Pulmonary atresia with intact ventricular septum percutaneous radiofrequency-assisted valvotomy and balloon dilation versus surgical valvotomy and Blalock Taussig shunt

OBJECTIVE: We compared the result of radiofrequency (RF)-assisted valvotomy and balloon dilation with closed surgical valvotomy and Blalock Taussig (BT) shunt as primary treatment in selected patients with pulmonary atresia and intact ventricular septum (PA-IVS). BACKGROUND: Patients with PA-IVS who have mild to moderate hypoplasia of the right ventricle (RV) and patent infundibulum have the greatest potential for complete biventricular circulation. The use of RF or laser wires to perforate the atretic valve followed by balloon dilation provides an alternative to surgery. METHODS: Between May 1990 and March 1998, 33 selected patients underwent either percutaneous RF valvotomy and balloon dilation (group 1, n = 21; two crossed over to group 2) or surgical valvotomy with concomitant BT shunt (group 2, n = 14). Second RV decompression by balloon dilation or right ventricular outflow tract (RVOT) reconstruction were performed if necessary. Patients who remained cyanosed were subjected to transcatheter trial closure of the interatrial communication. Partial biventricular repair was offered to those with inadequate growth of the RV. RESULTS: The primary procedure was successful in 19 patients in group 1. There was one in-hospital death and two late deaths. Of the remaining 16 survivors, 12 achieved complete biventricular circulation, 7 of whom required no further interventions. Two patients required repeat balloon dilation, 1 RVOT reconstruction and 2 transcatheter closure of interatrial communication. Two patients underwent partial biventricular repair. In group 2, there were 3 in-hospital deaths after the primary procedure and 1 patient died four months later. All survivors (n = 10) required a second RV decompression, 8 by balloon dilation and 2 by RVOT reconstruction, after which, two patients died. Of the final 8 survivors, 7 achieved complete biventricular circulation, 5 after coil occlusion of the BT shunt and 2 after closure of interatrial communication. CONCLUSIONS: Radiofrequency valvotomy and balloon dilation is more efficacious and safe compared with closed pulmonary valvotomy and BT shunt in selected patients with PA-IVS.     

Coarctation of the aorta in infancy

Twenty-five infants under 1 year of age (mean weight 3.4 kg) underwent repair of coarctation of the aorta between the years 1965 and 1982. Three patients had coarctation only, three had coarctation with patent ductus arteriosus (PDA), and 19 had associated intracardiac anomalies. Eleven patients underwent resection of the aorta and end-to-end anastomosis. Eight had subclavian flap arterioplasty, five had patch graft arterioplasty, and one had subclavian-to-aortic anastomosis. Additional procedures were performed on seven patients: banding of the pulmonary artery on one, repair of total anomalous pulmonary venous drainage on one, mitral valve replacement on one, aortic valvotomy on one, and aortic valvotomy plus pulmonary artery banding on one. Twenty-one survived the operation. All patients who died had associated intracardiac anomalies. The 21 survivors have been followed from 3 months to 13 years, with three late deaths that were associated with intracardiac anomalies. Five of the survivors underwent additional second operations: one had repair of the re-coarctation, one had replacement of the prosthetic mitral valve, one had aortic valvotomy, and two had pulmonary artery debanding and closure of a ventricular septal defect. Two of the 18 surviving patients have mild hypertension associated with a residual gradient. The others are in good health.     

Laser valvotomy with balloon valvoplasty for pulmonary atresia with intact ventricular septum: five years' experience.

OBJECTIVE: To assess immediate and medium term results of transcatheter laser valvotomy with balloon valvoplasty in selected infants with pulmonary atresia and intact ventricular septum. DESIGN: Prospective study. SETTING: Tertiary cardiac unit. PATIENTS: All infants with pulmonary atresia and intact septum with no more than minor tricuspid valve hypoplasia referred between November 1990 and June 1995. Laser valvotomy was attempted in nine infants of median age 4-5 days and median weight 3.6 kg. INTERVENTION: The pulmonary valve was perforated using a 0.018 inch fibreoptic guidewire attached to a NdYag laser and introduced through a catheter positioned beneath the valve. After perforation the valve was dilated with progressively larger balloons. MAIN OUTCOME MEASURES: Successful laser valvotomy and balloon dilatation, complications, pulse oximetry, right ventricular outflow velocities, and need for surgical treatment. RESULTS: Valvotomy was successful in all but one case, the failure being due to laser breakdown. After perforation the valve was dilated to 6-8 mm diameter. Prostaglandin E was withdrawn immediately in six of the eight duct dependent infants, and 28 and 49 days later in two. No patient required an aortopulmonary shunt. Two patients had repeat valvoplasty at 20 days and three months of age, respectively; one required infundibular resection and closure of the atrial septum at age four and one is awaiting similar treatment. CONCLUSIONS: Laser valvotomy with balloon valvoplasty is safe and effective treatment for selected patients with pulmonary atresia and intact ventricular septum and should be considered as first line treatment in place of surgical valvotomy.     

Management and outcome of infants and children with right atrial isomerism.

OBJECTIVES: To assess the current results and outcome of surgery in infants and children with right atrial isomerism and complex congenital heart disease. SETTING: Tertiary referral centre. METHODS: 20 consecutive children with right atrial isomerism and complex congenital heart disease underwent surgery over a 6 year period between August 1987 and July 1993. The results and outcome were analysed according to age, presentation, and surgical procedures. RESULTS: Patients were divided into two groups depending on age at presentation and initial surgery: group A comprised 11 patients who required surgical intervention in the first month of life (mean age 5 days); and group B comprised nine patients who required initial surgical intervention after the first month of life (mean age 6.8 months). Seven (64%) of the 11 patients in group A had obstructed pulmonary venous drainage and ten (91%) had pulmonary atresia. There were seven early deaths (64%), including the five patients who required systemic to pulmonary artery shunt and simultaneous repair of obstructed pulmonary veins. The long-term survival rate in this group was 18% (two of 11). Pulmonary venous obstruction was present in two (22%) of the nine patients in group B and four (44%) had pulmonary atresia. There were no early deaths. One patient died after a second palliative procedure. There was one late sudden death. Four patients had a Fontan operation with no deaths. Two of the remaining three patients meet the Fontan criteria. The long-term survival rate in this group was 78% (seven of nine). CONCLUSIONS: Surgical management of patients with right atrial isomerism who have complex congenital heart disease carries a high mortality and remains palliative. The overall survival rate was 45% (nine of 20); 18% in patients requiring surgery in the first month of life (group A) and 78% in patients requiring surgery after the first month of life (group B) (P < 0.001). Of the total of 20 patients, nine were potential candidates for a Fontan operation. Seven of these have undergone a Fontan procedure with five survivors.     

Determinants of successful balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum

Objectives. This study reviewed our experience with percutaneous balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum and defined the anatomic and hemodynamic characteristics of infants in whom this procedure is successful and provides definitive therapy.Background. Unlike children with valvular pulmonary stenosis, the follow-up of infants with critical pulmonary stenosis undergoing percutaneous balloon valvotomy is limited.Methods. Between December 1987 and August 1992, percutaneous balloon valvotomy was attempted in 12 infants with critical pulmonary stenosis (n = 10) or pulmonary atresia with intact ventricular septum (n = 2). Two outcome groups were identified: Group A patients are acyanotic, have mild residual pulmonary stenosis and have not required operation; Group B patients have required operation.Results. Of the 12 infants, 11 had a successful balloon valvotomy procedure. Group A patients (n = 7) have a residual gradient of 22 ± 18.7 mm Hg (mean ± SD) at follow-up of 3.2 years (range 1.2 to 5.0). In Group B (n = 5), operation was required for inability to cross the pulmonary valve (n = 1) or persistent severe hypoxemia for ⪰2 weeks after valvotomy (n = 4). Significant differences (p ≤0.01) between the two groups (Group A vs. Group B) were identified in pulmonary valve annulus (Z value) 8.1 mm (−1.1) versus 5.5 mm (−3.4); tricuspid valve annulus (Z value) 14.0 mm (0.8) versus 8.8 mm (−1.8); right ventricular volume 65 versus 29 ml/m²; and Lewis index 10.9 versus 8.9.Conclusions. Percutaneous balloon valvotomy is effective and likely to provide definitive therapy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum who have a tricuspid valve annulus >11 mm, pulmonary valve annulus ⪰7 mm and right ventricular volume >30 ml/m².     

Severe tricuspid stenosis presenting as tricuspid atresia. Echocargraphic diagnosis and surgical management.

Two cases of pulmonary atresia with intact ventricular septum and severe tricuspid stenosis are described in which the initial angiographic diagnosis was tricuspid atresia and pulmonary atresia. Two dimensional echocardiography showed the features of an imperforate tricuspid valve because in each case contrast echocardiography failed to show anterograde flow from the right atrium to right ventricle. Successful radical surgical repair was achieved in one patient with performing a tricuspid valvotomy and inserting an external valved conduit between the hypoplastic right ventricle and the main pulmonary artery. In the second case, an infant died four weeks after tricuspid valvotomy and right ventricular outflow tract reconstruction.     

Long-term follow-up of operated congenital heart disease

The long-term follow-up status of patients operated upon for the more common forms of congenital heart disease is reviewed. Among patients with large left-to-right shunts the long-term prognosis for those with ASD and VSD is generally excellent. With rare exceptions, these patients can look forward to a normal adulthood. Residual atrioventricular valve disease complicates the long-term course of infants and children operated upon for atrioventricular canal, but nevertheless, most patients have remained stable for many years. Among patients after relief of right ventricular outflow tract obstruction, excellent documentation of long-term stability has been reported for children with valvular pulmonary stenosis, and most patients with tetralogy of Fallot appear to tolerate residual right ventricular outflow stenosis and insufficiency without serious complications. Dysrrhythmias occur, but severe sequelae are rare. The prognosis for survivors of pulmonary atresia with VSD is more guarded. Conduit longevity appears to be the most important concern, and long-term studies indicate that xenografts rarely last beyond a decade. The modern types of homografts cannot yet be evaluated on a long-term basis. For patients with pulmonary atresia and intact ventricular septum, there are a number of long-term survivors, but most have chronic right heart dysfunction with only a rare, outstanding result. The long-term outlook for patients with left ventricular outflow obstruction is guarded. Most patients after repair of aortic stenosis have residual aortic stenosis and/or insufficiency. Postaortic valvotomy adults tend to be symptom-free, despite residual disease, but almost certainly late management will be required.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pulmonary atresia with intact ventricular septum

Opinion statement The prognosis for patients with pulmonary atresia with intact ventricular septum is poor with and without conventional surgical treatment. Because of this reason, a comprehensive program of medical, transcatheter, and surgical treatment is necessary to improve the long-term outlook of these infants. Algorithms of management plans should be developed based on the presence of right ventricular-dependent coronary circulation as well as size and morphology of the right ventricle. In a tripartite or bipartite right ventricle, transcatheter radiofrequency perforation is preferable. Alternatively, surgical valvotomy may be performed. Augmentation of pulmonary blood flow by prolonged infusion of prostaglandin E₁, stenting the ductus, or a surgical modified Blalock-Taussig shunt may be necessary in some of these patients. In patients with a unipartite or very small right ventricle or a right ventricular-dependent coronary circulation (Tricuspid valve Z score < -2.5), augmentation of pulmonary flow along with atrial septostomy should be undertaken. Follow-up studies to determine the feasibility of biventricular repair should be undertaken and, if feasible, surgical or transcatheter methods may be used to achieve the goals. If not suitable for biventricular repair, one-ventricle (Fontan) or one and one-half ventricular repair should be considered. Comprehensive and well-planned treatment algorithms may help improve survival rate.     

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries

INTRODUCTION: In order to establish the best strategy of treatment and predictors of outcome in infants with pulmonary atresia with ventricular septal defect and major aorto-pulmonary collateral arteries, we reviewed our institutional experience concerning 47 infants. METHODS: Inclusion criteria included an angiographic diagnosis of pulmonary atresia with ventricular septal defect and major aorto-pulmonary collateral arteries with or without central pulmonary arteries and a repair by the same surgeon. Thirty-one patients had confluent (type III) and 16 absent (type IV) central pulmonary arteries. Pulmonary arteries were considered to be adequate when they measured > or = 4 mm. RESULTS: There were 8 deaths (17%). Complete repair was performed in 24 patients (18 in group III and 6 in group IV) with 79.3% had a right/left ventricular pressure <0.5, 16; 5%<1 and 4.2%>1. Eleven patients are waiting for complete repair and 4 will be operated on pulmonary arteries. One stage complete repair was performed in 3 patients, two and third stage repair (after unifocalisation or right ventricle to pulmonary arteries conduit) was attained in 21 patients. Among patients having had a complete repair and showing a right/left ventricular pressure <0.5, 88.9% had a pulmonary atresia with ventricular septal defect type III and 50% a pulmonary atresia with ventricular septal defect type IV. Only 56% of type III patients with a right/left ventricular pressure <0.5 had adequate central pulmonary arteries. CONCLUSION: In our study, the small size and the absence of central pulmonary arteries do not prevent a positive outcome.     

Longevity of Neonatal Ductal Stenting for Congenital Heart Diseases with Duct‐dependent Pulmonary Circulation

Introduction. Ductal stent (DS) in duct‐dependent pulmonary circulation is less morbid than neonatal Blalock–Taussig shunt. However, there is concern if DS provides an adequately long palliation before definitive repair. Methods. This is a retrospective review of clinical follow‐up of all consecutive infants after successful DS performed by a single operator. They were divided into three anatomic groups. Group A neonates had balloon valvotomy for critical pulmonary stenosis or pulmonary atresia with intact ventricular septum, who needed DS patency until the right ventricle was adequate to provide antegrade pulmonary flows. Group B patients with tetralogy of Fallot and pulmonary atresia suited for later biventricular repair needed ductal patency until conduit surgery was completed. Group C patients with functionally univentricular hearts needed DS patency until bidirectional Glenn shunt completion. Results. Among 22 infants, four Group A patients followed for 26–54 months after balloon pulmonary valvotomy had adequate oxygen saturation and needed only short‐term DS patency. In six out of nine Group B patients, corrective biventricular repair using conduits was performed after 5–14 months at a body weight of 5–7.5 kg. Bidirectional Glenn shunt and confluence repair were performed in seven of nine Group C patients weighing 6–8.5 kg after 8–15 months. The hilar pulmonary artery growth in B and C groups was adequate for surgical repair. No patient needed stent redilatations or additional shunts on follow‐up for hypoxia. Four patients had sudden death. Conclusions. The short‐term patency of DS was adequate after balloon valvotomy for critical pulmonary stenosis or pulmonary atresia with intact ventricular septum. Duration of palliation by DS was also sufficient in univentricular hearts to allow adequate somatic growth before Glenn surgery. In patients with biventricular anatomy treated by DS, conduit repair had to be performed at a relatively early age. Interstage mortality was 18%.     

Surgical management of severe aortic outflow obstruction in lesions other than the hypoplastic left heart syndrome: use of a pulmonary artery to aorta anastomosis.

Between December 1985 and April 1990, 50 infants with a variety of congenital cardiac lesions other than the hypoplastic left heart syndrome underwent surgical relief of aortic outflow obstruction by creation of a pulmonary artery to aorta anastomosis. The patients were grouped anatomically by ventriculoarterial alignment. Nineteen had normally aligned great arteries (group I); 25 had transposition of the great arteries, all with a univentricular heart of left ventricular morphology (group II); and 6 had a double-outlet right ventricle (group III). All patients had either aortic stenosis with atresia, subaortic stenosis or a restrictive ventricular septal defect. Sixteen had normal arch anatomy; 34 had arch anomalies consisting of arch hypoplasia (n = 17), coarctation (n = 11), interruption of the arch (n = 4) and complex arch anomalies (n = 2). Surgery was performed at a median age of 10 days (range 2 to 184). Of the 50 infants, 33 survived. No significant difference in early survival (30 days) was noted among the groups of varying ventriculoarterial alignment (68% group I, 72% group II, 83% group III) (p greater than 0.05). Overall actuarial survival was 63% at 18 months. Analysis of actuarial survival by arch anatomy, although not statistically significant, revealed a trend toward better survival at 18 months postoperatively in infants with normal arch anatomy (81%) than in infants with arch anomalies (54%). Of the 33 survivors, 26 have proceeded to the next surgical stage, including the Fontan procedure in 8, superior cavopulmonary anastomosis in 13 and biventricular repair in 5.(ABSTRACT TRUNCATED AT 250 WORDS)     

Functional assessment of the Fontan operation: combined M-mode, two-dimensional and Doppler echocardiographic studies

Combined M-mode, two-dimensional and Doppler echocardiographic studies were used to assess the postoperative status of 33 patients who had undergone the modified Fontan procedure. Twenty-four patients had surgical repair with use of a simple direct right atrium to pulmonary artery anastomosis. The remaining patients had repair with use of a prosthesis or associated Glenn shunt. Twenty-seven patients were studied early in the postoperative period (2 months or less) and the remaining patients were studied up to 6 years postoperatively. A total of 36 examinations were performed. Of the 33 patients, 13 had tricuspid atresia, 12 had double inlet left ventricle with hypoplastic right ventricular outlet chamber and 8 had complex lesions with atrioventricular canal, double outlet right ventricle or a hypoplastic ventricle. Postoperative assessment by M-mode and two-dimensional echocardiography demonstrated normal or mildly reduced ventricular function (ejection fraction greater than 40%) in 22 patients. In 24 patients, a "normal" flow pattern was observed in the pulmonary artery by pulsed Doppler echocardiography, with predominant diastolic flow and accentuation by atrial systole somewhat similar to the venous flow pattern observed in the superior vena cava. "Abnormal" flow patterns (disorganized systolic flow, absence of atrial waves and little or no increase with inspiration) were observed in nine patients with reduced ventricular function or residual shunt. Continuous wave Doppler study also demonstrated mild dynamic subaortic obstruction in two patients. Combined pulsed and continuous wave studies showed atrioventricular valve insufficiency in 10 patients. Follow-up studies revealed a satisfactory clinical course in most patients. Three patients died approximately 4 to 8 months after their Fontan operation.     

Pulmonary valve atresia with intact interventricular septum. Mid-term results of the surgical treatment. Apropos of 45 cases]

Between 1980 and 1991, 45 patients with pulmonary atresia with intact interventricular septum (38 cases) or critical pulmonary stenosis (7 cases), underwent surgery. The right ventricle was tripartite in 23 children, bipartite in 12 and unipartite in 10. Sinusoid vessels were present in 20 cases. They reinjected a native coronary trunk in 10 cases (major sinusoids). During the initial operation performed in the neonatal period in 41 cases and later in 4 cases, 26 children had an isolated systemic-pulmonary shunt (3 deaths) and 19 had connection of the right ventricle and pulmonary artery (6 deaths). Perioperative mortality was 20% (9/45). Global actuarial survival at 5 years was 47%. Of the 22 patients referred later for univentricular repair, 8 (36%) died (6 perioperative and 2 sudden deaths). The actuarial survival for this group was 49% at 4 years. Of the 23 patients referred for biventricular repair, 11 (47%) died (9 perioperative and 2 sudden deaths) and 2 attained complete cure status. The actuarial survival is 43% at 4 years in this group. Children with intermediate forms (type II or tricuspid less than 8 mm) were orientated to univentricular repair, partly due to the high incidence of major sinusoids. None of the children with major sinusoids survived to over 3 years of age. When the anatomic form is favorable, the present strategy is to perform transpulmonary valvotomy under cardiopulmonary bypass associated with the implantation of a Gore-Tex tube between the innominate artery and right pulmonary artery during the neonatal period. The risk of secondary pulmonary regurgitation would seem to be less than the immediate risk of low pulmonary flow.(ABSTRACT TRUNCATED AT 250 WORDS)     

Muscular Infundibular Atresia Is Associated with Coronary Ostial Atresia in Pulmonary Atresia with Intact Ventricular Septum

Objective. To determine whether infundibular morphology is associated with coronary ostial atresia and/or right ventricle dependent coronary circulation in patients with pulmonary atresia with intact ventricular septum. Design. Neonatal echocardiograms of patients with pulmonary atresia with intact ventricular septum were evaluated for tricuspid valve size and infundibular anatomy (muscular pulmonary atresia vs. membranous pulmonary atresia). Right ventricle dependent coronary circulation and coronary ostial atresia were diagnosed angiographically. Medical record review determined patient outcome. Setting. Tertiary‐care pediatric hospital. Results. Of 72 patients, 24 had muscular pulmonary atresia including 23 with right ventricle dependent coronary circulation. Fourteen of these had unilateral or bilateral coronary ostial atresia. Of 46 patients with membranous pulmonary atresia, 5 five had right ventricle dependent coronary circulation and none had coronary ostial atresia. Muscular pulmonary atresia patients had smaller tricuspid valve z‐scores and were less likely to achieve a biventricular repair than those with membranous pulmonary atresia (P < .01). Muscular pulmonary atresia had 82% sensitivity, 98% specificity, and 96% positive predictive value for presence of right ventricle dependent coronary circulation. In the group with membranous pulmonary atresia, there were two deaths, no transplants, and 23 (48%) achieved a biventricular repair. In contrast, of the 24 with muscular pulmonary atresia, there were seven deaths, two transplants, and no biventricular repairs. Conclusions. In this cohort, muscular pulmonary atresia was strongly associated with right ventricle dependent coronary circulation and coronary ostial atresia, and appears to be a useful morphologic marker for poor outcome among pulmonary atresia with intact ventricular septum patients. This information may be useful during prenatal counseling and for presurgical evaluation.     

Pulmonary atresia with an intact interventricular septum and Ebstein's anomaly of the tricuspid valve

Patients with pulmonary atresia and intact ventricular septum have a poor prognosis with or without conventional surgical treatment. The best results of surgical treatment are obtained in those cases with a mild underdeveloped right ventricle and minor sinusoidal communication in the absence of important dysfunction of the tricuspid valve. We present five cases of pulmonary atresia with intact ventricular septum associated with dysfunction of the tricuspid valve. On the basis of radiographic, electrocardiographic and hemodynamic findings, this group of patients could not be distinguished from others without dysplasia of the tricuspid valve. Echocardiographic and angiocardiographic studies are mandatory in the differential diagnosis. A combination of systemic-pulmonary artery anastomosis associated with pulmonary valvotomy, when possible, and reconstruction of the right ventricular outflow tract are indicated for surgical solution of these malformations. However, tricuspid valve replacement is indicated in some cases.     

Absent left-sided atrioventricular connexion, with right atrium connected to left ventricle: prospective diagnosis in infancy, and outcome.

Prospective echocardiographic diagnosis of absence of the left atrioventricular connexion, with the right atrium connected to a morphologic left ventricle through a bileaflet morphologically mitral valve, was made in six infants. The rudimentary right ventricle was left-sided in all patients, and separated from the left atrium by sulcus tissue. The ventriculoarterial connexions were discordant. Associated defects included subpulmonary stenosis (2 patients), pulmonary atresia (1 patient), and a patent duct (4 patients). All patients developed early left atrial hypertension due to a restrictive interatrial septum, and required transcatheter septostomy (5 patients), or surgical septectomy (3 patients). One patient who had a severely restrictive ventricular septal defect died following cardiac catheterization. In three others the ventricular septal defect has become progressively restrictive on serial catheterization. Successful intermediate term palliation has been performed in two patients using a bidirectional Glenn anastomosis, together with enlargement of the ventricular septal defect and a Damus-Kay-Stansel procedure in one. It is possible to distinguish this malformation from "mitral atresia" using cross-sectional echocardiography. The long-term outlook is influenced by early relief of left atrial hypertension. Balloon atrial septostomy alone is usually inadequate, and either blade septostomy or surgical septectomy are required. Serial cardiac catheterization is mandatory for planning definitive palliation.     

Clinical and hemodynamic results of the Fontan operation for tricuspid atresia

Thirty patients aged 3 months to 26 years (median 9 years) with tricuspid atresia or severe tricuspid stenosis underwent a modified Fontan operation at Children's Hospital, Boston, between 1973 and 1980. Thirty-six palliative operations had been performed previously in 20 patients. Preoperative hemodynamic measurements included: mean pulmonary arterial pressure 6 to 18 mm Hg (median 9); pulmonary vascular resistance 0.3 to 2.2 Woods units/m² (median 1.5) and left ventricular end-diastolic pressure 3 to 14 mm Hg (median 7).There were five hospital deaths (17 percent), including two in the last 24 operations. The 25 survivors have been followed up for 4 to 85 months (median 23) with no late deaths. Twenty patients are in New York Heart Association class I and four in class II; one infant was not classified. Two patients have required reoperation, at, respectively, 71 and 26 months postoperatively; the former for late-developing conduit obstruction and residual bidirectional shunting; and the latter for a residual right to left shunt at atrial level. Early postoperative monitoring of hemodynamics in the intensive care unit revealed that a mean right atrial pressure of 17 mm Hg or greater, without obstruction of the anastomosis, was invariably associated with serious morbidity or mortality.Fifteen patients underwent elective cardiac catheterization 9 to 21 months (median 13) postoperatively. The mean right atrial pressure ranged between 5 and 12 mm Hg (median 8) and mean pulmonary arterial pressure between 4 and 11 mm Hg (median 7). The cardiac index (measured in 12 patients) was 1.9 to 4.0 liters/min per m² (median 3.2). The left ventricular ejection fraction measured in 11 patients did not change significantly postoperatively, remaining normal in 10. Three patients had a residual right to left shunt at atrial level while the arterial oxygen saturation in the remaining 12 patients was 93 to 97 percent. Thus the modified Fontan operation can be performed with excellent clinical and hemodynamic results in selected patients with tricuspid atresia.     

Anomalous left coronary artery arising from the pulmonary artery: A series of 27 infants undergoing operation in the first years of life

Between 1977 and 1987, 27 infants (aged 3 to 54 months) underwent surgical treatment for correction of an anomalous left coronary artery arising from the pulmonary artery (ALCAPA). All had a direct aortic reimplantation. The overall operative mortality was 18.5% (five deaths). The follow-up period was from 2 months to 8 years (mean, 38 months). No late deaths occurred. During the 10 years, modifications of the surgical procedure and myocardial preservation were introduced (is, complete resection of the pulmonary trunk made anastomosis easier; since 1982, cardioplegia in both coronary systems has been used, and left atrial-to-aortic assistance was introduced). The latter was carried out when surgical repair was associated with acute cardiac failure and a high left atrial filling pressure at the end of cardiopulmonary bypass.In view of the results during the latter part of this series, it is felt that surgery should not be restricted in younger patients (<12 months). If medical treatment is unsuccessful, surgery aims to avoid irreversible left ventricular dysfunction and development of severe endocardial fibroelastosis.     

Congenital pulmonary atresia with tricuspid insufficiency: Morphologic study

In an anatomic study of 21 cases of pulmonary atresia with tricuspid insufficiency (pulmonary atresia with intact ventricular septum, type II), the morphologic features of the tricuspid valve and the right ventricle were found to differ greatly from those seen in pulmonary atresia with tricuspid stenosis (pulmonary atresia with intact ventricular septum, type I). Morphologically, pulmonary atresia with tricuspid insufficiency (type II) has a greater resemblance to Ebstein's disease with pulmonary atresia than to type I pulmonary atresia. The anomaly may be more amenable to surgery than pulmonary atresia with tricuspid stenosis because the right ventricle in the former may be converted into a functional chamber by a valvotomy combined with a shunting procedure and atrial septostomy.     

Arrhythmias after the Fontan procedure.

OBJECTIVE--To study the determinants and outcome of arrhythmias after the Fontan type operation. DESIGN--Retrospective analysis of data in patients operated on between 1972 and 1986 (follow up 5-19 years (mean 12 years)). PATIENTS--All 60 patients undergoing a Fontan type procedure at the National Heart Hospital, London, during the study period (mean age (SD) 12.3 (6.8) years). RESULTS--Postoperative arrhythmias occurred in 34 patients (57%), and 11 (58%) of 19 early postoperative deaths (within seven days) were related to arrhythmias. Early arrhythmias occurred in 19 (32%) patients of whom 11 (58%) died. All patients with early atrial fibrillation and His bundle tachycardia died and only preoperative atrial fibrillation recurred early. There was a higher incidence of early arrhythmias, which were less well tolerated, in double inlet single ventricle patients (9/19) than in those with tricuspid atresia (8/37). There were no other preoperative determinants of early arrhythmias or deaths from early arrhythmia. Late (after seven days) arrhythmias occurred in 15 (37% of hospital survivors). They had higher right atrial (RA) pressures both early and late after operation and had lower ventricular ejection fractions late after operation. Of those with atrial arrhythmias 86% had RA obstruction and 57% had an RA thrombus or pulmonary embolism at presentation; this was also confirmed in two patients in whom late sudden deaths occurred. Atrial fibrillation early after reoperation for RA obstruction was fatal. The actuarial arrhythmia free survival for hospital survivors was 60% at 10 years. CONCLUSIONS--Early postoperative arrhythmias were poorly tolerated, particularly atrial fibrillation and His bundle tachycardia. Previous atrial fibrillation was a relative contraindication to this procedure. Late postoperative arrhythmias were associated with higher RA pressures measured both early and late after operation and worse late ventricular function. Late arrhythmias may be the first manifestation of RA obstruction, which must be sought. RA thrombus was common in patients with atrial arrhythmias and should be treated early with anticoagulants.