Double Inlet Left Ventricle Research Articles

7068 Paraganglioma in Cyanotic Congenital Heart Disease

Abstract Disclosure: R. Lis: None. V. Hamidi: None. Paragangliomas arise from the non-chromaffin cells of the parasympathetic nervous system, which are composed of glomus cells. These function as chemoreceptors in response to hypoxia. Carotid body paragangliomas have been linked to conditions with chronic hypoxia. This was first seen in residents of high altitude locations in a case report presented by Saldana et. al and regression of a paraganglioma with resolution of hypoxia was reported by Gruber et. al. There have been few additional case reports of Paraganglioma in other hypoxic states. We present a rare case of a functional Paraganglioma in a patient with Cyanotic Congenital Heart Disease (CCHD).This is a 19 year old female with a history of CCHD due to double inlet left ventricle and transposition of the great arteries. Soon after birth, she had a fontan procedure. She subsequently developed fontan associated liver disease as well as chronic kidney disease, hypertension, sinus node dysfunction, and complete heart block. Her baseline oxygen saturation was 78-85%. Her heart rate was 40-49 beats per minute with upcoming plans for pacemaker placement. Her blood pressure ranged 90/50 to 150/80 mmHg on Amlodipine 5 mg and Lisinopril 2.5 mg. She routinely received abdominal imaging to monitor her liver disease. A liver MRI incidentally revealed a retroperitoneal mass concerning for a paraganglioma. Serum Normetanephrines were found to be 26.60 nmol/L (reference range less than 0.89). 24 hr Urine Norepinephrine was 1,187 ug/d (reference range 14-20). Further imaging was negative for additional sites of disease. Given her complete heart block, she was started on alpha blockade only prior to pacemaker placement. After the pacemaker was placed, she was started on beta blockade and underwent surgical resection of her paraganglioma. The pathology confirmed paraganglioma and mutation testing was negative for germline mutations including SDH, VHL, RET, NF1 mutations. Paragangliomas in patients with CCHD have been scarcely described in the literature given the rarity of both of these conditions. However, there is a relatively increased incidence of paragangliomas in the CCHD population due to chronic hypoxia triggering the hypoxia pathway. EPAS1 mutated HIF2alpha is often found to be the culprit in these patients. In addition, case reports have often described these patients to either be asymptomatic or have potential symptoms masked by their cardiac disease. Therefore, CCHD patients are at increased risk for paragangliomas and warrant additional awareness and regular screening. Screening has been proposed to start after 10 years of hypoxia in a case review by Agarwal et. al. as paraganglioma incidence in CCHD populations has not been seen before age 11. Incidence of recurrence is not yet widely studied, but significant concern for recurrence is warranted if the etiology of hypoxia is not reversed. Presentation: 6/3/2024

Double inlet left ventricle: Comprehensive echocardiographic anatomy evaluation with special focus on the morphology of atrio-ventricular valves and ventricular septal defect: Case report and literature review

In double inlet left ventricle (DILV), the ventricle most frequently has left ventricle (LV) morphology, although right ventricle (RV), mixed, indeterminate, or undifferentiated morphologies have been reported. The main ventricle is largely a morphological LV with an outlet chamber connected to it which has a RV morphology. Ventricular septal defect (VSD) or bulboventricular foramen size and anatomy may be variable. The AV valves may be normal, or one of them may be hypoplastic, stenotic, or even atretic. The great arteries are most frequently transposed and the aorta arises from the hypoplastic RV, and the pulmonary artery comes off the main LV chamber. L-transposition of great arteries (L-TGA) happens more often than d-transposition of great arteries (D-TGA). The great vessels are normally related in 30% of cases. Double-outlet right ventricle (DORV) may be seen, in which both great vessels arise from the rudimentary RV. Pulmonary stenosis (PS) is present in two thirds of patients and such stenosis is seen irrespective of the great artery relationship. Rarely subaortic obstruction maybe present in patients with transposition of the great arteries. Here, we are presenting a case of complex cyanotic congenital cardiac defect of DILV with VSD complicated by L-TGA and severe tricuspid regurgitation (TR) in a 3-year-old male toddler.

Anatomical and physiological diagnostic discrepancies in fetuses with single-ventricle congenital heart disease in a contemporary cohort.

Image quality of fetal echocardiography (FE) has improved in the recent era, but few recent studies have reported the accuracy of FE, specifically in single ventricle (SV) congenital heart disease (CHD). This study aimed to assess the ability of FE to correctly predict SV-CHD postnatal anatomy and physiology in a contemporary cohort. The contemporary clinical reports of patients with SV-CHD, in which FE was performed between July 2017 and July 2021, were compared with postnatal echocardiograms from a formal quality assurance program. SV fetuses were grouped by anatomical subtype. Diagnostic errors were designated as major if the error would have caused significant alteration in parental counseling or postnatal management. The remaining errors were classified as minor. Physiological discrepancies, including prostaglandin-E (PGE) dependency, atrioventricular valve regurgitation (AVVR), pulmonary venous obstruction, and restrictive atrial septum (RAS) were assessed by chart review of the postnatal course. A total of 119 subjects were analyzed. SV subtypes in the cohort included hypoplastic left heart syndrome (HLHS) (n = 68), tricuspid atresia (n = 16), double-inlet left ventricle (n = 12), unbalanced atrioventricular canal (UAVC) (n = 11), heterotaxy (n = 9) and other (n = 3). The rate of major anatomical and physiological errors was low (n = 6 (5.0%)). A higher proportion of minor errors was noted in HLHS and tricuspid atresia, but the differences were not statistically significant. Physiological discrepancies were uncommon, with three major discrepancies, including underestimation of the degree of venous obstruction in one non-HLHS fetus with total anomalous pulmonary venous return, overestimation of RAS in one HLHS fetus and incorrect prediction of PGE dependency in one case false-negative for pulmonary blood flow. No discrepancy in degree of AVVR or RAS affected postnatal care. Minor physiological discrepancies included two false-positive predictions of PGE dependency with one false-positive for ductal-dependent systemic flow and one false-positive for pulmonary blood flow. In this contemporary review of FE at our center, there was high accuracy in describing anatomical and physiological findings in SV-CHD. Major physiological discrepancies were uncommon but included important cases of false-negative prediction of PGE dependency and underestimation of obstruction of total anomalous pulmonary venous return. These data can inform more accurate counseling of families with SV-CHD fetuses and guide diagnostic improvement efforts. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.

Atrioventricular valve surgery in patients with univentricular heart and two separate atrioventricular valves.

Atrioventricular valve regurgitation in patients with univentricular heart is a well-known risk factor for adverse outcomes and atrioventricular valve repair remains a particular surgical challenge. We reviewed all surgical atrioventricular valve procedures in patients with univentricular heart and two separate atrioventricular valves who underwent surgical palliation. Endpoints of the study were reoperation-free survival and cumulative incidence of reoperation. Between 1994 and 2021, 202 patients with univentricular heart and two separate atrioventricular valve morphology underwent surgical palliation, with 15.8% (32/202) requiring atrioventricular valve surgery. Primary diagnoses were double inlet left ventricle (n = 14, 43.8%), double outlet right ventricle (n = 7, 21.9%), and congenitally corrected transposition of the great arteries (n = 7, 21.9%). Median weight at valve surgery was 10.6 kg (interquartile range, 7.9-18.9). Isolated left or right atrioventricular valve surgery was required in nine (28.1%) and 22 patients (68.8%), respectively. Concomitant left and right atrioventricular valve surgery was performed in one patient (3.1%). Closure of the left valve was conducted in four patients (12.5%) and closure of the right valve in three (9.4%). Operative and late mortality were 3.1% and 9.7%, respectively. Reoperation-free survival and cumulative incidence of reoperation at 10 years after surgery were 62.3% (standard error of the mean: 6.9) and 30.9% (standard error of the mean: 9.6), respectively. In patients with univentricular heart and two separate atrioventricular valves, surgical intervention on these valves is required in a minority of patients and is associated with low mortality but high incidence of reoperation.

Quantifying Additional Procedures in Functionally Single-Ventricle Disease: A National Cohort Study

BackgroundGiven their importance as a metric for health care evaluation, this study’s aim was to evaluate the rates of surgical and catheter reinterventions for children with functionally single-ventricle (f-SV) congenital heart disease (CHD) undergoing staged palliation. MethodsWe undertook a retrospective cohort study of children born with f-SV CHD between 2000 and 2018 in England and Wales, using the national registry, with survival ascertained in 2020. Competing risk analysis was used to describe the incidence of additional procedures that occurred first, during follow-up, accounting for competing events of death or transplantation. ResultsOf 56,039 patients who received an intervention for CHD, 3307 (5.9%) had f-SV. The largest diagnostic subcategories were hypoplastic left heart syndrome (1266 [38.3%]), tricuspid atresia (448 [13.5%]), and double-inlet left ventricle (328 [9.9%]). During a median follow-up of 5.4 (interquartile range, 0.8-10.8) years, 921 (27.9%) patients had at least 1 additional interstage surgery and 1293 (39.1%) had at least 1 additional interstage catheter intervention. The cumulative incidence of additional surgery at 6 months after stage 1 was 17.6% (95% CI, 16.2%-19.0%); at 2 years after stage 2, 8.3% (7.2%-9.5%); and at 5 years after stage 3, 8.4% (7.0%-9.9%). The cumulative incidence of additional catheter at 6 months after stage 1 was 18.0% (16.6%-19.4%); at 2 years after stage 2, 14.7% (13.3%-16.2%); and at 5 years after stage 3, 23.7% (21.5%-26.0%). ConclusionsIt is important to quantify additional procedures for children with f-SV disease to inform parents and health professionals, potentially facilitating the development of interventions that aim to reduce these important adverse outcomes.

Comparing palliation strategies for single-ventricle anatomy with transposed great arteries and systemic outflow obstruction.

Patients with complex single-ventricle anatomy with transposed great arteries and systemic outflow obstruction (SV-TGA-SOO) undergo varied initial palliation with ultimate goal of Fontan circulation. We examine a longitudinal experience with multiple techniques, including the largest published cohort following palliative arterial switch operation (pASO), to describe outcomes and decision-making factors. Neonates with SV-TGA-SOO who underwent initial surgical palliation from 1995 to 2022 at a single institution were retrospectively reviewed. In total, 71 neonates with SV-TGA-SOO underwent index surgical palliation at a median age of 7days (interquartile range, 6-10) by pASO (n=23), pulmonary artery band (PAB) with or without arch repair (n=25), or modified Norwood with Damus-Kaye-Stansel aortopulmonary amalgamation (n=23). Single-ventricle pathology included double-inlet left ventricle (n=37, 52%), tricuspid atresia (n=27, 38%), and others (n=7, 10%). All mortalities (n=5, 7%) occurred in the first interstage period after PAB (n=3) and Norwood (n=2). Subaortic obstruction in the PAB group was addressed by operative resection (n=10 total, 7 at index operation) and/or delayed aortopulmonary amalgamation (n=13, 52%). Two patients with pASO (9%) had early postoperative coronary complications, 1 requiring operative revision. Median follow-up for survivors was 10.4years (interquartile range, 4.5-16.6years). Comparing patients by their initial palliation type, notable significant differences included size of bulboventricular foramen, weight at initial operation, operation duration, postoperative length of stay, time to second-stage palliation, multiple pulmonary artery reinterventions, and left pulmonary artery interventions. There were no significant differences in overall survival, Fontan completion, reintervention-free survival in the first interstage period, pulmonary artery reintervention-free survival, long-term systemic valve competency, or ventricular dysfunction. Excellent mid- to long-term outcomes are achievable following neonatal palliation for SV-TGA-SOO via pASO, PAB, and modified Norwood, with comparable survival and Fontan completion. Initial palliation strategy should be individualized to optimize anatomy and physiology for successful Fontan by ensuring an unobstructed subaortic pathway and accessible pulmonary arteries. pASO is a reasonable strategy to consider for these heterogeneous lesions.

Impact of pulsatile pulmonary blood flow on cardiopulmonary exercise performance after the Fontan procedure

ObjectiveTo evaluate the exercise capacity in patients following Fontan–Kreutzer, Fontan–Björk, and total cavopulmonary connection (TCPC). MethodsPatients who performed exercise capacity tests at least once after the Fontan procedure between 1979 and 2007 were included. Patients after Fontan–Björk procedure were divided into 2 groups according to the pulmonary blood flow (PBF) pattern: patients with pulsatile PBF and those without. Peak oxygen uptake (VO2) was measured and percent-predicted VO2 was calculated. ResultsA total of 227 patients were nominated. The types of Fontan procedure included Fontan–Kreutzer in 48 (21.1%) patients, Fontan–Björk in 38 (16.7%); 11 (4.8%) with pulsatile PBF and 27 (11.9%) without pulsatile PBF; and TCPC in 141 (62.1%). Median age at the Fontan procedure was 4.5 years (interquartile range, 2.1-8.2 years). A total of 978 cardiopulmonary exercise tests were performed at median follow-up of 17.7 years (interquartile range, 11.3-23.4 years) postoperatively. Analysis using linear mixed-effects models demonstrated that percent-predicted VO2 was greater in patients with pulsatile PBF after Fontan–Björk compared with patients after other types of Fontan procedure (P < .001). The same results were obtained when the longitudinal percent predicted VO2 was performed using only patients with tricuspid atresia and double inlet left ventricle (P < .001). ConclusionsAmong long-term survivors after various types of Fontan procedures, patients with pulsatile PBF after the Fontan–Björk procedure demonstrated better exercise performance compared to those after TCPC, those after the Fontan–Kreutzer procedure, and those after the Fontan-Björk procedure with non-pulsatile PBF. The results implicate the importance of pulsatile PBF to maintain the Fontan circulation.

A Tale of Intricate Connections: An Interesting Case of Congenital Heart Disease with Supraventricular Tachycardia

Double inlet left ventricle (DILV) is a rare complex congenital heart defect characterized by the presence of two atrioventricular valve orifices leading into a single left ventricle. Patients rarely survive beyond teenage years without surgical correction. Supraventricular tachycardia (SVT) in DILV is a rare occurrence that adds an additional layer of complexity to diagnosis and management. Here, we report an exceptional case of DILV who survived well into her fourth decade of life without any surgical correction. The patient presented to us with SVT which lead to the discovery of her unique cardiac anomaly.

Clinical course after Fontan palliation: Single center experience

BackgroundThe Fontan procedure has been the standard palliation for single-ventricle patients. We aimed to investigate the short- and midterm outcomes of patients who underwent Fontan palliation. ObjectivesTo assess and analyze the clinical course, including length of stay, complications, and mortality of single ventricle patients undergoing Fontan Palliation. MethodsRetrospective review of all patients who underwent Fontan palliation at the University of Minnesota, 2006–2020. The primary outcomes measured were length of stay, complications, and Fontan failure. ResultsOf the 55 patients the median age of Fontan was 3.9 yrs [2.63, 4.19] and the most common diagnoses were hypoplastic left heart syndrome (n = 20, 36 %), double inlet left ventricle (n = 11, 20 %) and double outlet right ventricle (n = 8, 15 %). Twenty-Seven were right ventricle dominant, 23 were left ventricle dominant, and 5 had biventricular morphology. The median hospital stay was 11 days [8, 17.5], an ICU stay of 6.5 days [4,9]. Fourteen late complications occurred in 10 patients. Three, all with HLHS, had Fontan failure leading to heart transplant. Four died: two prior to hospital discharge, one 6.7 yrs post-Fontan, and one 27 days after transplantation. Right ventricle morphology was associated with prolonged ICU (P 0.05), prolonged hospital stay (P < 0.01), and complications resulting in Fontan failure (P < 0.01). ConclusionThe survival following Fontan completion in the current era is excellent, with an overall and transplant-free survival at 5 and 10 years were at 96 % and 93 %. However, it is clear that univentricular physiology continues to present challenges, and patients remain at risk for morbidity, especially for patients with right ventricle morphology.

Preoperative versus postoperative survival in patients with univentricular heart: a nationwide, retrospective study of patients born in 1990–2015

ObjectivesFew data exist on mortality among patients with univentricular heart (UVH) before surgery. Our aim was to explore the results of intention to perform surgery by estimating preoperative vs postoperative...

Long-term outcomes of patients with single ventricle who do not undergo Fontan palliation

BackgroundPatients with single ventricle (SV) without Fontan palliation are uncommon, and their long-term outcomes remain unclear. MethodsRetrospective study of 35 adult patients with SV without Fontan from two tertiary centers. Primary outcome was mortality. ResultsMedian age at first follow-up was 31 years (IQR: 20–40). Most common defect was double inlet left ventricle (34%), and 69% had left ventricular morphology. Patients were unoperated (46%), had systemic-to-pulmonary artery shunt (31%) or bidirectional cavopulmonary shunt (23%) as final palliation. Most common reasons for not progressing to Fontan palliation were pulmonary vascular disease (54%), patient refusal (17%), Fontan takedown (14%), and hypoplastic pulmonary arteries (11%). Baseline mean hemoglobin was 195 ± 29 g/L, mean O2 saturation 83 ± 6.9%, and 4 patients in NYHA Class III‒IV. After a mean follow-up of 10 ± 8.3 years, there were 9 deaths with heart failure being the leading cause (n = 6). Age-adjusted survival of these adult SV survivors was 73% and 53% at 40 and 50 years of age, respectively. Deceased patients more frequently had renal impairment (50% vs 0%) and QRS prolongation (75% vs 16%) at baseline (all p < 0.05). During follow-up, 40% had a new arrhythmia (atrial: n = 14, ventricular: n = 3), 34% had one or more hospitalizations for heart failure, and 17% had a stroke. A greater proportion of patients with pre-existing or new atrial/ventricular arrhythmia died compared to those without (42% vs 6%, p = 0.02). ConclusionsPatients with SV without Fontan have high mortality and a substantial burden of cardiovascular complications, particularly arrhythmia. QRS prolongation and renal impairment were associated with mortality.

Leadless pacemaker implantation in a univentricular heart in a patient with double-inlet left ventricle and L-transposition of the great arteries.

Leadless pacemaker implantation in a univentricular heart in a patient with double-inlet left ventricle and L-transposition of the great arteries.

Maternal diabetes as a teratogenic factor for congenital heart defects in infants of diabetic mothers.

Congenital heart defects (CHDs) are shown to have an association with maternal diabetes mellitus. The Bahraini population has a high prevalence of diabetes 16.3% thus putting it at increased risk of developing CHDs in infants of diabetic mothers (IDMs). Describing the prevalence of CHDs in IDM in the Kingdom of Bahrain. A retrospective clinical study. Bahrain Defense Force Hospital, Kingdom of Bahrain. The study took place from January 1998 to January 2020. A history was recorded for all patients who were referred to the only tertiary cardiac center in Bahrain for echocardiography. Data was recorded on an Excel Sheet for analysis. A cardiac anatomy survey was conducted by an experienced pediatric cardiologist for each patient and the defects were categorized into acyanotic and cyanotic lesions. Five thousand five hundred sixty-nine patients were referred for cardiac echocardiography. Three thousand two hundred fifty-six patients were diagnosed with CHDs, 2,987 were non-IDM whereas 269 were IDM. Patients diagnosed with non-structural defects were excluded. Atrial septal defect (ASD) was identified in 744 patients and was more likely to occur in non-IDM (p-value=.005). Hypertrophic obstructive cardiomyopathy (HOCM) was identified in 35 patients and was more likely to occur in IDM (p-value < .001). Transposition of the great arteries (TGAs) was identified in 80 patients and was more likely to occur in IDM (p-value .002). Double inlet left ventricle (DILV), Hypoplastic Left Heart Syndrome (HPLHS), and Other Uni-Ventricular Hearts were all more likely to occur in IDM with p-values < .05. This study showed significant association between fetal exposure to diabetes and the development of ASD, HOCM, TGA, DILV, HPLHS, and Other Uni-Ventricular Hearts.

Longitudinal analysis of systemic ventricular function and atrioventricular valve function after the Fontan procedure.

This study aimed to determine the longitudinal change of systemic ventricular function and atrioventricular valve (AVV) regurgitation after total cavopulmonary connection (TCPC). In 620 patients who underwent TCPC between 1994 and 2021, 4219 longitudinal echocardiographic examinations of systemic ventricular function and AVV regurgitation were evaluated retrospectively. The most frequent primary diagnosis was hypoplastic left heart syndrome in 172, followed by single ventricle in 131, tricuspid atresia in 95 and double inlet left ventricle (LV) in 91 patients. Dominant right ventricle (RV) was observed in 329 (53%) and dominant LV in 291 (47%). The median age at TCPC was 2.3 (1.8-3.4) years. Transplant-free survival at 5, 10 and 15 years after TCPC was 96.3%, 94.7% and 93.6%, respectively, in patients with dominant RV and 97.3%, 94.6% and 94.6%, respectively, in those with dominant LV (P = 0.987). Longitudinal analysis of systemic ventricular function was similar in both groups during the first 10 years postoperatively. Thereafter, systemic ventricular function worsened significantly in patients with dominant RV, compared with those with dominant LV (15 years: P = 0.007, 20 years: P = 0.03). AVV regurgitation was more frequent after TCPC in patients with dominant RV compared with those with dominant LV (P < 0.001 at 3 months, 3 years, 5 years, 10 years and 15 years, P = 0.023 at 20 years). There was a significant correlation between postoperative systemic ventricular dysfunction and AVV regurgitation (P < 0.001). There were no transplant-free survival difference and no difference in ventricular function between dominant RV and dominant LV for the first 10 years after TCPC. Thereafter, ventricular function in dominant RV was inferior to that in dominant LV. The degree of AVV regurgitation was significantly higher in dominant RV, compared with dominant LV, and it was positively associated with ventricular dysfunction, especially in dominant RV.

Single cell evaluation of endocardial Hand2 gene regulatory networks reveals HAND2-dependent pathways that impact cardiac morphogenesis.

The transcription factor HAND2 plays essential roles during cardiogenesis. Hand2 endocardial deletion (H2CKO) results in tricuspid atresia or double inlet left ventricle with accompanying intraventricular septum defects, hypo-trabeculated ventricles and an increased density of coronary lumens. To understand the regulatory mechanisms of these phenotypes, single cell transcriptome analysis of mouse E11.5 H2CKO hearts was performed revealing a number of disrupted endocardial regulatory pathways. Using HAND2 DNA occupancy data, we identify several HAND2-dependent enhancers, including two endothelial enhancers for the shear-stress master regulator KLF2. A 1.8 kb enhancer located 50 kb upstream of the Klf2 TSS imparts specific endothelial/endocardial expression within the vasculature and endocardium. This enhancer is HAND2-dependent for ventricular endocardium expression but HAND2-independent for Klf2 vascular and valve expression. Deletion of this Klf2 enhancer results in reduced Klf2 expression within ventricular endocardium. These data reveal that HAND2 functions within endocardial gene regulatory networks including shear-stress response.

Holms Heart in a Fetus with Maternal Anticonvulsant Drug Exposure: A Case Report

Double inlet left ventricle (DILV) is a rare congenital cardiac malformation that is defined as an anomaly with univentricular atrioventricular (AV) connection, and single ventricular morphology. This variation can be associated with the inlet and outlet cardiac abnormalities. In the current report, we present a case of Holms heart, as a rare variant of double-inlet left ventricle.

Opportunities and short-comings of the axolotl salamander heart as a model system of human single ventricle and excessive trabeculation

Few experimental model systems are available for the rare congenital heart diseases of double inlet left ventricle (DILV), a subgroup of univentricular hearts, and excessive trabeculation (ET), or noncompaction. Here, we explore the heart of the axolotl salamander (Ambystoma mexicanum, Shaw 1789) as model system of these diseases. Using micro-echocardiography, we assessed the form and function of the heart of the axolotl, an amphibian, and compared this to human DILV (n = 3). The main finding was that both in the axolotl and DILV, blood flows of disparate oxygen saturation can stay separated in a single ventricle. In the axolotl there is a solitary ventricular inlet and outlet, whereas in DILV there are two separate inlets and outlets. Axolotls had a lower resting heart rate compared to DILV (22 vs. 72 beats per minute), lower ejection fraction (47 vs. 58%), and their oxygen consumption at rest was higher than peak oxygen consumption in DILV (30 vs. 17 ml min−1 kg−1). Concerning the ventricular myocardial organization, histology showed trabeculations in ET (n = 5) are much closer to the normal human setting than to the axolotl setting. We conclude that the axolotl heart resembles some aspects of DILV and ET albeit substantial species differences exist.

Association of Bulboventricular Foramen Size and Need for Early Intervention in Infants with Tricuspid Atresia or Double-Inlet Left Ventricle with Normally Related Great Arteries

The association of bulboventricular foramen (BVF) size and systemic outflow adequacy has been studied in patients with tricuspid atresia (TA) or double-inlet left ventricle (DILV) with transposed great arteries. The aim of this study was to determine the relationship between initial BVF size and risk for progressive pulmonary outflow obstruction requiring intervention to increase pulmonary blood flow in patients with TA or DILV with normally related great arteries. Patients with TA or DILV with normally related great arteries were identified by retrospective chart review at a single center from 2005 to 2021. Patients were stratified by indexed BVF area (iBVFA) to determine the relationship of iBVFA size and the need for intervention before the Glenn operation to establish supplemental pulmonary blood flow with either a Blalock-Taussig-Thomas shunt (BTTS) or patent ductus arteriosus (PDA) stent. Patients were followed through the time of their Glenn operations. Logistic regression analysis was performed to determine optimal iBVFA cut points. Thirty-seven patients with TA or DILV with normally related great arteries were included. Sixteen had iBVFA < 1cm2/m2, with all 16 (100%) requiring either a BTTS or PDA stent to increase pulmonary blood flow before the Glenn operation. Seventeen had iBVFAs of 1 to 2cm2/m2, with 10 (59%) requiring either a BTTS or PDA stent. Nine of those 10 demonstrated flow acceleration across the BVF and/or pulmonary outflow tract. Four had iBVFA > 2cm2/m2, with only one patient (25%) requiring a BTTS. Among our cohort, an iBVFA of <1.8cm2/m2 provided sensitivity of 96% with good positive and negative predictive values (81% and 80%, respectively) for requiring intervention with a BTTS or PDA stent before the Glenn operation. An iBVFA of ≤1.8cm2/m2 on initial postnatal echocardiography is associated with the development of subpulmonary obstruction requiring intervention with a BTTS or PDA stent before the Glenn operation, with the highest risk noted in those with iBVFA of ≤1cm2/m2. Factors such as BVF flow acceleration or pulmonary outflow tract narrowing should also be considered in the decision to augment pulmonary blood flow.

Long-term survival and center volume for functionally single-ventricle congenital heart disease in England and Wales

ObjectivesLong-term survival is an important metric for health care evaluation, especially in functionally single-ventricle (f-SV) congenital heart disease (CHD). This study's aim was to evaluate the relationship between center volume and long-term survival in f-SV CHD within the centralized health care service of England and Wales. MethodsThis was a retrospective cohort study of children born with f-SV CHD between 2000 and 2018, using the national CHD procedure registry, with survival ascertained in 2020. ResultsOf 56,039 patients, 3293 (5.9%) had f-SV CHD. Median age at first intervention was 7 days (interquartile range [IQR], 4, 27), and median follow-up time was 7.6 years (IQR, 1.0, 13.3). The largest diagnostic subcategories were hypoplastic left heart syndrome, 1276 (38.8%); tricuspid atresia, 440 (13.4%); and double-inlet left ventricle, 322 (9.8%). The survival rate at 1 year and 5 years was 76.8% (95% confidence interval [CI], 75.3%-78.2%) and 72.1% (95% CI, 70.6%-73.7%), respectively. The unadjusted hazard ratio for each 5 additional patients with f-SV starting treatment per center per year was 1.04 (95% CI, 1.02-1.06), P < .001. However, after adjustment for significant risk factors (diagnostic subcategory; antenatal diagnosis; younger age, low weight, acquired comorbidity, increased severity of illness at first procedure), the hazard ratio for f-SV center volume was 1.01 (95% CI, 0.99-1.04) P = .28. There was strong evidence that patients with more complex f-SV (hypoplastic left heart syndrome, Norwood pathway) were treated at centers with greater f-SV case volume (P < .001). ConclusionsAfter adjustment for case mix, there was no evidence that f-SV center volume was linked to longer-term survival in the centralized health service provided by the 10 children's cardiac centers in England and Wales.

Social life in adult patients after Fontan procedure

BackgroundLittle is known about the social life in adult patients after Fontan palliation. The study aimed to assess the long-term social life of patients after Fontan surgery, including school achievement, employment, and marital status.ResultsWe conducted a cross-sectional study on patients aged 18 years or above who had a Fontan operation for a single ventricle pathology. Our outcomes were the academic performance and marital and employment status of adult Fontan patients. Patients or their families were interviewed directly or by phone, and a set of questions were asked to address their educational level, employment, and marital status. Ninety-nine patients were included in the study. Their median age was 21 years (min–max: 18–41), and 60% were females (n= 59). The most common diagnoses were double inlet left ventricle (n= 24, 24%), tricuspid atresia (n= 21, 21%), and the unbalanced atrioventricular canal (n= 21, 21%). Fourteen patients (14%) were married, and 6 of them had children (two were females). Eleven percent were college graduates (either diploma or bachelor’s degree), and 47% were high school graduates.ConclusionsFontan operation could negatively affect the social life of the patients. It may affect employment patterns, educational levels, and marital status.