Complete atrioventricular canal and tetralogy of Fallot: surgical management.

Between 1982 and 1989, nine patients with complete atrioventricular (AV) canal and tetralogy of Fallot underwent successful repair of both anomalies. Seven patients had Down's syndrome. One or more previous palliative shunts had been used in six patients. Associated cardiovascular lesions included persistent left superior vena cava (two patients), patent ductus arteriosus with marked stenosis of the left pulmonary artery (one patient). The diagnosis was confirmed by cardiac catheterization and cineangiography. Closure of the ventricular septal defect was performed through a combined right atrial and ventricular approach in each patient. The right ventricular outflow obstruction was relieved with the use of a transannular patch (4), right ventricular infundibular patch (4) or a valved external conduit (1). There were no hospital deaths. One patient died as a result of meningitis 2 years after repair. Progressive left AV valve incompetence developed in one patient requiring valve replacement. A single atrial approach may not provide the best exposure of the subaortic end of the defect. In this regard, we recommend an additional right ventriculotomy in order to ensure safe closure of the septal defect. Although an adequately shaped isolated patch could be sufficient to close the anterior VSD, we favour a separate patch to minimize the possibility of producing subaortic obstruction. A further method to avoid this subaortic stenosis consists of dividing the common anterior leaflet to the right towards the lateral extent of the infundibular septum.

Phrenic nerve paralysis following pediatric cardiac surgery. Role of diaphragmatic plication.

Eighteen children sustained unilateral phrenic nerve paralysis (PNP) after cardiac surgical procedures. Ten (Group I), under 7 months (mean: 2.9 +/- 2.2), required long-term ventilatory assistance (mean: 23.9 +/- 13.0 days); they failed to be weaned from the ventilator. All underwent diaphragmatic plication (DP). DP was performed late in 7 cases (Group Ia) with a mean time of 30.8 days between surgery and DP, and early in 3 others (Group Ib) with a mean time of 10.2 days. Eight children (Group II), older than 1 year, tolerated PNP better and could be extubated early without diaphragmatic plication. In Group Ia severe lung infections were recorded in 5 before or/and after DP, and two died at 3 and 30 days after plication. Five children from Group Ia and all 3 from Group Ib were late survivors. They could be weaned from ventilatory support in a mean time of 3 days after DP, although those with severe lung infection (Group Ia) took the longest time. All from Group II were late survivors. We conclude: PNP is well tolerated without plication in children older than 1 year. However early DP offers excellent and immediate results in infants with PNP. Early DP in these children avoids or reduces severe lung infections and death.

[The pulmonary artery closure may be a thromboembolic risk factor in patients with bidirectional cavo-pulmonary (Glenn) shunt]. / El cierre arterial pulmonar puede ser un factor de riesgo tromboembólico en pacientes con anastomosis cavopulmonar (Glenn) bidireccional.

Postoperative thrombosis after the Fontan procedure has been well noted in the literature, and its risk factors are also well known. In contrast, thrombosis after the bilateral cavo-pulmonary shunt (Glenn) has been rarely reported and almost always occurs around the anastomosis itself or near it, mainly causing pulmonary embolism. We present 2 cases with cerebral embolism 2-7 months after pulmonary artery closure and Glenn procedure, due to dislodgement of a thrombus in the proximal pulmonary artery stump. Based on these two cases and a few others reported in the literature, we want to call the attention to this new cause of thromboembolism after Glenn and stimulate discussion about its incidence, risk factors and preventive measures.

[Atrioventricular septal defect with associated tetralogy of Fallot. Clinico-morphological aspects and surgical considerations]. / Defecto del septo atrioventricular con tetralogía de Fallot asociada. Aspectos clinicomorfológicos y consideraciones quirúrgicas.

In this work the clinical features, diagnostic methods, surgical procedures and postoperative follow-up are reviewed in 19 cases of atrioventricular septal defect with associated tetralogy of Fallot. The clinical situation was always related to the degree of pulmonary ischemia, that pointed out the urgency and need for a palliative surgical procedure before corrective surgery. There was no data in the ECG for the differential diagnosis of isolated forms of atrioventricular septal defect. In 50% of the cases the cardio-thoracic index was increased by right atrial enlargement. The diagnosis of the malformation was made in every case by means of 2D echocardiography. In 16 cases palliative surgery was done (aortic-pulmonary shunts), with one death (6.2%) due to facts unrelated to the technique. Of the 6 cases with corrective surgery (5 cases with previous shunts), one needed a mitral valve replacement (St Jude prosthesis). There was no mortality in this group and the functional status in the long-term follow-up was: 3 cases were in the functional class I and 3 cases in class II. The overall mortality for all surgical procedures carried out was 4.3%. In conclusion, we consider the use of the corrective surgery adequate to the clinical stability of the patients with atrioventricular septal defect with associated Fallot's tetralogy.

[Divided left atrium: operative results and follow-up in the cor triatriatum]. / Aurícula izquierda dividida: resultados quirúrgicos y seguimiento en el cor triatriatum.

INTRODUCTION AND OBJECTIVES: A divided left atrium because of cor triatriatum is a relatively rare cardiac anomaly requiring corrective surgery. We describe here our clinical and surgical experience with this congenital heart defect as well as the different medium and long term diagnostic, surgical and evolution aspects. PATIENTS AND METHODS: From 1981 to 1999, 15 children with cor triatriatum without complex associated cardiovascular defects underwent surgery at a mean age of 13 months (excision of the obstructive membrane). The surgical reports were reviewed and the clinical and echocardiographic data were analyzed before and after the intervention; six of these patients (40%) were referred to operating room only with the 2-D echo Doppler technique and color flow mapping information. The follow-up period ranged from 8 months to 19.3 years. RESULTS: Diagnosis was confirmed during the surgical procedure. One 9 month old patient died 60 days after a successful corrective surgery because of sepsis (7%). No late deaths or reoperations were found in the follow-up period. All 14 patients who survived the operation have a functional class I (NYHA), and they are asymptomatic in the follow-up. The overall survival rate was 93% (70% CI: 87-90). CONCLUSIONS: Corrective surgery with excision of the obstructive membrane dividing the left atrium restores normal anatomic, hemodynamic and clinical status in children with cor triatriatum without complex associated defects.

[Complete atrioventricular canal and tetralogy of Fallot: surgical considerations]. / Canal atrioventricular completo y tetralogía de Fallot: consideraciones quirúrgicas.

Nine patients with complete atrioventricular canal and tetralogy of Fallot underwent intracardiac repair of both anomalies between 1982 and 1989. The ages of the patients ranged from 6 months to 7 years. Six of the 9 had one or more previous systemic-pulmonary artery shunts. Two-dimensional echocardiography showed diagnostic characteristics of both malformations in all patients. The diagnosis was confirmed by cardiac catheterization and cineangiography. The ventricular septal defect was repaired by a combined right atrial and ventricular approach in every patient. Outflow tract reconstruction was performed with the use of a transannular patch (4), infundibular patch (4), and a valved conduit (1). There were no hospital deaths. Meningitis was responsible for the death of a patient 2 years after repair. We recommend early palliation, complete repair in those older than 4 or 5 years, surgical technique depending on the anatomical findings, combined atrial and ventricular approach, 2 separate patches to close the ventricular septal defect in required cases, adjusted correction of the right ventricular outflow tract, and careful postoperative care.

[The angiographic diagnosis of the connection of the persistent left vena cava superior to the left atrium by the absence of the roof of the coronary sinus in a child with a Holmes' heart]. / Diagnóstico angiográfico de la conexión de la vena cava superior izquierda persistente a la aurícula izquierda por ausencia de techo del seno coronario en un niño con corazón de Holmes.

We describe a patient with angiographic findings of a left superior persistent vena cava draining in the left atrium with absence of the coronary sinus' roof and coexisting with Holmes' heart. The diagnosis can be made by selective angiography in the left atrium, pulmonary vein or at the left superior vena cava. We want to emphasize the importance of knowing this sort of anomaly before surgical treatment.

[Use of aprotinin during pediatric heart surgery]. / Utilización de aprotinina en cirugía cardíaca pediátrica.

OBJECTIVE: To study the efficacy of high doses of aprotinin during cardiopulmonary bypass (CPBP) in pediatric patients with a tendency to intra- and postoperative bleeding: children undergoing repeated operations and those with cyanotic cardiopathy. PATIENTS AND METHODS: A simple retrospective, random sample of 71 pediatric patients with cyanogenic cardiopathy was studied. Group I (n = 36) received a test dose of 50,000 KIU in 2 min. The initial dose was 1,700,000 KIU (170 ml/m2 of body surface) i.v., at an infusion rate of 150 ml/h. CPBP circuit prime dose was 1,700,000 (170 ml/m2 of body surface), with replacement at a volume equivalent to the circuit prime. The maintenance dose was 400,000 KIU (40 ml/m2/h) i.v. Infusion was suspended 2 hours after a patient's arrival at the intensive care unit (ICU). We recorded red blood cell loss and blood product requirements in the first 24 hours in the ICU, duration of surgery, intra- and postoperative diuresis, and complications. RESULTS: Group I required 13.96 +/- 12.09 ml/kg/h of packed red cells whereas Group II required 22 +/- 16.22 ml/kg/h (p < 0.05). The volume lost was less in Group I than in Group II (0.77 +/- 0.48 ml/kg/h vs. 2.12 +/- 3.9 ml/kg/h; p < 0.05). The means for intra- and postoperative diuresis in the first 24 hours were not significantly different. CONCLUSIONS: Aprotinin significantly reduces red blood cell loss and homologous blood transfusion requirements. No complications attributable to aprotinin were observed.

[Extracorporeal membrane oxygenation in myocardial failure after pediatric heart surgery]. / Oxigenación con membrana extracorpórea en el fallo miocárdico después de cirugía cardíaca pediátrica.

In spite of progressive improvement not only in myocardial protection but also in anesthetic and surgical techniques, the repair of complex congenital heart defects can still lead to cardiopulmonary compromise refractory to conventional treatment. We describe two patients in whom successful surgical repair of congenital heart defects was followed by severe heart failure refractory to medical treatment. Both patients were managed by extracorporeal membrane oxygenation (ECMO). We discuss the indications, contraindications and use of ECMO in the postoperative intensive care unit.

[Neonatal laceration of liver and spleen (author's transl)]. / Ruptura neonatal de hígado y bazo.

A very unusual observation of survival after surgical treatment of neonatal maceration of liver and spleen is reported. The patient had a normal birthweight and the only possible etiologic factor was a oxitocin-induced rapid delivery. The literature on this topic is reviewed.

Infectious endocarditis in children.

With the object of analyzing current characteristics of infectious endocarditis (IE) in children, we carried out a retrospective study of 23 cases of IE in children under 15 years of age seen at the Hospital Ramón y Cajal in Madrid (Spain) between 1977 and 1985. The incidence was high (1.3 cases per 1000 children admitted). The male/female ratio was 2:1. Eight patients were under 2 years of age and 15 over 2 years, the majority being adolescents. The two groups presented marked etiological and prognostic differences. Congenital heart disease was the predisposing factor in 20 of the 23 cases. Streptococcus viridans (nine cases) and Staphylococcus aureus (eight cases) were the most frequent organisms. Fourteen cases were on a native valve and nine were secondary (seven of these on prosthetic patches). In spite of advances in therapy, IE continues to be a severe illness: the mortality rate in our series was 26%. Factors associated with a poor prognosis were: age less than 2 years, Staphylococcus aureus as the causative agent, and the presence of prosthetic material.

[Fever of undetermined origin in a case of total correction of persistent truncus arteriosus with agenesis of the left pulmonary artery (author's transl)]. / Fiebre de origen desconocido en un "truncus" con agenesia de la arteria pulmonar izquierda, intervenido de corrección total.

A 19 month-old girl diagnosed of truncus arteriosus with absence of the left pulmonary artery and severe truncal valve insufficiency underwent total correction. A non previously diagnosed severe mitral insufficiency required a new operation to implant a Bjork-Shiley supraanular mitral prosthesis. The postoperative period was hemodynamically normal, except for the presence of persistent fever of unexplained origin. The clinical picture was thought to be an infectious endocarditis due to the late appearance of an aortic prosthetic leak. Because of failure to medical treatment a reoperation was performed to replace aortic prosthesis. Patient died eight hours postoperatively. Autopsy revealed a correct surgical repair, absence of endocarditis and severe miliary disseminated tuberculosis. Authors consider of interest to present this case-report due to the rarity of: a) correction of truncus with a single pulmonary artery under two years of age; b) associated mitral insufficiency; c) the presence of tuberculosis, difficult differential diagnosis with infectious endocarditis in a patient with valvular prosthesis.

Tetralogy of Fallot associated with total anomalous pulmonary venous drainage.

We present a case of tetralogy of Fallot associated with total anomalous pulmonary venous return, with successful surgical correction. This association presents problems as regards surgical indications and technique.

Utilización de aprotinina en cirugía cardíaca pediátrica / Use of aprotinin during pediatric heart surgery

OBJETIVO. Estudiar la eficacia de altas dosis de aprotinina durante la circulación extracorpórea (CEC) en pacientes pediátricos con especial tendencia a la hemorragia intra y postoperatoria: niños reintervenidos y aquellos que presentan cardiopatía cianótica.PACIENTES Y MÉTODOS. Se escogió por muestreo simple aleatorio y de forma retrospectiva a 71 pacientes pediátricos con cardiopatía cianógena. El grupo I (n = 36) recibió una dosis test de 50.000 CIU (unidades inhibidoras de calicreína) en 2 min. La dosis inicial fue de 1.700.000 CIU (170 ml/m2 de superficie corporal) por vía intravenosa, a una velocidad de infusión de 150 ml/h. La dosis de cebado del circuito de CEC 1.700.000, 170 ml/m2 de superficie corporal, reemplazándose a un volumen equivalente del cebado del circuito. La dosis de mantenimiento fue de 400.000 CIU, 40 ml/m2/h por vía intravenosa. La perfusión fue suspendida a las 2 h de llegada a la UCI. Se valoraron las pérdidas hemáticas y las necesidades de hemoderivado en las primeras 24 h de estancia en la UCI, los tiempos quirúrgicos, la diuresis intra y postoperatoria, así como las complicaciones.RESULTADOS. El grupo I necesitó 13,96 ñ 12,09 ml/kg/h de concentrado de hematíes frente a los 22 ñ 16,22 ml/kg/h del grupo II (p < 0,05). El volumen drenado del grupo I fue menor que el del grupo II (0,77 ñ 0,48 ml/kg/h frente a 2,12 ñ 3,9 ml/kg/h; p < 0,05). No existieron diferencias significativas en la diuresis media tanto intraoperatoria como en el primer día postoperatorio.CONCLUSIONES. El uso de aprotinina reduce significativamente las pérdidas hemáticas y los requerimientos de sangre homóloga. No se objetivaron efectos clínicos adversos atribuibles a la aprotinina (AU)

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