Maurice P. Leung

Necrotizing enterocolitis in neonates with symptomatic congenital heart disease

10. Mollitt DL, Golladay ES. Postoperative neonatal necrotizing enterocolitis. J Pediatr Surg 1982;17:757-63. 11. Raziuddin K, Kim MH, Yao AC. Peripheral circulatory response to feeding in newborn low birthweight infants. J Pediatr Gastroenterol Nutr 1984;3:89-94. 12. Cooperstock MS. C. difficile, enterocolitis, and Hirschsprungs disease [Letter]. Lancet 1982;1:800. 13. Merkler RG, Solish SB, Scherzer AL. Meningomyelocele and Hirschsprungs disease: theoretical and clinical significance. Pediatrics 1985;76:299-300.

The role of cross sectional echocardiography and pulsed Doppler ultrasound in the management of neonates in whom congenital heart disease is suspected. A prospective study.

The application of cross sectional echocardiography and pulsed Doppler ultrasound to the management of symptomatic neonates with suspected congenital heart disease was studied in 96 consecutive cases. The ability of echocardiography to establish a complete and accurate diagnosis and a correct management plan was evaluated. Sequential segmental analysis of data from cardiac catheterisation and necropsy identified 536 cardiovascular anomalies. Of the 536 anomalies 512 were correctly diagnosed by echocardiography (sensitivity 95.5%). Seven false positive echocardiographic diagnoses were made (specificity 98.6%). Nearly all the missing diagnoses and all the false positive diagnoses made by echocardiography were extracardiac vascular anomalies. Normal cardiovascular anatomy was at all times correctly identified by echocardiography. In 12 babies (12.5%) a management plan could not be established because of inconclusive echocardiographic findings. Of the 84 proposed plans based on the echocardiographic findings, eight were found to be inappropriate after catheterisation. Thus, 76 babies (79.2%) could have been correctly managed without cardiac catheterisation. The combination of cross sectional echocardiography and pulsed Doppler ultrasound not only allows diagnosis of congenital cardiac anomalies in most neonates but can facilitate appropriate clinical management. Only a minority of neonates with suspected congenital heart disease require cardiac catheterisation and angiography.

Intraoperative TEE Assessment of Ventricular Septal Defect With Aortic Regurgitation

BACKGROUND It is desirable to repair but not replace the aortic valve in patients with ventricular septal defect and acquired aortic regurgitation. Precise definition of the valvar pathology with monitoring of its repair perioperatively would enhance the surgical management of this condition. METHODS Fourteen consecutive patients (age, 10.6 +/- 6 years; weight 29.7 +/- 5.7 kg) who underwent repair of ventricular septal defect with aortic regurgitation were studied by intraoperative transesophageal echocardiography. The severity of prolapse of each of the individual aortic cusps and its adjacent sinus was assessed and the valvar regurgitation quantified by Doppler-derived regurgitant indices. The echocardiographic and surgical findings were correlated and the preoperative and postoperative echocardiographic data were compared to assess the effectiveness of operation. RESULTS Eight subarterial and six perimembranous defects were located accurately and their sizes (11.8 +/- 3.0 mm) correlated well (r = 0.80) with the surgical measurements. Transesophageal echocardiography detected prolapse of the aortic valve and its sinus in all 14 patients. The severity of the prolapse was severe in 10, moderate in 4, and mild in 5 leaflets. One the basis of these findings, together with the Doppler-derived mean regurgitant indices, exploration of the valve and valvuloplasty were executed appropriately in 12 of 14 patients. In all 14 patients, transesophageal echocardiography after bypass revealed no further cuspal prolapse and significant reduction of the mean regurgitant index (0.55 +/- 0.23 to 0.17 +/- 0.15, p < 0.0001). Residual ventricular septal defect was detected in 5 patients and the only patient with significant shunting who required reexploration was identified correctly. CONCLUSIONS Intraoperative transesophageal echocardiography can assess effectively the surgical repair of ventricular septal defect with aortic regurgitation and provide information that directs and alters surgical plans to the benefit of patients.

Early and intermediate-term complications of self-expanding stents limit its potential application in children with congenital heart disease.

OBJECTIVES We report on the early and intermediate-term follow-up results of self-expanding Wallstent (Schneider, Switzerland) implanted in children with congenital heart disease. BACKGROUND The inherent shortcomings of balloon-expandable stents prompted the trial of an alternative stent. METHODS Twenty patients underwent 22 implantations of 25 self-expanding Wallstents between December 1993 and June 1997 in two institutions. The mean age and weight were 10.8+/-4.5 years and 30.5+/-14.2 kg, respectively. The patients were divided into two groups: 1) Group I comprised 17 patients with pulmonary arterial stenoses, 2) Group II comprised four patients with venous stenoses (one belonged to both groups). Sixteen patients underwent recatheterization at a median of 5.8 months (range 0.5 to 31, mean 8.1 months) after stenting. Hemodynamic and angiographic changes after the interventional procedures and complications were documented. RESULTS All the stents were successfully deployed in the intended position. In Group I, the narrowest diameter of the stented vessel increased from 4.1+/-1.5 to 8+/-2 mm (95% increase, p < 0.0001) while the systolic pressure gradient across decreased from 24.6+/-15.8 to 12.1+/-11.4 mm Hg (51% decrease, p = 0.001). In Group II, the dimensional changes of the narrowest segment increased from 4.3+/-0.5 to 7.5+/-0.4 mm (75% increase, p = 0.003), and the pressure gradient reduced from 5.0+/-2.9 to 0.9+/-1.0 mm Hg (82% decrease, p = 0.04) across the stented venous channel. Distal migration of two optimally positioned stents occurred within 24 h of implantation. At recatheterization, significant neointimal ingrowth (>30% of the expanded diameter) was noted in 7 (28%) of the 25 implanted stents. This responded poorly to balloon dilation. Predisposing factors for the neointimal ingrowth included stents of smaller diameter (<9 mm) and longer period after implantation. CONCLUSIONS Self-expanding Wallstent could be deployed easily and safely to relieve vascular stenoses in children. The complications of distal migration, significant neointimal ingrowth and its unyielding design to overdilation limit its application to this patient group.

Management evolution of pulmonary atresia and intact ventricular septum

To examine the impact on survival and clinical course of incorporating the morphologic classification of the right ventricle into the evolving management strategy for babies with pulmonary atresia and intact ventricular septum, the surgical results and follow-up status of the first 62 consecutive patients managed in this hospital between 1979 and 1990 were reviewed. Before 1984, all 23 babies from group I underwent primary right ventricular outflow reconstruction irrespective of right ventricular morphology and size. Since 1984, depending on the morphology and size of the right ventricle, 39 babies from group II had either closed transventricular pulmonary valvotomy (n = 31) or a shunt operation (n = 8). There were 10 hospital (43%) and 2 late deaths (total mortality 52%) in our group I patients. Three of the 11 long-term survivors had cyanosis at rest but none had any residual pressure gradient across the pulmonary outflow. Group II had 6 hospital (15%) and 4 late deaths (total mortality = 26%). Of the 29 long-term survivors, 9 had a second-stage right ventricular outflow reconstruction, 8 had balloon valvuloplasty and 2 had successful Fontan operation. At the latest follow-up, 5 children from this group have cyanosis at rest, 1 has a residual gradient (55 mm Hg) across the infundibulum, and 3 have right ventricular dysfunction. The hospital and total mortality for babies in group II was significantly lower than that in group I (p < 0.01). These data suggest that tailoring the treatment to the right ventricular anatomy results in a lower overall mortality although long term postoperative hemodynamic abnormalities are observed in both groups.

An echocardiographic study of perimembranous ventricular septal defect with left ventricular to right atrial shunting

Twenty nine patients with isolated perimembranous ventricular septal defects were investigated by M mode, cross sectional, and pulsed Doppler echocardiography. Tricuspid valve anomalies were present in all six patients with a left ventricular-right atrial shunt but in only six (26%) of 23 patients who had interventricular shunts only. Systolic flutter of the tricuspid valve was shown in five (83%) of the six patients with a ventriculoatrial shunt but not in the other patients. Systolic turbulence in both the right ventricle and right atrium was detected by Doppler echocardiography only in patients with ventriculoatrial shunting. A perimembranous ventricular septal defect with left ventricular to right atrial shunt can be diagnosed by its combined M mode, cross sectional, and pulsed Doppler echocardiographic features.

Management of branch pulmonary artery stenosis: balloon angioplasty or endovascular stenting.

1. The surgical outcome of congenital heart diseases may be adversely affected by residual branch pulmonary artery stenosis, which is difficult to treat surgically.

Abnormal radial artery in Down's syndrome.

Abnormal arterial patterns in the forearm were discovered in 12 of 77 patients (16%) with Downs syndrome. Eight patients had an enlarged anterior interosseous artery, the distal portion of which was palpable over the dorsum of the hand; in seven it was associated with absence of the radial artery and in one it coexisted with the radial and ulnar arteries. Three patients had a hypoplastic radial artery and a relatively dominant ulnar artery. One patient had a vestigial radial artery that ended as muscular branches in the forearm. The developmental aspects of these aberrations are discussed.

The management of symptomatic neonates with suspected congenital heart disease using combined cross-sectional echocardiography and pulsed Doppler flow study as the definitive investigations

We studied prospectively 140 consecutive symptomatic neonates with suspected congenital heart disease by combined cross-sectional and pulsed Doppler echocardiography. Using the sequential segmental approach, the anatomy at all cardiovascular junctions was clearly defined in 89 (64%) babies. Based on the non-invasive investigations, 47 went to surgery while 42 babies were treated medically. The other 51 babies underwent further cardiac catheterisation. Of these, only 23 (16%) required diagnostic catheterisation prior to their management decision. The remaining 28 babies were catheterised for (1) angiographic measurement of anatomical structures (n = 12), (2) haemodynamic measurement (n = 1), and (3) balloon atrial septostomy (n = 15). One hundred and two babies had ultimate verification of their echocardiographic diagnoses. A total of 612 cardiovascular segments were identified. There were 33 (5%) echocardiographic errors, 23 of missed or uncertain diagnosis and 10 wrong interpretations. The diagnostic sensitivity and specificity for the combined non-invasive technique were thus 96 and 98%, respectively. Only one death was attributed directly to an echocardiographic error. Hence when the clinical outcome was taken into consideration, 88 of the 89 neonates without an initial catheterisation were judged to be appropriately managed for their presentation.

Balloon valvuloplasty after pulmonary valvotomy for babies with pulmonary atresia and intact ventricular septum

During a 2 1/2-year period, staged procedures of transventricular closed pulmonary valvotomy followed by balloon valvuloplasty were attempted in 12 babies with pulmonary atresia and an intact ventricular septum. All babies immediately underwent valvotomy when echocardiography revealed a tripartite right ventricle with adequate inflow and outflow dimensions and without sinusoidal-coronary arterial fistulas. After valvotomy, the overall mortality rate was 25% (3/12), but the only surgical death (1/12, 8%) was due to failure to establish continuity between the right ventricular cavity and the pulmonary trunk. The other 2 babies died of neonatal complications after successful valvotomy. Angiocardiography performed 5 to 18 months after valvotomy documented substantial growth of the right ventricular inflow and outflow dimensions in the 9 survivors. Twelve balloon dilation procedures were then performed in 7 babies. All except 1 achieved a significant drop in the right ventricular to left ventricular peak systolic pressure ratio (0.96 +/- 0.40 to 0.56 +/- 0.28; p less than 0.01). Balloon valvuloplasty was not required in 1 baby and failed in the other, who then underwent successful right ventricular outflow tract reconstruction. After these staged procedures, follow-up at 1 month to 20 months (mean follow-up, 14.8 months) revealed resting cyanosis in 3 babies, which was related to severe residual infundibular stenosis (55 mm Hg) in 1 and a subnormal tricuspid valve annulus in 2. The remaining 5 babies (including 1 who required no valvuloplasty) were active and pink (saturation greater than 97%) and had a mean Doppler estimated gradient of 19 mm Hg (range, 8 to 36 mm Hg) across the pulmonary valve.(ABSTRACT TRUNCATED AT 250 WORDS)