Pulmonary Perfusion Asymmetry in Patients after Repair of Tetralogy of Fallot: A 4D Flow MRI-Based Study

Background: Repaired Tetralogy of Fallot (rTOF) patients may have residual lesions such as main (MPA) and branch pulmonary artery stenosis (BPAS). While MPA stenosis is well studied, few data are available on BPAS in rTOF. We aimed to describe pulmonary perfusion in a large paediatric cohort of rTOF and its impact on right ventricular and outflow-tract hemodynamics using 4D flow CMR. Methods: 130 consecutive patients (mean age at CMR 14.3 ± 4.6 years) were retrospectively reviewed. 96 patients had transannular patch without valve preservation while 34 patients had conserved annulus or valved conduit. A pulmonary blood flow ratio (right pulmonary artery (RPA)/left pulmonary artery (LPA)) between 0.75 and 1.56 was considered normal. Results: Asymmetric pulmonary perfusion was present in 59/130 patients (45%), with 54/59 (91%) having left lung hypoperfusion (blood flow ratio >1.56). RPA/LPA perfusion ratio in the whole cohort was independently associated with the LPA Z-score (−0.053, p = 0.007), the RPA regurgitant fraction (RF) (0.013, p = 0.011) and previous LPA stenting (0.648, p = 0.004). Decreasing LPA % perfusion (and conversely increasing RPA % perfusion) was significantly associated with higher MPA diameter Z-score (−0.06, p = 0.007). On multivariate analysis, MPA Z-score was independently associated with pulmonary RF (0.48, p < 0.001) and with right ventricular indexed volumes (coefficient 3.6, p = 0.023). In patients with transannular patch repair, asymmetric pulmonary flow was an independent predictor of right ventricular ejection fraction (RVEF) (−3.66, p = 0.04). Conclusions: Pulmonary perfusion asymmetry is frequent in rTOF and is associated with abnormal right ventricular and outflow-tract hemodynamics, including MPA dilatation and decreased RVEF in patients after transannular patch.     

Transcatheter Ventricular Septal Defect Closure with Nit-Occlud Lê VSD Device—Five Years’ Experience and Literature Review

Introduction: Transcatheter closure is an alternative to ventricular septal defect (VSD) occlusion surgery. Nit-Occlud Lê VSD coil is a new device yet to be evaluated. The study aimed to evaluate immediate and midterm results after transcatheter closure with the Nit-Occlud Lê VSD device. Methods: The retrospective analysis included 30 patients with VSD referred for closure during the period from October 2015 to December 2020. Results: At the time of intervention, the patients’ mean age and body weights were 7.5 ± 5.6 years and 29.3 ± 19.1 kg. The majority of the defects had perimembranous location (24/30), four defects had muscular and two outlet subaortic position. The mean effective right-side diameter of the VSDs was 3.6 ± 1.3 mm. Single ventricular fibrillation, device embolization, and hemolysis developed in different patients and were successfully treated. None of the patients had a complete atrioventricular block. The coil was successfully placed in 25/30 (83.3%) patients. The majority of the devices were 10 mm × 6 mm (11/25) and 12 mm × 8 mm (8/25) in size. Two patients required the implantation of a second device. The follow-up period was 2.1 ± 1.4 years. Complete VSD closure was achieved in 48% of cases immediately after the intervention, 74% during 2.1 ± 1.6 months after the procedure, and 81% over follow-up. The remaining patients had a trivial residual defect. During the follow-up, approximately one-third of patients developed trivial aortic and mitral valve regurgitation, and half of the patients acquired trace/mild tricuspid regurgitation. Standardized (z-score) left ventricular end-diastolic diameter (0.15 ± 0.37 vs. 0.92 ± 0.82, p = 0.005) and left atrium dimension (0.47 ± 0.58 vs. 1.89 ± 1.11, p = 0.005), as well as the left atrium to aortic root ratio (1.2 ± 0.1 vs. 1.4 ± 0.2, p = 0.005) showed a significant decrease over follow-up related to the period before intervention. Conclusion: Intervention with Nit-Occlud® Lê VSD coil showed appropriate results regarding VSD closure rate, complications, and chamber remodeling. The introduction of this device into clinical practice is a significant step forward in transcatheter perimembranous VSD occlusion.     

Mortality and Long-Term Outcome of Neonates with Congenital Heart Disease and Acute Perinatal Stroke: A Population-Based Case-Control Study

Objective: Neonates with congenital heart disease (CHD) and perinatal stroke have high mortality and survivors are at risk for poor long-term neurodevelopmental outcome. The aim of this study was to assess the risk factors and outcome of neonates with both CHD and MRI-confirmed perinatal stroke (Study Group) and compare those to the risk factors and outcome of infants matched for CHD without stroke (Control-1) and of infants matched for MRI-confirmed stroke without CHD (Control-2). Methods: We conducted a population-based case-control study enrolling 28 term neonates with CHD and MRI-confirmed acute perinatal stroke born between 2007–2017 in the Central-Hungarian Region. Each of the control groups included 56 infants. The Bayley Scales of Infant Development-II, the Brunet-Lézine test and the Binet Intelligence scales-V were used for neurodevelopmental follow-up at a median age of 61 months. Results: Mortality was highest in the Study Group (25% compared to 5% and 2%, respectively, p = 0.001). Adverse neurodevelopmental outcome was prevalent in the Study (53%) and Control-2 Groups (52%, p = 0.03). Significantly different parameters among the three groups included Apgar scores, mode of delivery, gestational age at birth, cardiac interventions and twin pregnancy. In a multivariable regression analysis adjusted for clinically relevant parameters, patients in the Study Group had significantly higher odds for mortality compared to patients in the Control-1 Group (OR: 6.5 95% CI: 1.1–39.4). Conclusions: Neonates with perinatal stroke and CHD are at a higher risk for dying compared to neonates with CHD without stroke. In addition, the stroke-associated direct insult to the brain likely plays an important role in the development of neurodevelopmental morbidity in these patients.     

Immediate and Long-Term Results of Transcatheter Closure of Patent Ductus Arteriosus—Comparison of Two Decades before and after Change in Antibiotic Infective Endocarditis Prophylaxis Guidelines

Objectives: To determine immediate and long-term follow-up of transcatheter closure of patent ductus arteriosus (PDA) in children. Background: National antibiotic prophylaxis (AP) guideline for infective endocarditis changed after 2009, the effect on practice of PDA closure is unknown. Methods: Observational single center study analyzing follow-up of PDA closure comparing two time periods before (2002–2009) and after (2010–2019) changes in AP guideline. Results: 332 patients (68.1% female), median (interquartile range) age 3.0 years (1.5–5.7) and body weight 14.0 kg (10.0–19.3), were enrolled. PDA morphology was conical type A (50.3%), window type B (1.2%), tubular type C (40.1%), complex type D (2.1%), elongated type E (0.9%) and other (5.4%). Minimal PDA diameter and length were 1.9 mm (1.3–2.5) and 8.0 mm (6.2–10.2). PDA was closed using coils (56.3%), Amplatzer Duct Occluders (41.9%) and others (1.8%). Complete closure rate was 61.1% at catheter intervention, 72.3% on day 1, 87.7% after 6 months and 98.4% at last follow-up on echocardiography. Moderate complication rate (severity level 3) was 4.2% and major complication rate (severity level 4) 0.3%, with no catastrophic complications (severity level 5). Annual PDA closure rate declined in the second time period (22.6/year vs. 15.5/ year, p = 0.018), PDA size increased (1.6 mm vs. 2.0 mm, p = 0.002) and proportion of coils decreased (72.4% vs. 37.1%, p < 0.001). Conclusions: Interventional closure of PDA is associated with excellent closure rates during follow-up (>98%) and only a small number of complications leading to reintervention or surgery. Change in AP guidelines changed indication for and practice of PDA closure.     

Assessment of Intracardiac and Extracardiac Deformities in Patients with Various Types of Pulmonary Atresia by Dual-Source Computed Tomography

Background: Pulmonary atresia (PA) is a group of heterogeneous complex congenital heart disease. Only one study modality might not get a correct diagnosis. This study aims to investigate the diagnostic power of dual-source computed tomography (DSCT) for all intracardiac and extracardiac deformities in patients with PA compared with transthoracic echocardiography (TTE). Materials and Methods: This retrospective study enrolled 79 patients and divided them into three groups according to their main diagnosis. All associated malformations and clinical information, including treatments, were recorded and compared among the three groups. The diagnostic power of DSCT and TTE on all associated malformations were compared. The surgical index (McGoon ratio, pulmonary arterials index (PAI), and total neopulmonary arterial index) and radiation dose were calculated on the basis of DSCT. Results: Of the patients, 32, 30, and 17 were divided into the groups of PA with ventricular septal defect (VSD), PA with VSD and major aortopulmonary collateral arteries, and PA with other major malformations, respectively. Consequently, 182, 162, and 13 intracardiac, extracardiac, and other major malformations were diagnosed, respectively. Moreover, DSCT showed a better diagnostic performance in extracardiac deformities (154 vs. 117, p < 0.001), whereas TTE could diagnose intracardiac deformities better (159 vs. 139, p = 0.001). The McGoon ratio, PAI, and treatment methods were significantly different among the three groups (p = 0.014, p = 0.008, and p = 0.018, respectively). Conclusion: More than one imaging modality should be used to make a correct diagnosis when clinically suspecting PA. DSCT is superior to TTE in diagnosing extracardiac deformities and could be used to roughly calculate surgical indices to optimize treatment strategy.     

Outcomes of Transcatheter Closure in Outlet-Type Ventricular Septal Defect after 1 Year

Background: Ventricular septal defect (VSD) is the most common congenital heart disease. Transcatheter VSD closure is an effective treatment for patients with muscular and perimembranous VSD. However, there is a limit data for outlet VSD, especially impact to the aortic valve leaflet after transcatheter closure. This study aims to assess the outcomes of transcatheter closure of the outlet-type ventricular septal defect (OVSD) after 1 postoperative year. Methods: A retrospective study was performed including 50 patients who underwent transcatheter (n = 25) and surgical (n = 25) OVSD closure during the exact time frame at two medical centres. Results: The median age and body weight of patients in the transcatheter group were significantly higher than those of patients in the surgical group (7.0 vs. 2.8 years; 27.0 vs. 11.4 kg; p < 0.01). The defect size in the surgical group was significantly larger than that in the transcatheter group (5.0 vs. 3.0 mm; p < 0.01). All OVSD patients have successful transcatheter closure (100%) as effective as surgical closure. Less than small residual shunt was present 20% and 8% immediately after the procedure in the transcatheter and surgical groups (p = 0.50), which decreased to 12% and 4% at the 1-year follow-up (p = 0.61), respectively. No incidence of complete atrioventricular block and other complications was observed in both groups, and no significant differences were noted in the new onset or worsening of the aortic regurgitation in both groups (p = 1.0). Conclusions: Transcatheter treatment could be effectively and safely achieved for OVSD closure at 1-year follow-up.     

Risk factors for hyperuricemia in congenital heart disease patients and its relation to cardiovascular death

Introduction: Hyperuricemia has been associated with cardiovascular risk factors but it remains controversial if uric acid is an independent predictor of cardiac mortality.
Methods: A total of 503 CHD patients (457 nonhypoxemic and 46 hypoxemic) and 772 control patients fulfilled inclusion criteria. Demographic, clinical, and analytical data [serum uric acid and 24h urine uric acid levels, N‐terminal pro‐B‐type natriuretic peptide (NT‐pro‐BNP), and C‐reactive‐protein (CRP) concentrations] were studied. Survivals curves to determine cardiac death and arterial thrombosis in CHD patients were also examined.
Results: Noncyanotic and cyanotic CHD patients had significant higher serum uric acid concentration (5.2 ± 1.5 vs 4.9 ± 1.3mg/dL, P = .007 and 6.7 ± 2.1 vs 4.9 ± 1.3mg/ dL, P < .001, respectively) and gout (1% vs 0%, P = .003 and 4% vs 0%, P < .01, respec‐ tively) than the control population. Among CHD patients, hyperuricemic patients were significant older and with overweight, used more diuretics, were more cyanotic and had higher serum creatinine, NT‐pro‐BNP and CRP concentrations than nonhy‐ peruricemic. In the multivariable analysis, the body mass index (BMI) (OR 1.09; 95% CI 1.01–1.18), cyanosis (OR 6.2; 95 CI 1.5–24.6), serum creatinine concentration (OR 49; 95% CI 44–538), and being under diuretic treatment (OR 4.5; 95% CI 1.4–14.5) proved to be risk factors for hyperuricemia in CHD patients. The Kaplan–Meier events free survival curves, during a 5.2 ± 2.7 years follow‐up of up time, showed that hyperuricemic CHD patients had significant higher cardiovascular death (P = .002). However, after applying the Cox regression analysis uric acid levels lost its statistical significance. No significant differences were seen in relation to thrombotic events between CHD patients with and without hyperuricemia.
Conclusions: CHD patients, noncyanotic and cyanotic, have higher serum uric acid levels and gout than patients in the general population. BMI, renal insufficiency, cya‐ nosis, and the use of diuretics were risk factor for hyperuricemia among CHD patients.     

Morphologic characterization of the patent ductus arteriosus in the premature infant and the choice of transcatheter occlusion device

Objectives : The aim of this study was to describe and differentiate the morphology of patent ductus arteriosus (PDA) seen in children born prematurely from other PDA types. Background : PDAs are currently classified as types A‐E using the Krichenko's classification. Children born prematurely with a PDA morphology that did not fit this classification were described as Type F PDA. Methods : A review of 100 consecutive children who underwent transcatheter device closure of PDA was performed. The diameter and length (L) of the PDA and the device diameter (D) were indexed to the descending aorta (DA) diameter. Results : Comparison of 26 Type F PDAs was performed against, 29 Type A, 7 Type C and 32 Type E PDAs. Children with Type F PDAs (median 27.5 weeks gestation) were younger during the device occlusion compared with types A, C, and E (median age: 6 vs. 32, 11, and 42 months; P = 0.002). Type F PDAs were longer and larger, requiring a relatively large device for occlusion than types A, C, and E (Mean L/DA: 1.88 vs. 0.9, 1.21, and 0.89, P ≤ 0.01 and Mean D/DA: 1.04 vs. 0.46, 0.87, and 0.34, P ≤0.01). The Amplatzer vascular plug‐II (AVP‐II) was preferred for occlusion of Type F PDAs (85%; P <0.001). Conclusions : Children born prematurely have relatively larger and longer PDAs. These “fetal type PDAs” are best classified separately. We propose to classify them as Type F PDAs to add to types A‐E currently in use. The AVP‐II was effective in occluding Type F PDAs. © 2015 Wiley Periodicals, Inc.     

Emotions,Perceptions and Health Behaviors of Adult Congenital Heart Disease Patients during COVID-19 in New York City

Background: Adults with congenital heart disease (ACHD) have increased prevalence of mood and anxiety disorders. There are limited data regarding the influence of the COVID-19 pandemic on the mental health and health behaviors of these patients. Objective: The purpose is to evaluate the perceptions, emotions, and health behaviors of ACHD patients during the COVID-19 pandemic. Methods: In this cross-sectional study of ACHD patients, we administered surveys evaluating self-reported emotions, perceptions and health behaviors. Logistic regressions were performed to determine the adjusted odds of displaying each perception, emotion and health behavior based on predictor variables. Results: Ninety-seven patients (mean age 38.3 years, 46.4% female, 85.6% moderate or complex lesion) completed the survey. The majority of patients reported feeling moderately or very sad (63.1%), and 48.4% of patients identified themselves as feeling moderately or very anxious. The majority of patients perceived their risk of COVID-19 as moderate or high. Females were more likely to report feeling sad and anxious (95% CI 1.06–10.96, p-value 0.039, and 95% CI 1.44–15.30, p-value = 0.012, respectively), and were associated with higher odds of having a perceived increased risk of COVID-19 (95% CI 1.33–10.59, p-value 0.012). There was no association between ACHD anatomic or physiologic classification and perceptions, emotions and health behaviors. Conclusions: Females were more likely to report feeling sad, anxious and an increased risk of COVID-19 in comparison to males. These findings indicate the need for mental health support and promotion of health behaviors during the pandemic amongst all ACHD patients, regardless of underlying condition.     

Preoperative Fasting of More Than 14 Hours Increases the Risk of Time-to-Death after Cardiothoracic Surgery in Children: A Retrospective Cohort Study

Background: Prolonged preoperative fasting can cause hypoglycemia, hyperglycemia, and intravascular volume depletion in children. We aimed to examine whether prolonged preoperative fasting is associated with in-hospital mortality and other morbidities in pediatric cardiothoracic surgery. Methods: This retrospective cohort study included children aged 0–3 years who underwent cardiac surgery between July 2014 and October 2020. The patient demographic data, surgery-related and anesthesia-related factors, and postoperative outcomes, including hypoglycemia, hyperglycemia, sepsis, length of intensive care unit stay, and in-hospital mortality, were recorded. The main exposure and outcome variables were prolonged fasting and time-to-death after surgery, respectively. The associations between prolonged fasting and perioperative death were analyzed using multivariate Cox regression analysis. Results: In total, 402 patients were recruited. The incidence of perioperative mortality was 21% (85/402). The proportion of perioperative deaths was significantly higher in the prolonged fasting group than that in the normal fasting group. The proportion of postoperative bacteremia and hypoglycemia was significantly higher in the very prolonged fasting group than that in the prolonged fasting group. After adjusting for preoperative conditions and anesthesia- and surgery-related factors, preoperative prolonged fasting >14.4 h was significantly associated with time-to-death (HR [95% CI]: 2.2 [1.2, 3.9], p = 0.036). The 30-day survival rates of fasting time >14.4 h, 9.25–14.4 h, and <9.25 h were 0.67 (0.55, 0.81), 0.79 (0.72, 0.87), and 0.85 (0.79, 0.91), respectively. Conclusions: Preoperative fasting of more than 14.4 h was associated with a two-fold increase in the hazard rate of time-to-death in children who underwent cardiac surgery.     

Exercise Catheterization for Hemodynamic Evaluation of Adults with Coarctation of the Aorta

Background: Coarctation of the aorta (CoA) is associated with a generalized arteriopathy and long-term complications despite repair. Data on invasive exercise hemodynamics in this population are lacking. Accordingly, we reviewed adults with CoA undergoing exercise catheterization to assess 1. hemodynamic profile; 2. feasibility for assessment of CoA severity. Methods: Twenty patients undergoing exercise cardiac catheterization (12 arm adduction and 8 supine cycle ergometry) at a quaternary care center between 2004 and 2021 were identified. Resting and exercise hemodynamic data were abstracted from the procedure logs. Results: Mean age was 43.6 ± 12.0 years. Eleven patients (55%) had resting pulmonary arterial wedge pressure (PAWP) >15 mmHg; among those undergoing arterial catheterization, left ventricular end-diastolic pressure was >15 mmHg in 63%. Eleven patients (55%) had pulmonary hypertension: 7 (35%) combined and 4 (20%) isolated post-capillary. At peak exercise, ΔPAWP/Δcardiac output (CO) ≥2 and Δmean pulmonary artery pressure/ΔCO ≥3 mmHg/l/min were found in 7 (78%) and 6 (67%) patients, respectively; the composite of exercise PAWP ≥25 mmHg or ΔPAWP/ΔCO >2 was seen in 12 (86%). CoA peak-to-peak gradients at baseline (n = 14) and during exercise (n = 9) were 12 (3–16) and 16 mmHg (9–28), respectively. Only 2 patients had an increase in CoA gradient to >20 mmHg with exercise. Conclusions: Diastolic dysfunction and pulmonary hypertension were highly prevalent, with exercise unmasking abnormal diastolic and pulmonary vascular reserve in some individuals. Most patients failed to show significant increases in CoA peak-to-peak gradients with exercise. Further studies are warranted to establish the best diagnostic method for CoA severity assessment.     

Outcomes of Self-Expanding Transcatheter Pulmonary Valves: Extended Follow-Up of a Prospective Trial

Background: The Venus-P valve was the first self-expanding valve used world-wide for transcatheter pulmonary valve replacement (TPVR) in patients with severe pulmonary regurgitation (PR). We intended to report the extended follow-up results from the prospective trial (No. NCT02590679). Methods: A total of 38 patients with severe PR (mean age 24.2 ± 13.2) were included. Follow-up data were obtained after implanted at 1, 6, and 12 months and yearly after. The frame geometry was assessed on post-implant computer tomography (CT) scanning by calculating the non-circularity [circularity ratio (minimum diameter/maximum diameter) < 0.9] and under-expansion [expansion ratio (derived external valve area/nominal external valve area) < 0.9). Adverse events (all-cause mortality, reintervention, valve dysfunction, stent fracture and endocarditis) were recorded. Results: All valves were implanted successfully with normal function at discharge. Geometric CT analysis showed under-expanded valve was detected in 22 patients (63%) and non-circular valve was seen in 16 patients (46%). During a median follow-up of 4.8 years (range 0.3–8.1), there were 1 death and 1 surgical explant, both resulting from endocarditis. Five-year freedom from valve dysfunction and stent fracture were 84.8% (95%CI 74.8–94.7) and 83.5% (95%CI 73.8–93.2). Endocarditis occurred in 3 patients at a median time of 7 months. Stent fracture was more common in patients with non-circularity stents. Conclusion: TPVR using Venus-P valve is associated with favorable outcomes at 5 years. Non-circular shapes in the valve level may have a higher risk of stent fracture.     

Diagnostic Yield of Non-Invasive Testing in Patients with Anomalous Aortic Origin of Coronary Arteries: A Multicentric Experience

Background: Anomalous aortic origin of a coronary artery (AAOCA) is a congenital heart disease with a 0.3%−0.5% prevalence. Diagnosis is challenging due to nonspecific clinical presentation. Risk stratification and treatment are currently based on expert consensus and single-center case series. Methods: Demographical and clinical data of AAOCA patients from 17 tertiary-care centers were analyzed. Diagnostic imaging studies (Bidimensional echocardiography, coronary computed tomography angiography [CCTA] were collected. Clinical correlations with anomalous coronary course and origin were evaluated. Results: Data from 239 patients (42% males, mean age 15 y) affected by AAOCA were collected; 154 had AAOCA involving the right coronary artery (AAORCA), 62 the left (AAOLCA), 23 other anomalies. 211 (88%) presented with an inter-arterial course. Basal electrocardiogram (ECG) was abnormal in 37 (16%). AAOCA was detected by transthoracic echocardiography and CCTA in 53% and 92% of patients, respectively. Half of the patients reported cardiac symptoms (119/239; 50%), mostly during exercise in 121/178 (68%). An ischemic response was demonstrated in 37/106 (35%) and 16/31 (52%) of patients undergoing ECG stress test and stress-rest single positron emission cardiac tomography. Compared with AAORCA, patients with AAOLCA presented more frequently with syncope (18% vs. 5%, P = 0.002), in particular when associated with inter-arterial course (22% vs. 5%, P < 0.001). Conclusion: Diagnosis of AAOCA is a clinical challenge due to nonspecific clinical presentations and low sensitivity of first-line cardiac screening exams. Syncope seems to be strictly correlated to AAOLCA with inter-arterial course.     

Alterations in Metabolites Associated with Hypoxemia in Neonates and Infants with Congenital Heart Disease

Objectives: (1) To measure the global shift in the metabolome in hypoxemic versus non-hypoxemic infants with congenital heart disease; (2) To identify metabolites and metabolic pathways that are altered in hypoxemia. Study Design: Analysis of serum samples obtained prior to cardiopulmonary bypass from 82 infants ≤120 days old with congenital heart disease requiring surgery at Children’s Hospital Colorado. Infants were divided into groups based on preoperative oxygen saturations: non-hypoxemic (>92%), mild hypoxemia (85–92%), and severe hypoxemia (<85%). Tandem mass spectrometry was used to analyze 165 targeted metabolites. Partial least squares discriminant analysis and t-tests were used to determine differences among metabolic profiles and individual metabolites respectively. Results: The broad metabolic fingerprint of neonates or older infants did not vary by degree of hypoxemia. There were 12 individual metabolites that differed between hypoxemic and non-hypoxemic neonates, including lower methylmalonic acid (p = 2.44 × 10^–4 ), glutamate (p = 0.001), and hypoxanthine (p = 0.003), and higher thymine (p = 8.67 × 10^–4 ) and myo-inositol (p = 0.014) seen in hypoxemic neonates. Individual metabolites did not vary significantly between older infants with or without hypoxemia. Conclusions: We did not find evidence supporting global metabolic changes associated with cyanotic congenital heart disease in neonates or older infants. However, specific metabolites did discriminate between hypoxemic and non-hypoxemic neonates. These include methylmalonic acid, as well as several metabolites known to change in hypoxia-reoxygenation states (hypoxanthine) and chronic hypoxemic states (glutamate, thymine, myo-inositol) and may represent specific metabolic changes triggered by hypoxemia among neonates with cyanotic congenital heart disease.     

Predictors of Health-Related Quality of Life in Children with Cyanotic Heart Disease Who Underwent Palliative and Total Repair

Background: Studies on predictors of health-related quality of life (HRQOL) in pediatric patients with cyanotic heart disease who are waiting for the next stage and those who have undergone total repair are scarce. Therefore, we aimed to identify such predictors in children who received the modified Blalock–Taussig shunt (MBTS) and those who underwent total repair. Methods: In this historical cohort and concurrent follow-up study, data of children who underwent MBTS at the age of 0–3 years between January 2005 and December 2016 at a super-tertiary care hospital in Southern Thailand were obtained. Children who were alive in December 2017 were recruited to evaluate the quality of life at least 1 year after their operation. Between January and December 2018, the “Pediatric Quality of Life Inventory 4.0 Generic Core Scales” with both child self-report and parent proxy-report scores were used to examine the HRQOL. Multivariate linear regression analysis was performed to identify independent predictors of HRQOL. Beta-coefficient (β) and 95% confidence intervals (95% CIs) were calculated and considered statistically significant at p < 0.05. Results: Among the 380 enrolled children, 148 died, 122 survived and waited for total repair, and 110 survived after total repair. In the multivariate analysis, chronic lung disease was a common predictor of lower physical and psychosocial HRQOL reported by the parents (β [95% CI]: −0.42 [−0.81, −0.03] and −0.49 [−0.89, −0.09], respectively). Total repair was a predictor of higher physical HRQOL according to both parents and children (β [95% CI]: 0.33 [0.09, 0.57] and 0.70 [0.36, 1.03], respectively). A predictor of higher psychosocial HRQOL reported by the parents was younger age during MBTS surgery compared with older age (β [95% CI]: 0.012 [0.001, 0.022]). In the total repair subgroup, undergoing the Fontan procedure (vs. Glenn procedure) was a predictor for lower physical HRQOL reported by the parents (β [95% CI]: −0.82 [−1.52, −0.13]). Higher socioeconomic status was a predictor of both physical and psychosocial HRQOL (β [95% CI]: 0.018 [0.001, 0.034] and 0.012 [0.0001, 0.04], respectively). Conclusions: Successful total repair was a predictor of higher physical HRQOL, and younger age during MBTS surgery was a predictor of higher psychological HRQOL in children with cyanotic heart disease. Higher socioeconomic status was a predictor of both physical and psychological HRQOL following total repair [Thai Clinical Trials Registry: TCTR20161221003].     

Safety and Feasibility of Transcatheter Closure of Large Patent Ductus Arteriosus Measuring ≥4 mm in Patients Weighing ≤6 kg

Objectives: The study aims to find safety of transcatheter closure of large patent ductus arteriosus (PDA) ≥4 mm in patients weighing ≤6 kg.
Background: Large PDA ≥4 mm in patients ≤6 kg challenge the interventionist due to need for large delivery sheath, kink of delivery sheath, and encroachment of aorta or pulmonary artery (PA) by the device. Many institutions refer them for surgery.
Methods: Preterm neonates and ducts with coarctation were excluded. All other patients were taken for catheter closure. Ducts with roomy ampulla were closed with multiple coils aided by bioptome, and others with Amplatzer duct occluders.
Results: Twenty-eight patients aged 2–18 months (median 5.5 months) and weighing 3.8–6 kg (median 4.7 kg) had large PDA (mean diameter 6.3 ± 2 mm) with hyperkinetic pulmonary hypertension. Four patients had bioptome-aided coil closure. Twenty-two other ducts were closed with devices. Two procedures failed due to sheath kink in one patient and device pulling through a duct in the other patient. Four infants needed blood transfusions. The mean procedural time was 42 ± 20 minutes. On a mean follow-up of 25.5 ± 14.8 months, there were no residual flows and no gradients across aorta or pulmonary artery.
Conclusions: Transcatheter closure of large ducts ≥4 mm might be considered safe and effective in infants weighing ≤6 kg also. Decision on coils versus devices depends on ductal morphology. On midterm follow-up with somatic growth, there was no occurrence of aortic or PA gradients.     

Natural History of Kawashima Palliation in Single-Ventricle and Interrupted Inferior Vena Cava Heart Disease in China 11 Years Result

Background: The long-term outcomes of patients treated with a Kawashima procedure and keeping the antegrade pulmonary blood flow (AnPBF) in single-ventricle (SV) and interrupted inferior vena cava (IVC) heart disease is still uncertain as yet. Methods: We investigated 18 patients who underwent the Kawashima procedure with SV physiology and an interrupted IVC between January 2009 and June 2018, perioperative, operative and postoperative characteristics were recorded. Results: A total of 18 patients underwent the Kawashima procedure at a median age of 2.7 years (range 0.5–24.7 years), of which 12 (66.7%) were male and 6 (33.3%) were female. The mean saturation was 76.2 ± 8.5% in preoperative period and 94.2 ± 2.2% in postoperative period. All patients had kept AnPBF. The median duration of mechanical ventilation was 12 h (range 2.5–22.5 h) and the median duration of pleural drainage was 5 days (range 2–27 days). The median hospital stay was 9 days (range 6–70 days). There was no operative death and no mortality was seen in early postoperative period. Follow-up was 100% completed, with an average follow-up period of 6.1 ± 2.7 years (range 1–11 years). 4 patients died during the follow-up. The overall 5 and 10 years’ survival rates estimated by Kaplan-Meier method were 88% and 68%, respectively. Although there were no significant differences in the duration of postoperative follow-up between the death group and the survival group (p > 0.05), the major systemic ventricular end-diastolic diameter (SVEDD) (p = 0.018) and the degree of AVVR (p = 0.001) showed significant difference between the two groups. The diameters of main pulmonary artery showed significant growth in both the death group (p = 0.015) and the survival group (p = 0.012) over time. SVEDD had no significant increase in the survival group (p = 0.665) but was significantly larger in the death group (p = 0.014). Multivariable risk factors of late mortality in patients treated with Kawashima procedure were follow-up AVVR (p = 0.044; HR: 3.124; 95%CI: 1.030–9.473) and SVEDD (p = 0.031; HR: 9.766; 95%CI: 1.226–77.8). 14 patients (100%) were all in New York Heart Association (NYHA) functional class I and the mean saturation was 93 ± 2% at last follow-up. Only one patient finished Fontan completion. Conclusions: The Kawashima procedure with AnPBF can be safely performed with acceptable early and long outcomes. Although some previous studies have shown the risk of pulmonary arteriovenous malformations (PAVMS) after Kawashima procedure in the mid- and long-term, our findings are in contradiction with it. No PAVMs occurred in all the survivors. Kawashima procedure with open AnPBF may be a good option for unsuitable Fontan candidates.     

Effectiveness of Bilateral Pulmonary Artery Banding in Patients with Hypoplastic Left Heart Syndrome and Congenital Heart Defects with A Functional Single Ventricle: A Single-Center Retrospective Study

Background: Bilateral banding of the branches of the pulmonary artery in patients with hypoplastic left heart syndrome (HLHS) and other duct dependent critical neonatal heart malformations can significantly reduce the incidence of severe complications in the postoperative period, especially in severely unstable patients. In our study we compared different surgical techniques of bilateral pulmonary artery banding (PAB) in respect to their success in balancing systemic and pulmonary blood flow. Methods: We included 44 neonates with a HLHS and congenital heart diseases (CHD) with a functional single ventricle underwent a hybrid operation: bilateral PAB and patent ductus arteriosus stenting. The hybrid surgery for method No. 1 is performed as a one-stage procedure, together with patent ductus arteriosus (PDA) stenting. After median sternotomy, two Gore-Tex 1–2 mm wide bands with a diameter of 3–3.5 mm are put. When we apply method No. 2 then the thread is used to create bands. Method No. 3 is distinguished by intraoperative assessment of blood flow at the site of narrowing of the branches of the pulmonary artery and optional stenting of the PDA. The cuff for banding is made of Gore-Tex tubing. Effectiveness when applying method Nos. 1 and 2 is assessed by the change in invasive blood pressure and oxygen saturation after narrowing of the branches of the pulmonary artery. Also, with these techniques PDA stenting by inserting the introducer via pulmonary artery trunk is performed. Results: HLHS with mitral or aortic valve atresia or both was present in 19 patients (43.1%), with severe left heart obstruction resulting in PDA dependent systemic circulation in 16 babies (36.4%). CHD with single ventricle physiology occurred in 9 patients (20.5%). 14 babies (31.8%) undergo the procedure following the method No. 1, 8 patients (18.2%) method No. 2 and 22 patients (50%) method No. 3. Qp/Qs = 1/1 was achieved in 30 patients (30/44, 68.1%): as a result of the method No. 1 was achieved in 5 patients (5/14, 35.7%), method No. 2 in 4 patients (4/8, 50%), method No. 3 in 21 patients (21/22, 95.5%). Multivariate regression analysis revealed that method No. 3 significantly increases the chances of hemodynamic efficacy operations (OR = 35.0; p = 0.005; CI (95%) 3–411.5). Conclusion: Application of the operation technique No. 3 in combination with the intraoperative assessment of blood flow parameters at the site of banding of the branches of the pulmonary artery are the most optimal criteria for achieving Qp/Qs = 1/1. If there are signs of restriction at the level of the foramen ovale, atrioseptostomy should be done in the second stage after bilateral pulmonary banding.     

Anatomic Correlates of Mitral Systolic Anterior Motion in Transposition of the Great Arteries Following Atrial Switch Operation

Introduction: We sought to investigate whether the development of sub-pulmonic systolic anterior motion (SAM) may be inherent to the anatomy of the the mitral valve (MV) or affected by external factors, such as a dilated right ventricle or chest abnormalities in d-looped transposition of the great arteries post atrial switch operation (d-TGA/AtS). Methods: Analysis was performed of clinical and cardiac imaging studies acquired on 19 adult patients with d-TGA/AtS (age 42 ± 6 years old, 56% male) between 2015–2019. Echocardiography data included mitral apparatus anatomy, and CT/MRI data included biventricular dimensions, function, and Haller index (HI) for pectus deformity. Results: Patients with leaflet SAM (n = 6) compared to patients without SAM (n = 13) had higher MV protrusion height (2.3 ± 0.5 vs. 1.5 ± 0.4 cm, p ≤ 0.01) and longer anterior MV leaflet length (3.1 ± 0.4 cm vs. 2.6 ± 0.3 cm p ≤ 0.05), when compared to those without. CT/MRI showed higher sub-pulmonic left ventricular ejection fraction (LVEF) in the SAM group (71% ± 8% vs. 54% ± 7%, respectively). RV size and function, significant chest deformity (HI > 3.5), presence of a ventricular lead pacemaker, and septal thickness did not play a role in development of SAM. Conclusions: An elongated mitral apparatus is associated with the development of SAM, and the development of left ventricular outflow tract obstruction (LVOTO), in d-TGA/AtS. LV hyperkinesia is associated with SAM. Systemic RV dimensions, septal thickness, and degree of chest deformity did not differ significantly between subjects with SAM and those without.     

Long-Term Outcome and Risk Factor Analysis of Surgical Pulmonary Valve Replacement in Congenital Heart Disease

Objectives: To establish long-term outcome of surgical pulmonary valve replacement (PVR) in congenital heart disease (CHD) and to identify risk factors for overall mortality, operative mortality, and repetitive PVR. Methods: This is a retrospective study of 375 surgical PVR in 293 patients who underwent surgical PVR for CHD between January 2000 and May 2020. We only included patients with index PVR with previous open-heart surgery regardless of the number of PVRs. The previous surgical history of patients who underwent PVR during the study period was also included. Patients who underwent the Rastelli operation, and those who underwent single PVR without previous open-heart surgery were excluded. Results: The median age of the patients at the time of surgical PVR was 14.9 years (Interquartile range, IQR, 11.0–22.0). The median follow-up duration was 10.5 years (IQR, 5.5–14.8 years). There were 3 patients with operative mortality (1.0%) and 15 patients with overall mortality (5.1%). The survival rate was 95.1% over 20 years follow-up period. Multivariate analysis demonstrated that more than 3 times of previous open-heart surgeries before surgical PVR, older age at the first operation, longer cardiopulmonary bypass (CPB) time and longer intensive care unit (ICU) stay were predictors for overall mortality. Patients who underwent surgical PVR after more than 3 times of previous open-heart surgeries had significantly higher mortality than those who underwent open-heart surgeries less than 3 times (P < 0.001). Age younger than 10 years, male, multiple valve problems and longer ICU stay were significant predictors for repetitive PVR by multivariate analysis. Conclusions: Though surgical PVR has excellent long-term outcome, it should be performed with caution for those who previously underwent multiple open-heart surgeries, especially if patient received more than 3 times of open-heart surgeries.