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The results of 180 cases of congenital heart disease with diminished pulmonary flow operated upon with Waterston's technique are presented. It is considered that Waterston's operation is to be preferred in children under 2 years of age and in older children who have had a previous thrombotic or insufficient Blalock operation and in whom total correction is not indicated. The problem of pseudotruncus with hypoplastic pulmonary arteries is discussed. The convenience of the Waterston operation in these cases, and the importance of creating an anastomosis at the pulmonary bifurcation and as far back as possible in the aorta, is emphasized. The need to perform this technique in the correct way is stressed. This will avoid the kinking and pulling of the right pulmonary artery, which are causes of preferential blood flow to the right lung, as we have demonstrated experimentally. The possible complications caused by such a technical failure are discussed. The necessity for previous angiocardiographic study, in order properly to repair the defect during total correction, is also considered. [ABSTRACT FROM PUBLISHER]

We present a case involving a rare form of cyanotic congenital heart disease in a 2-month-old neonate. The initial diagnosis was thought to be tricuspid dysplasia with right-to-left shunting through an interatrial communication. However, surgery showed the presence of a pedunculated mass that prolapsed into the tricuspid valve orifice, thereby causing severe obstruction to right ventricular filling. This mass was attached to the lower part of the atrial septum. Pathological examination showed fibrotic myxoid tissue. After the mass was resected surgically, the tricuspid valve appeared normal. We comment on the pathogenesis and the differential diagnosis of this rare entity.

Ebstein's malformation of the tricuspid valve is a rare but complex congenital cardiac lesion characterised by a variable degree of dysplasia and displacement of the proximal attachments of its inferior and septal leaflets from the true atrioventricular junction. The aim of our retrospective study is to report the risk factors for mortality, and to determine the clinical profile as seen in 52 cases diagnosed in our service between 1978 and 2002, concentrating in particular on the outcome for the neonatal patient. There were 26 females and 26 males, and the age at presentation ranged from 30 weeks gestational age to 46 years. We found 23 associated cardiac anomalies in 20 cases. Of the patients, 11 patients (21%) died. Actuarial survival at 30 years was 65%. Predictors of death included fetal or neonatal presentation, presence of associated defects, a grade within the Celermajer index of 3 or 4, and a cardiothoracic ratio equal to or greater than 65%. The diagnosis was made in the neonatal period in 24 patients, nine of whom died. Of the 15 survivors, only 4 are free of symptoms. Surgical treatment was undertaken in 9 patients, with a mortality rate of 33% without late deaths, with all the survivors being in good condition. The mean period of follow-up for the 41 living patients was 16.5 years. An arrhythmia of variable severity appeared during the evolution of 27 patients (66%). At present, only 7 cases are in the functional class III or IV of the grading system of the New York Heart Association, but 25 patients (61%) needed some medical treatment, meanly for arrhythmic events. Thus, fetal and neonatal presentation of Ebstein's malformation is associated with a poor outcome. Moreover, the echocardiographic appearance, marked cardiomegaly, and the presence of associated lesions are all risk factors for mortality. Arrhythmia and need of medical treatment are common in older children and adults. Survival after surgical treatment is associated with a good outcome.

Introduction and Objectives: The Fontan procedure was designed to palliate complex congenital heart disease with univentricular physiology. A retrospective study was made to document the determinants of early (/= 31 days) mortality with the modified Fontan procedure performed in one-stage over a 22-year period., Material and Methods: Between 1978 and 2000, 102 atriopulmonary, 16 cavopulmonary, and 6 Kawashima type anastomoses were performed to palliate complex congenital heart defects in 124 patients with a mean age of 7.3 4.7 years. Forty-five patient and procedure-related variables were analyzed in relation to mortality. All events were verified., Results: There were 29 early (23%) and 20 late (16%) deaths. Estimated survival at 30 days, 2 years, 5 years, and 20 years was 78, 75, 66, and 50%, respectively. Subaortic stenosis, protein-losing enteropathy, and arrythmia were observed in 8, 5 and 33 patients, respectively, after surgery. Univariate and multivariable analysis indicated that left ventricular end-diastolic pressure (>/= 13 mmHg), mean pulmonary pressure (>/= 19 mmHg), mitral stenosis/atresia, atrioventricular valve regurgitation, visceral heterotaxia, absence of fenestration, risk factors criteria, duration of extracorporeal circulation, and operative technique were associated with early mortality. Reoperation, arrhythmia, and pacemaker implantation were predictors of late death. Forty percent remained free from surgical or catheter reintervention after Fontan operation at 20 years., Conclusions: The outcome of Fontan procedure is profoundly affected by patient-related variables (ventricular function and pulmonary circulation). Postoperative arrhythmia and reoperation shortened the lifespan of the Fontan circulation model in patients with atriopulmonary connections. Total cavopulmonary anastomosis improves the physiology of univentricular circulation. In the light of our findings, the modified Fontan procedure (one or two stages) should be performed early in life to better preserve ventricular and pulmonary vascular function.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

112 cases of coarctation of the aorta and 8 cases of tubular hypoplasia of the aortic isthmus operated upon in the Children's Hospital "La Paz" from Madrid, are reviewed. All children were under 7 years of age. 64.2% of the cases of aortic coarctation were in the first year of life, 47.3% of them had associated lesions, being the most frequently present persistent ductus arteriosus and ventricular septal defect. Hospital mortality was 14.2%, what is considered as very acceptable. All the children operated upon for the correction of tubular hypoplasia of the aortic isthmus were in the first year of age. 75% of them had associated ductus arteriosus and ventricular septal defect, being hospital mortality of 62.5%. Most frequent postoperative complications and cause of death were due to broncopulmonary disorders secondary to the existence of a previous pulmonary hypertension.