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.

[Interventional catheterization in congenital heart diseases]. / El cateterismo intervencionista en las cardiopatías congénitas.

Since 1953 when the first percutaneous valvotomy procedure was performed at the Instituto Nacional de Cardiología "Ignacio Chávez", interventional cardiac catheterization applied to congenital heart disease has developed greatly, mostly in the past 15 years. From atrial septostomy to occlusion of intracardiac defects, this therapeutical modality has increased its applications, and has given us additional resources to treat our patients with congenital heart defects. In this article we present a general perspective of our experience with these new techniques.

[Radiofrequency ablation and simultaneous insertion of an Amplatzer device in a patient with atrial septal defect and nodal reentry tachycardia]. / Ablación con radiofrecuencia y colocación simultánea de un dispositivo Amplatzer en un paciente con comunicación interatrial y taquicardia por reentrada intranodal.

The association of AV nodal reentry tachycardia and interatrial septal defect has not been described previously. This article reports a 47-year-old woman with an atrial septal defect (ostium secundum) and history of palpitations without documental tachycardia through resting and ambulatory electrocardiography. Rapid atrial pacing under the influence of isoproterenol, during an electrophysiology study, elicited and AV nodal reentry tachycardia of the common type. Successful radiofrequency catheter ablation was performed targeting the slow pathway. Immediately an Amplatzer device was used for closure of the septal defect. Both procedures were successful and without complications. What makes this case unusual is that both interventional procedures were performed during the same session.

[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.

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.

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.

[Percutaneous closure of an interatrial communication with the Amplatzer device. The first case in Mexico]. / Cierre percutáneo de comunicación interatrial con el dispositivo Amplatzer. Primer caso en México.

The Amplatzer septal occluder is a new device intended for the percutaneous closure of secundum atrial septal defects that has distinctive characteristics and important advantages over other available devices. We present the first successful closure of an atrial septal defect with this device in Mexico in a 19 year old man with a 16 mm secundum type septal defect.

[Fenestrated Fontan surgery in high risk patients]. / Cirugía de fontan fenestrado en pacientes de alto riesgo.

A retrospective analysis is presented of all patients who had fenestrated Fontan procedure between january 1990 and may 1996. Surgery was indicated in the presence of anyone of the following risk factors: mean pulmonary pressure higher than 20 mmHg; pulmonary vascular resistance higher than 2 UW; ejection fraction less than 60%; systemic ventricular end diastolic pressure higher than 8 mmHg; Nakata index less than 200 mm2/m2, McGoon index less than 2. The diagnosis were: Absent right atrio-ventricular connection with concordance ventriculo-arterial connection 10 patients; pulmonary atresia with intact septum, 1 patient; Ebstein's malformation, 1 patient and absent left A-V connection with discordance VA connection, 1 patient. The mean of age was 6.7 years (range 2.5-11 years). Overall mortality was 23%. No significant difference in risk factors was found between survivals and no survivals. Nonsurvivors had between two an four risks factors. Postoperative complications were 1 patient with protein losing enteropathy and stroke (1 patient). The mean duration of pleural effusion was 16 days (range 4-45 days). We consider fenestrated Fontan procedure useful for patients with congenital heart disease with a one hypoplastic ventricle and one o more risks factors.

[Tetralogy of Fallot in adults]. / Tetralogía de Fallot en adultos.

We present the clinical and hemodynamic profile of 33 patients, older than the 18 year with tetralogy of Fallot. Cardiac catheterization and selective angiocardiography were performed in all cases. We excluded patients with valvular pulmonary atresia associated with tetralogy of Fallot. Most of the patients were in functional class II or IV of the New York Heart Association and all presented with cyanosis. In the electrocardiogram of 23 patients we found right bundle branch block. None had significant cardiomegaly, 19 of 20 cases had reduced pulmonary blood flow and reticular pattern in the lung fields as observed in the chest X-ray. We did not find correlation between the age and the degree of cyanosis, but the level of arterial desaturation correlated with the functional class. The right ventricular systolic pressure was elevated in all patients. In all cases but one the pulmonary arterial systolic pressure were normal of slightly increased. One case with mild pulmonary infundibular stenosis had pulmonary systolic pressure similar to the systemic pressure. Multivariate analysis for functional class showed significant value for cyanosis. Systemic-pulmonary shunt was performed in 10 patients with hypoplastic pulmonary arteries plus reduced pulmonary blood flow, with one postoperative death. We did not find postoperative mortality in patients who underwent corrective surgery. The anatomic and functional behavior of patients who underwent surgery, allowed better tolerance to their heart defects.

[Reconstructive surgery of rheumatic mitral valvulopathy in patients under 18 years of age]. / Cirugía reconstructiva de valvulopatía mitral reumática en menores de 18 años.

OBJECTIVE: To present our experience in 58 children (37 female, 21 male) aged 4 to 18 years (mean +/- SD = 14.9 +/- 2.7) with rheumatic mitral valve disease who underwent surgical reconstruction. METHODS: They were divided in four groups according to the abnormalities of the mitral apparatus: Group I had mitral regurgitation due to lesions located in the valvar structures with normal valvar movement (n = 1, 2%), group II mitral regurgitation with lesions located mainly in subvalvular structures with valve prolapse (n = 11, 19%), group III with mitral regurgitation due to lesion located both in valvular and subvalvular structures and restricted valvar motion (n = 38, 65%), and group IV included patients with stenosis (n = 8, 14%). RESULTS: Surgical mortality was 5% (3/58) and three patients (5%) were reoperated immediately. The 52 remaining patients were followed from 6 to 108 months (mean 45.8 +/- 30.1 months, 199 patient-years). Six cases were reoperated in the follow-up (3.0% per patient-year). The functional class using the criteria of the New York Heart Association was evaluated before and after the procedure: class I raised from two to 42 patients whereas it decreased in the others (class II from 13 to 7, class III from 38 to 3, class IV from 5 to none; p < 0.001). The cardiothoracic index decreased from 0.61 +/- 0.064 before surgery to 0.55 +/- 0.069 after surgery (p < 0.001). Thirty patients (57%) were evaluated with echo-Doppler in the follow-up period. There were no deaths in the follow-up period. CONCLUSIONS: Our data shows that reconstructive surgery of the mitral valve with rheumatic involvement offers good immediate and late results, with a low reoperation rate, avoiding the use of prosthetic valves as a first option.

[Corrected transposition of the great arteries in patients over 65]. / Transposición corregida de las grandes arterias en pacientes mayores de 65 años.

Three cases with corrected transposition of the great arteries in patients older than 65 years are described. Two had atrial situs solitus and one atrial situs inversus. One had pulmonary valvular stenosis with valvular calcification and a small ventricular septal defect in association with ischemic heart disease. This patient died due to cardiac failure at the age of 80 years. The second case was associated with ventricular septal defect, atrial septal defect and pulmonary hypertension. The third patient presented with mild tricuspid regurgitation. Although this congenital heart malformation is theoretically compatible with normal life, few patients have long survival because of associated congenital defects or the subsequent development of tricuspid regurgitation or atrioventricular block.

[Radiofrequency ablation of an accessory left lateral pathway in an infant, using a transeptal approach]. / Ablación por radiofrecuencia de una vía accesoria lateral izquierda en un lactante, utilizando un abordaje transeptal.

We describe the case of a 4 month old child with supraventricular tachycardia detected on his second day of life, refractory to medical treatment, who was treated with radiofrequency catheter ablation of an accessory auriculoventricular pathway located on the left lateral wall. A venous femoral approach was used and the left atrium was entered through a patent foramen ovale, avoiding the risks of the arterial puncture and allowing us to administrate the radiofrequency current on the proper site. We discuss the potential risks of the therapeutic procedure in children and the benefits of the transeptal over the retrograde approaches for the treatment of left sided accessory pathways. Our conclusion is that catheter radiofrequency ablation of accessory pathways in children is possible with low risk, which can be diminished with the use of the transseptal approach when indicated.

[Truncus arteriosus communis and interrupted aortic arch]. / Tronco arterioso común e interrupción del arco aórtico.

The association between persistent truncus arteriosus and interrupted aortic arch is a rare but important condition since it has special clinical and therapeutic implications. In this article we present three cases with this malformation. The clinical characteristics are similar to patients with isolated truncus such as heart failure and cyanosis since birth, a protosystolic click, an ejection murmur over the left parasternal line, heart enlargement, increased pulmonary vascular markings and biventricular hypertrophy. The clinical suspicion of interrupted aortic arch is bases on the presence of asymmetric pulses in the upper and lower limbs, but this difference is present only when the arterial duct is restrictive. Because of that, the echocardiographic analysis, mainly, and the angiocardiogram, secondarily, are very important diagnostic tools. We analyze the echocardiographic diagnostic findings, as well as the angiocardiographic anatomy. Also, some important pathophysiological aspects are discussed.

[Pulmonary balloon valvulotomy. The results in 42 cases]. / Valvulotomía pulmonar con balon. Resultados en 42 casos.

The percutaneous balloon valvulotomy is nowadays the treatment of choice for isolated pulmonary stenosis. Forty two patients with congenital pulmonary stenosis underwent balloon valvulotomy at the Instituto Nacional de Cardiología "Ignacio Chávez". Ages 1 month to 24 years, mean 7.6 +/- 5.9 years, with a follow-up of 28.3 +/- 14.8 months. With an infundibular gradient of 50 mm Hg or more a treatment with propranolol was given. After valvulotomy the total basal pressure gradient decrease from 82.9 +/- 40.6 mm Hg to 31.2 +/- 27.1 mm Hg (p < 0.00001). The patient's results were divided in two groups: with and without reactive infundibular obstruction. The 31 patients without infundibular obstruction had a total initial gradient of 71.6 +/- 33.5 mm Hg, and after valvulotomy the gradient was reduced to 18.9 +/- 11.2 mm Hg (p < 0.00001). The 11 patients with infundibular obstruction had a total initial gradient of 114.8 +/- 43.2 mm Hg, and after valvulotomy the valvular gradient was 12.4 +/- 8.5, the infundibular gradient 53.4 +/- 22.9 and the total gradient 65.9 +/- 29.1 mm Hg (p = 0.002 in relation to the basal gradient). All patients with infundibular obstruction (8 treated with Propranolol) and a follow-up of 8.5 +/- 9.8 months underwent Doppler examination, showing a progressive reduction of the total gradient. By six months no one had a total gradient greater than 50 mm Hg. These data confirm that balloon valvulotomy in valvular pulmonary stenosis is safe and effective, and that restenosis is very low. Good results relay on the use of proper balloon diameters (balloon diameter/pulmonary annulus relation of 1.2 to 1.5).(ABSTRACT TRUNCATED AT 250 WORDS)

Primary hypertrophic osteoarthropathy: another heritable disorder associated with patent ductus arteriosus.

Patent 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.