A common systolic ejection murmur of the neonate has been attributed to physiologic peripheral pulmonic stenosis. We investigated this auscultatory finding using duplex pulsed Doppler. Three groups of normal fuliterm neonates less than one week old were studied—10 without murmurs, 10 with grade 1/6 murmurs and nine with at least grade 2/6 murmurs. We measured the anatomical size and peak flow velocities in the main pulmonary artery and left and right branches, the peak velocity in the right ventricular outflow tract, and the bifurcation angle. Flow gradients were calculated as 4 (Vmax)2 Groups were compared by t-tests. A loud peripheral pulmonic stenosis murmur was associated with increased pulmonary artery velocities, with left pulmonary artery velocity the most discriminating variable (1.3 ± 0.29 vs 0.94 ± 0.19 m/s; p ≤ 0.05). Although the peak gradient never exceeded 12 mmHg, there was an increased gradient in the loud murmur group (8.7 ± 2.6 vs 5.7 ± 2.2 mmHg; p ≤ 0.05) which may even be underestimated by the lack of angle correction. The left pulmonary artery diameter was also larger in the loud murmur group, but there were no other anatomic or volumetric flow differences between groups. The soft murmur group could not be separated from normals. We conclude that Doppler techniques can confirm the physiologic basis of peripheral pulmonic stenosis murmurs.

Obstruction of the left ventricular outflow tract continues to represent a challenging surgical problem, particularly in children. Failure to relieve the obstruction by either valvotomy or myectomy requires enlargement of the ventriculoaortic junction and outflow tract. Alternatively, the obstruction can be by passed using an apical valved veritriculo aortic conduit, althoughthis is associatedwith a high rate oflong-term complications. The use of a small xenograft in children is less than ideal because of early calcification, and the insertion of a mechanical valve may be feasible only after considerable enlargement of the ventriculoaortic junction. Aortoventriculoplasty is particularly suited for this purpose, and it is usually associated with good early results. Yet, long-term experience with this procedure, as far as we know, has not been reported previously. We describe our experience with the use of aortoventriculoplasty in four patients, three of whom have been followed-up for more than five years. Two patients have required reoperation for replacement of their prosthetic valves, one for bacterial endocarditis and the other for fracture of a continuous prolene suture which secured the prosthetic valve to the Dacron septal patch. All patients are currently in good health with continuing satisfactory relief of obstruction to their left ventricular outflow tract.

We describe a new method of transvenous laser or radiofrequency perforation of the pulmonary valve with subsequent balloon pulmonary valvoplasty. The technique obviates the need for arterial catheterisation and considerably shortens the time needed for the procedure. Uncomplicated and successful relief of pulmonary valvar atresia was achieved in both patients attempted, although one ultimately required a Blalock-Taussig shunt because of increasing muscular obstruction of the right ventricular outflow tract.

The use of an obstructing balloon catheter positioned in the outflow tract of the pulmonary ventricle may be an alternative method for the adaptation of the left ventricle to an increased afterload prior to an arterial switch procedure in patients with complete transposition requiring a two-stage approach. We tested this approach in an acute hemodynamic study in a piglet model. Seven piglets were anesthetized with halothane and ventilated at FiO2 1.0. Through a median sternotomy, the great vessels were exposed. The right ventricular pressure and the aortic blood pressure were recorded using microtip manometers, cardiac output was measured by an electromagnetic flow probe on the ascending aorta. A 6F or 7F Berman angiographic catheter was placed in the lumen of the pulmonary artery and the balloon inflated to decrease cardiac output no more than 30%. The hemodynamic changes observed after balloon inflation included an 100% increase in systolic right ventricular pressure (control vs balloon inflation (mean±SE) 20.4 ± 1.5 mm Hg vs 46.6 ± 4 mm Hg, p<0.001), a slight reduction in systolic aortic pressure (75.5 ± 3.8 mm Hg vs 65.0 ± 5.8 mm Hg; p<0.05), and a reduction in cardiac output by 30% (1.1 ± 0.081/min vs 0.8 ±0.08 1/min, p<0.01). The systolic right ventricular to aortic pressure ratio was increased from 0.27 ± 0.02 to 0.69 ± 0.08, p<0.001. This study shows that a balloon catheter may be useful in creating systolic hypertension in the pulmonary ventricle in patients with complete transposition requiring a two-stage approach.

Between February 1986 and December 1991, 41 patients with pulmonary atresia and intact ventricular septum were treated in our institution following a multistage protocol of management. In all cases, the first step was to construct a right modified Blalock-Taussig shunt during the neonatal period regardless of the right ventricular anatomy. Then, in patients with well-developed right ventricles possessing all three components, we proceeded to early surgical repair. In contrast, in patients with right ventricles having obliteration of some components, yet deemed to be recoverable, the next step was to provide palliative relief of obstruction in the right ventricular outflow tract followed, if possible, by subsequent repair. Fontan's operation was performed in patients with right ventricles considered unsuitable from the outset to support the pulmonary circulation. Only one patient died following the initial shunt procedure (mortality of 2.43%). The subsequent program of treatment has now been concluded in 22 patients. In all those deemed to have favorable native anatomy (10 cases), the subsequent complete repair was successful. Among the 24 patients who required palliation of the outflow tract, five died while total repair was subsequently performed in eight. Fontan's operation was performed without mortality in five patients with small right ventricles, although one patient died while waiting for surgery. In all, 89 procedures were performed with an overall mortality of 14%.

Obstruction to the pulmonary venous return is a frequent associated anomaly in patients with isomerism of the right atrial appendages. Yet, preoperative diagnosis by means of either cross-sectional echocardiography or cardiac catheterization can be intriguing. Indeed, the presence of two morphologically right lungs reduce considerably the size of window for precordial echocardiography. Also, in the presence of severe pulmonary stenosis or atresia, it can be difficult at cardiac catheterization to enter the pulmonary trunk. In these patients, construction of a systemic-to-pulmonary artery anastomosis will almost inevitably result in pulmonary edema. Between May 1984 and December 1988, five patients with isomerism of the right atrial appendages, severely decreased pulmonary blood flow and concealed obstruction to the pulmonary venous return were admitted to our hospital. A modified Blalock Taussig shunt by interposition of a polytetrafluoroethylene prosthesis was performed in each patients and all of them developed pulmonary edema. Three patients died despite appropriate medical treatment. The remaining two patients were successfully treated by banding of the Blalock shunt. This was performed in the first patient at the time of the initial surgery, when prior to closure of chest, pulmonary edema became manifest. The second patient who developed pulmonary edema early postoperatively, underwent cardiac catheterization to confirm the clinical diagnosis of obstruction to the pulmonary venous return. Reduction of blood flow through the Blalock shunt with resolution of edema was initially achieved by means of a partially occluding balloon catheter. Pulmonary edema recurred one week later because of rupture of the balloon and the patient eventually underwent a successful banding of the Blalock shunt through a left thoracotomy. We conclude that preoperative assessment of the pulmonary venous return is mandatory in patients with right isomerism and reduced pulmonary blood flow. Construction of a modified Blalock-Taussig shunt in the presence of concealed obstruction to the pulmonary venous return will almost inevitably cause pulmonary edema. Banding of the Blalock shunt can be successful, as observed in our experience, for the management of this serious complication.

Noninvasive translumbar renal artery Doppler blood flow patterns were obtained in 20 normal children, 11 with successfully repaired coarctation, and eight with native or restenosed coarctation. Native or restenosed lesions result in decreased relative systolic velocity and prolonged acceleration time. After successful surgery, the renal flow returns to near normal. This technique may provide a useful tool to explore and predict hemodynamic and renal physiologic changes, and follow the course of therapy.

Non-surgical occlusion of the arterial duct (ductus arteriosus) has been shown to be effective. Its limitations are yet to be fully defined. We have attempted to elucidate this issue in the setting of our early experience with the procedure, utilizing the Rashkind double disc occluder. After clinical and echocardiographic evaluation, 56 consecutive patients were studied invasively. In two patients, the patent duct was considered too large for occlusion and in one patient it could not be crossed with the diagnostic catheter. In the remaining 53 patients (95%), an attempt to implant the double disc occluder was made. The mean age of all patients was 7.9 years (range 0.16 year to 29 years). There were 35 females (63%) and 21 males. The mean weight was 24.2 kg (range 6.7 kg–85 kg). In five patients (9.5%), the procedure failed. This was due to pull through of the 17 mm device in three patients. In another patient, because of a very small duct (post surgical ligation), it was not possible to deliver the distal legs of the 12mm device. In the last patient, the device embolized to the left pulmonary artery and was retrieved to the groin. Subsequent replacement was not attempted. In 48/53 patients (90.5%) the device was successfully implanted. Nine cases of the 48 in whom the device was implanted (18.8%) showed complete occlusion at 10 minutes and 22 more (45.8%) five to 24 hours after the procedure. A total of3 1/48 patients (64.5%) were discharged home the day following the procedure with no residual shunt, as judged by color flow Doppler studies. The mean duration of the procedure in all patients was 94 minutes (range 47 to 244). The fluoroscopy time ranged from 3 to 81 minutes with a mean of 17 minutes. Follow-up data (between five days and 18 months) are available in 20/48 patients. Three patients had a clinically detectable residual shunt, with an audible continuous murmur six months after the procedure. All three have undergone complete occlusion using a second device. One more patient showed spontaneous occlusion on follow-up. So far, a total of 35/48 patients (72.9%) have had complete occlusion of the patent duct. We conclude that not all patients with an arterial duct are amenable to occlusion using the Rashkind device. The procedure appears to be safe when performed by a suitably trained novice, nonetheless, and successful implantation can be achieved in over 90% of patients.

The feasibility and efficacy of radiofrequency catheter ablation of medically resistant supraventricular tachycardia is a promising technique with applications in the pediatric age group. The limitations of this procedure in the pediatric population, however, have yet to be delineated. We herein report a case of incessant reentrant supraventricular tachycardia in a two month old infant which was resistant to pharmacologic therapy, yet successfully treated by radiofrequency catheter ablation.

This study aimed to assess the prevalence of “silent” valvar regurgitation in healthy Brazilian children; to determine whether variations in the gain settings of the machine interfered with its documentation; and to compare Doppler color flow mapping with the pulsed wave Doppler. Twenty-five healthy children were selected randomly. Echocardiographic equipment used was ALOKA 870. Fixed parameters were 3.5 MHz transducer, 400 Hz filter, reject of 7/14 and aperture of 30/80. Variable parameters were Doppler color flow mapping and pulsed wave Doppler gains. A protocol for evaluation of each cardiac valve was developed. Regurgitation was noted in 93% for the pulmonary valve, 74% for the tricuspid valve and 27% for the mitral valve. In the aortic valve, jets suggestive of mild valvar regurgitation were observed with the Doppler color flow mapping technique in two children but these findings were not corroborated by pulsed wave Doppler. Only in the aortic valve were findings from Doppler color flow mapping not in agreement with those from pulsed wave Doppler. Variations in the settings for gain did not interfere significantly in the documentation of regurgitation. Inter-observer variations were not significant (two independent operators). The clinical implications of these findings are yet to be established. Labeling these regurgitant jets as “normal” may be oversimplifying their meaning and perhaps overlooking the possibility of predisposition to endocarditis or progression of the valvar abnormality. Conversely, labeling them as “abnormal” can generate a more serious problem, which is that of iatrogenic heart disease. Prospective follow-up studies should be carried out in these subjects.

Review of an institutional experience of 81 patients admitted to the Royal Liverpool Children's Hospital between January 1958 and December 1990 with a diagnosis of pulmonary atresia and intact ventricular septum revealed that successful definitive repair was achieved in about one-third of those who underwent surgical treatment. A three-stage protocol, consisting of a systemic-pulmonary anastomosis followed by right ventricular decompression on cardiopulmonary bypass and subsequent separation of the systemic and pulmonary circulations, facilitated a biventricular correction but rarely led to a successful Fontan procedure. Both functional class and probability of survival improved after each stage of management.

Tricuspid valve endocarditis on a normal valve has been rarely documented in children. In all five previous cases the infecting organism was Staphylococcus aureus. We report five infants and children with tricuspid valve endocarditis, whose ages ranged from four months to four years. In all of them large vegetations were identified by two-dimensional echocardiography. Positive blood cultures were found in four of five patients. Staphylococcus aureus, Corynebacterium species, Haemophilus influenzae and Candida albicans were the respective isolates. There were three deaths, all relatively sudden, 10–33 days after commencement of antibiotic therapy. At the only post mortem carried out in the patient with Candida albicans endocarditis and Down syndrome, there were two large fungating masses straddling the tricuspid valve. They appeared to have obstructed the circulation. Microscopic examination of a lymph node and the thymus showed features consistent with an immune deficiency syndrome. One child has been lost to follow-up. In the remaining survivor, progressively severe tricuspid regurgitation with congestive cardiac failure, necessitated open heart surgery 13 months later. A homograft replacement of the destroyed septal leaflet was carried out. Tricuspid valve endocarditis in an infant or child is a serious and potentially lethal condition. Early surgical intervention to remove large obstructive vegetations appears to be warranted.

We studied the cardiac conduction system in a case of Hurler syndrome. There was dense fibrosis of the supporting matrix of the sinus node and accumulation of mucopolysaccharide in the nodal cells. The bundle branches showed prominent hydropic degeneration, being encased and punctuated by dense fibrous tissue. These changes in the conduction system may predispose to the development of arrhythmias, accounting for the sudden deaths in Hurler syndrome.

A complex malformation of the heart was diagnosed by fetal echocardiography at 27 weeks of gestation. The study of the relationship of the abdominal great vessels, showing the inferior caval vein anterior and to the left of the aorta, made possible the diagnosis of right isomerism. This inferential guide to the arrangement of the thoraco abdominal organs proves to be as valid in the fetus as it is in the neonate and child. The examination of the abdominal great vessels as part of the fetal scan is of great value, enabling us to counsel mothers appropriately in the light of the poor prognosis of neonates with isomerism of atrial appendages and complex intracardiac lesions.

A 21-year-old woman with tetralogy of Fallot, who had undergone a right Blalock-Taussig shunt at one year of age, developed endocarditis of the aortic valve. Septic thrombosis of the right pulmonary artery was diagnosed by intraoperative transesophageal echocardiography. Replacement of the aortic valve initially performed by a St. Jude Medical prosthesis, was unsuccessful due to immobilization of the valve occluder. Subsequent replacement of the St. Jude valve by a Carpentier-Edwards valve was uneventful. Thrombectomy and patch enlargement of the right pulmonary artery were also performed successfully. We suggest that the St. Jude Medical valve should be employed with caution for replacement of the aortic valve in patients with tetralogy of Fallot who have not undergone intracardiac repair. Transesophageal echocardiography is extremely useful in discovering undiagnosed associated lesion such as septic thrombosis of the pulmonary arteries.