En Bloc Rotation of the Outflow Tracts - Double Root Translocation.

En bloc rotation of the outflow tracts or double root translocation offers an anatomic repair of transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction and closely related forms of double outlet right ventricle. The technical principle is to excise aortic and pulmonary root en bloc, rotate them as a whole by 180°, and reimplant them. The left ventricular outflow tract is enlarged with the patch closing the ventricular septal defect. In our experience, two thirds of the pulmonary valves could be preserved. Growth of the aortic and pulmonary root could be demonstrated in several studies performed by our group. It is still a complex and technically demanding procedure with long cardiopulmonary bypass periods and cross-clamp times. However, perioperative mortality and complications do not differ significantly from other forms of reconstruction. The reoperation rate is significantly lower. Presently, the best time to perform this operation seems to be after the newborn period within the first year of life.

Descending aortic translocation for left aortic arch with right descending aorta and coarctation of the aorta.

Left aortic arch with right descending aorta associated with coarctation of the aorta is a rare congenital cardiac anomaly. Conventional aortic arch repair in this condition may cause airway compression by the abnormally coursing descending aorta. We present the case of a neonate with this anomaly who underwent successful descending aortic translocation to prevent postoperative left main bronchial stenosis.

Descending aortic translocation procedure for relief of bronchial compression in left aortic arch and right descending aorta.

Compression of the left bronchus by a right-sided descending aorta arising from a left aortic arch constitutes an exceedingly rare form of vascular ring. This manuscript describes the surgical technique that involves translocation of the descending aorta directly to the proximal ascending aorta, thereby relieving the bronchial compression.

Left aortic arch with right descending aorta and severe coarctation: an unusual "vascular clamp" with airway compression.

Left aortic arch with right descending aorta is a rare congenital anomaly. We describe the clinical presentation of this unusual anomaly associated with cardiorespiratory compromise from severe aortic obstruction and left main bronchus compression. The anatomical peculiarities, embryological basis, and surgical solutions are presented.

Long-term results of anatomic correction for congenitally corrected transposition of the great arteries: A 19-year experience.

OBJECTIVE: The surgical indication, timing, strategy, and surgical technique for anatomic correction of congenitally corrected transposition of the great arteries are challenging. We evaluated the long-term results at The Royal Children's Hospital Melbourne. METHODS: Review of 32 successive anatomic corrections between 1996 and 2015. RESULTS: Twenty-one double-switch (66%), 6 Senning/Bex-Nikaidoh (19%), and 5 Senning/Rastelli (16%) procedures were performed (median age, 1.9 years). Median follow-up was 5.4 years with 4 deaths and 1 heart transplant. Cumulative incidence of late reoperation was 8%, 29%, and 59% at 1, 5, and 10 years, respectively. Twenty-six patients had full follow-up with native hearts. Nineteen had normal left ventricle (LV) function. Late LV dysfunction, mostly mild, was not related to needing a pacemaker (P = .4) or a pulmonary artery band (PAB) (P = .08). Previous PAB was linked to the need for aortic valve surgery or neoaortic regurgitation moderate or greater (P = .03). Six required Senning revision. The introduction of the Shumacker modification of the Senning has generated stable pulmonary venous pathways. Six patients developed postoperative iatrogenic atrioventricular block dependent on a permanent pacemaker. CONCLUSIONS: Anatomic correction is a surgical challenge. It provides excellent functional outcomes in survivors with a significant need for reoperation and a definite risk of death or transplantation. Normal LV function should be expected in most patients. LV dysfunction was not linked to PAB or pacemaker requirement but surgery without LV training had better long-term LV function. The Shumacker modification provided stable venous pathways. Iatrogenic atrioventricular block remains a challenge.

Cirugía de Nikaidoh modificada: experiencia inicial y resultados a corto y a mediano plazos / The Modified Nikaidoh Procedure: Initial Experience and Short and Mid-term Outcomes

Introducción: El tratamiento convencional de los pacientes con transposición de los grandes vasos con comunicación interventricular y estenosis pulmonar es la cirugía de Rastelli. Sin embargo, sus resultados son subóptimos, razón por la cual se han propuesto otras alternativas quirúrgicas. El procedimiento de Nikaidoh es una técnica reciente que surge como una opción terapéutica atractiva. Objetivo: Analizar nuestra experiencia inicial con la cirugía de Nikaidoh modificada: translocación aórtica y reconstrucción de ambos tractos de salida ventriculares. Material y métodos: Se analizaron 11 pacientes consecutivos operados con técnica de Nikaidoh modificada en nuestra institución en el período 2005-2014, con edad mediana de 3 años (intervalo intercuartil 25-75%: 1-5,5 años) y peso medio de 13,8 ± 5,4 kg, 9 de ellos con transposición de los grandes vasos con comunicación interventricular y estenosis pulmonar y 2 con doble salida del ventrículo derecho con estenosis pulmonar y fisiología de transposición. Todos tenían comunicación interventricular no relacionada con la aorta y un paciente tenía además ventrículo derecho hipoplásico. Resultados: En el posquirúrgico inmediato no hubo mortalidad, un paciente desarrolló endocarditis infecciosa y requirió reoperación. Seis pacientes tuvieron disfunción ventricular transitoria; solo uno necesitó asistencia ventricular. No se registró obstrucción a los tractos de salida ventriculares ni insuficiencia aórtica mayor que leve. En el seguimiento a mediano plazo, con una media de seguimiento de 5,5 ± 2,1 años (rango: 2-8,6 años), no hubo mortalidad ni reoperaciones. Se efectuó un cateterismo intervencionista para dilatación del conducto ventrículo derecho-arteria pulmonar. Todos los pacientes permanecían en clase funcional I, con buena función biventricular, sin arritmias, sin obstrucción al tracto de salida ventricular izquierdo y sin insuficiencia aórtica significativa. Conclusiones: La cirugía de Nikaidoh y sus modificaciones constituyen una buena opción quirúrgica para los pacientes con transposición de los grandes vasos con comunicación interventricular y estenosis pulmonar, que por su morfología son malos candidatos para cirugía de Rastelli. En esta serie no hubo mortalidad y todos los pacientes se encuentran en clase funcional I. En el seguimiento a mediano plazo, el tracto de salida del ventrículo izquierdo permaneció sin obstrucción y no se registró insuficiencia aórtica significativa.

Background: The Rastelli operation is the conventional procedure for transposition of the great vessels with ventricular septal defect and pulmonary stenosis. Its results, however, are suboptimal and other surgical alternatives have been postulated. A recent surgical technique, the Nikaidoh procedure, appears as a new therapeutic option. Objective: The aim of this study was to review our initial experience using the modified Nikaidoh procedure consisting in aortic translocation and biventricular outflow tract reconstruction. Methods: The study analyzed 11 consecutive patients operated on with the modified Nikaidoh procedure at our institution between 2005 and 2014. Median age was 3 years (25-75% interquartile range: 1-5.5 years) and mean weight was 13.8 ± 5.4 kg. Nine patients had transposition of the great vessels with ventricular septal defect and pulmonary stenosis and 2 had double outlet right ventricle with pulmonary stenosis and transposition physiology. All patients had a ventricular septal defect unrelated to the aorta and one patient also had a hypoplastic right ventricle. Results: There were no deaths in the immediate postoperative period. One patient developed infective endocarditis needing reoperation. Six patients had some degree of transient left ventricular dysfunction, and one patient only needed ventricular assistance. There was no involved right or left ventricular outflow tract obstruction and only mild aortic regurgitation. At mid-term follow-up (mean of 5.5 ± 2.1 years, range: 2-8.6 years) there were no deaths or reoperations. An interventional cardiac catheterization to dilate the right ventricle to pulmonary artery conduit was performed in one patient. All patients remained in functional class I, with good biventricular function, free from arrhythmias, no left ventricular outflow tract obstruction and no significant aortic regurgitation. Conclusions: The Nikaidoh operation and its variants are a satisfactory surgical option for patients presenting transposition of the great vessels with ventricular septal defect and pulmonary stenosis, who due to their morphology are inadequate candidates for a Rastelli procedure. In the mid-term follow-up, the left ventricular outflow tract remained without obstruction and there was no significant aortic valve regurgitation.