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Pulmonary hypertension associated with pediatric congenital heart defects is a major cause of postoperative morbidity and death. Sildenafil has been combined with inhaled nitric oxide to treat pulmonary hypertension. We retrospectively studied the pre- and postoperative effects of oral sildenafil as monotherapy in children with pulmonary hypertension who underwent surgery to correct congenital cardiac defects. From September 2005 through November 2009, 38 children with moderate-to-severe pulmonary arterial hypertension (pulmonary arterial/aortic pressure ratio, >0.7) underwent cardiac surgery at our institution. Fifteen patients were given sildenafil (0.35 mg/kg, every 4 hr) orally or through nasogastric tubes 1 week before and 1 week after surgery. Twenty-three patients of comparable medical status were given sildenafil only upon the institution of cardiopulmonary bypass and for 1 week after surgery. Postoperatively, the 15 patients who were given preoperative sildenafil had significantly lower mean pulmonary arterial pressures (25.6 ± 3.1 vs. 30.4 ± 5.7 mmHg; P = 0.005) and pulmonary arterial/aortic pressure ratios (0.35 ± 0.05 vs. 0.42 ± 0.07; P = 0.002) than did the other 23 patients. The preoperative therapy also shortened cardiopulmonary bypass time, mechanical ventilation time, and lengths of intensive care unit and hospital stays. No sildenafil-related hypertensive crises or sequelae occurred. As monotherapy, oral sildenafil in low doses appears to control pulmonary hypertension safely and effectively in children undergoing operations to correct congenital heart defects, particularly when it is given both preoperatively and postoperatively. Further study is warranted.

During the past 10 years, minimally invasive surgical techniques have been applied more and more widely in both adult and pediatric populations, especially in female patients. Right anterolateral minithoracotomy is an alternative to median sternotomy that yields a better cosmetic outcome. From May 1997 through September 2008, 132 patients (107 females, 25 males) underwent open-heart surgery through right anterolateral minithoracotomy. Ages ranged from 1 to 49 years (mean, 10.12 yr). Mean body weight was 21.3 kg (range, 9.4-78 kg). Corrected defects included atrial septal defect, partial anomalous pulmonary venous connection, partial atrioventricular canal defect, ventricular septal defect alone or with pulmonary valve stenosis, tetralogy of Fallot, cor triatriatum, mitral valve defect, and pericardial cyst. The anterolateral skin incision was 5 to 7 cm. Our control group--which in the same period underwent the same interventional procedures through median sternotomy--consisted of 415 patients, 245 of whom were female (59%). There was no early or late death or major illness as a sequela. No patient required conversion to full sternotomy. All patients had gratifying cosmetic results at longer follow-up. Indeed the mortality and morbidity rates obtained through our approach were almost the same as those obtained through median sternotomy, and there were no significant differences in cardiopulmonary bypass time, aortic cross-clamp time, ventilation time, or postoperative hospital stay. We conclude that the right anterolateral minithoracotomy for correction of congenital heart defects offers superior cosmetic results without increasing morbidity or mortality rates and confers upon patients psychological and social satisfaction.

Background: Although surgical shunt is still considered as the best palliation of congenital heart disease with reduced pulmonary blood flow, stent implantation may be technically simpler, safer and more cost-effective than surgery in high-risk patients. This study evaluated the feasibility and results of this option in patients with duct-dependent pulmonary blood flow or systemic-to-pulmonary shunt malfunction., Methods: Between April 2003 and July 2004, 9 patients (age 11 days-52 years, weight 2.1-52 kg) with complex congenital heart disease underwent stent implantation inside the patent ductus arteriosus (4 patients) or a stenotic surgical shunt (5 patients)., Results: The stenting procedure was successfully completed in all cases. The procedural time was 162 +/- 36 min (range 90-225 min). The fluoroscopy time was 33.8 +/- 6.8 min. No patient died. The morbidity rate was 22.2% (1 patient had local infection at the site of puncture and 1 had transient femoral artery pulse loss). After the procedure, the ductus/shunt diameter increased from 1.2 +/- 0.6 to 3.6 +/- 0.6 mm (p < 0.0001) and oxygen saturation improved from 74.0 +/- 6.5 to 85.2 +/- 3.3% (p < 0.01). Three patients underwent corrective surgery without technical problems after 8.0 +/- 1.0 months while oxygen saturation remained constantly >80% in patients still waiting for surgical repair (follow-up 5.3 +/- 3.1 months)., Conclusions: Stent implantation is a technically feasible, safe and effective palliative option in high-risk surgical patients with congenital heart disease and reduced pulmonary blood flow. Although larger series are required to define the cost-effective clinical impact of this therapeutic option, it is reasonable to hypothesize a further extension of its indication even to elective and low-risk surgical patients.

A 3-month-old infant with tetralogy of Fallot presented with progressive severe cyanosis and intractable acidosis about 2 months after a successful modified right-sided Blalock-Taussig shunt. At cardiac catheterization, the suspected shunt malfunction was confirmed. It was due to a bulky, pear-like mass arising from the right subclavian artery and compressing the polytetrafluoroethylene conduit. Any attempt to recanalize the shunt by percutaneous techniques proved unsuccessful. At surgery, a huge dilation of the anterior wall of the right subclavian artery, that sharply bent the prosthetic conduit, was found. Pathologic examination revealed that the compressing mass was pseudo-aneurysmal in nature. Despite a second successful shunt operation with a dramatic clinical improvement, the patient died due to multiorgan failure 72 hours following surgery. Extrinsic compression by a false aneurysm is a rare cause of shunt occlusion that should always be suspected in patients presenting with a rapidly progressive shunt malfunction late after a successful shunt procedure.