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      Balloon valvuloplasty as an initial palliation in the treatment of newborns and young infants with severely symptomatic tetralogy of Fallot.

      Radiology
      Male, Catheterization, Female, Follow-Up Studies, Humans, Infant, Infant Welfare, Infant, Newborn, Postoperative Complications, etiology, Pulmonary Artery, surgery, Pulmonary Valve, Severity of Illness Index, Tetralogy of Fallot, therapy, Time, Treatment Outcome

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          Abstract

          Balloon valvuloplasty in infants with symptomatic tetralogy of Fallot (TOF) may increase the pulmonary flow and prompt the growth of pulmonary arteries. From 1994 to 2002, percutaneous transluminal balloon valvuloplasty (PTPV) was performed in 22 consecutive newborns and young infants (<3 months of age) with TOF. The indication included severe hypoxemia (systemic oxygen saturation below 75%, 10 cases) and repeated hypoxic spells (12 cases). The age at PTPV was 8-88 days (38 +/- 34, median 27) and the body weight 2-5 kg (3.45 +/- 1.15, median 3). A balloon catheter (4- 7 mm in diameter and 2 cm in length) was used to dilate the pulmonary valve. No major procedure-related complications occurred. The systemic oxygen saturation increased significantly (14 +/- 9%). A subsequent palliative shunt operation was avoided in 12 patients (54.5%), but 10 still needed operation (median 11 days after the PTPV). The presence of recurrent hypoxic spells before PTPV was the most important indicator for PTPV failure (p = 0.02). PTPV was safe and effective for symptomatic newborns and young infants with TOF, but a palliative shunt operation was still needed due to short effect, especially in those with recurrent spells before the dilation. Copyright 2006 S. Karger AG, Basel.

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          The modified Blalock-Taussig shunt: clinical impact and morbidity in Fallot's tetralogy in the current era.

          The Blalock-Taussig shunt is considered a low-risk management option for palliation in tetralogy of Fallot, but the morbidity associated with a Blalock-Taussig shunt can have a significant impact on patient care. We reviewed the outcome for this operation in the current era. Between 1990 and 1994, 65 children with tetralogy of Fallot received a modified Blalock-Taussig shunt. Sixty patients who had follow-up angiography were assessed for clinical outcomes and shunt-related morbidity and mortality. From the same study period, 68 of 247 pediatric patients who underwent angiography and tetralogy repair, but did not receive palliation, were randomly selected to comprise a comparison group. Palliation was more likely in the presence of a complicated tetralogy malformation or if there was an associated medical condition. Median age at palliation was 58 days (range: 1 to 535 days). Ninety-five percent of shunts were right-sided. Self-limited morbidity complicated 11% of shunt operations. Significantly smaller distal right pulmonary arteries were observed in the palliated group before total repair compared with findings in the group without palliation and 33% of patients who underwent palliation had angiographic evidence of pulmonary artery distortion. Shunt stenosis was common and correlated with younger age at palliation. Shunt occlusion resulted in one death. Excluding noncardiac causes of death, overall survival was 90% in the palliated group versus 97% in the nonpalliated group (p = 0.09). Pulmonary artery hypoplasia and angiographic evidence of pulmonary artery distortion are common after initial palliation by a modified Blalock-Taussig shunt. Neonatal palliation was associated with significantly smaller pulmonary arteries before repair, which necessitated additional interventions.
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            Tetralogy of Fallot: surgical management individualized to the patient.

            Over the past four decades, the surgical trend has been toward early, complete repair of tetralogy of Fallot (TOF). Many centers currently promote all neonates for total correction irrespective of anatomy and symptoms, with some surgeons advocating hypothermic circulatory arrest for repair in small infants. We believe this approach increases morbidity. Based on approximately 40 years' experience in 2,175 patients, we developed a management protocol focused on patient size, systemic arterial saturations, and anatomy. Symptomatic patients (hypercyanotic spells, ductal dependent pulmonary circulation) weighing less than 4 kg undergo palliative modified Blalock-Taussig shunt (BTS) followed by complete repair at 6 to 12 months. Asymptomatic patients, weighing less than 4 kg who have threatened pulmonary artery isolation, undergo BTS and repair at 6 to 12 months. All other patients undergo complete repair after 6 months. From July 1, 1995, to December 1, 1999, 144 patients underwent operation for TOF (129 patients) or TOF with atrioventricular septal defect (TOF/AVSD, 15 patients). Ninety-four patients underwent one stage complete repair (88 TOF, 6 TOF/AVSD). Thirty-nine patients underwent repair after initial BTS (32 TOF, 7 TOF/AVSD). Ten patients are awaiting repair after BTS. The mean age and weight at complete repair were 18 months and 9 kg. There were no operative deaths. There have been 3 late deaths with complete follow-up (mortality 3 of 144 [2.1%]). Four of 133 patients (3%) have required reoperation after total correction. This management strategy optimizes outcomes by individualizing the operation to the patient. Advantages include avoidance of circulatory arrest, low morbidity and mortality, and low incidence of reoperation after complete repair.
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              Early and late results and the effects on pulmonary arteries of balloon dilatation of the right ventricular outflow tract in tetralogy of Fallot.

              Balloon dilatation of the pulmonary valve remains controversial as a palliative procedure in tetralogy of Fallot. To determine the clinical outcome, growth of the pulmonary vascular tree and findings at surgery of balloon dilatation of the pulmonary valve performed as a palliation in infants with tetralogy of Fallot. Thirty-three severely cyanosed infants (mean age of 3 months for the whole population, including seven neonates) underwent this procedure from June 1990 to January 1997. After balloon dilatation, systemic oxygen saturation increased from a mean value of 76 +/- 9% to 88 +/- 7% (P < 0.001). The procedure was accomplished without complications. Four patients had recurrent hypoxic spells after dilatation leading to surgical repair within 30 days of dilatation (three modified Blalock-Taussig shunts and one complete repair). A control study was performed at a mean of 6.1 +/- 4.5 months after dilatation in 16 patients to establish growth of the pulmonary vascular tree (repeat catheterization in nine patients and surgical pulmonary annulus calibration in seven). The pulmonary annulus increased from a mean Z score of -4.1 +/- 0.9 SD to -2.5 +/- 1.1 SD (P < 0.001). Z score for the right pulmonary artery from -3.0 +/- 0.6 SD to -1.9 +/- 1.2 SD (P = 0.007) and the Z score for the left pulmonary artery from -2.7 +/- 0.7 SD to -1.6 +/- 1.3 SD (P = 0.021). At late follow-up (mean of 9 months after dilatation, range 3 to 54 months), 28 patients underwent complete repair. Trans-annular patching was required in 43% of patients. Four post-operative deaths were observed, none dilatation related. Balloon dilatation of the pulmonary valve is an effective and safe palliation in tetralogy of Fallot. It promotes growth of the pulmonary vascular tree, reducing the need for trans-annular patching and is recommended in symptomatic infants of very young age, with a small pulmonary annulus (Z value below -4 SD) and associated cardiac anomaly.
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