hypoplasia of pulmonary annulus, main pulmonary artery and its branches in a 42-year-old female patient. (Ao: aorta; MPA: main pulmonary artery; RVOT: right ventricular outflow tract.)

occluded systematically to avoid volume overload during the postoperative period. Myocardial protection of these markedly hypertrophied hearts must be extremely careful. Closing coronary fistulas before aortic crossclamping could be a useful maneuver to avoid losing cardioplegia.

When the pulmonary annulus is hypoplastic or infundibular resection is not possible through a right atriotomy, a ventriculotomy in the RVOT must be performed. This approach was necessary in almost all our patients, unlike what happened in other series, in which the use of the atrial approach reached 41% of the cases [12]. When annulus hypoplasia is present, the ventriculotomy is extended to the main pulmonary artery, transecting the annulus. This procedure was performed in 25% of our patients. If this is not necessary, the incision is limited to the RVOT. Section and resection of the hypertrophic muscle bands releases obstruction. Care must be taken not to weaken the right ventricular free wall. For VSD closure, the use of separate pledgeted stitches is recommended to prevent damage of the friable tissue of this region. The ventriculotomy confined to the RVOT is usually closed with glutaraldehyde treated pericardium or Dacron®. The latter is rigid and reduces the incidence of RVOT aneurysm. In contrast, untreated pericardium significantly increases the incidence of such aneurysms [13]. The transannular patch can be complemented with a monocuspid valve made of pericardium or thin expanded polytetrafluoroethylene. This monocusp is sutured to the inner surface of the patch or to the internal edges of the ventriculotomy. This could avoid severe pulmonary regurgitation, protecting the hypertrophic ventricle from volume overload of sudden installation in the immediate postoperative period.

Despite the severe clinical condition of these patients, the complex surgical correction and the high mortality rate associated to this procedure, many studies have demonstrated the benefits of the repair of TF in adulthood. The long-term survival has been shown to be higher compared to patients treated only with medication [12,14]. Similarly, NYHA FC and quality of life improve substantially after surgery [15].

5. Conclusion

Surgical correction of TF in adulthood is a procedure rarely performed. The short-term results depend mostly on the anatomic substrate and the preoperative clinical condition of each patient. Coronary fistulas and hypoplastic pulmonary artery branches have a statistically significant negative impact on mortality. Severe insaturation is also a predictor of augmented surgical risk. Mortality remains high in this subset of patients. Therefore, identifying good candidates for surgical repair becomes extremely important. Young acyanotic patients showing favorable anatomy and acceptable preoperative condition can be safely treated. On the other hand, older cyanotic patients with severe impairment and coronary fistulas or hypoplastic pulmonary artery branches have a substantially higher risk of early postoperative death. Such cases must be carefully analyzed before an aggressive surgical approach is chosen.


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