Two-dimensional echocardiographic recognition and repair of subvalvular mitral aneurysm of the left ventricle in an infant.

The clinical features, diagnostic studies and surgical treatment of a subvalvular mitral aneurysm of the left ventricle are described. The infant presented at 9 weeks of age with large apical ventricular septal defects and pulmonary hypertension. The subvalvular aneurysm was an incidental finding. Both lesions were treated surgically.

Pulmonary artery origin of the left coronary artery: diagnosis by two-dimensional echocardiography, pulsed Doppler ultrasound and color flow mapping.

Five children, aged 0.2 to 6.7 years, with pulmonary artery origin of the left coronary artery proved by angiography underwent echocardiographic examination from 1985 through 1986. Prospective identification of this condition was achieved by two-dimensional echocardiography alone in two patients. Using a parasagittal plane from the second or third intercostal space, identification of the anomalous origin from the pulmonary artery was also possible in the other children on subsequent examination. In all of these studies the right coronary artery was considerably enlarged. The ratio of the diameters of the right coronary artery and the aortic root was 0.25 to 0.33 (normal 0.12 +/- 0.02). Flow mapping by color Doppler ultrasound in three children studied most recently and peripheral arterial or aortic root contrast echocardiography identified the site of entry of the coronary flow into the pulmonary artery. The flow disturbance was detected on the aortic surface of the pulmonary artery opposite the portion from where the flow from a patent ductus is usually identified. Pulsed Doppler ultrasound identified the flow disturbance in the pulmonary artery in only three of the children. The two youngest patients did not demonstrate turbulence on pulsed Doppler ultrasound, but they were not examined by color Doppler ultrasound. The accuracy of noninvasive detection of pulmonary artery origin of the left coronary artery by ultrasound is improved by the use of a combined echocardiographic approach.

The levoatriocardinal vein: morphology and echocardiographic identification of the pulmonary-systemic connection.

OBJECTIVES: This study considers the array of pulmonary-systemic connections made by the levoatriocardinal vein. The primary and associated lesions that play a role in forming this vein are examined, and echocardiography is discussed as a method for its rapid identification. BACKGROUND: The levoatriocardinal vein is a pulmonary-systemic connection that provides an alternative egress for pulmonary venous blood in left-sided obstructive lesions. It is thought to result from the persistence of anastomotic channels that connect the capillary plexus of the embryonic foregut to the cardinal veins. Only 12 cases of levoatriocardinal vein have been reported since its first description in 1926. A comprehensive description of the morphology and echocardiographic identification of this lesion has been unavailable because of its rarity. METHODS: A retrospective study was performed in 13 patients with a levoatriocardinal vein from the University of California, San Francisco. Echocardiographic findings were compared with those obtained by angiography or at necropsy. In addition, the details of 12 previously published case reports were reviewed. Age at presentation, primary obstruction to pulmonary venous return, integrity of the atrial septum and origin and drainage of the levoatriocardinal vein were compared. RESULTS: Patient age at presentation was < 2 years, with most patients presenting before age 6 months. Variations of the hypoplastic left heart syndrome accounted for the majority of primary defects encountered, although multiple but less severe left-sided lesions were seen. The atrial septum was functionally intact in most patients. The levoatriocardinal vein, defined echocardiographically, originated predominantly from the smooth-walled left atrium and drained to the superior vena cava or innominate vein; however, variations of this pattern existed. CONCLUSIONS: As a physiologic entity, the levoatriocardinal vein provides a mechanism for decompression of pulmonary venous return primarily in patients with left ventricular inflow obstruction. A levoatriocardinal vein is thought to form when the atrial septum fails to provide an alternate egress for left atrial blood. However, when a septal defect or alternative shunt occurs in conjunction with a levoatriocardinal vein, the clinical presentation may be postponed. Echocardiography provides a rapid, noninvasive modality for identifying the pulmonary-systemic connection, which may masquerade as the vertical vein in anomalous pulmonary venous connection or act as an occult source of left to right shunting in patients undergoing surgery for hypoplastic left heart syndrome.

Doubly committed subarterial ventricular septal defects: echocardiographic features and surgical implications.

Doubly committed subarterial (supracristal, subpulmonary) ventricular septal defects are often complicated by aortic regurgitation resulting from aortic valve herniation into the defect. The clinical, echocardiographic and catheterization findings in 48 patients aged 0.3 to 46.4 years (median 9.5) with a doubly committed subarterial ventricular septal defect were reviewed. Aortic valve herniation was present in 38 (79%) and 55% of these had aortic regurgitation. The prevalence of both findings increased gradually with advancing age. The defect was closed surgically in 41 patients. Surgery during the first 2 years of life (median 0.4 year) was performed in 13 patients (group I), mainly because of a large shunt with a pulmonary to systemic flow ratio (Qp/Qs) 3.8 +/- 1.4 (mean +/- SD). Aortic regurgitation was present preoperatively in two patients (15%), persisted postoperatively in one patient and did not develop in any after repair (median duration of follow-up 2.3 years, range 0.1 to 7.4). In the other 28 patients (group II) surgery was performed between 4.8 and 46.4 years of age (median 11.5). These patients were generally less symptomatic and had a smaller shunt (Qp/Qs 1.5 +/- 0.5, p less than 0.001). Preoperative aortic regurgitation was present in 18 (64%). It persisted in 15 postoperatively, but in 13 of these it had diminished. Two-dimensional echocardiography in multiple views identified the site of the ventricular septal defect in all patients. Serial echocardiographic examinations demonstrated the progressive nature of aortic valve herniation, the partial occlusion of the defect by the herniated sinus and the development of aortic regurgitation. These findings suggest that timely surgical closure of these defects may prevent aortic regurgitation.

Radiofrequency catheter ablation of supraventricular arrhythmias in patients with congenital heart disease: results and technical considerations.

OBJECTIVES: The aim of this study was to report the results and techniques of radiofrequency ablation for treatment of supraventricular arrhythmias in patients with congenital structural heart disease. BACKGROUND: The management of patients with congenital and other structural heart disease may be complicated by serious arrhythmias due to Wolff-Parkinson-White syndrome or by atrial arrhythmias after cardiac surgery. Ablation techniques using radiofrequency current are revolutionizing the management of arrhythmias, but reports have included few with structural heart disease. METHODS: Fifteen patients with significant heart disease underwent radiofrequency ablation: 11 with Wolff-Parkinson-White syndrome and 4 with intraatrial reentrant tachycardia after atrial surgery. Seven had Ebstein's anomaly, complex in two, and the rest had other defects. Coexistence of structural defects introduced significant technical difficulties to radiofrequency ablation in patients with the Wolff-Parkinson-White syndrome and was accomplished by adaptation of current techniques. Ablation of intraatrial reentrant tachycardia was performed by finding early atrial activation sites with electrogram fractionation for radio-frequency application. RESULTS: Radiofrequency ablation was initially successful in 14 of 15 patients, with cure in 10 and clinical improvement in 14. Two patients subsequently underwent cardiac surgery without perioperative arrhythmias. CONCLUSIONS: Radiofrequency ablation in patients with congenital heart disease and arrhythmias in both safe and effective and may be the preferred approach to treatment in some patients. In patients who are to undergo surgical correction or palliation, preoperative radiofrequency ablation of the tachycardia substrate is effective and may be preferred to operative accessory pathway division. The ablation of intraatrial reentrant tachycardia shows promise in the management of patients who have undergone extensive atrial surgery, and it may eventually become the preferred approach, particularly when there are contraindications to the use of antiarrhythmic agents.

Complications of pediatric cardiac catheterization: a 3-year study.

To determine the current risk of pediatric cardiac catheterization, the complications and incidents of all catheterizations performed in a pediatric laboratory between January 1986 and October 1988 were prospectively recorded and compared with results from a 1974 study from the same institution. In the current study 1,037 catheterizations, 885 diagnostic and 152 diagnostic/interventional procedures, were performed in 888 patients (aged 1 day to 27 years, median 15.6 months). There were 15 major complications (1.4%), 70 minor complications (6.8%) and 30 incidents (2.9%). Two patients died as a result of the procedure and two as a result of pericatheterization clinical deterioration caused by the cardiac abnormality. The great majority of complications were successfully treated or were self-limited and the patients had no residua. Of patients with 13 nonfatal major complications and 70 minor complications, residua were evident in 7 patients and 3 without evident residua had the potential for sequelae (0.7% and 0.3% of catheterizations). A comparison of the diagnostic and balloon atrial septostomy cases in the present study with similar cases in the 1974 study shows that the incidence of major complications has decreased from 2.9% to 0.9% (p less than 0.0001); minor complications and incidents have decreased from 11.7% to 7.9% (p less than 0.006) and pericatheterization deaths not attributable to catheterization have decreased from 2.8% to 0.2% (p less than 0.0001). Changes in pericatheterization medical management, patient selection for catheterization and catheterization techniques probably account for these improvements.

Electrocardiograph monitor artifacts in a neonatal intensive care unit.

A wide variety of artifacts may be found when monitoring the ECG in a neonatal intensive care unit. Many of the artifacts resemble arrhythmias, and unless they are recognized as artifacts they may lead to serious errors of diagnosis and therapy. Many of the artifacts are caused by patient movement such as seizures, tremulousness, or hiccups. Others may be introduced by the monitor itself or be caused by electrical equipment in the vicinity. A group of ECG tracings is presented to illustrate the various artifacts encountered. Features that distinguish the artifacts from the arrhythmias they mimic are described, as are suggestions for elimination of the artifacts.

Transient prolongation of the QTc interval after balloon valvuloplasty and angioplasty in children.

QTc-interval changes were evaluated in 39 children who had undergone balloon dilation procedures. In 30 children who had undergone balloon pulmonary valvuloplasty (BPV), QTc intervals were measured on standard electrocardiograms recorded before and 16 to 24 hours after the procedure and at follow-up during the next 1 to 15 months (mean 7). The QTc interval increased from 0.380 +/- 0.027 second before BPV to 0.429 +/- 0.027 second after BPV (p less than 0.002) and returned to 0.377 +/- 0.026 second at follow-up. To determine when during the catheterization-dilation procedure the QTc interval increased, QTc intervals were measured on monitor electrocardiograms recorded during cardiac catheterization. The baseline QTc was 0.382 +/- 0.041 second, was unchanged after angiography and increased to 0.427 +/- 0.043 second after BPV (p less than 0.002). A similar prolongation occurred in 3 of 4 children who had undergone balloon aortic valvuloplasty and in 4 of 5 who had undergone coarctation angioplasty. In control children, who had undergone catheterization without dilation, QTc was unchanged. Thus, transient increases in QTc interval occur after semilunar valvuloplasty and coarctation angioplasty. These dilation procedures are a cause of QTc prolongation; however, the mechanism is unknown. It is possible that the sudden unilateral increase in afterload affects the myocardium or myocardial sympathetic receptors. Because QTc prolongation may predispose children to the R-on-T phenomenon, postprocedure monitoring appears to be warranted.

Ventricular tachycardia and accelerated ventricular rhythm presenting in the first month of life.

Fourteen infants aged less than 1 month presented to our institution during the last 22 years with ventricular tachycardia (VT) or accelerated ventricular rhythm and a structurally normal heart. In 2, VT was associated with long QT syndrome. Both are alive on beta-blocker therapy, 1 with an implanted pacemaker. Twelve infants had accelerated ventricular rhythm, and 2 of these died in the first 2 months of life of unrelated conditions. The other 10 are alive at a median age of 4 years (range 2 months to 11 years), and none were lost to follow-up. Hemodynamic compromise did not occur with accelerated ventricular rhythm. The ventricular rate was very close to the sinus rate in all 12, less than 12% above the sinus rate. The mean QRS duration during accelerated ventricular rhythm was 92.5 ms, and averaged twice the QRS duration during sinus rhythm. Fusion beats were seen in all 12, and there was atrioventricular dissociation with capture beats in 10. In 2, ventriculoatrial conduction was present. Treatment was attempted in 5 of the 10 survivors with accelerated ventricular rhythm, and was thought to be successful in 4. Treatment was later successfully withdrawn in all 5, so that all 10 survivors were free of accelerated ventricular rhythm and were not receiving antiarrhythmic medications at last follow-up. Because of the excellent long-term outcome and the lack of hemodynamic compromise during the rhythm, it seems reasonable to withhold antiarrhythmic therapy in infants with accelerated ventricular rhythm and await resolution of the rhythm.

Diagnostic accuracy of pediatric echocardiograms performed in adult laboratories.

Although cardiologists who treat adults have been evaluating pediatric patients using echocardiography since the early 1980s, the diagnostic accuracy of such studies has never been tested. To prospectively assess diagnostic accuracy of pediatric echocardiograms performed in adult laboratories (adult-lab echos) compared with evaluations of the same patients in pediatric laboratories (pediatric-lab echos), we gathered data from 66 patients, ages 1 day to 18 years (median 19 months), who underwent adult-lab echos in community hospitals or private offices before evaluation by a pediatric cardiologist; subsequently, 65 underwent pediatric-lab echos in a university hospital laboratory. Echocardiographic diagnoses were compared with (1) diagnoses proved by catheterization or surgery (25 patients), and (2) echocardiographic diagnoses verified by blinded duplicate-observer review (41 patients). Eighteen patients had no cardiac disease; 42 had simple lesions, 5 had intermediate lesions, and 1 had a complex lesion. In 25 patients with 46 procedure-proven diagnoses, the most important error per adult-lab echo was major in 11 (44%), moderate in 7 (28%), and minor in 3 (12%); in pediatric-lab echos it was major in 0 (0%), moderate in 1 (4%), and minor in 1 (4%). In 41 patients with 62 duplicate-observer-verified diagnoses, the most important error per adult-lab echo was major in 5 (12%), moderate in 12 (29%), and minor in 5 (12%); pediatric-lab echos had no errors. In 35 of 66 adult-lab echos (53%), the most important error was major or moderate. Of these, 71% were interpretive, 17% technical, and 11% both. Error incidences were not related to patient age, study year, use of color Doppler, or complexity of diagnoses. In 29 of these 35 patients, pediatric-lab echos resulted in altered clinical management, including 12 surgeries and 2 averted surgeries. In 3 of the 29, delayed diagnoses were associated with fixed pulmonary vascular disease, hypoxemic spells, and vascular collapse with severe metabolic acidosis. This study reveals a high incidence of diagnostic errors in pediatric echocardiograms performed in community-based adult laboratories, despite a preponderance of patients with simple diagnoses or no heart disease.

Effects of activity on ascending aortic velocity in children with valvar aortic stenosis.

The peak velocity of aortic blood flow was measured in 12 normal children and 20 children with valvar aortic stenosis (AS) by continuous-wave Doppler ultrasound (CWD). Measurements were made at rest, after 2 forms of exercise and, in the 11 children who underwent cardiac catheterization, while under sedation. The exercise was both formal cycle ergometry and a 200-meter jog. The peak velocity in normal children was 1.5 +/- 0.2 m/s at rest, increasing to 2.1 +/- 0.4 m/s (p less than 0.01) after running and to 1.9 +/- 0.4 m/s (p less than 0.01) after cycling. Peak velocity in children with AS was 3.5 +/- 0.8 m/s at rest, increasing to 4.4 +/- 0.8 m/s (p less than 0.01) after running and to 4.5 +/- 0.5 m/s (p less than 0.01) after cycling. The increase in peak velocity was greater in children with AS than in normal children. Measurements of left ventricular (LV) aortic pressure differences by cardiac catheterization were compared to those made by CWD, using the modified Bernoulli equation. The best correlation of the CWD prediction of LV aortic pressure differences was achieved when the children were sedated. The peak velocity of blood flow in the ascending aorta varied with level of activity, and this variability must be considered when using the Bernoulli equation to predict LV aortic pressure differences. CWD prediction of the LV aortic pressure difference was best when the child was sedated (r = 0.98). It is therefore prudent to consider the velocity measurement proximal to the AS to calculate LV aortic pressure differences more accurately unless the child is sedated.(ABSTRACT TRUNCATED AT 250 WORDS)

Balloon pulmonary valvuloplasty: results of the Valvuloplasty and Angioplasty of Congenital Anomalies Registry.

Eight hundred twenty-two balloon pulmonary valvuloplasties were reported to the Valvuloplasty and Angioplasty of Congenital Anomalies Registry. Before and after systolic outflow gradients were recorded in 784 valvuloplasties, and the gradient decreased from 71 +/- 33 to 28 +/- 21 mm Hg. The sites of residual obstructions could be ascertained in 196 patients. In these, the total systolic outflow gradients decreased from 85 +/- 41 mm Hg to 33 +/- 27 mm Hg. Of this total residual gradient, 16 +/- 15 mm Hg was transvalvar and 18 +/- 24 mm Hg was infundibular. The degree to which infundibular obstruction subsequently resolved was not determined in this study. The procedure was less effective in reducing outflow gradients in patients with dysplastic valves with or without Noonan's syndrome. There were 5 major complications (0.6%), including 2 deaths (0.2%), a cardiac perforation with tamponade (0.1%) and 2 tricuspid insufficiencies (0.2%). There were 11 minor complications (1.3%) and 21 incidents (2.6%). The incidence of major complications, minor complications and incidents was inversely related to age; it was substantially higher in infants and, in particular, neonates. Balloon pulmonary valvuloplasty is a safe and effective method of lowering pulmonary outflow gradients in infants, children and adults. Small transvalvar and varied infundibular gradients commonly are present at the end of the procedure. Assessing the full effect of the procedure requires intermediate-term follow-up and assessing the duration of relief requires long-term follow-up.

Pulmonary artery conduits in infants younger than six months of age.

Ten infants under 6 months of age with right ventricular-pulmonary artery discontinuity have undergone reparative operations. Five infants had either Type I or Type II truncus and 5 had pulmonary atersia with some discontinuity between right and left pulmonary arteries. There have been 4 survivors in each group. The one death in each category was due to pulmonary hypertension. The remaining 8 patients have low pulmonary vascular resistance. The survival rate after the reparative technique in infancy appears better than that with other forms of palliative treatment, such as banding of shunts, in these severly ill patients.

Successful aortic valvotomy for severe congenital valvular aortic stenosis in the newborn infant.

The preoperative evaluation, surgical course, and early follow-up results of 11 newborn infants less than 30 days of age who underwent aortic valvotomy for severe valvular aortic stenosis between 1976 and 1983 were reviewed to determine our current surgical mortality and the early prognosis of these neonates. Ten of the 11 patients had severe congestive heart failure and dyspnea. Preoperative cardiac catheterization and angiography detected features characteristic of congenital aortic valvular stenosis in newborn infants. Emergency aortic valvotomy was performed in all during cardiopulmonary bypass, for which a cold (4 degrees C) blood prime and moderate systemic hypothermia were used. An effort was made to achieve maximal relief of the stenosis without causing aortic insufficiency. Mean cardiopulmonary bypass time was 21 minutes and mean cross-clamp time was 6.4 minutes. There was a single operative death, and there have been no late deaths during a mean follow-up period of 2.2 years. All patients are currently free of heart failure. Four patients underwent postoperative cardiac catheterization for clinical suspicion of severe residual stenosis. However, three had only mild or moderate residual stenosis. The fourth had a large gradient, 70 mm Hg, and has since undergone successful repeat valvotomy. These results indicate that neonates with severe valvular aortic stenosis can undergo valvotomy safely and have a favorable early prognosis. The factors responsible for the low mortality appear to include prompt recognition and diagnosis followed by emergency operation, use of a 4 degree C cold pump prime, brief cross-clamp times, and conservative valvotomy to avoid the development of significant aortic insufficiency.

Pediatric angiocardiography with iohexol.

A nonionic contrast medium (iohexol) was evaluated for safety and efficacy in pediatric angiocardiography in this study of 15 patients, age 6 to 82 months. Patients carried a preliminary diagnosis of congenital heart disease. Subjects were injected with iohexol, 350 mg iodine/ml of solution with an average volume of 2.46 ml/kg of body weight at a rate of 9 to 14.5 ml/sec. The parameters evaluated included vital signs, intravascular BP, ECG changes, discomfort, and adverse reactions. No adverse reactions were noted in 14 of 15 patients. No significant changes in axillary temperature of ECG were observed. Intravascular blood pressure showed only moderate changes. After 24 hours, creatine phosphokinase (CPK) plasma concentrations increased significantly. Serum electrolytes remained unchanged. Image quality was deemed excellent considering variations in injection site and flow condition of the heart. Iohexol caused remarkably little discomfort and no adverse effects.

Left pulmonary artery kinking caused by outflow tract dilatation after transannular patch repair of tetralogy of Fallot.

BACKGROUND: Stenosis of the branch pulmonary arteries after tetralogy of Fallot repair can result from several mechanisms. In patients with free pulmonary regurgitation and right ventricular dilatation after transannular patch repair, we have observed that the pulmonary outflow tract can dilate and elongate craniad and rotate to the left, resulting in kinking and obstruction of the previously normal left pulmonary artery. METHODS: Ten patients referred for reoperation after tetralogy repair with severe pulmonary regurgitation and right ventricular outflow tract dilatation were found to have left pulmonary artery kinking. In 8 of these patients kinking was the sole or partial cause of left pulmonary artery obstruction, whereas there was no documented obstruction in the other 2. All patients underwent right ventricular outflow tract reconstruction and left pulmonary artery repair with removal of the redundancy at the kink point. Patching alone is not an effective method of repair in this condition, because the mechanism of obstruction is not corrected and the patch material can become redundant and lead to recurrent obstruction. RESULTS: All patients underwent successful pulmonary outflow tract reconstruction. Left pulmonary artery kinking was corrected in all patients, and relief of obstruction was attained in the 8 patients with stenosis. At follow-up ranging from 9 to 58 months, no patient has evidence of recurrent left pulmonary artery stenosis. CONCLUSIONS: Left pulmonary artery kinking should be suspected at long-term follow-up after tetralogy repair in patients with significant pulmonary regurgitation and right-sided dilatation, even if previous evaluations showed no evidence of left pulmonary arterial abnormality. Because unilateral obstruction caused by kinking may lead to asymmetric pulmonary flow, it can exacerbate pulmonary regurgitation and right ventricular dilatation, in effect accelerating the processes that led to kinking in the first place. Kinking can be relieved successfully with the techniques described in this report.

Repair of congenital tricuspid valve abnormalities with artificial chordae tendineae.

BACKGROUND: Congenital abnormalities of the tricuspid valve, including Ebstein's malformation, dysplasia, straddling, and those found in pulmonary atresia with intact septum and congenitally corrected transposition, are an uncommon cause of tricuspid regurgitation. Congenital tricuspid valve anomalies are found as a spectrum of disease in which both the leaflets and the subvalvar apparatus are often involved. Tricuspid valve repair is complicated in such patients because the chordae tendineae are often abnormally short and thick. Replacement or augmentation of chordae tendineae has proved to be a useful component of mitral valve repair. In the present report, we describe the techniques and results of chordal augmentation in the repair of congenital tricuspid valve abnormalities. METHODS: Since July 1992, tricuspid valve repair has been performed in 5 children with severe tricuspid regurgitation secondary to congenital abnormalities of the tricuspid valve with significant chordal pathology. As a component of the repair, chordal replacement or augmentation was performed using expanded polytetrafluoroethylene suture. RESULTS: Intraoperative and postoperative echocardiographic assessment showed good mobility of the tricuspid valve leaflets and trivial to mild tricuspid regurgitation. There were no complications and no early or late mortality. At follow-up of 34 to 60 months (median, 49 months), tricuspid valve function has remained excellent in 4 of the 5 patients. In the remaining patient, progressive regurgitation of the right ventricle to pulmonary artery allograft conduit has led to right ventricular dilatation, with a secondary increase in tricuspid regurgitation from trivial to moderate. CONCLUSIONS: Chordal replacement or augmentation with expanded polytetrafluoroethylene suture is a useful technique in the repair of congenitally dysplastic tricuspid valves with abnormal chordal structures.

Routine primary repair of tetralogy of Fallot in neonates and infants less than three months of age.

BACKGROUND: Although primary repair of tetralogy of Fallot is increasingly undertaken in infancy, complete repair is generally performed in only selected symptomatic neonates. METHODS: From July 1992 through March 1995, 30 consecutive neonates and young infants with tetralogy of Fallot underwent routine primary repair. Group I (n = 10) consisted of patients with tetralogy of Fallot and pulmonary atresia (n = 5) or severe pulmonary stenosis (n = 5) who were duct dependent and were repaired in the neonatal period. Group II (n = 11) consisted of patients who were asymptomatic with arterial oxygen saturation between 75% and 90% (adequate pulmonary blood flow). Group III (n = 9) consisted of patients with "pink" tetralogy of Fallot (arterial oxygen saturation > 90%). Patients in groups II and III were electively scheduled for repair at about 2 months of age. RESULTS: The postrepair peak systolic right ventricular-to-peak systolic left ventricular pressure ratio did not correlate (p = 0.96) with the branch pulmonary artery size. One patient died 2 months after operation, despite good hemodynamics, of uncontrollable diffuse subcutaneous edema due to familial distichiasis lymphedema syndrome. There were no late deaths. At a median follow-up of 19 months, 1 patient underwent balloon dilation of branch pulmonary arteries. Follow-up echocardiography revealed a 30 to 60 mm Hg right ventricle-to-pulmonary artery gradient in 3 patients. CONCLUSIONS: Excellent early and midterm results can be accomplished with routine primary repair of tetralogy of Fallot in early infancy regardless of age, symptomatic status, coronary anatomy, and the size of branch pulmonary arteries as long as they arborize normally.

Surgical management of tetralogy of Fallot with absent pulmonary valve.

Tetralogy of Fallot and absent pulmonary valve (T-APV) is associated with massively dilated pulmonary arteries that cause tracheobronchial compression in the newborn and heart failure and cyanosis in older patients. Corrective operations have been attended by high mortality rates due to pulmonary insufficiency causing right heart failure (RHF) and pulmonary complications. Pulmonic valve insertion (PVI) with complete repair has resulted in improved survival. During the last 5 years, 152 patients with tetralogy were corrected. Of these, 10 patients (ages 51 days to 34 years) had absent pulmonary valve. One patient (age 51 days) presented with severe RHF and pulmonary insufficiency and 9 patients presented with mild RHF and cyanosis. Chest roentgenograms showed increased cardiothoracic ratio and pulmonary prominence in all. Arteriography revealed massively enlarged pulmonary arteries with a mean ratio of 2:1 for size of pulmonary artery to aorta. Associated pulmonic stenosis and insufficiency was present in all. Seven patients underwent closure of ventricular septal defect (VSD) and PVI. Of these, 3 had PVI (2 tissue and 1 prosthetic) with outflow patch and 4 had right ventricle to pulmonary artery (RV-PA) tissue valved conduits. Two patients had repair without PVI, and 1 had repair with a monocusp pericardial valve patch. Nine patients have done well with no episodes of thromboembolism or infection. Death occurred in a 51-day-old infant who had VSD closure and relief of pulmonic stenosis. Pulmonary valve insertion seems to be indicated in these patients because it lowers peak pulmonary artery pressure and, thus, reduces compression effects on the trachea and bronchi. When PVI was used, RHF did not occur post-operatively.