Brian Kogon

Features of portal hypertension are associated with major adverse events in Fontan patients: The VAST study

BACKGROUND Chronic congestive hepatopathy is known to cause hepatic fibrosis and portal hypertension in patients post-Fontan operation for single ventricle palliation. The clinical significance of these findings is not clear. We hypothesized that features of portal hypertension would be significantly related to major adverse events. METHODS A retrospective review of 73 adult and pediatric post-Fontan patients referred for a liver evaluation from 2001 to 2011 was performed. The relationship between features of portal hypertension (VAST score ≥2, 1 point each for Varices, Ascites, Splenomegaly or Thrombocytopenia) and a major adverse event (death, need for transplant, or hepatocellular carcinoma) was examined using logistic regression. RESULTS 73 post-Fontan patients (30% female, 73% Caucasian, 66% systemic left ventricle (SLV), mean age 24±11 years, mean interval from Fontan 17±6 years) were included in analysis. Features of portal hypertension (VAST score ≥2) were present in 26 (36%), and there were 19 major adverse events: death (n=12), transplant (n=6), and HCC (n=1). A significant relationship was found between VAST score ≥2 and major adverse events (OR=9.8, 95% CI [2.9-32.7]). After adjusting for time since Fontan, SLV, age, hemoglobin and type of failure, VAST score ≥2 remained significant (OR=9.1, 95% CI [1.4-57.6]). CONCLUSION Fontan patients with features of portal hypertension have a 9-fold increased risk for a major adverse event. Therapies targeted to manage clinical manifestations of portal hypertension, and early referral to heart transplant may help delay major adverse events. Future prospective studies are needed to confirm these findings.

Heart Transplantation in Children With a Fontan Procedure

BACKGROUND Previous studies have reported that children with a prior Fontan procedure have decreased survival after heart transplantation. We examined 190 primary pediatric heart transplants. METHODS Since 1988, 27 (14.2%) of 190 children less than 18 years old undergoing primary heart transplantation had a Fontan procedure 3.7 ± 4.3 years before transplantation. Compared with 163 (85.8%) non-Fontan primary transplants, the Fontan patients were similar in age (8.2 ± 5.0 vs 6.5 ± 6.0 years), presensitization, and pretransplant clinical status. More Fontan patients had prior operations (100% vs 50%; p < 0.0001) and needed pulmonary artery reconstruction (100% vs 23.5%; p < 0.0001). Twelve (44%) had protein-losing enteropathy. RESULTS Donor ischemic times (211 ± 72 vs 170 ± 61 minutes; p = 0.0018) and cardiopulmonary bypass times (197 ± 91 vs 121 ± 53 minutes; p < 0.0001) were greater in the Fontan group as were durations of ventilator support (4.9 ± 6.6 vs 2.6 ± 3.9 days; p = 0.018) and hospital stay (20.2 ± 17.5 vs 14.3 ± 12.4 days; p = 0.0435). The Fontan group had one 30-day mortality. One-year actuarial survival (81.5% vs 84.6%, Fontan vs non-Fontan) and five-year actuarial survival (65.5% vs 66.2%, Fontan vs non-Fontan) were similar, as was rejection incidence at one year (2.0 ± 2.0 vs 1.7 ± 1.9 episodes per patient; p = 0.3972). Five Fontan patients (18.5%) required retransplantation 4.9 ± 3.6 years posttransplant compared with 18 non-Fontan patients (11.0%) retransplanted 5.2 ± 3.4 years posttransplant (p = 0.3346). CONCLUSIONS Contrary to prior reports, we did not identify any early or midterm disadvantage for children undergoing heart transplantation after a previous Fontan procedure despite more complex transplant operations. We contend that carefully selected children with a failing Fontan circulation can do as well as other children with heart transplantation.

The bidirectional Glenn operation: a risk factor analysis for morbidity and mortality.

OBJECTIVE Patients with single ventricle heart defects often undergo a palliative bidirectional Glenn operation. For this operation, we analyzed potential risk factors for morbidity and mortality. We also evaluated the effects of a persistent left superior vena cava by comparing the outcomes of unilateral and bilateral operations. METHODS We reviewed the clinical records of 270 consecutive patients who underwent a bidirectional Glenn operation between 2001 and 2007. A total of 226 patients underwent unilateral operations and 44 patients underwent bilateral operations. Patient characteristics included weight and age, single ventricle morphology, vena caval anatomy, and previous surgery. Operative details included cardiopulmonary bypass technique and duration, pulmonary artery management, hemi-Fontan construction, concomitant procedures, and hemodynamics. Outcome data included duration of chest tube drainage, lengths of intensive care unit and hospital stay, morbidity, and mortality (<30 days). RESULTS The median length of chest tube drainage was 2.4 days (range 1-20 days). Risk factors for prolonged drainage were elevated central venous pressure (P = .015) and transpulmonary gradient (P = .011). The median lengths of stay in the intensive care unit and hospital were 50 hours (range 20-1628 hours) and 5 days (range 2-83 days), respectively. Risk factors for both included prolonged cardiopulmonary bypass time, elevated central venous pressure and transpulmonary gradient, and right ventricular morphology. Overall, 72 of 270 patients (27%) had 116 postoperative complications. Risk factors included prolonged cardiopulmonary bypass time (P = .002) and elevated central venous pressure (P = .029). Mortality was 2 of 270 patients (0.7%). No risk factors for death were identified. Weight (median 6.8 kg vs 6.2 kg, P = .038) and age (median 186 days vs 159 days, P = .001) at the time of surgery were significantly greater in the bilateral bidirectional Glenn group compared with the unilateral group. However, there was no difference in any of the outcome variables. CONCLUSION Outcomes were adversely affected primarily by prolonged cardiopulmonary bypass time, elevated central venous pressure and transpulmonary gradient, and right ventricular morphology. Specifically, outcomes were unaffected by the presence of a left superior vena cava, cannulation strategy, or antegrade pulmonary blood flow. There were few differences between the unilateral and bilateral groups, none of which were postoperative outcomes.

Extracorporeal Cardiopulmonary Resuscitation for Pediatric Cardiac Patients

BACKGROUND Extracorporeal cardiopulmonary resuscitation (ECPR) has been shown to improve survival after in-hospital pediatric cardiac arrest. We describe our experience with ECPR for refractory cardiac arrest in pediatric cardiac patients. METHODS We performed a retrospective analysis of the use of venoarterial extracorporeal membrane oxygenation (ECMO) for in-hospital cardiac arrest from 2002 to 2011. The primary endpoint was survival to discharge, and the secondary endpoint was long-term functional neurologic status. RESULTS Of 160 total uses of cardiac ECMO in 159 patients, 90 (56%) were ECPR (mean age 2.05 years; range, 0 days to 16.5 years). Sixty-four patients (71%) were postoperative, of which 36 were single ventricle and 28 were biventricular. Nine patients (10%) had cardiomyopathy-myocarditis, and 17 patients (19%) were nonpostoperative (5 single ventricle; 12 biventricular). Fifty-nine patients (66%) had open chest cannulation, and 31 (34%) had peripheral cannulation. Fifty patients (56%) survived to discharge. Duration of ECMO was 4.3±4.0 days (median 3) for survivors and 6.3±5.4 days (median 5) for nonsurvivors (p<0.05). On follow-up, almost half of survivors without genetic syndromes had normal neurologic status. CONCLUSIONS Extracorporeal cardiopulmonary resuscitation is an appropriate application of ECMO in pediatric cardiac patients. We report overall survival of 56%. Cardiomyopathy patients have favorable outcomes (89% survival). Biventricular patients have better outcomes then single ventricle patients (p<0.01). Extracorporeal cardiopulmonary resuscitation also seems to be a good strategy for nonpostoperative patients (71% survival). Nearly half of postoperative patients (46%) resuscitated with ECPR survived to hospital discharge.

Symptomatic Neonatal Tetralogy of Fallot: Repair or Shunt?

BACKGROUND The management of neonates with symptomatic tetralogy of Fallot (TOF) requiring surgical intervention in the first month of life remains controversial. We reviewed our experience with neonates 30 days or greater with TOF from 2002 to 2008 requiring surgical intervention. METHODS Thirty-seven consecutive symptomatic nonrandomized neonates with TOF or its variants (including TOF with complete atrioventricular septal defect or absent pulmonary valve but excluding pulmonary atresia) had either a shunt (n = 17) or primary repair (n = 20). The shunted patients more commonly required emergency operation (24% vs 0%; p = 0.036); otherwise, the two groups were similar. RESULTS One infant with preoperative bowel ischemia died one day postoperatively after emergency shunting. There were two late deaths 11 and 12 months postoperatively, both in primary repair patients. The 16 surviving shunted patients had TOF repair 216 +/- 99 days after the original shunt. The shunted patients had shorter intensive care unit and hospital stays for the first operation, which became equivalent when the second hospitalization (repair) values were added. The primary repair patients more frequently had a transannular patch and a tendency to more frequent delayed sternal closure. Four primary repair (20%) and two shunted (12.5%) patients required subsequent cardiac operations after complete repair (p = not significant). CONCLUSIONS Shunting or primary repair of neonates with symptomatic TOF provides equivalent mortality and results. Shunted patients had fewer transannular patch repairs despite having more emergent initial operations. Compared with the primary repair group, shunted patients had decreased intensive care unit and hospital stays for the first hospitalization, which were neutralized when the second operation (repair) values were added.

One Thousand Repeat Sternotomies for Congenital Cardiac Surgery: Risk Factors for Reentry Injury

BACKGROUND Reentry injury is a risk associated with repeat sternotomy for cardiac surgery. This risk has been well defined for adults, but there is less information available for patients with congenital heart disease. The goal of this review was to identify the incidence, risk factors, and outcomes for reentry injury in patients with congenital heart disease. METHODS Eight hundred two patients with congenital heart disease had 1,000 consecutive repeat sternotomies between August 2000 and November 2007. Records were reviewed for demographics, history, operative techniques, and outcomes. Univariate risk factors for reentry injury and operative mortality were assessed. RESULTS Median age and weight were 2.1 years (range, 0.1 to 34.6 years) and 11 kg (range, 2.5 to 123 kg). There were 639 second, 287 third, and 74 fourth or higher sternotomies. There were 13 reentry injuries (1.3%) involving right ventricle-pulmonary artery conduits (n = 4), aorta or aortic conduits (n = 3), right ventricular outflow tract patches or pseudoaneurysms (n = 3), and others (n = 3). Risk factors for injury were presence of a right ventricle-pulmonary artery conduit (6 of 115 with conduit [5.2%] versus 7 of 885 without [0.8%]; p < 0.001) and sternotomy number (relative risk, 2.28; p < 0.001). Reentry injury was associated with longer procedure times (median, 420 minutes with injury versus 248 without; p < 0.001). Operative mortality occurred in 18 patients and was associated with sternotomy number and procedure time (p < 0.001), but not reentry injury (p = 0.2). CONCLUSIONS Risk of reentry injury during repeat sternotomy for congenital heart disease is low. Increasing sternotomy number and the presence of a right ventricle-pulmonary artery conduit are risk factors for reentry injury. However, reentry injury is not associated with increased risk of operative mortality.

Feeding Difficulty in Newborns Following Congenital Heart Surgery

OBJECTIVE Following neonatal congenital heart surgery, one of the factors impacting patient recovery is feeding difficulty. The aim of this study is to identify the risk factors. METHODS Patients who underwent surgery for congenital heart defects within the first 15 days of life were reviewed. Endpoints for feeding difficulty included: (1) a prolonged time to reach goal feeds; (2) a prolonged transition to oral feeds requiring tube feeds at discharge; and (3) additional procedures to facilitate feeding. Preoperative, operative, and postoperative data were examined to identify risk factors for feeding difficulty. RESULTS A total of 83 records were reviewed and showed the following feeding difficulties: 9 patients (10.8%) had a prolonged time to reach goal feeds, 37 (44.6%) had a prolonged time to transition to oral feeds, and 8 (9.6%) required subsequent procedures to facilitate feeding. Significant risk factors for all endpoints included an increased risk adjusted congenital heart surgery (RACHS) score and prolonged intubation. Significant risk factors for individual endpoints included return to the intensive care unit with an open chest for endpoint 1, and a single functional ventricle and the presence of a shunt for endpoint 3. The remaining factors (gestational age, weight at the time of surgery, being intubated at the time of surgery, underlying disease, utilization and time of cardiopulmonary bypass, utilization of trans-esophageal echocardiography, and surgical proximity to the aortic arch) had no significant effect on postoperative feeding. CONCLUSIONS Feeding difficulties are not uncommon following surgery for the correction of congenital heart defects, especially in the neonate. The most important risk factors appear to be an increased RACHS score and prolonged postoperative intubation. Hopefully, by defining the risk factors, proactive management strategies can be developed to minimize these problems following neonatal congenital heart surgery.

Force frequency relationship of the human ventricle increases during early postnatal development.

Understanding developmental changes in contractility is critical to improving therapies for young cardiac patients. Isometric developed force was measured in human ventricular muscle strips from two age groups: newborns (<2 wk) and infants (3–14 mo) undergoing repair for congenital heart defects. Muscle strips were paced at several cycle lengths (CLs) to determine the force frequency response (FFR). Changes in Na/Ca exchanger (NCX), sarcoplasmic reticulum Ca-ATPase (SERCA), and phospholamban (PLB) were characterized. At CL 2000 ms, developed force was similar in the two groups. Decreasing CL increased developed force in the infant group to 131 ± 8% (CL 1000 ms) and 157 ± 18% (CL 500 ms) demonstrating a positive FFR. The FFR in the newborn group was flat. NCX mRNA and protein levels were significantly larger in the newborn than infant group whereas SERCA levels were unchanged. PLB mRNA levels and PLB/SERCA ratio increased with age. Immunostaining for NCX in isolated newborn cells showed peripheral staining. In infant cells, NCX was also found in T-tubules. SERCA staining was regular and striated in both groups. This study shows for the first time that the newborn human ventricle has a flat FFR, which increases with age and may be caused by developmental changes in calcium handling.

Low-weight infants are at increased mortality risk after palliative or corrective cardiac surgery.

BACKGROUND Low weight is an established risk factor for mortality after congenital cardiac surgery. Given the advances in the care of neonates and infants after surgery, we sought to examine the effect of low weight on outcomes in the current era. METHODS From 2002 to 2012, 2051 infants aged 90 days or less underwent cardiac surgery including 534 (26.0%) with single-ventricle pathology. Regression models examined the effect of low weight (≤ 2.5 kg; n = 274, 13.4%) on early and late outcomes. RESULTS Overall, the incidence of prematurity, associated chromosomal/extracardiac abnormalities was higher in infants who weighed 2.5 kg or less than in those who weighed more than 2.5 kg; the incidence of single-ventricle pathology was comparable between the 2 groups. In addition, infants who weighed 2.5 kg or less underwent more palliation and had a higher proportion of STAT (Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery) risk category 4 and 5 procedures. Adjusted regression models showed that low weight (≤ 2.5 kg) did not increase unplanned reoperation (odds ratio [OR], 0.90; 95% confidence interval [CI], 0.48-1.67; P = .73) or extracorporeal membrane oxygenation requirement (OR, 1.23; 95% CI, 0.68-2.22; P = .49), however it was associated with significant increase in hospital mortality (OR, 2.15; 95% CI, 1.33-3.50; P = .002). In addition, there was a significant association between low weight and increased duration of postoperative mechanical ventilation and intensive care unit and hospital stays. Adjusted hazard analysis showed that weight equal to or less than 2.5 kg was associated with diminished late survival (hazard ratio, 1.89; 95% CI, 1.39-2.55; P < .001) and that was evident in all patients subgroups (P < .001 for all). CONCLUSIONS In a large single-center series, low weight continues to be associated with increased early mortality risk and resource utilization after palliative and corrective cardiac surgery. The hazard of death in low-weight patients continues beyond the perioperative period for at least 1 year before normalizing. Strategies to improve outcomes for this high-risk population must address perioperative care, outpatient surveillance, and management.

Adult congenital heart surgery: adult or pediatric facility? Adult or pediatric surgeon?

BACKGROUND One of the current controversies in the field of adult congenital heart disease is whether patients should be cared for at an adult or pediatric facility and by an adult or pediatric heart surgeon. After transitioning our program from the childrens hospital to the adult hospital, we analyzed our experience with each system. METHODS Between 2000 and 2007, 303 operations were performed on adults (age >or= 18 years) with congenital heart disease. One hundred eighty-five operations were performed in an adult hospital and 118 in a pediatric hospital. Forty-six operations were performed by an adult heart surgeon and 257 by a congenital heart surgeon. RESULTS Mean age, coexisting medical problems, and preoperative risk factors were higher in both the adult hospital group and adult surgeon group compared with the respective pediatric groups. Mortality was similar at the adult and pediatric hospitals (4.3% versus 5.1%), but was markedly higher in the adult surgeon group compared with the pediatric surgeon group (15.2% versus 2.7%; p = 0.0008). By multivariate analysis, risk factors for mortality included older age at the time of surgery (p = 0.028), surgery performed at a childrens hospital (p = 0.013), and surgery performed by an adult heart surgeon (p = 0.0004). CONCLUSIONS Congenital heart surgery can be performed in adults with reasonable morbidity and mortality. Caring for an anticipated aging adult congenital population with increasingly numerous coexisting medical problems and risk factors is best facilitated in an adult hospital setting. Also, when surgery becomes necessary, these adult patients are best served by a congenital heart surgeon.