Francisco A. Guzmán-Pruneda

Incidence and treatment of chylothorax after cardiac surgery in children: analysis of a large multi-institution database.

OBJECTIVE There is limited information regarding the true incidence of and risk factors for chylothorax after pediatric cardiac surgery. The objective of this study was to determine, from a large multi-institution database, incidence, associated factors, and treatment strategy in patients undergoing pediatric cardiac surgery. METHODS All patients younger than 18 years in the Pediatric Health Information System (PHIS) database who underwent congenital heart surgery or heart transplant from 2004 to 2011 were included. Procedure complexity was assessed by Risk Adjustment for Congenital Heart Surgery-1. RESULTS In all, 77,777 patients (55% male) of median age 6.7 months were included. Overall incidence of chylothorax was 2.8% (n = 2205), significantly associated with increased procedure complexity, younger age, genetic syndromes, vein thrombosis, and higher annual hospital volume. Patients with multiple congenital procedures had the highest incidence. Incidence increased with time, from 2% in 2004 to 3.7% in 2011 (P < .0001). Chylothorax was associated with longer stay (P < .0001), increased adjusted risk for in-hospital mortality (odds ratio, 2.13; 95% confidence interval, 1.75-2.61), and higher cost (P < .0001), regardless of procedure complexity. Of all patients with chylothorax, 196 (8.9%) underwent thoracic duct ligation or pleurodesis a median of 18 days after surgery. Total parenteral nutrition, medium-chain fatty acid supplementation, and octreotide were used in 56%, 1.7%, and 16% of patients, respectively. CONCLUSIONS Chylothorax is a significant problem in pediatric cardiac surgery and is associated with increased mortality, cost, and length of stay. Strategies should be developed to improve prevention and treatment.

Surgical pulmonary valve replacement: a benchmark for outcomes comparisons.

BACKGROUND Patients with right heart obstructive lesions develop residual or recurrent right ventricle outflow tract pathology as a result of native or implanted pulmonary valve (PV) dysfunction. Until recently, the standard of care has been surgical placement of a PV or valved right ventricle to pulmonary artery conduit. Catheter-based options are being increasingly applied in patients with PV dysfunction. The purpose of our study was to evaluate outcomes of surgical pulmonary valve/conduit replacement (PVR) at a large pediatric hospital to provide contemporary benchmark data for comparison with developing technologies. METHODS Retrospective review of patients undergoing PVR not associated with complex concomitant procedures from July 1995 to December 2010 was completed. Inclusion criteria were designed to generally match those applied to patients promoted for catheter-based valve replacement based on age and weight (age≥5 years and weight≥30 kg). RESULTS There were 148 PVRs with all patients having undergone ≥1 previous interventions (tetralogy of Fallot [53%] and pulmonary atresia [17%]). Surgical indications were PV insufficiency (60%), PV stenosis (26%), and both (13%). Valves used included bioprosthetic (n=108; 73%) and homografts (n=40; 27%). Time-to-extubation, intensive care unit stay, and hospital length of stay were <1 day (interquartile range, 0-1 day), 2 days (interquartile range, 1-2 days), and 5 days (interquartile range, 4-6 days), respectively, with no hospital deaths. Freedom from PV reintervention at 1, 3, and 5 years was 99%, 99%, and 94%, respectively. Multivariable analysis showed age<13 years (P=.003), and smaller valve size (P=.025) were associated with increased risk of valve reintervention. Patient survival at follow-up (mean, 5.0±3.9 years) was 99%. CONCLUSIONS Surgical PVR is safe with low in-hospital and midterm follow-up mortality and reoperation rates. These outcomes provide a useful benchmark for treatment strategy comparisons.

Risk factors for development of endocarditis and reintervention in patients undergoing right ventricle to pulmonary artery valved conduit placement

OBJECTIVE To determine the incidence and risk factors for endocarditis and reintervention in patients undergoing placement of right ventricle-to-pulmonary artery valve conduits. METHODS All right ventricle-to-pulmonary artery valved conduits placed between 1995 and 2014 were included. Freedom from endocarditis, reintervention, and replacement were analyzed using the Kaplan-Meier method and parametric survival regression models. RESULTS A total of 586 patients underwent placement of a total of 792 valved conduits, including 289 (36%) pulmonary homografts, 121 (15%) aortic homografts, 245 (31%) bovine jugular grafts, and 137 (17%) porcine heterografts. There were 474 (60%) primary placements and 318 (40%) replacements. The median duration of conduit follow-up was 7 years; 23 conduits developed endocarditis at a median of 5 years after surgery. The use of bovine jugular grafts was the sole significant risk factor associated with endocarditis (hazard ratio, 9.05; 95% confidence interval, 2.6-31.8 compared with homografts). The hazard was greater for bovine jugular grafts compared with the other conduit types and increased with time; however, bovine jugular grafts were associated with a lower risk for reintervention (P < .0001) and replacement (P = .0002). Factors associated with greater risk of both reintervention and replacement were younger age and smaller conduit size. In addition, a diagnosis of truncus arteriosus was associated with a greater risk for replacement (P = .03). CONCLUSIONS Bovine jugular grafts are associated with a significantly greater risk of late endocarditis but with lower reintervention rates compared with other valved conduits. The risk of endocarditis and durability must be balanced during conduit selection. Antibiotic prophylaxis and a high index of suspicion for endocarditis are warranted in patients with bovine jugular grafts.

Aortic Arch Advancement for Aortic Coarctation and Hypoplastic Aortic Arch in Neonates and Infants

BACKGROUND The optimal treatment for infants with aortic coarctation and hypoplastic aortic arch is controversial. The goal of this study was to report the short-term and mid-term outcomes of aortic arch advancement (AAA) in infants with hypoplastic aortic arch. METHODS All infants who underwent AAA at our institution from 1995 to 2012 were included. AAA consisted of coarctectomy and end-to-side anastomosis of the descending aorta to the distal ascending aorta/proximal arch through a median sternotomy. The cohort was divided into four groups: (1) isolated AAA (n=29, 11%), (2) AAA with closure of ventricular septal defect (n=56, 20%), (3) AAA with other biventricular repairs (n=115, 42%), and (4) AAA as part of single-ventricle palliation (n=75, 27%). RESULTS The cohort included 275 patients: 125 (45%) were female, and the median age was 14 days (interquartile range, 7-34 days). Genetic abnormalities were present in 48 patients (17%). Neurologic adverse events occurred in 3 patients (1%), all in group 4. Left bronchial compression was seen in 2 patients (0.7%); only one required intervention. Vocal cord dysfunction was noted in 36 of 95 patients (38%) on routine laryngoscopy. Only 1 patient had clinical residual dysfunction at the last follow-up visit. Perioperative mortality was 3% (n=8). At a median follow-up time of 6 years, 8 patients (3%) had reinterventions at a median time of 5 months (3-17 months) after repair. CONCLUSIONS AAA is a safe, effective, and durable operation with low rates of adverse events and mid-term reintervention. The advantages include native tissue-to-tissue reconstruction and preserved potential for growth. As such, it is the ideal technique for the management of hypoplastic aortic arch in neonates and infants.

Evolution and impact of ventricular assist device program on children awaiting heart transplantation.

BACKGROUND We sought to evaluate the impact of the evolution of a pediatric mechanical circulatory support (MCS) program on outcomes of children listed for heart transplantation at our institution. METHODS All patients listed for isolated heart transplantation from 1995 to 2013 were included. The use of MCS while on the wait-list was recorded. Wait-list and posttransplant outcomes were compared before and after 2005, which was when we became capable of providing long-term MCS without size limitation. RESULTS In total, 259 patients were listed for transplant and 201 (78%) reached transplant. The use of MCS was significantly increased between the eras (13% and 37%, p = 0.0001). Wait-list mortality was significantly decreased (25% and 11%, p = 0.0006). Among transplant recipients, the proportion of patients who underwent MCS was significantly increased (13% and 37%, p = 0.0002). Of these MCS patients, the use of long-term devices was significantly increased (50% and 98%, p = 0.0004). Median duration of MCS was significantly increased (12 and 78 days, p = 0.004). Kaplan-Meier estimates showed a trend (p = 0.08) toward improved survival after bridge-to-transplant both at 1 year (70% in the early era and 88% in the late era) and at 5 years (60% and 78%, respectively). CONCLUSIONS Outcomes of pediatric heart transplantation have significantly improved over the last 2 decades. We believe such improvement is, at least in part, attributable to maturation of MCS strategy, characterized by avoiding the use of temporary devices such as extracorporeal membrane oxygenation as a bridge-to-transplant and a more aggressive use of long-term devices.

A modified implantation technique of the HeartWare ventricular assist device for pediatric patients

Limitations to our study include the lack of a control group or a different treatment arm. Owing to recurrent, lifethreatening, and refractory bleeding in these patients, we concluded that withholding a therapy that we had previously shown would benefit these patients would be unethical. We also did not assess the relative effect of thalidomide in reduction of LVAD-associated GIAD compared with other therapeutic agents. Other investigators have used agents, including octreotide, to reduce bleeding due to GIAD in LVAD patients, although octreotide is difficult to tolerate due to its mode of delivery (injection) and adverse effects, including nausea and bradycardia. Lenalidomide, a synthetic analog of thalidomide, has been shown to be as potent as the parent drug, with less of the non-hematologic adverse effects, and is another potential treatment option for these patients. In summary, thalidomide can be used safely in patients with LVAD-associated GIAD when prescribed as part of a treatment protocol. These findings must be replicated in multicenter, randomized studies to corroborate our results. Disclosure statement

Contemporary Results of Aortic Coarctation Repair Through Left Thoracotomy

BACKGROUND Although surgical results for repair of coarctation of the aorta (CoA) have steadily improved, management of this condition remains controversial. The purposes of this study were to analyze the long-term outcomes of patients undergoing CoA repair through left thoracotomy and to define risk factors for reintervention. METHODS All patients who were less than 18 years old and who underwent initial repair of CoA through left thoracotomy from 1995 to 2013 at Texas Childrens Hospital (Houston, TX) were included. Patients were classified into 3 groups: 143 (42%) neonates (0 to 30 days old), 122 (36%) infants (31 days to 1 year old), and 78 (23%) older children (1 to 18 years old). Univariate and multivariate analyses were performed. RESULTS A total of 343 patients (129 [38%] girls) with median age of 53 days (interquartile range [IQR],12 days to 9 months) and weight of 4.1 kg (IQR, 3.1 to 8.0) underwent repair with extended end-to-end anastomosis (291 patients [85%]), end-to-end anastomosis (44 patients [13%]), interposition graft (2 patients [0.6%]), or subclavian flap (6 patients [2%]). Concomitant diagnoses included genetic abnormalities (48 patients [14%]), isolated ventricular septal defects (58 patients [17%]), small left-sided structures (53 patients,16%), or other complex congenital heart disease (18 patients [5%]). Perioperative mortality was 1% (n = 4, all neonates). At a median follow-up of 6 years (7 days to 19 years), only 14 (4%) patients required reintervention (10 catheter-based procedures, 6 surgical repairs). A postoperative peak velocity of 2.5 m/s or greater was an independent risk factor for reintervention (odds ratio [OR], 4.0; 95% confidence interval [CI], 1.4 to 11.6). Within the cohort, 95 (33%) patients were hypertensive or remained on cardiac medications a median of 12 years (6 months to 19 years) after the surgical procedure. Development of perioperative hypertension was associated with higher risk of chronic hypertension or cardiac medication dependency (OR, 1.9; 95% CI, 1.1 to 3.3). CONCLUSIONS CoA repair through left thoracotomy is associated with low rates of morbidity, mortality, and reintervention. Aortic arch obstruction should be completely relieved at the time of surgical intervention to minimize the risk of long-term recoarctation.

Contemporary results of surgical repair of recurrent aortic arch obstruction.

BACKGROUND There is a paucity of data on the current outcomes of surgical intervention for recurrent aortic arch obstruction (RAAO) after initial aortic arch repair in children. The goal of this study is to report the long-term results in these patients. METHODS All patients undergoing surgical intervention for RAAO at Texas Childrens Hospital from 1995 to 2012 were included. The cohort was divided into four groups based on initial procedure: (1) simple coarctation repair, (2) Norwood procedure, (3) complex congenital heart disease, and (4) interrupted aortic arch. RESULTS A total of 48 patients age 9 months (range, 22 days to 36 years) underwent 49 procedures for RAAO. All patients had an anatomic repair consisting of either patch aortoplasty (n=27, 55%), aortic arch advancement (n=8, 16%), sliding arch aortoplasty (n=6, 12%), placement of an interposition graft (n=2, 17%), reconstruction with donor allograft (n=4, 8%), extended end-to-end anastomosis (n=1, 2%), or redo Norwood-type reconstruction (n=1, 2%). Most procedures (n=46, 94%) were performed through a median sternotomy using cardiopulmonary bypass. At a median follow-up of 6.1 years (range, 9 days to 17 years), only 2 patients required surgical or catheter-based intervention for RAAO. Hypertension was present in 10% of patients at last follow-up. There were no neurologic or renal complications. There was 1 perioperative death after an aortic arch advancement in group 1. Four other patients have died during follow-up, none of the deaths related to RAAO. CONCLUSIONS Anatomic repair of RAAO is a safe procedure associated with low morbidity and mortality, and low long-term reintervention rates.

Bronchial artery revascularization and en bloc lung transplant in children

BACKGROUND Long-term success in pediatric lung transplantation is limited by infection and bronchiolitis obliterans syndrome (BOS). The bilateral sequential lung transplantation (BSLT) technique may result in airway ischemia leading to bronchial stenosis, dehiscence, or loss of small airways. En bloc lung transplant (EBLT) with bronchial artery revascularization (BAR) minimizes airway ischemia, thus promoting superior airway healing. BAR also allows for safe tracheal anastomosis, circumventing the need for bilateral bronchial anastomoses in small children. METHODS This was a retrospective review of bilateral transplantations from 2005 to 2014. Both techniques were used in parallel. Redo and multiorgan transplants were excluded. RESULTS There were 119 recipients comprising 88 BSLTs and 31 EBLTs. Follow-up time was 3 years (interquartile range, 1-5 years). Donor ischemic and cardiopulmonary bypass times were not different between techniques (p = 0.48 and p = 0.18, respectively). Degree of graft dysfunction and cellular rejection scores were not different (p = 0.83 and p = 0.93, respectively). There were 3 hospital deaths after BSLT and 2 after EBLT (p = 0.60). Overall survival was 61% for the BSLT group and 77% for the EBLT group (p = 0.54). Freedom from BOS was 71% in the BSLT group and 94% in the EBLT group (p = 0.08). On routine bronchoscopy, 57% BSLT and 16% EBLT patients had 1 or more airway ischemic findings (p < 0.0001). Multivariate analysis showed BSLT was associated with higher ischemic injury (relative risk, 2.86; 95 confidence interval, 1.3-6.5; p = 0.01) and non-airway complications (relative risk, 4.62; 95% confidence interval, 1.1-20.2; p = 0.04) but not airway reinterventions (p = 0.07). Airway dehiscence occurred in 3 BSLT patients. CONCLUSIONS Pediatric EBLT with BAR can be safely performed without increasing operative or graft ischemic times. Airway ischemia and non-airway complications were significantly reduced when BAR was combined with tracheal anastomosis, potentially diminishing morbidity caused by anastomotic healing complications.

Simultaneous Repair of Right-Sided Coarctation and Vascular Ring

We present the case of a 4-year old girl with posterior fossa-hemangioma-arterial lesions-cardiac abnormalities/coarctation-eye abnormalities (PHACE) syndrome, an atypical, long-segment, right-sided coarctation of the aorta and vascular ring secondary to an aberrant left subclavian artery and left ligamentum. Simultaneous repair of both lesions was accomplished using a novel technique that included reimplantation of the aberrant left subclavian artery and translocation of the descending aorta to the proximal ascending aorta.