Brief reports

Usefulness of laser-assisted valvotomy with balloon valvoplasty for pulmonary valve atresia with intact ventricular septum

Los neonatos afectos de atresia pulmonar con tabique interventricular íntegro y estenosis pulmonar crítica representan un espectro amplio, incluyendo aquellos con hipoplasia significativa del ventrículo derecho. La presencia de fístulas arteriales coronarias a ventrículo derecho puede ser una contraindicación para la descompresión del ventrículo derecho. El principal objetivo del presente trabajo es analizar los resultados a corto y largo plazo durante 20 años de estos pacientes, e identificar los factores diferenciales entre ambos grupos incluyendo aquellos pacientes afectos por fístulas arteriales coronarias.

Estudio retrospectivo donde se identificaron todos los pacientes diagnosticados de atresia pulmonar con septo interventricular íntegro y estenosis pulmonar crítica entre los meses de enero de 1996 y enero de 2018. Se recogieron y analizaron las características morfológicas del ventrículo derecho, el manejo quirúrgico, la intervención percutánea y la evolución a corto y a largo plazo.

Fueron incluidos cincuenta y un pacientes. Un total de 9 (17,6%) fallecieron durante el seguimiento. Ninguno de ellos presentaba fístulas arteriales coronarias a ventrículo derecho. La mediana de seguimiento de los restantes 42 supervivientes fue de 8,9 años (rango: 1-16).

La clase funcional según la New York Heart Association en la revisión más reciente fue de 1,2. Los supervivientes del grupo de estenosis pulmonar crítica presentaban una clase funcional de 1,1 y los del grupo de atresia pulmonar con tabique interventricular íntegro de 1,6. No hubo diferencias entre los pacientes que presentaban fístulas arteriales coronarias a ventrículo derecho y los que no.

La presencia de fístulas arteriales coronarias a ventrículo derecho no es una contraindicación para la vía biventricular. Los pacientes con estenosis pulmonar crítica presentan una mejor evolución que los afectos de atresia pulmonar con tabique interventricular íntegro. La estrategia de apertura agresiva y precoz de la válvula pulmonar tiene una buena supervivencia global correlacionada con una buena clase funcional.

Pulmonary atresia with intact ventricular septum and critical pulmonary stenosis in newborns encompasses a wide spectrum of disease, including cases with significant right ventricular hypoplasia and coronary artery to right ventricle fistulae, which may be considered a contraindication for decompression of the right ventricle. The aim of this study was to review the middle- and long-term outcomes of these patients over 20 years and identify differential factors between both groups, including patients with coronary artery fistulae.

We performed a descriptive retrospective study by identifying all patients that received a diagnosis of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis between January 1996 and January 2018. We collected and analysed data regarding right ventricular morphology, surgical management, percutaneous intervention and medium- and long-term outcomes.

51 patients were admitted. A total of 9 patients (17.6%) died during the followup. None of the deceased patients had coronary artery to right ventricle fistulae. The median length of follow up in the 42 survivors was 8.9 years (1-16).

The functional class based on the latest revision of the New York Heart Association classification was 1.2 for the overall sample. Survivors of critical pulmonary stenosis had a functional class of 1.1, and survivors of pulmonary atresia with intact ventricular septum a functional class of 1.6. There were no differences based on the presence or absence of coronary artery to right ventricle fistulae.

Coronary artery to right ventricle fistulae may not be a contraindication for biventricular strategy. Patients with critical pulmonary stenosis had better outcomes compared to patients with pulmonary atresia with intact ventricular septum. The aggressive strategy of opening the pulmonary valve early on was associated with a good overall survival and correlated to a good functional class.

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.

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.

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.

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.

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.

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.

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.

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.

Outcomes for pulmonary atresia with intact ventricular septum are suboptimal, while initial management remains controversial. This study was undertaken to determine the effect of catheter-based therapy on the need for early surgical intervention.

A single-institution retrospective chart review was made of all 25 neonates with pulmonary atresia with intact ventricular septum from 1999 to 2005.

Mean age at first intervention was 3.1 ± 2.2 days, mean weight 3.3 ± 0.5 kg. Right ventricular hypoplasia varied: 20% normal, 16% mild, 28% moderate, 28% moderately severe or severe, 8% not classified. Median tricuspid valve z-score was −2.3 ± 2.6. First intervention was catheter-based therapy in 56% (14 of 25), operative in 36% (9 of 25), and no therapy in 2. Technically adequate valvotomy was achieved in 79% (11 of 14). Serious catheter-related complications occurred in 3 of 14 (21%). Only 5 of 14 (36%) with catheter-based therapy weaned from prostaglandins without surgery. Of these, 2 required surgery for cyanosis at 1 and 3 months. Surgery after catheter-based therapy consisted of right ventricular outflow patch in 36% (4 of 11), systemic to pulmonary shunt in 64% (7 of 11). Median time between catheter-based therapy and surgery was 8.5 days (range, 1 to 89). Only 3 of the 23 treated patients avoided operation during infancy. There was 1 early and 1 late death after operation after initial catheter-based therapy, and 1 late death after primary surgery alone during a mean follow-up of 33 months (range, 1.5 to 79).

Balloon valvotomy alone for pulmonary atresia with intact ventricular septum rarely obviates the need for an additional source of pulmonary blood flow—either shunt or ductal stenting.

Survival of patients with PAIVS has improved significantly with adoption of a selective management approach based on RV and coronary arterial anatomy. We sought to determine the right (RV) and left ventricular (LV) function of patients with pulmonary atresia and intact ventricular septum (PAIVS) long-term after biventricular repair.

The regional ventricular function of 22 patients (11 males) aged 12.8 ± 5.6 years with PAIVS after biventricular repair was assessed by tissue Doppler echocardiography (TDE). The results were compared to those of 22 age-matched controls.

When compared with controls, patients had significantly reduced myocardial tissue velocities, systolic and diastolic strain rate, and peak systolic strain in the basal, mid-, and apical segments of the RV free wall, interventricular septum and LV lateral wall (all with p < 0.01). Heterogeneity of systolic and diastolic strain rate was observed in the RV free wall and interventricular septum in patients but not in controls.

Impairment of RV and LV regional longitudinal myocardial function occurs in patients with PAIVS late after biventricular repair.