Alfonso Buendía

Surgical treatment for secundum atrial septal defects in patients >40 years old: A randomized clinical trial

We prospectively examined whether surgical treatment of secundum atrial septal defects (ASDs) in patients > or =40 years old improves their long-term clinical outcome. Surgical treatment of secundum ASDs in adults > 40 years old is a subject of controversy because of the perception of good long-term clinical outcomes in patients with unrepaired ASDs and the lack of data from randomized trials. We recruited 521 patients > 40 years old with secundum ASDs referred for treatment; 48 were excluded. Patients were randomly assigned to surgical closure (n = 232) or medical treatment (n = 241). The primary and secondary end points were a composite of major cardiovascular events (death, pulmonary embolism, major arrhythmic event, embolic cerebrovascular event, recurrent pulmonary infection, functional class deterioration or heart failure) and overall mortality, respectively. We assessed possible prognostic markers. The analysis was performed on an intention-to-treat basis. The median follow-up period was 7.3 years (range 2 to 13). The risk of having the primary end point was significantly higher in the medical group, which had a univariate hazards ratio of 1.99 (95% confidence interval [CI] 1.23 to 3.22) and a multivariate hazards ratio of 1.85 (95% CI 1.08 to 3.17). Although the survival analysis did not reveal differences in overall mortality between the surgical and medical treatments (hazards ratio 1.71, 95% CI 0.76 to 3.86), the multivariate analysis, adjusted by age at entry, mean pulmonary artery pressure and cardiac index, demonstrated significant differences between the study groups (hazards ratio 4.09, 95% CI 1.41 to 11.89). Surgical closure was superior to medical treatment in improving both the composite of major cardiovascular events and overall mortality in patients > 40 years old with secundum ASDs. This superiority was related to the mean pulmonary artery pressure, age at diagnosis and cardiac index. Because of the higher risk of morbidity and mortality, we believe that anatomic closure should always be attempted as the initial treatment for ASDs in adults > 40 years old with pulmonary artery systolic pressure < 70 mm Hg and a pulmonary/systemic output ratio > or =1.7. The operation must be performed as soon as possible, even if the symptoms or the hemodynamic impact seems to be minimal.

Clinical study: adult congenital heart diseaseSurgical treatment for secundum atrial septal defects in patients >40 years old: A randomized clinical trial

We prospectively examined whether surgical treatment of secundum atrial septal defects (ASDs) in patients > or =40 years old improves their long-term clinical outcome. Surgical treatment of secundum ASDs in adults > 40 years old is a subject of controversy because of the perception of good long-term clinical outcomes in patients with unrepaired ASDs and the lack of data from randomized trials. We recruited 521 patients > 40 years old with secundum ASDs referred for treatment; 48 were excluded. Patients were randomly assigned to surgical closure (n = 232) or medical treatment (n = 241). The primary and secondary end points were a composite of major cardiovascular events (death, pulmonary embolism, major arrhythmic event, embolic cerebrovascular event, recurrent pulmonary infection, functional class deterioration or heart failure) and overall mortality, respectively. We assessed possible prognostic markers. The analysis was performed on an intention-to-treat basis. The median follow-up period was 7.3 years (range 2 to 13). The risk of having the primary end point was significantly higher in the medical group, which had a univariate hazards ratio of 1.99 (95% confidence interval [CI] 1.23 to 3.22) and a multivariate hazards ratio of 1.85 (95% CI 1.08 to 3.17). Although the survival analysis did not reveal differences in overall mortality between the surgical and medical treatments (hazards ratio 1.71, 95% CI 0.76 to 3.86), the multivariate analysis, adjusted by age at entry, mean pulmonary artery pressure and cardiac index, demonstrated significant differences between the study groups (hazards ratio 4.09, 95% CI 1.41 to 11.89). Surgical closure was superior to medical treatment in improving both the composite of major cardiovascular events and overall mortality in patients > 40 years old with secundum ASDs. This superiority was related to the mean pulmonary artery pressure, age at diagnosis and cardiac index. Because of the higher risk of morbidity and mortality, we believe that anatomic closure should always be attempted as the initial treatment for ASDs in adults > 40 years old with pulmonary artery systolic pressure < 70 mm Hg and a pulmonary/systemic output ratio > or =1.7. The operation must be performed as soon as possible, even if the symptoms or the hemodynamic impact seems to be minimal.

The Adult Patient with Ebstein Anomaly: Outcome in 72 Unoperated Patients

Knowledge of the long-term outcome in unoperated adult patients with Ebstein anomaly is limited, and the therapeutic approach is still controversial. We studied unoperated adult patients with Ebstein anomaly to define the patterns of presentation, anatomic characteristics, outcome, and predictive factors for survival. Seventy-two unoperated survivors of Ebstein anomaly aged over 25 years attended from 1972 to 1997 were reviewed and followed-up from 1.6 to 22.0 years. Patients were classified in 3 groups of severity according to the echocardiographic appearance of the septal leaflet attachment of tricuspid valve. The mean age at diagnosis was 23.9 +/- 10.4 years, and the most common clinical presentation was an arrhythmic event (51.4%). There were 30 (42%) deaths, including 6 from arrhythmia, 12 related to heart failure, 7 sudden, 2 unrelated, and 3 unascertained. According to Cox regression analysis, predictors of cardiac-related death included age at diagnosis (hazard ratio 0.89 for each year of age, 95% confidence intervals CI[ 0.84-0.94), male sex (3.93, 95% CI, 1.50-10.29), degree of echocardiographic severity (3.34, 95% CI, 1.78-6.24), and cardiothoracic ratio > or = 0.65 (3.57, 95% CI, 1.15-11.03). During follow-up, morbidity was mainly related to arrhythmia and refractory late hemodynamic deterioration. The magnitude of tricuspid regurgitation, cyanosis, and the New York Heart Association (NYHA) functional class at time zero were significant risk factors according to the univariate analysis, but not after multivariable confrontation. The results of this study suggest that pattern of presentation, clinical course, and prognosis of unoperated adult patients with Ebstein anomaly are influenced by several factors. Although the initial symptoms are usually mild and commonly related to supraventricular arrhythmias, these are not associated with the long-term outcome. The severity of the morbid anatomy was the main determinant of survival only in extreme cases, but not in those with mild or moderate deformations, which are more common in adults. Other independent risk factors such as cardiothoracic ratio, sex, age at diagnosis, and the echocardiographic evaluation may help to determine the therapeutic approach. Adult patients with Ebstein anomaly should not be considered as a simple low-risk group.

Transcatheter versus Surgical Closure of Secundum Atrial Septal Defect in Adults: Impact of Age at Intervention. A Concurrent Matched Comparative Study

OBJECTIVES To compare the short- and mid-term outcomes of surgical (SUR) vs. transcatheter closure of secundum atrial septal defect (ASD) using Amplatzer septal occluder (ASO) in adults with a very similar spectrum of the disease; and to identify predictors for the primary end point. DESIGN Single-center, concurrent comparative study. Surgically treated patients were randomly matched (2:1) by age, sex, date of procedure, ASD size, and hemodynamic profile. SETTING Tertiary referral center. PATIENTS One hundred sixty-two concurrent patients with ASD submitted to ASO (n = 54) or SUR closure (n = 108) according with their preferences. MAIN OUTCOME MEASURES Primary end point was a composite index of major events including failure of the procedure, important bleeding, critical arrhythmias, serious infections, embolism, or any major cardiovascular intervention-related complication. Predictors of these major events were investigated. RESULTS Atrial septal defects were successfully closed in all patients, and there was no mortality. The primary event rate was 13.2% in ASO vs. 25.0% in SUR (P = .001). Multivariate analysis showed that higher rate of events was significantly associated with age >40 years; systemic/pulmonary output ratio <2.1; and systolic pulmonary arterial pressure >50 mm Hg; while in the ASO group the event rate was only associated with the ASD size (>15 cm(2)/m(2); relative risk = 1.75, 95% confidence interval 1.01-8.8). There were no differences in the event-free survival curves in adults with ages <40 years. CONCLUSIONS The efficacy for closure ASD was similar in both groups. The higher morbidity observed in SUR group was observed only in the patients submitted to the procedure with age >40 years. The length of hospital stay was shorter in the ASO group. Surgical closure is a safe and effective treatment, especially in young adults. There is certainly nothing wrong with continuing to do surgery in countries where the resources are limited.

Atrial septal defect in adults ≥40 years old: negative impact of low arterial oxygen saturation

BACKGROUND Although good prognosis and clinical long-term outcome have been commonly reported in minimally symptomatic adult patients with ASD, this information has been based on studies with a relatively small number of adult patients. We studied unoperated patients aged over 40 years to define the patterns of presentation, anatomical characteristics, outcome and predictive factors for free-event survival of major cardiovascular and pulmonary events. METHODS AND RESULTS Two-hundred survivors of atrial septal defect aged over 40-yr attended from 1985 to 1998 were reviewed and followed-up from 1.6 to 22 years. Patients were classified in three groups according to age at entry: Group 1, between 40 and 49; Group 2; 50 and 59; and Group 3, > or =60 years old. The mean age at presentation was 48.8+/-9.2 years, and the most common clinical presentations were arrhythmia and dyspnea (51.4%). There were 37 (18.5%) events: 7 heart failure-related, 5 sudden death, 13 severe pulmonary infections, 5 embolisms, and 4 strokes. According to Coxs regression analysis, predictors of primary end point included age group at presentation (hazard ratio 1.71, 95% confidence limits 1.16 to 2.54), and either pulmonary hypertension (mean pulmonary pressure >35 mmHg; hazard ratio=0.65 (4.6, confidence limits 2.2 to 9.5) or, arterial oxygen saturation <80% (hazard ratio 1.71, 95% confidence limits 1.16 to 2.54). CONCLUSIONS This study supports that long term outcome of patients aged >40 years with unoperated ASD is importantly determined by the mPAP (>35 mmHg), SaO2% (_80) and the age at diagnosis. Nevertheless we identified an inverse association between the mPAP level and SaO2% (interaction). The event-free survival expectancy may be estimated using the age at diagnosis and either SaO2% or mPAP. This prognostic stratification based on pathophysiological principles, may help in making decisions for therapeutic interventions. SaO2% should always be measured as a part of the initial clinical approach of those patients with atrial septal defect aged over 40 years.

Radiofrequency catheter ablation for the treatment of supraventricular tachycardias in children and adolescents

We report our experience in radiofrequency catheter ablation between April, 1992 and December, 1998, in which we treated 287 patients less than 18 years of age (mean 14.3 +/- 3.1 years) with supraventricular tachycardia. Accessory, pathways were the arrhythmic substrate in 252 of the patients (87.8%), the patients having a total of 265 accessory pathways. Atrioventricular nodal re-entry was the cause of tachycardia in 26 patients (9.0%), while atrial flutter was detected in the remaining 9 patients (3.1%). We were able successfully to eliminate the accessory pathway in 236 patients (89%), but 25 patients had recurrent arrhythmias. Ablation proved successful in all cases of atrioventricular node re-entry tachycardia, the slow pathway being ablated in 25 patients, and the fast pathway in only one case. Recurrence of the arrhythmia occurred in three patients (11.5%). We performed a second ablation in these children, all then proving successful. The ablation was successful in all cases of atrial flutter, with one recurrence (11.1%). Overall, therefore, ablation was immediately successful in 271 patients (94.4%), with a recurrence of the arrhythmia in 29 cases (10.7%). The incidence of serious complications was 2.09%. There was one late death due to infective endocarditis, 3 patients suffered complete heart block, 1 had mild mitral regurgitation, and 1 patient developed an haematoma in the groin. We conclude that radiofrequency catheter ablation can now be considered a standard option for the management of paroxysmal supraventricular tachycardias in children and young adults.

Primary hypertrophic osteoarthropathy: Another heritable disorder associated with patent ductus arteriosus

SummaryPatent ductus arteriosus (PDA) is a congenital malformation that has been linked to diverse heritable and chromosomal disorders. Primary hypertrophic osteoarthropathy (HOA) is a rare heritable syndrome in which digital clubbing and periostosis become evident without any underlying illness.The objective of this article is to describe four patients with coexisting PDA and primary HOA. Surgical closure of the ductus showed no effect on the skeletal changes.Primary HOA should be included among the heritable disorders that may be associated with PDA.

Resultados del reimplante coronario para la corrección del origen anómalo de la coronaria izquierda a partir de la arteria pulmonar

INTRODUCTION AND OBJECTIVES We report our experience in the surgical correction of anomalous origin of left coronary artery from pulmonary artery (ALCAPA), with an emphasis on the coronary reimplantation technique and its outcome. METHODS We designed a retrospective, longitudinal, descriptive study that included patients with ALCAPA who underwent surgery involving coronary reimplantation over a 19-year period. We describe perioperative details such as variations in the surgical technique and the postoperative outcome in terms of morbidity and mortality. RESULTS We studied 15 patients (86% females) with a mean age of 6.2 years (range, 2 months to 24 years). Heart failure was the principal cause for hospitalization in 80% of our patients. Left ventricular dysfunction was present in 67%, and 27% had significant or severe mitral valve regurgitation. We describe 3 surgical techniques for coronary reimplantation, the choice of which depends on the site of origin of the anomalous left coronary artery. Four patients underwent an additional mitral valve procedure. The most common immediate postoperative complications were low cardiac output (38%), pleural effusion (17%), and transient ischemia (13%). There was no operative or medium-term mortality. CONCLUSIONS Coronary reimplantation is the technique of choice for surgical correction of ALCAPA due to the excellent postoperative survival and low operative morbidity.

Congenitally corrected transposition with atrioventricular septal defect

We describe 4 cases of congenitally corrected transposition associated with atrioventricular septal defect, diagnosed by echocardiography and angiocardiography. Two had usual atrial arrangement and two had mirror imaged atrial arrangement . All cases were associated with subpulmonary valvar stenosis. All patients presented with cyanosis and were in sinus rhythm. Atrioventricular septal defect with common atrioventricular junction was easily diagnosed on the basis of a common atrioventricular valve permitting interatrial and interventricular communications. All patients had balanced right and left ventricles. The echocardiographic recognition of the ventricles was based on the presence of the moderator band within the morphologically right ventricle, the characteristics of the apical septal trabeculations, and the shape of the ventricles. Angiocardiographic recognition of the ventricles was achieved on the basis of right and left ventriculography. In one case with usual atrial arrangement, we recorded two His bundle potentials, one anteriorly and another posteriorly. Atrial stimulation revealed blocked atrioventricular conduction at the level of the posterior bundle, and normal atrioventricular conduction through the anterior bundle. In both cases with atrial mirror-imagery, only a posterior His bundle potential was found, with normal atrioventricular conduction revealed by atrial stimulation The clinical course with this combination depends on the other lesions present in addition to the common atrioventricular valve. Our electrophysiological studies show that the conduction system in presence of a common atrioventricular valve is as expected for congenitally corrected transposition with two atrioventricular valves.

Co-existence of congenital and rheumatic heart disease in 404 post-mortem cases—comparison with findings in 2,352 cases with isolated rheumatic disease

We reviewed 2,756 necropsy studies of patients who died because of rheumatic heart disease. The anatomic material was divided into two groups. The first group was made up of 404 cases (14.6%) having the association of rheumatic and congenital heartdisease. The second group comprised 2,352 cases with isolated rheumatic heart disease. The analysis of the site and types of congenital malformations in the first group showed 160 cases (39.6%) with aortic, 112 (27.7%) with pulmonary, 15(3.7%) with tricuspid and six (1.5%) with mitral lesions. Twenty patients (4.9%) had an atrial septal defect whilenine (2.2%) had patency of the arterial duct and four (1 %) a ventricular septal defect. The incidence of rheumatic aortic valvar disease in cases also having congenital lesions of the aortic valve (83.7%) was higher than in patients with isolated rheumatic involvement of the aortic valve (67.7%) (p