubMed1998

Transarterial approach of the pulmonary artery in anatomically corrected malposition of the great arteries by manipulating a catheter inverted with balloon floating maneuver.

We describe two neonates with anatomically corrected malposition of the great arteries (ACM). A 1-day-old female newborn, who had the salient clinical findings of Goldenhar syndrome, behaved like a patient with the tetralogy of Fallot. The cardiac malformations included ACM, perimembranous inlet and outlet ventricular septal defect with tricuspid valve straddling, conoventricular septal malaligement, bilateral cori without aortic-mitral continuity, pulmonary stenosis at subvalvular, valvular, and supravalvular levels, and bicuspid semilunar valves. After the procedure of left Blalock-Taussig shunt at 3 months of age, cyanosis was alleviated. The other 1-month-old male newborn, who presented with prolonged feeding, tachypnea and tachycardia, likened to a patient with the hypoplastic left heart syndrome. He had a combination of ACM, mitral stenosis, hypoplastic left atrium and left ventricle, a restrictive patent foramen ovale, ventricular septal defect, subaortic conus without aortic-mitral continuity, subaortic stenosis, and a narrow ascending aortic root. Rashkind balloon atrioseptostomy was done with limited success. He survived a Norwood procedure. Transarterial entry of the catheter, which was inverted with the aid of balloon floating maneuver, to hook the retro-aortic pulmonary artery in ACM is recommended.

Angiography↗
PubMed1998

Clinical implications of isolated double aortic arch and its complex with intracardiac anomalies.

From July 1, 1989 to June 30, 1996, a total of six patients, four male and two female, with double aortic arch (DAA) were registered. They aged 16 days to 6.5 years. There were two patients with isolated DAA, and four complicated with intracardiac anomalies. All six patients presented respiratory distress and feeding problem early in life. However, cyanosis, owing to decreased pulmonary blood flow, predominated the clinical picture in each patient having DAA intricated with tetralogy of Fallot (TOF) and pulmonary stenosis, TOF with absent pulmonary valve syndrome, and type II-A tricuspid atresia. DiGeorge syndrome was documented in one patient with TOF and pulmonary stenosis. Expiratory wheezing was remarkable in the patient with TOF and absent pulmonary valve syndrome. Mild cyanosis and heart failure was noted in one patient with ventricular septal defect (VSD), and bicuspid pulmonary valve with mild valvular stenosis. Barium esophagogram showed indentations on esophagus in five patients. Echocardiography was yielding in three infants. Four patients, two isolated and two complex DAA, survived the surgical division of the smaller left aortic arch. One-stage and two-stage operation was performed in patients having DAA intricated with VSD and TOF, respectively. The other two patients with complex DAA remained in close observation owing to the tolerance of symptoms related to intracardiac anomalies.

Aorta, Thoracic↗
PubMed1998

Usefulness of electron beam computed tomography in children with heterotaxy syndrome.

Children with heterotaxy syndrome frequently have complex cardiac and noncardiac malformations requiring detailed diagnostic evaluation for management planning. Direct delineation of these structures by electron beam computed tomography (EBCT) is validated as a means of diagnosis. From July 1995 to March 1997, 32 patients (16 girls, 16 boys) with clinically impressed heterotaxy syndrome were enrolled in this study. After evaluation by echocardiography, EBCT studies were performed. Interpretation of cardiac anomalies was performed by sequential analysis based on these cross-sectional images. The diagnoses were subsequently confirmed by angiocardiography and surgical findings. Twenty-eight patients had bilateral trifurcated bronchi, and most of these (24 of 28) did not have a spleen. Four patients had bilateral bifurcated bronchi, 2 patients had polysplenia, and the other 2 patients had a lobulated single spleen. We found that laterality could be identified by EBCT in all patients. Comparison of diagnostic yield between echocardiography, catheterization, and EBCT showed that EBCT is superior to echocardiography and catheterization in demonstration of pulmonary venous anatomy and presence of a very small rudimentary ventricle. In addition, associated visceral, bronchopulmonary, mediastinal, and intracardiac anomalies could all be clearly delineated by EBCT at the same time. Thus, EBCT is a promising complementary modality for an overall understanding of heterotaxy syndrome.

Abnormalities, Multiple↗
PubMed1998

Supraventricular tachycardia in patients with right atrial isomerism.

OBJECTIVES: To clarify the prevalence and mechanism of supraventricular tachycardia in patients with right atrial isomerism. BACKGROUND: Paired SA and dual atrioventricular (AV) nodes have been described in patients with right atrial isomerism. However, the clinical significance remains unclear. METHODS: From 1987 to 1996, a total of 101 patients (61 male, 40 female) and four fetuses were identified with right atrial isomerism. The diagnosis of supraventricular tachycardia exclude the tachycardia with prolonged QRS duration or AV dissociation, and primary atrial tachycardia. RESULTS: The median follow-up duration was 38 months (range 0.2-270 months). Supraventricular tachycardia was documented in 25 patients (24.8%) and one fetus (25%) (onset age ranged from prenatal to 14 years old; median 4 years old). Actuarial Kaplan-Meier analysis revealed that the probability of being free from tachycardia was 67% and 50% at 6 and 10 years of age, respectively. These tachycardias could be converted by vagal maneuvers in one, verapamil in seven, propranolol in four, digoxin in two, procainamide in one, and rapid pacing in five. Spontaneous conversion was noted in six (including the fetus). Seven cases had received electrophysiological studies. Reciprocating AV tachycardia could be induced in five and echo beats in one. The tachycardia in three patients was documented as incorporating a posterior AV node (antegrade) and an anterior or a lateral AV node (retrograde). Two of them received radiofrequency ablation. Successful ablation in both was obtained by delivering energy during tachycardia, aimed at the earliest retrograde atrial activity and accompanied by junctional ectopic rhythm. The patient with echo beats developed tachycardia soon after operation. CONCLUSIONS: Supraventricular tachycardia is common in patients with right atrial isomerism and can occur during the prenatal stage. Drugs to slow conduction through the AV node may help to terminate the tachycardia. Radiofrequency ablation is a safe and effective treatment alternative to eliminate tachycardia.

Actuarial Analysis↗
PubMed1998

Long QT syndrome manifested as fetal ventricular tachycardia and intermittent AV block.

A case of long QT syndrome diagnosed in the early neonatal period is described. This full-term female baby had intermittent atrioventricular (AV) block and ventricular tachycardia detected antenatally at the gestational age of 26 weeks. Sinus rhythm with prolonged QT interval (QTc = 0.636 sec) was found soon after birth. She developed variable degree of AV block with alternating left and right bundle branch block, which suggested the presence of multilevel AV block. Her mother had no lupus autoantibodies. Auditory brain stem evoked potential was normal. Family study revealed QT prolongation in her grandmother. Her condition improved after pacemaker implantation and oral beta-blocker usage.

Electrocardiography↗
PubMed1998

Evolution of dilated cardiomyopathy from hypertrophic obstructive cardiomyopathy in a child.

Hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) are two different diseases and have distinct etiologies. HCM is rare in children and rarely progresses to DCM. We report a 5-year-old Taiwanese girl with a definite diagnosis of hypertrophic obstructive cardiomyopathy. The diagnosis was based on echocardiograms, cardiac catheterization, and endomyocardial biopsy. However, the clinical features progressed to a picture of nonobstructive HCM at age 9, and the patient developed dilated poorly contractile ventricles with intractable congestive heart failure at age 10. She died from ventricular tachycardia at age 13. This report emphasizes the importance of periodic reevaluation for children with HCM.

Cardiomyopathy, Dilated↗
PubMed1996

Malalignment-type ventricular septal defect in double-chambered right ventricle.

Double-chambered right ventricle (DCRV) is commonly associated with ventricular septal defect (VSD). In this study, an assessment was made of the relevance of a malalignment-type VSD to hemodynamic and morphologic features in DCRV. During an 8.5-year period, 53 patients with DCRV were enrolled after study with echocardiography, catheterization, and angiography. Patients were divided into 2 groups: group I included 40 patients, aged 3.7 +/- 3.2 years, with a malalignment-type VSD; group II consisted of 13 patients, aged 8.6 +/- 2.7 years, without a malalignment-type VSD. History of congestive heart failure in infancy was present in 21 group I and 2 group II patients (53% vs 15%, respectively, p <0.05). The mean pulmonary-to-systemic flow ratio was significantly higher in group I than in group II (1.89 +/- 0.74 vs 1.14 +/- 0.21, respectively, p <0.05). The mean pressure gradient across the right ventricular outflow tract was lower in group I than in group II (41 +/- 16 vs, 73 +/- 33 mm Hg, respectively, p <0.05). Among 42 patients who had a series of echocardiograms recorded, progression of pressure gradient was evident in 35: 28 in group I and 7 in group II. A subaortic ridge was present exclusively in 29 group I patients (73%). Prolapse of the aortic valve was present in 26 (49%): 20 group I (50%) and 6 group II (46%) patients. Aneurysm formation of the septal defect was found in 17 (43%) and 7 (54%) group I and II patients, respectively. It can be concluded that a history of congestive heart failure was more common in DCRV patients with a malalignment-type VSD. Malalignment-type VSD is significantly associated with a larger pulmonary-to-systemic flow ratio and subaortic ridge.

Adolescent↗
PubMed1996

Angiographic evidence of long-axis rotation in addition to short-axis aortopulmonary rotation: its implication in transposition of the great arteries.

To assess the pattern of aortic sinus in varied aortopulmonary rotations and its clinical implications, three aortic sinuses at the diastolic phase in true lateral view are identified in 53 angiograms of complete transposition of the great arteries recorded between 1988 and 1993. The patients with the high takeoff of the coronary arteries found at surgery and in the literature were selected for additional investigation. Six regions were defined on the horizontal plane. From left anterior 89 degrees to 61 degrees, the non-facing sinus moved toward the anterior aspect (one patient). From left anterior 60 degrees to left anterior 1 degree, the left-hand sinus moved gradually from an anterior toward a posterior location (two patients). From directly anterior 0 degree to right anterior 59 degrees, the right-hand sinus moved from posterior toward an anterior position (30 patients). From right anterior 60 degrees to right posterior 105 degrees, the non-facing sinus moved posteriorly (20 patients). On approaching directly anterior 0 degree and toward right anterior 60 degrees, the left-hand sinus was the lowest in anterior transposition of the great arteries instead of the non-facing sinus, as in left anterior 90 degrees and in posterior transposition of the great arteries. High takeoff occurred commonly above the lowest left-hand sinus in anterior transposition of the great arteries (2 cases here and 5 in the literature, 100%). In conclusion, aortopulmonary rotations about the short and long axes were both evident on identification of the aortic sinus in various rotations. The aortic sinus did not rotate along the long-axis in anterior transposition of the great arteries, thus making the left-hand sinus, the lowest of this group, vulnerable to the high takeoff of the coronary arteries in anterior transposition of the great arteries.

Aorta↗
PubMed1996

Anomalous pulmonary venous pathway traversing pulmonary parenchyma. Diagnosis and implication.

STUDY OBJECTIVES: To describe four patients having total anomalous pulmonary venous connection with an intrapulmonary vertical vein, rendering difficulty in diagnosis and surgery. SETTING: a tertiary referral center. PATIENTS AND METHODS: By reviewing medical records, 4 of 25 patients with right atrial isomerism and total anomalous pulmonary venous connection were identified to have an intrapulmonary vertical vein. All four patients underwent echocardiography, catheterization, and angiography. One underwent MRI. Two underwent open-heart surgery and one received a modified Blalock-Taussig shunt. RESULTS: Right atrial isomerism was present in all four patients. On chest x-ray films, an abnormal shadow resembling scimitar syndrome was seen in two patients. Imaging the vertical vein was unsuccessful with an echocardiogram in all four patients. The intrapulmonary course of the vertical vein was depicted with a pulmonary venogram in two patients and with magnetic resonance in one patient. The intrapulmonary segment remained undetected until autopsy in one patient. All four patients died. At autopsy, the pulmonary venous confluence was hypoplastic in all four hearts. The vertical vein was buried in pulmonary parenchyma and drained to superior vena cava with significant obstruction. CONCLUSION: In the presence of right atrial isomerism and total anomalous pulmonary venous connection, there may be an intrapulmonary pulmonary venous connection that may be obstructed. Anastomosing the pulmonary venous confluence to the atrium may be difficult because of hypoplasia of the pulmonary venous confluence.

Anastomosis, Surgical↗
PubMed1996

Three- versus four-week administration of benzathine penicillin G: effects on incidence of streptococcal infections and recurrences of rheumatic fever.

OBJECTIVE: To investigate the effects of 3-week versus 4-week administration of benzathine penicillin G (BPG) on the incidence of Group A streptococcal infections and the recurrences of rheumatic fever (RF). STUDY DESIGN: We started, in 1979, randomly allocating all patients with RF to a 3-week or 4-week BPG prophylaxis program. They were examined at the RF clinic, every 3 to 6 months, and at any time they did not feel well. During 1979 to 1989, throat cultures and sera for antistreptolysin O and streptozyme titers were obtained at each clinic visit. Chest radiographs, electrocardiogram, color Doppler echocardiograms, and acute phase reactants were obtained. SUBJECTS: Two hundred forty-nine patients fulfilled the revised Jones criteria and were followed until December 1991: 124 in the 3-week and 125 in the 4-week program. Their age, sex, weight, percentage with history of RF, severity of cardiac involvement, follow-up duration, and compliance to program were comparable. Eight hundred eighty throat cultures were collected in the 3-week program and 770 were collected in the 4-week program. Six hundred sixteen and 627 sera were determined in each program for antistreptolysin O, and 582 and 592 sera for streptozyme titers. RESULTS: True streptococcal infections occurred in both programs: 39 infections in the 3-week program, and 59 infections in the 4-week program (7.5 vs 12.7 per 100 patient-years). Four infections with no antibody response occurred in the 3-week program, and three such infections in the 4-week program. Nine RF recurrences occurred in 8 patients in the 3-week program, and 16 recurrences in 16 patients in the 4-week program. Prophylaxis failure occurred in 2 of 124 patients in the 3-week program, and in 10 of 125 patients in the 4-week program (0.25 vs 1.29 per 100 patient-years). The overall recurrences/infections rate in each program was comparable, 13.6% vs 15.5%, but the recurrences/ infections rate due to prophylaxis failure was higher in the 4-week program than in the 3-week program, 3.0% versus 9.7%. CONCLUSIONS: This 12-year prospective and controlled study documented that streptococcal infections and RF recurrences occurred more often in the 4-week program than in the 3-week program. The risk of prophylaxis failure was fivefold greater in the 4-week program than in the 3-week program.

Adolescent↗
PubMed1995

Visualization of pulmonary vein obstruction by pulmonary artery wedge injection and documentation by pressure tracings: report of one case with persistent wheezing following correction of total anomalous pulmonary venous connection.

A 21-month-old boy presented with persistent wheezing 16.5 months after cardiac surgery for supracardiac total anomalous pulmonary venous connection. He had presented at birth with cyanosis and at the age of 25 days, underwent initial cardiac surgery in the form of anastomosis of the pulmonary venous confluence to the left atrium. Non-invasive investigations including transthoracic and transesophageal echocardiograms revealed stenosis of the right pulmonary veins. Visualization and documentation of right pulmonary vein obstruction by pulmonary arterial wedge angiography and pressure tracings are presented.

Anastomosis, Surgical↗
PubMed1995

Implication of anterior septal malalignment in isolated ventricular septal defect.

OBJECTIVE: The aim was to define the long term prognosis of isolated ventricular septal defect (VSD) with anteriorly malaligned outlet septum. DESIGN: Cohort study. SETTING: University hospital, tertiary medical care centre. PATIENTS: Between July 1986 and June 1993, 63 patients were studied with an isolated VSD and anteriorly malaligned outlet septum (59 perimembranous; 4 muscular outlet). MAIN OUTCOME MEASURES: The diagnosis of septal malalignment, aneurysmal transformation, right ventricular obstruction, subaortic ridge, and aortic valve prolapse was based on echocardiographic criteria, then confirmed by angiography in 33 patients and by surgery in 28. An actuarial curve for each event was obtained by Kaplan-Meier non-parametric analysis and the significance was examined by log-rank test. RESULTS: Aneurysmal transformation decreased the size of the VSD in 52% of the patients, but was also associated with the appearance of subaortic ridge (p < 0.05). Progressive obstruction in the right ventricle was observed in 51%, more often in those without aneurysmal transformation (p < 0.05). Aortic valve prolapse was quite common whether or not aneurysmal transformation occurred (33% and 23%, respectively). This was attributed to the location of the VSD and the anterior malalignment of the outlet septum. Surgery was performed in 28 patients at a median age of 50 months because of significant left to right shunt (n = 5), or the development of obstruction in right ventricle (n = 9), aortic valve prolapse (n = 3), or combinations (n = 11). The presence of subaortic ridge per se was not considered to be a surgical indication. CONCLUSIONS: Anteriorly malaligned VSDs have variable presentation. Careful echocardiographic evaluation is needed to identify various combinations of progressive right ventricular obstruction, aneurysmal transformation, subaortic ridge, or aortic valve prolapse. In extreme cases a patient may have a pathology complex comprising right ventricular outflow obstruction, subaortic ridge, aortic valve prolapse, and anteriorly malaligned VSD.

Child, Preschool↗
PubMed1995

Left ventricular thrombi in children with dilated cardiomyopathy.

To define the nature and course of left ventricular (LV) thrombi in pediatric patients, the charts and echocardiographic records of 32 pediatric patients with dilated cardiomyopathy from 1986 to 1991 were retrospectively reviewed. Five patients who were identified according to echocardiographic evidence of LV thrombi fulfilled the criteria of dilated cardiomyopathy. Systemic embolization occurred in three patients with LV thrombi who invariably died despite anticoagulant treatment. Although it was not statistically significant, patients with LV thrombi tended to have a lower ejection fraction compared to patients without LV thrombi. The morphology of LV thrombi in patients with systemic embolization showed nonprotruding features. Thus, children with dilated cardiomyopathy are at risk of developing LV thrombi and systemic embolization. Although it was not possible to identify the risk factors, the risk of developing LV thrombi and systemic embolization may be higher in patients with lower ejection fraction of the left ventricle.

Adolescent↗
PubMed1995

Doppler echocardiography for detection of ventriculo-coronary communication: report of a case.

Ventriculo-coronary communication is frequently encountered in pulmonary atresia patients who have an intact ventricular septum. Preoperative documentation is important to decide the feasibility of right ventricular decompression procedure. Experience of Doppler echocardiographic visualization of this anomaly in a case of critical pulmonary stenosis with intact ventricular septum is reported. Doppler echocardiography is a promising method of detection and should be used in every patient with a high-pressured hypoplastic ventricle before intervention.

Coronary Vessel Anomalies↗