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We present our experience for patients who have undergone an anatomic repair (AR) for congenitally corrected transposition of the great arteries (CCTGA) at the Children's Hospital of Wisconsin. A retrospective chart review of patients who underwent AR for CCTGA from 2001 to 2015 was performed. The cohort consisted of 15 patients (74% male). Median age of anatomic repair was 15 months (range 4.5-45.6 months). Four patients had a bidirectional Glenn (BDG) prior to AR. At the time of AR,-9 (60%) underwent Senning/Rastelli procedure, 4 (26.6%) had double switch operation, and 2 (13.3%) underwent only Senning with VSD closure. Median duration of follow-up was 5.5 years (0.05-14 years). Reoperations prior to discharge included BDG, revision of pulmonary venous baffle, closure of residual VSD, and pacemaker placement. Late reoperations included left ventricular outflow tract obstruction repair, conduit replacement, melody valve placement, and pacemaker implantation. At their most recent follow-up, no patient had heart failure symptoms and only 1 had severely diminished function that improved with cardiac resynchronization therapy. Moderate mitral regurgitation was noted in 15% (2/13), and severe in 7% (1/13). Moderate tricuspid regurgitation was noted in 15% (2/13). One patient, 7% (1/13), developed moderate aortic insufficiency. There was a 100% survival at the time of the most recent follow-up. Patients with CCTGA who have undergone AR have excellent functional status and mid-term survival but reinterventions are common. Longer term studies are needed to determine both the extent and spectrum of reinterventions as well as long term survival.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart lesion that if left untreated, results in high mortality. Surgical treatment options include reimplantation of the anomalous coronary ostia, use of extracardiac arterial blood supply, or the Takeuchi procedure. The Takeuchi procedure involves creation of an aortopulmonary window and an intrapulmonary tunnel that baffles the aorta to the ostium of the anomalous left coronary artery. The incidence of late complications following the Takeuchi repair is unknown. The goal of our study was to determine the long-term outcome after palliation of ALCAPA using the Takeuchi Procedure. A total of 9 patients with a history of ALCAPA palliated with the Takeuchi procedure were identified from our surgical database. Chart review was performed. The mean age at time of Takeuchi procedure was 49.6 months (range 5 weeks-14.6 years). There was one late death, of unknown cause. Of the remaining 8 patients, the mean length of follow-up after surgery was 15.9 years (13.5-19.7 years). All 8 survivors had some degree of main pulmonary artery stenosis in the area of the intrapulmonary baffle, with moderate stenosis in 2 and severe stenosis in 1. Three late survivors (38 %) had a baffle leak. Two patients (25 %) had decreased left ventricular systolic function and 3 (38 %) had developed at least moderate mitral valve regurgitation. Three of the 8 late survivors (38 %) required a reoperation for repair of mitral valve regurgitation, baffle leak, and main pulmonary artery (MPA) stenosis. Review of literature demonstrated similar complication rates and need for reoperation following the Takeuchi procedure. Compared with patients after direct reimplantation for ALCAPA at our institution, there was no significant difference in late survival or freedom from reoperation. The Takeuchi procedure is a method to establish a two-coronary repair for ALCAPA. Late complications are common, necessitating lifelong care in a center experienced with caring for adults with congenital heart disease.

Background: Neonates with hypoplastic left heart syndrome have circulatory vulnerability that results in shock and high risk of mortality without intervention. High arterial saturation (SaO(2)) is often used as a proxy for inadequate systemic oxygen delivery and triggers the use of invasive therapies to restore circulatory balance. We hypothesized that preoperative use of near-infrared spectroscopy (NIRS) would reduce the need for invasive therapies, including controlled ventilation and inspired gas manipulation., Methods: A Human Research Review Board-approved retrospective review of patients who had stage 1 palliation from January 2000 to January 2006 was conducted. Preoperative patient characteristics, cardiorespiratory support, and monitored data were collected for all patients. Cerebral and somatic tissue oxyhemoglobin saturations were recorded for patients with preoperative NIRS monitoring., Results: The studied cohort included 92 patients, 47 without and 45 with preoperative NIRS. Patient characteristics were similar between groups. Differences were observed in preoperative respiratory support. Controlled ventilation was less common in the NIRS group (51% versus 79%, p = 0.005) as was the use of inspired nitrogen (16% versus 70%, p = 0.001). The NIRS patients had higher mean SaO(2) (92% versus 88%, p = 0.001). Age at surgery was similar between groups (5.7 +/- 3.2 versus 6.5 +/- 5.2 days, p = 0.3). Early survival was 96% in each group., Conclusions: Near-infrared spectroscopy monitoring of patients with hypoplastic left heart syndrome awaiting palliation provides noninvasive assessment of oxygen delivery and simplified management, with reduced use of controlled ventilation and inspired gas. Higher SaO(2) in the NIRS group was not associated with impaired systemic oxygen delivery, and did not lead to earlier palliation or postoperative mortality.

Severe tricuspid regurgitation resulting from a flail leaflet is a rare cause of neonatal cyanosis. We report two neonates with profound cyanosis and severe tricuspid regurgitation caused by rupture of the papillary muscle supporting the anterior leaflet, without other structural heart defects. Ductal patency could not be established. Repair of the tricuspid valve was performed by reimplantation of the ruptured papillary muscle head, after initial stabilization using extracorporeal membrane oxygenation. Early recognition and treatment of this otherwise fatal condition can be lifesaving.

Background: A comprehensive assessment of 48 hour postoperative hemodynamics in neonates randomized to the right ventricle-to-pulmonary artery (RV-PA) conduit or modified Blalock-Taussig (BT) shunt for stage 1 palliation of hypoplastic left heart syndrome was performed to determine the potential benefits of the modified technique., Methods: Randomization to either RV-PA conduit or BT shunt was stratified by surgeon and the presence of aortic atresia. The designated procedure was performed by using hypothermic cardiopulmonary bypass with phenoxybenzamine, continuous cerebral perfusion, pH-stat blood gas management, and continuous postoperative venous oximetry. Differences between treatments were analyzed by time-series generalized least-squares regression, chi2 tests, two-way repeated measures analysis of variance, and the Levene variance ratio test for variability in parameters, as appropriate., Results: All patients underwent the procedure to which they were randomized. There were no differences in age, weight, deep hypothermic circulatory arrest, or cardiopulmonary bypass times between patients receiving the BT shunt (n = 8) or the RV-PA conduit (n = 9). There was one early and one late death in the RV-PA conduit group, and one interstage death in the BT shunt group. Other than diastolic blood pressure (39 mm Hg in BT shunt versus 46 mm Hg in RV-PA conduit, p < 0.001), there were no differences in the mean values of arterial saturation, venous oximetry, mean arterial blood pressure, pulmonary-to-systemic flow ratio (Qp/Qs), or any other physiologic or inotropic support variable between groups. The variability of physiologic values related to pulmonary blood flow was greater in the RV-PA group (Qp/Qs coefficient of variation, 0.91 versus 2.50, p < 0.001)., Conclusions: In this randomized prospective study, no hemodynamic benefits of the RV-PA modification for stage 1 palliation of hypoplastic left heart syndrome were found. Pulmonary blood flow was more variable, and the diastolic blood pressure was higher. These findings did not influence indicators of systemic oxygen delivery with our afterload reduction strategy.

Background: Children with univentricular hearts and aortic arch obstruction are treated sequentially with Norwood procedure, superior cavopulmonary anastomosis (SCPA), and Fontan operation. Early SCPA results in lower initial O2 saturation and longer hospitalization, but not increased mortality. We sought to determine the impact of early SCPA on Fontan candidacy and outcomes., Methods: Eighty-five consecutive patients undergoing Norwood operation between January 1998 and February 2003 were divided into group 1 (SCPA at less than 4 months, n = 33) and group 2 (SCPA at more than 4 months, n = 52). Of the original cohort, 69 have undergone Fontan operation, 7 await Fontan, 1 was transplanted, 3 are not Fontan candidates, and 5 died late after SCPA. Group 1 (n = 25) and group 2 (n = 44) patients who have completed Fontan operation were compared for preoperative and perioperative variables: age, size, O2 saturation, pulmonary artery pressure and size, prevalence of tricuspid regurgitation and ventricular dysfunction, extubation rate in operating room, duration of pleural drainage, hospital stay, and discharge O2 saturation. Late functional status and ventricular function were also compared. Survival was compared for original groups 1 and 2., Results: There were no differences for any preoperative or perioperative variable, or late functional assessment. Actuarial survival at 6 years was also not different (88% +/- 5% for group 1 and 94% +/- 4% for group 2, p = 0.72)., Conclusions: Although initially more cyanotic and hospitalized longer than older peers, younger SCPA patients achieve clinical equivalence by the time of Fontan operation and afterward. We conclude that both short- and long-term outcomes support performance of early SCPA.

Despite improved early results with the Norwood procedure (stage one palliation), patients remain with at-risk anatomy and interstage mortality continues to be a limitation of staged single ventricle palliation. Retrospective analyses have implicated residual or recurrent anatomic lesions as well as intercurrent illness as causes of interstage mortality. We hypothesized that potentially life-threatening anatomic lesions and illnesses would be manifest before serious physiologic impact by alteration in arterial saturation, failure to gain weight or in the case of dehydration, acute weight loss. As a result, we developed a home monitoring program of daily weights and oxygen saturations to earlier identify those patients at increased risk for interstage death. Frequent monitoring of these physiologic variables between stage one and two palliation identified life-threatening anatomic lesions and illness and permitted timely intervention that ultimately improved survival. All 36 survivors of the stage one palliation discharged from the hospital and entered into the home monitoring program survived the interstage period.