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1.
J Pediatr ; 268: 113955, 2024 May.
Article in English | MEDLINE | ID: mdl-38340889

ABSTRACT

OBJECTIVE: To assess rates of cardiac surgery and the clinical and demographic features that influence surgical vs nonsurgical treatment of congenital heart disease (CHD) in patients with trisomy 13 (T13) and trisomy 18 (T18) in the United States. STUDY DESIGN: A retrospective study was performed using the Pediatric Health Information System. All hospital admissions of children (<18 years of age) with T13 and T18 in the United States were identified from 2003 through 2022. International Classifications of Disease (ICD) codes were used to identify presence of CHD, extracardiac comorbidities/malformations, and performance of cardiac surgery. RESULTS: Seven thousand one hundred thirteen patients were identified. CHD was present in 62% (1625/2610) of patients with T13 and 73% (3288/4503) of patients with T18. The most common CHD morphologies were isolated atrial/ventricular septal defects (T13 40%, T18 42%) and aortic hypoplasia/coarctation (T13 21%, T18 23%). Single-ventricle morphologies comprised 6% (100/1625) of the T13 and 5% (167/3288) of the T18 CHD cohorts. Surgery was performed in 12% of patients with T13 plus CHD and 17% of patients with T18 plus CHD. For all cardiac diagnoses, <50% of patients received surgery. Nonsurgical patients were more likely to be born prematurely (P < .05 for T13 and T18). The number of extracardiac comorbidities was similar between surgical/nonsurgical patients with T13 (median 2 vs 2, P = .215) and greater in surgical vs nonsurgical patients with T18 (median 3 vs 2, P < .001). Hospital mortality was <10% for both surgical cohorts. CONCLUSIONS: Patients with T13 or T18 and CHD receive surgical palliation, but at a low prevalence (≤17%) nationally. Given operative mortality <10%, opportunity exists perhaps for quality improvement in the performance of cardiac surgery for these vulnerable patient populations.


Subject(s)
Cardiac Surgical Procedures , Heart Defects, Congenital , Trisomy 13 Syndrome , Trisomy 18 Syndrome , Humans , Retrospective Studies , United States/epidemiology , Female , Male , Heart Defects, Congenital/surgery , Heart Defects, Congenital/epidemiology , Cardiac Surgical Procedures/methods , Trisomy 18 Syndrome/surgery , Infant , Child, Preschool , Infant, Newborn , Child , Adolescent , Hospitalization/statistics & numerical data , Chromosomes, Human, Pair 18 , Trisomy , Chromosome Disorders/epidemiology
2.
J Thorac Cardiovasc Surg ; 167(5): 1556-1563.e2, 2024 May.
Article in English | MEDLINE | ID: mdl-37414356

ABSTRACT

OBJECTIVES: Ventricular assist devices (VADs) are associated with a mortality benefit in children. Database-driven analyses have associated VADs with reduction of modifiable risk factors (MRFs), but validation with institutional data is required. The authors studied MRF reduction on VAD and the influence of persistent MRFs on survival after heart transplant. METHODS: All patients at the authors' institution requiring a VAD at transplant (2011-2022) were retrospectively identified. MRFs included renal dysfunction (estimated glomerular filtration rate <60 mL/min/1.73 m2), hepatic dysfunction (total bilirubin ≥1.2 mg/dL), total parenteral nutrition dependence, sedatives, paralytics, inotropes, and mechanical ventilation. RESULTS: Thirty-nine patients were identified. At time of VAD implantation, 18 patients had ≥3 MRFs, 21 had 1 to 2 MRFs, and 0 had 0 MRFs. At time of transplant, 6 patients had ≥3 MRFs, 17 had 1 to 2 MRFs, and 16 had 0 MRFs. Hospital mortality occurred in 50% (3 out of 6) patients with ≥3 MRFs at transplant vs 0% of patients with 1 to 2 and 0 MRFs (P = .01 for ≥3 vs 1-2 and 0 MRFs). MRFs independently associated with hospital mortality included paralytics (1.76 [range, 1.32-2.30]), ventilator (1.59 [range, 1.28-1.97]), total parenteral nutrition dependence (1.49 [range, 1.07-2.07]), and renal dysfunction (1.31 [range, 1.02-1.67]). Two late mortalities occurred (3.6 and 5.7 y), both in patients with 1 to 2 MRFs at transplant. Overall posttransplant survival was significantly worse for ≥3 versus 0 MRFs (P = .006) but comparable between other cohorts (P > .1). CONCLUSIONS: VADs are associated with MRF reduction in children, yet those with persistent MRFs at transplant experience a high burden of mortality. Transplanting VAD patients with ≥3 MRFs may not be prudent. Time should be given on VAD support to achieve aggressive pre-transplant optimization of MRFs.


Subject(s)
Heart Failure , Heart Transplantation , Heart-Assist Devices , Kidney Diseases , Child , Humans , Heart-Assist Devices/adverse effects , Retrospective Studies , Heart Transplantation/adverse effects , Risk Factors , Heart Failure/diagnosis , Heart Failure/surgery , Treatment Outcome
3.
J Thorac Cardiovasc Surg ; 167(2): 422-430, 2024 Feb.
Article in English | MEDLINE | ID: mdl-37385525

ABSTRACT

OBJECTIVE: To characterize national experience with surgical aortic valve repair in pediatric patients. METHODS: Patients in the Pediatric Health Information System database aged 17 years or younger with International Statistical Classification of Diseases and Related Health Problems codes for open aortic valve repair from 2003 to 2022 were included (n = 5582). Outcomes of reintervention during index admission (repeat repair, n = 54; replacement, n = 48; and endovascular intervention, n = 1), readmission (n = 2176), and in-hospital mortality (n = 178) were compared. A logistic regression was performed for in-hospital mortality. RESULTS: One-quarter (26%) of patients were infants. The majority (61%) were boys. Heart failure was present in 16% of patients, congenital heart disease in 73%, and rheumatic disease in 4%. Valve disease was insufficiency in 22% of patients, stenosis in 29%, and mixed in 15%. The highest quartile of centers by volume (median, 101 cases; interquartile range, 55-155 cases) performed half (n = 2768) of cases. Infants had the highest prevalence of reintervention (3%; P < .001), readmission (53%; P < .001), and in-hospital mortality (10%; P < .001). Previously hospitalized patients (median, 6 days; interquartile range, 4-13 days) were at higher risk for reintervention (4%; P < .001), readmission (55%; P < .001), and in-hospital mortality (11%; P < .001), as were patients with heart failure (reintervention [6%; P < .001], readmission [42%; P = .050], and in-hospital mortality [10%; P < .001]). Stenosis was associated with reduced reintervention (1%; P < .001) and readmission (35%; P = .002). The median number of readmissions was 1 (range, 0-6) and time to readmission was 28 days (interquartile range, 7-125 days). A regression of in-hospital mortality identified heart failure (odds ratio, 3.05; 95% CI, 1.59-5.49), inpatient status (odds ratio, 2.40; 95% CI, 1.19-4.82), and infancy (odds ratio, 5.70; 95% CI, 2.60-12.46) as significant. CONCLUSIONS: The Pediatric Health Information System cohort demonstrated success with aortic valve repair; however, early mortality remains high in infants, hospitalized patients, and patients with heart failure.


Subject(s)
Aortic Valve Stenosis , Health Information Systems , Heart Failure , Transcatheter Aortic Valve Replacement , Male , Infant , Humans , Child , Female , Aortic Valve/surgery , Aortic Valve/abnormalities , Aortic Valve Stenosis/surgery , Constriction, Pathologic/surgery , Treatment Outcome , Heart Failure/surgery , Patient Readmission , Risk Factors
4.
Transplantation ; 108(3): 750-758, 2024 Mar 01.
Article in English | MEDLINE | ID: mdl-38062571

ABSTRACT

BACKGROUND: With rates of potential donor heart discard as high as 66% nationally, quality improvement efforts must seek to optimize donor utilization. Whether the timing of donor brain death declaration (BDD) influences organ acceptance is understudied. The authors sought to characterize the impacts of time between donor hospital admission and BDD on heart utilization and posttransplant outcomes. METHODS: All potential heart donors and recipients in the United Network for Organ Sharing database were identified (2006-2021). Admission-to-BDD cohorts were: 1 to 2 d (n = 52 469), 3 to 4 d (n = 44 033), 5 to 7 d (n = 24 509), and 8 to 10 d (n = 8576). Donor clinical characteristics were compared between cohorts, and donor acceptance was assessed using multivariable binary logistic regression. Recipient posttransplant survival was assessed with the Kaplan-Meier method. RESULTS: Donor demographics and comorbidity profiles (diabetes and hypertension) were comparable across cohorts. Anoxia/overdose deaths were more common (10% > 21% > 24% > 18%, respectively) and cardiopulmonary resuscitation requirements were higher (37% > 52% > 58% > 47%) when BDD occurred longer after admission. Renal dysfunction (44% > 44% > 35% > 29%) and inotrope requirements (52% > 25% > 36% > 29%) were lower in the later BDD cohorts. Proportions of hepatic dysfunction (18%-21%) and left ventricular ejection fraction <50% (13%-16%) were clinically equivalent. Donor acceptance differed by admission-to-BDD cohort (36% [1-2 d], 34% [3-4 d], 30% [5-7 d], and 28% [8-10 d]). Admission-to-BDD >4 d was independently associated with lower odds of acceptance on multivariable analysis (odds ratio 0.79, P < 0.001). Recipients experienced equivalent posttransplant survival for all donor admission-to-BDD cohorts ( P = 0.999 adults and P = 0.260 pediatrics). CONCLUSIONS: Heart donors with later BDD were disproportionately discarded despite similar-to-favorable overall clinical profiles, resulting in nearly 3000 fewer transplants during the study. Increased utilization of donors with later BDD and "high-risk" characteristics (eg, anoxia/overdose, cardiopulmonary resuscitation requirement) can improve rates of transplantation without compromising outcomes.


Subject(s)
Drug Overdose , Heart Transplantation , Tissue and Organ Procurement , Adult , Humans , Child , Tissue Donors , Stroke Volume , Brain Death , Ventricular Function, Left , Hypoxia , Retrospective Studies
5.
J Heart Lung Transplant ; 43(3): 461-470, 2024 Mar.
Article in English | MEDLINE | ID: mdl-37863451

ABSTRACT

BACKGROUND: Recently, several centers in the United States have begun performing donation after circulatory death (DCD) heart transplants (HTs) in adults. We sought to characterize the recent use of DCD HT, waitlist time, and outcomes compared to donation after brain death (DBD). METHODS: Using the United Network for Organ Sharing database, 10,402 adult (aged >18 years) HT recipients from January 2019 to June 2022 were identified: 425 (4%) were DCD and 9,977 (96%) were DBD recipients. Posttransplant outcomes in matched and unmatched cohorts and waitlist times were compared between groups. RESULTS: DCD and DBD recipients had similar age (57 years for both, p = 0.791). DCD recipients were more likely White (67% vs 60%, p = 0.002), on left ventricular assist device (LVAD; 40% vs 32%, p < 0.001), and listed as status 4 to 6 (60% vs 24%, p < 0.001); however, less likely to require inotropes (22% vs 40%, p < 0.001) and preoperative extracorporeal membrane oxygenation (0.9% vs 6%, p < 0.001). DCD donors were younger (29 vs 32 years, p < 0.001) and had less renal dysfunction (15% vs 39%, p < 0.001), diabetes (1.9% vs 3.8%, p = 0.050), or hypertension (9.9% vs 16%, p = 0.001). In matched and unmatched cohorts, early survival was similar (p = 0.22). Adjusted waitlist time was shorter in DCD group (21 vs 31 days, p < 0.001) compared to DBD cohort and 5-fold shorter (DCD: 22 days vs DBD: 115 days, p < 0.001) for candidates in status 4 to 6, which was 60% of DCD cohort. CONCLUSIONS: The community is using DCD mostly for those recipients who are expected to have extended waitlist times (e.g., durable LVADs, status >4). DCD recipients had similar posttransplant early survival and shorter adjusted waitlist time compared to DBD group. Given this early success, efforts should be made to expand the donor pool using DCD, especially for traditionally disadvantaged recipients on the waitlist.


Subject(s)
Heart Transplantation , Tissue and Organ Procurement , Adult , Humans , Tissue Donors , Brain Death , Time Factors , Graft Survival , Retrospective Studies , Death
6.
Ann Thorac Surg ; 117(3): 611-618, 2024 Mar.
Article in English | MEDLINE | ID: mdl-37271442

ABSTRACT

BACKGROUND: In pediatric heart transplantation, surgeons historically avoided donors requiring cardiopulmonary resuscitation (CPR), despite evidence that donor CPR does not change posttransplant survival (PTS). This study sought to determine whether CPR duration affects PTS. METHODS: All potential brain-dead donors aged <40 years from 2001 to 2021 consented for heart procurement were identified in the United Network for Organ Sharing database (n = 54,671). Organ acceptance was compared by CPR administration and duration. All recipients aged <18 years with donor CPR data were then identified (n = 5680). Survival analyses were conducted using increasing CPR duration as a cut point to identify the shortest duration beyond which PTS worsened. Additional analyses were performed with multivariable and cubic spline regression. RESULTS: Fifty-one percent of donors (28,012 of 54,671) received CPR. Donor acceptance was lower after CPR (54% vs 66%; P < .001) and across successive quartiles of CPR duration (P < .001). Of the transplant recipients, 48% (2753 of 5680) belonged to the no-CPR group, and 52% (2927 of 5680) belonged to the CPR group. Kaplan-Meier analyses of CPR duration attained significance at 55 minutes, after which PTS worsened (11.1 years vs 9.2 years; P = .025). There was no survival difference between the CPR ≤55 minutes group and the no-CPR group (11.1 years vs 11.2 years; P = .571). A cubic spline regression model confirmed that PTS worsened at more than 55 minutes of CPR. A Cox regression demonstrated that CPR >55 minutes predicted worsened PTS relative to no CPR (HR, 1.51; P = .007) but CPR ≤55 minutes did not (HR, 1.01; P = .864). CONCLUSIONS: Donor CPR decreases organ acceptance for transplantation; however, shorter durations (≤55 minutes) had equivalent PTS when controlling for other risk factors.


Subject(s)
Cardiopulmonary Resuscitation , Heart Transplantation , Humans , Child , Cardiopulmonary Resuscitation/adverse effects , Tissue Donors , Time Factors , Survival Analysis , Graft Survival , Retrospective Studies , Treatment Outcome
7.
Perfusion ; : 2676591231220816, 2023 Dec 05.
Article in English | MEDLINE | ID: mdl-38053305

ABSTRACT

INTRODUCTION: In children with myocarditis or dilated cardiomyopathy (DCM) on extracorporeal membrane oxygenation (ECMO) for cardiogenic shock, it is often necessary to decompress the left heart to minimize distension and promote myocardial recovery. We compare outcomes in those who underwent balloon atrial septostomy (BAS) versus direct left atrial (LA) drainage for left heart decompression in this population. METHODS: Retrospective study of the Extracorporeal Life Support Organization (ELSO) multicenter registry of patients ≤ 18 years with myocarditis or DCM on ECMO who underwent LA decompression. Descriptive and univariate statistics assessed association of patient factors with decompression type. Multivariable logistic regression sought independent associations with outcomes. RESULTS: 369 pediatric ECMO runs were identified. 52% myocarditis, 48% DCM, overall survival 74%. 65% underwent BAS and 35% LA drainage. Patient demographics including age, weight, gender, race/ethnicity, diagnosis, pre-ECMO pH, mean airway pressure, and arrest status were similar. 89% in the BAS group were peripherally cannulated onto ECMO, versus 3% in the LA drainage group (p < .001). On multivariable analysis, LA drainage (OR 3.96; 95% CI, 1.47-10.711; p = .007), renal complication (OR 2.37; 95% CI, 1.41-4.01; p = .001), cardiac complication (OR 3.14; 95% CI, 1.70-5.82; p < .001), and non-white race/ethnicity (OR 1.75; 95% CI, 1.04-2.94; p = .035) were associated with greater odds of mortality. There was a trend toward more episodes of pulmonary hemorrhage in BAS (n = 17) versus LA drainage group (n = 3), p = .08. Comparing only those with central cannulation, LA drainage group was more likely to be discontinued from ECMO due to recovery (72%) versus the BAS group (48%), p = .032. CONCLUSIONS: In children with myocarditis or DCM, there was a three times greater likelihood for mortality with LA drainage versus BAS for LA decompression. When adjusted for central cannulation groups only, there was better recovery in the LA drainage group and no difference in mortality. Further prospective evaluation is warranted.

8.
World J Pediatr Congenit Heart Surg ; 14(6): 758-760, 2023 Nov.
Article in English | MEDLINE | ID: mdl-37710988

ABSTRACT

Tricuspid valve (TV) dysplasia is a rare congenital defect that, in severe cases, can cause heart failure necessitating surgical correction. TV replacement options are limited in neonates since few commercial valves are available and the rates of failure and postoperative mortality are high. The authors report a neonate with TV dysplasia who underwent replacement with a hand-constructed tube valve using small intestinal submucosal extracellular matrix, which functioned well for >5 years.


Subject(s)
Heart Defects, Congenital , Heart Valve Diseases , Heart Valve Prosthesis Implantation , Tricuspid Valve Insufficiency , Infant, Newborn , Humans , Tricuspid Valve/surgery , Tricuspid Valve Insufficiency/surgery , Follow-Up Studies , Treatment Outcome , Heart Valve Diseases/surgery , Heart Defects, Congenital/surgery , Retrospective Studies
9.
Article in English | MEDLINE | ID: mdl-37774778

ABSTRACT

OBJECTIVE: Many pediatric Fontan patients require heart transplant, but this cohort is understudied given the difficulty in identifying these patients in national registries. We sought to characterize survival post-transplant in a large cohort of pediatric patients undergoing the Fontan. METHODS: The United Network for Organ Sharing and Pediatric Health Information System were used to identify Fontan heart transplant recipients aged less than 18 years (n = 241) between 2005 and 2022. Decompensation was defined as the presence of extracorporeal membrane oxygenation, ventilation, hepatic/renal dysfunction, paralytics, or total parenteral nutrition at transplant. RESULTS: Median age at transplant was 9 (interquartile range, 5-12) years. Median waitlist time was 107 (37-229) days. Median volume across 32 center was 8 (3-11) cases. Approximately half (n = 107, 45%) of recipients had 1A/1 initial listing status. Sixty-four patients (28%) were functionally impaired at transplant, 10 patients (4%) were ventilated, and 18 patients (8%) had ventricular assist device support. Fifty-nine patients (25%) had hepatic dysfunction, and 15 patients (6%) had renal dysfunction. Twenty-one patients (9%) were dependent on total parenteral nutrition. Median postoperative stay was 24 (14-46) days, and in-hospital mortality was 7%. Kaplan-Meier analysis showed 1- and 5-year survivals of 89% (95% CI, 85-94) and 74% (95% CI, 81-86), respectively. Kaplan-Meier of Fontan patients without decompensation (n = 154) at transplant demonstrated 1- and 5-year survivals of 93% (95% CI, 88-97) and 88% (95% CI, 82-94), respectively. In-hospital mortality was higher in decompensated patients (11% vs 4%, P = .023). Multivariable analysis showed that decompensation predicted worse post-transplant survival (hazard ratio, 2.47; 95% CI, 1.16-5.22; P = .018), whereas older age at transplant predicted superior post-transplant survival (hazard ratio, 0.89/year; 95% CI, 0.80-0.98; P = .019). CONCLUSIONS: Pediatric Fontan post-transplant outcomes are promising, although early mortality remains high. For nondecompensated pediatric patients at transplant without end-organ disease (>63% of cohort), early mortality is circumvented and post-transplant survival is excellent and similar to all pediatric transplantation.

10.
Article in English | MEDLINE | ID: mdl-37321290

ABSTRACT

OBJECTIVE: Ventricular assist devices (VADs) and inotropes are feasible modalities to bridge children to heart transplant (HT) in outpatient settings. However, it is unclear which modality yields superior clinical status at HT and posttransplant survival. METHODS: The United Network for Organ Sharing was used to identify patients aged 18 years or younger, weighing >25 kg, from 2012 to 2022 who were outpatients at HT (n = 835). Patients were grouped by bridging modality at HT: VAD (n = 235 [28%]), inotropes (n = 176 [21%]), or neither (no support) (n = 424 [50%]). RESULTS: VAD patients were of similar age (P = .260) but heavier (P = .007) and more likely to have dilated cardiomyopathy (P < .001) than their inotrope counterparts. VAD patients had similar clinical status at HT but superior functional status (performance scale >70%) (59% vs 31%) (P < .001). Overall posttransplant survival in VAD patients (1-year and 5-year survival, 97% and 88%, respectively) was comparable to patients with no support (93% and 87%, respectively) (P = .090) and those on inotropes (98% and 83%, respectively) (P = .089). One-year conditional survival was superior for VAD vs inotrope (2-year and 6-year survival, 96% and 91%, respectively vs 97% and 79%, respectively) (P = .030) and 5-year conditional survival for VAD patients was superior to inotrope (7-year and 10-year survival, 100% and 100%, respectively vs 100% and 88%, respectively) (P = .022) and no support (100% and 83%, respectively) (P = .011). CONCLUSIONS: Consistent with prior studies, short-term outcomes for pediatric patients bridged to HT in the outpatient setting with VAD or inotropes is excellent. However, compared with outpatients bridged to HT on inotropes, outpatient VAD support allowed for better functional status at HT and superior late posttransplant survival.

11.
J Thorac Cardiovasc Surg ; 166(5): e164-e173, 2023 Nov.
Article in English | MEDLINE | ID: mdl-37164051

ABSTRACT

OBJECTIVE: Several human studies have associated nitric oxide administration via the cardiopulmonary bypass circuit with decreased incidence of cardiopulmonary bypass-associated acute kidney injury, but histopathologic and serologic evidence of nitric oxide efficacy for acute kidney injury attenuation are lacking. METHODS: By using a survival ovine model (72 hours), acute kidney injury was induced by implementing low-flow cardiopulmonary bypass for 2 hours, followed by full-flow cardiopulmonary bypass for 2 hours. The nitric oxide cohort (n = 6) received exogenous nitric oxide through the cardiopulmonary bypass circuit via the oxygenator, and the control group (n = 5) received no nitric oxide. Serial serologic biomarkers and renal histopathology were obtained. RESULTS: Baseline characteristics (age, weight) and intraoperative parameters (cardiopulmonary bypass time, urine output, heart rate, arterial pH, and lactate) were equivalent (P > .10) between groups. Postoperatively, urine output, heart rate, respiratory rate, and peripheral arterial saturation were equivalent (P > .10) between groups. Post-cardiopulmonary bypass creatinine elevations from baseline were significantly greater in the control group versus the nitric oxide group at 16, 24, and 48 hours (all P < .05). Histopathologic evidence of moderate/severe acute kidney injury (epithelial necrosis, tubular slough, cast formation, glomerular edema) occurred in 60% (3/5) of the control group versus 0% (0/6) of the nitric oxide group. Cortical tubular epithelial cilia lengthening (a sensitive sign of cellular injury) was significantly greater in the control group than in the nitric oxide group (P = .012). CONCLUSIONS: In a survival ovine cardiopulmonary bypass model, nitric oxide administered with cardiopulmonary bypass demonstrated serologic and histologic evidence of renal protection from acute kidney injury. These results provide insight into 1 potential mechanism for cardiopulmonary bypass-associated acute kidney injury and supports continued study of nitric oxide via cardiopulmonary bypass circuit for prevention of acute kidney injury.

12.
World J Pediatr Congenit Heart Surg ; 14(1): 40-46, 2023 01.
Article in English | MEDLINE | ID: mdl-36847765

ABSTRACT

BACKGROUND: Overall one-year non-mortality outcomes for surgically palliated hypoplastic left heart syndrome (HLHS) patients remain understudied. Using the metric Days Alive and Outside of Hospital (DAOH), the present study sought to characterize expectations for surgically palliated patients' first year of life. METHODS: The Pediatric Health Information System database was used to identify by ICD-10 code all HLHS patients who underwent surgical palliation (Norwood/hybrid and/or heart transplantation [HTx]) during their index neonatal admission and were successfully discharged alive (n = 2227) and for whom one-year DAOH could be calculated. DAOH quartiles were used to group patients for analysis. RESULTS: Median one-year DAOH was 304 (interquartile range [IQR] 250-327), including a median index admission length of stay of 43 days (IQR 28-77). Patients required a median 2 (IQR 1-3) readmissions, each spanning 9 days (IQR 4-20). One-year readmission mortality or hospice discharge occurred in 6% of patients. Patients with lower-quartile DAOH had a median DAOH of 187 (IQR 124-226), whereas upper-quartile DAOH patients had a median DAOH of 335 (IQR 331-340) (P < .001). Readmission mortality/hospice-discharge rates were 14% and 1%, respectively (P < .01). On multivariable analysis, factors independently associated with lower-quartile DAOH included interstage hospitalization (odds ratio [OR] 44.78 [95% confidence interval [CI] 25.1-80.2]), index-admission HTx (8.73 [4.66-16.3]), preterm birth (1.97 [1.34-2.90]), chromosomal abnormality (1.85 [1.26-2.73]), age >7 days at surgery (1.50 [1.14-1.99]), and non-white race/ethnicity (1.33 [1.01-1.75]). CONCLUSIONS: In the current era, surgically palliated HLHS infants spend approximately 10 months alive and outside of the hospital, although outcomes are highly variable. Knowledge of the factors associated with lower DAOH can inform expectations and guide management decisions.


Subject(s)
Hypoplastic Left Heart Syndrome , Premature Birth , Infant, Newborn , Humans , Child , Infant , Female , Hypoplastic Left Heart Syndrome/surgery , Motivation , Hospitalization , Patient Discharge
13.
Pediatr Cardiol ; 2023 Feb 22.
Article in English | MEDLINE | ID: mdl-36811659

ABSTRACT

Patients with heterotaxy syndrome and congenital heart disease (CHD) experience inferior cardiac surgical outcomes. Heart transplantation outcomes are understudied, however, particularly compared to non-CHD patients. Data from UNOS and PHIS were used to identify 4803 children (< 18 years) undergoing first-time heart transplant between 2003 and 2022 with diagnoses of heterotaxy (n = 278), other-CHD (n = 2236), and non-CHD cardiomyopathy (n = 2289). Heterotaxy patients were older (median 5 yr) and heavier (median 17 kg) at transplant than other-CHD (median 2 yr and 12 kg), and younger and lighter than cardiomyopathy (median 7 yr and 24 kg) (all p < 0.001). UNOS status 1A/1 at listing was not different between groups (65-67%; p = 0.683). At transplant, heterotaxy and other-CHD patients had similar rates of renal dysfunction (12 and 17%), inotropes (10% and 11%), and ventilator-dependence (19 and 18%). Compared to cardiomyopathy, heterotaxy patients had comparable renal dysfunction (9%, p = 0.058) and inotropes (46%, p = 0.097) but more hepatic dysfunction (17%, p < 0.001) and ventilator-dependence (12%, p = 0.003). Rates of ventricular assist device (VAD) were: heterotaxy-10%, other-CHD-11% (p = 0.839 vs. heterotaxy), cardiomyopathy-37% (p < 0.001 vs. heterotaxy). The 1-year incidence of acute rejection post-transplant was comparable between heterotaxy and others (p > 0.05). While overall post-transplant survival was significantly worse for heterotaxy than others (p < 0.05 vs. both), conditional 1-year survival was comparable (p > 0.3 vs. both). Children with heterotaxy syndrome experience inferior post-heart transplant survival, although early mortality appears to influence this trend, with 1-year survivors having equivalent outcomes. Given similar pre-transplant clinical status to others, heterotaxy patients are potentially under risk-stratified. Increased VAD utilization and pre-transplant end-organ function optimization may portend improved outcomes.

14.
J Heart Lung Transplant ; 42(5): 585-592, 2023 05.
Article in English | MEDLINE | ID: mdl-36710094

ABSTRACT

PURPOSE: Children of minority race and ethnicity experience inferior outcomes postheart transplantation (HTx). Studies have associated ventricular assist device (VAD) bridge-to-transplant (BTT) with similar-to-superior post-transplant-survival (PTS) compared to no mechanical circulatory support. It is unclear whether racial and ethnic discrepancies exist in VAD utilization and outcomes. METHODS: The United Network for Organ Sharing (UNOS) database was used to identify 6,121 children (<18 years) listed for HTx between 2006 and 2021: black (B-22% of cohort), Hispanic (H-21%), and white (W-57%). VAD utilization, outcomes, and PTS were compared between race/ethnicity groups. Multivariable Cox proportional analyses were used to study the association of race and ethnicity on PTS with VAD BTT, using backward selection for covariates. RESULTS: Black children were most ill at listing, with greater proportions of UNOS status 1A/1 (p < 0.001 vs H & W), severe functional limitation (p < 0.001 vs H & W), and greater inotrope requirements (p < 0.05 vs H). Non-white children had higher proportions of public insurance. VAD utilization at listing was: B-11%, H-8%, W-8% (p = 0.001 for B vs H & W). VAD at transplant was: B-24%, H-21%, W-19% (p = 0.001 for B vs H). At transplant, all VAD patients had comparable clinical status (functional limitation, renal/hepatic dysfunction, inotropes, mechanical ventilation; all p > 0.05 between groups). Following VAD, hospital outcomes and one-year PTS were equivalent but long-term PTS was significantly worse among non-whites-(p < 0.01 for W vs B & H). On multivariable analysis, black race independently predicted mortality (hazard ratio 1.67 [95% confidence interval 1.22-2.28]) while white race was protective (0.54 [0.40-0.74]). CONCLUSIONS: Pediatric VAD use is, seemingly, equitable; the most ill patients receive the most VADs. Despite similar pretransplant and early post-transplant benefits, non-white children experience inferior overall PTS after VAD BTT.


Subject(s)
Heart Failure , Heart Transplantation , Heart-Assist Devices , Child , Humans , Treatment Outcome , Proportional Hazards Models , Graft Survival , Heart Failure/surgery , Retrospective Studies
15.
J Heart Lung Transplant ; 42(3): 345-353, 2023 03.
Article in English | MEDLINE | ID: mdl-36509608

ABSTRACT

BACKGROUND: The existence of a "weekend effect" in heart transplantation (HTx) is understudied. The present study sought to determine whether the odds of (HTx) offer acceptance differed for adult and pediatric candidates depending upon the day on which the offer occurred. METHODS: United Network for Organ Sharing data were used to identify all HTx offers to adult (listing age ≥18) and pediatric candidates from 2000-2019. Odds of offer acceptance were studied, comparing weekends, holidays, and conferences (Society of Thoracic Surgeons [STS], American Association for Thoracic Surgery [AATS], International Society for Heart and Lung Transplantation [ISHLT]) to "baseline" (all other days). Multivariable binary logistic regression analyses were performed to determine independent predictors of offer nonacceptance, controlling for the impacts of program transplant volume, region, and candidate characteristics. RESULTS: A total of 323,953 offers occurred - 298,405 to adults and 25,548 to pediatric candidates. Clinically significant differences did not exist in donor or candidate characteristics between baseline or other events. The number of offers per day was stable throughout the year for both adults (p = 0.191) and pediatrics (p = 0.976). In adults, independently lower odds of acceptance existed on weekends (OR 0.88 [95% CI 0.84-0.92]), conferences in aggregate (0.86 [0.77-0.95]), and holidays in aggregate (0.81 [0.72-0.91]). In children, independently lower odds of acceptance were seen on weekends (0.88 [0.79-0.98]), during STS (0.46 [0.25-0.83], and during Christmas (0.32 [0.14-0.76]). CONCLUSIONS: The day on which a HTx offer occurs significantly impacts its likelihood of acceptance. Further work can determine the impacts of human behavior or resource distribution, but knowledge of this phenomenon can inform efforts to ensure ideal organ allocation throughout the year.


Subject(s)
Heart Transplantation , Heart-Lung Transplantation , Adult , Child , Humans , United States , Holidays , Tissue Donors , Severity of Illness Index
16.
Pediatr Transplant ; 27(1): e14414, 2023 02.
Article in English | MEDLINE | ID: mdl-36261871

ABSTRACT

BACKGROUND: Both gender- and weight-matching between donor and recipient are thought to impact survival in pediatric heart transplantation, with clinical dogma holding that male donor hearts and "ideal" weight-matching yield superior survival. The composite impacts of gender and weight on post-transplant survival (PTS) are understudied. METHODS: All pediatric (age <18) heart recipients between 1989 and 2021 with the complete recipient and donor gender and weight data were identified in the United Network for Organ Sharing database. Patients were grouped by recipient-donor gender (M & F) and donor-to-recipient weight ratio (DRWR; undersized [<0.8], ideal-sized [0.8-1.5], oversized [>1.5]). RESULTS: A total of 10 697 patients were identified. Among male recipients, PTS was greatest with oversized DRWR from either male or female donors (median 22.4 and 20.6 years; p < .001 vs. others) and lowest for undersized DRWR from either male or female donors (median 13.4 and 13.2 years; p < .001 vs. others). The majority (64%) of male recipients received ideal-sized DRWR, among which female donor hearts yielded superior survival to males (median 18.9 vs. 17.4 years, p = .014). No differences in PTS existed for female recipients on the basis of gender-match, DRWR, and gender/DRWR together (all p > .1). CONCLUSIONS: When considered together, gender and DRWR pairings impact PTS in male-but not female-pediatric heart transplant recipients. For males receiving ideal-sized DRWR organs (most common pairing, >60%), male recipients achieve superior survival when female donor hearts are transplanted. These findings suggest that if weight is being used for size-matching, donor gender should also be considered, particularly for male recipients.


Subject(s)
Heart Transplantation , Tissue Donors , Humans , Child , Male , Retrospective Studies , Heart , Transplant Recipients , Graft Survival
17.
Pediatr Transplant ; 27(3): e14433, 2023 05.
Article in English | MEDLINE | ID: mdl-36345131

ABSTRACT

BACKGROUND: Mechanical ventilation prior to pediatric heart transplantation predicts inferior post-transplant survival, but the impact of ventilation duration on survival is unclear. METHODS: Data from the United Network for Organ Sharing and Pediatric Health Information System were used to identify pediatric (<18 years) heart transplant recipients from 2003 to 2020. Patients ventilated pretransplant were first compared to no ventilation, then ventilation durations were compared across quartiles of ventilation (≤1 week, 8 days-5 weeks, >5 weeks). RESULTS: At transplant, 11% (511/4506) of patients required ventilation. Ventilated patients were younger, had more congenital heart disease, more urgent listing-status, and greater rates of nephropathy, TPN-dependence, and inotrope and ECMO requirements (p < .001 for all). Post-transplant, previously ventilated patients experienced longer ventilation durations, ICU and hospital stays, and inferior survival (all p < .001). Hospital outcomes and survival worsened with longer pretransplant ventilation. One-year and overall survival were similar between the no-ventilation and ≤1 week groups (p = .703 & p = .433, respectively) but were significantly worse for ventilation durations >1 week (p < .001). On multivariable analysis, ventilation ≤1 week did not predict mortality (HR 0.98 [95% CI 0.85-1.43]), whereas ventilation >1 week did (HR: 1.18 [1.01-1.39]). CONCLUSIONS: Longer pretransplant ventilation portends worse outcomes, although only ventilation >1 week predicts mortality. These findings can inform pretransplant prognostication.


Subject(s)
Health Information Systems , Heart Transplantation , Humans , Child , Respiration, Artificial , Length of Stay , Time Factors , Retrospective Studies , Treatment Outcome
18.
J Thorac Cardiovasc Surg ; 166(6): 1766-1779, 2023 12.
Article in English | MEDLINE | ID: mdl-36163295

ABSTRACT

OBJECTIVE: The purpose of this study was to assess post-transplantation outcomes in recipients with increased pulmonary vascular resistance (PVR) in relation to donor size. METHODS: The United Network for Organ Sharing database was used to identify patients ages 0 to 18 years at time of listing who underwent transplantation from 2010 to 2019 and for whom cardiac catheterization and donor-recipient weight ratio data were available. Patients were divided according to listing PVR into <3, 3 to 6, and >6 Wood units. Donor-recipient weight ratio was categorized as undersized (≤0.80), midsize (0.81-1.2), and oversized (>1.2). Subgroup analysis was done with an additional supersized group (>2.0). RESULTS: Fourteen hundred ninety-one patients met study criteria. Median age was 10 (interquartile range, 3-15) years and 45% were female. Four percent of heart transplantation cases used undersized, 45% used midsize, and 51% used oversized organs. More patients with PVR >6 were received an oversized organ transplant compared with patients with PVR <3; 59% (148/252) versus 48% (430/894); P = .003. There was no difference in survival among organ size groups regardless of PVR; this includes patients with PVR >6 at listing who received an oversized organ transplant versus an undersized (P = .359) or midsized (P = .956) organ. In subgroup analysis, even in patients who received a supersized organ transplant, there was no survival difference noted regardless of PVR. CONCLUSIONS: Despite a persistent practice pattern to transplant oversized organs in high-PVR patients, there remains no difference in post-transplantation survival among these patients and those who received smaller organ transplants. Therefore, transplants in patients with high PVR should not be delayed by waiting for larger donors.


Subject(s)
Heart Transplantation , Transplants , Humans , Female , Child , Male , Retrospective Studies , Heart Transplantation/adverse effects , Vascular Resistance , Tissue Donors
19.
Article in English | MEDLINE | ID: mdl-36455711

ABSTRACT

Congenital heart disease (CHD) is a well-established risk factor for inferior waitlist and post-heart transplant survival in children. Differences in outcomes between CHD subgroups are understudied. The present study compared outcomes for palliated hypoplastic left heart syndrome (HLHS) patients to other non-single ventricle CHD (non-SVCHD) and non-CHD patients. United Network for Organ Sharing was used to identify children (age < 18) listed for heart transplant in the United States between 2016 and 2021. CHD sub-diagnoses were only available for United Network for Organ Sharing status 1a after 2015, thereby defining the cohort. Waitlist outcomes were studied using competing-risk time-to-event analysis for transplantation, mortality/decompensation, and alive-on-waitlist. Multivariable Cox proportional hazards regression analyses were used to identify factors associated with inferior post-transplant survival. Patients included: palliated-HLHS (n = 477), non-SVCHD (n = 686), and non-CHD (n = 1261). At listing, Palliated-HLHS patients were older than non-SVCHD (median 2-year [IQR 0-8] vs median 0-year [0-3], respectively) and younger than non-CHD (median 7-year [0-14]) (P < 0.001 vs both), and were more likely to be white (P < 0.01 vs both). Upon time-to-event analysis, rates of waitlist mortality/decompensation rates were greater among non-SVCHD than palliated-HLHS. Post-transplant survival was comparable between palliated-HLHS and non-SVCHD (P = 0.920) but worse compared to non-CHD (P < 0.001). Both palliated-HLHS (HR 2.40 [95% CI 1.68-3.42]) and non-SVSCHD (2.04 [1.39-2.99]) were independently associated with post-transplant mortality. Palliated-HLHS patients with heart failure experience significantly worse post-transplant outcomes than non-CHD but, compared to other CHD patients, experience superior waitlist and comparable post-transplant survival. While a high-risk cohort, HLHS patients can achieve gratifying waitlist and post-transplant survival.

20.
Article in English | MEDLINE | ID: mdl-36356907

ABSTRACT

The demand for organs for lung transplantation (LTx) continues to outweigh supply. However, nearly 75% of donor lungs are never transplanted. LTx offer acceptance practices and the effects on waitlist/post-transplant outcomes by candidate clinical acuity are understudied. UNOS was used to identify all LTx candidates, donors, and offers from 2005 to 2019. Candidates were grouped by Lung Allocation Score (LAS; applicable post-2005, ages ≥12 years): LAS<40, 40-60, 61-80, and >80. Offer acceptance patterns, waitlist death/decompensation, and post-transplant survival (PTS) were compared. "Acceptable organ offers" were those from donors whose organs were accepted for transplantation. Approximately 3 million offers to 34,531 candidates were reviewed. Median waitlist durations were: 9 days-(LAS>80), 17 days-(LAS 61-80), 42 days-(LAS 40-60), 125 days-(LAS<40) (P < 0.001 between all). Per waitlist-day, offer rates were: total offers - 0.8/day-(LAS>80), 0.7/day-(LAS 61-80), 0.6/day-(LAS 40-60), 0.4/day-(LAS<40); acceptable offers - 0.34/day-(LAS>80), 0.32/day-(LAS 61-80), 0.24/day-(LAS 40-60), 0.15/day-(LAS<40) (both P < 0.001 between all LAS). Among patients who experienced waitlist mortality/decompensation, ≥1 acceptable offer was declined in 92% (3939/4270) of patients - 78% for LAS >80, 88% for LAS 61-80, 93% for LAS 40-60, and 96% for LAS <40. Thirty-day waitlist mortality/decompensation rates were: 46%-(LAS>80), 24%-(LAS 61-80), 5%-(LAS 40-60), <1%-(LAS<40) (P < 0.001 between all). PTS was equivalent between patients for whom the first/second offer vs later offers were accepted (all LAS P > 0.4). The first offers that LTx candidates receive (including acceptable organs) are declined for nearly all candidates. Healthier candidates can afford offer selectivity but more ill patients (LAS>60) cannot, experiencing exceedingly high 30-day waitlist mortality.

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