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Aortopulmonary window is rarely associated with other cardiac anomalies, such as anomalous origin of one coronary artery. This exceptional association has been reported in 15 patients, with different surgical solutions. We describe 2 patients with aortopulmonary window with anomalous origin of the right coronary artery, one of which presented with tetralogy of Fallot-pulmonary atresia. A neonatal modified Blalock-Taussig shunt and ligature of the distal pulmonary artery at the level of pulmonary bifurcation was performed in the first patient, and the second patient underwent aortopulmonary window division with anomalous coronary artery reimplantation. Both patients had an uneventful postoperative course., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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.

A newborn, with SDS (S = situs viscero-atrialis solitus, D = D-loop of the ventricles, S = solitus, normally related great arteries) anomalous drainage of the right superior vena cava in the left atrium, intact atrial septum, and anomalous drainage of the right superior pulmonary veins in the right superior vena cava, underwent surgical repair at our institution. This rare cyanotic, congenital, cardiac malformation is herein described with particular regard to its anatomical, embryological, and surgical implications.

Atrioventricular valve duplication, also known as double orifice valve, is an excitingly rare anomaly that can involve either the mitral or more uncommonly the tricuspid valve. Herein we describe a case of a double orifice tricuspid valve that prejudiced the complete repair in a symptomatic infant with tetralogy of Fallot.

Background: Genetic syndromes occur in 20% of patients with tetralogy of Fallot (TOF). The impact of genetic syndromes on surgical repair of TOF in infancy is still under investigation., Methods: This retrospective study reviews the outcome of 306 consecutive patients (median age, 5.1 months) who underwent primary (266) or staged (40) repair of TOF between 1994 and 2004. Total follow-up was 1,188 patient-years (mean, 57 months)., Results: Genetic syndromes were documented in 85 patients (27.8%), including 22q11 deletion (27), trisomy 21 (13), vertebral, anal, cardiac, tracheoesophageal, renal, and limb abnormalities (VACTERL, 12), and others (33). Hypoplastic pulmonary arteries (PA) were more common in syndromic (19/85 = 22.3%) than nonsyndromic TOF (20/221 = 9.04%) (p < 0.001). Primary repair was performed in 82.4% syndromic and 88.7% nonsyndromic TOF (p = not significant [NS]). Ten-year actuarial survival was 94.1 +/- 2.3% in nonsyndromic and 84.3 +/-4.2% in syndromic TOF (p < 0.001). Ten-year survival was 96.3 +/- 3.6% for del22q11, 100% for trisomy 21, 63.6 +/- 14.5% for VACTERL, and 78.5 +/- 7.3% for patients with other syndromes (p = 0.022). Survival in syndromic TOF with normal PA anatomy was 89.6 +/- 4.2% for primary repair and 85.7 +/- 12.8% for staged repair (p = NS); freedom from reoperation after complete repair was 74.4 +/- 6.4% for primary correction and 56.3 +/- 11.9% for staged repair (p = 0.04). Cox proportional hazard identified the presence of genetic syndrome (p = 0.011) and central PA hypoplasia (p = 0.002) as independent predictors of mortality., Conclusions: Pulmonary arborization defects and genetic syndromes other than del22q11 or trisomy 21, are associated with worse outcome after correction of TOF. Primary TOF repair in syndromic patients with normal PA anatomy is a valid surgical strategy, with no additional risk for mortality and higher freedom from reintervention.

Aneurysm development after repair of aortic coarctation is not a rare complication, but it is highly related to hypoplasia of the transverse aortic arch. The optimal management of such cases is not clearly established. We propose a single-stage transmediastinal re-repair using moderate hypothermic cardiopulmonary bypass and antegrade selective cerebral perfusion.

Background: Several studies have shown that Down's syndrome is not a risk factor for biventricular repair of complete atrioventricular septal defects. However, few data are available about the comprehensive outcome of all the cardiac surgical procedures in patients with trisomy 21, including palliative surgery., Methods: This is a retrospective study of 206 consecutive patients who underwent cardiac surgery from January 1992 to January 2002. Data about mortality and morbidity were analyzed and the impact of Down's syndrome was evaluated., Results: Overall mortality was 7.7%. Actuarial survival was 94% among patients with Down's syndrome versus 86% of the group with normal karyotype (p = 0.12). The presence of unbalanced ventricles was the only independent risk factor affecting survival at multivariate analysis (p < 0.0001). The need for a Norwood type surgery was more frequent among non-Down patients (12.0% vs 1.5%, p = 0.02) as was the prevalence of pulmonary artery banding operations (22.9% vs 9.3%, p = 0.04). Cumulative mortality after palliation was higher in non-Down patients (44% vs 2.9%, p = 0.0001). Freedom from reoperation was lower in the group with normal chromosomes in respect to patients with Down's syndrome (81.4% vs 94.6%, p = 0.04), due to the higher prevalence of anomalies of the mitral valve (4.9% vs 1.8%, p = 0.03) or left ventricular outflow tract (7.3% vs 0%, p = 0.01)., Conclusions: Down patients showed a decreased risk for biventricular repair and lower mortality and morbidity in cases of complex cardiac malformations requiring complex palliative operations.

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

Aortic coarctation and cutaneous hemangioma is a rare association. We describe the case of a neonate with abnormal looping of the aortic arch associated with hemangioma of the head and neck who underwent complex surgical repair without cardiopulmonary bypass.

The midterm follow-up of 2 patients with left ventricle-to-aorta conduit who underwent percutaneous closure with the Amplatzer device is described. Complete occlusion was achieved immediately in 1 patient. In the other patient, a trivial residual shunt was still present after 12 months, but disappeared after 18 months. Occlusion of dysfunctional left ventricle-to-aorta conduits by the Amplatzer device is feasible, provided that enough time is allowed for complete occlusion.

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.