Mucocutaneous lymph node syndrome (Kawasaki disease): delayed aortic and mitral insufficiency secondary to active valvulitis.

A 2 8/12 year old girl with acute mucocutaneous lymph node syndrome (Kawasaki disease), presented 17 months later with a new onset of mitral and aortic insufficiency. Congestive heart failure rapidly developed and double valve replacement was performed. Pathologic study of the excised valve tissue disclosed active valvulitis. This is the first report of late onset aortic and mitral valvulitis associated with Kawasaki disease.

Two-dimensional echocardiographic and angiocardiographic diagnosis of subpulmonary stenosis due to tricuspid valve pouch in complete transposition of the great arteries.

Two-dimensional echocardiograms and angiocardiograms were obtained in six infants and children, each with complete transposition of the great arteries, a ventricular septal defect and subvalvular pulmonary stenosis. In each case, the subpulmonary stenosis resulted from redundant tricuspid valve tissue which protruded through the ventricular septal defect and into the left ventricular outflow tract. Angio-echocardiographic correlations demonstrated features that allowed preoperative recognition of this unusual type of subpulmonary stenosis. The left ventricular angiogram in an anteroposterior orientation revealed a characteristic filling defect in the outflow tract during systole in each patient. Echocardiographic images of the left ventricular outflow tract and of the tricuspid valve demonstrated subpulmonary stenosis caused by protruding redundant tricuspid valve tissue (resembling a "pouch") in all six patients. These findings were contrasted with those from three patients with normally related great arteries and a similar, but nonobstructive, tricuspid valve pouch. Echocardiographic and angiocardiographic examination and correlation allowed preoperative diagnosis of this unusual form of subpulmonary stenosis in complete transposition of the great arteries. This is especially important because limited left ventricular intraoperative exploration may fail to reveal subpulmonary obstruction as the pouch may be flaccid in a relaxed arrested heart.

Successful surgical repair of aortic atresia associated with normal left ventricle.

Surgical repair of aortic atresia with a large ventricular septal defect and a normally developed left ventricle was accomplished in a 14 month old infant. Palliative surgery at age 23 days consisted of bilateral banding of pulmonary artery branches and replacement of the ductus arteriosus with a Goretex conduit. Corrective surgery was accomplished by closure of the ventricular septal defect, insertion of a valved conduit between the apex of the left ventricle and the subdiaphragmatic aorta, removal of the pulmonary artery bands and division of the pulmonary-aortic conduit. The patient has a mild coarctation of the aorta and remains asymptomatic at 2 years of age.

Two dimensional echocardiography in evaluation of right atrial masses: five cases in pediatric patients.

Five unusual cases of a right atrial mass in children are described to illustrate the very valuable contribution that two dimensional echocardiographic examinations can bring to both the initial diagnosis and the subsequent management of patients with these findings. One patient had a large benign hemangioendothelioma of the right atrium. Two infants had extension of a Wilms' tumor from the kidney by way of the inferior vena cava to the right atrium. A fourth patient, an 8 year old girl, had no cardiac disease, and manifested Staphylococcus aureus endocarditis of the tricuspid valve with a large pedunculated mass and subsequent pulmonary embolus. A fifth patient, a premature infant with a central hyperalimentation catheter in the right atrium, had a large thrombus on the catheter that was successfully eradicated with urokinase-induced thrombolysis. Two dimensional echocardiography provides real time imaging of the entire right atrium, interatrial septum, inferior and superior venae cavae and tricuspid orifice and hence is valuable in the diagnosis and management of these clinical problems.

Masked subaortic stenosis in ostium primum atrial septal defect: recognition and treatment.

Five patients with ostium primum atrial septal defect (ASD) and a cleft mitral valve had no hemodynamic evidence of left ventricular (LV) outflow tract obstruction on preoperative cardiac catheterization. After surgical closure of the ASD and repair of the mitral cleft, all 5 patients manifested subaortic stenosis with pressure gradients ranging from 10 to 120 mm Hg. Postoperative LV angiograms revealed systolic narrowing of the outflow tract, and the same outflow tract dynamics were recognized on reviewing the preoperative angiograms and echocardiograms. Persistence or exaggeration of the characteristic diastolic "goose-neck" deformity during LV systole in atrioventricular canal defects is diagnostic of a potential or actual subaortic obstruction. This diagnostic sign is also readily recognizable by 2-dimensional echocardiography, and when present, the surgeon should be alerted to explore the LV outflow tract because the outflow tract anatomy is not readily apparent at operation aimed solely at closing the ASD and repairing the cleft mitral valve.

Development of neo-coarctation in patients with transposed great arteries and hypoplastic aortic arch after Lecompte modification of anatomical correction.

We report on three patients with kinking in the proximal aortic arch that developed after Lecompte modification of the arterial switch operation. Two patients had a previous subclavian patch repair of coarctation of the aorta and had an associated hypoplasia of the transverse aortic arch, and one patient had hemodynamically mild coarctation at the anatomical repair. A severe pressure gradient across the kinked area ("neo-coarctation") necessitating reoperation developed in one patient. The acute arch angulation appears to be due to an excessive posterior displacement of the ascending aorta by the anterior relocation of either the right or left main pulmonary artery branch from underneath the aortic arch. A foreshortened and frequently hypoplastic transverse aortic arch, a common association with coarctation of the aorta, appears to be especially vulnerable to the development of "neo-coarctation" after the Lecompte modification of the anatomical repair of transposed great arteries.

Transmediastinal repair of complex coarctation and interrupted aortic arch.

In the past 2 years, eight patients have had repair of coarctation of the aorta or interrupted aortic arch along with an intracardiac procedure performed through a simple midsternotomy. Five underwent total repair and three had palliation for the intracardiac lesion. Four patients did well (including one who required a resection for recoarctation), two died intraoperatively, and two died postoperatively (11 and 21 days), one of them from clostridial sepsis and the other from cerebral anoxia that followed a cardiac arrest precipitated by a massive hemoptysis. We found that with this approach the aortic obstruction could be readily relieved and the intracardiac procedure carried out. Since the majority of these patients have compromised hemodynamic status, the ease of instituting immediate bypass, avoidance of a separate incision, and feasibility of total repair are major advantages.

Vascular anomalies causing tracheoesophageal compression. Review of experience in children.

Two hundred four infants and children (mean age 13 months) have undergone operation for the relief of tracheoesophageal obstruction resulting from vascular anomalies. One hundred thirteen patients had complete vascular rings (group I), 61 with double aortic arch and 52 with right aortic arch with a left ligamentum. Nine patients had a pulmonary artery sling (group II), 71 had innominate artery compression (group III), and 11 had miscellaneous anomalies (group IV). Patients were admitted with respiratory distress, stridor, apnea, dysphagia, or recurrent respiratory infections. Diagnosis was established by barium esophagogram in group I; barium esophagogram, bronchoscopy, and computed tomography or angiography in group II; bronchoscopy in group III; and barium esophagogram or angiography in group IV. The operative approach was through a left thoracotomy in group I, II and IV (93% of these patients) and through a right thoracotomy for group III (96% of these patients). The operative mortality rate was 4.9% and there were seven late deaths (3.4%). There have been no operative deaths in patients with isolated vascular anomalies in the past 28 years. Follow-up data from 1 month to 20 years (mean 8.5 months) were available on 159 patients; 141 (92%) were essentially free of symptoms, and 12 (8%) had residual respiratory problems. Five of six patients in group II having a lung scan postoperatively had a patent left pulmonary artery. A strong index of suspicion is necessary to avoid the complications of vascular rings in children. Barium swallow is the best single diagnostic technique for patients with complete vascular rings. A bronchoscopic study is required to diagnose innominate artery compression. Angiograms or computed tomographic scans are used to confirm the diagnosis of pulmonary artery sling. Left thoracotomy provides excellent exposure for all vascular rings except the displaced innominate artery, for which a right thoracotomy is the best approach.

Extended aortic valvuloplasty for recurrent valvular stenosis and regurgitation in children.

Recurrent significant aortic valvular stenosis or regurgitation, or both, after balloon or open valvotomy in pediatric patients often necessitates aortic valve replacement. In an attempt to preserve the aortic valve, we performed extended aortic valvuloplasty in 21 children with recurrent aortic valve stenosis or regurgitation from January 1989 to March 1993. Previous related procedures were one open aortic valvotomy or more (n = 15), balloon valvotomy (n = 4), balloon valvotomy after surgical valvotomy (n = 1), and repair of iatrogenic valve tear (n = 1). Mean age at the time of the extended aortic valvuloplasty was 6 +/- 3.4 years. Mean pressure gradient across the aortic valve was 56 +/- 12 torr. Regurgitation was moderate (grade 2 to 3) in nine and severe (grade 4) in 12 patients. Extended aortic valvuloplasty techniques consisted of thinning of valve leaflets (n = 15), augmentation of scarred and retracted leaflets with autologous pericardium (n = 11), resuspension of the augmented leaflet (n = 14), release of the rudimentary commissure from the aortic wall (n = 5), extension of the valvotomy incision into the aortic wall on both sides of the commissure (n = 20), patch repair of the sinus of Valsalva perforation (n = 1), reapproximation of tears (n = 5), and narrowing of the ventriculoaortic junction (n = 2). No operative deaths occurred. The postoperative mean pressure gradient, assessed by most recent Doppler echocardiography or cardiac catheterization at a follow-up of 18 +/- 6 months, was 19 +/- 6 torr (p < 0.01 versus the preoperative gradient). Aortic regurgitation was absent in 13, mild in 6, and moderate-to-severe, necessitating subsequent aortic valve replacement, in 2. This short-term experience indicates that extended aortic valvuloplasty is a safe and effective surgical approach that minimizes the need for aortic valve replacement in children with significant recurrent aortic valve stenosis or regurgitation.

Dose dependency of L-arginine in neonatal myocardial protection: the nitric oxide paradox.

OBJECTIVES: Recent experimental studies have suggested that enriching cardioplegic solution with L-arginine improves myocardial protection by increasing nitric oxide production. Nitric oxide, however, also generates the toxic oxygen-derived free radical peroxynitrite; thus these beneficial effects may be dose dependent, especially in vulnerable (stressed) hearts. METHODS: Fifteen neonatal piglets underwent 60 minutes of ventilator hypoxia (inspired oxygen fraction 8%-10%) followed by 20 minutes of normothermic ischemia on cardiopulmonary bypass (stress). They were then protected for 70 minutes with multiple doses of blood cardioplegic solution. In 5 (group 1), the cardioplegic solution contained no L-arginine, in 5 (group 2), it was enriched with a 4 mmol/L concentration of L-arginine, and in 5 (group 3), a 10 mmol/L concentration of L-arginine. Myocardial function was assessed by means of pressure volume loops and expressed as a percentage of control, and coronary vascular resistance and conjugated diene production were measured during infusions of cardioplegic solution. RESULTS: Compared with the protection afforded by blood cardioplegic solution without L-arginine (group 1), the addition of a 4 mmol/L concentration of L-arginine (group 2) significantly improved myocardial protection, resulting in complete return of systolic function (end-systolic elastance 38% vs 100%; P <.001 vs 4 mmol/L L-arginine) and preload recruitable stroke work (40% vs 100%; P <. 001 vs 4 mmol/L L-arginine); minimal increase in diastolic stiffness (239% vs 158%; P <.001 vs 4 mmol/L L-arginine); and lower coronary vascular resistance, conjugated diene production, and myeloperoxidase activity (P <.001 vs 4 mmol/L L-arginine in each case). Conversely, supplementing the cardioplegic solution with a 10 mmol/L dose of L-arginine (group 3) negated these beneficial effects, resulting in depressed systolic function (end-systolic elastance 41% +/- 2%; P <.001 vs 4 mmol/L L-arginine) and preload recruitable stroke work (40% +/- 2%; P <.001 vs 4 mmol/L L-arginine); increased diastolic stiffness (246% +/- 7%; P <.001 vs 4 mmol/L L-arginine); and higher conjugated diene production, myeloperoxidase activity, and coronary vascular resistance (P <.001 vs 4 mmol/L L-arginine in each case). CONCLUSIONS: Enriching cardioplegic solution with a 4 mmol/L concentration of L-arginine significantly improves myocardial protection by reducing oxygen-derived free radical formation by white blood cells, thus preserving vascular and myocardial function. However, these beneficial effects are dose dependent because 10 mmol/L concentrations of L-arginine increase oxygen-derived free radical production, resulting in vascular and myocardial dysfunction.

L-arginine, prostaglandin, and white cell filtration equally improve myocardial protection in stressed neonatal hearts.

OBJECTIVES: L-Arginine may improve cardioplegic protection by increasing nitric oxide production. However, L-arginine may also be detrimental because it generates the free radical peroxynitrite. It would, therefore, be advantageous if the benefits of L-arginine could be achieved by another means. METHODS: Twenty neonatal piglets underwent 60 minutes of ventilator hypoxia (inspired oxygen fraction 8%-10%) followed by 20 minutes of ischemia on cardiopulmonary bypass (stress) and were then protected for 70 minutes with multiple doses of blood cardioplegic solution. In 5 piglets (group 1), the cardioplegic solution was not modified; in 5 (group 2), low-dose L-arginine (4 mmol/L) was added; in 5 (group 3), prostaglandin E(1) (alprostadil, 4 microgram/L) was added; and in 5 (group 4), the cardioplegic solution was passed through a leukodepleting filter. Myocardial function was assessed by pressure volume loops and expressed as percentage of control, and coronary vascular resistance was measured with each cardioplegic infusion. RESULTS: Unmodified blood cardioplegic solution (group 1) was unable to protect the severely stressed myocardium, resulting in depressed systolic function (39% +/- 1%) and preload recruitable stroke work (40% +/- 1%), increased diastolic stiffness (239% +/- 3%), and high conjugated diene production, myeloperoxidase activity, and coronary vascular resistance. In contrast, cardioplegic solutions modified with L-arginine, prostaglandin E(1), or leukodepletion, resuscitated the stressed myocardium, resulting in complete return of systolic function (100% vs 101% vs 101%; P <.001 vs group 1) and preload recruitable stroke work (100% vs 101% vs 101%; P <.001 vs group 1), minimal increase in diastolic stiffness (160% vs 162% vs 160%; P <. 001 vs group 1), and lowered conjugated diene production, myeloperoxidase activity, and coronary vascular resistance (P <.001 vs group 1 for each). CONCLUSIONS: (1) Unmodified blood cardioplegic solution is unable to protect the severely stressed myocardium. (2) L-Arginine, prostaglandin E(1), and leukocyte filtration all improve myocardial protection equally and appear to work by limiting a white blood cell-mediated injury. This reduces oxygen-derived free radical formation, maintains vascular function, and restores functional recovery. Since L-arginine may be detrimental, surgeons should consider using prostaglandin E(1) and/or a leukocyte filter instead.

Preparation of the left ventricle for anatomical correction in patients with simple transposition of the great arteries. Surgical guidelines.

Pulmonary artery banding in combination with an aortopulmonary shunt was performed on 16 patients with simple transposition of the great arteries to prepare the left ventricle for anatomical correction. Three groups were identified after operation: Group I (four patients) had increased pulmonary blood flow and tight pulmonary artery banding; Group II (four patients) had increased pulmonary blood flow and moderate pulmonary artery banding; Group III (eight patients) had normal pulmonary blood flow and moderate pulmonary artery banding. Postoperative low cardiac output was present in all patients in Group I, whereas mild heart failure was present in two patients in Group II and in two in Group III. There was one hospital death (6%). The follow-up period was 125 patient-months. Left ventricular systolic pressure rose from 63 +/- 11 torr before the operation to 101 +/- 35 torr after the procedure in Group I (p less than 0.05), from 59 +/- 10 to 93 +/- 33 torr in Group II (p less than 0.05), and from 55 +/- 10 to 84 +/- 16 torr in Group III (p less than 0.005). The increase in left ventricular muscle mass was from 44 +/- 2 gm/m2 preoperatively to 108 +/- 12 gm/m2 after operation in Group I (p less than 0.01), from 43 +/- 3 to 93 +/- 8 gm/m2 in Group II (p less than 0.02), and from 46 +/- 3 to 55 +/- 14 gm/m2 in Group III (p = no statistically significant difference). The postoperative change in left ventricular end-diastolic volume was from 100% +/- 17% to 133% +/- 23% of normal in Groups I and II (p less than 0.05) and from 123% +/- 29% to 107% +/- 36% of normal in Group III (p = no statistically significant difference). In preparing the left ventricle for anatomical correction, avoidance of severe pulmonary artery banding decreases the incidence of postoperative myocardial dysfunction, a moderate degree of volume overload and pulmonary artery banding provides the most effective stimulus for ventricular growth, and a small to moderate atrial septal defect is advantageous because it ensures the volume preload necessary for the development of the left ventricle.

Transposition of the great arteries with intact ventricular septum. Arterial switch in the first month of life.

Twenty-three infants with simple transposition of the great arteries and intact ventricular septum were operated on from October 1983 to October 1986. The age at operation in 22 infants ranged from 2 to 21 days and in one was 35 days (mean 9.82 +/- 6.86 days). The infants were evaluated with cardiac catheterization at 1 to 27 days of age. Twenty-two infants had balloon atrial septostomy, and 22 received prostaglandin E1 infusion. The left ventricular diastolic wall thickness, assessed by M-mode echocardiograms, varied between 2.8 and 4 mm. There were two hospital deaths in this group of 23 infants (mortality 8.6%), and there were no late deaths. All surviving patients are doing well clinically. One patient had asymptomatic nonsustained ventricular tachycardia necessitating phenytoin. Postoperative echocardiographic assessment performed on 15 patients at 0.93 +/- 0.61 years of age and cardiac catheterization and angiographic studies on seven patients at 1.07 +/- 0.13 years after operation revealed excellent ventricular performance, good semilunar valve function, and mild gradient at the right ventricular outflow with a mean right ventricular pressure of 37.4 +/- 4.1 torr.

Arterial switch in simple and complex transposition of the great arteries.

Arterial switch for repair of transposition of the great arteries was performed on 53 patients since October 1983. These patients were divided into three groups: group I, 25 infants with an intact ventricular septum who had primary repair in the first month of life (2 to 34 days of age, mean 9.7 +/- 6.6); group II, 13 patients with an intact ventricular septum who had anatomic repair after a preliminary procedure (pulmonary artery banding in 13, shunt in 10, atrial septectomy in 1); and group III, 15 infants with transposition of the great arteries and ventricular septal defect. In group III, six patients had Taussig-Bing abnormality, nine had previous pulmonary artery banding, three had coarctation of the aorta repaired earlier in life, and four were less than 2 weeks old. Overall early mortality was 9.4% (5/53: group I 8%, group II 7.6%, group III 13.3%). Two late deaths occurred in group II 10 and 12 weeks postoperatively after infection and high fever. A third late death 18 weeks postoperatively was due to aspiration in an infant with Goldenhar's syndrome. Mortality and morbidity decreased significantly after an initial learning period (no deaths from July 1985 to March 1987 overall, and none in the last 15 infants operated on in group I). The surviving 45 patients are doing well. All have normal sinus rhythm. Two had transient asymptomatic arrhythmias. Left and right ventricular function assessed by echocardiogram and postoperative cardiac catheterization were within normal ranges in all but two patients, one with pulmonary artery stenosis and one (Taussig-Bing abnormality with two large ventricular septal defects) with severe pulmonary vascular disease (9.6 units) observed before anatomic repair. The right ventricular pressure at catheterization ranged from 27 to 42 mm Hg in 12 patients and was 55 mm Hg in two. There was no aortic stenosis. Aortic insufficiency was trivial in three patients and mild in one. We conclude that excellent results can be obtained with arterial switch for transposition of the great arteries with or without ventricular septal defect, especially in neonates.

Modified Blalock-Taussig shunt in newborn infants.

The modified Blalock-Taussig shunt, interposing an expanded polytetrafluoroethylene graft between the subclavian and pulmonary arteries, was performed in 30 neonates with a mean age of 8.8 days and a mean weight of 3.14 kg. Underlying lesions included severe tetralogy of Fallot or its variant (N = 10), transposition complex (with pulmonary stenosis or atresia) (N = 6), single ventricle equivalents (with pulmonary atresia or stenosis) (N = 9), and pulmonary atresia with intact ventricular septum (N = 5). The mean preoperative arterial oxygen tension prior to prostaglandin E1 therapy was 29.5 torr. The shunt was performed through a right thoracotomy in 18 patients, through a left thoracotomy in nine, and through a median sternotomy in three. A 5 mm graft was used in 21 patients and a 6 mm graft in nine patients. The mean postoperative arterial oxygen tension was 64.1 torr (p less than 0.001). The incidence of early shunt occlusion was 3.3% and the hospital mortality was 3.3%. Actuarial functional life of the shunt (no death or reoperation related to shunt failure) was 91% at 3 years' follow-up. Nine patients were recatheterized. There was no distortion of the pulmonary artery. The ratios of the diameter of the right pulmonary artery and pulmonary valve anulus to that of the descending aorta increased after the operation by 50% (p less than 0.001) and 52% (p less than 0.05), respectively. Our experience indicates that the modified Blalock-Taussig shunt has an excellent function, offers several technical advantages, and lacks most of the drawbacks of other systemic-pulmonary artery shunts. It may be the shunt of choice in patients less than 1 month of age.

The surgical management of left ventricular outflow tract obstruction due to tricuspid valve pouch in complete transposition of the great arteries.

Subpulmonary stenosis in transposition of the great arteries, resulting from a tricuspid valve pouch bulging into the left ventricular outflow tract through a ventricular septal defect, can be missed at the time of operation in the flaccid, nonbeating heart unless preoperative diagnosis has been established. In our experience, six patients were found to have this lesion. In four patients the tricuspid valve pouch was recognized preoperatively. At operation, retraction of the tricuspid valve pouch into the right ventricle, patch closure of the ventricular septal defect, and a Mustard procedure were performed in three patients; the fourth is awaiting correction following initial palliation with a subclavian-pulmonary shunt. In the other two, the ventricular septal defect was partially or completely obliterated by a tricuspid valve pouch that was missed preoperatively and during exploration at the time of the Mustard procedure. Residual left ventricular outflow tract obstruction was subsequently corrected with a left ventricle-pulmonary artery valved conduit. Echocardiographic and angiocardiographic examinations offer helpful signs for the diagnosis of tricuspid valve pouch. Transatrial retraction of the redundant tricuspid valve tissue into the right ventricle, patch closure of the ventricular septal defect, and Mustard operation are the procedures of choice. A left ventricle-pulmonary artery valved conduit may be required for residual unresectable left ventricular outflow tract obstruction.

Tracheoplasty with pericardial patch for extensive tracheal stenosis in infants and children.

Five infants with long tracheal stenosis were operated upon by means of a pericardial patch tracheoplasty. The approach was through a median sternotomy with extracorporeal circulation for respiratory support. In four, the obstruction was due to complete rings; in the other, there was an associated tracheal trauma which had occurred during resuscitation. This patient requires prolonged stenting with a tracheostomy tube. All others are asymptomatic postoperatively, with the longest follow-up being 22 months. There were no deaths or infections. We conclude from this experience that median sternotomy provides an excellent approach to the trachea, that autogenous pericardium is advantageous, and that there is no need for prolonged tracheal stenting in most patients.

Technical modifications for improved results in total anomalous pulmonary venous drainage.

To delineate factors that contribute to improved surgical outcome in patients with total anomalous pulmonary venous drainage, we reviewed the records of 52 consecutive patients. Venous drainage was supracardiac in 25 (48%), cardiac in 12 (23%), infracardiac in 10 (19%), and mixed in five (10%). Preoperative pulmonary venous obstruction was present in 18 patients (35%). Median age at the time of repair was 35 days and weight, 3.7 kg. Repair was performed with deep hypothermia, low-flow cardiopulmonary bypass, and occasional short periods of circulatory arrest. In patients with coronary sinus drainage, the veins were tunneled to the left atrium through an enlarged atrial septal defect, with a mortality of 8% (1/12) and no postoperative stenosis. The approach in patients with supracardiac, infracardiac, and mixed drainage varied with time. In 16 patients, the condition was managed by apical or right-sided exposure of the common vein, anastomosis of the common vein to the left atrium with continuous sutures, and primary closure of the atrial septal defect (type I repair). In the other 24 patients the common vein was approached from the right side through the right atrium and the interatrial septum. Common vein-left atrium anastomosis was performed with interrupted sutures and a piece of pericardium used to augment the anastomosis, prevent common vein distortion, and close the atrial septal defect (type II repair). Mortality in type I repair was 25% (4/16) and in type II repair, 4% (1/24). Follow-up was 7.86 +/- 4.0 years with no late deaths. Postoperative stenosis occurred in five of 14 (36%) patients who had type I repair versus two of 23 (9%) who had type II repair. Multivariate analysis showed that type I repair was a positive risk factor for hospital mortality (p = 0.05) and restenosis (p = 0.04). The technique of transatrial exposure of the common venous chamber, interrupted suturing of the common vein to the left atrium, and pericardial patch augmentation significantly improves survival and decreases risk of restenosis.

Factors that exaggerate the deleterious effects of pulmonary insufficiency on the right ventricle after tetralogy repair. Surgical implications.

Postoperative cardiac catheterization data of 74 patients with pulmonary insufficiency after tetralogy repair were analyzed. Two groups were identified: Group A, 26 patients with normal right ventricular function (ejection fraction 95% +/- 5.5%, end-systolic volume 110% +/- 17% of predicted normal) and Group B, 48 patients with right ventricular dysfunction (ejection fraction 80% +/- 18% [p less than 0.001], and end-systolic volume 218% +/- 75% of predicted normal [p less than 0.001]). There was no significant difference between the two groups with respect to frequency of previous palliative procedures, age at operative repair, operative techniques, methods of myocardial protection, and follow-up period. Right ventricular dysfunction in Group B was associated with significant distal pulmonary stenosis (right ventricle-pulmonary artery pressure gradient 28 +/- 13 torr in Group A versus 55 +/- 20 torr in Group B, p less than 0.001), moderate pulmonary regurgitation (regurgitant fraction 18% +/- 11% in Group A versus 32% +/- 10% in Group B, p less than 0.001), and large transannular outflow patch (ratio of patch diameter to descending aorta diameter 1.31 +/- 0.16 in Group A versus 2.50 +/- 0.28 in Group B, p less than 0.001). Pulmonary valve insertion was performed in 42 patients in Group B. Eighteen had subsequent cardiac catheterization. Right ventricular function recovered completely (end-systolic volume 122% +/- 24%, and ejection fraction 92% +/- 7% of predicted) in five of six patients (83%) who had valve insertion within the first 2 years after tetralogy repair. In contrast, right ventricular function remained abnormal in all 12 patients who had valve insertion later than 2 years after tetralogy repair (p less than 0.05). Patients with residual pulmonary stenosis and/or a large transannular outflow patch are at risk for the development of right ventricular dysfunction from pulmonary insufficiency after tetralogy repair. Early correction of these residual lesions and control of pulmonary insufficiency may prevent long-term deterioration in right ventricular function.

Experience with St. Jude Medical valve prosthesis in children. A word of caution regarding right-sided placement.

Thirty-six children aged 6 months to 18 years, underwent insertion of 37 St. Jude Medical cardiac prostheses. In 20, the valve was placed in the aortic or mitral position, and in 16 in the pulmonary or tricuspid position. There was one (2.8%) hospital death. All patients received maintenance doses of salicylates and dipyridamole after the operation. Follow-up data are available for all patients for 12 to 24 postoperative months. There was no incidence of valve dysfunction or thromboembolic complication in any of the 20 patients with valves in the systemic (left) side of the circulation, and all manifested improvement in their functional class. In contrast, six (37%) of the 16 patients with valves in the pulmonary (right) side of the circulation developed dysfunction of the prosthesis 1 to 6 months after insertion. Prosthesis failure was associated with fibrous tissue growing into the struts, leading to leaflet immobilization. At 2 years, the actuarial functional life was 100% for mitral and aortic valves and 70% for pulmonary and tricuspid valves. The data illustrate the excellent hemodynamic function of the St. Jude Medical valve in children. The absence of thromboembolic complications warrant continued implantation of the prosthesis in the left side without warfarin anticoagulation therapy, but the high incidence of valve dysfunction in the pulmonary position does not justify its continued use in the right side.