Balloon angioplasty for obstructed modified systemic-pulmonary artery shunts and pulmonary artery stenoses.

OBJECTIVES: The results of percutaneous balloon angioplasty for obstructed modified Blalock-Taussig (BT) or central shunts and pulmonary artery (PA) stenoses were studied to assess its role as an alternative to second shunt and surgical PA angioplasty. BACKGROUND: Obstruction of a modified shunt and PA stenosis related to the shunt or ductus are not infrequent. A second shunt with or without PA angioplastv is required if the PA size, morphology or age of the patient is suboptimal for definitive surgery. METHODS: From June 1994 to May 1999, balloon angioplasty for obstructed systemic-to-PA shunts was performed in 46 patients, with ages ranging from 1 month to 7.4 years (2.2 +/- 1.9 years). Among the 46 patients, 32 had modified BT shunts, 5 had bilateral shunts, 7 had modified central shunts, and 2 had both modified BT and central shunts. Stenoses were seen in 27 main branch PAs, and interruption was present in three. A concurrent balloon angioplasty was attempted in 28 main branch PAs, but it was performed in only 25 vessels. RESULTS: Balloon dilation for obstructed modified shunts was considered to be effective in 42 patients (91%), while angioplasty for PA stenosis was effective in 14 vessels and not effective in 11 vessels. After balloon dilation angioplastv, oxygen saturation in the aorta increased from 74.4 +/- 4.3% to 80.8 +/- 3.6% (p < 0.01) in these 46 patients. One patient died of pneumonia. Eight patients required an additional modified BT shunt soon after the procedure because of severe stenosis or interruption at main branch PA. After a mean follow-up period of 11.6 +/- 5.4 months, 29 patients underwent a repeated imaging study to evaluate the morphology and size of the PAs. Of these 29 patients, 26 underwent open-heart surgery, with two mortalities. CONCLUSIONS: When a second shunt is under consideration because of obstruction of the modified shunt, balloon angioplasty is a possible alternative procedure. Pulmonary artery stenosis, if present, can be simultaneously dilated.

The assessment of the proximal left pulmonary artery by transesophageal echocardiography and computed tomography in neonates and infants: a case series.

IMPLICATIONS: Transesophageal echocardiography (TEE) is often used during surgical repair of congenital heart disease. In our case series of 256 newborns and infants, we found that a left paracarinal view of TEE could visualize the proximal left pulmonary artery, a frequent blind spot for TEE, in most patients, except in a few cases with anatomic variations of the esophagus in the right lateral to the vertebra.

Retroesophageal aortic arch: diagnostic and therapeutic implications of a rare vascular ring.

Retroesophageal aortic arch (REAA) can be further divided to right or left retroesophageal aortic arch. The right REAA has right ascending and left descending aorta with retroesophageal segment, whereas the left REAA has left ascending and right descending aorta. The REAA with retroesophageal segment may cause tracheoesophageal compression by the formation of a vascular ring. A total of eight patients were identified from the database from 1996 to 1999. Among them, five were right REAA and three were left REAA. The age at diagnosis varied from 4 days to 16 years (median 8 years). The clinical findings and initial diagnostic investigations, including the chest X-ray, echocardiography and esophagogram, suspected the presence of this anomaly. The diagnosis can be confirmed by ultrafast computed tomography with or without three-dimensional reconstruction. Half of the total patients, two of right and two of left REAA were symptomatic and needed operation. The symptoms related to the retroesophageal aortic arch appeared during infancy and early childhood (six cases), and only rarely in late childhood and adults (two cases). Half of the patients were associated with congenital heart disease, but no specific patterns of congenital heart disease were identified. The sensitivity of the early diagnosis of REAA by chest roentgenogram, echocardiography, esophagogram and angiography was 37% (3/8), 0% (0/7), 100% (1/1) and 60% (3/5), respectively. The sensitivity of ultrafast CT was 100% (8) in delineating both retroesophageal segment and complete vascular ring. Operation to relieve the vascular compression was performed in two cases with right REAA and two with left REAA. Only one died after the operation, due to pulmonary arterial sling and tracheal stenosis. The surviving patients had been asymptomatic during follow up. In conclusion, REAA can be best diagnosed by ultrafast computed tomography with or without three-dimensional reconstruction. If symptomatic, surgery to relieve the compression is effective and safe.

Valveless outflow reconstruction using autologous tissue as a posterior wall for pulmonary atresia with ventricular septal defect.

BACKGROUND AND PURPOSE: Reoperation is inevitable for some patients with pulmonary atresia who receive a heterograft or homograft in a primary Rastelli operation. Nonetheless, the need for reoperation in patients with classic Fallot's tetralogy who have undergone total correction with a transannular patch is unusual. We sought to change pulmonary atresia into Fallot's tetralogy and used a transannular patch instead of the conventional Rastelli operation. PATIENTS AND METHODS: Valveless outflow direct reconstruction was performed on 10 consecutive patients with pulmonary atresia and ventricular septal defect between August 1997 and 1999. Patient ages ranged from 1.3 to 11.5 years. A Blalock-Taussig shunt was previously constructed in four of these patients and a central shunt was constructed in five. The major aortopulmonary collateral arteries were occluded in one patient by repeated coil embolization after the central shunt. The strategy was to connect the right ventriculotomy with the pulmonary arteries directly, even if there was a gap with a long atretic cord. In patients with a previous central shunt covered with a Gore-Tex membrane, the reactive visceral pericardium over the in situ tissue (the left atrium, right ventricle, or aorta) was used as the autologous posterior wall. Thus, only autologous, fresh pericardium without a valve was used to cover the anterior part of the right ventricular outflow tract, as in the repair of classic Fallot's tetralogy with a transannular patch. RESULTS: There was no mortality, and the postoperative central venous pressure was low in all patients. No gradient was noted across the right ventricular outflow tract. Follow-up echocardiography revealed a competent tricuspid valve with mild pulmonary regurgitation in all patients. CONCLUSIONS: The results of this study suggest that valveless outflow direct reconstruction provides adequate pulmonary circulation without hypertension in pulmonary atresia patients with a ventricular septal defect if the tricuspid valve is competent.

Heart transplantation and the Batista operation for children with refractory heart failure.

Medically refractory heart failure may be present in children with cardiomyopathy (CMP) or complex congenital heart disease (CHD). In adults, the surgical management of this condition is either heart transplantation or the Batista operation. From March 1995 to January 2000, a total of 6 children, aged from 1 to 16 years, with medically refractory heart failure associated with CMP or complex CHD underwent cardiac transplantation and one of them also had the Batista operation as a bridge to transplantation. One of the 6 patients died of intractable sepsis 17 days after the operation, but the other 5 were discharged with satisfactory hemodynamics. Immunosuppressive agents, including azathioprine, cyclosporin or FK-506, were given. One patient experienced moderate acute rejection, but it was controlled by FK-506, OKT-3 and solumedrol. However, another suffered from lymphoproliferative disease 8 months after transplant, but it was controlled by intravenous immunoglubulin, alpha-interferon and acyclovir. Cardiac function during serial follow-up (range, 1 month to 5 years) revealed normal systolic and diastolic function and none received any anticongestive medications. Almost all patients received an oversized donor heart. The left ventricle (LV) mass was remodeled, initially as an decrease and later as an increase. The patient who underwent the Batista operation was discharged 1 month after the operation with an increased LV ejection fraction (from 10% to 22%). She was successfully bridged to heart transplantation 7 months after the Batista operation. The results of cardiac transplantation in growing children are satisfactory and remain the mainstay of surgical treatment for medically refractory heart failure in these patients. However, with a shortage of donor hearts, the Batista operation may be adopted as a bridge to heart transplant with a fair response.

Preoperative diagnosis by electron beam computed tomography and perioperative management of primary tracheal anomalies in tetralogy of Fallot.

PURPOSE: To investigate the usefulness of electron beam computed tomography (EBCT) in the preoperative detection and perioperative management of insidious concomitant primary tracheobronchial anomalies in patients with tetralogy of Fallot (TF). METHODS: From July 1995 to October 1999, 88 children (38 girls, 50 boys) with TF were enrolled in this study. EBCT examinations provided information needed to plan management. The final diagnoses of airway abnormalities were correlated with the findings of bronchoscopy in five patients and confirmed during surgery. RESULTS: Fourteen (16%) of the 88 patients had associated primary tracheobronchial anomalies. Nine patients had tracheal bronchi, which were combined with tracheobronchial stenosis in two patients and with tracheal stenosis in one patient. Two patients had tracheal diverticulum, which was combined with lower tracheal stenosis in one patient. Two patients had congenital tracheal stenosis. Tracheomalasia was found in one patient. Three patients with ventilation difficulties died postoperatively. Special attention was given to the care of the diseased airways perioperatively, and the remaining 11 patients had a smooth course of hospitalization and discharge. CONCLUSIONS: Our results show that EBCT provides good delineation of both cardiac and tracheobronchial anomalies and suggest that it should be used perioperatively to detect associated airway anomalies in TF, to facilitate the design of an appropriate ventilation care strategy.

Combined atrial and arterial switch operations for congenitally corrected transposition.

Conventional repair of congenitally corrected transposition of the great arteries (CCTGA) is directed at eliminating the associated defects and leaves the right ventricle in a systemic position. The long-term outcome of this procedure may involve deterioration of right ventricular function with tricuspid regurgitation and failure of the conduction system. We describe two consecutive patients with CCTGA, one of whom had apicocaval juxtaposition. The patients were aged 19 and 16 months, respectively, and both underwent a combination of atrial and arterial switch. These are the first two reported cases of successful completion of this type of operation in Taiwan. Our review of previously reported cases suggested that no significant difference exists in the outcome of patients with this condition who undergo either arterial switch or Rastelli-type repair plus atrial redirection. However, reported patients who underwent anatomic repair had lower early mortality, late mortality, and incidence of complete heart block than those who underwent conventional repair. The present two cases and our review of the literature suggest that, among patients with apicocaval juxtaposition, 1) Mustard operation is optimal for patients with small atrial volume; 2) one-and-one-half ventricular repair may be helpful to the outcome, especially when treatment is combined with Rastelli-type repair; and 3) excellent access to the ventricular septal defect through the tricuspid valve is afforded via a left atriotomy. From the present two cases and our review of the literature, we conclude that anatomic repair is superior to conventional repair of CCTGA in terms of protection against dysfunction and failure of the anatomic right ventricle, tricuspid valve, and conduction system. Long-term follow-up is mandatory.

Validation of pulmonary venous obstruction by electron beam computed tomography in children with congenital heart disease.

The purpose of this study was to explore the useful imaging findings of electron beam computed tomography (EBCT) for diagnosing pulmonary venous obstruction (PVO) in children with congenital heart disease. From July 1995 to March 1998, 17 children (9 girls and 8 boys, aged 7 days to 14 years and 9 months [median 3 months]) with the diagnosis of PVO were enrolled in this study. All images were obtained by EBCT at the end-diastolic phase of the cardiac cycle after administration of intravenous iodinated contrast medium. The findings of 25 EBCT studies were retrospectively analyzed by 2 radiologists and were correlated with echocardiography, angiocardiography, and surgical findings. Main findings on EBCT for PVO were (1) structural narrowing, (2) thickened interlobular septa, (3) peribronchovascular cuffing, and (4) ground-glass opacity of the alveoli. Structural narrowing along the course of the pulmonary venous drainage was the most important finding in all examinations (25 of 25). Lung parenchymal changes secondary to PVO included thickened interlobular septa (17 of 25), peribronchovascular cuffing (15 of 25), and ground-glass opacity of the alveoli (8 of 25). Thus, the combination of these findings provides very useful data for the definitive diagnosis of PVO. Characteristic electron beam computed tomographic findings can validate suspected PVO noninvasively.

Transvenous permanent pacemaker implantation in children and adolescent.

In pediatric patients cardiac pacing has been traditionally carried out by the epicardial approach in Taiwan. This study was to define the long-term results of transvenous endocardial pacemaker therapy in growing children. From 1994 to 1997, patients with sympatomatic bradycardia referred for permanent pacemaker were enrolled. Permanent pacemaker was implanted under propofol anesthesia and direct puncture of subclavian vein and creation of prepectoral pocket. A total of 10 patients ( 5 male, 5 female) aged from 5 to 17 years (13 +/- 4 yrs), constituted the study population. Follow-up period ranged from 14 to 48 months (29 +/- 14 months) . Underlying rhythm disturbances and pacemaker type implanted were: complete atrioventricular (AV) block 5 (VDD pacemaker in 3 and VVIR in 2), sick sinus syndrome 2 (DDDR pacemaker in both), sick sinus syndrome with abnormal AV conduction 1 (WIR), long QT syndrome 1 (VVIR) and hypertrophic cardiomyopathy 1 (DDDR). Pacemaker dysfunction occurred in only one patient in whom the endocardial lead was fixed by absorbable thread with an aim to have more flexibility of the lead. The endocardial lead was dislodged but properly reimplanted about 3 months after the initial implantation. All patients had satisfactory lead sensing and pacing threshold during the long-term follow-up. The pacing threshold was much better than that usually neededfor epicardial leads. With growing, none have the problems of lead length. Quality of life was reported to be improved in all. Tranvenous permanent pacemaker implantation is feasible in children aged 5 or older The long-term efficacy is satisfactory. Physiological pacing using a single lead (VDD) is recommended for younger patients with impaired AV conduction.

Echocardiographic assessment of total anomalous pulmonary venous connections in pediatric patients.

BACKGROUND AND PURPOSE: Cardiac catheterization can be superfluous and risky for sick babies, infants, and young children with total anomalous pulmonary venous connection (TAPVC). This study assessed the accuracy of echocardiography in the clinical assessment of pediatric patients with TAPVC before cardiac surgery. PATIENTS AND METHODS: A total of 15 consecutive patients with TAPVC treated between July 1, 1993, and December 31, 1999, were included in this retrospective study. Patients with TAPVC with heterotaxy syndrome were excluded. We assessed this cardiac anomaly preoperatively using plain chest roentgenograms, echocardiography, and magnetic resonance imaging. A combination of suprasternal, parasternal, subcostal, and apical four-chamber views and their tilting scans were employed for diagnosis and to trace the course of the anomalous pulmonary venous connection. RESULTS: Interatrial right-to-left shunt via atrial septal defects were documented by two-dimensional echocardiography with color Doppler mapping in all 15 patients. Patent ductus arteriosus was found in six patients. Cardiomegaly with enlargement of the right atrium and the right ventricle could be seen on plain chest roentgenograms and by echocardiography in all but two patients with infracardiac TAPVC. In all patients, the left innominate vein, coronary sinus or right atrium, and portal vein were the draining sites of supracardiac, cardiac, and infracardiac TAPVC, respectively. A pattern of continuous flow without phasic variation, which is suggestive of stenosis of the pulmonary vein, was found in two patients with infracardiac TAPVC with obstruction. The surgical findings were reviewed and correlated well with those of the echocardiography. CONCLUSIONS: In infants and children with TAPVC, the drainage sites and flow profiles of the pulmonary veins can be exactly determined by Doppler echocardiography preoperatively, which makes cardiac catheterization and angiocardiography unnecessary.

Cardiac rhythm disturbances in patients with left atrial isomerism.

This long-term study sought to determine the clinical implication of defective sinus node and AV conduction tissue in patients with left atrial isomerism (LAI). From 1984 to 1998, a total of 22 patients were identified as LAI. Patient age at the last follow-up ranged from 2 to 276 months (90+/-70 months). Associated cardiac anomalies were interruption of the inferior vena cava (n = 18, 82%), common atrium (n = 9, 41%), AV canal (n = 14, 64%), double-outlet right ventricle (n = 8, 36%), and pulmonary stenosis (n = 15, 68%). Palliative interventions were performed in 16 patients (Fontan-type operation in 4 patients, shunt followed by Fontan-type operation in 2, repair of septal defect in 4, and extracardiac intervention in 6). During the follow-up, over half of the patients (n = 14, 64%) developed bradyarrhythmia (onset age: from 1 to 264 months; median 78 months): junctional rhythm (n = 11), sinus bradycardia (n = 8) (5 patients also had junctional rhythm), and AV block (n = 2, both also had junctional rhythm). The probability free from bradyarrhythmia was 80% and 46% at the age of 2 and 6 years, respectively. None of the bradyarrhythmias were directly related to open-heart surgery. Besides, junctional ectopic tachycardia occurred after Fontan-type operation in three of six patients. In two patients, a Mahaim-like pathway was identified during the electrophysiological study. The patients with LAI had a high probability of developing bradyarrhythmias due to abnormal sinus node function. Varied AV conduction abnormalities may include compromised AV conduction, junctional ectopic tachycardia after Fontan-type operation, and an association of Mahaim-like pathway.

Modified arterial switch operation by spiral reconstruction of the great arteries in transposition.

BACKGROUND: Spiral relationship of the normally related great arteries (SRGA) has never been reconstructed in an arterial switch operation. METHODS: From March 1998 to April 1999, 9 consecutive cases of transposition of the great arteries (TGA) family (from 2 days to 1.6 years old) underwent arterial switch operations with SRGA at our hospital. Two had a congenitally corrected TGA (plus atrial redirection). Lecompte maneuver was not used in all. The posterior wall of pulmonary trunk was not divided but three were reattached, two of whom had had previous pulmonary trunk banding. Thus the wall was shared between the great arteries facing each other. RESULTS: All survived the operation. Supraaortic stenosis was balloon-dilated in 2 cases of early series, but technical modifications later were able to avoid it. Angiogram showed smooth flow into SRGA without upward and anterior tilting of the pulmonary bifurcation. All great and coronary arteries were patent. All were doing well on follow-up (16.5 +/- 4.2 months). CONCLUSIONS: We concluded that the techniques to relocate the coronary arteries using common wall and in situ switch could also be applied to pulmonary arterial reconstruction, so that SRGA can be resumed in TGA.

Epidemiology of leprosy in Taiwan; its pattern in children.

OBJECTIVE: To study the epidemiology of leprosy in children in Taiwan. SETTING: Taiwan, with a population increase from 3.3 to 21.7 million, several tides of immigration and national leprosy control programs, from 1910 to 1997. DESIGN: To collect and analyze the documents of Taiwan leprosy surveys and charts of the National Leprosy Control Center. PATIENTS AND MEASUREMENTS: Cumulative and new number of all-age and pediatric-age patients, prevalence rates, new case detection rates, and results of skin bacterial smears. RESULTS AND CONCLUSIONS: The prevalence rates of all-age leprosy ranged between 1.54 and 3.22 per 10,000 population. The proportion of children among all-age patients reached the highest of 4.93% in 1966, dropping to 0% in 1984 and thereafter, until 1988 and 1991 when two and one pediatric-age patients appeared, respectively, following the influx of immigrants from leprosy-endemic countries. The rise and fall of new patients younger than 15 years and 15 years or older were slightly correlated (r = 0.935, p < 0.001). Detection and confirmation of leprosy in children are usually belated. Physicians should still be acquainted with the clinical diagnosis of leprosy since sporadic cases of leprosy can reappear, particularly among children coming from endemic countries.

Radiofrequency catheter ablation of tachycardia in children with and without congenital heart disease: indications and limitations.

From 1993 to 1998, a total of 100 consecutive pediatric patients with tachycardia (45 male and 55 female, aged 1 year 10 months to 17 years, 11+/-4 year) who underwent electrophysiological study were reviewed. Eleven of them were younger than 5 years. Two had tachycardia-related cerebrovascular accident. Congenital heart disease was found in 12 patients. After propofol anesthesia, the clinical tachycardia could not be induced in three (two atrial tachycardia and one AV nodal re-entrant tachycardia) and became nonsustained in five (atrial tachycardia). Mechanical ablation occurred in three and two had subsequent recurrences. Among the 85 cases who received radiofrequency ablation, the overall final success rate of RF ablation for all diagnoses was 94% with a diagnosis-specific success rate ranging from 100 to 57%. Tachycardia cardiomyopathy was noted in four (three atrial tachycardia and one junctional ectopic tachycardia) and all regressed after successful ablation. Success in two patients with left posterioseptal accessory pathway could only be achieved by delivering the energy at the middle cardiac vein. Two patients with right atrial isomerism had an 'AV nodal-to-AV nodal tachycardia' which was eliminated by ablation. Total recurrence rate was 13% but final success was achieved in all during re-study except the three patients who refused re-intervention. The atrial tachycardia developed in postoperative congenital heart disease was associated with the lowest success rate (57%) and highest recurrence rate (25%). Procedure-related complications occurred in four; two with transient brachial palsy, one with first-degree AV block and one with blood loss requiring blood transfusion. In conclusion, the experience of this single center confirmed the efficacy and safety of radiofrequency catheter ablation in treating pediatric arrhythmias, but the limitations in postoperative arrhythmias and the effects of propofol on tachycardia induction (especially the atrial tachycardia) need to be improved.

Right lung agenesis with left pulmonary artery sling.

We report on a 2-month-old infant girl who had right pulmonary agenesis and an unusual course of the left pulmonary artery. Computed tomography and cardiac catheterization showed that the left pulmonary artery arose from the main pulmonary artery, crossing the midline, and reaching the left lung via an aberrant course between the esophagus and trachea. The coexistence of right pulmonary agenesis and left pulmonary sling is extremely rare. Unlike in other reports, our patient remained symptom-free and in good health, with normal growth and development until age 2 years, when she died from complications during an attack of bronchiolitis caused by respiratory syncytial virus.

Influence of aortopulmonary rotation on the anomalous coronary artery pattern in tetralogy of fallot.

Three main patterns of aortic sinus rotation were defined on 517 lateral angiograms of tetralogy of Fallot with 14 cases of anomalous coronary artery pattern occurring only in patients with a right anterior or right lateral aorta. The significant dependence of coronary artery types on the aortic sinus pattern made it possible to predict the predisposing coronary artery pattern in tetralogy of Fallot based on the aortic sinus pattern.

Outcomes of transcatheter valvotomy in patients with pulmonary atresia and intact ventricular septum.

The results of transcatheter valvotomy in pulmonary atresia with intact ventricular septum (PA-IVS) patients are presented with an attempt to identify the predictive factors for pulmonary valvotomy alone as definitive treatment. Between June 1995 and December 1997, 14 PA-IVS neonates with tripartite right ventricle underwent an attempted pulmonary valvotomy. For perforation of the pulmonary valve, a guidewire was used in 4, and a radiofrequency guidewire in 10 patients. Two outcome groups were identified. Group I included those in whom transcatheter treatment achieved a definitive success; group II patients required surgery despite an initial successful valvotomy. The attempt failed in 3 patients, 1 of whom had pericardial effusion. Perforation of the pulmonary valve was achieved in 11 patients: 2 with a guidewire and 9 with a radiofrequency guidewire. A subsequent balloon valvuloplasty was performed in these 11 patients. After valvuloplasty, mean right ventricular pressure decreased from 124 +/- 24 to 60 +/- 15 mm Hg (p <0.01). One died of heart failure and infection 10 days later, despite successful weaning from prostaglandin E1. Group I patients (n = 6) were treated with transcatheter valvotomy alone. Group II patients (n = 4) required right ventricular outflow patch. Significant differences between the 2 groups (group I vs II) were identified in tricuspid valve Z value (0.52 +/- 0.37 vs -1.25 +/- 0.48, p <0.05), pulmonary valve Z value (-3.47 +/- 0.59 vs -5.43 +/- 0.94, p <0.05), and ratio of right-to-left ventricular area on the apical 4-chamber view (0.73 +/- 0.06 vs 0.49 +/- 0.03, p <0.05). There were no significant differences in hemodynamic characteristics between the 2 groups. After a follow-up period ranging from 7 to 35 months (mean 18 +/- 10.3), the most recent echocardiograms in the 10 patients showed a mean pressure gradient across the pulmonary valve of 17 +/- 15 mm Hg. All 10 patients had an oxygen saturation of >92%. Transcatheter valvotomy using a radiofrequency guidewire is a safe and effective treatment in selected patients with PA-IVS. Transcatheter valvotomy can be a definitive treatment in PA-IVS patients with a tricuspid valve Z value > or = -0.1, pulmonary valve Z value > or = -4.1 and ratio of right-to-left ventricular area > or = 0.65.

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.