Aortic cusp extension valvuloplasty with or without tricuspidization in children and adolescents: Long-term results and freedom from aortic valve replacement

ObjectiveAortic cusp extension valvuloplasty is increasingly used in the management of children and adolescents with aortic stenosis or regurgitation. The durability of this approach and the freedom from valve replacement are not well defined. A study was undertaken to investigate outcomes.MethodsFrom July 1987 to November 2008, 142 patients aged less than 19 years underwent aortic cusp extension valvuloplasty in the form of pericardial cusp extension and tricuspidization (when needed). Three patients with truncus arteriosus and severe truncal valve insufficiency were excluded. From the available follow-up data of 139 patients, 50 had bicuspid aortic valves, 40 had congenital aortic valve stenosis, 41 had combined congenital aortic valve stenosis/insufficiency, and 8 had other diagnoses. Median follow-up was 14.4 years (0.1–21.4). Long-term mortality and freedom from aortic valve replacement were studied.ResultsThere were no early, intermediate, or late deaths. Z-values of left ventricular end-diastolic dimension, aortic annulus, aortic sinus diameter, and sinotubular junction diameter before aortic valve replacement were 4.2 ± 3.11, 2.3 ± 1.25, 4.4 ± 1.23, and 1.84 ± 1.28, respectively. During the follow-up period, 64 patients underwent aortic valve reinterventions. The Ross procedure was performed in 32 of 139 patients (23%) undergoing aortic cusp extension valvuloplasty. Other aortic valve replacements were undertaken after 16 aortic cusp extension valvuloplasties (11.5%). Freedom from a second aortic cusp extension valvuloplasty or aortic valve replacement at 18 years was 82.1% ± 4.2% and 60.0% ± 7.2%, respectively.ConclusionAortic cusp extension valvuloplasty is a safe and effective surgical option with excellent survival and good long-term outcomes in children and adolescents. The procedure provides acceptable durability and satisfactory freedom from aortic valve replacement

Repair of superior sinus venosus atrial septal defect using a modified two-patch technique

Background: Surgical repair of superior sinus venosus atrial septal defect associated with partial anomalous pulmonary venous connection is usually performed with either a two-patch technique or the Warden procedure. However, both techniques can lead to superior vena cava or pulmonary vein stenosis, as well as sinus node dysfunction. To prevent these complications, we have utilized a modified two-patch technique. Methods: Between January 2000 and December 2015, 44 patients underwent modified two-patch repair of superior sinus venosus atrial septal defect associated with partial anomalous pulmonary venous connection at our institution. The technique utilizes the right atrial appendage to patch the superior vena cava and avoid superior vena cava obstruction. Patients\u27 data were collected from reviewing hospital charts and clinic records. Results: Mean age was 10.3 years (range, 0.68 to 61 years). There was no hospital mortality. Six patients (13.6%) had sinus node dysfunction after surgery. All of them recovered their normal sinus node function at a mean follow-up period of 45.7 months. No superior vena cava or pulmonary vein stenosis was observed at long-term follow-up. Conclusions: A modified two-patch technique is a valid alternative to other methods of superior sinus venosus atrial septal defect and partial anomalous pulmonary venous connection repair, and prevents late systemic/pulmonary venous pathway obstruction while maintaining sinus rhyth

Effects of modified ultrafiltration on thromboelastographic profile after pediatric cardiac surgery

Modified ultrafiltration (MUF) is still used after pediatric cardiopulmonary bypass (CPB) in some pediatric cardiac surgery centers to decrease transfusion requirements. Other potential benefits of MUF include clearance of inflammatory markers and improvement in myocardial function. Our hypothesis is that MUF will hemoconcentrate coagulation factors and improve thromboelastography (TEG) parameters after pediatric CPB. Patients younger than 6 months were prospectively enrolled over a year. TEG was carried out before MUF, after MUF, and after protamine administration. Paired t tests were conducted to compare values pre-MUF and post-MUF as well as post-MUF and post-protamine administration. Thirty patients were enrolled in the study, with 20 (67%) neonates in the cohort. Seven arterial switch operations and nine Norwood procedures were found to be performed among the cohort. Reaction time (R), angle (α), and maximum amplitude (MA) were significantly worse post-MUF compared with pre-MUF (p \u3c .001). They improved significantly after protamine administration compared with post-MUF (p \u3c .001). The amount of fluid removal was significantly associated with a worse post-MUF R, angle, and MA and worse post-protamine administration, angle, and MA but with no effect on post-protamine R. MUF caused worsening of TEG parameters that is reversed by protamine administration

Technical modifications that might improve long-term outcome of the ross procedure in children

BACKGROUND: Failure of the pulmonary autograft (PVA) following the Ross procedure (RP) has discouraged its widespread use and led to modifications or alternatives to the procedure. We sought to analyze whether certain technical modifications could improve results of the RP in children. METHODS: Sixty-nine patients (median age 12 years, range 0.25 to 17.9) underwent the RP between 01/1996 and 12/2018. Concomitant Konno procedure was performed on 20/69 (29%). Prior interventions included balloon valvuloplasty in 30/69 (44%), and/or surgical valvuloplasty in 39/69 (57%). Technical modifications included utilizing the native aortic root for external annuloplasty, implanting the autograft using uniplanar horizontal sutures through the aortic wall, normalizing the sinotubular junction and wrapping the native root remnant around the PVA. RESULTS: Operative mortality was 1/69 (1.5%), with no late death. No patient had neoaortic valvar stenosis and 7/68 (10%) had mild regurgitation on discharge echocardiogram. At latest follow-up (median 9.4 years, range 0.4 - 21.3) there was no significant change in the latest follow up Z scores of annulus, sinus or sinotubular junction diameters when compared to those at discharge. Three patients (4.4%) required late autograft replacement, two PVA repair, and two resection of pseudoaneurysm. Actuarial freedom from PVA replacement was 87% at 20 yrs. Freedom from right ventricular outflow tract catheter reintervention or reoperation was 83% and 80% respectively. CONCLUSIONS: Technical modifications of the RP used in this cohort might successfully prolong the life of the PVA without compromising its growth, an important advantage in pediatric patients

Efficacy of nitric oxide administration in attenuating ischemia/reperfusion injury during neonatal cardiopulmonary bypass

OBJECTIVE: Nitric oxide (NO) plays several protective roles in ischemia/reperfusion (I/R) injury. Neonates undergoing the Norwood procedure are subject to develop I/R injury due to the immaturity of their organs and the potential need to interrupt or decrease systemic flow during surgery. We hypothesized that NO administration during cardiopulmonary bypass (CPB) ameliorates the I/R and could help the postoperative recovery after the Norwood procedure. METHODS: Twenty-four neonates who underwent a Norwood procedure were enrolled in a prospective randomized blinded controlled trial to receive NO (12 patients) or placebo (12 patients) into the oxygenator of the CPB circuit during the Norwood procedure. Markers of I/R injury were collected at baseline (T0), after weaning from CPB before modified ultrafiltration (T1), after modified ultrafiltration (T2), and at 12 hours (T3) and 24 hours (T4) after surgery, and they were compared between both groups, as well as other postoperative clinical variables. RESULTS: There was no difference in age, weight, anatomical diagnosis, CPB, and aortic cross-clamp time between both groups. Troponin levels were lower in the study group at T1 (0.62 ± 58 ng/mL vs 0.87 ± 0.58 ng/mL, CONCLUSION: Systemic administration of NO during the Norwood procedure has myocardial protective effects (lower Troponin levels) but we observed no effect on postoperative recovery. Larger sample size may be needed to show clinical differences

Outcomes from three decades of infant and pediatric heart transplantation

Infants are a unique transplant population due to a suspected immunologic advantage, in addition to differences in size and physiology. Consequently, we expect infants to have significantly different diagnoses, comorbidities, and outcomes than pediatric transplant recipients. In this study, we compare patterns and trends in pediatric and infant heart transplantation during three decades. The United Network for Organ Sharing (UNOS) database was queried for transplants occurring between January 1990 and December 2018. Patients were categorized as pediatric (1-17) or infant (0-1). Congenital heart disease (CHD) primary diagnoses have increased from 37% to 42% in pediatric patients (p = 0.001) and decreased from 80% to 61% in infants during the 1990s and 2010s (p \u3c 0.001). Those with CHD had worse outcomes in both age groups (p \u3c 0.001). Infants who underwent ABO-incompatible transplants had similar survival as compared to those with compatible transplants (p = 0.18). Overall, infants had better long-term survival and long-term graft survival than pediatric patients; however, they had worse short-term survival (p \u3c 0.001). Death due to rejection or graft failure was less likely in infants (p = 0.034). However, death from infection was over twice as common (p \u3c 0.001). In summary, pediatric and infant heart transplant recipients differ in diagnoses, comorbidities, and outcomes, necessitating different care for these populations

Pulmonary hypertension and mitral regurgitation in an infant with an anatomically normal mitral valve

A full-term, female presented on her date of birth with severe pulmonary hypertension (PH) and mitral regurgitation (MR), requiring veno-arterial extracorporeal membrane oxygenation. After the treatment, her PH and MR were resolved with no anatomic abnormality present. We propose a positive feedback loop of PH causing right ventricular dilation and interventricular septal shifts, worsening MR, and elevated left atrial, and potentially pulmonary, pressures

Anomalous origin of the left coronary artery from the right pulmonary artery of an infant

A female presented 2 weeks after birth with an unbalanced atrioventricular canal, double outlet right ventricle, mild pulmonary stenosis, and patent ductus arteriosus that eventually caused pulmonary over circulation. After pulmonary artery banding, she experienced myocardial ischemia, suggesting interference with coronary blood flow by the band that had been placed on the main pulmonary trunk. The band was removed and revised to bilateral branch pulmonary artery banding, and cardiac function improved. An anomalous left coronary artery from the underside of the right pulmonary artery was identified. Eight weeks later, the patient underwent coronary transfer and reimplantation of the left coronary artery into the aorta followed by main pulmonary artery banding. She subsequently underwent bidirectional Glenn

Tricuspid atresia and common arterial trunk: A rare form of CHD

Tricuspid atresia with common arterial trunk is a very rare association in complex CHD. This association has even more infrequently been documented concomitantly with interrupted aortic arch. We present the diagnosis and initial surgical management of an infant with a fetal diagnosis of tricuspid atresia and common arterial trunk, with additional postnatal finding of interrupted aortic arch with interruption between the left common carotid and left subclavian artery. Due to the infant\u27s small size, she was initially palliated with bilateral pulmonary artery bands and a ductal stent. This was followed by septation of the common arterial trunk and interrupted aortic arch repair and 4 mm right subclavian artery to main pulmonary artery shunt placement at two months of age. She was discharged home on day of life 81