Quality of life and metrics of achievement in long-term adult survivors of pediatric heart transplant.
2015; 19 (1): 76-81
Ventricular lead redundancy to prevent cardiovascular events and sudden death from lead fracture in pacemaker-dependent children
2015; 12 (1): 111-116
Many children who undergo heart transplantation will survive into adulthood. We sought to examine the QOL and capacity for achievement in long-term adult survivors of pediatric heart transplantation. Adults >18 yr of age who received transplants as children (≤18 yr old) and had survived for at least 10 yr post-transplant completed two self-report questionnaires: (i) Ferrans & Powers QLI, in which life satisfaction is reported as an overall score and in four subscale domains and is then indexed from 0 (very dissatisfied) to 1 (very satisfied); and (ii) a "Metrics of Life Achievement" questionnaire regarding income, education, relationships, housing status, and access to health care. A total of 20 subjects completed the survey. The overall mean QLI score was 0.77 ± 0.16. Subjects were most satisfied in the family domain (0.84 ± 0.21) and least satisfied in the psychological/spiritual domain (0.7 ± 0.28). Satisfaction in the domains of health/functioning and socioeconomic were intermediate at 0.78 and 0.76, respectively. Most respondents had graduated from high school, reported a median annual income >$50 000/yr, and lived independently. Adult survivors of pediatric heart transplant report a good QOL and demonstrate the ability to obtain an education, work, and live independently.
View details for DOI 10.1111/petr.12384
View details for PubMedID 25388808
HLA desensitization with bortezomib in a highly sensitized pediatric patient
2014; 18 (8): E280-E282
Molecular diagnosis of long QT syndrome at 10 days of life by rapid whole genome sequencing
2014; 11 (10): 1707-1713
Tetralogy of Fallot: aorto-pulmonary collaterals and pulmonary arteries have distinctly different transcriptomes
2014; 76 (4): 341-346
An inpatient rehabilitation program utilizing standardized care pathways after paracorporeal ventricular assist device placement in children
JOURNAL OF HEART AND LUNG TRANSPLANTATION
2014; 33 (6): 587-592
Children requiring a permanent epicardial pacemaker(PM) traditionally have a single lead placed on the right ventricle. Lead failure in pacemaker dependent(PMD) children, however, can result in cardiovascular events(CVE) and death.To determine if redundant ventricular lead systems(RVLS) can safeguard against CVE and death in PMD children.Single-center study of PMD patients undergoing placement of RVLS from 2002-2013. Patients ≤ 21 years of age who were PMD were included. Patients with biventricular systems(BiV) systems placed for standard resynchronization indications were excluded. RVLS patients were compared to PMD patients with only a single pacing lead on the ventricle(SiV).769 patients underwent PM/ICD placement with 76 BiV implants and there were 49 PMD patients(6%). 13 patients underwent implantation of a RVLS. There was no difference between the RVLS group(n=13) and SiV PMD control group(n=24) with regard to age(RVLS 9.5±5.8 vs. SiV 9.4±6.7 years; p=0.52), weight(RVLS 38.2±32.6 vs. SiV 35.2±29.3 kg; p=0.62), indication for pacing, procedural complications or time to follow-up. There were 2 lead fractures (17%) in the RVLS group(mean follow-up 3.8±2.9 years) with no deaths or presentations with CVE. The SiV control group had 3 lead fractures (13%)(mean follow-up 2.8±2.9 years), with no deaths, but all 3 patients presented with CVE and required emergent PM placement.RVLS systems should be considered in children who are PMD and require permanent epicardial pacing. BiV pacing and RVLS may decrease the risk of CVEs in the event of lead failure in PMD patients.
View details for DOI 10.1016/j.hrthm2014.09.056
View details for Web of Science ID 000346857100022
A child with purulent pericarditis and Streptococcus intermedius in the presence of a pericardial teratoma: an unusual presentation.
journal of thoracic and cardiovascular surgery
2014; 147 (3): e23-4
Successful Bridge to Transplant with a Continuous Flow Ventricular Assist Device in a Single Ventricle Patient with an Aortopulmonary Shunt
2014; 60 (1): 119-121
Structured rehabilitation programs in adults after ventricular assist device (VAD) placement result in improvements in physical function and exercise capacity, and have been shown to improve survival and accelerate post-transplant recovery. The objective of this study was to determine the safety and feasibility of an acute inpatient rehabilitation program for children utilizing standardized, age-appropriate, family-centered care pathways after paracorporeal VAD placement in both the ICU and acute-care inpatient settings.Between November 12, 2010 and March 15, 2013, 17 patients were referred to therapy after VAD implantation, 14 of whom were medically stable enough to participate. Beginning in the ICU, a structured physical and occupational therapy program was implemented utilizing novel age-appropriate, standardized care pathways for infants (age <1 year) and children (age 1 to 12 years). The infant and child pathways consisted of 8 and 10 goals, respectively. Retrospective review was conducted to ascertain the number of phases achieved per patient. Adverse events, defined as bleeding, physiologic instability, stroke, or device disruption during therapy, were also analyzed.The median age was 1.1 (range 0.5 to 14.4) years in the 14 patients considered medically stable enough to participate in rehabilitation. Nine of them were female. Eight patients participated in the infant standardized care pathway (SCP) and 6 participated in the child SCP. Seven patients were on biventricular support. Twelve patients were transplanted and survived. Two patients died while awaiting transplantation. There were 1,473 total days on the VAD (range 40 to 229 days). The median time to extubation was 2 days (range 1 to 8) and the median ICU stay was 6.5 days (range 3 to 152). Eleven patients achieved all goals of the SCP, including all of the patients in the child group. For the infant group, 5 patients achieved all goals of the SCP (range 5 to 8), and all but 1 patient achieved at least 7 goals of the SCP. There were no adverse events related to therapy.Standardized, family-centered inpatient rehabilitation care paths are safe for infants and children after paracorporeal device placement. Structured rehabilitation goals can be achieved by the majority of pediatric patients during VAD support. Early mobilization and inpatient rehabilitation in this cohort promotes normalization of function while awaiting cardiac transplantation.
View details for DOI 10.1016/j.healun.2013.12.009
View details for Web of Science ID 000336637100005
Lower socioeconomic status is associated with worse outcomes after both listing and transplanting children with heart failure
2013; 17 (6): 573-581
Ventricular assist devices are frequently used to bridge pediatric patients to cardiac transplantation; however, experience in single ventricle patients with aortopulmonary shunts remains limited. This case report addresses the challenge of balancing pulmonary and systemic circulation with a focus on the role of continuous versus pulsatile ventricular assist device support.
View details for DOI 10.1097/MAT.0000000000000007
View details for Web of Science ID 000329368600021
Fetal cardiac intervention: Improved results of fetal cardiac bypass in immature fetuses using the TinyPump device
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
2013; 145 (6): 1460-1464
The relationship between SES and outcomes surrounding pediatric cardiac transplantation is complex and influenced by recipient race. Broad-based studies of SES have not been performed. A retrospective review of all 5125 primary pediatric heart transplants performed in the United States between 2000 and 2011. Patients were stratified by SES based on zip code of residence and U.S. census data (low SES: 1637; mid-SES: 2253; high SES: 1235). Survival following listing and transplantation was compared across strata. Risk-adjusted long-term mortality on the waitlist was higher among low SES patients (hazard 1.32, CI 1.07-1.63). The relationship between SES and outcomes varied by race. Early risk-adjusted post-transplant outcomes were worst among high SES patients (10.8% vs. low SES: 8.9%, p < 0.05). The incidence of non-compliance was higher among low SES patients (p < 0.0001). Long-term risk-adjusted patient survival was poorer among low (hazard 1.41, CI 1.10-1.80) and mid-SES (1.29, 1.04-1.59) groups. Low SES is associated with worse outcomes on both the waitlist and late following transplantation. Higher SES patients had more complex transplants with higher early mortality. Further research should be directed at identifying and addressing underlying causal factors for these disparities.
View details for DOI 10.1111/petr.12117
View details for Web of Science ID 000322317700015
View details for PubMedID 23834560
Intermediate-term outcomes after combined heart-liver transplantation in children with a univentricular heart
JOURNAL OF HEART AND LUNG TRANSPLANTATION
2013; 32 (3): 368-370
Fetal cardiac surgery is a potential innovative treatment for certain congenital heart defects that have significant mortality and morbidity in utero or after birth, but it has been limited by placental dysfunction after fetal cardiac bypass. We have used the TinyPump device for fetal cardiac bypass in sheep fetuses at 90 to 110 days gestation.Ten mixed-breed pregnant ewes were used over a period of 6 months, and 10 fetuses were placed on bypass for 30 minutes. Five fetuses with a mean gestational age of 104 ± 4.5 days and mean weight of 1.4 ± 0.4 kg were placed on bypass using the TinyPump device, and 5 fetuses with a mean gestational age of 119 ± 4.5 days and mean weight of 3.4 ± 0.4 kg were placed on bypass using the roller head pump. The fetuses were monitored for up to 3 hours after bypass or until earlier demise.Progressive respiratory and metabolic acidosis developed in all fetuses. The TinyPump group had a lower gestational age and weight compared with the roller head pump group. However, the rate of postbypass deterioration in the TinyPump group, as measured with blood gases, was noted to be significantly slower compared with the roller head pump group.We demonstrate the feasibility of the TinyPump device for fetal cardiac bypass in a fetal sheep model. The TinyPump group showed improved results compared with the roller head group despite more immature fetuses. The TinyPump device seems to be a promising device for future studies of fetal cardiac bypass in immature fetal sheep and in primates.
View details for DOI 10.1016/j.jtcvs.2012.08.014
View details for Web of Science ID 000319066300024
Midterm Results of the Modified Ross/Konno Procedure in Neonates and Infants
ANNALS OF THORACIC SURGERY
2012; 94 (1): 156-163
For patients with end-stage hepatic failure secondary to failing hemodynamics, combined heart-liver transplant (H-LT) remains the only option for long-term survival. We report a series of three pediatric patients who successfully underwent orthotopic H-LT for failed single-ventricle palliation. All three patients are currently living, now two, three, and five years post-transplant, and remain completely free of cardiac cellular allograft rejection despite reduced immunosuppression protocols. One patient, however, did develop acute antibody-mediated rejection in the immediate post-transplant period, suggesting that this protective effect may be less effective in attenuating humoral mechanisms of rejection.
View details for DOI 10.1016/j.healun.2012.11.023
View details for Web of Science ID 000315664600014
Pediatric combined heart-liver transplantation performed en bloc: A single-center experience
2012; 16 (4): 392-397
The management of congenital aortic stenosis in neonates and infants continues to be a surgical challenge. We have performed the modified Ross-Konno procedure for patients who have severe aortic insufficiency or significant residual stenosis after balloon aortic dilation. The midterm results of this procedure were evaluated in this subset of patients.Between 1994 and 2010, a total of 24 patients younger than 1 year of age underwent the modified Ross-Konno procedure. The diagnoses were aortic stenosis with or without subaortic stenosis (n = 16), Shone's complex (n = 7), and interrupted aortic arch with subaortic stenosis (n = 1). The aortic root was replaced with a pulmonary autograft, and the left ventricular outflow tract (LVOT) was enlarged with a right ventricular infundibular free wall muscular extension harvested with the autograft.Age at operation ranged from 1 to 236 days (median 28 days). The median follow-up period was 81 months (range 1-173 months). There was 1 early death and no late mortality. Overall the 1-, 2-, and 5-year survival rate was 95% ± 4.5%. Freedom from aortic stenosis was 94.7% ± 5.1% at 1, 2, and 5 years. Less than mild aortic insufficiency was 93.3% ± 6.4% at 2 years, and 74.7% ± 12.9% at 5 years. In total, 23 reoperations and reinterventions were performed; 14 were allograft conduit replacements. Two patients required aortic valve plasty. None required valve replacement. The reintervention-free rate was 64.6% ± 10.8% at 2 years and 36.9% ± 11.3% at 5 years.Pulmonary autografts demonstrated good durability with low mortality and morbidity. This study shows that the modified Ross-Konno procedure can be a practical choice in selective cases for complex LVOT stenosis in neonates and infants.
View details for DOI 10.1016/j.athoracsur.2012.03.007
View details for Web of Science ID 000305801600033
View details for PubMedID 22626750
Use of the Impella 5.0 as a bridge from ECMO to implantation of the HeartMate II left ventricular assist device in a pediatric patient
2012; 16 (2): 205-206
Recovery During Mid-Term Mechanical Support of Fontan Circulation in Sheep
2009; 55 (4): 406-411
Pediatric CHLT is rarely performed in transplant centers and even fewer are performed en bloc. In the hands of an experienced surgeon with the appropriate patient selection, CHLT performed en bloc may have several operative and immunologic benefits, thereby resulting in improved outcomes for the transplant recipient. A single-institutional, retrospective review from 1/1/06 to 12/31/10 was conducted. Three pediatric patients with end-stage heart and liver disease who were considered low immunologic risk were included. All were managed by the same surgeon with a herein-described CHLT donor and recipient operation. Data were collected on patient and graft survival, rejection episodes, infectious complications, operative time, intraoperative transfusion requirements, and immunosuppression regimens. One-yr patient and graft survival rates were 100%. No patients experienced antibody-mediated or cell-mediated rejection. No patients had postoperative infections, and all patients were free of opportunistic infections at one-yr post-transplant. All patients were maintained safely on steroid-free immunosuppression. There were no intraoperative complications. In pediatric end-stage heart and liver disease patients with low immunologic risk, it is reasonable to proceed with en bloc CHLT so long as there is an experienced surgeon to perform the case. This offers operative and immunologic advantages to the recipient while maintaining equivalent, if not improved, recipient and graft outcomes.
View details for DOI 10.1111/j.1399-3046.2012.01695.x
View details for Web of Science ID 000303998800024
View details for PubMedID 22583978
"Arteries within the artery" after Kawasaki disease - A lotus root appearance by intravascular ultrasound
2002; 106 (7): 887-887
Total cavopulmonary connection (CPC) has a significant incidence of late failure due to increased systemic venous pressure and low cardiac output. Mechanical support could prevent failure by correcting hemodynamics. We established a model of inferior CPC using an axial flow pump (Thoratec HeartMate II, Thoratec Corp. Pleasanton, CA) in a group of ten 47-57 kg sheep and assessed hemodynamics and metabolism as a potential chronic treatment option for failed Fontan circulation. After pilot studies (n = 7), three animals underwent pump-supported inferior CPC to assess hemodynamic and metabolic responses. Pump inflow was connected to the inferior vena cava (IVC) and outflow to the main pulmonary artery. The IVC was ligated at the right atrium. Hemodynamic and biochemical parameters were recorded over four days. The first seven animals died from pump-related causes (graft kinking, three; pump thrombosis, one) or other causes (GI bleeding, one; suspected stroke, two). The subsequent three animals were electively euthanized on postoperative day four due to IRB requirements. Over the four day postoperative period, pump flow was 3.43 +/- 0.62 L/min and IVC pressure 4.05 +/- 3.21 mm Hg (mean +/- SD). Lactate levels remained normal. Low pressure and high-volume IVC flow was sustained by mechanical support. We will next attempt chronic pump implantation.
View details for DOI 10.1097/MAT.0b013e3181a0a570
View details for Web of Science ID 000267559100016
View details for PubMedID 19471161