Emergency ligation of anomalous left coronary artery arising from the pulmonary artery.

We report two cases of successful emergency ligation of anomalous left coronary artery arising from the pulmonary artery (ALCAPA) in patients with previous cardiac arrest. Both patients had regained marginal cardiac output after cardiopulmonary resuscitation and had maximal doses of inotropic support. The ALCAPA ligation was then performed as a life-saving procedure in the absence of any kind of mechanical circulatory support.

Five- to fifteen-year follow-up of fresh autologous pericardial valved conduits.

OBJECTIVE: Evaluate long-term results of autologous pericardial valved conduits in the pulmonary outflow. METHODS: Between June 1983 and October 1993, 82 conduits were placed in the outflow of the venous ventricle. Patients who received homografts (n = 2 patients), heterografts (n = 3 patients), and valveless conduits (n = 19 patients) and those patients who died within 90 days after the operation were excluded. Fifty-four survivors of pulmonary outflow reconstruction with fresh autologous pericardial valved conduits were followed up from 5 to 15 years (mean, 7.47 +/- 2.8 years). Diagnosis include d -transposition of great arteries (n = 16 patients), L -transposition of great arteries (n = 14 patients), tetralogy of Fallot, pulmonary atresia with ventricular septal defect (n = 11 patients), truncus arteriosus (n = 10 patients), and double-outlet ventricle (n = 3 patients). Implantation age ranged from 0.25 to 24 years (mean, 5.2 +/- 4.2 years). Median conduit diameter was 16 mm. Two-dimensional echocardiographic Doppler evaluations were made yearly; 9 patients underwent cardiac catheterization. Reintervention for stenosis was indicated when the pressure gradient exceeded 50 mm Hg. RESULTS: Three late deaths were unrelated to the conduit. Thirty-five autologous pericardial valved conduits increased in diameter (1-7 mm), remained unchanged in 15 patients, and reduced 1 to 2 mm in 4 patients. The median diameter was 18 mm at the last evaluation (P =.0001). Eight patients required conduit-related reoperation 3 to 8 years after the implantation. Two patients underwent balloon dilation of the autologous pericardial valved conduit. No conduit had to be replaced. Freedom from reintervention at 5 and 10 years was 92% and 76%, being 100% at 10 years for conduits larger than 16 mm at time of implantation. CONCLUSIONS: Autologous pericardial valved conduits show excellent long-term results and compare favorably with other conduits.

[Evaluation of the complications of different types of right ventricular bypass by contrast echocardiography]. / Evaluación de las complicaciones de los diferentes bypass del ventrículo derecho por ecocardiografía con contraste.

We evaluated 54 patients with different right heart by-pass by means of contrast echocardiography with rapid venous injection of shaken saline (3 cc, < 20 k; 6cc, > 20 k), in order to detect venous collateral circulation in partial by-pass, pulmonary arteriovenous fistulae in partial and total by-pass, and right-to-left shunt in total by-pass. Forty three patients had partial by-pass: 29 had a bidirectional cavopulmonary anastomosis with additional pulmonary flow (systemic-pulmonary anastomosis and/or restrictive anterograde ventricular flow); 10 had a partial ventricular correction (bidirectional cavopulmonary anastomosis and non restrictive anterograde ventricular flow; two had classical Glenn procedures; two had Kawashima operations (bidirectional cavopulmonary anastomosis and non restrictive anterograde ventricular flow; two had classical Glenn procedures; two had kawashima operations (bidirectional cavopulmonary anastomosis with inferior vena cava interruption), and 11 with total by-pass (10 atriopulmonary anastomosis and 1 with total cavopulmonary anastomosis). The age ranged from 2.5 to 33 years (x = 12.2 years), and the mean postoperative period was 4.3 years. Venous collateral circulation: 32/43 patients (74%) with partial by-pass, specially in those without partial biventricular correction: 29/33 patients (88%) compared to those with partial biventricular correction: 3/10 (30%) p < 0.01. Pulmonary arteriovenous fistulae: 6/43 patients (14%) with partial by-pass; 6/33 (18%) with partial biventricular correction, 0/10 patients with partial biventricular correction, and 0/11 with total by-pass. Right-to-left shunt: 2/11 patients (18%) with total by-pass, all of them with atriopulmonary anastomosis. Contrast echocardiography is an excellent noninvasive method for the initial evaluation of specific dysfunctions of different right heart by-pass. We emphasize the higher frequency of collateral venous circulation in cavopulmonary anastomosis, that would explain the dysfunction with progressive hypoxia in the evolution of these patients. Pulmonary arteriovenous fistulae were detected only in partial by-pass, without partial biventricular correction (p < 0.01). The minor frequency of these fistulae in bidirectional cavopulmonary anastomosis would be due to additional pulmonary flow.

Experience with one and a half ventricle repair.

OBJECTIVE: This article presents a 10-year experience with one and a half ventricle repair for right ventricular hypoplasia or dysfunction. METHODS: From November 1986 to December 1996, 30 patients (mean age 6.7 +/- 8.5 years, range 4 months-40 years) with functionally abnormal right ventricles underwent a bidirectional Glenn shunt as part of the repair. Diagnoses included pulmonary atresia with intact ventricular septum (n = 15), Ebstein anomaly (n = 5), levotransposition of the great arteries (n = 3), pulmonary stenosis with right ventricular hypoplasia (n = 2), tetralogy of Fallot (n = 3), dextrotransposition of the great arteries (n = l), and Uhl anomaly (n = l). Concomitantly performed cardiac procedures included atrial septal defect closure (n = 27), fenestration of the atrial septum (n = 2), right ventricular cavity augmentation (n = 8), right ventricular outflow tract enlargement (n = 6), transannular patch (n = 13), modified Blalock-Taussig shunt closure (n = 16), tricuspid replacement (n = 3), tricuspid repair (n = 2), Rastelli procedure (n = 3), tricuspid commissurotomy (n = 2), and double switch (n = l). RESULTS: There were 2 early deaths (6.6%) and 1 late death. Mean early postoperative superior vena caval pressure was 14. 12 +/- 3.55 mm Hg and mean right atrial pressure was 10.3 +/- 5.16 mm Hg. Early oxygen saturation in the operating room with an inspired oxygen fraction of 1 was 97.2 +/- 2.5; oxygen saturation was 92.3 +/- 4.8 on room air at discharge. Mean oxygen saturations were 93.6% +/- 3.6% at 1 year of follow-up (P =.10) and 93.5% +/- 4. 1% at 5 years (P =.12). Overall survival was 90% at 5 years, and 21 patients (77%) were in New York Heart Association class I, 5 (18%) were in class II, and 1 (2.7%) was in class III. CONCLUSION: This procedure provides a valid alternative for correction of right ventricle hypoplasia or dysfunction. Early and intermediate follow-up results compare favorably with those of the Fontan procedure, but long-term follow-up is needed.

Preoperative management of congestive heart failure in neonates: the closed hood.

In this study we report the results of the use of a closed hood with no external administration of CO2 to increase pulmonary vascular resistance by lowering the inspired fraction of oxygen (FiO2) and raising the inspired fraction of carbon dioxide (FiCO2) in patients with congenital heart disease and increased pulmonary blood flow. Between December 1995 and May 1996, 9 neonates (F:5, M:4) were admitted. Each study patient was assigned to clinical classes using a 1 to 4 classification. Ages ranged between 2 and 30 days (mean 18), weight between 2.25 and 3.65 kg (mean 2.89). A plastic hood, closed on the top with a plastic membrane and with the gas entrance open to room air was placed over the head of the patients. Patients increase pCO2 by rebreathing their own expired CO2. After 24 h of the onset of the treatment the media of points of congestive heart failure 1 to 4 classification decrease from a mean of 4 to a mean of 2.28+/-0.44 (p=0.001). A statistically significant improvement in symptoms and lowering of PO2 and pH while raising pCO2 has been demonstrated in this study.

Cavo atriopulmonary anastomosis via a nonprosthetic medial tunnel.

A surgical technique is described to perform a total bypass of the venous ventricle (TBPVV) via a cavo atriopulmonary anastomosis wherein a medial atrial tunnel is constructed using autologous tissue. The procedure offers the advantage of maintaining low atrial pressure at the sinus node area without the use of prosthetic material. It also represents a good method for conversion of a bidirectional Glenn to a TBPVV avoiding surgical damage of the sinus node area.

Long-term follow-up of autologous pericardial valved conduits.

BACKGROUND: The aim of this study was to evaluate the long-term results of the use of an autologous pericardial valved conduit in the outflow tract of the venous ventricle in congenital heart malformations. METHODS: Fifty-one patients were followed up for a period of 12 to 120 months; 30 for more than 36 months and 13 for more than 72 months. All were evaluated clinically and by two-dimensional and Doppler echocardiography. Eight patients were recatheterized. Postoperative evaluation included serial measurement of pressure gradients and the conduit's diameter at the proximal, valvular, and distal levels. Reoperation because of stenosis was indicated when the gradient across the right ventricular outflow was greater than 50 mm Hg. The reoperation rate in relation with postoperative time, diameter of the autologous pericardial valved conduit at the time of implantation, and malformation was statistically analyzed. RESULTS: In 27 patients the conduit increased its diameter 1 to 7 mm. In 20 patients the diameter remained unchanged, whereas a reduction was noted in 4. Conduit survival free of reoperation for the whole group was 89.9% at 5 years. Conduit survival free of reoperation was 100% at 5 and 7 years for conduits larger than 16 mm at the time of implantation. It was 95% (standard deviation = 4.8%) at 5 years and 72.3% at 7 years for those 16 mm or less. For patients operated after January 1, 1986 (technical modification), conduit survival free of reoperation was 95.4% at 7 years postoperatively. CONCLUSIONS: These results compare favorably with those of other available conduits.

A comparative long-term follow-up of the results of anterior and posterior approaches in bypassing the rudimentary right ventricle in patients with tricuspid atresia.

We have studied 44 patients with classical tricuspid atresia who underwent radical surgery between 1971 and 1985. Bypassing of the incomplete and rudimentary right ventricle was performed, in anterior fashion, in 17 patients operated on from 1971 to 1980. Subsequently, from 1980 to 1985, we used a technique of posterior retroaortic atriopulmonary anastomosis. This was undertaken in 27 patients. We have now compared the results in the two groups. Hospital and late mortality was 23.5 and 17.6% in those undergoing an anterior anastomosis, while it was 7.4 and 3.7% in those having a retroaortic connexion. When we compared the results in survivors, according to a previously designed clinical assessment score, we found that 41.1% of those undergoing the anterior approach were in excellent clinical status in comparison to 85.1% of those having a posterior anastomosis. The actuarial survival curve at 6 years showed 88.8% survival (70% confidence limit 44-77) for the posterior approach and 64.7% for the anterior. On the basis of our findings we felt able to remove from consideration as risk factors three of the features initially identified by Choussat and his colleagues. We conclude that better immediate and late results are obtained with the posterior retroaortic approach. We also found that those patients with excellent long-term outcome had postoperative right atrial pressures less than 14 mm Hg. Late arrhythmias were associated with increased right atrial pressures and were a relevant risk factor in both groups.

Tetralogy of Fallot with subarterial ventricular septal defect. Diagnostic and surgical considerations.

Since 1964, 77 patients underwent repair for tetralogy of Fallot with subarterial ventricular septal defect. Median age at operation was 5 years. Cyanosis was commonly mild, and hypoxic episodes were infrequent. Accordingly, only 12 patients (15.58%) needed either palliative or corrective operations before the age of 2 years. Characteristic angiographic and echocardiographic features were observed, which allowed this entity to be differentiated from either classic tetralogy or other types of double-outlet right ventricle. The earliest series of operations (in which no right ventricular outflow patch was used) was followed by a prohibitive mortality (50%). Residual subpulmonary obstruction was the cause of all of these deaths. In a second series of patients, a transannular patch was frequently used (79.59%), with the rationale that closure of the ventricular septal defect would make restrictive the deficient infundibulum of these patients. In a later series, we observed that most of the patients had a nonrestrictive pulmonary anulus (80%); the patch therefore was limited to the ventriculotomy. In the absence of pulmonary annular hypoplasia, the need for patching of the right ventricular outflow tract in this entity has to be confined to preventing the subpulmonary obstruction induced by closure of the ventricular septal defect. A considerable improvement in the results followed our first series (4.3% mortality). Follow-up of the survivors (mean 7 years) has been satisfactory.

Antithrombotic therapy in children and adolescents.

This report documents our experience with long term antithrombotic therapy (acenocoumarol plus aspirin) in 31 children and adolescents, from 5 months to 16 years of age. The valves replaced were mitral in 20 patients, aortic in 4, mitral-aortic in 4 and tricuspid in 3; the overall follow-up time was of 1336 months. Anticoagulant requirement in each children was not in correlation with age, but a significant increase (p less than 0.01) was found in association with sexual development. Our total incidence of embolic episodes was 1.49/1000 patient-months. The embolic incidence on adequate anticoagulated patients was 0.74/1000 patient-months and 93.7% of all patients were free of thrombo-embolic accidents up to 96 months of follow-up. Minor haemorrhage in relation to an excess of anticoagulant was 1.49/1000 patient-months. There has been only one major bleeding episode associated with severe sepsis, with an incidence of 0.74/1000 patient-months. No major difficulties were found in the management of anticoagulant treatment and its association with antiplatelet drugs in children.

Repair of corrected transposition associated with ventricular septal defect and pulmonary stenosis.

Five patients with the diagnosis of classically corrected transposition of the great arteries, ventricular septal defect (VSD), and pulmonary outflow tract obstruction underwent surgical repair. A variant of a previously described technique was used to avoid injury to conduction tissue. Through an incision into the anatomical left ventricle, the VSD patch was sutured inferiorly to the right and away from the edges of the defect and superiorly to the epicardial border of the ventriculotomy. The pulmonary artery was opened, and its proximal end was closed with a suture. A pouch containing the conduction tissue was therefore obtained. Pulmonary ventriculoarterial continuity was reestablished using a valved or nonvalved Dacron or pericardial conduit. The postoperative course of the patients was uneventful. No changes were demonstrated on comparison with preoperative cardiac rhythm. Good hemodynamic performance was noted in 2 patients in whom postoperative catheterization was performed.

A surgical technique for correction of total anomalous pulmonary venous drainage.

A technique was employed successfully for correction of total anomalous pulmonary venous drainage into the upper right superior vena cava. A J-shaped right atriotomy was performed; the posterior flap was sutured to the anterior border of a previously enlarged atrial septal defect. The right superior vena cava was divided above the site of drainage of the pulmonary veins, and its proximal opening closed with a suture. The pulmonary venous return was directed to the left atrium in this way. The right atrial-right superior vena caval continuity was then reestablished by an anastomosis between the previously opened right atrial appendage and the distal end of the right superior vena cava. Finally the remaining atriotomy was closed. The azygos vein must be ligated to avoid systemic unsaturation. For correction of anomalous pulmonary venous drainage into the azygos vein with this technique, ligature of the azygos vein must be placed distally to the site of anomalous drainage. Three patients, aged 2 months, 7 years, and 16 years, respectively, with different anatomic types of the anomaly, were successfully operated on with this procedure. Findings displayed from the postoperative hemodynamic, echocardiographic, and clinical evaluation are encouraging, after a follow-up period that ranges from 4 months to 4 years. The advantages of the repair are discussed.

Atriopulmonary anastomosis.

Atriopulmonary anastomosis (APA) has been performed in 29 patients, 3 to 22 years of age, since 1971. The diagnoses were tricuspid atresia in 21, single ventricle with low pulmonary vascular resistance in seven, and one case of dextro-transposition of the great arteries with ventricular septal defect and pulmonary stenosis. Four different techniques were used: Technique I (anterior end-to-end APA with a homograft or Dacron tube); Technique II (anterior end-to-end APA with the patient's own pulmonary artery); Technique III (nonvalved anterior anastomosis between the right atrium and the right ventricle); and Technique IV (largest, posterior, nonvalved direct APA between the right atrium and the main pulmonary artery and its right branch). Since the right atrium does not function as a pump, caval valves were never used. The total hospital mortality was 17.2%. Proper patient selection and the development of Technique IV reduced the mortality to 9%. Low end-diastolic ventricular pressure and a nonrestrictive APA are mandatory to obtain a good clinical result without pleural effusion. Twenty-one survivors are in Functional Class I, 17 of them without medication. Twelve of the 24 survivors were recatheterized. The best clinical and hemodynamic results were achieved in patients with low right atrial pressure and low end-diastolic ventricular pressure. The follow-up demonstrated a consistent superiority of the posterior nonvalved APA (Technique IV) in comparison with other techniques described. Therefore, this technique is proposed as the procedure of choice for the performance of an APA, irrespective of the precise diagnosis (tricuspid atresia or single ventricle) and irrespective of the type of great arterial relationship.