Persistent patency of the ductus arteriosus (PDA) is less frequent among infants treated with caffeine for apnea of prematurity. Caffeine acts to inhibit A1, A2, and A3 adenosine receptors (ARs). Adenosine is typically vasodilatory, and serum adenosine levels are elevated in preterm newborns, suggesting a potential mechanism for caffeine-associated reduction in PDA. We hypothesized that caffeine has an indirect vasoconstrictive effect on the ductus by antagonizing specific ARs. The expression of AR subtypes in the mouse ductus was analyzed by RT-PCR on days 15, 17, 19 (full term) of gestation, and postnatal day 1. Pressure myography was used to examine responses of the isolated ductus to adenosine, caffeine (citrate or base), or preincubation with either caffeine or adenosine. AR immuno-localization and adenosine-mediated cyclic AMP (cAMP) generation were evaluated in human ductus smooth muscle cells (SMCs). A1AR, A2AAR, A2BAR, and A3AR were present and developmentally regulated in the mouse ductus. Adenosine promoted ductus dilation under fetal and newborn O2 conditions. Caffeine had little or no effect on ductus tone with concentrations spanning the therapeutic range and failed to augment O2-induced or cyclooxygenase inhibitor-stimulated ductus constriction. However, pretreatment with caffeine or selective A1AR and A2AAR antagonists prevented adenosine-induced ductus dilation. Caffeine also blocked adenosine-stimulated cAMP release in human ductus SMCs. In conclusion, caffeine did not induce direct ductus constriction ex vivo. However, caffeine exposure prevented adenosine-induced ductus relaxation, suggesting the inhibition of an endogenous vasodilator as a mechanism for the reduction in PDA.NEW & NOTEWORTHY Caffeine is a respiratory stimulant and one of the most commonly prescribed drugs in the NICU. Primarily used to reduce apnea of prematurity, additional therapeutic benefits have been noted, including decreasing the incidence of patent ductus arteriosus. Using mouse and human ductus models, we have identified a mechanism by which caffeine can promote ductus constriction by inhibiting adenosine-mediated vasodilation.

Background:Persistent left superior vena cava (LSVC) is a common congenital anomaly requiring operative management when it causes significant right-to-left shunting or during heart transplantation. Comparative patency of various LSVC repatriation methods to the right side of the heart has not been systematically studied. Methods:This is a single-institution, retrospective review of patients undergoing surgical management of LSVC from 2013 to 2023. Patients were grouped on the basis of the LSVC to right atrium path: coronary sinus (CS) group with LSVC-CS anastomosis; systemic vein (SV) group with LSVC drainage through donor superior vena cava, right atrial appendage, or innominate vein; or atrial baffle (AB) group. Our primary objective is LSVC patency rate in each group. Results:Twenty-two patients with a median age of 27 months met inclusion criteria; 4 patients were in the CS group, 9 in the SV group, and 9 in the AB group. At the time of collection, 3 (75%) patients in the CS group, 3 (33%) in the SV group, and 9 (100%) in the AB group showed LSVC patency. All patients demonstrating patency in CS and SV groups were older than 2 years, whereas all patients with occlusion (except for a 35-year-old patient) were younger than 2 years. Conclusions:Of the methods redirecting LSVC to the right atrium, patency may be best preserved with an undistorted LSVC reunited with a retained CS or redirected by an intracardiac baffle compared with methods that displace the LSVC. Older patients with higher weight may have better patency rates with LSVC reconstruction.

Collaborative multicenter research has significantly increased our understanding of fetal Ebstein anomaly, delineating risk factors for adverse outcomes as well as predictors of postnatal management. These data are incorporated into prenatal care and therapeutic strategies and inform family counseling and delivery planning to optimize care. This report details the translation of findings from multicenter studies into multidisciplinary prenatal care for a fetus with Ebstein anomaly, supraventricular tachycardia, and a circular shunt, including transplacental therapy to control arrhythmias and achieve ductal constriction, informed and coordinated delivery room management, and planned univentricular surgical palliation.

A 31-year-old woman with transposition of the great arteries status post-Senning operation presents with severe pulmonary venous baffle obstruction. Both standards of care (percutaneous stenting or open repair) were deemed suboptimal and/or high risk. A multidisciplinary, hybrid approach via subxiphoid incision, guided by 3-dimensional modeling, provided a lower risk and minimally invasive intervention.

Background: Complications following the Fontan procedure include prolonged pleural drainage and readmission for effusions. To address these complications, a post-Fontan management pathway was implemented with primary goals of reducing chest tube duration/reinsertion rates and decreasing hospital length of stay and readmissions.Methods: Fontan patients were identified by retrospective chart review (2017-2019) to obtain baseline data for chest tube duration/reinsertion rates, hospital length of stay, and readmission rates for effusion. A post-Fontan management pathway was implemented (2020-2021) utilising post-operative vasopressin, nasal cannula oxygen until chest tube removal, and discharge regimen of three times daily diuretics, sildenafil, and afterload reducing medications. Patients were followed to evaluate primary outcomes.Results: The pre- and post-pathway groups were similar in single ventricle morphology, demographics, and pre-operative haemodynamics. Forty-three and 36 patients were included in the pre- and post-pathway cohorts, respectively. There were statistically significant reductions in chest tube duration (8 vs. 5 days, p <= 0.001), chest tube output on post-operative day 4 (20.4 vs. 9.9 mL/kg/day, p = 0.003), and hospital readmission rates for effusion (13[30%] vs. 3[8%], p = 0.02) compared to baseline. There was an absolute reduction in hospital length of stay (11 vs. 9.5 days, p = 0.052). When combining average cost savings for the Fontan hospitalisations, readmissions for effusion, and cardiac catheterisations within 6 months of Fontan completion, there was a $325,144 total cost savings for 36 patients following pathway implementation.Conclusion: Implementation of a post-Fontan management pathway resulted in significant reductions in chest tube duration and output, and readmission rates for effusion in the perioperative period.

BACKGROUND:The aims of the study were to assess the performance of a clinically available cell-free DNA (cfDNA) assay in a large cohort of pediatric and adult heart transplant recipients and to evaluate performance at specific cut points in detection of rejection. METHODS:Observational, non-interventional, prospective study enrolled pediatric and adult heart transplant recipients from seven centers. Biopsy-associated plasma samples were used for cfDNA measurements. Pre-determined cut points were tested for analytic performance. RESULTS:A total of 487 samples from 160 subjects were used for the analysis. There were significant differences for df-cfDNA values between rejection [0.21% (IQR 0.12-0.69)] and healthy samples [0.05% (IQR 0.01-0.14), p < .0001]. The pediatric rejection group had a median df-cfDNA value of 0.93% (IQR 0.28-2.84) compared to 0.09% (IQR 0.04-0.23) for healthy samples, p = .005. Overall negative predictive value was 0.94 while it was 0.99 for pediatric patients. Cut points of 0.13% and 0.15% were tested for various types of rejection profiles and were appropriate to rule out rejection. CONCLUSION:The study suggests that pediatric patients with rejection show higher levels of circulating df-cfDNA compared to adults and supports the specific cut points for clinical use in pediatric and adult patients with overall acceptable performance.

Background:During cardiomyocyte maturation, the centrosome, which functions as a microtubule organizing center in cardiomyocytes, undergoes dramatic structural reorganization where its components reorganize from being localized at the centriole to the nuclear envelope. This developmentally programmed process, referred to as centrosome reduction, has been previously associated with cell cycle exit. However, understanding of how this process influences cardiomyocyte cell biology, and whether its disruption results in human cardiac disease, remains unknown. We studied this phenomenon in an infant with a rare case of infantile dilated cardiomyopathy (iDCM) who presented with left ventricular ejection fraction of 18% and disrupted sarcomere and mitochondria structure. Methods:We performed an analysis beginning with an infant who presented with a rare case of iDCM. We derived induced pluripotent stem cells from the patient to model iDCM in vitro. We performed whole exome sequencing on the patient and his parents for causal gene analysis. CRISPR/Cas9-mediated gene knockout and correction in vitro were used to confirm whole exome sequencing results. Zebrafish and Drosophila models were used for in vivo validation of the causal gene. Matrigel mattress technology and single-cell RNA sequencing were used to characterize iDCM cardiomyocytes further. Results:Whole exome sequencing and CRISPR/Cas9 gene knockout/correction identified RTTN, the gene encoding the centrosomal protein RTTN (rotatin), as the causal gene underlying the patient's condition, representing the first time a centrosome defect has been implicated in a nonsyndromic dilated cardiomyopathy. Genetic knockdowns in zebrafish and Drosophila confirmed an evolutionarily conserved requirement of RTTN for cardiac structure and function. Single-cell RNA sequencing of iDCM cardiomyocytes showed impaired maturation of iDCM cardiomyocytes, which underlie the observed cardiomyocyte structural and functional deficits. We also observed persistent localization of the centrosome at the centriole, contrasting with expected programmed perinuclear reorganization, which led to subsequent global microtubule network defects. In addition, we identified a small molecule that restored centrosome reorganization and improved the structure and contractility of iDCM cardiomyocytes. Conclusions:This study is the first to demonstrate a case of human disease caused by a defect in centrosome reduction. We also uncovered a novel role for RTTN in perinatal cardiac development and identified a potential therapeutic strategy for centrosome-related iDCM. Future study aimed at identifying variants in centrosome components may uncover additional contributors to human cardiac disease.

Central MessageDonor heart oversizing confers no advantage for adult or pediatric recipients, with or without pulmonary hypertension.See Article page 1766. Donor heart oversizing confers no advantage for adult or pediatric recipients, with or without pulmonary hypertension. See Article page 1766. Emerging from the mist of anecdote, small series, and flawed metrics came the concept that a larger donor heart's larger stroke volume should be better suited to face recipient pulmonary hypertension. This hypothetical and conflictingly supported idea, once elevated to common practice, now may be joining bloodletting, cupping, and ingestion of mercury among practices that rose from similar mist and seemed like reasoned ideas only for a while. Logically, any advantage of a larger stroke volume to combat greater afterload pressure may be offset by the logic of Laplace that says, when applying the same pressure afterload to a large diameter, unacclimated, thin-walled chamber versus a smaller chamber, the larger chamber suffers greater radial wall stress. Data do or do not support the practice of donor oversizing. Donor recipient weight ratio (DRWR) data produced evidence that undersized donor hearts underperform in the setting of pulmonary hypertension,1Patel N.D. Weiss E.S. Nwakanma L.U. Russell S.D. Baumgartner W.A. Shah A.S. et al.Impact of donor-to-recipient weight ratio on survival after heart transplantation.Circulation. 2008; 118: S83-S88Crossref PubMed Scopus (119) Google Scholar or that oversized hearts perform better,2Yeoh T.K. Frist W.H. Lagerstrom C. Kasper E.K. Groves J. Merrill W. Relationship of cardiac allograft size and pulmonary vascular resistance to long-term cardiopulmonary function.J Heart Lung Transplant. 1992; 11: 1168-1176PubMed Google Scholar or that over- or undersizing doesn't matter, even in the setting of pulmonary hypertension.3Kwon M.H. Wong S. Kittleson M. Ardehali A. Laks H. Shemin R.J. et al.Selecting oversized donor cardiac allografts for patients with pulmonary hypertension may be unnecessary.Transplant Proc. 2014; 46: 1497-1501Crossref PubMed Scopus (14) Google Scholar At the root of some of the conflicting data is a problem of how to estimate heart size. An analysis of data from the United Network for Organ Sharing registry (31,634 patients) has been used to calculate predicted heart mass (pHM). The study showed that although 2 people may have similar weights, their hearts could have vastly different sizes, that weight differences do not affect outcome but pHM does, with poorer outcome for donor hearts undersized by pHM.4Reed R.M. Netzer G. Hunsicker L. Mitchell B.D. Rajagopal K. Scharf S. et al.Cardiac size and sex-matching in heart transplantation size matters in matters of sex and the heart.JACC Heart Fail. 2014; 2: 73-83Crossref PubMed Scopus (168) Google Scholar A study of 3215 pediatric heart transplant recipients from the Organ Procurement and Transplant Network database found predicted left ventricular mass no better metric than weight alone in the pediatric population, and similar findings that oversizing does not confer advantage, but undersizing donor hearts carry a greater risk of mortality.5Singh T.P. Colan S.D. Gauvreau K. Matching donor and recipient size in pediatric heart transplantation.Transplant Int. 2022; 36: 10226Crossref Scopus (5) Google Scholar Thangappan and colleagues,6Thangappan K. Zafar F. Ahmed H.F. Greenberg J.W. Ashfaq A. Hirsch R. et al.Is the practice of using oversized organs for recipients with elevated pulmonary vascular resistance justified?.J Thorac Cardiovasc Surg. 2023; 166: 1766-1779Abstract Full Text Full Text PDF Scopus (1) Google Scholar in this issue of the Journal, return to simple weight ratio instead of heart mass predictors to again challenge the practice of transplanting oversized donor hearts into pediatric recipients with pulmonary hypertension. The study uses the United Network for Organ Sharing registry data to assess 1491 patients, with attention to posttransplant outcomes for recipients with elevated pulmonary resistance. Not surprisingly, no difference was found in posttransplantation survival between patients with DRWR >1.8 and those transplanted with smaller organs, regardless of pulmonary resistance. Only 2 patients with elevated pulmonary resistance were transplanted with undersized hearts (DRWR <0.8), and the study is underpowered to make conclusions about undersizing. The study adds to evidence that the practice of oversizing delivers no advantage, and it may contribute to increased wait time. It seems established that oversizing confers no advantage for adults or children, with or without pulmonary hypertension, and so another tenet of common practice is headed for the dustbin. The more consistent and consequential message is that undersizing is bad, irrespective of pulmonary resistance. Fitting 5 pounds of matter in a 10-pound bag is of little consequence. Stuffing 10 pounds of matter into a 5-pound bag is problematic. Is the practice of using oversized organs for recipients with elevated pulmonary vascular resistance justified?The Journal of Thoracic and Cardiovascular SurgeryVol. 166Issue 6PreviewThe purpose of this study was to assess post-transplantation outcomes in recipients with increased pulmonary vascular resistance (PVR) in relation to donor size. Full-Text PDF

Objective:To identify risk factors for aortopulmonary collateral (APC) development and assess the impact of severe APCs in children undergoing staged single ventricle palliation.Methods:Children undergoing a bidirectional Glenn operation between January 1, 2016, and March 31, 2021, at our center were included. All underwent angiography prior to Glenn and Fontan; APC flow was graded on a scale of 0 (no appreciable collateral flow) to 4 (severe burden). Demographic data, congenital diagnosis, clinical history, and outcomes were stratified by Glenn assessment; Fontan outcomes were stratified by pre-Fontan grade.Results:Sixty patients met the inclusion criteria, all of whom had angiographic evidence of APCs. There were 7 transplants and 9 deaths in the cohort. There were no significant differences in demographics among the patients. Right ventricular morphology was more common in patients with severe pre-Glenn collaterals (24 of 44 vs 2 of 6 vs 7 of 8; P = .014). Longer stage 1 aortic cross-clamp duration was associated with greater severity pre-Glenn (44 minutes vs 34 minutes vs 66 minutes; P = .023). Patients with grade 3 pre-Glenn collaterals more commonly required transplantation than those with grade 1 collaterals (P < .001) and had lower overall transplant-free survival than those with grade 1 (P = .005) or grade 2 (P = .04) collaterals.Conclusions:The ubiquity of APCs in this study demonstrates their prevalence in single ventricle disease. Right ventricular morphology and prolonged aortic cross-clamp duration are associated with higher burden. Greater severity was associated with decreased transplant-free survival. These data emphasize the negative long-term impact of these collaterals.

We describe a 35-month-old girl with a complex congenital heart defect including double-inlet left ventricle who presented with an occluded ductal stent. We performed pulmonary artery unifocalization and central shunt placement to bridge to Fontan or as final palliation. Anticipating a prolonged role for the shunt, we used a novel approach to aortopulmonary shunt construction consisting of a stent-restricted shunt that allows extended shunt longevity through an ability to "grow" with the patient's somatic growth. This technique demonstrates a safe way to dilate aortopulmonary shunts without repeated open surgical intervention.

Turner syndrome (TS) is associated with left-sided cardiac lesions, including hypoplastic left heart syndrome (HLHS). Mortality as high as 80–90

Children from developing nations with advanced, complex congenital heart disease including single ventricles can be successfully treated.

Objectives: Donor-specific cell-free DNA shows promise as a noninvasive marker for allograft rejection, but as yet has not been validated in both adult and pediatric recipients. The study objective was to validate donor fraction cell- free DNA as a noninvasive test to assess for risk of acute cellular rejection and antibodymediated rejection after heart transplantation in pediatric and adult recipients. Methods: Pediatric and adult heart transplant recipients were enrolled from 7 participating sites and followed for 12 months or more with plasma samples collected immediately before all endomyocardial biopsies. Donor fraction cellfree DNA was extracted, and quantitative genotyping was performed. Blinded donor fraction cell-free DNA and clinical data were analyzed and compared with a previously determined threshold of 0.14%. Sensitivity, specificity, negative predictive value, positive predictive value, and receiver operating characteristic curves were calculated. Results: A total of 987 samples from 144 subjects were collected. After applying predefined clinical and technical exclusions, 745 samples from 130 subjects produced 54 rejection samples associated with the composite outcome of acute cellular rejection grade 2R or greater and pathologic antibody- mediated rejection 2 or greater and 323 healthy samples. For all participants, donor fraction cell-free DNA at a threshold of 0.14% had a sensitivity of 67%, a specificity of 79%, a positive predictive value of 34%, and a negative predictive value of 94% with an area under the curve of 0.78 for detecting rejection. When analyzed independently, these results held true for both pediatric and adult cohorts at the same threshold of 0.14% (negative predictive value 92% and 95%, respectively). Conclusions: Donor fraction cell-free DNA at a threshold of 0.14% can be used to assess for risk of rejection after heart transplantation in both pediatric and adult patients with excellent negative predictive value. (J Thorac Cardiovasc Surg 2023;165:460-8)

A cDNA clone encoding grapevine (Vitis vinifera L. cv Sultanina) NAD(H)-glutamate dehydrogenase (GDH) was isolated from a cDNA expression library by immunoscreening with a polyclonal antibody raised against grapevine GDH. Nucleotide sequence analysis revealed an open reading frame (ORF) encoding a precursor protein of 411 amino acids (aa) with a calculated molecular mass of 44.517 kDa. The deduced aa sequence showed relatively higher homology to GDH from archaebacteria species, than to those from eukaryotes and eubacteria. This resemblance indicated a functional and/or evolutionary relationship in this class of enzymes which might be relevant to the stress-related function of plant GDH. We have shown that the bacterially produced plant GDH was thermostable.

Background: Obesity increases the risk of post-operative arrhythmias in adults undergoing cardiac surgery, but little is known regarding the impact of obesity on post-operative arrhythmias after CHD surgery. Methods: Patients undergoing CHD surgery from 2007 to 2019 were prospectively enrolled in the parent study. Telemetry was assessed daily, with documentation of all arrhythmias. Patients aged 2-20 years were categorised by body mass index percentile for age and sex (underweight <5, normal 5-85, overweight 85-95, and obese >95). Patients aged >20 years were categorised using absolute body mass index. We investigated the impact of body mass index category on arrhythmias using univariate and multivariate analysis. Results: There were 1250 operative cases: 12% underweight, 65% normal weight, 12% overweight, and 11% obese. Post-operative arrhythmias were observed in 38%. Body mass index was significantly higher in those with arrhythmias (18.8 versus 17.8, p = 0.003). There was a linear relationship between body mass index category and incidence of arrhythmias: underweight 33%, normal 38%, overweight 42%, and obese 45% (p = 0.017 for trend). In multivariate analysis, body mass index category was independently associated with post-operative arrhythmias (p = 0.021), with odds ratio 1.64 in obese patients as compared to normal-weight patients (p = 0.036). In addition, aortic cross-clamp time (OR 1.007, p = 0.002) and maximal vasoactive-inotropic score in the first 48 hours (OR 1.03, p = 0.04) were associated with post-operative arrhythmias. Conclusion: Body mass index is independently associated with incidence of post-operative arrhythmias in children after CHD surgery.

Partial anomalous pulmonary venous return is a rare congenital heart defect that can escape detection until adulthood and can be misdiagnosed as pulmonary hypertension and managed with vasodilators before the anomaly is identified. This report describes the cases of 3 patients with pulmonary hypertension whose hemodynamics and symptoms improved after repair of anomalous veins. Anomalous pulmonary veins are difficult to identify on a transthoracic echocardiogram, so a high index of suspicion and early use of additional imaging modalities are important to avoid a delayed diagnosis and progression to irreversible disease.

Central MessageOutcome is driven more by cardiac output, less by shunt resistance. A generous stroke volume can afford to support a big shunt. Lower output phases of recovery demand nuanced attention to shunt size.See Article page 206. Outcome is driven more by cardiac output, less by shunt resistance. A generous stroke volume can afford to support a big shunt. Lower output phases of recovery demand nuanced attention to shunt size. See Article page 206. Dividing a single ventricle's cardiac output between pulmonary and systemic circulations is a dicey zero-sum game, particularly in the postoperative 12 to 24 hours when a nadir of cardiac output renders the stroke volume an especially meager resource to divide. A combination of pharmacologic and mechanical maneuvers to modify pulmonary resistance, systemic resistance, and shunt resistance aims to keep pulmonary overperfusion from siphoning away systemic perfusion as the shifting dynamics of the first few postoperative days are navigated. Aortopulmonary shunt resistance is the subject of the current study by Spigel and colleagues.1Spigel Z.A. Qureshi A.M. Kalustian A. Binsalamah A.M. Imamura M. Caldarone C.A. Shunt resistance is associated with clinically important outcomes after the Norwood operation.J Thorac Cardiovasc Surg Open. 2022; 9: 206-214Scopus (1) Google Scholar The authors use data from the Single Ventricle Reconstruction (SVR) trial that include measurements to calculate resistance across aortopulmonary shunts. Death or transplant at 1 year, branch pulmonary artery size at second-stage surgery, and the use of supplemental oxygen were analyzed. A corroborative dataset was obtained from the authors' institution, validating calculated resistances by direct catheter measurement for 12 patients who survived to pre-Glenn catheterization. Both SVR trial data and institutional data showed worse 1-year transplant-free survival for patients with greater shunt resistance (smaller shunts), suggesting a survival benefit to a bigger aortopulmonary shunt. Only patients with aortopulmonary shunts were used for the analysis, so extrapolation of findings to the now more prevalent ventriculopulmonary conduit approach is not straightforward. Targeting optimal tissue oxygen delivery, the best early survival after stage 1 palliation is enjoyed by patients with high cardiac output and low systemic resistance in the early postoperative period.2Hoffman G.M. Scott J.P. Ghanayem N.S. Stuth E.A. Mitchell M.E. Woods R.K. et al.Identification of time-dependent risks of hemodynamic states after stage 1 Norwood palliation.Ann Thorac Surg. 2020; 109: 155-162Abstract Full Text Full Text PDF PubMed Scopus (11) Google Scholar A predictable nadir in cardiac output in the early postoperative hours challenges the ability to optimally lower systemic resistance, at risk of organ malperfusion. Excessively low aortopulmonary shunt resistance in the early postoperative period may exacerbate low output, coronary steal, and contribute to early mortality.3Bartram U. Grünenfelder J. Praagh R.V. Causes of death after the modified Norwood procedure: a study of 122 postmortem cases.Ann Thorac Surg. 1997; 64: 1795-1802Abstract Full Text Full Text PDF PubMed Scopus (232) Google Scholar,4Migliavacca F. Pennati G. Dubini G. Fumero R. Pietrabissa R. Urcelay G. et al.Modeling of the Norwood circulation: effects of shunt size, vascular resistances, and heart rate.Am J Physiol Heart Circ Physiol. 2001; 280: H2076-H2086Crossref PubMed Google Scholar The institutional data of the current study include only patients surviving to pre-Glenn catheterization and do not exclude the possibility of a survivorship bias that may hide an early disadvantage of a big shunt even as it celebrates a late advantage. Collapse may in fact be averted in the early postoperative state for some patients with more restrictive aortopulmonary shunts. Temporarily applying shunt restriction in the early postoperative period occasionally adjusts the distribution of flow favorably when the systemic and pulmonary resistances are in dynamic mismatch. After the nadir has passed, there is more stroke volume to share between vascular beds, and sensitivity to shunt size lessens. In the low cardiac output phase of early recovery, a big shunt may be a liability, even as it is advantageous later. Those with robust cardiac output can tolerate a bigger shunt, and secondary benefit of tolerating a bigger shunt may also be better pulmonary artery growth. Tolerance of low shunt resistance may be a proxy for identifying those with best cardiac output, and cardiac output likely has more to do with outcome than does shunt size. Shunt resistance is associated with clinically important outcomes after the Norwood operationJTCVS OpenVol. 9PreviewIn single-ventricle physiology, focus on pulmonary vascular resistance neglects the resistance in the conduit supplying the pulmonary inflow. Full-Text PDF Open Access

BACKGROUND:Congenitally corrected transposition of the great arteries (ccTGA) has many management strategies, with the emergence of anatomic repair increasing the available surgical options. Contemporary surgical practices have not been described in multicenter analyses. This study describes the distribution of heart surgery in patients with ccTGA and defines contemporary outcomes in a large multicenter cohort.METHODS:Index cardiovascular operations in patients with primary or fundamental diagnosis of ccTGA were identified in The Society of Thoracic Surgeons Congenital Heart Surgery Database from 2010 to 2019. Operations of interest were combined into mutually exclusive groups designating overall ccTGA management strategies. Outcomes were defined with standard Society of Thoracic Surgeons Congenital Heart Surgery Database definitions. Pearson χ2 and Kruskal-Wallis tests were used for statistical comparisons.RESULTS:One hundred one centers performed 985 index operations, with anatomic repair the most common approach. Twenty-six centers performed more than 10 operations. Atrial switch plus Rastelli operations had the highest rate of operative mortality (8.4%) and major complications (38.2%). Heart transplant operations had the longest postoperative length of stay among survivors (18 days [interquartile range, 13.5-26]).CONCLUSIONS:Patients with ccTGA remain a challenging cohort, with significant diversity in the operations used and a substantial burden of operative mortality and morbidity.