Patients born with obstructed total anomalous pulmonary venous return have a high risk of morbidity and mortality in the neonatal period, which only increases when combined with single ventricle ...physiology and non-cardiac congenital anomalies such as heterotaxy syndrome. Despite advances in management of congenital heart disease, surgery within the first weeks of life to repair the pulmonary venous connection and establish pulmonary blood flow with a systemic-to-pulmonary shunt has historically led to disappointing outcomes. A multidisciplinary approach with pediatric interventional cardiology and cardiac surgery is required to reduce morbidity and mortality in this extremely high-risk patient population. Extending the time between birth and cardiac surgery can lessen postoperative complications and mortality risk, especially in patients with abnormal thoracoabdominal relationships. Our team was able to successfully utilize transcatheter stent placement in a vertical vein and patent ductus arteriosus to delay and stage cardiac surgeries in an infant born with obstructed total anomalous pulmonary venous return, unbalanced atrioventricular septal defect with pulmonary atresia and heterotaxy, thus reducing the morbidity and mortality associated with this diagnosis.
The purpose of this study was to assess the outcomes of slide tracheoplasty and tracheal resection in pediatric patients and analyze the data for predictors of outcomes.
A retrospective review of ...tracheal surgery from January 1, 1993 to May 1, 2018 was performed. Demographic data, operative details, perioperative data, and clinical outcomes were collected. The study investigators' management strategy has evolved over time, with less rigid bronchoscopy, more reliance on postoperative computed tomographic imaging, and the use of inhaled Ciprodex (combination of ciprofloxacin and dexamethasone) since 2007.
The study included 41 patients, with a median age of 4.1 months and a median weight of 4.2 kg. There were 6 neonates and 24 infants. Slide tracheoplasty was performed in 27 patients (66%), and resection with end-to-end anastomosis was performed in 14 (34%). Eleven patients (27%) had a pulmonary artery sling. Simultaneous intracardiac repairs requiring cross-clamp and cardioplegia were performed in 9 patients (22%). Lung agenesis (n = 6) or severe hypoplasia (n = 2) was present in 8 patients (20%). Complications included tracheostomy in 5 patients (12%) and in-hospital death in 3 patients (7%). There were no cases of mediastinitis. Inhaled Ciprodex was used to decrease granulation tissue at suture lines. Median intubation time was 7 days, and median length of stay was 25.0 days. There was no difference in outcomes when comparing intracardiac repairs with the remaining patients, lung agenesis or hypoplasia vs the remaining cohort, or neonates vs infants.
Slide tracheoplasty and tracheal resection are effective operative strategies for infants and children with tracheal stenosis, including patients with lung agenesis/hypoplasia. Simultaneous repair of intracardiac anomalies and pulmonary artery sling is recommended.
Objective Two randomized trials of hematocrit strategy during hypothermic cardiopulmonary bypass in infant heart surgery have been performed. The first suggested worse outcomes were concentrated in ...patients with lower hematocrit levels (approximately 20%), whereas the second suggested there was little benefit to increasing the hematocrit level above 25%. The form of the relationship between continuous hematocrit levels and outcomes requires further study. Methods In the two trials, 271 infants who underwent biventricular repair not involving the aortic arch were enrolled. Analysis was undertaken of the effects of hematocrit level, as a continuous variable, at the onset of low-flow cardiopulmonary bypass. Results Psychomotor Development Index scores at age 1 year varied nonlinearly with hematocrit levels, with increasing scores up to 23.5% hematocrit ( P < .001) and a plateau effect beyond 23.5% ( P = .42), based on a piecewise linear model. Lower hematocrit levels were associated with more positive intraoperative fluid balance ( P < .001 for linear trend) and marginally associated with higher serum lactate levels at 60 minutes after bypass ( P = .08 for linear trend), but not with blood products given, nadir of cardiac index in the first 24 hours, or Mental Development Index scores. Conclusions A hematocrit level at the onset of low-flow cardiopulmonary bypass of approximately 24% or higher is associated with higher Psychomotor Development Index scores and reduced lactate levels. Because the effects of hemodilution may vary according to diagnosis, age at operation, bypass variables such as pH strategy and flow rate, and other perioperative factors, this study cannot ascertain a universally “safe” hemodilution level.
Diabetic cardiomyopathy (DCM) lacks diagnostic biomarkers. Circulating long non-coding RNAs (lncRNAs) can serve as valuable diagnostic biomarkers in cardiovascular disease. To seek potential lncRNAs ...as a diagnostic biomarker for DCM, we investigated the genome-wide expression profiling of circulating lncRNAs and mRNAs in type 2 diabetic db/db mice with and without DCM and performed bioinformatic analyses of the deregulated lncRNA-mRNA co-expression network. Db/db mice had obesity and hyperglycemia with normal cardiac function at 6 weeks of age (diabetes without DCM) but with an impaired cardiac function at 20 weeks of age (DCM) on an isolated Langendorff apparatus. Compared with the age-matched controls, 152 circulating lncRNAs, 127 mRNAs and 3355 lncRNAs, 2580 mRNAs were deregulated in db/db mice without and with DCM, respectively. The lncRNA-mRNA co-expression network analysis showed that five deregulated lncRNAs, XLOC015617, AK035192, Gm10435, TCR-α chain, and MouselincRNA0135, have the maximum connections with differentially expressed mRNAs. Bioinformatic analysis revealed that these five lncRNAs were highly associated with the development and motion of myofilaments, regulation of inflammatory and immune responses, and apoptosis. This finding was validated by the ultrastructural examination of myocardial samples from the db/db mice with DCM using electron microscopy and changes in the expression of myocardial tumor necrosis factor-α and phosphorylated p38 mitogen-activated protein kinase in db/db mice with DCM. These results indicate that XLOC015617, AK035192, Gm10435, TCR-α chain, and MouselincRNA0135 are crucial circulating lncRNAs in the pathogenesis of DCM. These five circulating lncRNAs may have high potential as a diagnostic biomarker for DCM.
Objectives Bilateral pulmonary artery banding with or without ductal stenting has been performed as a resuscitative intervention for patients considered at too high risk for conventional single ...ventricle palliation. The purpose of the present study was to determine the outcomes using this strategy. Methods We performed a retrospective review of 24 patients with single ventricle anatomy who were younger than 3 months who had undergone bilateral pulmonary artery banding and ductal stenting or maintenance of prostaglandin E1 from January 2007 to October 2011 at our institution. The echocardiographic, angiographic, operative, and clinical data were reviewed. Follow-up data were available for 100% of the patients. Results All 24 patients (13 male patients) underwent bilateral pulmonary artery banding at a median age of 8 days (range, 2-44 days). Their gestational age was 38 weeks (range, 27-41 weeks), and their weight was 3.01 kg (range, 1.5-4.4 kg). The cardiac diagnoses included hypoplastic left heart syndrome/variant hypoplastic left heart syndrome in 18, unbalanced atrioventricular canal in 4, and tricuspid atresia in 2. In the hypoplastic left heart syndrome group, 9 (50%) had an intact or a highly restrictive atrial septum requiring open (n = 1) or transcatheter (n = 8) atrial septostomy with or without atrial stent placement (n = 4). Ductal stenting was performed in 14 patients, and 10 patients were continued with prostaglandin E1 . Fifteen patients (62.5%) survived to undergo a Norwood procedure (n = 7), comprehensive stage 2 (n = 1), or primary cardiac transplantation (n = 7). Of the 9 who died, support was withdrawn in 5 because of a contraindication to transplantation, 1 because of sepsis and/or multiorgan system failure, and 1 for whom palliative care was desired. Two died awaiting transplantation. All 7 patients who underwent a conventional Norwood operation survived to discharge, and 6 of the 7 (85.7%) underwent bidirectional Glenn shunt placement. Of the 7 patients who underwent transplantation, 6 (85.7%) were alive at a median follow-up of 33.6 months. Conclusions Bilateral pulmonary artery banding with or without ductal stenting is an effective method of resuscitation for high-risk neonates and infants with a single ventricle, allowing for reasonable survival to conventional first-stage palliation or primary transplantation.
Diabetic cardiomyopathy is one of the main causes of heart failure and death in patients with diabetes. There are no effective approaches to preventing its development in the clinic. Long noncoding ...RNAs (lncRNA) are increasingly recognized as important molecular players in cardiovascular disease. Herein we investigated the profiling of cardiac lncRNA and mRNA expression in type 2 diabetic db/db mice with and without early diabetic cardiomyopathy. We found that db/db mice developed cardiac hypertrophy with normal cardiac function at 6 weeks of age but with a decreased diastolic function at 20 weeks of age. LncRNA and mRNA transcripts were remarkably different in 20-week-old db/db mouse hearts compared with both nondiabetic and diabetic controls. Overall 1479 lncRNA transcripts and 1109 mRNA transcripts were aberrantly expressed in 6- and 20-week-old db/db hearts compared with nondiabetic controls. The lncRNA-mRNA co-expression network analysis revealed that 5 deregulated lncRNAs having maximum connections with differentially expressed mRNAs were BC038927, G730013B05Rik, 2700054A10Rik, AK089884, and Daw1. Bioinformatics analysis revealed that these 5 lncRNAs are closely associated with membrane depolarization, action potential conduction, contraction of cardiac myocytes, and actin filament-based movement of cardiac cells. This study profiles differently expressed lncRNAs in type 2 mice with and without early diabetic cardiomyopathy and identifies BC038927, G730013B05Rik, 2700054A10Rik, AK089884, and Daw1 as the core lncRNA with high significance in diabetic cardiomyopathy.
Partial heart transplantation is a new type of transplant that delivers growing heart valve replacements for babies. Partial heart transplantation differs from orthotopic heart transplantation ...because only the part of the heart containing the heart valve is transplanted. It also differs from homograft valve replacement because viability of the graft is preserved by tissue matching, minimizing donor ischemia times, and recipient immunosuppression. This preserves partial heart transplant viability and allows the grafts to fulfill biological functions such as growth and self‐repair. These advantages over conventional heart valve prostheses are balanced by similar disadvantages as other organ transplants, most importantly limitations in donor graft availability. Prodigious progress in xenotransplantation promises to solve this problem by providing an unlimited source of donor grafts. In order to study partial heart xenotransplantation, a suitable large animal model is important. Here we describe our research protocol for partial heart xenotransplantation in nonhuman primates.
Partial heart xenotransplantation promises an unlimited source of growing heart valves for pediatric cardiac surgery. Here we describe our model for porcine to nonhuman primate partial heart xenotransplantation. Figure created with biorender.com.
The integrated 6-year thoracic surgery (I-6) residency model was developed in part to promote early interest in cardiothoracic surgery in diverse trainees. To determine gaps in and opportunities for ...recruitment of women and minority groups in the pipeline for I-6 residency, we quantified rates of progression at each training level and trends over time.
We obtained 2015 to 2019 medical student, I-6 applicant, and I-6 resident gender and race/ethnicity demographic data from the American Association of Medical Colleges and Electronic Residency Application Service public databases and Accreditation Council for Graduate Medical Education Data Resource Books. We performed χ
, Fisher exact, and Cochran-Armitage tests for trend to compare 2015 and 2019.
Our cross-sectional analysis found increased representation of women and all non-White races/ethnicities, except Native American, at each training level from 2015 to 2019 (P < .001 for all). The greatest trends in increases were seen in the proportions of women (28% vs 22%, P = .46) and Asian/Pacific Islander (25% vs 15%, P = .08) applicants. There was also an increase in the proportions of women (28% vs 24%, P = .024) and White (61% vs 58%, P = .007) I-6 residents, with a trend for Asian/Pacific Islanders (20% vs 17%, P = .08). The proportions of Hispanic (5%) and Black/African American (2%) I-6 residents in 2019 remained low.
I-6 residency matriculation is not representative of medical student demographics and spotlights a need to foster early interest in cardiothoracic surgery among all groups underrepresented in medicine while ensuring that we mitigate bias in residency recruitment.
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