Implantación de stents en cardiología pediátrica. Resultados iniciales / Use of stents in pediatric cardiology. Initial results

Antecedentes y objetivo: Un stent es un dispositivo útil en el tratamiento de lesiones estenóticas vasculares asociadas a cardiopatías congénitas. En el año 1997 iniciamos nuestra experiencia, cuyos resultados se exponen en este trabajo. Pacientes y método: Se han implantado 17 stents, en 12 niños con edad media de 82 meses (2-168 meses) mediante 13 procedimientos. Ocho pacientes tenían estenosis de ramas pulmonares posquirúrgicas; 3 pacientes recoartaciones de aorta y uno, cardiopatía compleja dependiente del ductus. En los primeros 8 pacientes se efectuó cateterismo derecho y se siguió la técnica habitual de implantación con guía de alto soporte y vaina de Mullins. En los restantes el abordaje fue por vía arterial femoral. Se utilizaron 13 stents de Palmaz; 3 stents Express; y uno, Intrastent. Resultados: El diámetro de las lesiones se incrementó tras el implante desde 3,1 (2,8-5,6) hasta 12 (10-15) mm (p<=0,002), y el gradiente a través de las estenosis descendió desde 32 (21-45) a 8,5 (0-11,5) mmHg (p<=0,002). En el paciente dependiente del ductus permitió la suspensión de prostaglandinas y el alta hospitalaria. Tras un seguimiento medio de 27 meses (1-60) ha habido una oclusión completa tardía de rama pulmonar y una parcial de arteria lobular superior derecha. Un enfermo falleció por causas ajenas al stent (shock cardiogénico previo), el resto están asintomáticos y no precisan medicación. Conclusión: El stent es un dispositivo seguro y eficaz. Puede ser utilizado en una amplia variedad de lesiones estenóticas (AU)

[Use of stents in pediatric cardiology. Initial results]. / Implantación de stents en cardiología pediátrica. Resultados iniciales.

BACKGROUND AND AIM: Balloon-expandable stents are useful in treating vascular stenotic lesions associated with congenital heart defects. We report our experience of this device since 1997. PATIENTS AND METHOD: Seventeen stents were implanted in 12 children in 13 procedures. The mean age of the patients was 82 months (range: 2-168 months). Eight patients had postsurgical stenoses in branch pulmonary arteries, three patients had aortic re-coarctation and one patient had a ductus-dependent complex heart defect. In the first eight patients we performed right catheterization, following the usual technique of percutaneous implantation with super-stiff wire and Mullins sheath. In the remaining patients, vascular access was through the femoral artery. The Palmaz stent was used in 13 patients, Express stents in three and Intrastent in one patient. RESULTS: After stent implantation, the diameter of the narrowings increased from 3.1 mm (2.8-5.6) to 12 mm (10-15) (p < 0.002) and the gradient was reduced from 32 mmHg (21-45) to 8.5 mmHg (0-11.5) (p < 0.002). In the patient with ductus-dependent heart defect, the procedure allowed prostaglandin E1 withdrawal and hospital discharge. After a mean follow-up of 27 months (range: 1-60 months) one occurrence of late complete occlusion of branch pulmonary artery and one case of side-branch partial occlusion (right superior lobe branch) have been detected. One patient died from causes unrelated to the stent (prior cardiogenic shock). The remaining patients are asymptomatic and medication free. CONCLUSION: The stent is a safe and effective device that could be used in a wide variety of stenotic vascular lesions.

[Fibrinolytic treatment with tissue plasminogen activator in a thrombosed modified Blalock-Taussig shunt]. / Tratamiento fibrinolítico con activador tisular de plasminógeno de una fístula modificada de Blalock-Taussig trombosada.

Thrombolytic therapy of a systemic pulmonary fistula is a rare method of treatment in newborns with thrombosed systemic-pulmonary shunt. We report the case of a newborn girl with a complex congenital heart defect. On the ninth day of life a modified right Blalock-Taussig shunt was performed. The ductus arteriosus was not ligated. Six days later the baby developed severe hypoxemia. The results of echocardiography and cardiac catheterization showed a total thrombosis of the fistula and complete absence of flow throughout the shunt. After insertion of an arterial catheter into the proximal end of the shunt we started fibrinolytic treatment with recombinant tissue plasminogen activator (r-TPA) in continuous infusion. After 14 hours of treatment we confirmed by angiography complete clot dissolution. The baby left the hospital in good condition when she was 23 days old. In the follow-up (4th month of life) the shunt is still permeable. Thrombolytic therapy with r-TPA locally applied in case of acute thrombosis of a systemic-pulmonary shunt appears to be a good therapeutic option avoiding the risks of a new surgical procedure.

[Congenital coronary fistula to right ventricle. Treatment with transcatheter coil embolization]. / Fístula coronaria congénita a ventrículo derecho. Tratamiento mediante embolización transcateter con coils.

We report the case of a 3 year old girl with a congenital right coronary artery-right ventricle fistula demonstrated by two dimensional echocardiography with color-flow Doppler and cardiac catheterization and angiography. The treatment was percutaneous transcatheter embolization by two detachable coils (7 mm x 20 cm and 6 mm x 20 cm) with successful complete occlusion. There were no complications.

[Progressive subaortic stenosis caused by accessory mitral valve tissue]. / Estenosis subaórtica progresiva causada por tejido mitral accesorio.

Accessory mitral valve tissue is a rare cause of subaortic obstruction. The reported case correspond to a two days old patient diagnosed by 2D-Echocardiography. Serial doppler flow analysis showed progressive left ventricular outflow obstruction. A cardiac catheterization at the age of 9 months confirmed the obstruction without defining the cause. Five months later, transesophageal echocardiography clearly defined the intraventricular connections of the accessory mitral tissue. At the age of 18 months the patient suffered from cardiac failure and underwent surgery for removing the accessory tissue, assisted by intraoperative transesophageal echocardiography pre and post by-pass. Follow-up echocardiography at 25 months showed no left ventricle outflow obstruction and the patient is now asymptomatic.

[Subpulmonary obstruction caused by an aneurysm of the membranous ventricular septum. A study of 2 cases]. / Obstrucción subpulmonar causada por aneurisma del septo ventricular membranoso. Estudio de dos casos.

Aneurysms of the membranous ventricular septum can exceptionally produce subpulmonary obstruction. We report two patients, an 8-year-old and a 2-year-old respectively, with a perimembranous ventricular septal defect and subpulmonic stenosis caused by an aneurysm of the membranous septum. Diagnosis was made by Doppler two-dimensional echocardiography and was confirmed by cardiac catheterization and was surgery. It is shown that this obstruction may appear late due to the growth of the aneurysm. In conclusion, we believe that the appearance of an aneurysm on a ventricular septal defect is not always beneficial, as it may result in right ventricular outflow tract obstruction or other kinds of complications which may require cardiac surgery.

[Angioplasty of residual lesions after correcting surgery of transposition of great vessels]. / Angioplastia de lesiones residuales tras cirugía correctora de transposición de grandes vasos.

BACKGROUND: Surgical correction in infants born with transposition of the great arteries, venous rerouting procedure (Mustard or Senning operation) or an arterial switch operation, can be complicated by vascular narrowings. Balloon dilatation angioplasty may be the treatment for these stenotic lesions and is attempted to avoid a reoperation of these patients. MATERIAL AND METHODS: Angioplasty was attempted in nine patients after the surgical correction of transposition of the great arteries. Senning procedure was performed in sixty-one patients; two patients (3.2%) were successfully balloon-dilated for systemic venous obstruction: superior vena caval obstruction (case 1) and inferior vena caval obstruction (case 2). Arterial switch operation was performed in forty patients, balloon angioplasty was attempted in seven patients (17.5%) who developed supravalvar pulmonary artery stenosis. Mean age at dilation was 32.5 +/- 20.7 months (range 11 to 67) in the switch arterial group. Age at dilation in the Senning group was 8 and 106 months. The balloon/stenosis diameter was 2 to 3 in the switch arterial operation and 3 to 8 in the Senning procedure. RESULTS: Balloon dilatation angioplasty was successful in three patients with supravalvar pulmonary stenosis postarterial switch operation (mean age was 16.6 months) and reduced the peak-to-peak gradient from 78.6 +/- 36.6 mmHg to 44 +/- 15 mmHg. There was no success after angioplasty in three patients and it was not possible to cross the stenosis with the guidewire in one (mean age was 46 months). Balloon dilatation angioplasty was successful in the obstruction following Senning procedure. CONCLUSIONS: These data indicate that although the success rate of balloon angioplasty for supravalvar pulmonary stenosis after the arterial switch operation is low, balloon angioplasty can be the first therapeutic choice owing to its low complication rate and the potential benefit of the procedure. The success rate should be higher if angioplasty is performed at a younger age after operating and a balloon of adequate size is used. Postoperative systemic venous obstructions can be successfully managed with balloon dilatation.

[Restrictive myocardiopathy in children. A study of 4 patients]. / Miocardiopatía restrictiva en el niño. Estudio de cuatro pacientes.

Restrictive cardiomyopathy is an unusual form of cardiomyopathy during childhood. Only occasional cases or reduced populations have been reported in international journals. The purpose of this report is to document the clinical, echocardiographic and hemodynamic profile and outcome of restrictive cardiomyopathy in a group of four children (two boys and two girls). The mean age at admission was 3.5 +/- 2.7 years (range 1(3/12) to 7(10/12) years). The diagnosis was made by clinical, echocardiographic and hemodynamic criteria. The main symptoms were bronchial disease (two patients), hepatomegaly and a cardiac murmur. All of the patients had impaired growth and physical development, normal or near-normal Rx cardiothoracic ratio, evidence of congestive heart failure and only one of them showed a cardiac murmur. The most common ECG finding was biatrial enlargement and incomplete right-bundle branch block. The echocardiographic feature was severe biatrial dilatation in the presence of normal ventricular cavity size. Left ventricular shortening was normal and there was diastolic dysfunction. Doppler mitral flow was restrictive, with an E/A ration > 1.5. Marked elevation of left ventricular end-diastolic pressure was noted in cardiac catheterization (x = 32 +/- 7 mmHg). Right ventricular end-diastolic pressure was elevated, but less than the left side (x = 19 +/- 12 mmHg). The outcome was very poor. Two patients died, one remains with heart failure NYHA II and the other with heart failure NYHA IV. We conclude that restrictive cardiomyopathy during childhood may simulate a bronchial or liver disease, so we must bear this in mind in order to make the differential diagnosis. Because of the poor prognosis and lack of specific treatment, the best therapeutic option may be heart transplantation.

[Doppler echocardiographic evaluation of pulmonary hypertension in children]. / Valoración con ecocardiografía Doppler de la hipertensión pulmonar del niño.

INTRODUCTION: We analysed the usefulness of Doppler echocardiography to determine the presence and severity of pulmonary hypertension (PH) in children. METHOD: The whole group consisted of 63 patients, 42 with congenital heart disease that underwent cardiac catheterization (32 of whom had PH = study group) and 21 healthy children. These 21 patients and the remaining 10 without PH at cardiac catheterization made up the control group. All children were studied with Doppler Echocardiography to evaluate the pulmonary flow pattern with the sample volume placed in the pulmonary artery trunk, 1 cm distal to the pulmonic valve. The preejection period (PEP), ejection period (EP), acceleration time (AcT), the indexes PEP/EP, PEP/AcT, AcT/EP and the morphologic pattern of the pulmonary flow (type I: with peak flow velocity at midsystole; type II: with peak flow velocity in early systole; type III: with midsystolic notching) were analysed and quantitative parameters corrected according to hear rate by dividing theirs value by the square root of R-R interval. In the hemodynamic study we analysed the systolic (SPAP), diastolic and mean pulmonary artery pressure, and the mean pulmonary pressure/mean systemic pressure ratio (Pp/Sp). We compared the echocardiographic variables in both, study and control groups, and analysed the hemodynamic and echocardiographic correlation between the variables in question. RESULTS: Pattern I of pulmonary flow was associated with absence of PH and pattern II and III with PH (p < 0.001). The best results of quantitative variables were either corrected AcT (AccT) rather less in the study group than in control group (2.89 +/- 0.56 vs 4.05 +/- 0.56 ms, p < 0.001) and PPE/AcT index, 1.28 +/- 0.3 in the hypertensive group and 0.78 +/- 0.16 in the control group (p < 0.001). The best correlation were AcT with SPAP (r = -0.82) and Act with Pp/Sp ratio (r = -0.84). CONCLUSIONS: We consider that pulmonary flow analysed with Doppler echocardiography is a reliable, suitable and non-invasive method to evaluate PH in children.

[An aneurysm in a balloon-catheter-dilated Blalock-Taussig fistula]. / Aneurisma en fístula de Blalock-Taussig dilatada con catéter-balón.

The development of aneurysm at the Blalock-Taussig shunt, dilated two years before is described. It is rounded with a diameter of 19 mm. and located at the junction of the right subclavian artery and the right pulmonary artery, that is, at the site of previous balloon dilatation. We think that the pathogenesis is the same as that of the aortic aneurysms which develop after the balloon catheter dilation at the same level. The actual prognosis is unknown being an undescribed pathology.

[Balloon percutaneous angioplasty in postoperative aortic coarctation]. / Angioplastia percutánea con balón en la coartación de aorta posquirúrgica.

BACKGROUND: Percutaneous transluminal balloon angioplasty has been documented as a useful procedure in the treatment of recurrent coarctation of the aorta. In this project we present our experience and results. PATIENTS AND METHODS: Percutaneous transluminal balloon angioplasty was performed in 7 children with postsurgical coarctation of the aorta. Six of them had previous coarctation of the aorta and one had a iatrogenic result of a persistent ductus arteriosus operation. RESULTS: Mean pressure difference across the coarctation decreased from mean = 51 +/- 16 mmHg to mean = 14 +/- 6 mmHg after the angioplasty and mean diameter of the aortic lumen at the coarctation site increased from mean = 3.6 +/- 1.4 to mean = 8.7 +/- 1.2 mm. All patients are asymptomatic and normotensive at least during the following two years after the procedure. Only the patient who had iatrogenic coarctation still has a residual gradient of 30 mmHg. There were not any major complications during the procedure or after it. CONCLUSIONS: Percutaneous transluminal balloon angioplasty is a safe and effective procedure in the treatment of postsurgical coarctation of the aorta.

[Transluminal percutaneous pulmonary valvuloplasty. Follow up of 34 patients]. / Valvuloplastia transluminal percutánea pulmonar. Seguimiento en 34 pacientes.

Thirty four children aged 2 to 11 years (means = 4.8 +/- 2.4 years) with pulmonary valve stenosis were treated by percutaneous transluminal valvuloplasty with catheter-balloon. All of them were reevaluated one month and six months after valvuloplasty, and once more year after the procedure. The evaluation consisted on clinical examination, chest Rx, ECG and Doppler 2 D-Echocardiography. We found a close correlation between transpulmonic gradients by Doppler and manometry. After pulmonary valvuloplasty we detected 12 cases of slight pulmonary insufficiency. Changes in ECG were significant since signs of right heart hypertrophy disappeared. Immediate good results got after valvuloplasty, remained and even improved throughout checking. We conclude that, faced with our results, we consider valvuloplasty as a definitive treatment of pulmonary stenosis, Doppler-two-Dimensional Echocardiography, is also useful for evaluating patients without need for a new catheterisation and EECG is an ideal complement to 2 D-Doppler Echocardiography.

[Percutaneous transluminal pulmonary valvuloplasty. Results in 34 cases]. / Valvuloplastia transluminal percutánea pulmonar. Resultados en 34 pacientes.

Percutaneous transluminal valvuloplasty with catheter-balloon were performed in thirty-four patients with pulmonary valve stenosis, aged 2 to 11 years (mean = 4.8 +/- 2.4 years). It was a slight stenosis in 24 cases (70.5%) and severe in 10 cases (29.5%). In three of them it was a dysplastic valve. We achieved good results, since right ventricle systolic pressure, decreased from mean = 67 +/- 18 mmHg to mean = 23 +/- 12 mmHg (p less than 0.0001). In the three cases with dysplastic valve, results were rather poor. Procedure was well stood-up and there were no serious complication. We conclude that at the present time valvuloplasty its the elective treatment in pulmonary stenosis in childhood except in cases with dysplastic valve.

[Balloon angioplasty of stenosed Blalock-Taussig shunts. Study of 4 cases]. / Angioplastia con balón de fístulas de Blalock-Taussig estenosadas. Presentación de 4 casos.

Four patients with complex cyanotic congenital heart disease, who had previously undergone Blalock-Taussig operations, developed severe stenoses of the shunt. All were treated with balloon dilatation. This procedure was successfully in three of them.

[Severe aortic stenosis in the neonate and infant under 6 months old]. / Estenosis aórtica severa en el recién nacido y lactante menor de seis meses.

24 patients with diagnosed aortic stenosis are reported. Clinical symptoms started before 6 months of age (between 24 hours and 6 months, average 87 days). In all cases aortic stenosis was the only existing malformation, except in one patient who had a small muscular ventricular septal defect and in another case who had a patent ductus arteriosus. Clinical symptoms were early and notable. In nineteen there was congestive heart failure whose appearance in the first week of like was a bad prognosis. Relationship between severity of aortic stenosis and electrocardiographic findings was closer than in older patients. Death rate was very high in children who had not been operated (5 out of 12). Of children who received surgery only one died out of 12. Treatment, therefore, in this pathology must be aggressive.

El síndrome de Halasz en el niño / Halasz syndrome in the child

El síndrome de Halasz es una malformación poco frecuente y con características bien definidas. Aportamos el estudio realizado en tres niños, menores de tres años, con estudio clínico, radiográfico, isotópico y hemodinámico (cateterismo y angiocardiografía) y se revisan los casos existentes en la bibliografía de pacientes menores de 15 años; se perfilan los síntomas y signos que conducen al diagnóstico y a la orientación terapéutica (AU)