Search

Background: Although deep hypothermic circulatory arrest has been extensively used in neonates for aortic arch surgery, the brain and other organs might be adversely affected by prolonged ischemia and deep hypothermia., Methods: Between December 1997 and January 2005, 70 consecutive neonates underwent Norwood stage I procedure for hypoplastic left heart syndrome (group A, n = 30), or aortic arch repair for interruption or coarctation with arch hypoplasia (group B, n = 40), with antegrade selective cerebral perfusion (ASCP). Mean weights were 3.0 +/- 0.2 kg and 2.8 +/- 0.07 kg, and mean ages were 10 +/- 3.5 days and 14 +/- 10.6 days in groups A and B, respectively. Only 2 patients were older than 30 days. Core body temperature was lowered to 25 degrees C, and mean pump flow during ASCP was initiated at 10 to 20 mL/(kg x min) and adjusted to guarantee a radial/temporal artery pressure of 30 to 40 mm Hg and venous oxygen saturation of more than 70%. Hematocrit was maintained at 30%., Results: Early mortality was 17% (group A, 23%; group B, 12.5%; p = 0.19). Six late deaths occurred (3 in each group), and at 36 months, Kaplan-Meier overall survival was 64% +/- 9.2% in group A and 85% +/- 5.7% in group B. One patient had postoperative seizures. Age, weight, sex, prematurity, group A, and ASCP duration did not influence early mortality., Conclusions: Antegrade selective cerebral perfusion is a safe and effective procedure and might improve outcome of neonatal aortic arch surgery, minimizing neurologic impact without the need for deep hypothermia.

Background: Junctional ectopic tachycardia (JET) is a life-threatening arrhythmia producing severe hemodynamic dysfunction, which may complicate the postoperative course of surgery for congenital heart disease. Strict care and a fast and effective antiarrhythmic strategy are essential, because mortality largely depends on the duration of the arrhythmia., Methods: Seven consecutive neonates with postoperative JET without any evidence of myocardial ischemia received intravenous flecainide after conventional therapies proved ineffective. Atrial pacing at the minimal rate for atrioventricular synchrony was followed by a 10-min intravenous infusion of 1.6 mg/kg flecainide, then continuous infusion of 0.4 mg/kg flecainide per hour. Treatment was considered effective based on restoration of sinus rhythm or a JET rate no higher than 170 bpm within 4 hours of flecainide loading. Overall mean flecainide infusion lasted 31.2 hours (range 25 to 53 hours). Side effects were assessed by monitoring plasma flecainide levels, electrocardiogram, arterial pressure, and central venous pressure., Results: Flecainide was effective in all 7 patients after an infusion duration of 3.6 +/- 1.5 hours. Sinus rhythm was restored after 7.2 +/- 9.7 hours. After 4 hours of loading, heart rate fell from 219 +/- 14 to 136 +/- 7 bpm (p < 0.0001), arterial pressure increased from 69 +/- 8 to 93 +/- 10 mm Hg (p < 0.0001), while central venous pressure decreased from 8.0 +/- 1.6 to 5.2 +/- 1.9 mm Hg (p = 0.0007). No side effect or recurrence was noted., Conclusions: Flecainide can exert a fast antiarrhythmic effect on postoperative JET, and its infusion can be modulated to maintain the concentration within the therapeutic range, thus avoiding toxicity. We propose further consideration of flecainide for treatment of JET in neonates without myocardial ischemia.

A very rare case of double outlet right ventricle with intact ventricular septum and unrestricted pulmonary flow was successfully palliated with pulmonary banding and delayed bidirectional cavopulmonary anastomosis and mitral avulsion. This is the only case of pulmonary banding with mitral avulsion reported in the literature for this type of heart defect

The problems related to the pediatric pulmonary homograft availability and the possible transmission of viral infection led us to design a new patch for aortic enlargement in the Norwood procedure for hypoplastic left heart syndrome. This sterile bovine pericardial patch is not expensive and can be tailor-made.

Aortic arch interruption associated with an aortopulmonary window is a rare congenital malformation that needs an early diagnosis and surgical treatment to avoid irreversible pulmonary lesions. Here we describe a case of a successful one-stage surgical repair in a 3-day-old neonate, without the use of prosthetic material, for the correction of the aortic arch interruption.

Hypoplasia of the transverse aortic arch is frequently associated with isthmic coarctation in many patients referred for operation in early infancy, and the surgical technique should be adjusted to suit each type of anatomic lesion. Referring to the anatomic description of hypoplastic aortic arch reported by Moulaert and associates, between January 1988 and July 1991 we operated on 32 consecutive infants (< or = 3 months old) using a surgical approach based on the echocardiographic and angiographic findings; 20 patients (62%) were younger than 2 weeks of age and 20 patients (62%) had associated intracardiac lesions. According to the location, extension, and size of the hypoplasia of the aortic arch, we had three groups of patients: in group 1 (21 patients) we performed resection and extended end-to-end anastomosis, as previously described in 1985; in group 2 (5 patients) we performed resection, posterior end-to-end anastomosis, and anterior subclavian flap enlargement; and in group 3 (6 patients) we performed direct side-to-end anastomosis between ascending and descending aorta through a median sternotomy. One patient died during the postoperative course in group 3. With a mean follow-up time of 26 months we had 4 cases (13%) of "residual" or "recurrent" coarctation in group 1, successfully repaired at 2 months of age by an anterior approach in 2 patients and by percutaneous angioplasty in the others. In conclusion, hypoplastic aortic arch in neonates represents a common difficulty, and optimal reconstruction of the entire aortic arch is mandatory to reduce operative mortality and incidence of recoarctation, especially when there are complex associated intracardiac lesions or left ventricular dysfunction.

The diffuse form of supravalvar aortic stenosis represents a surgical challenge when ascending aorta and proximal aortic arch are involved. We describe a technique performed on a 14-year-old patient with normal aortic annulus and severe diffuse supravalvar aortic stenosis in which the replacement of ascending aorta and proximal aortic arch with a tubular prosthetic graft completely relieved the obstruction as confirmed by cardiac catheterization.