Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) is a complex and diverse form of congenital heart defect. Although most patients with ...PA/VSD/MAPCAs can wait until they are 3 to 6 months of age to undergo surgical reconstruction, there are three specific criteria that merit an earlier repair. These 3 criteria are (1) unremitting heart failure; (2) a ductus to one lung and MAPCAs to the other; and (3) hemitruncus to one lung and MAPCAs to the other. The purpose of this study was to evaluate our surgical experience with early complete repair of PA/VSD/MAPCAs.
This was a retrospective review of patients undergoing complete repair of PA/VSD/MAPCAs within the first 60 days of life. Twenty-seven patients were identified in our database (2002 to 2013) who met these criteria. Fifteen had congestive heart failure, 9 had a ductus plus MAPCAs, and 3 had hemitruncus plus MAPCAs. The median age at surgery was 5 weeks.
There was no operative mortality in this cohort of 27 patients. Hemodynamics at the conclusion of the complete repair demonstrated an average right ventricular peak systolic pressure of 32 ± 5 mm Hg and an average right ventricle to aortic pressure ratio of 0.36 ± 0.06. The median length of hospital stay was 26 days. There have been 2 subsequent mortalities (7%), with a median follow-up duration of 4 years. Eight of the 27 patients have subsequently undergone conduit replacements at our institution. The hemodynamics at the conclusion of the conduit change were statistically unchanged compared with the hemodynamics after complete repair.
The data demonstrate that early complete repair of PA/VSD/MAPCAs can be accomplished with low mortality and excellent postoperative hemodynamics. These early hemodynamic results are maintained at medium-term follow-up. We conclude that early complete repair is an appropriate choice for this highly select subgroup of patients.
The purpose of this study is to determine the outcome and performance of bovine pericardial valves in the pulmonary position.
This is a retrospective review of all patients with congenital heart ...disease who had pulmonary valve replacement using a bovine pericardial valve from 2002 to 2009 at a single institution.
There were 73 consecutive patients, with a median age of 17.3 years (range, 2.1 to 64.4). Their diagnosis was tetralogy of Fallot (n = 47), pulmonary stenosis (n = 11), or other (n = 15). Sixty-nine patients had 91 previous surgical procedures. The mean time from last surgery was 19.9 ± 11.6 years. Forty-three patients had concomitant surgical procedures. There were no perioperative deaths. Clinical follow-up was available in 68 patients (93%). There were no late deaths, and all patients were in New York Heart Association functional class I during a median follow-up period of 2.6 years (range, 0.2 to 8.0). One patient had endocarditis necessitating valve removal 2 years after surgery. Freedom from pulmonary valve reoperation was 100%, 97.7%, and 97.7% at 1, 3, and 5 years, respectively (95% confidence interval: 93.2% to 100%). Mean pulmonary valve gradient at follow-up was 19 ± 14 mm Hg. Degree of pulmonary insufficiency was less than moderate in 62 patients, moderate in 4, and more than moderate in 2. Freedom from moderate-severe or severe pulmonary insufficiency was 97.7%, 89.1%, and 89.1% at 1, 3, and 5 years, respectively (5-year 95% confidence interval: 77.0% to 100%).
Pulmonary valve replacement using a bovine pericardial valve can be accomplished with low perioperative morbidity and favorable midterm outcomes. Further follow-up is necessary to evaluate the long-term performance of bovine pericardial valves in the pulmonary position.
For many decades, patients with d-transposition of the great arteries underwent an atrial switch procedure. Although many of these patients have continued to do well, a subset experience profound ...right ventricular failure. Some may be candidates for left ventricular (LV) retraining and late arterial switch. The purpose of this study was to review our experience with LV retraining and late arterial switch.
This was a retrospective review of 32 patients with d-transposition. Thirty patients underwent a previous atrial switch and subsequently experienced right ventricular failure, whereas 2 presented late (8 months and 6 years) without previous intervention. The median age at the time of enrollment in this program was 15 years. Seven patients proceeded directly to late arterial switch owing to systemic LV pressures. The remaining 25 underwent a pulmonary artery band for LV retraining.
Twenty of the 32 (63%) patients enrolled in this program were able to undergo a late arterial switch. There were 2 operative mortalities (10%). Two additional patients survived surgery but died in the early outpatient time period. There has been no late mortality after the arterial switch with a median follow-up of 5 years. Twelve patients underwent one or more pulmonary artery band procedures without evidence of effective LV retraining. There have been 2 early and 3 late (42%) deaths in this subgroup.
The outcomes after arterial switch are encouraging and suggest that LV retraining and late arterial switch provide a viable option for this complex group of patients.
Objective The optimal shunt size for patients who have the Norwood operation with a right ventricle-to-pulmonary artery conduit is controversial. The goal of this study is to compare outcomes of 2 ...shunt sizes in this population. Methods Between 2002 and 2010, 75 consecutive patients diagnosed with hypoplastic left heart syndrome and its variants underwent the Norwood procedure with a right ventricle-to-pulmonary artery conduit. The outcomes of 20 neonates weighing <3 kg were analyzed. Results The cumulative 30-day stage 1 mortality was 97% (95% confidence interval, 88%-99%) for all patients (73/75), 20 of whom weighed <3 kg. Nine patients had a 6-mm (group 1) and 11 patients had a 5-mm (group 2) right ventricle–to-pulmonary artery conduit. Thirty-day stage 1 survival was 88% (8/9) in group 1 and 90% (10/11) in group 2 ( P = .88). The central pulmonary artery confluence size at prebidirectional cavopulmonary shunt catheterization was 4.5 ± 1.2 mm in group 1 and 2.5 ± 1.0 mm in group 2 ( P = .009). The mean transpulmonary gradient was higher in group 2 (7.8 ± 3.1 mm Hg vs 4.2 ± 1.9 mm Hg; P = .036). The incidence of pulmonary artery intervention was 16% (1/7) in group 1 and 75% (6/8) in group 2 ( P = .030). Survival rate at 6 months was 66% (6/9) in group 1 and 70% (7/10) in group 2 ( P = .89). Conclusions Use of a 6-mm right ventricle-to-pulmonary artery conduit showed better central pulmonary artery growth and less need for pulmonary artery intervention in the authors’ experience.
A modification of the Fontan operation was recently applied, which includes anastomoses of the extracardiac conduit to the right pulmonary artery and inferior vena cava using simple clamping with no ...additional circulatory support, venous shunting, pulmonary artery preparation, or prior maintenance of azygos vein patency. The objective of this study is to assess the outcomes of this novel off-pump "clamp and sew" Fontan procedure.
This is a retrospective review of all patients having a Fontan procedure between January 2009 and October 2010 at a single institution.
Twelve patients had a Fontan procedure with the use of cardiopulmonary bypass (CPB group), and 12 had an off-pump Fontan procedure (off-pump group). Preoperative demographic and hemodynamic data were similar except for higher mean pulmonary artery pressure in the CPB group (12.2±1.6 mm Hg versus 9.9±2.4 mm Hg; p=0.02). No patients in the off-pump group required conversion to CPB. The mean inferior vena cava clamp time in the off-pump group patients was 10±3 minutes. There were no early or midterm deaths. No patients exhibited postoperative hepatic or renal dysfunction. Postoperative maximal serum creatinine and aspartate transaminase were significantly lower in the off-pump group compared with the CPB group (0.59±0.12 versus 0.77±0.22 mg/dL; p=0.03 and 35.5±8.3 versus 53.1±19.0 U/L; p=0.02, respectively). At median follow-up of 13 months (range, 1 to 20 months), all but 1 patient in the CPB group are in New York Heart Association class I with unobstructed Fontan circulation.
The clamp and sew technique for completion of an extracardiac conduit Fontan procedure appears safe and feasible for selected patients.
The Norwood procedure with the right ventricle–to–pulmonary artery (RV-PA) conduit has been the standard procedure for hypoplastic left heart syndrome. However, postoperative management can be ...challenging related to finding the correct balance between pulmonary and systemic blood flow. One can use hemostatic clips on the RV-PA conduit to temporarily restrict pulmonary blood flow. However, the hemodynamics would become unstable after clip removal. Here we describe a novel technique that allows precise pulmonary blood flow without the addition and removal of clips on the RV-PA conduit, allowing primary chest closure after the Norwood procedure.
Median sternotomy has been the standard surgical approach for pediatric cardiac surgery. The downside is that it leaves a notable and visible scar. Right axillary mini-thoracotomy provides a more ...aesthetically pleasing incision in addition to an earlier recovery time for patients. However, this contemporary thoracotomy provides a smaller and deeper view of the heart that can make the procedure more difficult. This report describes the modified techniques used to provide an excellent surgical exposure through the right axillary mini-thoracotomy, which also allows patients to have an “invisible” incision.
We describe an extremely rare case of a 15-year-old female, who underwent a complete repair for transitional atrioventricular canal with Scimitar syndrome by a two-patch technique including ...translocation of the Scimitar vein. This surgical technique offered superior patch shapes in order to better repair both anomalies.
Over the last few decades, due to evolving surgical techniques and medical management, there has been a significant decrease in the rate of mortality and complications for neonates born with critical ...pulmonary valve stenosis. Median sternotomy is the standard approach; however, this longitudinal midline incision is invasive and leaves a significant scar. A right mini thoracotomy approach to this surgical repair decreases recovery time and the chance of possible future psychological distress from a visible median sternotomy scar. This is the first article to describe a right mini thoracotomy approach for critical pulmonary stenosis during the neonatal period.
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