Hybrid Approach to Right Ventricle Decompression in Muscular Pulmonary Atresia with Intact Ventricular Septum.

Muscular pulmonary atresia with intact ventricular septum (PA/IVS) in neonates is traditionally managed by surgery. We describe hybrid approach to decompress the right ventricle (RV) and establish RV to pulmonary artery connection in a neonate avoiding cardiopulmonary bypass. A 21-gauge access needle was used to perforate the atretic pulmonary valve via periventricular approach followed by stent placement. This case is an example of how patients with congenital heart disease can be palliated in creative ways through thoughtful collaboration between surgical and interventional cardiology teams.

Single ventricle palliation: greater risk of complications with the Fontan procedure than with the bidirectional Glenn procedure alone.

BACKGROUND: This study was performed to evaluate and compare the early, intermediate, and long-term outcomes of the bidirectional Glenn procedure and Fontan procedure in patients who live at moderately high altitude. METHODS: The outcome of each method of palliation for patients with a functionally single ventricle was retrospectively evaluated from a review of medical records. RESULTS: The bidirectional Glenn procedure was performed in 177 patients from October 1984 to June 2004. The Fontan procedure was performed in 149 patients from June 1978 to June 2004. Cardiovascular death or heart transplantation occurred in 8% of patients after the bidirectional Glenn procedure and 17% of patients after the Fontan procedure. Complications of systemic thromboembolic events, bleeding associated with anticoagulation therapy, protein losing enteropathy, and arrhythmias requiring implantation of a pacemaker, cardioversion, or radiofrequency ablation occurred in 7% of patients after the bidirectional Glenn procedure and 47% of patients after the Fontan procedure. Cardiovascular deaths and heart transplantation occurred less frequently when the Fontan procedure was performed in patients with a previous bidirectional Glenn procedure. However, the actuarial transplant-free survival and freedom from complications was not superior for a subgroup of patients who had a Fontan procedure after a bidirectional Glenn procedure in comparison to a subgroup of patients who had a bidirectional Glenn procedure alone. CONCLUSIONS: The bidirectional Glenn procedure can be used for long-term palliation of patients with a functionally single ventricle. Additional palliation with a Fontan procedure may increase the risk of stroke, protein losing enteropathy and arrhythmias without improving survival.

Biventricular Repair of Pulmonary Atresia After Fontan Palliation.

Fontan palliation is used when biventricular repair (BVR) is not possible. Early outcomes are acceptable; however, the long-term sequelae include protein-losing enteropathy, declining functional status, increased pulmonary vascular resistance, heart failure, and hepatic and renal dysfunction. These adverse events are characteristic of persistent venous hypertension and may be avoided if restoring biventricular circulation is possible. Arrhythmias are a common adverse event, particularly in patients with an atriopulmonary connection, which may lead to acute decompensation and early death. We describe a 30-year-old woman who underwent successful BVR for pulmonary atresia with intact ventricular septum and demonstrate that where favorable anatomy exists with a failing Fontan, BVR should be considered.

Immunogenicity of decellularized cryopreserved allografts in pediatric cardiac surgery: comparison with standard cryopreserved allografts.

BACKGROUND: Recognition of the immunogenicity of standard cryopreserved allografts has led to the development of new decellularized allografts (CryoValve SG; CryoLife, Inc, Kennesaw, Ga). This preliminary study examined the HLA antibody response to these decellularized allografts and compared it with the response to standard allograft material. METHODS: We prospectively measured the frequency of panel-reactive HLA class I (HLA-A, HLA-B, and HLA-C) and class II (HLA-DR/DQ) alloantibodies in 14 children (age 8.5 +/- 7.9 years) receiving decellularized, cryopreserved allografts, including 6 undergoing allograft patch insertion and 8 with a valved pulmonary allograft. We compared them with 20 historical control subjects (age 1.7 +/- 2.4 years) undergoing implantation of standard cryopreserved allografts, 8 with valves and 12 with allograft patch. All patients had panel-reactive antibody levels measured before and at 1, 3, and 12 months after the operation. HLA class I and class II panel-reactive antibody levels were determined with a sensitive flow cytometry technique. RESULTS: We found panel-reactive antibody levels in decellularized allografts to be elevated slightly from preoperative levels for both class I and class II antibodies at 1, 3, and 12 months (P >.05). The panel-reactive antibody level for both class I and class II antibodies were significantly lower for decellularized allografts as compared to standard allografts. Functionally, the allografts were similar with decellularized valved grafts showing a peak echo-determined systolic gradient of 13 +/- 15 mm Hg at 8 +/- 2.6 months postoperatively as compared to a gradient of 24 +/- 18 mm Hg measured 12 +/- 6 months postoperatively in standard allografts (P =.11). CONCLUSIONS: Decellularized grafts elicited significantly lower levels of class I and class II HLA antibody formation at 1, 3, and 12 months after implantation than did standard cryopreserved allografts. Early hemodynamic function of decellularized grafts was similar to that of standard cryopreserved allograft valves. Further experience is necessary to determine whether the reduced immunogenicity of decellularized allografts will truly allow tissue ingrowth and improved long-term durability in patients.

Current treatment of pediatric empyema.

Pneumonia with complicated parapneumonic effusion and empyema is increasing in incidence and continues to be a source of morbidity in children seen in our institution. Current diagnostic modalities include chest radiographs and CT scanning with ultrasound being helpful in some situations. Exact management of empyema remains controversial. Although open thoracotomy drainage is well accepted in children, video-assisted thoracoscopic surgery (VATS) drainage has become more prevalent in the current era. Over the last 4 years, we have treated 58 children with intrapleural placement of pigtail catheters and administration fibrinolytics consisting of tissue plasminogen activator (tPA). Successful drainage and resolution of 54 of the 58 effusions was achieved with percutaneous methods alone. There was no mortality or 30-day recurrence. Mean hospital stay was 9.1 days (range 5 to 21) and mean chest catheter removal was 6 days post placement (range 1.5 to 20). Of the four patients that failed percutaneous tube therapy, 3 underwent video assisted thoracic surgery (VATS), and one had open thoracotomy with decortication. Based on our experience, tPA administered through a small bore chest tube for drainage of complicated parapneumonic effusions has become our standard practice. We reserve VATS for treatment failures and open thoracotomy and decortication for patients with VATS failure.

The Ross operation in children: effects of aortic annuloplasty.

BACKGROUND: Autograft dilatation and progressive neoaortic regurgitation after the Ross procedure prompted us to perform routine aortic annuloplasty. The purpose of this review is to evaluate the success of this technical modification in preventing autograft failure requiring reoperation. METHODS: From 1994 to 2005, 46 children and young adults with a mean age of 12.9 +/- 4.9 years (range, 14 months to 21 years) underwent a Ross procedure; 19 of 46 patients had prior aortic valve surgery. Neoaortic valve function and need for reintervention were compared between patients who had a Ross procedure without annuloplasty (n = 20) and those who had an annular reduction prior to the autograft anastomosis (n = 26). RESULTS: There were no early or late deaths during a mean follow-up of 65 +/- 36 months. Mean hospital stay was 6.6 +/- 2.9 days. Two patients required early intervention (eight days) for significant neoaortic regurgitation; one patient required repair of a left ventricular outflow tract pseudoaneurysm a month after emergent Ross procedure for endocarditis, and one patient required replacement of a stenotic homograft at five years. Five patients (13%) required autograft repair (n = 3) or replacement (n = 2) for progressive neoaortic regurgitation, two of the 26 patients had reduction annuloplasty (8%), and three of the 20 patients did not (15%) (p = 0.6). There was a similar incidence of neo-sinus of Valsalva dilatation 37 mm or greater in patients with (53%) and without (36%) annuloplasty (p = 0.5). CONCLUSIONS: The Ross procedure remains an excellent option for valve replacement in children and young adults given the alternatives and can be performed with very low mortality. However, in this series of Ross operations in children, routine use of aortic annuloplasty failed to prevent neoaortic regurgitation requiring reoperation.

Current status of surgery for rheumatic carditis in children.

BACKGROUND: The incidence of rheumatic heart disease (RHD) has increased recently in the western United States. We reviewed our 18-year surgical experience with RHD in children to examine current surgical techniques and results. METHODS: From 1985 until 2003, 596 children (<21 years) with rheumatic fever were seen at Primary Children's Medical Center. Rheumatic carditis was diagnosed in 366 patients (61.4%). Twenty-six with carditis (26/366, 7.1%) required operation for rheumatic valve disease including 8 for mitral regurgitation, 7 for mitral and aortic regurgitation, 4 for aortic regurgitation, 4 for mitral regurgitation and stenosis, 2 for combined mitral stenosis and regurgitation with aortic insufficiency, and 1 for mitral and tricuspid regurgitation. RESULTS: Mean age at operation was 13.5 +/- 4 years. Three patients required operation during the acute phase of rheumatic fever (< 6 weeks), 2 during the subacute phase (< 6 months), and 21 during the chronic phase after the episode of rheumatic fever (6.7 +/- 3 years). Mitral valve repair was possible in 19 of 22 patients who required mitral operation. Aortic valve repair was possible in 4 patients whereas replacement was necessary in 9, including 2 Ross procedures. No operative deaths were recorded and 2 late deaths occurred at 4.6 and 10 years. Actuarial survival was 94% at 5 years and 78% at 10 years. Six patients required reoperation; actuarial freedom from reoperation was 78% at 5 years, 65% at 10 years, and 49% at 15 years. All survivors are in New York Heart Association class I or II. CONCLUSIONS: Children with RHD in the United States uncommonly require valve operation. Mitral repair with a technique that allows annular growth is possible in most children with good long-term functional results. Long-term surveillance of children with RHD is necessary because of the possible need for late valve operation.