Waterston Anastomosis Research Articles

Clinical Case Reports 1976 - 79

In this paper five case reports were presented and include growth of hypoplastic right ventricle following pulmonary valvotomy, intracavitary electrocardiography in the diagnosis of Ebstein’s anomaly of the left atrioventricular valve in congenital corrected transposition of the great arteries, growth phenomenon as a cause of kinking of the right pulmonary artery in Waterston anastomosis, the usefulness of pulmonary vein wedge angiography in the visualization of an obstructed pulmonary artery, false aneurysm of the right pulmonary artery as a new complication of the Waterston anastomosis. For each case report, clinical, chest x-ray, electrocardiogram, angiogram, and other pertinent findings were presented. This was followed by discussion of etiology, diagnosis, and treatment options, as appropriate.

Experiencia con el stent de Palmaz en la estenosis de rama pulmonar

Since 1989, the balloon expandable Palmaz stent (Johnson & Johnson Interventional Systems) has been used for the treatment of congenital heart disease. This study reports the results and the technique used for its implantation in native and post surgical retraction pulmonary branch stenosis. From february 1995 to june 1996, we have performed this technique in 11 symptomatic children/adolescents with pulmonary branch stenosis, the mean age was 11 +/- 2 years. Fourteen procedures were made and 16 stents were implanted. The mean condition of the patients was: 7 with tetralogy of Fallot after a previous complete correction, 1 with Noonan syndrome and a surgically corrected atrial septal defect, 1 with pulmonary trunk aneurysm and a previous surgical correction for pulmonary valve and branch stenosis, 1 with pulmonary atresia and an intact septum who underwent a surgical valvotomy and posterior percutaneous valvuloplasty with a good ventricular development, and final was a tricuspid atresia with a previous Waterston anastomosis which produced a stenosis at the right pulmonary branch who underwent a modified Fontan operation with the result of a severe stenosis at the medial and inferior right pulmonary artery. The branch ostium was affected in five cases, and in one case with diffuse hypoplasia of the right branch, the stenosis was at both sites. The stenosis was native in nine cases and post surgery in the other five (2 Waterston, 1 Blalock-Taussig, 1 Fontan). We used the right and/or left femoral vein in all cases except two, in which we used the internal jugular vein. We followed the technique reported by the Boston-Houston experience, with an extra stiff guide and Mullins sheath towards the site of the stenosis. The procedure was effective in all cases except one. The mean pulmonary branch and balloon diameter was 11 +/- 3 mm and 14 +/- 2 mm, respectively, rate 1.2. The stenosis diameter rose from 4.8 +/- 1 to 11 +/- 2 mm (p < 0.001) with a percentage fall from 63 +/- 5 to 2 +/- 1% (p < 0.001). The pressure gradient fell from 30 +/- 5 to 5 +/- 1 mmHg (p < 0.001) and the right ventricle systolic pressure fell from 77 +/- 6 to 48 +/- 3 mmHg (p < 0.01). The mean followup was 10 +/- 3 months, a symptomatic and pulmonary perfusion improvement was seen. After 8 +/- 4 months, we evaluated six patients/nine stents with angiography, and we excluded thrombosis, aneurysms and occlusion of lobar or segmental branches. One case had a proximal stent re-stenosis as it did not cover the whole stenosis, so a new stent was implanted. Three patients/four stents underwent redilatation getting a slightly higher diameter than previously. One patient had a stent migration and he underwent surgical stent retrieval. Implantation of the Palmaz stent is a useful procedure for the treatment of native or post-operative pulmonary stenosis. It is possible to apply it to hypoplastic and ostial pulmonary stenosis. We confirm the effectiveness of redilatation at mid term.

Balloon dilatation of a stenotic Waterston shunt with long-term follow-up

After its successful introduction in 1982 for the treatment of congenital heart lesions, percutaneous balloon dilatation has been applied to many stenotic lesions found in preoperative and postoperative congenital heart disease. 1,2 There have been rare reports of this technique's application to surgical aortopulmonary connection with varying results. Fischer et al 3 described successful dilatation of a Blalock-Taussig anastomosis, and Gibbs et al 4 described balloon dilatation of a Waterston anastomosis after which pulmonary edema developed and the patient died 12 hours after the procedure. A recent case report of long-term follow-up after initially successful balloon dilatation of a variant of a Blalock-Taussig shunt documented late remodeling at the angioplasty site and distal pulmonary hypertension. 5 We describe the successful long-term outcome after balloon dilatation of a stenotic Waterston anastomosis.

Successful modified Fontan procedure in an adolescent after left pneumonectomy

We performed a Waterston's anastomosis on a patient with complicated tricuspid atresia when she was two months of age. At age 14 years she required left pneumonectomy for massive, idiopathic hemoptysis. Four months after the pneumonectomy we substituted a modified Fontan anastomosis for the Waterston anastomosis. Restudy when she was 17 years of age showed continued satisfactory function.

Pulmonary vascular disease in shunted and nonshunted patients with tetralogy of fallot.

Histometric analysis of pulmonary vascular disease was performed in 13 shunted and 21 nonshunted patients with tetralogy of Fallot, and in 29 normal controls. There was no significant difference in the medial thickness of the small pulmonary arteries between cases of tetralogy of Fallot and normal controls, but the media in shunted cases of tetralogy of Fallot were thicker than in the nonshunted cases. Intimal fibrosis, regarded as organized thrombi, and thrombi of small pulmonary arteries were observed generally after 4 years of age in both the shunted and nonshunted cases of tetralogy of Fallot. Intimal proliferation of musculoelastosis which was formed of longitudinal smooth muscle bundles and elastic fibers was characteristic in shunted patients, especially after the central palliation procedure, Waterston anastomosis or modified Blalock-Taussig (BT) anastomosis using the Gore-Tex tube graft. However, it was not usually seen in the BT anastomosis cases. Unexpected pulmonary hypertension due to the shunt operation is thought to be the cause of musculoelastosis, because musculoelastosis was observed even in a patient with pulmonary hypertension only 3 weeks following surgery. We therefore recommend the original BT anastomosis as the shunt procedure. When considering the use of other shunt operations in which there is a possibility of placing a pressure load on the pulmonary vascular bed, attention must be given to the size of the anastomosis or artificial tube graft.

Ventricular inversion with normal ventriculoarterial connection and left atrial isomerism: Correction by the mustard operation

A 2 year old boy with ventricular inversion and normal ventriculoarterial connection is described. Associated cardiac lesions included single atrium, absence of the coronary sinus, bilateral superior venae cavae, absence of the hepatic segment of the inferior vena cava with azygos and hemiazygos continuation, right aortic arch, levocardia and left atrial isomerism. At 5 days of age, the patient underwent a Waterston (aortopulmonary) anastomosis because of suspected pulmonary atresia. The correct diagnosis was established at 2 years of age and the patient had a successful Mustard operation (interatrial baffle procedure) and closure of the Waterston anastomosis. Accurate preoperative diagnosis is difficult in this rare cardiac anomaly and the reported mortality rate is high.

Waterston Anastomosis in Two-Stage Correction of Severe Tetralogy of Fallot: Ten Years of Experience

Of 724 cases of tetralogy of Fallot surgically corrected from 1971 to 1981, 142 patients under 24 months of age, severely cyanotic and with hypoplasia of the pulmonary vasculature and pulmonary valvular atresia, underwent a modified Waterston-type anastomosis. These patients ranged in age from 6 days to 24 months (mean age, 9 months from 1971 to 1974, 5.5 months from 1975 to 1981). Early and late mortality (from 1 to 9 years) was 7.7% (11 deaths). No deaths occurred in the last 78 patients. After a minimum of 2 years, 131 patients had matured sufficiently at reevaluation for intracardiac surgical correction. Cardiopulmonary bypass with moderate hypothermia and intermittent aortic cross-clamping was used. Early and late mortality was 3.8% (5 deaths); only 1 of the last 81 patients died. Two patients early in the series developed severe pulmonary hypertension, 1 developed right ventricular infundibular atresia, and kinking of the pulmonary artery was found in 1. Acceptable postoperative right ventricular pressure/left ventricular pressure ratio was considered to be 0.55 or less. Transannular patch was required in only 2 patients (1.5%). All survivors are asymptomatic and physically normoactive. We believe that the Waterston anastomosis, if employed with accuracy by experienced surgeons, has clear advantages over other types of systemic-pulmonary artery fistula. These include stimulation of pulmonary arterial and valvular ring development; avoidance of physiological and functional arm deficiency; no need for deep hypothermia and its consequences; more possibility of preserving natural outflow of the right ventricular tract and better right ventricular function (low incidence of transannular patch); and avoidance of prosthetic material (Gore-Tex).

The waterston anastomosis for initial palliation of tetralogy of fallot: L. Parenzan, O. Alfieri, V. Vanini, T. Bianchi, M. Villani, R. Tiraboschi, G. Crupi, and G. Locatelli. J Thorac Cardiovasc Surg 82:176–181, 1981

The waterston anastomosis for initial palliation of tetralogy of fallot: L. Parenzan, O. Alfieri, V. Vanini, T. Bianchi, M. Villani, R. Tiraboschi, G. Crupi, and G. Locatelli. J Thorac Cardiovasc Surg 82:176–181, 1981

Persistent stenosis and deformity of the right pulmonary artery after correction of the waterston anastomosis: J. M. Wilson, J. W. Mack, K. Turley, and P. A. Ebert. J Thorac Cardiovasc Surg 82:169–175, 1981

Persistent stenosis and deformity of the right pulmonary artery after correction of the waterston anastomosis: J. M. Wilson, J. W. Mack, K. Turley, and P. A. Ebert. J Thorac Cardiovasc Surg 82:169–175, 1981

Waterston anastomosis for initial palliation of tetralogy of Fallot

Two hundred twenty-seven patients (median age 5.4 months) in whom a Waterston anastomosis was done for initial palliation of tetralogy of Fallot between 1966 and 1979 were studied. Twelve patients died in the hospital (5.3%; 70% confidence limits, 3.8% to 7.3%). Young age, low weight, and poor clinical condition did not appear to be incremental risk factors, whereas a too large or a too small shunt was largely responsible for the hospital mortality and morbidity. Follow-up information was available in all the 215 patients discharged from the hospital. At the last follow-up visit, before any further surgical procedure, 74% of the patients were clinically in good condition. By actuarial methods, 97.7% of hospital survivors were alive and 95.8% were event-free at and beyond 3 years postoperatively. Eighty-six patients have been catheterized in preparation for secondary repair (mean interval between Waterston shunt and catheterization, 2.9 +/- 1.38 years). One patient developed pulmonary vascular disease, four acquired pulmonary atresia, and 14 had a severe kinking of the right pulmonary artery at the site of the anastomosis.

Persistent stenosis and deformity of the right pulmonary artery after correction of the Waterston anastomosis

Between July, 1975, and July, 1979, 16 patients with cyanotic heart disease and previously constructed ascending aorta-right pulmonary artery (AA-RPA) shunts underwent subsequent operations. Fourteen of the 16 had significant physiological and anatomic problems associated with the shunts. Eight had kinking and preferential flow to the right pulmonary artery (RPA), four had preferential flow without kinking, and two had small pulmonary arteries bilaterally. Various techniques were used to close the RPA after Waterston takedown: direct suture in four, pericardial patch in five, prosthetic patch in four, and conduit in three. Five patients required reoperation 3 months to 3 years after Waterston dismantling because of persistent deformity of the RPA. Four patients had pericardial patch reconstruction of the RPA and one had direct closure of the opening in the RPA when the Waterston shunt was dismantled. All five patients received prosthetic patch enlargment of the RPA either alone or with a conduit. The four surviving patients are doing well 7 to 15 months following repair. Three have been studied angiographically and have been found to have no pulmonary artery deformity.

Scanning suprasternal echocardiography.

Scanning suprasternal echocardiography was performed in 280 patients with a variety of cardiac anomalies. By using the special suprasternal transducer on the suprasternal notch, the aortic arch, right pulmonary artery, and left atrium were recorded superoinferiorly. From this reference position various scanning techniques were made to record the main pulmonary artery, pulmonary valve, ascending aorta, aortic valve, and distal aortic arch, wherever possible. These scans made it possible to assess (a) the integrity and size of right pulmonary artery and main pulmonary artery in pulmonary atresia, stenosis of origin of right pulmonary artery, pulmonary artery banding, kinked Waterston anastomosis, and aneurysm of pulmonary artery; (b) relative positions of aortic valve and pulmonary valve in malposition complexes; (c) the position and size of the aortic arch in tetralogy of Fallot and aortic coarctation. Measurements of the left atrium made by suprasternal echocardiography were consistently larger than those made by praecordial echocardiography. Changes in relative sizes of aortic arch, right pulmonary artery, main pulmonary artery, and left atrium were also documented in the various cardiac anomalies. The atrial baffle after Mustard repair for d-transposition of the great arteries and the atrial membrane in cor triatriatum were also demonstrated. The introduction of scanning techniques has thus widened the scope of suprasternal echocardiography.

Growth of the pulmonary anulus and pulmonary arteries after the Waterston anastomosis

Twenty-six patients with tetralogy of Fallot underwent angiocardiography before a Waterston shunt and again several years later, prior to complete repair (mean interval 40 +/- 15.6 [SD] months). The ratio between the diameter of the pulmonary valve anulus (PVA) and that of the descending thoracic aorta (Ao) increased by 0.32 +/- 0.233, significantly more than is reported to occur after a Blalock-Taussig anastomosis (p = 0.0001). This increase had some relation to the interval between the two angiographic studies (r = 0.34, p = 0.08) and to the mean pressure in the main pulmonary artery (MPA) at the time of the second study (r = 0.35, p = 0.08). The ratio of diameter of the right (RPA) and left pulmonary arteries (LPA) and that of the Ao also increased by 0.24 +/- 0.226 and 0.20 +/- 0.201, respectively. These data suggest that an initial large aortopulmonary shunt may result in less need for transannular patching at complete repair.

Routine primary repair vs two-stage repair of tetralogy of Fallot.

Fifteen of 194 patients (7.7%) with tetralogy of Fallot operated upon since January 1, 1972 under a protocol of routine primary repair despite young age died in-hospital. Most deaths were from low cardiac output. Young age and smallness of size increased the risk of operation. No deaths occurred among patients older than 4 years. High hematocrit was also a risk factor. Transannular patching has an independent effect in increasing risk. The post-repair ratio of peak pressure in the right ventricle to that in the left did not exert an independent effect. To project current risks of a two-stage approach, we determined that five of 158 patients (3.2%) died in-hospital after secondary intracardiac repair after a previous Blalock-Taussig or Waterston anastomosis between 1967--1978. Using these data and those we have published on the risk of shunting, we project that except in very small babies, the risks of hospital death of a two-stage approach are not less than those of primary repair done without a transannular patch, except when body surface area is less than about 0.35 m2. When a transannular patch is used in the primary repair, the two-stage approach is projected to be safer when the child has a body surface area of about 0.48 m2 or smaller.

Repair of tetralogy of Fallot after Waterston anastomosis

Sixty-three corrective operations have been performed in patients with Tetralogy of Fallot and a previously constructed Waterston shunt. The patients were from 1.4 to 8 years of age (median 4.3 years), and the mean interval between the Waterston shunt and the repair was 39.3 +/- 16.05 months. Three patients developed pulmonary atresia and six required a second anastomosis prior to intracardiac repair. Kinking and stenosis of the right pulmonary artery at the site of the Waterston anastomosis occurred in 12 (19 percent; 70 percent confidence limits 14 to 26 percent) patients who required patch enlargement of the right pulmonary artery at repair. Seven (11 percent; 70 percent confidence limits 7 to 17 percent) patients died in the hospital and three (5 percent; 70 percent confidence limits 2 to 11 percent) during the period of late follow-up. None of the early or late deaths was specifically related to the presence of the previously performed Waterston anastomosis.

False aneurysm of right pulmonary artery: New complication of aorta-right pulmonary artery anastomosis

The case history of an infant with tetralogy of Fallot who had a Waterston anastomosis performed at the age of 1 year is presented. Subsequent chest roentgenograms revealed a ringlike calcification which was shown at angiography and operation to be due to a false aneurysm of the right pulmonary artery at the site of the Waterston anastomosis. The aneurysm also produced unidirectional obstruction of the right pulmonary artery. A review of the literature suggested that a false aneurysm of the right pulmonary artery after a Waterson anatomosis had not been reported previously.

The cause of kinking of the right pulmonary artery in the Waterston anastomosis

The case is presented of an infant, referred at the age of 8 hours, who underwent a Waterston anastomosis for pulmonary atresia with intact ventricular septum and a hypoplastic right ventricle. Angiographic studies performed 3 days and 12 months after the Waterston anastomosis clearly demonstrated that kinking was not present soon after the operation but developed later. Kinking of the right pulmonary artery is a widely recognized complication of the Waterston anastomosis. Data from other workers show that severe kinking occurs more frequently in infants operated during the first year of life than in those treated after 1 year of age. These data and the case herein presented support the hypothesis that the kinking occurring after the Waterston anastomosis develops because of growth and realignment of the great arteries. Because of this and other problems encountered after the Waterston anastomosis, it is recommended that either primary correction or, in case of an uncorrectable cardiac defect, a Blalock-Taussig shunt should be performed.

Current Results with Construction and Interruption of the Waterston Anastomosis

The Waterston anastomosis was constructed in 21 infants and neonates between 1973 and 1977. Sixteen neonates were 1 week old or less and 8 were less than 24 hours old. There were 2 operative deaths, giving a hospital survival of 90%. There were 3 late deaths. All surviving infants received satisfactory palliation except 1 who required a Potts anastomosis one year later. During the same time interval, 9 other patients who had had a Waterston anastomosis underwent complete intracardiac repair. Seven of them had significant angulation of the right pulmonary artery necessitating patch reconstruction. All patients survived operation, and follow-up pulmonary angiograms demonstrated only a slight persistent narrowing of the right pulmonary artery in 2 patients. We conclude that the Waterston anastomosis can be constructed with a low operative mortality even in the severely cyanotic neonate and that it can be taken down at the time of complete repair with minimal morbidity and no mortality even if it has significantly angulated the right pulmonary artery.

A Technique to Narrow the Lumen of a Waterston Anastomosis

A technique is described for narrowing the Waterston anastomosis by placing a horizontal mattress suture buttressed with felt pledgets through the aortic wall at the cephalad side of the anastomosis. This maneuver produces a slight but safe narrowing of the anastomotic lumen.

The Waterston anastomosis with no deaths in the neonate

The Waterston anastomosis has been performed with no deaths in 11 consecutive neonates during the past 24 months. Seven neonates had pulmonary atresia with either single ventricle or hypoplastic right ventricle, 3 had tetralogy of Fallot, and one had severe pulmonary stenosis with a normal right ventricle. Six neonates were one day old and only one was older than 7 days. All have obtained symptomatic and documented (increase Po2) benefit. This level of success is attributed to (1) minimal delay between the recognition of cyanosis and operation (mean time between hospital admission and catheterization was 3 hours and between catheterization and operation, 4 hours); (2) correction of any base deficit prior to, during, and after operation; (3) accurate construction of the anastomosis to avoid excessive size, and (4) careful postoperative management of the pulmonary subsystem by experienced personnel. We have demonstrated that the high mortality rate previously reported for the Waterston anastomosis in the neonate can be markedly improved by an appropriate patient management program.