Idiopathic thoracic aortic aneurysm at pediatric age.

A 6-year-old-boy presented with epigastric pain and vomiting over 1 year. Chest X-ray and esophagogastric transit showed a mediastinal mass. A chest computerized tomography angiogram demonstrated a descending thoracic aortic aneurysm. Analytical determinations carried out were all negative. The aneurysm was surgically repaired using a Dacron patch. The anatomopathological study described atherosclerotic lesions with calcifications, compatible with an atherosclerotic aneurysm wall. Aneurysms are uncommon in the pediatric population. Usually, no pathogenesis can be determined, and thus, such cases are grouped as idiopathic. Direct repair with or without patch is a therapeutic alternative in pediatric aneurysms and can allow the growth of the aortic circumference.

[HIV related Foix-Chavany-Marie syndrome]. / Síndrome de Foix-Chavany-Marie asociado a VIH.

Automatic-voluntary motor dissociation of the face and lower cranial nerves of brainstem is the hallmark of Foix-Chavany-Marie syndrome (biopercular syndrome). It is commonly caused by ischemic strokes. We present a biopercular syndrome associated with HIV. A 41 year old female who presents a progressive automatic- voluntary motor dissociation of face and lower cranial nerves with later involvement of limbs. She was immunodepressed as a result of HIV. MRI showed bilateral lesions in opercular areas and ventrolateral thalamic nucleus, likely as a result of retrograde neuronal degeneration. Automatic-voluntary dissociation occur after biopercular lesions with sparing of motor pathway proceeding from the cingulate cortex.

[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.

[Interrupted aortic arch with aortopulmonary window. Total primary correction without extracorporeal circulation in the newborn]. / Interrupción del arco aórtico con ventana aortopulmonar. Corrección total primaria sin circulación extracorpórea en el neonato.

This report describes the unusual association between the interruption of the aortic arch type B and aortopulmonary window type II in a neonate. When the patient was 20 days old, a one-stage surgical repair was done through left side thoracotomy without circulatory by-pass, making a left carotid artery to descending aorta anastomosis, closing the window with a hemaclip, respectively. A routine 2-D Doppler color echocardiography performed in the immediate postoperative period showed the absence of blood flow in the right pulmonary artery. The patient required a new intervention, changing the position of the clip in order to restore the normal pulmonary blood flow. Eight months after surgery, the "neoaortic arch" grows in harmony with the body surface area, without signs of cerebral circulatory deficiency or significant gradient at any level.

[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.

Probable diltiazem-induced acute interstitial nephritis.

OBJECTIVE: To describe a case of acute interstitial nephritis (AIN) probably related to administration of diltiazem. CASE SUMMARY: A 53-year-old white man presented to the hospital experiencing abdominal pain radiating to both renal fossae, as well as dysuria. Diltiazem and atenolol had been prescribed to treat an episode of precordial pain associated with effort. An erythematous maculopapular rash developed approximately 2 hours after administration of a single dose of diltiazem, and acute renal failure, associated with elevated liver function test results, developed 6 days later. DISCUSSION: To the best of our knowledge, this is the third reported case of acute renal failure believed to be induced by diltiazem. In all cases, there was an obvious temporal relationship between administration of diltiazem and the onset of acute renal failure. Previous reports failed to discuss a probable pathogenic mechanism. AIN is the most likely etiology of acute renal failure in our patient. Favorable resolution with no relapse, the presence of the skin rash, and the liver sequelae suggest a common immunoallergic mechanism. CONCLUSIONS: Healthcare professionals should consider diltiazem-induced AIN in the differential diagnosis of a patient taking diltiazem who develops acute renal failure.

[The role of the stent in non-coronary cardiopathies]. / Papel del stent en las cardiopatías no coronarias.

INTRODUCTION: The stent has demonstrated to be a useful device in the prevention of postangioplasty coronary restenosis and it is expected to have a favourable effect as an alternative or complementary treatment of stenotic lesions in arteries or veins associated with congenital defects. The aim of this study is to analyze our experience in this setting. MATERIAL AND METHODS: From February 1992 to March 1996, 28 stenting procedures were performed in 26 patients (mean age: 8.6 +/- 0.7 years; mean weight: 26.2 +/- 3 kg). In 12 patients, stenting was single, and a iliac Palmaz stents were always used. Stenting location was: pulmonary artery branches in 17 patients, right ventricular outflow in 2 patients, in the junction of right atrium with pulmonary artery in 2 patients, systemic veins in 2 patients and in post Mustard intratrial channel stenosis in 2 patients. 25 patients had previously undergone at least one surgical procedure. RESULTS: The stenotic diameter of the treated lesions increased significantly after the procedure (4.4 +/- 0.3 mm before stenting vs 11.6 +/- 0.3 mm after stenting, p < 0.0001) and the transtenotic gradient decreased from 38.1 +/- 5 to 12 +/- 3.8 mmHg. Those changes were associated with a diminution of right ventricular pressure (81.6 +/- 3 vs 56.7 +/- 6 mmHg, p < 0.0001) in patients with pulmonary branch stenosis without septal defects. There was no mortality among the percutaneously treated patients and only one patient needed surgery. Nevertheless, one patient died after bilateral intraoperative stenting. CONCLUSION: The treatment of proximal or distal stenotic lesions in the pulmonary tree, systemic veins, and obstructed intraatrial channels with stents, can replace or complement conventional balloon angioplasty. It also offers a useful and effective alternative to surgery, when it is impossible or carries a risk.

[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.

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.

[Systemic-pulmonary fistula. Percentage of children attaining corrective surgery]. / Fístula sistémico-pulmonar. Porcentaje de niños que alcanzan la cirugía correctora.

Because of the lack of information on definitive long term results of the systemic pulmonary fistulae (SPF) we decided to review our experience. From 1978/1980, SPF was carried out on 91 children with a minimal follow-up period of 53 months, 80 (88%) survive the immediate postoperative period, 65 (71%) the late period, although only 48 (53%) reached corrective surgery. 14 (50%) of the 28 newborn children died and only 9 (32%) had corrective surgery. 12 (19%) of the 63 children of two months or older died and 39 (62 por 100) had definitive surgery. 72% with Fallot syndrome, 58% with TGA and pulmonary stenosis and less than 40% of other pathologies also reached definitive corrective surgery. Frequently the SPF is the only alternative to achieve total correction although there may be a high mortality and morbidity rate. The age (newborn children) and the complex pathology are unfavourable in the end results.

[Surgical management of aortic coarctation in the newborn infant]. / Manejo quirúrgico de la coartación aórtica en el neonato.

Between 1978 and 1983, 35 neonates with coarctation of the aorta (from a group of 200 cases with the same pathology) underwent coarctation relief. Indications for operation were persistent heart failure and/or hypertension. Three surgical techniques were employed: Aortoplasty with prosthetic patch (politetrafluoroethylen) in 23 cases. Repair with subclavian flap in 11 cases, and resection of coarctate segment with and-to-end anastomosis in only one case. It is an important fact the high incidence of congenital cardiac malformations associated with coarctation of the aorta in neonates. There were 8 deaths (six of them with associated cardiac malformations, and other with miocardiophaty). There were 8 late deaths and 5 cases with restenosis, and no hypertension cases. All others are well in the follow-up. Some of them are waiting for definitive correction of their major cardiac malformation. The high risk of mortality in this group, mark again the importance of adequate medical treatment previous to surgical correction in optimal conditions and as early as possible.

Temas de ecocardiografía: III. Evaluación ecocardiográfica previa a la cirugía de los enfermos con conexión atrioventricular univentricular / Echocardiography: III. Early echocardiographic evaluation in surgery of patients with univentricular atrioventricular connection

Los pacientes con conexión atrioventricular univentricular pueden beneficiarse hoy día de diversos procedimientos quirúrgicos paliativos. La indicación quirúrgica óptima debe estar basada en un diagnóstico anatómico y funcional exactos. Para valorar hasta qué punto la ecocardiografía, puede contribuir al diagnóstico revisamos 45 pacientes operados con conexión atrioventricular tipo univentricular. Las técnicas quirúrgicas empleadas fueron las siguientes: banding de la arteria pulmonar en 10 pacientes, de los cuales en 8 se asoció la técnica de Blalock-Hanlon; fístula sistémico-pulmonar en 34 casos, de los que en 6 se realizó también atrioseptectomía de Blalock-Hanlong; anastomosis atriopulmonar modificada (técnica de Kreutzer) en 4 enfermos. La ecocardiografía, fue satisfactoria para el diagnóstico de la malformación y el estado del septo interatrial, el foramen interventrículo-cameral y las válvulas atrioventriculares y semilunares. También fue útil para conocer las características funcionales de las cámaras ventriculares. Sin embargo, la información que facilitó la ecocardiografía, en lo referente al arco aórtico y a las ramas de la arteria pulmonar, fue inexacta y no fiable. En conclusión: 1) El tratamiento quirúrgicos de los pacientes con conexión atrioventricular univentricular está justificado, pues con él se consigue mejorar la calidad de vida del enfermo y de su entorno y, a largo plazo, reducir los costos de la asistencia médica. 2) La ecocardiografía, proporciona una parte muy sustancial de la información que necesitamos para realizar un diagnóstico preoperatorio correcto (AU)

Temas de ecocardiografía: III. Evaluación ecocardiográfica previa a la cirugía de los enfermos con conexión atrioventricular univentricular / Echocardiography: III. Early echocardiographic evaluation in surgery of patients with univentricular atrioventricular connection

Los pacientes con conexión atrioventricular univentricular pueden beneficiarse hoy día de diversos procedimientos quirúrgicos paliativos. La indicación quirúrgica óptima debe estar basada en un diagnóstico anatómico y funcional exactos. Para valorar hasta qué punto la ecocardiografía, puede contribuir al diagnóstico revisamos 45 pacientes operados con conexión atrioventricular tipo univentricular. Las técnicas quirúrgicas empleadas fueron las siguientes: banding de la arteria pulmonar en 10 pacientes, de los cuales en 8 se asoció la técnica de Blalock-Hanlon; fístula sistémico-pulmonar en 34 casos, de los que en 6 se realizó también atrioseptectomía de Blalock-Hanlong; anastomosis atriopulmonar modificada (técnica de Kreutzer) en 4 enfermos. La ecocardiografía, fue satisfactoria para el diagnóstico de la malformación y el estado del septo interatrial, el foramen interventrículo-cameral y las válvulas atrioventriculares y semilunares. También fue útil para conocer las características funcionales de las cámaras ventriculares. Sin embargo, la información que facilitó la ecocardiografía, en lo referente al arco aórtico y a las ramas de la arteria pulmonar, fue inexacta y no fiable. En conclusión: 1) El tratamiento quirúrgicos de los pacientes con conexión atrioventricular univentricular está justificado, pues con él se consigue mejorar la calidad de vida del enfermo y de su entorno y, a largo plazo, reducir los costos de la asistencia médica. 2) La ecocardiografía, proporciona una parte muy sustancial de la información que necesitamos para realizar un diagnóstico preoperatorio correcto