The adult patient with native coarctation of the aorta: balloon angioplasty or primary stenting?

OBJECTIVE: To compare results of dilatation of native coarctation of the aorta with and without stent implantation. DESIGN: Open, observational, non-randomised study. PATIENTS: 54 consecutive adult patients: 32 with balloon angioplasty alone (group 1) and 22 with stent placement (group 2). INTERVENTIONS: Balloon dilatation from 1995 to 1997; dilatation with Palmaz stent placement from 1997 to 1999. MAIN OUTCOME MEASURES: The primary end point was a composite index of failure including heart related death, a residual gradient of > 20 mm Hg, the need of reintervention, and aneurysm formation. RESULTS: Peak systolic gradient (mean (SD)) was reduced both in group 1 (from 63.3 (22.8) to 10.7 (10.8) mm Hg, p < 0.001) and group 2 (from 63.9 (20.8) to 2.7 (4.3) mm Hg, p < 0.001), but Delta change was significantly greater in group 2. A residual gradient of > 10 mm Hg was shown to be the best cut off point to separate risk groups, representing a hazard ratio (HR) of 9.59 compared with a residual gradient of < or = 10 mm Hg (95% confidence interval (CI) 1.92 to 47.8). From multivariate Cox regression analysis, the only risk marker was the residual gradient (HR 8.9, 95% CI 1.2 to 63.0). The type of the coarctation and the use of stent were the factors associated with a residual gradient of < or = 10 mm Hg. CONCLUSIONS: Mid term outcome in adult patients with native aortic coarctation receiving percutaneous treatment is strongly related to the immediate residual gradient. When treating these cases, efforts should be made to obtain gradients under 10 mm Hg, either by angioplasty alone or by placing a stent. Patients with discrete aortic coarctation have similar mid term results when the immediate residual gradient is < or = 10 mm Hg despite the implantation of a stent. To achieve these gradients, patients with hypoplastic isthmus or tubular coarctation should be treated with primary stenting. Further studies including exercise tests and non-invasive imaging are still needed before definitive conclusions can be drawn.

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.

[Fast pathway for heart surgery]. / Vía rápida para cirugía cardiaca.

The increasing demographic and life expectancy rates, together with the vertiginous technological development during the last two decades, have raised the number of cardiac patients requiring surgical treatment. Therefore, several institutions have been forced to give priority to advanced or more serious cases and to postpone those that do not demand an urgent surgery. This analysis was made from June 15, 1999 to June 15, 2000 and demonstrates the results obtained from maintaining a fast track at the National Institute of Cardiology "Ignacio Chávez". This has favored the practice of surgeries in cases with noncomplex pathologies, null mortality, and low morbidity rates. The analysis also compares results from patients who had been subjected to surgery for the same type of pathologies but following the usual course of admittance and surgery scheduling, proving to be cost-efficient. It also demonstrates the high incidence of inter-auricular communication and the Von Willebrand disease that exist in our environment. Based on the results, we propose to maintain and increase this type of surgeries, addressing their limitations.

[Congenital cardiopathies in adults]. / Cardiopatías congénitas en el adulto.

In the last 30 years, major advances have been made in the diagnosis and treatment of congenital heart disease in children. As a result, many children survive to adulthood, some of them with sequelae. In adults, the most common congenital heart disease is atrial septal defect. The most common causes of cyanotic congenital heart disease are the tetralogy of Fallot and Eisenmenger's syndrome. In this review we discuss the more common acyanotic and cyanotic congenital heart diseases that physicians who care for adults are likely to encounter.

Two- and three-dimensional echocardiographic unroofed coronary sinus.

We present the 2-dimensional findings and 3-dimensional reconstruction of images from an 18-year-old patient with unroofed coronary sinus, persistent left superior vena cava, a common atrium with levoisomerism, ventricular septal defect, and double-outlet right ventricle. The left superior vena cava showed continuity with the floor of the coronary sinus. Diagnosis of the constellation of anomalies established by transesophageal reconstruction clarified the continuity of the coronary sinus with left superior vena cava and atrial wall.

[Chromosome 22 (22q.11.2) deletion. Etiology of conotruncal heart abnormalities]. / Deleción en el cromosoma 22 (22q.11.2). Etiología de cardiopatías congénitas troncoconales.

Important advances in the diagnosis and treatment of congenital heart disease (CHD) have been made in the past 50 years. Nowadays echocardiogram plays an important role in the diagnosis. This procedure is able to identify a wide range of malformations. Cardiac catheterization is mainly a therapeutic tool, surgery is now performed much earlier because CHDS are diagnosed sometimes before birth or very early in life. All this advances in the diagnosis and treatment of this group of patients, allows them not only a better quality of life but also the possibility of reaching adulthood and having children. The study of the etiology of CHD is a field that has not evolved as fast as the assessment and treatment. Nowadays we have a larger population of adults with CHD. The discovery of a microdeletion of chromosome 22 q11.2 associated with conotruncal cardiac defects, proves a common etiology for clinical phenotypes and conotruncal malformations. In order to identify, which of these patients share the same etiology and presented with this syndrome, we collected a group that shared not only heart defects of the conotruncal type but also specific phenotypic alterations such as broad nasal bridge, nasal dimple, high palate, and digitalization of the first finger among others. The first two patients studied with FISH technique were positive to monosomy of a locus on chromosome 22. Those patients with CHD of conotruncal type should undergo microdeletion testing so genetic counseling can be offered as well as appropriate treatment in areas such as cardiology and developmental psychology.

[Electrocardiography and echocardiography aspects of hypertrophic myocardiopathy in pediatrics]. / Aspectos electrocardiográficos y ecocardiográficos de la miocardiopatía hipertrófica en edad pediátrica.

Hypertrophic cardiomyopathy (HCM) is a heterogeneous disease, characterized by asymmetric hypertrophy of the left and/or right ventricle with disarray of myocardial fibers. In order to know its clinical and electrocardiographic manifestation in the pediatric age group, we made a retrospective study of 24 cases from 1986 to 1995. There were: 15 girls and 9 boys, with a mean age of 6 years (age range: 1 month to 17 years). Clinical manifestations were dyspnea (71%), syncope (42%) and palpitations (42%). Physical examination disclosed an aortic systolic murmur in all patients, a mitral regurgitation in 42% and physical signs of congestive heart failure in 54% of patients. Chest X rays showed cardiac enlargement in 71% and pulmonary capillary hypertension in 42%. The most frequent ECG abnormalities were: a prolonged time in the intrinsecoid deflection onset on leads corresponding to the affected region, more or less deep and clean Q waves on leads aVF, aVL, V5 and V6, as well as supraventricular and ventricular rhythm disturbances in 11 patients (46%) with and without congestive heart failure. Bidimensional echocardiography confirmed antero-septal hypertrophy in all patients. The mortality rate was 17%. HCM is rare disease in the pediatric age group. Mortality increases when congestive heart failure and arrhythmias are present. Treatment must be individualized in all cases.

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.

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.

The syndrome of pericarditis, arthritis, and camptodactyly: an under-recognized cause of pericardial constriction in children?

The syndrome encompassing the combination of pericarditis, arthritis, and camptodactyly is a rarely described cause of pericardial constriction in children. It is likely that this association is being under-recognized. We report a new case in which the skeletal abnormalities were subtle. The syndrome should be included in the differential diagnosis of any child with persistent non-inflammatory pericardial effusion. A careful search at the bedside for the associated skeletal abnormalities should lead to the correct diagnosis. Pericardiectomy is the treatment of choice.

Ebstein's anomaly. Clinical profile in 174 patients.

The study population consisted of 148 patients who did not undergo surgical treatment and 26 who were operated, most of them diagnosed after the age of 2, with a follow-up from 6 months to 25.3 years. Patients were divided in three groups of clinical deterioration according to their functional class and cardiothoracic index (CTR) long-term follow-up in 148 nonoperated patients showed significant differences for mortality between groups I and III (p < 0.001), and between groups II and III (p < 0.02). Predictors of death included the association among functional class III or IV CTR > or = 65% with either cyanosis or arrhythmias (p < 0.05). The multivariate analysis showed that clinical deterioration (p < 0.0001), CTR (p < 0.0002) and functional class (p < 0.001), were significant for mortality. Kaplan-Meier analysis showed a survival rate of 81% in the overall patients free from surgical treatment. According to Kaplan-Meier analysis, the rate of survival was lower in patients with CTR > or = 65% (63.5%), in patients who had functional class IV (52.5%) and in patients included in group III of clinical deterioration (38.2%). Despite the fact that the association of functional class III or IV plus CTR > or = 65% with either cyanosis or arrhythmias is a good predictor for death, the mortality in patients who had only one of these variables was lower. Patients included in group II of clinical deterioration in stable condition presented long survival with medical treatment. Due to the high mortality rate found in group III, surgical treatment of Ebstein's anomaly must be done before deteriorating into group III. Surgical indication must be done considering the surgical risk of each group according to the experience of the Institution and comparing the rate of surgical mortality with the rate of survival without surgery.

Interventional cardiology in congenital heart disease.

Interventional procedures for congenital heart disease have evolved dramatically in the last decade. Beginning with pulmonary and aortic valvuloplasty, nowadays, stents and various devices are placed inside the heart and vessels to palliate or correct different congenital defects. The present communication summarizes the experience with interventional cardiology in congenital heart disease of the Instituto Nacional de Cardiología "Ignacio Chávez" during the last five years.

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.

[Endocarditis due to an alga of the genus Prototheca sp. A saprophyte of water and of tree sap?]. / Endocarditis por alga del género Prototheca sp. Saprofito del agua y de la savia de los árboles?

We report a case of a preterm neonate with very low birth weight (775 grs), He stayed 77 days in a neonatal care unit due to respiratory distress syndrome, with respiratory support for 27 days, and complications in gastrointestinal and nervous systems. In a structural normal heart, he developed infective endocarditis that was treated with good bacteriologic results. An echocardiogram showed the persistence of a mass in the right atrium, the mass was a thrombus and it was resected. Histopathological analysis and cultures of the thrombus reported Prototheca sp. that is an algae. The patient received medical treatment with amphothericin B with satisfactory clinical evolution. He is doing well 6 months later. This is the first report of literature of an algae causing endocarditis.

[Radiofrequency ablation in the treatment of supraventricular arrhythmias in pediatrics. Experience with 203 consecutive patients]. / Ablación con radiofrecuencia en tel tratamiento de las arritmias supraventriculares en pediatría. Experiencia en 203 Pacientes consecutivos.

We report the results of radiofrequency catheter ablation in 203 patients under 18 years of age with supraventricular tachyarrhythmias between April 1992 and June 1997. The presence of an accessory pathway caused the tachyarrhythmia in 181 patients (89.1%) with a total of 187 accessory pathways; atrioventricular nodal reentry caused the arrhythmia in 18 patients (8.8%) and atrial flutter in only 4 patients (1.9%). We eliminated the accessory pathway in 171 patients (91.4%), 23 patients showed recurrence of the tachycardia and we had complications in 4 patients (2.1%). The procedure was successful in the treatment of the atrioventricular nodal reentry in the 18 cases, with ablation of the slow pathway in 17 cases and in only one patient of the fast pathway, one patient showed total A-V block, and recurrence of the arrhythmia in 3 cases (16.6%). Finally the procedure was successful in the 4 cases of atrial flutter with one recurrence (25%). In the total of the series, the radiofrequency catheter ablation was successful in 193 patients (95%), with recurrence of the arrhythmia in 27 cases (13.3%) and with complications in only 5 patients (2.6%). Radiofrequency catheter ablation is a safe and effective procedure for the definitive treatment of supraventricular tachyarrhythmias in children.

[Fontan surgery in tricuspid atresia. Experience at the National Institute of Cardiology "Ignacio Chávez"]. / Cirugía de Fontan en atresia tricuspídea. Experiencia del Instituto Nacional de Cardiología "Ignacio Chávez".

A retrospective analysis is presented of all patients under 18 years of age with tricuspid atresia and concordant ventriculo-arterial connection who underwent a Fontan procedure in the National Cardiology Institute "Ignacio Chávez", México-City, between January 1989 and December 1995. In this period Fontan procedure was performed in 22 patients with a mean age of 6.52 +/- 1.4 years. 14 of them were females. Thirteen patients had previous palliative procedure. Age of palliation was 10 +/- 12 months with period of 5 +/- 2 years between palliative and Fontan procedure. Overall mortality was 31% (7 patients); with a mortality of 42% between 1989 and 1992, of 22% between 1992 and 1995 and of 17% since 1994 with all 5 patients operated in 1995 surviving. Mean pulmonary pressure was 13.9 +/- 4.15 mmHg, mean pulmonary vascular resistance 1.66 +/- 0.57 U.W/m2, left ventricular end diastolic pressure 7.5 +/- 2.46 mmHg, ejection fraction 58 +/- 11% and Nakata and McGoon indexes 219 +/- 56 mm2/m2 and 2 +/- 0.1 respectively. No significant differences were found survivors and non-survivors, with exception of the left ventricular end-diastolic pressure (p < 0.05). Causes of death were multiple organ failure in 2, ventricular fibrillation in 3 and septic shock in 2.