[Pulmonary atresia with intact ventricular septum. Perforation and pulmonary valvuloplasty using a modified mechanical technique. Medium-term follow-up]. / Atresia pulmonar con septo íntegro. Perforación y valvuloplastia pulmonar mediante técnica mecánica modificada. Seguimiento a medio plazo.

INTRODUCTION AND OBJECTIVES: In patients with pulmonary atresia with intact ventricular septum (PAIVS), radiofrequency-assisted perforation of the valve is the most widely used initial therapy when the anatomy is favorable. We report our experience with a modified mechanical technique that gave good results. METHODS: Between November 2001 and October 2006, valve opening was carried out successfully in 11 consecutive neonates with a favorable anatomy (i.e., Alwi groups A and B, and tricuspid valve Z-score -1.1 [1.3]). The technique involved snare-assisted anterograde or retrograde perforation with the soft tip of a special guidewire used for chronic total coronary artery occlusions, use of an arteriovenous loop, and progressive balloon dilatation from a diameter of 2 mm to a maximum diameter of 9.6 [1.2] mm. RESULTS: Valve opening was achieved in all patients, and right ventricular (RV) systolic pressure fell from 97 [17] mmHg to 48 [13] mmHg (P< .001). No pericardial effusion or cardiac tamponade was observed, though one neonate died 24 hours after the procedure due to pulmonary embolism. Six patients (54%) were discharged without any further intervention, while 4 (36%) required an additional increase in pulmonary blood flow. During the follow-up period of 25 [21] months, two patients died. Eight (72%) survived and were in New York Heart Association functional class 1. Two required additional surgery on the outflow tract, one of whom also needed a one-and-a-half ventricular repair. Data indicate that the valves remain open as RV structures grow, though without any change in the tricuspid valve Z-score. CONCLUSIONS: Pulmonary valvuloplasty using a mechanical technique proved effective in patients with PAIVS. Modification of the standard mechanical technique by using the soft tip of a special guidewire used for chronic total coronary artery occlusions was less aggressive and improved results. In patients with a favorable anatomy, results were comparable to those obtained using the radiofrequency technique.

Atresia pulmonar con septo íntegro. Perforación y valvuloplastia pulmonar mediante técnica mecánica modificada. Seguimiento a medio plazo / Pulmonary atresia with intact ventricular septum. Perforation and pulmonary valvuloplasty using a modified mechanical technique. Medium-term follow-up

Introducción y objetivos. En la atresia pulmonar con septo íntegro, si la anatomía es favorable, el tratamiento inicial más extendido es la apertura valvular mediante radiofrecuencia. Presentamos nuestra experiencia mediante una técnica mecánica modificada con buenos resultados. Métodos. Entre noviembre de 2001 y octubre de 2006 realizamos apertura valvular en 11 casos consecutivos con anatomía favorable (grupo A y B de Alwi e índice z tricúspide de ­1,1 ± 1,3), mediante perforación anterógrada guiada por lazo o retrógrada mediante guías especiales para la obstrucción crónica total coronaria por su parte blanda y circuito arteriovenoso y dilatación progresiva con balón monorraíl coronario desde 2 mm de diámetro hasta un máximo de 9,6 ± 1,2 mm. Resultados. Se realizó la apertura valvular en todos los casos con reducción de la presión sistólica del ventrículo derecho de 97 ± 17 a 48 ± 13 mmHg (p < 0,001). No se observaron derrame ni taponamiento en ningún caso, y un neonato falleció a las 24 h por un tromboembolismo pulmonar. Seis casos (54%) fueron dados de alta sin otro procedimiento, y 4 (36%) precisaron un flujo pulmonar adicional. El seguimiento fue de 25 ± 21 meses. Dos pacientes fallecieron, mientras que 8 (72%) sobrevivieron y se encontraban en situación funcional I. Dos precisaron cirugía adicional del tracto de salida y en uno de ellos, además, del ventrículo y medio. Persisten datos de desobstrucción valvular con crecimiento de estructuras en el ventrículo derecho, aunque sin cambios en el índice z valvular. Conclusiones. La valvuloplastia pulmonar con técnica mecánica sigue siendo válida en la atresia pulmonar con septo íntegro. La modificación de la técnica mecánica clásica mediante guías especiales para la obstrucción crónica total coronaria por su parte blanda es menos agresiva y mejora sus resultados. En esta serie con anatomía favorable los resultados son superponibles a los obtenidos mediante radiofrecuencia (AU)

Introduction and objectives. In patients with pulmonary atresia with intact ventricular septum (PAIVS), radiofrequency-assisted perforation of the valve is the most widely used initial therapy when the anatomy is favorable. We report our experience with a modified mechanical technique that gave good results. Methods. Between November 2001 and October 2006, valve opening was carried out successfully in 11 consecutive neonates with a favorable anatomy (i.e., Alwi groups A and B, and tricuspid valve Z-score ­1.1 [1.3]). The technique involved snare-assisted anterograde or retrograde perforation with the soft tip of a special guidewire used for chronic total coronary artery occlusions, use of an arteriovenous loop, and progressive balloon dilatation from a diameter of 2 mm to a maximum diameter of 9.6 [1.2] mm. Results. Valve opening was achieved in all patients, and right ventricular (RV) systolic pressure fell from 97 [17] mmHg to 48 [13] mmHg (P<.001). No pericardial effusion or cardiac tamponade was observed, though one neonate died 24 hours after the procedure due to pulmonary embolism. Six patients (54%) were discharged without any further intervention, while 4 (36%) required an additional increase in pulmonary blood flow. During the follow-up period of 25 [21] months, two patients died. Eight (72%) survived and were in New York Heart Association functional class 1. Two required additional surgery on the outflow tract, one of whom also needed a one-and-a-half ventricular repair. Data indicate that the valves remain open as RV structures grow, though without any change in the tricuspid valve Z-score. Conclusions. Pulmonary valvuloplasty using a mechanical technique proved effective in patients with PAIVS. Modification of the standard mechanical technique by using the soft tip of a special guidewire used for chronic total coronary artery occlusions was less aggressive and improved results. In patients with a favorable anatomy, results were comparable to those obtained using the radiofrequency technique (AU)

Isolated pseudohypoplasia of the right ventricle.

We present a case of a 12-year-old girl with severe cyanosis due to abnormal moderator band producing a hidden trabecular component of the right ventricle, mimicking isolated hypoplasia of the right ventricle. A marked hypoplasia was confirmed by echocardiography and catheterization. At operation an anomalously thickened moderator band obstructing the apical infundibulum was found. Repair consisted of a section of the moderator band and closure of the atrial septal defect. Postoperatively the right ventricle showed normal dimension and function.