--Connecting Transcriptomics with Computational Modeling to Reveal Developmental Adaptations in Pediatric Human Atrial Tissue.

Nearly 1% of babies are born with congenital heart disease (CHD) - many of whom will require heart surgery within the first few years of life. A detailed understanding of cardiac maturation can help to expand our knowledge on cardiac diseases that develop during gestation, identify age-appropriate drug therapies, and inform clinical care decisions related to surgical repair and postoperative management. Yet, to date, our knowledge of the temporal changes that cardiomyocytes undergo during postnatal development is limited. In this study, we collected right atrial tissue samples from pediatric patients (n=117) undergoing heart surgery. Patients were stratified into five age groups. We measured age-dependent adaptations in cardiac gene expression, and used computational modeling to simulate action potential and calcium transients. Enrichment of differentially expressed genes (DEGs) revealed age-dependent changes in several key biological processes (e.g., cell cycle, structural organization), cardiac ion channels, and calcium handling genes. Gene-associated changes in ionic currents exhibited age-dependent trends, with changes in calcium handling (INCX) and repolarization (IK1) most strongly associated with an age-dependent decrease in the action potential plateau potential and increase in triangulation, respectively. We observed a shift in repolarization reserve, with lower IKr expression in younger patients, a finding potentially tied to an increased amplitude of IKs that could be triggered by elevated sympathetic activation in pediatric patients. Collectively, this study provides valuable insights into age-dependent changes in human cardiac gene expression and electrophysiology, shedding light on molecular mechanisms underlying cardiac maturation and function throughout development.

Reconstruction of Fibrous Skeleton of the Heart for Double-Valve Replacement in A Pediatric Patient - Modified Commando Procedure.

The so-called Commando procedure, initially described by David and colleagues, consists in the reconstruction of the mitro-aortic fibrous lamina by a patch that enlarges both annuli. Its use has been described to upsize the aortic and mitral annulus for double valve replacement in adolescents. We describe a modified technique of this reconstruction of the fibrous skeleton of the heart, combined with Konno procedure to further enlarge the aortic annulus. In modified Commando procedure, following the reconstruction of aortomitral continuity with a bovine pericardium CardioCel patch (Admedus Regen Pty Ltd, Perth, WA, Australia), an aortic valved conduit that was made on the bench in order to have bottom skirt that enabled the suturing of the composite conduit far inside the left ventricle outflow tract. Coronary buttons were implanted at the supra-commissural level. The advantages of this modified Commando procedure are (1) the creation of a new aortic annulus when the integrity of this annulus has been compromised, (2) the upsizing of both annuli to any possible size of aortic and mitral prostheses, and (3) the relief of any residual left ventricular outflow tract obstruction.

The Children's National Hospital Outcomes Registry: a plea for a benchmarking tool that provides longitudinal outcomes for patients and families.

Surgical advancements in paediatric cardiovascular surgery have led to improved survival rates for those patients with the most complex CHDs leading to greater numbers of patients who are living well into adulthood. Despite this new era of long-term survival, our current reporting systems continue to focus largely on using short-term postoperative outcomes as the criteria to both rate and rank hospitals. Using such limited criteria to rate and rank hospitals may mislead the intended audiences: patients and families. The goal of this article is to describe the creation of a local benchmarking report which aims to retrospectively review long-term outcomes from our single centre. This report is updated annually and published on our cardiac surgery webpage in an effort to be as transparent as possible for our patient and family communities.

Pediatric Tele-Critical Care: Initial Experience with a Continuous Surveillance Model Aiming to Prevent Cardiac Arrest in Children with Critical Heart Disease.

Introduction: Despite advances in treatment of children with critical heart disease, cardiac arrest (CA) remains a common occurrence. We provided virtual support to bedside teams (BTs) from a tele-critical care (TCC) unit in a pediatric cardiac intensive care unit (CICU) and focused on early detection of concerning trends (CT) and avoidance of CA. Virtual surveillance workflows included a review of remote monitoring, video feed from patient room cameras, medical records, and artificial intelligence tools. We present our initial experience with a focus on critical communications (CCs) to BTs. Methods: A retrospective, descriptive review of TCC activities was conducted from January 2019 to December 2022, involving electronic databases and electronic medical records of patients in the CICU, including related CCs to BTs, responses from BTs, and related CA. Results: We conducted 18,171 TCC activities, including 2,678 non-CCs and 248 CCs. Over time, there was a significant increase in the proportion of CCs related with CT (p = 0.002), respiratory concerns (<0.001), and abnormalities in cardiac rhythm (p = 0.04). Among a sample of 244 CCs, subsequent interventions by BTs resulted in adjustment of medical treatment (127), respiratory support (68), surgery or intervention (19), cardiac rhythm control (17), imaging study (14), early resuscitation (9), and others (10). Conclusions: CCs from a TCC unit in a pediatric CICU changed over time with an increased focus on CT and resulted in early interventions, potentially contributing to avoiding CA. This model of care in pediatric cardiac critical care has the potential to improve patient safety.

Cerebral Sinus Venous Thrombosis in Infants after Surgery for Congenital Heart Disease.

OBJECTIVE: To determine the prevalence of and risk factors for cerebral sinus venous thrombosis (CSVT) in neonates undergoing congenital heart disease (CHD) repair. STUDY DESIGN: Neonates who had CHD repair with cardiopulmonary bypass and postoperative brain magnetic resonance imaging (MRI) between 2013 and 2019 at a single tertiary care center were identified from institutional databases. Demographic, clinical, and surgical data were abstracted from these databases and from the medical record; 278 neonates with CHD had cardiopulmonary bypass, 184 of whom had a postoperative brain MRI. RESULTS: Eight patients (4.3%) had a CSVT. Transposition of the great arteries with an intact ventricular septum (P < .01) and interrupted aortic arch (P = .02) were associated with an increased risk for CSVT. Other risk factors for CSVT included cross-clamp time (98 [IQR, 77.5-120] minutes vs 67 [IQR, 44-102] minutes; P = .03), units of platelets (3.63 [IQR, 3-4] vs 2.17 [IQR, 1-4]; P < .01) and packed red blood cells (0.81 [IQR, 0.25-1] vs 1.21 [IQR, 1-1]; P = .03) transfused intraoperatively, and time between surgery and MRI (10 [IQR, 7-12.5] days vs 20 [IQR, 12-35] days; P < .01). Five patients (62.5%) were treated with anticoagulation. All patients had complete or partial resolution of their CSVT, regardless of treatment. CONCLUSIONS: Brain MRI after cardiopulmonary bypass in neonates revealed a low prevalence of CSVT (4.3%). Further studies are needed to establish best practices for surveillance, prevention, and treatment of CSVT in this population.

Predictors of Neurological Outcome Following Infant Cardiac Surgery Without Deep Hypothermic Circulatory Arrest.

The aim of this study is to describe the clinical characteristics, perioperative course and neuroimaging abnormalities of infants with congenital heart disease (CHD) undergoing heart surgery without deep hypothermic circulatory arrest (DHCA) and identify variables associated with neurological outcome. Infants with CHD undergoing open-heart surgery without DHCA between 2009 and 2017 were identified from a cardiac surgery database. Full-term infants < 10 weeks of age at the time of surgery who had both a pre- and postoperative brain magnetic resonance imaging exam (MRI) were included. Clinical characteristics and perioperative variables were collected from the electronic medical record. Brain Injury Scores (BIS) were assigned to pre- and postoperative brain MRIs. Variables were examined for association with neurological outcome at 12 months of age or greater. Forty-two infants were enrolled in the study, of whom 69% (n = 29) had a neurological assessment ≥ to 12 months of age. Adverse neurological outcome was associated with longer intensive care unit (ICU) stay (P = 0.003), lengthier mechanical ventilation (P = 0.031), modified Blalock-Taussig (MBT) shunt procedure (P = 0.005) and postoperative seizures (P = 0.005). Total BIS scores did not predict outcome but postoperative infarction and/or intraparenchymal hemorrhage (IPH) was associated with worse outcome by multivariable analysis (P = 0.018). Infants with CHD undergoing open-heart surgery without DHCA are at increased risk of worse neurological outcome when their ICU stay is prolonged, mechanical ventilation is extended, MBT shunt is performed or when postoperative seizures are present. Cerebral infarctions and IPH on postoperative MRI are also associated with worse outcome.

Determinants of neurological outcome in neonates with congenital heart disease following heart surgery.

BACKGROUND: The objective was to determine the association between perioperative risk factors and brain imaging abnormalities on neurologic outcome in neonates with hypoplastic left heart syndrome (HLHS) or d-Transposition of the great arteries (d-TGA) who underwent cardiac surgery including cardiopulmonary bypass. METHODS: A retrospective analysis of neonates with HLHS or d-TGA undergoing cardiac surgery including cardiopulmonary bypass between 2009 and 2017 was performed. Perioperative risk factors and Andropoulos' Brain Injury Scores from pre- and postoperative brain magnetic resonant images (MRI) were correlated to outcome assessments on patients between 5 and 23 months of age. Neurologic outcome was measured using the Pediatric Stroke Outcome Measure (PSOM) and Pediatric Version of the Glasgow Outcome Scale-Extended (GOS-E). RESULTS: Fifty-three neonates met our enrollment criteria (24 HLHS, 29 d-TGA). Mechanical ventilation > 12 days and DHCA > 40 min were associated with worse outcome. MRI measures of brain injuries were not associated with worse outcome by PSOM or GOS-E. CONCLUSION: For HLHS and d-TGA patients, duration of mechanical ventilation and DHCA are associated with adverse neurologic outcome. Neonatal brain MRI commonly demonstrates acquired brain injuries, but the clinical impact of these abnormalities are not often seen before 2 years of age. IMPACT: Acquired brain injury is common in high-risk neonates with CHD but poor neurological outcome was not predicted by severity of injury or lesion subtype. Longer stay in ICU is associated with postoperative brain injuries on MRI. Total duration of ventilation > 12 days is predictive of adverse neurological outcome scores. DHCA > 40 min is associated with adverse neurological outcome scores. Neurological outcome before 2 years of age is more affected by the clinical course than by cardiac diagnosis.

Surgical pericardial adhesions do not preclude minimally invasive epicardial pacemaker lead placement in an infant porcine model.

BACKGROUND: Pericardial adhesions in infants and small children following cardiac surgery can impede access to the epicardium. We previously described minimally invasive epicardial lead placement under direct visualization in an infant porcine model using a single subxiphoid incision. The objective of this study was to assess the acute feasibility of this approach in the presence of postoperative pericardial adhesions. METHODS: Adhesion group piglets underwent left thoracotomy with pericardiotomy followed by a recovery period to develop pericardial adhesions. Control group piglets did not undergo surgery. Both groups underwent minimally invasive epicardial lead placement using a 2-channel access port (PeriPath) inserted through a 1 cm subxiphoid incision. Under direct thoracoscopic visualization, pericardial access was obtained with a 7-French sheath, and a pacing lead was affixed against the ventricular epicardium. Sensed R-wave amplitudes, lead impedances and capture thresholds were measured. RESULTS: Eight piglets underwent successful pericardiectomy and developed adhesions after a median recovery time of 45 days. Epicardial lead placement was successful in adhesion (9.5 ± 2.7 kg, n = 8) and control (5.6 ± 1.5 kg, n = 7) piglets. There were no acute complications. There were no significant differences in capture thresholds or sensing between groups. Procedure times in the adhesion group were longer than in controls, and while lead impedances were significantly higher in the adhesion group, all were within normal range. CONCLUSIONS: Pericardial adhesions do not preclude minimally invasive placement of epicardial leads in an infant porcine model. This minimally invasive approach could potentially be applied to pediatric patients with prior cardiac surgery.

Levels of agreement between cardiac magnetic resonance and conductance catheter measurements of right ventricular volumes after pulmonary artery banding.

BACKGROUND: Pressure-volume analysis is the gold standard for quantifying pump function of the right ventricle (RV); however, volume measurements based on a conductive catheter may be imprecise. The reference method for volume assessment is cardiac magnetic resonance (CMR). PURPOSE: To determine the levels of agreement between RV volume measurements obtained by cine CMR, phase-contrast CMR (PC CMR), and a conductance catheter in an animal model. MATERIAL AND METHODS: CMR was performed in 20 sheep three months after pulmonary artery banding. Ejection fraction (EF), end-diastolic (EDV), end-systolic (ESV), and stroke volumes (SV) were obtained by cine CMR and conductance catheter. RESULTS: Statistically significant differences between cine CMR and conductance catheter derived volume measurements were found for EDV (P < 0.001), ESV (P < 0.05), and SV (P < 0.05). Bland-Altman analysis showed very poor agreement between the two methods: EDV, bias 36.27 mL, agreement of limits 1.96-70.57 mL; ESV, bias 15.33 mL, agreement of limits -6.89-37.55 mL; and SV, bias 20.69 mL, agreement of limits 8.01-49.10 mL. Good agreement was found for SV between cine CMR and PC CMR (bias -7.0 mL, agreement of limits -24.01-9.98 mL), while SV derived from PC CMR measurements showed poor agreement with conductance catheter (bias 27.76 mL, agreement of limits -3.84-59.26 mL). CONCLUSION: Poor agreement between the conductance catheter and CMR RV volume measurements was found. PC CMR and cine CMR measurements of SV agreed well.

Endocardial Fibroelastosis of the Left Ventricle Affects Right Ventricular Performance in Fetuses with Hypoplastic Left Heart Syndrome: A Prospective Study Using M-Mode, PW- and Tissue Doppler Techniques.

PURPOSE: Myocardial function (MF) of the systemic right ventricle (RV) influences the postnatal course of neonates with hypoplastic left heart syndrome (HLHS). Our study examines whether the presence of endocardial fibroelastosis of the left ventricle (LV EFE) influences MF of the RV in HLHS fetuses. MATERIALS AND METHODS: A prospective study was conducted including 10 controls (group 1), 10 HLHS fetuses with (group 2) and 10 without LV EFE (group 3) - all matched for gestational age. M-mode was used to assess tricuspid plane systolic excursion (TAPSE) and the shortening fraction (SF). PW-Doppler-derived and PW-TDI-derived velocities were assessed. E/A, E/e', e'/a' ratios and the myocardial performance index (mpi') were calculated. RESULTS: The examination of MF revealed significantly lower s' velocities (p < 0.05) and higher values for SF in group 2 compared to group 3. e'/a' ratio, et' (ejection time), E wave velocity, E/e' and SF showed significantly higher values in group 2 compared to group 1. In group 2 a' velocity increased significantly over gestational age. In group 3 but not in group 2, TAPSE increased during gestation. CONCLUSION: These significant differences in MF between the groups might lend support to the notion of negative ventricular-ventricular interaction in the case of HLHS with LV EFE possibly influencing surgical outcomes.

Ninety-two days of extracorporeal membrane oxygenation support following congenital heart surgery.

We report on a case involving a 10-month-old infant who received prolonged ECMO therapy following cardiac surgery for multiple ventricular septal defects (VSD). The patient was successfully weaned from 92 days of ECMO support without any long-term deficits.

Prenatal diagnosis of functionally univentricular heart, associations and perinatal outcomes.

OBJECTIVE: Functionally univentricular hearts (UVHs) represent cardiac anomalies in which either the pulmonary or systemic circulation cannot be supported independently. The purpose of our study was to determine perinatal outcomes after prenatal diagnosis of functionally UVH. METHODS: We retrospectively evaluated patients who presented between 2008 and June 2015 in our centre and in prenatal practice praenatal.de in Cologne. We included double inlet left ventricle (DILV), tricuspid valve atresia (TA), pulmonary valve atresia and intact ventricular septum (PA:IVS), unbalanced atrioventricular septal defect (AVSD), heterotaxy, hypoplastic left heart syndrome (HLHS) and hypoplastic left heart complex (HLHC). RESULTS: Of initially 155 patients, 128 were liveborn (82.6%). Ten neonates (7.8%) were lost to follow-up, in three (2.5%) neonates, parents decided for compassionate care. Overall survival after prenatal diagnosis of functionally UVH was 67.1%, and 90.4% on an intention-to-treat basis. Survival after surgery reached 93.7%. The majority of deaths occurred within the group of dominant RV (10/74, 13.5%). High risk HLHS with restrictive foramen ovale was associated with the lowest survival rate (13/17, 76.5%) with significant difference compared to survival rate in dominant LV (40/41, 97.6%, p < 0.05). CONCLUSION: These results should be explained to parents to ensure informed decisions and counselling. © 2016 John Wiley & Sons, Ltd.

Mid-Term Results of Congenital Supravalvar Mitral Ring Resection.

BACKGROUND: Supravalvar mitral ring is a rare congenital anomaly leading to left ventricle inflow obstruction. We present our surgical experience and mid-term results in patients operated for supravalvar mitral ring and associated congenital heart defects. MATERIALS AND METHODS: A retrospective analysis was performed in order to evaluate the cases surgically treated with the diagnosis of supravalvar mitral ring between 2001 and 2014. Ten patients were identified, seven of whom had accompanying congenital heart defects. Median age at the operation was 4.5 years. RESULTS: Ventricular septal defects were encountered in half of the cases. Two of the patients had mitral annular hypoplasia, another two had the components of Shone's complex. The mean preoperative gradient across the supravalvar mitral ring decreased from 14.1 ± 4.2 mmHg to 4.6 ± 2.2 mmHg. All patients are alive and remain in a good clinical condition after a mean follow-up of five years. CONCLUSION: Supravalvar mitral ring is a surgically treatable cause of left ventricular inflow obstruction. Although residual gradients may be encountered in patients with mitral annular hypoplasia, surgical resection of the ring is encouraged.

Reverse anastomosis technique for the treatment of a rare coronary artery anomaly in D-transposition of the great arteries.

Arterial switch operation remains the gold standard surgical therapy for D-transposition of the great vessels. Coronary anomalies may complicate the surgical strategy and lead to an increased morbidity and mortality. However, as of today, even challenging coronary anomalies should not be a contraindication for the performance of complete repair. Here, we describe a technique for the translocation of an intramural, high-origin conal branch in a 5-month-old patient with D-transposition of the great vessels and ventricular septal defect.

Development and assessment of novel assist device for cardiac cannulation.

PURPOSE: Congenital heart defects are the most common birth defects in the USA and in 25% of cases need to be treated with cardiovascular interventions. One of such interventions is the postoperative use of an extracorporeal membrane oxygenation (ECMO) machine for the treatment of cardiorespiratory failure. The process of placing the patient on the ECMO is extremely time-critical and requires the use of cardiac cannulation. For the first time, our team developed and evaluated a new quick-connect cannulation system that allows for rapid, easy, and safe ECMO cannulation in the pediatric population. The design should eliminate the need for purse-string sutures that are currently used to secure cannulas, as the cannulas will be inserted through a port that is glued to the cardiovascular tissue. METHODS: The rapid cannulation assistance device was designed on the SolidWorks computer-aided design software using the dimensions of the commercially available arterial and venous catheters. These designs were then 3D printed, and tensile testing was performed. Then, a flow loop was developed, and cannulation was performed and analyzed on both 3D-printed hearts and porcine hearts. RESULTS: The rapid cannulation assistance device was designed and 3D printed. Tensile testing found that the parts were strong enough to withstand forces that may be introduced in studies. 3D-printed and porcine heart tests with a flow loop found no leakage with the 3D-printed hearts but minimal leaking with the porcine hearts. However, this leakage was observed at the junction between the device and the heart, leading us to believe that a glue better suited to attach the device to the heart would prevent leakage in the future. CONCLUSIONS: This project successfully demonstrated how a rapid cannulation assistance device could be developed and tested. Future studies will be conducted that address device adhesion to the cardiovascular tissue so that accurate pressure and flow rates can be measured. Future studies will also include testing the device in a fluid environment to more effectively analyze the device success and comparing the time required to cannulate using our device compared to the standard of care.

Excellent Survival With a Focus on Pulmonary Artery Reinterventions After the Arterial Switch Operation.

Background: We sought to evaluate the outcomes in patients who underwent the arterial switch operation (ASO) over a 20-year period at a single institution. Methods: The current study is a retrospective review of 180 consecutive patients who underwent the ASO for biventricular surgical correction of dextro-transposition of the great arteries (d-TGA) between 2002 and 2022. Results: Among 180 patients, 121 had TGA-intact ventricular septum, 47 had TGA-ventricular septal defect and 12 had Taussig-Bing Anomaly (TBA). The median follow-up time was 6.7 years (interquartile range: 3.9-8.7 years). There were five early (2.8%) and one late (0.6%) mortality. Survival was 96.6% at one year and beyond. Reoperations were performed in 31 patients (17%). Taussig Bing Anomaly was found to increase the risk of reoperation by 17 times (P < .0001). A total of 37 (21%) patients underwent 53 reinterventions (14 surgical procedures, 39 catheter interventions) specifically addressing pulmonary artery (PA) stenosis. Freedom from PA reintervention was 97%, 87%, 70%, and 55% at 1, 5, 10, and 15 years, respectively. By bivariable analysis, TBA (P = .003, odds ratio [OR]: 6.4, 95% confidence interval [CI]: 1.9-21.7), mild PA stenosis at discharge (P ≤ .001, OR: 6.1, 95% CI: 2.7-13.6), and moderate or severe PA stenosis at discharge (P ≤ .001, OR: 12.7, 95% CI: 5-32.2) were identified as predictors of reintervention on PA. In the last follow-up of 174 survivors, 24 patients (14%) had moderate or greater PA stenosis, two (1%) had moderate neoaortic valve regurgitation, and 168 were New York Heart Association status I. Conclusions: Our results demonstrated excellent survival and functional status following the ASO for d-TGA; however, patients remain subject to frequent reinterventions especially on the pulmonary arteries.

Young infants with symptomatic tetralogy of Fallot: Shunt or primary repair?

Objectives: The optimal treatment strategy for symptomatic young infants with tetralogy of Fallot (TOF) is unclear. We sought to compare the outcomes of staged repair (SR) (shunt palliation followed by second-stage complete repair) versus primary repair (PR) at 2 institutions that have exclusively adopted each strategy. Methods: We performed propensity score-matched comparison of 143 infants under 4 months of age who underwent shunt palliation at one institution between 1993 and 2021 with 122 infants who underwent PR between 2004 and 2018 at another institution. The primary outcome was mortality. Secondary outcomes were postoperative complications, durations of perioperative support and hospital stays, and reinterventions. Median follow-up was 8.3 years (interquartile range, 8.1-13.4 years). Results: After the initial procedure, hospital mortality (shunt, 2.8% vs PR, 2.5%; P = .86) and 10-year survival (shunt, 95%; 95% confidence interval [CI], 90%-98% vs PR, 90%; 95% CI, 81%-95%; P = .65) were similar. The SR group had a greater risk of early reinterventions but similar rates of late reinterventions. Propensity score matching yielded 57 well-balanced pairs. In the matched cohort, the SR group had similar freedom from reintervention (55%; 95% CI, 39%-68% vs 59%; 95% CI, 43%-71%; P = .85) and greater survival (98%; 95% CI, 88%-99.8% vs 85%; 95% CI, 69%-93%; P = .02) at 10 years, as the result of more noncardiac-related mortalities in the PR group. Conclusions: In symptomatic young infants with TOF operated at 2 institutions with exclusive treatment protocols, the SR strategy was associated with similar cardiac-related mortality and reinterventions as the PR strategy at medium-term follow-up.

Alarming rate of liver cirrhosis after the small conduit extracardiac Fontan: A comparative analysis with the lateral tunnel.

BACKGROUND: The association between the prevalence of cirrhosis and the types of Fontan operations remains unclear. METHODS: We conducted a retrospective chart review of 332 patients who underwent the Fontan procedure at our institution. Four patients who underwent the atriopulmonary connection Fontan were excluded from the analysis. Patients who had intracardiac-extracardiac conduit (126) between 1989 and 2021 were pooled with those having extracardiac conduit (ECC) (134). The 260 patients who underwent the ECC and the 68 patients who had the lateral tunnel (LT) Fontan constitute the core of the study. RESULTS: Median age at the Fontan procedure was 23.7 months (interquartile range [IQR], 20.8-32.6) in the LT group, compared with 28.8 months (IQR, 24.6-39.5) in the ECC group (P < .01). The median follow-up was 14.8 years (IQR, 12.5-16.5) in the LT group and 7 years (IQR, 2.8-10.4) in the extracardiac conduit group. During the follow-up period, 3 patients (4.4%) with LT and 17 patients (6.5%) with ECC (11 patients with 16 mm or less conduit size) were diagnosed with cirrhosis. The prevalence of cirrhosis at 1, 5, 10, and 15 years was 0%, 0%, 0%, and 4.4% in the LT group, respectively, and 0%, 0.9%, 7.7%, and 29.8% in the ECC group (P < .01) Rates of mortality, Fontan revision, Fontan takedown, transplant, and complications were comparable between the 2 groups. CONCLUSIONS: The extracardiac conduit Fontan seems to be associated with faster development of cirrhosis.

Left Atrioventricular Valve Regurgitation After Atrioventricular Septal Defect Repair.

BACKGROUND: Whether patients with moderate left atrioventricular valve regurgitation (LAVVR) after surgical repair of complete atrioventricular septal defect (CAVSD) should be observed or undergo reoperation remains unclear. METHODS: Moderate LAVVR was diagnosed in 87 of 220 patients who underwent CAVSD repair: 47 during the initial hospital stay and 40 after a median of 7 months (interquartile range, 2-18 months) after the initial operation. RESULTS: Of these 87 patients who had moderate LAVVR, 15 died, for an overall mortality of 17%. The regurgitation became severe in 39 patients (45%) within a median of 2 months (interquartile range, 1-7 months) leading to 33 reoperations and 10 deaths. In 23 of 87 patients (26%), regurgitation remained at a moderate level over a median follow-up period of 8 months (interquartile range, 1-48 months). In 25 of 87 patients (29%), the regurgitation decreased to mild after a median of 9 months (interquartile range, 5-19 months). The only independent risk factor for increased severity of regurgitation and reoperation was the echocardiographic appearance of the jet centered around the cleft rather than central at the time of diagnosis of moderate regurgitation (odds ratio, 3.5; 95% CI, 1.5-9.0; P = .007). CONCLUSIONS: Moderate LAVVR after CAVSD repair is often linked to death and reoperation, but regurgitation remains stable in one-quarter of patients and improves in one-third. The deterioration usually occurs within the first year after surgery. The initial observation of patients with residual or new moderate regurgitation for up to 1 year or until further deterioration seems reasonable, as long as the regurgitation is centrally located.