Complex Heart Disease Research Articles

P005 CHILDREN WITH REPAIRED COARCTATION OF THE AORTA: FOLLOW-UP AND HYPERTENSIO5

Background and objective: Coarctation of the aorta (CoA) is a congenital heart disease with high morbidity and mortality due to cardiovascular disease even after adequate therapeutic management. A fundamental factor is the development of high blood pressure over the years. The objective of this work is to analyze the blood pressure phenotype of the population under follow-up in a high blood pressure clinic. Methods: Retrospective review of medical records of patients aged 0 to 22 years with CoA corrected by surgery or interventional catheterizations, which were followed up between January 2017 and February 2023 in the high blood pressure clinic of a pediatric center. Variables to analyze: age at the time of correction, technique, time since correction, associated cardiac or extracardiac pathologies, office blood pressure, left ventricular hypertrophy. In patients with adequate height and tolerance to the procedure: Ambulatory Blood Pressure Monitoring (ABPM) and pulse wave velocity study were performed. Results: 142 medical records were reviewed (see table 1). Two groups were formed according to the age of the patients due to the possibility of performing ABPM. There were no significant differences between the age of surgical approach and the association with complex heart diseases between the groups. The younger population presented a greater number of extended terminal anastomosis technique and increased concentric remodeling. The prevalence of hypertension and overweight obesity increased over the years. The older group had 25% masked hypertension and 25% increased pulse wave velocity Conclusion: Our population has 28% of hypertension among those under 8 years of age and it increases to 46% among the eldest, coinciding with international reports that indicate that the evolution towards arterial hypertension in this population is progressive. Lifelong follow-up of these patients with corrected CoA is important to detect recoarctation or the development of hypertension and act accordingly to improve morbidity and mortality.

3D regional evaluation of right ventricular myocardial work from cineCT.

Regional myocardial work (MW) is not measured in the right ventricle (RV) due to a lack of high spatial resolution regional strain (RS) estimates throughout the ventricle. We present a cineCT-based approach to evaluate regional RV performance and demonstrate its ability to phenotype three complex populations: end-stage LV failure (HF), chronic thromboembolic pulmonary hypertension (CTEPH), and repaired tetralogy of Fallot (rTOF). 49 patients (19 HF, 11 CTEPH, 19 rTOF) underwent cineCT and right heart catheterization (RHC). RS was estimated from full-cycle ECG-gated cineCT and combined with RHC pressure waveforms to create regional pressure-strain loops; endocardial MW was measured as the loop area. Detailed, 3D mapping of RS and MW enabled spatial visualization of strain and work strength, and phenotyping of patients. HF patients demonstrated more overall impaired strain and work compared to the CTEPH and rTOF cohorts. For example, the HF patients had more akinetic areas (median: 9%) than CTEPH (median: <1%, p=0.02) and rTOF (median: 1%, p<0.01) and performed more low work (median: 69%) than the rTOF cohort (median: 38%, p<0.01). The CTEPH cohort had more impairment in the septal wall; <1% of the free wall and 16% of the septal wall performed negative work. The rTOF cohort demonstrated a wide distribution of strain and work, ranging from hypokinetic to hyperkinetic strain and low to medium-high work. Impaired strain (-0.15≤RS) and negative work were strongly-to-very strongly correlated with RVEF (R=-0.89, p<0.01; R=-0.70, p<0.01 respectively), while impaired work (MW≤5 mmHg) was moderately correlated with RVEF (R=-0.53, p<0.01). Regional RV MW maps can be derived from clinical CT and RHC studies and can provide patient-specific phenotyping of RV function in complex heart disease patients. Evaluating regional variations in right ventricular (RV) performance can be challenging, particularly in patients with significant impairments due to the need for 3D spatial coverage with high spatial resolution. ECG-gated cineCT can fully visualize the RV and be used to quantify regional strain with high spatial resolution. However, strain is influenced by loading conditions. Myocardial work (MW) - measured clinically derived as the ventricular pressure-strain loop area - is considered a more comprehensive metric due to its independence of preload and afterload. In this study, we sought to develop regional RV myocardial work (MW) assessments in 3D with high spatial resolution by combining cineCT-derived regional strain with RV pressure waveforms from right heart catheterization (RHC). We developed our method using data from three clinical cohorts who routinely undergo cineCT and RHC: patients in heart failure, patients with chronic thromboembolic pulmonary hypertension, and adults with repaired tetralogy of Fallot.We demonstrate that regional strain and work provide different perspectives on RV performance. While strain can be used to evaluate apparent function, similar profiles of RV strain can lead to different MW estimates. Specifically, MW integrates apparent strain with measures of afterload, and timing information helps to account for dyssynchrony. As a result, CT-based assessment of RV MW appears to be a useful new metric for the care of patients with dysfunction.

Utilizing Fetal Echocardiography to Risk Stratify and Predict Neonatal Outcomes in Fetuses Diagnosed with Congenital Heart Disease.

Risk stratification of fetuses diagnosed with congenital heart disease (CHD) helps provide a delivery plan and prepare families and medical teams on expected course in the delivery room. Our aim was to assess the accuracy of echocardiographically determined risk-stratification assignments in predicting postnatal cardiac outcomes beyond the delivery room. This was a retrospective study at a single center evaluating all fetuses with CHD who were risk-stratified by echocardiographically determined level of care (LOC) assignment, ranging from 1a (lowest risk) to 4 (highest risk). All data were collected from January 1, 2017, to November 1, 2021. Outcomes included any unexpected cardiac interventions and neonatal clinical outcomes including in-hospital mortality, the need for prostaglandins or inotropes, and defined critical illness. These outcomes were assessed for each LOC assignment by Fisher's exact test. Out of 817 patients assigned a LOC, a total of 747 fetuses were included in our final cohort with a separate subanalysis of 70 fetuses diagnosed with coarctation of the aorta. The sensitivity and specificity were high for all LOC levels in predicting delivery room needs (93-100%). Higher LOC levels (3-4) had a lower positive predictive value (66-67%) indicating a high false-positive rate. Subjects with higher LOC assignments had a greater frequency of critical illness, hospital mortality, need for inotropes, need for neonatal surgical or catheterization interventions, and need for prostaglandins (p < 0.001 for all outcomes). A post-hoc analysis reviewing LOC assignments revealed a greater tendency to over-assign LOC at higher assignments (19% for LOC 3 and 4) compared to lower assignments (4% for LOC 1 and 2). Risk stratification based on fetal echocardiography can predict neonatal clinical outcomes and acuity of postnatal management needs. However, there is greater variability in expected clinical events and an expected degree of false positives for those with higher LOC assignments. · Risk stratification utilizing fetal echocardiography can be used to predict neonatal needs.. · Complex heart disease has lower positive predictive value in predicting postnatal clinical needs.. · There is a tendency to over-assign risk of acute hemodynamic instability for complex heart disease.. · False positives are expected when planning high-risk deliveries to avoid compromising situations..

Double outlet right ventricle accompanied by complex congenital anomalies: A rare association

Double outlet right ventricle (DORV) comprises a heterogeneous series of associated cardiac anomalies that involve the RV outflow tract in which both of the great arteries arise entirely or predominantly from the RV. The anatomic dysmorphology is variable, and there may be associated cardiac anomalies. We report here a case of a complex cyanotic heart disease in a 14 year old adolescent comprising of DORV accompanied by multiple congenital cardiac anomalies: single atrium, complete AV canal defect, severe AV valve regurgitation, D-malposition of great arteries, subaortic obstruction and pulmonary valvular stenosis.

Bilateral pulmonary artery banding facilitates the systemic ventricular outflow tract growth for biventricular and univentricular repair candidates of complex arch anomaly.

Various surgical approaches address complex heart disease with arch anomalies. Bilateral pulmonary artery banding (bPAB) is a strategy for critically ill patients with complex arch anomalies. Some reports argued the potential effect of bPAB on the growth of the left ventricular outflow tract (LVOT) during inter-stage after bPAB. This study aimed to analyze the LVOT growth for biventricular repair candidates with arch anomaly and systemic ventricular outflow tract (SVOT) for univentricular repair candidates with arch anomaly. This retrospective study analyzed 17 patients undergoing initial bPAB followed by arch repair. The Z-scores of LVOT and SVOT were compared between pre-bPAB and pre-arch repair. Patient characteristics, transthoracic echocardiogram data, and PAB circumferences were reviewed. The diameter of the minimum LVOT for biventricular repair (BVR) candidates, the pulmonary valve (neo-aortic valve, neo-AoV) and the pulmonary trunk (the neo-ascending aorta, neo-AAo) for univentricular repair (UVR) candidates, and the degree of aortic or neo-aortic insufficiency in each candidate was statistically analyzed. 17 patients were divided into the UVR candidates (group U) with 9 patients and the BVR candidates (group B) with 8 patients. In group B, the median value of the Z-score of the minimum LVOT increased from -3.2 (range: - 4.1 ~ - 1.0) at pre-PAB to -2.8 (range: - 3.6 ~ - 0.3) at pre-arch repair with a significant difference (p = 0.012). In group U, the median value of the Z-score of the neo-AoV increased from 0.5 (range: - 1.0 ~ 1.7) at pre-bPAB to 1.2 (range: 0.2 ~ 1.9) at pre-arch repair with a significant difference (p < 0.01). The median value of the Z-score of the neo-AAo was also increased from 3.1 (range: 1.5 ~ 4.6) to 4.3 (range: 3.1 ~ 5.9) with a significant difference (p = 0.028). The growth of the LVOT for BVR candidates and SVOT for UVR candidates during the inter-stage between bPAB and arch repair was observed. These results suggest the potential advantage of bPAB in surgical strategies. Further research is needed to validate these findings and refine surgical approaches.

DX-HeartNet: Explainable Model for Detecting Heart Diseases

In specific medical contexts, prioritizing the detection of complex cardiac conditions surpasses the importance of identifying common heart ailments. Unique insights into diagnostics have the potential to reveal crucial cardiovascular intricacies that may be crucial in real-world medical scenarios. In this research, we introduce an innovative framework called DX-HeartNet, drawing inspiration from deep learning methodologies while upholding the principles of interpretability in medical decision-making [1]. The primary objective of this study is to empirically demonstrate the effectiveness of the DX-HeartNet model in accurately and transparently detecting complex heart diseases. The proposed model employs an intricate architecture that captures intricate patterns within the data, elucidating the factors that contribute to the prediction of the disease. Unlike other machine learning approaches, DX-HeartNet successfully combines intricate features to uncover latent diagnostic attributes, facilitating comprehensive disease detection. The model’s performance is evaluated using diverse datasets, and its diagnostic capabilities are benchmarked against conventional methods. The results underscore the superiority of DX-HeartNet in identifying heart diseases, thereby outshining the prevailing deep-learning techniques in the realm of cardiac health assessment.

Complete Abnormal Venous Return Associated with Complex Heart Disease: Case Report S. Faiz, M. Boussif, B. Zouita, D. Basraoui, H. Jalal Published: April 27, 2024 | 4 2 DOI: 10.36347/sjmcr.2024.v12i04.035 Pages: 531-533 Downloads Abstract Total anomalous pulmonary venous return (TAPVR) is a cardiac malformation defined by a complete absence of communication between the pulmonary veins and the left atrium. All

Total anomalous pulmonary venous return (TAPVR) is a cardiac malformation defined by a complete absence of communication between the pulmonary veins and the left atrium. All oxygenated pulmonary venous return flows directly or indirectly into the right atrium. Survival is only possible via a right-to-left atrial shunt. Non-blocked forms present as high-flow shunts with moderate cyanosis. Symptoms in blocked forms are dominated by obstruction of venous return, and life-threatening distress with intense cyanosis and severe pulmonary hypertension sets in from the very first days of life. Treatment consists in connecting the pulmonary venous receptacle to the left atrium, tying off the collector and closing the inter-atrial septum. The risks of surgery remain high in severe forms, and failures usually occur in the first year of life. In the case of good surgical correction, the long-term prognosis is excellent.

Socioeconomic Influences on Outcomes Following Congenital Heart Disease Surgery.

Associations between social determinants of health (SDOH) and adverse outcomes for children with congenital heart disease (CHD) are starting to be recognized; however, such links remain understudied. We examined the relationship between community-level material deprivation on mortality, readmission, and length of stay (LOS) for children undergoing surgery for CHD. We performed a retrospective cohort study of patients who underwent cardiac surgery at our institution from 2015 to 2018. A community-level deprivation index (DI), a marker of community material deprivation, was generated to contextualize the lived experience of children with CHD. Generalized mixed-effects models were used to assess links between the DI and outcomes of mortality, readmission, and LOS following cardiac surgery. The DI and components were scaled to provide mean differences for a one standard deviation (SD) increase in deprivation. We identified 1,187 unique patients with surgical admissions. The median LOS was 11days, with an overall mortality rate of 4.6% and readmission rate of 7.6%. The DI ranged from 0.08 to 0.85 with a mean of 0.37 (SD 0.12). The DI was associated with increased LOS for patients with more complex heart disease (STAT 3, 4, and 5), which persisted after adjusting for factors that could prolong LOS (all p < 0.05). The DI approached but did not meet a significant association with mortality (p = 0.0528); it was not associated with readmission (p = 0.36). Community-level deprivation is associated with increased LOS for patients undergoing cardiac surgery. Future work to identify the specific health-related social needs contributing to LOS and identify targets for intervention is needed.

The burden of psychological trauma and post-traumatic stress disorder among adults with congenital heart disease: PTSD in ACHD

BackgroundPsychological trauma, symptoms of post-traumatic stress disorder (PTSD), and mental health conditions are common in adult congenital heart disease (ACHD). There is a gap in the literature examining PTSD in ACHD using the current DSM-5 criteria, while assessing patient characteristics and experiences with trauma-focused treatment. MethodsSurveys were offered to outpatients over a six-month enrollment period to be completed via QR code on their personal smart phone. Patient-reported items include a detailed medical and psychosocial history, the Oslo Social Support Scale (OSSS-3), Adverse Childhood Experiences (ACEs) survey, and the PTSD Checklist for DSM-5 (PCL-5). ResultsOf 158 patients (77% moderate or complex heart disease) who provided complete data, a provisional diagnosis of PTSD was found in 48 patients (30%), using a PCL-5 cutoff score of ≥ 31. A positive PTSD screen was associated with younger age, non-white race, presence of heart failure, lower New York Heart Association Functional Class, lower linear Quality of Life score, lower OSSS-3 score, an insecure caregiver relationship, period of unemployment, emergency department visits, medication nonadherence, and coexisting mental health disorders. Complexity of heart disease and number of surgical and/or catheter-based interventions was not associated with PTSD, although having undergone no cardiac surgeries until adulthood (≥ 18 years old) was associated with a lower prevalence of PTSD. Those who screened positive for PTSD were more likely to report multiple traumatic events, including noncardiac traumatic events. Only 14/48 patients (29%) reported a known diagnosis of PTSD, although 44 patients (92%) reported having ever seen a mental health provider. Eighteen patients (38%) reported currently having a mental health provider. Thirty patients (62%) had heard of at least one evidence-based trauma-informed therapy, and 14 (29%) had tried at least one. ConclusionsUsing the DSM-5 criteria, we observed a high prevalence of potential PTSD in ACHD associated with several novel cardiac and psychosocial patient factors. Future longitudinal studies will be necessary to establish causality. Few individuals with ACHD have been formally diagnosed with PTSD or have experience with evidence-based trauma-informed therapies.

Patients' prognosis with congenital heart disease followed by ten years: survival and associated factors.

To evaluate the prognosis and influence of associated factors in patients with congenital heart disease admitted for the first time to the Intensive Care Unit of the Hospital da Criança Santo Antônio/Irmandade da Santa Casa de Misericórdia de Porto Alegre, especially those factors associated with death. Patients were prospectively and consecutively allocated over a period of one year (August 2005 to July 2006). Now, 15 years after the initial selection, we collected data from these patients in the database of the Cytogenetics Laboratory of the Universidade Federal de Ciências da Saúde de Porto Alegre and in the medical records of the hospital. Of the 96 patients, 11 died and 85 were alive until 20 years old. Four patients died in the Intensive Care Unit. The survival probability up to 365 days of life was 95.8%. The survival assessment identified that the deaths occurred mainly before the patients completed one thousand days of life. We found that complex heart disease was independently associated with an odds ratio of 5.19 (95% confidence interval - CI:1.09-24.71; p=0.038) for death. Knowledge about the factors that interfere with the prognosis can be crucial in care practice planning, especially considering that congenital heart disease is an important cause of mortality in the first year of life.

Increased cesarean section rate and premature birth according to modified WHO maternal cardiovascular risk in pregnant women with congenital heart disease

Background During pregnancy and delivery, hemodynamics are altered and complex congenital heart disease has been associated with adverse maternal and neonatal outcomes. We sought to investigate pregnancy outcome and complications in relation to complexity of heart condition. Materials and methods We studied women with ACHD discussed at multidisciplinary conferences at Lund University Hospital March 2009-May 2021. We studied 149 pregnancies in 101 women. We scored each woman retrospectively according to the modified World Health Organization (mWHO) risk classification and included patients in risk class I (n = 36, 24.1%), II (n = 43, 28.9%), II-III (n = 43, 28.9%), III (n = 24, 16.1%) and IV (n = 3, 2.0%). Results Women with mWHO class ≥III underwent cesarean section more often than women in less complex mWHO classes, (OR, 5.1; 95% CI, 2.0–12.5; p&lt;0.001). The odds of premature delivery were significantly higher among pregnant women with mWHO class ≥III (OR, 6.7; 95% CI, 2.6–17.4; p&lt;0.001). We found no difference in incidence of preeclampsia, gestational hypertension, gestational diabetes, hemorrhage &gt;1000 ml or cardiac defect in the neonate depending on WHO-class. Women in mWHO classes III-IV had a higher rate of fetal growth restriction (FGR) compared to women in mWHO classes I, II, II-III (p&lt;0.007). Conclusions Our findings indicate that women with more complex heart disease (mWHO classes III or IV) tend to have a higher rate of cesarean section, premature birth and FGR.

Regional Variation in Pulmonary Arterial Hypertension in the United States: The Pulmonary Hypertension Association Registry.

Rationale: Pulmonary arterial hypertension (PAH) is a heterogeneous disease within a complex diagnostic and treatment environment. Other complex heart and lung diseases have substantial regional variation in characteristics and outcomes; however, this has not been previously described in PAH. Objectives: To identify baseline differences between U.S. census regions in the characteristics and outcomes for participants in the Pulmonary Hypertension Association Registry (PHAR). Methods: Adults with PAH were divided into regional groups (Northeast, South, Midwest, and West), and baseline differences between census regions were presented. Kaplan-Meier survival analyses and Cox proportional hazards were used to estimate the association between region and mortality in unadjusted and adjusted models. Results: Substantial differences by census regions were seen in age, race, ethnicity, marital status, employment, insurance payor breakdown, active smoking, and current alcohol use. Differences were also seen in PAH etiology and baseline 6-minute walk distance test results. Treatment characteristics varied by census region, and mortality appeared to be lower in PHAR participants in the West (hazard ratio, 0.60; 95% confidence interval, 0.43-0.83, P = 0.005). This difference was not readily explained by differences in demographic characteristics, PAH etiology, baseline severity, baseline medication regimen, or disease prevalence. Conclusions: The present study suggests significant regional variation among participants at accredited pulmonary vascular disease centers in multiple baseline characteristics and mortality. This variation may have implications for clinical research planning and represent an important focus for further study to better understand whether there are remediable care aspects that can be addressed in the pursuit of providing equitable care in the United States.

Anomalous venoatrial connections – CT and MRI assessment

Abnormal venous atrial (VA) connections present a congenital heart disease (CHD) challenge for pediatric cardiologists. Fully anatomical evaluation is very difficult in prenatal and perinatal follow-up, but it has a profound impact on surgical correction and outcome. The echocardiogram is first-line imaging and represents the gold standard tool for simple abnormal VA connection. CT and MRI are mandatory for more complex heart disease and “nightmare cases”. 3D post-processing of volumetric CT and MRI acquisition helps to clarify anatomical relationships and allows for the creation of 3D printing models that can become crucial in customizing surgical strategy.Our article describes a ten-year (2013–2022) tertiary referral CHD center of abnormal AV connections investigated with CT and MRI, illustrating most of these complex diseases with the help of volume rendering (VR) or multiplanar reconstructions (MPR). The nightmarish cases will also be addressed due to the complex cardiovascular arrangement that requires a challenging surgical solution for correction along with the post-surgical complications.

Transcriptome data analysis of primary cardiomyopathies reveals perturbations in arachidonic acid metabolism.

Cardiomyopathies are complex heart diseases with significant prevalence around the world. Among these, primary forms are the major contributors to heart failure and sudden cardiac death. As a high-energy demanding engine, the heart utilizes fatty acids, glucose, amino acid, lactate and ketone bodies for energy to meet its requirement. However, continuous myocardial stress and cardiomyopathies drive towards metabolic impairment that advances heart failure (HF) pathogenesis. So far, metabolic profile correlation across different cardiomyopathies remains poorly understood. In this study, we systematically explore metabolic differences amongst primary cardiomyopathies. By assessing the metabolic gene expression of all primary cardiomyopathies, we highlight the significantly shared and distinct metabolic pathways that may represent specialized adaptations to unique cellular demands. We utilized publicly available RNA-seq datasets to profile global changes in the above diseases (|log2FC| ≥ 0.28 and BHadjusted p-val 0.1) and performed gene set analysis (GSA) using the PAGE statistics on KEGG pathways. Our analysis demonstrates that genes in arachidonic acid metabolism (AA) are significantly perturbed across cardiomyopathies. In particular, the arachidonic acid metabolism gene PLA2G2A interacts with fibroblast marker genes and can potentially influence fibrosis during cardiomyopathy. The profound significance of AA metabolism within the cardiovascular system renders it a key player in modulating the phenotypes of cardiomyopathies.

P310 CARDIOPULMONARY EXERCISE TEST IN THE EVALUATION OF A PATIENT WITH HFREF: THE IMPORTANCE IN THE ANALYSIS OF GAS KINETICS

Abstract Introduction exertional dyspnea is a symptom present in several diseases, identifying the origin is of fundamental diagnostic and therapeutic importance. Cardiorespiratory exercise testing (CPET) is a valuable tool not only to assess functional capacity but also for diagnostic and prognostic purposes. Clinical case 55–year–old patient with a history of episode of acute pulmonary edema. Echocardiogram showed FE 35% with diffuse left ventricular (LV) hypokinesis and mild–to–moderate mitral insufficiency (MI). Diagnostic coronarography showed lesion–free coronary tree. An interrupted CPET at 140W showed peak O2 consumption of 23.6 mL/kg/min that excluded indication for cardiac transplantation. An echocardiogram from us confirmed dilated cardiomyopathy (CMPD) with FE=32%, diffuse VS hypokinesia and mild–to–moderate functional MI. CPET was repeated, maximal for respiratory quotient (1.42) interrupted due to muscle exhaustion, indicative of moderate reduction in functional capacity with peak VO2 at 64% of predicted, preserved anaerobic threshold at 51% of VO2 max and reduced VO2/W and Pulse Oxygen Ratio (VO2/HR) values from cardiogenic limitation. Also evident drop in VO2/HR during the final stages of the test and associated change in slope of the VO2/Work relationship a sign of decline in stroke volume and cardiogenic efficiency. Absent signs of ventilatory limitation, pulmonary vascular (Ve/VCO2=20.8 in normal range), or exercise desaturation. In the final phase of the test increase in VCO2 and Ve/VCO2 and Ve/VO2 equivalents. In order to find the explanation to the data collected by CPET, exercise echocardiogram was performed, which revealed the development of severe mitral valvular insufficiency and subsequent pulmonary hypertension justifying the behavior of the parameters collected by CPET. Discussion The oxygen pulse represents a metabolic surrogate for stroke volume. Normally during the active phases of exertion there is a progressive increase followed by a plateau. The evident flattening of the VO2/Work curve during the final phases of exercise and the concomitant decrease in oxygen pulse denote inadequate cardiac performance relative to the increased energy demands during exercise. Conclusions CPET proves to be a valuable clinical tool to guide the diagnostic–therapeutic pathway, particularly in the presence of complex heart disease (CMPD or IM).

Utility of fetal cardiovascular magnetic resonance imaging in assessing the fetuses with complete vascular ring.

This study aimed to report our experience in qualitative and quantitative evaluation of fetal complete vascular ring (CVR) using fetal cardiovascular magnetic resonance imaging (MRI) to improve prenatal diagnosis and make early postnatal management possible. A retrospective case-control study was performed on cases of CVR diagnosed using fetal cardiovascular MRI, and confirmed by postnatal imaging diagnosis. Associated abnormalities were recorded. The diameters of aortic arch isthmus (AoI) and ductus arteriosus (DA), and tracheal diameters in fetuses with tracheal compression were measured and compared with those of the control group. All fetal CVR cases in this study included right aortic arch (RAA) with aberrant left subclavian artery (ALSA) and left DA (n = 93), double aortic arch (DAA) (n = 29), RAA with mirror-image branching and retroesophageal left ductus arteriosus (RLDA) (n = 8). Compared with the control group, the diameters of AoI in fetuses with DAA were decreased (p < 0.001), and the diameters of DA in fetuses with RAA with ALSA and left DA were increased (p < 0.001). The diameters of AoI and DA were positively correlated with gestational age (GA) in the normal control group (both p < 0.001); The diameters of AoI and DA were also positively correlated with GA in RAA with ALSA and left DA subgroup (both p < 0.001) and RAA with mirror-image branching and RLDA subgroup (AoI: p = 0.003; DA: p = 0.002); The diameters of DA were positively associated with GA in DAA subgroup (p < 0.001), however, there was no linear tendency between the diameters of AoI and GA in the DAA subgroup (p = 0.074). There were CVR fetuses with associated intracardiac malformation (n = 13), especially ventricular septal defect rather than complex heart disease, and extracardiac malformation (n = 14). Sixteen fetuses were shown the airway compression whose tracheal diameters were smaller than the normal (p < 0.001). The altered diameters of AoI and DA can be detected and measured in CVR fetuses using fetal cardiovascular MRI. Fetal CVR can occur alone or with intracardiac and extracardiac malformation. Fetal CVR can be associated with prenatal airway compression.

Echocardiographic findings in infants of diabetic mothers and their relation to maternal glycemic control

Background Diabetes mellitus during pregnancy is a common risk factor for both mothers and infants. Prediction of cardiac problems in infants of diabetic mothers is challenging. Aim The purpose of the study is to determine the type and incidence of congenital heart diseases in infants whose mothers are diabetic in the NICU of Alexandria university maternity hospital, as well as to evaluate functional and morphological changes in the myocardium and its relationship to maternal glycemic control. Patients and methods A case-control study was conducted in the NICU of Alexandria University Hospital. Thirty-five infants of diabetic mothers (IDM) were compared to 37 healthy control infants. All diabetic mothers had their HBA1C levels measured before delivery. Therefore, the infants of diabetic mothers were further divided into 22 infants whose mothers had controlled diabetes (controlled DMM group; controlled diabetes mellitus mothers group) during pregnancy (predelivery HBA1C≤6.5) and 13 infants whose mothers had uncontrolled diabetes during pregnancy (uncontrolled DMM group). All studied infants were scanned for the presence of congenital heart diseases. Right and left ventricular systolic functions were assessed, and septal and left ventricular dimensions were measured using functional echocardiography (FE). Followup scans were done 1–2 months later. This work was registered on clinical trial.gov NCT05050058 Results Incidence of congenital heart diseases is significantly increased in infants of diabetic mothers compared to healthy control group with P value =0.008. Intraventricular septal thickness and left ventricular posterior wall during systole and diastole (IVSs, IVSd, LVPWs, LVPWd and IVSd/ LVPWd ratio) significantly increased in IDM group. Patients whose mothers had uncontrolled diabetes had their IVSd and IVSd/ LVPWd ratio markedly increased over the uncontrolled group. After 45–60 days the septal and posterior wall measures during systole and diastole decreased significantly. Tricuspid regurge (TR) gradient is markedly increased in IDM in the first 24 h after birth, specifically in infants with mothers with poor glycemic control during pregnancy. Conclusion Infants of diabetic mothers were significantly more likely to suffer from ASD, VSD, and complex heart disease. Complex heart diseases were significantly associated with pregestational maternal diabetes. Infants of diabetic mothers with poor glycemic control have affected IVSd and IVSd/LVPWd ratios. Ventricular septal and LVPW measures were markedly improved after 45–60 days of age .Neither the presence of maternal diabetes nor the diabetic control of mothers affected the right and left ventricular functions of their infants.

Emerging role of positron emission tomography (PET) imaging in cardiac surgery.

Historically, structural and anatomical imaging has been the mainstay in the diagnosis and management of cardiovascular diseases. In recent years there has been a shift toward increased use of functional imaging studies, including positron emission tomography (PET). PET is a noninvasive nuclear medicine-imaging technique that uses radiotracers to generate images of a radionucleotide distribution by detecting the physiologic substrates that emit positron radionuclides. This article will focus on the applications of PET imaging for the cardiac surgeon and highlight the collaborative nature of using PET imaging for the management of complex heart disease. We present cases that demonstrate the value of using PET imaging in the diagnosis of coronary artery disease and management of complex endocarditis, and in targeted cardiovascular therapies.

Utilization of a Prenatal Palliative Care Consultation Pathway for Congenital Heart Disease.

Over 4 decades, short- and long-term survival for children with congenital heart disease (CHD) has significantly increased. Those with complex CHD have a 1-year mortality rate of approximately 25%. Prenatal access to palliative care ensures a comprehensive approach to the infant's physical, emotional, spiritual, and psychosocial well-being and offers enhanced support for parents and families while providing an interprofessional approach to care. Pediatric cardiac provider attitudes, knowledge, patterns of prenatal palliative care, and consultation for fetuses with complex heart disease to guide increased and appropriate prenatal consultation were explored for a quality improvement project completed at a single institution. A retrospective cardiac database review identified that 21.5% of maternal patients seen prenatally and carrying a fetus with complex CHD would have qualified for prenatal palliative care consultation. To increase prenatal palliative care consultation, a pathway for referral was developed. A pre-evaluation of pediatric cardiac provider attitudes regarding palliative care was measured. Survey data informed the intervention phase of prenatal palliative care educational material development, which was presented to pediatric cardiac providers. The number of prenatal palliative care consults was tracked. A postintervention survey completed by pediatric cardiac providers showed increased understanding and utilization of palliative care for comfort and support and not just reserved for death preparation. Greater than 93% agreed or strongly agreed that they gained a better understanding of appropriate use of palliative/supportive care. As pediatric cardiac providers become more knowledgeable about early integration of palliative care, parents and infants will benefit greatly from referrals and initiation of palliative care at the time of prenatal diagnosis.

20 years of experience with the Fontan procedure: characteristics and clinical outcomes of children in a tertiary referral hospital.

Without participating in a contractile chamber, the Fontan procedure seeks to create a separation of oxygenated and deoxygenated blood in patients with univentricular heart, reducing the risks of long-term hypoxemia and improving their survival. This study describes the clinical outcomes of children undergoing the Fontan procedure between 2000 and 2020 in a tertiary referral hospital care centre in southwestern Colombia. A retrospective observational descriptive study. The 81 patients who underwent the Fontan procedure were included. Categorical variables were presented with percentages and continuous variables with measures of central tendency according to the distribution of the data evaluated through the Shapiro-Wilk test. Sociodemographic, clinical, surgical variables, complications, and mortality were described. Between 2000 and 2020, 81 patients underwent the Fontan procedure: 43 (53.1%) males and a median age of 5.3years (interquartile range 4.3-6.6). The most common diagnosis was tricuspid atresia (49.4%). The median mean pulmonary arterial pressure was 12mmHg (interquartile range 10-15), the Nakata index 272mm2/m2 (interquartile range 204-327), and the McGoon index (interquartile range 1.86-2.3). Seventy-two (88.9%) patients underwent extracardiac Fontan and 44 (54.3%) patients underwent fenestration. The median hospitalisation days were 19days. The main complication was coagulopathy (19.8%), mortality in the first month between 2000 and 2010 was 8.6%, and after 2010 was 1.2%. The Fontan procedure is a palliative surgery for children with complex heart disease. According to anatomical and physiological variables, the proper choice of patients determines the short- and long-term results.