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We present the results and follow up of ductus arteriosus closure with the Rashkind device. Sixty-six devices were implanted in 63 patients, 41 women and 22 men, with a mean age of 8.2 years. We determined the diameter of the duct, the presence or absence of immediate residual leak at 3 months, 6 months, 1 year, and every year of follow-up by ecocardiography. The hemodynamic determinations showed: type A morphology in 49, type C in 7 and type E in 7; with a mean diameter of 4.2 +/- 1.4 mm (range of 2.3 to 8.7 mm) and a Qp/Qs 2.2 +/- 1.5 (range 0.7 to 8.6). Forty-five 17 mm devices and 18 of 12 mm were used. The incidence of immediate residual leak was 65% (n-41); at 24 hrs. Post-procedure was 31.7% (n-20) and at one year 7.9% (n-5). Three patients with persistent leak had implantation of a second Rashkind device and one more patient an endovascular coil, the other patient is waiting for a second occlusor. Minor complications occurred in 15.8% (n-10). Results show an occlusion rate at 24 hrs of 68% and at one year of 92%; no patient presented migration of the device, hemolysis or obstruction of the pulmonary artery branches. We consider that occlusion of the ductus arteriosus with the Rashkind device is a safe and effective option for the treatment of this pathology.

We present the initial experience of closing of patent ductus arteriosus (PDA) with a new device; Gianturco-Grifka, at the General Hospital of The Medical Center "La Raza". The patient was a 4 year's old girl, in whom we detected continuous murmur in the second intercostal space, echocardiography showed a long conical patent ductus arteriosus 4.9 mm of diameter, systolic pressure of the pulmonary artery was 35 mm Hg with QP/QS 1.6:1. Hemodynamic study revealed a long conical ductus arteriosus 5 mm of diameter, type A1 from Krichenko classification. We proceeded to occlude the PDA with a Gianturco-Grifka device of 7 mm. Immediately after the PDA occlusion the shunt disappeared, there were no complications during the procedure. More cases are needed to determine long term benefits and limitations, of this procedure. However we conclude that technically it is easy to use. There is greater decrease of residual shunt that the one reported with other devices.

This study was performed to evaluate the efficacy of transcatheter Coil and Grifka closure of the patent ductus arteriosus in comparison to our experience with the Rashkind umbrella device. Ninety seven patients (36 male and 61 females) underwent occlusion of the PDA. The following variables were compared: age, sex, ductus diameter, morphology, device, occlusion time, residual shunt, multiple device and complications. Data obtained were compared using the Student's test and Chi 2. Median patient age was 7.82 +/- 6.89, forty five patients underwent transcatheter Rashkind-17 closure, 19 patients with Rashkind-12, 18 patients with Coil and 13 patients with Grifka device, in two patients the closure could not be performed. There were differences between the morphology and device used (p = 0.008), between the ductus diameter and device used (p < 0.001). There was immediate closure in 26.7% in the Rashkind-17 group compared with, 57.9% for the Rashkind-12 group, 83.3% for Coil group and of 91.7% for Grifka group. The occlusion within 24 hrs of implantation was 60% with Rashkind-17, 78.9% with Rashkind-12, 94.4% with Coil and 100% with Grifka. Residual shunt persisted for more than a year in 7 patients with Rashkind-17 and 1 with Rashkind 12, (p = 0.001), in 4 patients two devices wore placed. The complications were, 15.5% for Rashkind-17, 26.3% for Rashkind-12, 5.2% for Coil and 30% for Grifka, (p = 0.004), one coil and one Grifka embolized. Transcatheter Coil and Grifka occlusion are more effective in achieving immediate closure than the Rashkind device. The indication to use each device is made according to the morphology and size of the ductus.

We present our early experience with the Gianturco-Grifka device for transcatheter closure of patent ductus arteriosus. Grifka occlusion was attempted in 13 patients (7 female, 6 male), median patient age was 6.5 years. Echocardiographic evaluation showed a mean ductal diameter of 5.47 +/- 1.09 mm, and the morphology was type C in 7 patients, type A in 5 and type E in 1 patient. The angiographic ductal morphology was 6 type A, 5 type C and 2 type E. Mean PDA diameter was 5.69 +/- 1.15 mm. Three patients had pulmonary hypertension. They were given supplemental oxygen (100%), decreasing their pulmonary pressures. A total of 13 devices were implanted, 5 of 7 mm and 8 of 9 mm. Twelve patients had complete ductal occlusion documented by aortic angiography (92%), one patient presented residual shunt (8%). Color echocardiography at 24 hrs documented complete occlusion in 12 cases. One device embolized to the descending aorta 2 hours after closure, and it was successfully retrieved in the catheterization lab. We conclude that the Gianturco-Grifka device is an appropriate alternative for transcatheter closure of the PDA. This technique can be performed in ductus arteriosus type C, D and E, with diameter < 9 mm. More clinical trials are needed to establish the long-term results of this technique.

The purpose of this study was to evaluate the immediate and long term results of pulmonary valve ballon dilation, and to determine the prognostic factors of failure in 109 patients (60 female and 49 male), aged 7.04 +/- 8.4 years. Seventy two patients (66.1%) had isolated valvular stenosis and 33.9% presented associated lesions. Peak systolic pressure gradient across the pulmonary valve decreased from 89.53 +/- 37.23 to 20.8 +/- 19.41 mmHg (p < 0.0001) after valvuloplasty. Forty three patients developed reactive infundibular stenosis after valvuloplasty with a systolic gradient of 19.65 +/- 35.64 mmHg. At a mean period of 8 years of follow-up the pressure gradient was 20.75 +/- 14.32 (p < 0.001). Valvuloplasty was successful in 86.2% of the cases with a global mortality of 1.9%, minor complications in 15.2%, and a failure rate of 13.8%. At follow-up restenosis developed in 6.7%. The comparative analysis between the groups of success and failure yield as significant risk factors for failure an age younger than 3.5 years and a pulmonary valve with displastic (p < 0.05) or combined morphology (p < 0.05). This group had also more complications and higher systolic gradients and right ventricular pressures post-dilation (p < 0.05). Pulmonary valvuloplasty is a safe and effective procedure for the treatment of pulmonary valve stenosis with a good immediate and long-term results, and is now considered the treatment of choice.

Deep venus thrombosis may result in pulmonary embolism. In rare instances, embolization has occurred, not directly to the pulmonary arterial tree, but to the right heart chambers. Although the value of echocardiography in the diagnosis is well recognised, their is no consensus for the appropriate treatment. We report herein six cases of floating right atrial thrombi, diagnosed by echocardiography, in patients with pulmonary embolism, or unexplained shock or syncope. Surgical embolectomy was carried out in 4 patients, and thrombolytic therapy in 2, without in-hospital mortality. The high mortality associated to this entity may be improved by rapid echocardiographic recognition and emergency treatment with thrombolysis or surgery. Our data suggest the possible use of thrombolysis as a first-choice therapy in selected 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.

The congenital junctional ectopic tachycardia, is an unusual tachyarrhythmia, with early clinic manifestation and poor antiarrhythmic drugs response with a great infant mortality reaching rates of 35%. It deserves a special attention in its timely detection as well as in its appropriate handling with different modalities of pharmacological and nonpharmacological therapy. We reported two cases in which age of presentation of tachyarrhythmia was at three months and whose particularity was the good response to antiarrhythmic drugs; verapamil and later propafenone, used for the acute stages and a combination of propafenone plus propranolol initially for the chronic stage. Due to intolerance it was necessary to change the treatment after a year for sotalol and digital with good response. We review the literature about this topic.

From April 1986 to June 1994 we performed percutaneous transvenous mitral commissurotomy in 689 patients with rheumatic mitral stenosis in a multicenter study. Mean age was 40 +/- 11 years, of then 84.9% female, 2.7% to had previous surgical treatment and in 1.4% the procedure was performed during pregnancy. Inoue balloon was used in 89.4%, double balloon 9.7% and monoballoon 0.9%. Mitral valve area (MVA) increased from 0.93 +/- 0.20 to 1.85 +/- 0.37 cm2 (p < 0.001) and mean pulmonary artery pressure from 31.5 +/- 15.8 to 22.4 +/- 11.5 mmHg (p < 0.001), mean left atrial pressure decreased from 20.9 +/- 8.1 to 10.0 +/- 5.9 mmHg (p < 0.001), transvalvular gradient (TVG) from 15.4 +/- 6.4 to 3.4 +/- 3.1 mmHg (p < 0.001) and mean pulmonary artery pressure from 31.5 +/- 15.8 to 22.4 +/- 11.5 mmHg (p < 0.001). Complete procedure without mayor complications was achieved in 93.1%. Severe mitral regurgitation (MR) was present in 3.9%. Optimal result in 82.1%, suboptimal in 8.2% and failure in 9.7%. Major complications 4.7%. Mortality was 0.9%. Six months follow-up MVA decreased to 1.77 +/- 0.38 (p < 0.001) and no changes to 24 months (1.78 +/- 0.37 p ns). Twenty four months follow-up 93.3% are in NYHA class I. Only MVA (> 1 cm2) and good predilatation NYHA class were predictors of optimal results. Severe MR were more frecuently in patients with atrial fibrillation and with high score (> 8). Our results were similar the international experience. We conclude that the technique of PTMC is a safe and effective technique.

From October 1985 to February 1992 we performed 80 percutaneous transluminal angioplasty (PTA) in 76 patients with coarctation of the aorta (CoAo). Sixteen of them with ages ranging from 12 to 62 years (mean = 21.1). We describe the experience in these cases. Fifteen with native and one with post-surgical coarctation. The gradient decreased from 72 +/- 33 to 18 +/- 17 mmHg immediately after dilation, in the follow-up (1 to 69 months m = 25) was 23 +/- 20 mmHg. In one patient we performed simultaneously angioplasty of CoAo and mitral valvuloplasty with excellent results in both lesions. We redilated two cases for residual gradient successfully. We had one failure in a patient with long coarctation. He needed surgery. In the initial experience we had one severe complication (cerebral stroke). No deaths or aneurysms. In conclusion we believe that PTA is an adequate alternative in adolescents and adults with native or post-surgical coarctation of the aorta with minimum incidence of complications.

The rate of conjoined twins is 1 in 50,000 births. These patients usually have a common pericardial sac (90%) and conjoined hearts (75%). In case of shared heart there is a communication by a vascular channel between both atria and ventricles. If there is not ventricular communication, surgical separation is possible. We describe such a case in whom in spite that interventricular connections were present; surgical separation was decided because of the critical conditions of twin A in an effort to preserve the survival of twin B.

We present the long term follow-up of two groups of patients under 16 years of age in whom two different types of mitral valve prostheses were used for the treatment of mitral valve disease. Group I was formed by 74 patients with Starr-Edwards (6120) prosthesis and group II by 36 cases with Bjork-Shiley prosthesis. Group I was followed for a mean period of 11 years and group II for a mean period of 7.5 years. The clinical evolution of both groups assessed according to the New York Heart Association criteria showed improvement of the functional class (p less than 0.006). Twenty seven cases of group I and 21 of group II underwent cardiac catheterization for the evaluation of surgical results. In group I, the mean pulmonary arterial systolic pressure (PASP) decreased from 57 mmHg to 33 mmHg (p less than 0.001) and the mean pulmonary arterial wedge pressure (PAWP) from 20.8 mmHg to 12.1 mmHg (p less than 0.05). In group II, the mean PASP decreased from 59.2 mmHg to 28.5 mmHg (p less than 0.001) and the mean PAWP from 24.6 mmHg to 8.5 mmHg (p less than 0.001). There was statistically significant difference for a greater incidence of thromboembolism in group I (p = 0.037). No differences were found for bacterial endocarditis, dysfunction and death.

Percutaneous transluminal angioplasty (PTA) was performed in 34 patients with aortic coarctation (Ao Co). One of them with coarctation after surgical correction, the rest were native Ao Co. We used one balloon in 28 patients and two balloons simultaneously in 6. They were separated in three groups according to the degree of aortic arc hypoplasia. Group I (mild to moderate hypoplasia N = 9) the gradient dropped 39% with angiographic improvement of 48% during the follow-up (m = 13.1 months). Three cases with restenosis, 2 were satisfactory dilated and one was sent to surgery. In Group II (severe hypoplasia N = 4) the gradient dropped 31% with angiographic improvement of 30% (follow-up 16.3 months). Two cases with recoarctation were sent to surgery. In Group III (without hypoplasia N = 21) we obtained dropped of gradient of 71% with angiographic improvement of 60% (follow-up 18.5 months). Two cases were redilated successfully. The complications were: cerebral hemorrhage with death due to hypertensive crisis, (1) cerebral embolism, (1) thrombosis in the puncture site 1 and small aneurysm in dilated zone. (1) We think PTA is a good choice to conventional surgery with low rate of morbidity-mortality. The results depend basically on the anatomic type of coarctation and degree of aortic arch hypoplasia.

Double-outlet right ventricle (DORV) is a type of ventriculo-arterial connection, in which both great arteries are connected to the morphologically right ventricle in at least 50% of their diameter. The relation of the great arteries with the ventricular septal defect (VSD) and the pulmonary stenosis, are important anatomical aspects in the election of the surgical technique. In this study we describe four patients with DORV, in whom different surgical techniques were used according to the specific anatomy of each case. Two patients had subaortic restrictive VSD, with subpulmonary stenosis in one of them. In the other two patients the VSD was subpulmonary with the aorta anterior and to the left, in one of these existed moreover a severe mixed pulmonary stenosis. It is concluded, that with different surgical techniques according to the specific anatomy of each patient, good results can be obtained in the surgical treatment of these complex congenital malformations.

It is well known, that in complex congenital heart disease, the bronchial anatomy reflects the atrial situs in the majority of patients. Few exceptions to this rule have been reported in the literature. We report four patients in whom we found discordance between the anatomy of the bronchial tree and the external aspect of the atrial appendages. Two patients had bronchial situs solitus with atrial levoisomerism, the third patient had bronchial levoisomerism with atrial situs solitus and in the fourth one we found inverted bronchial anatomy with levoisomerism of the atrial appendages. In three patients, broncho-atrial discordance was diagnosed clinically by bronchial tomography and selective atrial angiography, and in the other one the diagnosis was made by anatomical study. In this last patient, external atrial anatomy was found to be of levoisomerism, with an internal atrial aspect of situs inversus. Prediction of atrial arrangement by bronchial anatomy retains its diagnostic value. However, it is necessary to bear in mind that exceptions do exist. It is discussed how to suspect these exceptions, and the final diagnostic conduct is indicated.

We describe one patient with crossed atrioventricular connection in situs inversus, concordant atrioventricular connection, double outlet right ventricle, and straddling of the right atrioventricular valve. Angiographic features are analyzed, pointing out the importance of selective angiography in order to define the atrioventricular and ventriculoarterial connections. The utility of bidimensional echocardiographic study before cardiac catheterization is emphasized in order to know the associated malformations.

We describe the first case of balloon pulmonary valvuloplasty performed in this country. This 12 year old girl had a transvalvular gradient of 94 mmHg and after the procedure it improved to 20 mmHg. Four months later, cardiac catheterization did not show any significative change in pulmonary valvular gradient (26 mmHg). We describe the technique, hemodynamic findings, complications, and advantages of this procedure. We also review the literature. We consider that balloon valvuloplasty should be the first choice procedure in the treatment of patients with congenital pulmonary valve stenosis.

We describe the first case of BAS in our country in a three months old child with transposition of the great arteries, restrictive atrial septal defect (RASD) and intact interventricular septum. When he was 15 days old, we performed a balloon atrial septostomy. He had temporal improvement and six weeks later his cyanosis increased, and a new catheterization showed systemic arterial oxygen saturation of 30%, RASD and an interatrial pressure gradient of 2.1 mmHg (left atrium LA: 3.9 and right atrium RA: 1.8). We decided to perform a new septostomy with Park's blade atrial septostomy catheter. After the procedure the interatrial pressure gradient decreased to 0.2 mmHg (RA: 4.3 and LA: 4.5), the angiography shunt and atrial pressures increased. Five months later the child is alive and the systemic arterial oxygen saturation is 51.3%. The technique, advantages and complications are described.

Percutaneous transcatheter balloon mitral commissurotomy was performed in an 11 year old girl. The atrial septum was perforated and an 8 mm angioplasty balloon was advanced the atrial septal perforation was then dilated to allow passage of 25 mm balloon valvuloplasty catheter across the mitral annulus. Transmitral balloon inflation acutely decreased the end diastolic transmitral gradient (23 mm Hg to 2 mm Hg). Immediate gradient reduction was associated with increases in cardiac output (3.4 to 5.3 L/min). Murmur intensity diminished immediately post-commissurotomy. Balloon commissurotomy did not produced mitral regurgitation. Five weeks later, follow-up catheterization was performed and did not show change in mitral valvular gradient (2 mmHg). Clinical improvement was evident.

Congenital diverticulum of the heart is an unusual malformation. It is frequently associated with thoracic or abdominal midline alterations and also with various types of congenital heart disease. Complications of this anomaly are rupture, peripheral embolism and heart failure. Surgical management consists of resection of the diverticulum either as an isolated operation or at the time of correction of accompanying malformations. We present six cases of congenital diverticulum of the heart, four on the right ventricle and two on the left. In one patient with Fallot's tetralogy a diverticulum was found at necropsy; in two cases the underlying malformation was double outlet ventricle; one case had pulmonary artery stenosis and two did not have associated malformations.

We describe the first case in our hospital of percutaneous transluminal angioplasty in a 16 years old boy with congenital coarctation of the aorta. The technique, hemodynamic findings, angiography before and after dilation and the advantages in comparison with the conventional cardiac surgery were analyzed. In our case the gradient decreased from 60 mmHg to 28 mmHg. We conclude that this procedure is an alternative treatment for patients with congenital coarctation of the aorta.

The clinical presentation and long-term follow-up of 14 cases of tricuspid atresia associated with pulmonary atresia were reviewed. The electrocardiograms, hemodynamic findings and a definition of anatomic types were outlined in order to facilitate therapeutic decisions. In these types of tricuspid atresia the clinical presentation depends on the patent ductus. Despite the caliber of the ductus arteriosus, the poor tolerance to the malformation is frequent and the clinical presentation is similar to those malformations with decreased pulmonary blood flow (cyanosis since birth and hypoxic spells). In considering the age of these patients, the modified Blalock-Taussig anastomosis is the initial procedure. The Fontan procedure should be considered carefully as an alternative in older children.

Patients with pulmonary atresia and intact ventricular septum have a poor prognosis with or without conventional surgical treatment. The best results of surgical treatment are obtained in those cases with a mild underdeveloped right ventricle and minor sinusoidal communication in the absence of important dysfunction of the tricuspid valve. We present five cases of pulmonary atresia with intact ventricular septum associated with dysfunction of the tricuspid valve. On the basis of radiographic, electrocardiographic and hemodynamic findings, this group of patients could not be distinguished from others without dysplasia of the tricuspid valve. Echocardiographic and angiocardiographic studies are mandatory in the differential diagnosis. A combination of systemic-pulmonary artery anastomosis associated with pulmonary valvotomy, when possible, and reconstruction of the right ventricular outflow tract are indicated for surgical solution of these malformations. However, tricuspid valve replacement is indicated in some cases.

We performed catheter balloon valvuloplasty (CBV) on 8 stenotic operatively-excised bioprosthetic valves (2 Hancock and 6 Ionescu Shiley). Pathology of valves before CBV included degenerative changes: commissural fusion by mounds of calcific deposits (2 valves), fibrotic and focally calcified leaflets (7 valves) and stiff and thick valves (1 valve). Inflation of the balloon resulted in commissural splitting (2 valves), leaflet cracks and fractures (3 valves). Removal of the deflated balloon catheter was associated with debris dislodgement (3 valves). In one case the valve was unable to close with potential for acute regurgitation. Thus, CBV of bioprosthetic valves can split fused commissures by similar mechanisms as in native valves. CBV may fracture calcific deposits causing acute emboli. It can also disrupt the leaflets causing acute insufficiency. The findings suggest a limited role of CBV in the treatment of stenotic bioprosthetic valves in mitral and aortic position.