Search

Advances in the management of congenital heart disease (CHD) have significantly decreased mortality rates, indicating a need for continuous care as a chronic condition throughout the child's lifespan. This study examined the association of nurse–mother partnerships with parenting stress and family resilience among South Korean mothers of children with CHD. This descriptive study involved 93 mothers of children aged six years or younger with CHD admitted to a hospital. Data were collected from September to November 2020 and analyzed using SPSS/WIN (version 29.0) for t -tests, analyses of variance, Pearson's correlation coefficient, and multiple regression analyses. The means and standard deviations of the nurse – mother partnership, parenting stress, and family resilience were 4.13 ± 0.47, 76.98 ± 16.6, and 56.54 ± 7.86 points, respectively. Parenting stress increased as the number of hospitalizations and surgeries increased and with complex types of CHD. Nurse–mother partnerships were stronger with longer hospital stays. Family resilience was higher with younger children, fewer rehospitalizations, and shorter hospital stays. A positive correlation was found between nurse–mother partnerships and family resilience, and a negative correlation between parenting stress and family resilience. Factors influencing parenting stress included family resilience, rehospitalizations, and complex types of CHD, and those affecting family resilience were nurse–mother partnerships, parenting stress, and the child's age. Nurse–mother partnerships significantly affect family resilience. Enhancing nurse–mother partnerships can improve family resilience, which in turn can reduce parenting stress, thus offering guidance for future nursing interventions. [ABSTRACT FROM AUTHOR]

Neonates with critical congenital heart disease of the ductal-dependent pulmonary circulation type (CCHD-DDPC) require prostaglandin E1 (PGE1) to maintain oxygen saturation until surgery. However, the factors contributing to the maintenance doses of PGE1 remain unclear. This study aimed to determine the predictors of high maintenance PGE1 doses in these neonates. This retrospective cohort study included neonates with CCHD-DDPC at Songklanagarind Hospital between January 1, 2006, and December 31, 2021. Factors associated with high maintenance PGE1 doses (> 0.01 mcg/kg/min) were analyzed to identify predictors. Odds ratios were calculated using tabulation and logistic regression analysis. A prediction score was developed for high maintenance PGE1 doses. Among 96 neonates with CCHD-DDPC, 55 % required high maintenance doses of PGE1. Three factors significantly associated with high maintenance PGE1 doses were patent ductus arteriosus (PDA) size-to-birthweight ratio ≤1.3 mm/kg, initial PGE1 dose >0.03 mcg/kg/min, and preoperative invasive mechanical ventilation. The area under the receiver operating characteristic curve for these three predictors was 0.7409. A predictive score of 0–3 was created based on these factors. The probabilities of receiving a high maintenance dose of PGE1 for patients with overall scores of 0, 1, 2, and 3 were 0.19 (95 % CI: 0.04–0.33), 0.42 (95 % CI: 0.30–0.54), 0.69 (95 % CI: 0.57–0.81), and 0.87 (95 % CI: 0.76–0.99), respectively. In neonates with CCHD-DDPC, a PDA size-to-birth weight ratio ≤1.3 mm/kg, an initial dose of PGE1 > 0.03 mcg/kg/min, and preoperative invasive mechanical ventilation were predictors of high maintenance PGE1 doses during the preoperative period. [ABSTRACT FROM AUTHOR]

We report a case of an active-duty diver who developed severe decompression sickness with concomitant patent foramen ovale that was successfully closed contrary to standard guideline recommendations. This case should prompt evaluation of the role of cardiac screening in occupational divers, including tactical athletes, relative to recreational divers. [ABSTRACT FROM AUTHOR]

Neonates with congenital heart disease (CHD) have smaller brain volume at birth. High rates of placental vascular malperfusion lesions may play a role in disrupted brain development. This is a single-center retrospective cohort study of infants born between 2010 and 2019 who were diagnosed with a major cardiac defect requiring surgery in the first year of life. Doppler ultrasound RI of the middle cerebral artery (MCA) and anterior cerebral artery were calculated within the first 72 hours of life. Placentas were evaluated using a standardized approach. Over the study period, there were 52 patients with hypoplastic left heart syndrome (HLHS), 22 with single-ventricle right ventricular outflow tract obstruction (SV-RVOTO), 75 with a two-ventricle cardiac defect (2V), and 25 with transposition of the great arteries (TGA). MCA Doppler RI were significantly higher for all subgroups of CHD compared with control subjects (0.68 ± 0.11 in control subjects compared with 0.78 ± 0.13 in HLHS, P = 0.03; 0.77 ± 0.10 in SV-RVOTO, P = 0.002; 0.78 ± 0.13 in 2V, P = 0.03; and 0.80 ± 0.14 in TGA; P = 0.001) with the highest average MCA RI in the TGA group. In subgroup analyses, placental fetal vascular malperfusion in the 2V group was associated with higher MCA RI, but this relationship was not present in other subgroups, nor in regards to maternal vascular malperfusion. Major forms of CHD are associated with significantly higher cerebral artery RI postnatally, but placental vascular malperfusion lesions may not contribute to this hemodynamic adaptation. [ABSTRACT FROM AUTHOR]

Heart failure results in significant morbidity and mortality for young children with hypoplastic left heart syndrome (HLHS) following the Norwood procedure. The trajectory in later childhood is not well described. We studied the outcome into adolescence of participants enrolled in the Single Ventricle Reconstruction trial who underwent the Fontan procedure or survived to 6 years without having undergone Fontan procedure. The primary outcome was heart failure events, defined as heart transplant listing or death attributable to heart failure. Symptomatic heart failure for participants surviving 10 or more years was also assessed utilizing the Pediatric Quality of Life Inventory (PedsQL). Of the 345 participants who underwent a Fontan operation or survived to 6 years without Fontan, 25 (7.2%) had a heart failure event before the age of 12 years. Among these, 21 were listed for heart transplant, and 4 died from heart failure. Nineteen participants underwent heart transplant, all of whom survived to age 12 years. Factors associated with a heart failure event included longer Norwood hospital length of stay, aortic atresia, and no Fontan operation by age 6 years. Assessment of heart failure symptoms at 12 years of age revealed that 24 (12.2%) of 196 PedsQL respondents "often" or "almost always" had difficulty walking more than one block. Heart failure events occur in over 5% of children with palliated HLHS between preschool age and adolescence. Outcomes for children listed for transplant are excellent. However, a substantial portion of palliated HLHS children have significant symptoms of heart failure at 12 years of age. [ABSTRACT FROM AUTHOR]

Interrupted aortic arch (IAA) is a rare congenital heart condition where there is a complete discontinuation between the ascending and descending aorta. The association with a patent ductus arteriosus or developed arterial collateral allows survival until adulthood in extremely rare cases. We report a case series of adult forms of IAA. Even if the incidence is very rare, IAA should be could excluded in the setting of resistant asymmetrical hypertension. The first case is singular regarding its association with a bicuspid aortic valve and aortic aneurysm, while the second case is characterized by massive left ventricular hypertrophy. The diagnosis was suspected on echocardiographic findings and confirmed by computed tomography angiography. Both patients refused surgical repair given the high operative risk. -The evaluation of severe resistant high blood pressure should include complementary investigation for adult form interrupted aortic arch. -To understand the major impact of multimodality imaging for the recognition, localization, and stratification of interrupted aortic arch. [ABSTRACT FROM AUTHOR]

Necropsy of a 52-day-old Camborough pig revealed numerous cardiac malformations. The positional relationship of the atria, ventricles and great vessels was a mirror image type (I, L and L): inverted arrangement of the atria, with a left-sided right atrium and right-sided left atrium (situs inversus); inverted arrangement of the ventricles, with a left-sided morphological right ventricle and right-sided morphological left ventricle (L-loop); and aortic valve to the front left relative to the pulmonary valve (L-malposed). The major malformations included an ostium secundum atrial septal defect, cor triatriatum sinister (CTS), a subpulmonary ventricular septal defect and a bicuspid pulmonary valve. Histological examination revealed myocyte hypertrophy, focal myocardial necrosis and calcification in the left morphological right ventricle of the heart. To the best of our knowledge, this is the first report of CTS in pigs. Although the individual malformations found in the present case are not unique, an unusual combination of these cardiac malformations has not been described in animals. [Display omitted] [ABSTRACT FROM AUTHOR]

This exploratory study examines differences in parents' quality of life by treatment decision and the child's survival outcome in the context of life-threatening congenital heart disease (CHD). Parents of a fetus or neonate diagnosed with severe CHD enrolled in the observational control group of a clinical trial (NCT04437069) and completed quality of life (i.e., contact with clinicians, social support, partner relationship, state of mind), mental and physical health survey measures. Comparisons were made between parents who chose comfort-directed care or surgery and between those whose child did and did not survive. Parents who chose surgery and their child did not survive reported the most contact with their clinicians. Parents who chose comfort-directed care reported lower social support than parents who chose surgery and their child did not survive as well as poorer state of mind compared to parents who chose surgery. Some aspects of parents' quality of life differed based on their treatment decision. Parents who choose comfort-directed care are vulnerable to some negative outcomes. Decision support tools and bereavement resources to assist parents with making and coping with a complex treatment decision is important for clinical care. • Parents who chose comfort-directed care reported less contact with clinicians. • Parents who chose comfort-directed had less social support and poor state of mind. • Parents who chose surgery (child did not survive) were highly connected with clinicians. • Parents who chose comfort-directed care may need enhanced support and counseling. • Equitable support regardless of treatment decision is needed in clinical care. [ABSTRACT FROM AUTHOR]

• CHD care & COVID-19 risk factors predict negative parental mental health. • Resilience and support are protective factors for anxiety, depression, and stress. • Interventions that promote resilience in CHD parents are needed. Little is known about the mental health burden or the factors that contribute to mental health variables in parents of children with congenital heart defects (CHD) during the COVID-19 pandemic. The purpose of this study was to examine risk and protective factors associated with anxiety symptoms, depression symptoms, and perceived stress in parents of young children with CHD during the COVID-19 pandemic. A nonexperimental design was used in this study of 127 parents of children aged newborn to five years with CHD during the COVID-19 pandemic. Regression analyses were conducted to examine associations between COVID-19 stressors, CHD care-related factors, parental resilience, external support, and mental health variables. Parental resilience, emotional support, and informational support were key protective factors for anxiety, depression, and stress. However, resilience was remarkably low in CHD parents. Increased levels of anxiety symptoms, depression symptoms, and perceived stress were associated with 1) Exposure to a greater number of COVID-19-related stressors, 2) Distress from family visitation restrictions during healthcare encounters, 3) Worry related to the perceived risk of their CHD child's exposure to COVID-19 during healthcare encounters, and 4) Worry about their CHD child's risk of death or serious illness from COVID-19. The additive impact of the COVID-19 pandemic plus CHD care-related worries on this parent population's mental health is significant. Interventions that promote resilience, address the effect of healthcare system changes, and support the needs of parents of young children with CHD during this and future pandemics are needed. [ABSTRACT FROM AUTHOR]

• Quadricuspid aortic valve is a rare congenital heart defect. • Echocardiography is a useful tool for quadricuspid aortic valve diagnosis. • Prevalence of this congenital heart defect remains unknown in the horse. A 16-year-old horse, 400 kg, male, Quarter horse gelding was examined for a cardiac murmur that had not been previously heard. Physical examination revealed a regularly irregular pulse and a grade III/VI, decrescendo, diastolic murmur with a point of maximum intensity over the left heart base. Base-apex standard electrocardiographic examination at rest showed sinus rhythm with second-degree atrio-ventricular blocks. Echocardiography identified the presence of four aortic valve cusps, two equal larger and two unequal smaller cusps. Color flow Doppler examination showed a diastolic regurgitant jet emerging from the central region of closed aortic valve. Based on clinical and echocardiographic findings a diagnosis of mild to moderate aortic valve insufficiency secondary to quadricuspid aortic valve was made. This report describes a rare congenital heart defect that can be detected by transthoracic echocardiography in the horse. [ABSTRACT FROM AUTHOR]

Enhanced techniques for single-ventricle palliation (SVP), in which the Fontan procedure is the final phase, have improved the survival and quality of life in patients not eligible for biventricular repair (BiVR). However, Fontan physiology also is associated with predictable long-term consequences that ultimately affect quality of life and freedom from adverse events. Given this harsh reality, the advances in ventricular rehabilitation strategies and the conversion from SVP increasingly are being used to achieve biventricular circulation in patients with left-heart hypoplasia who previously were considered to be marginal or unacceptable candidates for BiVR. The techniques for ventricular recruitment include restriction of the atrial septal defect (ASD), atrioventricular valve repair, repair of left ventricular outflow obstruction, endocardial fibroelastosis resection, an augmented pulmonary blood flow, and catheter-based interventions. In addition, reverse double-switch surgery has been used as an option for patients with a left ventricle of insufficient size and function to support the systemic circulation, who would otherwise require a Fontan surgery. Despite improved management and surgical technique, the outcomes after biventricular staging repair and conversion remain variable, and the complications ultimately may exclude patients from future single-ventricle palliation or transplant. The anesthesiologist should have a basic understanding of the anatomy and physiology of this growing patient population in order to develop adequate perioperative management options. [ABSTRACT FROM AUTHOR]

The Fontan surgery was developed as a palliative intervention for congenital heart disease (CHD) patients with single-ventricle physiology who are not candidates for a biventricular repair. Improvements in the surgery and medical management of these patients have increased survival, yet this population remains at risk for complications and end-organ dysfunction due to Fontan failure. Lymphatic vessels maintain a fluid balance within the extracellular space, participate in fat reabsorption from the small intestine, and play an important role in the body's immune response. Altered Starling forces at the capillary level, capillary leak, and lymphatic obstruction contribute to lymphatic dysfunction in patients with Fontan physiology. These lymphatic complications include edema, pleural effusions, plastic bronchitis (PB), and protein-losing enteropathy (PLE). Over the past decade, there have been innovations in lymphatic imaging. These new imaging techniques include noncontrast magnetic resonance (MR) lymphangiography, intranodal lymphangiography (IL), dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL), and liver lymphangiography. These imaging techniques help in delineating anatomy and guiding the appropriate therapeutic approach. Lymphatic interventions then may be performed to decompress the lymphatic system or to identify and occlude abnormal lymphatic vessels and drainage pathways. The anesthesiologist should have an understanding of the effects of lymphatic disorders on the Fontan circulation and apply appropriate management techniques for the associated interventions. The Fontan surgery was developed as a palliative intervention for CHD patients with single-ventricle physiology who are not candidates for a biventricular repair. The surgery creates a series systemic and pulmonary circulation with the energy necessary to provide gradient-driven pulmonary blood flow generated by the ventricle. 1 In the past decades, improvements in the surgery and medical management of these patients have increased survival, with 30-year survival rates close to 85%. 2 Despite these improvements, this population remains at risk for complications and end-organ dysfunction due to Fontan failure, which is characterized by elevated systemic venous pressures and low cardiac output. These complications include arrhythmias, cardiac dysfunction, ascites, liver fibrosis/cirrhosis, renal dysfunction, pulmonary failure, and lymphatic complications such as edema, pleural effusions, PB, and PLE. Complications ultimately contribute to increased risk for hospitalization, death, and need for heart transplantation. 3,4 For this reason, there has been increasing interest in the role of abnormal lymphatic circulation in the genesis of Fontan failure. The authors characterize the lymphatic pathophysiology associated with Fontan physiology and review the imaging and interventional strategies used to treat these patients. [ABSTRACT FROM AUTHOR]

Ebstein anomaly (EA) is a congenital heart defect affecting the right heart. Heart failure (HF) is a significant complication in adults with EA. It may result not only from the right ventricle (RV), but also from the left ventricle (LV) abnormalities. We evaluate the size and function of both ventricles in patients with EA in cardiac magnetic resonance (CMR); to assess their association with the clinical markers of HF. Study group: 37 unoperated adults with EA (mean age 43.0 ± 14.4y, 21[56.8%] males). Controls: 25 volunteers (mean age 39.9 ± 10.9y, 15[60%] males). Study protocol included: CMR [ejection fraction (EF), end-diastolic (EDVind) and stroke volumes (SVind) indexed by body surface area]; cardiopulmonary test (peak VO2, %peak VO2, VE/VCO2 slope). Size and systolic function of LV were reduced comparing to the controls [LVEDVind (ml/m2): 63.7(range 38.7–94.2) vs. 79.3(48.7–105.1), p < 0.001; LV SVind (ml/m2): 35.8(22.9–55.1) vs. 49.2(37.8–71.7), p < 0.0001; LVEF(%): 58.3(34–70.5) vs. 62.0(52.0–77.0), p = 0.009]. RV was enlarged comparing to the controls [RVEDVind (ml/m2): 124.3(52.8–378.9) vs. 83.0(64.0–102.0), p < 0.0001) with impaired systolic function (RV SVind (ml/m2): 22.7(11.1–74.1) vs. 48.0(37.8–71.7), p < 0.0001; RVEF(%): 38.0(21.0–66.1) vs. 59.0(49.0–69.0), p < 0.0001). A significant correlation was found between LVEDVind vs. peakVO2 (r = 0.52, p = 0.001); LV SVind vs. peakVO2 (r = 0.47, p = 0.005). There was no correlation between the right ventricular status and exercise capacity. In adults with Ebstein anomaly the size of left ventricle is reduced, right ventricle is enlarged; the function of both is impaired. Abnormal exercise capacity is associated with left ventricular status. Ventricular interdependence probably plays a role in heart failure pathomechanism. • Ebstein anomaly is a right heart congenital defect, but data on the left heart are scarce. • Left ventricle is reduced, the right is enlarged, the function of both is impaired. • Heart failure is associated with the status of left, but not right, ventricle. • ventricular interdependence may be a key player in heart failure pathomechanism. [ABSTRACT FROM AUTHOR]

• Specimens of the heart with congenital heart defects were scanned using micro CT. • A 3D interactive atlas of congenital heart defects was developed and published. • The resolution of the data was 41 um, faithfully representing delicate structures. • The specimens were successfully replicated using a 3D printer. • The 3D data of the 88 specimens are available for nonprofit research or education. Postmortem heart specimens are essential for education and research on the anatomy, morphology, and pathology of congenital heart defects. However, such specimens are rarely obtained these days, and the specimens stored in formalin are inexorably deteriorating. This study aimed to develop methods to archive three-dimensional data of rare human heart specimens and to publish the data. All wax-infiltrated human postmortem heart specimens stored in the Cardiac Registry, Boston Children's Hospital were scanned using microfocus computed tomography (X-Tek HMXST225, Nikon Metrology, Inc.), and reproduced using a three-dimensional printer (Form 3B, Formlabs Inc.). The digital models were published as an interactive three-dimensional online atlas. The resolution of the three-dimensional data was evaluated. The primary diagnoses in the 88 specimens included in the study include normal cardiac anatomy (11 cases), transposition of the great arteries {S,D,D} (11 cases), ventricular septal defect (10 cases), double-outlet right ventricle (9 cases), hypoplastic left heart syndrome (9 cases), and common atrioventricular canal (7 cases). Twenty-five cases (28%) underwent previous surgical or percutaneous interventions to the heart, including Mustard procedure (1 case), Senning procedure (2 cases, one was performed on a postmortem heart specimen). The median voxel size of the three-dimensional data was 40.5 um (IQR, 32.8–64.2). All intracardiac structures were precisely reproduced as digital and physical three-dimensional models. The methods and resultant models were considered useful for archiving and furthering the utilization of these invaluable specimens. The atlas is available at https://www.sketchfab.com/heartmodels/collections. [Display omitted] [ABSTRACT FROM AUTHOR]

Objectives: To assess the safety and efficacy of the Amplatzer Septal Occluder in the closure of secundum type atrial septal defects.Background: The Amplatzer Septal Occluder (ASO; Abbott, St. Paul, MN) is an FDA-approved device for percutaneous closure of secundum type atrial septal defects (ASD). Previous small cohort trials have shown a favorable safety and technical efficacy profile.Methods: We conducted a systemic review and meta-analysis of all prospective case series and controlled trials that evaluated the ASO's safety and implant efficacy. The primary endpoint was the technical success rate of implantations. Secondary outcomes included proportions of arrhythmias and embolism specific-adverse events.Results: We included a total of 12 studies with 2972 patients. The ratio of device implantation was 2:1 by sex [female: male]. Pooled technical success rate of implantation was 98% (95% CI: 0.968-0.990, P < 0.01). The cumulative adverse event rate was 5.1% (95% CI: 0.035-0.068, P < 0.01), which included arrhythmia and embolism specific adverse event rates of 1.8% (95% CI: 0.007-0.032, P < 0.01) and 0.7% (95% CI: 0.002-0.013, P < 0.01), respectively. Sensitivity analysis did not significantly affect pooled outcomes for success rate and adverse events; both forest plot and Begg's and Egger's regression tests supported symmetricity.Conclusion: A high likelihood of technical success can be expected when implanting the ASO in secundum type ASDs. Adverse event rates are expected for one in twenty patients, and thus, our results support the safe use of ASO in secundum type ASDs closure.Condensed Abstract: The AMPLATZER Septal Occluder is an FDA-approved device for percutaneous closure of secundum type atrial septal defects (ASD). We conducted a systemic review and meta-analysis of all prospective case series and controlled trials that evaluated the ASO's safety and implant efficacy. We included a total of 12 studies with 2972 patients. Pooled technical success rate of implantation was 98% (P < 0.01). The cumulative adverse event rate was 5.1% (P < 0.01), 1.8% (P < 0.01) rate of arrhythmias, and 0.7% (P < 0.01) rate of embolisms. A high likelihood of technical success can be expected with a low rate of adverse events. [ABSTRACT FROM AUTHOR]

To explore siblings' perceptions of having a brother or sister with congenital heart disease in the UK during the COVID-19 pandemic. Siblings of children with congenital heart disease aged 8–17 years old were interviewed via video call technology between September 2020 and February 2021. We conducted reflexive thematic analysis of these interviews to generate themes. Interviews took place with 17 siblings, predominantly of white ethnicity n = 15 (86%). Most siblings interviewed were first born in the family n = 15 (88%), and most children with CHD were the youngest n = 15(88%). Four themes were generated; My sibling is vulnerable, what does this mean for my family, I have a responsibility to protect my brother or sister, our family time during the pandemic and adjustment and adaptations to pandemic life. Siblings identified difficult aspects of the pandemic and these related to concerns about their brother's or sister's vulnerability, family impact, and keeping their sibling safe. They also identified adjustments they made to keep their family functioning throughout the COVID-19 pandemic. Despite the worry and uncertainty siblings experienced, they valued increased family cohesion which helped to mitigate some challenges of the restrictions imposed in the UK. Honest and open communication is valued by siblings. It is vital to ensure siblings receive the support they need to ensure they keep up with their schooling and social commitments as pandemic related restrictions ease. • Siblings of children with congenital heart disease were affected by the COVID-19 pandemic. • Participants recognised their sibling was vulnerable to COVID-19 and explained their responsibility to protect them. • Siblings had increased household chores and caretaking during the pandemic, this kept their family functioning. • Siblings reported positive and negative aspects of shielding during the COVID-19 pandemic. [ABSTRACT FROM AUTHOR]

To assess the feasibility and outcomes of biventricular conversion following takedown of Fontan circulation. Retrospective analysis of patients who had takedown of Fontan circulation and conversion to biventricular circulation at a single center from September 2007 to April 2020. Failing Fontan physiology was defined as Fontan circulation pressure >15 mm Hg and/or the presence of associated complications. Biventricular conversion was performed in 23 patients at a median age of 10.0 (7.5-13.0) years. Indications included failing Fontan physiology in 15 (65%) and elective takedown in 8 (35%) patients. A subset of patients (n = 6) underwent procedures for staged recruitment of the nondominant ventricle before conversion. Median z score of end-diastolic volume of borderline ventricle before takedown was –2.3 (–3.3, –1.3). Hypoplastic left heart syndrome (P <.01) and sub-/aortic stenosis (P <.01) were more common in these patients. Biventricular conversion with or without staged ventricular recruitment led to a significant increase in indexed end-diastolic volume (P <.01), indexed end-systolic volume (P <.01), and ventricular mass (P <.01) of the nondominant ventricle (14 right, 9 left ventricle). There were 5 (22%) deaths (1 [4%] early death). All who underwent elective biventricular conversion survived, whereas 2-year survival rate for patients with a failing Fontan circulation was 72.7% (95% confidence interval, 37%-90%). The overall, 3-year reoperation-free survival was 86.7% (95% confidence interval, 56%-96%). Left dominant atrioventricular canal defect (P <.01) and early era of biventricular conversion (P =.02) were significant predictors for mortality. A primary as well as a staged biventricular conversion is feasible in patients who have had previous Fontan procedure. Although this provides an alternative to transplantation in patients with failing Fontan, outcomes are worse in those with failing Fontan compared with elective takedown of Fontan circulation. Optimal timing needs further evaluation. [Display omitted] [ABSTRACT FROM AUTHOR]

This study aimed to assess the associations between maternal drug use, cytochrome P450 (CYP450) genetic polymorphisms, and their interactions with the risk of congenital heart defects (CHDs) in offspring. A case-control study involving 569 mothers of CHD cases and 652 controls was conducted from November 2017 to January 2020. After adjusting for potential confounding factors, the results show that mothers who used ovulatory drugs (adjusted odds ratio [ aOR ] = 2.12; 95% confidence interval [ CI ]: 1.08–4.16), antidepressants (a OR = 2.56; 95% CI : 1.36–4.82), antiabortifacients (a OR = 1.55; 95% CI : 1.00–2.40), or traditional Chinese drugs (a OR = 1.97; 95% CI : 1.26–3.09) during pregnancy were at a significantly higher risk of CHDs in offspring. Maternal CYP450 genetic polymorphisms at rs1065852 (A/T vs. A/A: OR = 1.53, 95% CI : 1.10–2.14; T/T vs. A/A: OR = 1.57, 95% CI : 1.07–2.31) and rs16947 (G/G vs. C/C: OR = 3.41, 95% CI: 1.82–6.39) were also significantly associated with the risk of CHDs in offspring. Additionally, significant interactions were observed between the CYP450 genetic variants and drug use on the development of CHDs. In those of Chinese descent, ovulatory drugs, antidepressants, antiabortifacients, and traditional Chinese medicines may be associated with the risk of CHDs in offspring. Maternal CYP450 genes may regulate the effects of maternal drug exposure on fetal heart development. [ABSTRACT FROM AUTHOR]

Treacher Collins syndrome (TCS) is a congenital malformation of the craniofacial structures derived from the first and second pharyngeal arches. The craniofacial deformities are well described in the literature. However, little is known about whether there are associated extracraniofacial anomalies. A retrospective study was conducted using data from four craniofacial units. Medical charts were reviewed for the presence and type of extracraniofacial anomalies, as well as age at diagnosis. A possible correlation between the severity of the phenotype and the presence of extracraniofacial anomalies was assessed using the Hayashi classification. A total of 248 patients with TCS were identified; 240 were confirmed to have TCS, of whom 61 (25.4%) were diagnosed with one or more extracraniofacial anomalies. Ninety-five different extracraniofacial anomalies were found; vertebral (n = 32) and cardiac (n = 13) anomalies were most frequently seen, followed by reproductive system (n = 11), central nervous system (n = 7), and limb (n = 7) anomalies. No correlations between tracts were found. Extracraniofacial anomalies were more prevalent in these patients with TCS compared to the general population (25.4% vs 0.001–2%, respectively). Furthermore, a positive trend was seen between the severity of the syndrome and the presence of extracraniofacial anomalies. A full clinical examination should be performed on any new TCS patient to detect any extracraniofacial anomalies on first encounter with the craniofacial team. [ABSTRACT FROM AUTHOR]

Impaired placentation is an important contributing factor to intra-uterine growth restriction and pre-eclampsia in fetuses with congenital heart defects (CHD). These pregnancy complications occur more frequently in pregnancies with fetal CHD. One of the most important factors influencing the life of children with CHD is neurodevelopmental delay, which seems to start already in utero. Delayed neurodevelopment in utero may be correlated or even (partly) explained by impaired placentation in CHD cases. This systematic review provides an overview of published literature on placental development in pregnancies with fetal CHD. A systematic search was performed and the Newcastle-Ottawa scale was used to access data quality. Primary outcomes were placenta size and weight, vascular and villous architecture, immunohistochemistry, angiogenic biomarkers and/or placental gene expression. A total of 1161 articles were reviewed and 21 studies were included. Studies including CHD with a genetic disorder or syndrome and/or multiple pregnancies were excluded. Lower placental weight and elevated rates of abnormal umbilical cord insertions were found in CHD. Cases with CHD more frequently showed microscopic placental abnormalities (i.e. abnormal villous maturation and increased maternal vascular malperfusion lesions), reduced levels of angiogenic biomarkers and increased levels of anti-angiogenic biomarkers in maternal serum and umbilical cord blood. Altered gene expression involved in placental development and fetal growth were found in maternal serum and CHD placentas. In conclusion, abnormal placentation is found in CHD. More extensive studies are needed to elucidate the contribution of impaired placentation to delayed neurodevelopment in CHD cases. [ABSTRACT FROM AUTHOR]

• Modern open-heart surgery induces minimal lung edema in neonates. • Stress-dose corticosteroid regimen reduced radiographically assessed lung edema. • Stress-dose corticosteroid improved postoperative dynamic lung compliance. • No benefit for need of postoperative ventilator support or oxygenation was observed. • No benefit for inflammatory response in tracheal aspirate was observed. The present study was performed to determine whether lung injury manifests as lung edema in neonates after congenital cardiac surgery and whether a stress-dose corticosteroid (SDC) regimen attenuates postoperative lung injury in neonates after congenital cardiac surgery. A supplementary report of a randomized, double-blinded, placebo-controlled clinical trial. A pediatric tertiary university hospital. Forty neonates (age ≤28 days) undergoing congenital cardiac surgery with cardiopulmonary bypass. After anesthesia induction, patients were assigned randomly to receive intravenously either 2 mg/kg methylprednisolone or placebo b, which was followed by hydrocortisone or placebo bolus six hours after weaning from CPB for five days as follows: 0.2 mg/kg/h for 48 hours, 0.1 mg/kg/h for the next 48 hours, and 0.05 mg/kg/h for the following 24 hours. The chest radiography lung edema score was lower in the SDC than in the placebo group on the first postoperative day (POD one) (p = 0.03) and on PODs two and three (p = 0.03). Furthermore, a modest increase in the edema score of 0.9 was noted in the placebo group, whereas the edema score remained at the preoperative level in the SDC group. Postoperative dynamic respiratory system compliance was higher in the SDC group until POD three (p < 0.01). However, postoperative oxygenation; length of mechanical ventilation; and tracheal aspirate biomarkers of inflammation and oxidative stress, namely interleukin-6, interleukin-8, resistin, and 8-isoprostane, showed no differences between the groups. The SDC regimen reduced the development of mild and likely clinically insignificant radiographic lung edema and improved postoperative dynamic respiratory system compliance without adverse events, but it failed to improve postoperative oxygenation and length of mechanical ventilation. [ABSTRACT FROM AUTHOR]

We describe an extremely rare case of a 37-year-old female patient who presented with exertional angina and was diagnosed with a unique coronary anomaly with an anomalous right coronary artery with origin in the left anterior descending artery and a fistula between this anomalous coronary artery and the pulmonary artery. Most patients with coronary anomalies are asymptomatic but some may have angina caused by a coronary steal phenomenon, myocardial infarction, or even sudden death depending on the circuit and characteristics of the anomaly. The combination of multiple coronary anomalies is extremely rare. Despite being a rare diagnosis, coronary anomalies should always be considered as a cause of myocardial ischemia, especially in young patients with low probability for coronary obstructive disease. [ABSTRACT FROM AUTHOR]

A gain-of-function mutation in germline ABL1 causes a syndrome including congenital heart defects. However, the molecular mechanisms of this syndrome remain unknown. In this study, we found a novel ABL1 mutation in a Japanese family with ventricular septal defect, finger contracture, skin abnormalities and failure to thrive, and the molecular mechanisms of these phenotypes were investigated. Whole-exome sequencing on several family members revealed a novel mutation (c.1522A > C, p.I508L) in the tyrosine kinase domain of ABL1 , and complete co-segregation with clinical presentations was confirmed in all members. Wild-type and mutant ABL1 were transfected into human embryonic kidney 293 cells for functional analysis. Western blotting confirmed that tyrosine phosphorylation in STAT5, a substrate of ABL1, was enhanced, and the novel mutation was proved to be a gain-of-function mutation. Since this novel mutation in ABL1 enhances tyrosine kinase activity, phosphorylated proteome analysis was used to elucidate the molecular pathology. The proteome analysis showed that phosphorylation in proteins such as UFD1, AXIN1, ATRX, which may be involved in the phenotypes, was enhanced in the mutant group. The onset of congenital heart defects associated with this syndrome appears to involve a mechanism caused by UFD1 common to 22q.11.2 deletion syndrome. On the other hand, AXIN1 and ATRX may be important in elucidating the mechanisms of other phenotypes, such as finger contracture and failure to thrive. Verification of these hypotheses would lead to further understanding of the pathophysiology and the development of treatment methods. • A gain-of-function mutation in ABL1 causes a syndrome with congenital heart defects. • A novel mutation (p.I508L) in ABL1 causes a gain-of-function. • Phosphorylation of UFD1L, AXIN1, and ATRX may be involved in the phenotypes. [ABSTRACT FROM AUTHOR]

Tricuspid valve dysplasia (TVD) is a congenital malformation of the right atrioventricular valve characterized by restricted leaflet motion, annular dilation, and tricuspid regurgitation (TR). Severe cases typically exhibit progressive right-sided congestive heart failure, affecting the quality of life and survival. This article describes a technique for surgical repair of TVD and a case report with long-term follow-up. A 1.5-year-old intact male Labrador retriever with severe TR underwent surgical repair for TVD. Valve repair was performed under cardiopulmonary bypass and consisted of neochord mobilization of the valve leaflets and partial band annuloplasty. Transthoracic echocardiogram performed 5 days after surgery showed mild TR, a 93% decrease in anatomic regurgitant orifice area, and decreased right chamber dimensions. Forty-eight months after repair, the patient was free of clinical signs, did not have a heart murmur, and was receiving no cardiac medications. Based on this case, surgical repair of TVD is feasible with long-term durability, and the outcome suggests that the described technique may be a viable treatment option for patients with severe TVD. [ABSTRACT FROM AUTHOR]

To compare postoperative arterial blood gas samples and requirement for respiratory support between patients who received sugammadex versus neostigmine reversal before extubation after congenital cardiac surgery. Retrospective, cross-sectional study. Single-center, university-based, tertiary care hospital. Patients with congenital heart disease undergoing surgery with cardiopulmonary bypass. Chart review. The first postoperative arterial blood gas measurements were abstracted from electronic medical records, and reintubation or use of positive- pressure respiratory support within the first 24 postoperative hours was documented. Of the 237 charts reviewed, 111 (47%) patients received sugammadex reversal and 126 (53%) received neostigmine. Multivariate models showed that patients with 2-ventricle congenital heart disease who received sugammadex had lower postoperative arterial carbon dioxide partial pressure (PaCO 2) values (coefficient –3.1, 95% confidence interval [CI] –5.9 to –0.4; p = 0.026) and required less- noninvasive positive- pressure ventilation (odds ratio 0.3, 95% CI 0.1–0.8; p = 0.021). Single-ventricle congenital heart disease patients who received sugammadex had higher postoperative pH values (coefficient 0.04, 95% CI 0.01-0.06; p = 0.01) and lower PaCO 2 values (coefficient –5.2, 95% CI –9.6 to –0.8; p = 0.021). Sugammadex reversal was associated with lower postoperative PaCO 2 values. In addition, sugammadex reversal was associated with less need for noninvasive positive- pressure ventilation in 2-ventricle patients. The magnitude of the effect appears modest, therefore the clinical significance remains unclear. Additional studies focused on investigating particular patient populations, such as infants, single-ventricle congenital heart disease, or patients with pulmonary hypertension, are needed to identify whether these patients appreciate a greater benefit from sugammadex reversal. [ABSTRACT FROM AUTHOR]

• We investigated the effect of two inodilators in infants post cardiac surgery. • The effect of levosimendan and milrinone on myocardial function was compared. • We used conventional and two-dimensional speckle tracking strain echocardiography. • Biventricular strain demonstrated deterioration initially and improvement later. • The two inodilators had comparable effect on cardiac function in this setting. The present study aimed to determine the differential effects of intraoperative administration of milrinone versus levosimendan on myocardial function after pediatric cardiac surgery. Transthoracic echocardiography was used for myocardial function evaluation using biventricular longitudinal strain with 2-dimensional speckle tracking echocardiography in addition to conventional echocardiographic variables. A secondary analysis of a randomized, prospective, double-blinded clinical drug trial. Two pediatric tertiary university hospitals. Infants between 1 and 12 months old diagnosed with ventricular septal defect, complete atrioventricular septal defect, or tetralogy of Fallot who were scheduled for corrective surgery with cardiopulmonary bypass. The patients were randomly assigned to receive an infusion of milrinone or levosimendan at the start of cardiopulmonary bypass and for 26 consecutive hours. Biventricular longitudinal strain and conventional echocardiographic variables were measured preoperatively, on the first postoperative morning, and before hospital discharge. The association between perioperative parameters and postoperative myocardial function also was investigated. Images were analyzed for left ventricular (n = 67) and right ventricular (n = 44) function. The day after surgery, left ventricular longitudinal strain deteriorated in both the milrinone and levosimendan groups (33% and 39%, respectively). The difference was not significant. The corresponding deterioration in right ventricular longitudinal strain was 42% and 50% (nonsignificant difference). For both groups, biventricular longitudinal strain approached preoperative values at hospital discharge. Preoperative N-terminal pro-brain natriuretic peptide could predict the left ventricular strain on postoperative day 1 (p = 0.014). Levosimendan was comparable with milrinone for left and right ventricular inotropic support in pediatric cardiac surgery. [ABSTRACT FROM AUTHOR]

Corticosteroids attenuate an inflammatory reaction in pediatric heart surgery. Inflammation is a source of free oxygen radicals. Children with a cyanotic heart defect are prone to increased radical stress during heart surgery. The authors hypothesized that high-dose methylprednisolone reduces inflammatory reaction and thereby also oxidative stress in infants with a univentricular heart defect undergoing the bidirectional Glenn procedure. A double-blind, placebo-controlled, randomized clinical trial. Operating room and pediatric intensive care unit of a university hospital. The study comprised 29 infants undergoing the bidirectional Glenn procedure with or without aortic arch or pulmonary arterial repair. After anesthesia induction, the patients received intravenously either 30 mg/kg of methylprednisolone (n = 15) or the same volume of saline as placebo (n = 14). Plasma interleukin-6, interleukin-8, interleukin-10 (biomarkers of inflammation), and 8-hydroxydeoxyguanosine concentrations (a biomarker of oxidative stress) were measured at the following 4 time points: preoperatively, during cardiopulmonary bypass, after protamine administration, and 6 hours postoperatively. The study parameters did not differ between the study groups preoperatively. Methylprednisolone reduced the proinflammatory cytokines interleukin-6 and interleukin-8 and increased the anti-inflammatory cytokine interleukin-10 postoperatively. Despite reduced inflammation, there were no differences in 8-hydroxydeoxyguanosine between the methylprednisolone and placebo groups. The proinflammatory reaction and increase in free radical stress were not interrelated during congenital heart surgery in cyanotic infants with a univentricular heart defect undergoing the bidirectional Glenn procedure. High-dose methylprednisolone was ineffective in attenuating free radical stress. [ABSTRACT FROM AUTHOR]

• Antiplatelet therapy after the percutaneous closure of ASD/PFO effective. • ASA after percutaneous ASD/PFO closure was related to low rates of bleeding events. • ASA ensures a low incidence of device thrombosis and/or thrombotic events. The optimal antiplatelet therapy after atrial septal defect (ASD) or patent foramen ovale device closure (PFO) remain to be established. We evaluated the safety and effectiveness of 6-month 100 mg Aspirin (ASA) regimen in the very long-term follow up after intracardiac echocardiography (ICE) –aided interatrial shunt closure in a large retrospective cohort. Single-center retrospective analysis which included medical and instrumental data of 789 consecutive patients referred to our institution over a 15 years period due interatrial shunt catheter-based closure. Finally, 734 patients were included in the analysis (mean age 46.1 ± 14.6 years, 398 females): ASD and PFO closure were successfully performed in 281 and 453 patients, respectively. Over the follow-up period, no major bleeding events were observed while the rate of minor bleeding events was 0.9%. Tolerance problems were detected in 3.4% of cases (n ==28). In 11 patients (1.5%) an abnormal complete blood count was detected and monitored up to normalization. On a mean follow –up of 10.3 ± 3.0 years (range 1–15), 10 patients (1.36%) had a persistent moderate shunt 12 months after closure and maintained Aspirin 100 mg life-long, whereas the remaining 724 suspended the treatment after 6 months. Device thrombosis in ASD and PFO groups were 0% and 0.2%, respectively. A cerebral ischemic event occurred in 0.4% of the entire cohort. A regimen of 100 mg of ASA for six months after ASD/PFO closure appeared to be both safe and effective in preventing device thrombosis and major bleedings. [ABSTRACT FROM AUTHOR]

There is no established transplacental treatment for heart failure (HF) in utero , and no animal models or experimental systems of fetal HF have been established. This study aimed to investigate the effect of maternal tadalafil administration on fetal cardiovascular function and uteroplacental circulation in a murine model of fetal HF. We first used an ultra-high-frequency ultrasound imaging system in utero and demonstrated that Hey2 −/− embryos had worsening right ventricular hypoplasia and marked left ventricular (LV) dilatation as gestation progressed. In both ventricles, fractional shortening (FS) and the E/A ratio were significantly lower in Hey2 −/− embryos than in wild-type embryos, indicating that the embryos can be used as a murine model of fetal HF. Subsequently, we evaluated the effect of tadalafil treatment (0.04 or 0.08 mg/ml; T0.04 or T0.08 groups, respectively) on fetoplacental circulation in Hey2 −/− embryos. LV FS was significantly higher in the T0.04 group than in control (P < 0.01), whereas LV dilation, mitral E/A ratio, and umbilical artery resistance index were not significantly different among all groups. The thinness of the LV compacted layer did not differ between the T0.04 and vehicle-treated Hey2 −/− embryos. A phenotype comprising marked dilatation and reduced FS of the left ventricles was identified in Hey2 −/− embryos, suggesting these embryos as a murine model of fetal HF. In addition, maternal administration of tadalafil improved LV systolic function without altering LV morphological abnormalities in Hey2 −/− embryos. Our findings suggest that tadalafil is a potential agent to treat impaired fetal ventricular systolic function. • Hey2 knockout embryos can be used as a murine model of fetal heart failure. • Tadalafil improved fetal left ventricular systolic function in Hey2 knockout embryos. • Tadalafil is a potential agent to treat impaired fetal ventricular systolic function. • Ultra-high-frequency ultrasound imaging is useful for assessing fetal heart failure. [ABSTRACT FROM AUTHOR]

• Phenotypic association exist between copy number variant syndromes (CNVs) mediated neurocognitive deficits(NCDs) and congenital heart defects(CHDs), in the absence of surgical intervention. • The size of the CNV is associated with the severity of NCDs and number of CHDs, with no gender bias. • CHDs are often co-morbid with cognitive deficits and specific CHDs are associated with severe neurocognitive deficits. • Current standards of care do not encompass comorbidity of these phenotypes; potentially leading to underprovided clinical management and treatment. Copy number variant (CNV) syndromes are often associated with both neurocognitive deficits (NCDs) and congenital heart defects (CHDs). Children and adults with cardiac developmental defects likely to have NCDs leading to increased risk of hospitalisation and reduced level of independence. To date, the association between these two phenotypes have not been explored in relation to CNV syndromes. In order to address this question, we systematically reviewed the prevalence of CHDs in a range of CNV syndromes associated with NCDs. A meta-analysis showed a relationship with the size of CNV and its association with both NCDs and CHDs, and also inheritance pattern. To our knowledge, this is the first review to establish association between NCD and CHDs in CNV patients, specifically in relation to the severity of NCD. Importantly, we also found specific types of CHDs were associated with severe neurocognitive deficits. Finally, we discuss the implications of these results for patients in the clinical setting which warrants further exploration of this association in order to lead an improvement in the quality of patient's life. [ABSTRACT FROM AUTHOR]

Right-sided mechanical circulatory support for failing Fontan physiology has been largely unsuccessful due to inherent hemodynamic differences between these patients and the target populations for most assist devices. This study uses advanced benchtop modeling of Fontan physiology to examine the use of PediMag and CentriMag to improve failing Fontan hemodynamics. Each device was attached to a compliance-matched, patient-specific total cavopulmonary connection in vitro model that used resistances, compliances, and programmable waveforms to establish "failing Fontan" baseline hemodynamics (cardiac output [CO] = 3.5 L/min and central venous pressure ∼17 mm Hg). The ability of the assist devices to improve failing Fontan hemodynamics (reduce inferior vena cava pressure and augment CO) was investigated. Requiring complete Fontan pathway restriction, PediMag reduced inferior vena cava pressure by ∼10 mm Hg and supported CO augmentation up to 5 L/min. This was accompanied by an increase in superior vena cava pressure of ∼6 mm Hg. CentriMag produced similar hemodynamic changes without the need for pathway restriction or an increase in superior vena cava pressure. PediMag and CentriMag right-sided support led to a decrease in inferior vena cava pressure and augmentation of cardiac output. In the case of CentriMag, this is accomplished without an increase in superior vena cava pressure or the need for restrictive banding. This work provides further data to help with the optimal design of a Fontan assist device to ameliorate the growing need. In vitro testing of the PediMag and CentriMag devices for failing Fontan support showed beneficial hemodynamic changes, leading to a conceptual design of an intracorporeal durable centrifugal pump for failing Fontan support. [ABSTRACT FROM AUTHOR]

Pulmonary artery banding (PAB) is a viable but underreported palliative option for hemodynamically significant ventricular septal defect in small animals. A significant challenge associated with PAB is judging the degree of band tightening, which can be further complicated when animals are immature and still growing at the time of PAB. If a pulmonary artery band is overtightened or becomes progressively too tight after surgery, the result can be reversal of shunt flow with potentially devastating consequences. Placement of a band that could be percutaneously dilated using a balloon catheter affords a minimally invasive option for partially or completely relieving the band should it become too tight after PAB. This report describes a surgical technique for placement of a dilatable pulmonary artery band, reviews guidelines for tightening the band, and reports the outcome of three cats undergoing the procedure. All three cats showed evidence of reduced hemodynamic load after PAB for a period of up to three years after PAB. [ABSTRACT FROM AUTHOR]

Introduction: Natriuretic peptides (NPs) play a pivotal role in maintaining fetal circulation; however, little is known about their metabolism. The aim of the present study was to elucidate the metabolism of plasma NPs in the fetoplacental circulation.Methods: Plasma NP concentrations in maternal vein and umbilical artery (UA) and vein (UV) samples from fetuses with congenital heart defect (n = 86) or arrhythmia (n = 31) and controls (n = 127) were analyzed.Results: Levels of plasma atrial NP (ANP) and brain NP (BNP) showed good correlation between UV versus UA samples (p < 0.01). In all three fetus groups, the regression coefficients between UV and UA plasma ANP levels were close to 0.5, while those between UV and UA plasma BNP levels were close to 1. The molecular forms of immunoreactive ANP in UA plasma showed a single peak corresponding to mature ANP, while those of immunoreactive BNP in UA plasma showed two major peaks and several minor peaks corresponding to mature BNP-32 and its partially digested peptides, as well as glycosylated and non-glycosylated BNP precursors (proBNP). No correlation was found between fetuses and mothers in terms of either plasma ANP or BNP levels.Conclusions: The mother and fetus independently secrete and metabolize both ANP and BNP. Fetal plasma ANP consists exclusively of the mature form, and the placenta and umbilical vessels are possible major sites of ANP metabolism. In contrast, fetal plasma BNP consists predominantly of the precursor forms, which may contribute to protecting BNP from metabolism in the fetoplacental circulation. [ABSTRACT FROM AUTHOR]

Aim of this study was to assess the accuracy of ventricular septal defects (VSD) using high pitch computed tomography angiography (CTA) of the chest in children below 1 year of age, compared to the intraoperative findings and echocardiography. Out of 154 patients that underwent Dual-Source CTA of the chest using a high-pitch protocol at low tube voltages (70–80 kV), 55 underwent surgical repair of a VSD (median age 8 days, range 1–348 days). The margins of the VSDs and their relation to the surrounding structures were reproduced by en-face views using multiplanar reformations (MPR). Absolute diameter, normalized area and relative area compared to the aortic valve annulus were used for discrimination between restrictive and non-restrictive defects. Localization was classified into four subtypes. The results were compared to two-dimensional echocardiography and intraoperative findings. Median absolute size of VSDs did not differ significantly between CTA-measurements (10.8 mm, range 2.8–18.1 mm) and intraoperative findings (12.0 mm, 3.0–25.0 mm, p = 0.09). Echocardiographic values were significantly lower (9.6 mm, 3.0–18.5 mm, both p < 0.01). The classification of the location and orientation matched the intraoperative situs in 96.4% of all cases using CT and in 87.3% using echocardiography. Echocardiography missed the relation to valves in 11% of all cases. Pre-interventional sensitivity and specificity for detection of a VSD were 97.2/98.9% compared to echocardiography. Median radiation dose was 0.32 mSv (range 0.12–2.00 mSv) and differed significantly between second and third generation Dual-Source CT (0.43 vs. 0.22 mSv, p = 0.003). Size and subtype of VSDs can be accurately assessed by CTA of the chest in patients with complex congenital heart defects at a very low radiation dose. [ABSTRACT FROM AUTHOR]

Abstract Background Advances in congenital heart disease (CHD) have transferred the mortality from childhood to adulthood. Exercise capacity in young patients with CHD remains lower than in the general population, resulting in deconditioning and impaired quality of life. Evidence based-medicine in cardiac rehabilitation in this age group with CHD remains limited. We present the QUALI-REHAB study rationale, design and methods. Methods The QUALI-REHAB trial is a nationwide, multicentre, randomised, controlled study, aiming to assess the impact of a combined centre and home-based cardiac rehabilitation program on the quality of life of adolescents and young adults (13 to 25 years old) with CHD. Patients with a maximum oxygen uptake (VO2 max) < 80% and/or a ventilatory anaerobic threshold (VAT) < 55% of predicted VO2 max , will be eligible. Patients will be randomised into 2 groups (12-week cardiac rehabilitation program vs. controls). The primary outcome is the change in the PedsQL quality of life score between baseline and 12-month follow-up. A total of 130 patients are required to observe a significant increase of 7 ± 13.5 points in the PedsQL, with a power of 80% and an alpha risk of 5%. The secondary outcomes are: VO2 max , VAT, stroke volume, clinical outcomes, physical and psychological status, safety and acceptability. Conclusion After focusing on the survival in CHD, current research is opening on secondary prevention and patient-related outcomes. The QUALI-REHAB trial intends to assess if a combined centre and home-based rehabilitation program, could improve the quality of life and the exercise capacity in youth with CHD. Trial registration: Clinicaltrials.gov (NCT03690518). Highlights • Exercise capacity in young patients with CHD is lower than in the general population. • It results in deconditioning and impaired quality of life. • Evidence based-medicine in cardiac rehabilitation in CHD patients remains limited. • The QUALI-REHAB randomised trial aims to assess the impact of rehabilitation program on young CHD patients' quality of life. • The QUALI-REHAB randomised trial uses a combined centre and home-based cardiac rehabilitation program. [ABSTRACT FROM AUTHOR]

This paper represents the research results of echocardiographic study for early prediction of mortality. The classification task is solved by analyzing the echocardiographic data from medical information system. Echocardiographic data of 90000 hearts condition extracted directly from medical information system were analyzed. The considered echocardiographic studies were conducted for patients with CHF, CHD, hypertension, heart arrhythmia, valvular heart disease, autoimmune disease, congenital heart defect, cardiomyopathy, endocrine disease, heart failure, mixed connective tissue disease, cancer, aneurism. Using machine learning methods and neural networks it is possible to make an early prediction of mortality based on instrumental echocardiographic study. It can be offered to clinicians as support for accurate, reasonable saving clinical decisions with minimization risks for patient's health. The classification task mortality prediction is solved by machine learning methods with 97% ROC curve. The simple echocardiographic test results like FV Simpson, systolic volume, the valves condition, the condition of the ascending aorta and other echocardiographic data are used as predictors. Such a simple approach to solving critical tasks can make the method widely used in clinical practice. [ABSTRACT FROM AUTHOR]

Abstract Objectives Bioprosthetic pulmonary valve conduits have been reported with an increased risk of endocarditis. Thrombus formation is considered as source of these serious and life-threatening infections. We reviewed a series of explanted valved pulmonary conduits for histological evidence for thrombus formation. Materials and methods Explanted bioprosthetic pulmonary valves were fixed in formalin and embedded in paraffin or in methylmethacrylate. Standard staining as well as immunohistochemical staining techniques were applied. Native pulmonary valves of German domestic pigs served as controls. Results 47 valved pulmonary conduits (Hancock n = 23, Homograft n = 7, Contegra n = 7, Melody n = 7, other n = 3) were analyzed histologically. Average time of implantation had been 63 months (6 to 342 months). Indications for explantation included significant obstruction (n = 45), regurgitation (n = 7), and/or endocarditis (n = 6). In 44/47 (93%) specimen, we found accumulation of thrombotic material at the basis of the semilunar valve sinus to a variable degree. 11 patients had been treated with antiplatelet agents, 2 had received anticoagulants at the time of explantation. There was no suspicion of thrombus formation clinically or echocardiographically prior to explantation in any of the patients. Control porcine pulmonary valves (n = 5) did not show any evidence of accumulation of thrombotic material. Conclusions In a large series of explanted valved pulmonary conduits, formation of subclinical, mostly non-infectious thrombotic material was an almost ubiquitous finding. We speculate that high incidence of endocarditis in bioprosthetic valves may in part be explained by thrombus apposition at the basis of conduit valve sinus. Highlights • Histology reveals subclinical thrombus formation in bioprosthetic valves. • Incidence of subclinical thrombus formation in bioprosthetic pulmonary valves is high. • Aspirin does prevent formation of fibrin condensations at bioprosthetic valves. [ABSTRACT FROM AUTHOR]

Purpose: Although congenital heart defects (CHD) are one of the most common types of birth defects in the United States, subnational prevalence estimates beyond early childhood are limited.Methods: We used capture-recapture methodology and logistic regression to estimate CHD prevalence per 1000 residents as of January 1, 2010, separately for adolescents and adults treated and living within five metropolitan Atlanta, Georgia counties, during 2008-2010.Results: Data sources differed by age. Adolescents (n = 1621, aged 11-20 years) and adults (n = 3176, aged 21-64 years) were captured from at least one source. We estimated CHD prevalence to be 7.85 per 1000 adolescents (estimated n = 3718 [95% CI: 3471-4004]) and 6.08 per 1000 adults (estimated n = 12,969 [95% CI: 13,873-18,915]). When we included persons found in age-inappropriate sources, prevalence estimates increased to 11 per 1000 adolescents and 6.5 per 1000 adults.Conclusions: This method for obtaining subnational prevalence estimates provided reasonable prevalence results and identified needs for service improvement. Only one half of adolescents and one-quarter of adults with CHD were in health care within a 3-year time frame, suggesting need for better access to health insurance, transition care, and an increased number of physicians specializing in CHD care. [ABSTRACT FROM AUTHOR]

Highlights • CMR reveals signs of diffuse myocardial fibrosis in young ASO TGA patients. • Extracellular volume fraction was higher in patients compared to controls. • MR marker of diffuse myocardial fibrosis was increased in all coronary territories. [ABSTRACT FROM AUTHOR]

Abstract A two-month-old Red Angus heifer calf presented to the University of Wisconsin Veterinary Care for evaluation of suspected severe bronchopneumonia. Pertinent physical exam findings included tachycardia, tachypnea, dyspnea with a significant abdominal component, and cyanotic mucous membranes. On thoracic auscultation, wheezes were present bilaterally, as well as a grade 2/6 right apical systolic murmur. Thoracic radiographs revealed cardiomegaly, most severely affecting the right side. Echocardiography showed tricuspid valve dysplasia, resulting in severe tricuspid regurgitation and right-heart dilation, as well as a patent foramen ovale. A postmortem examination confirmed the presence of the aforementioned cardiac abnormalities and revealed only mild pulmonary changes. This case report is the first to describe tricuspid dysplasia in the absence of multiple, complex congenital cardiac abnormalities in a calf, and it highlights the value of echocardiography for an antemortem diagnosis. [ABSTRACT FROM AUTHOR]

Abstract Objective Congenital Heart Disease (CHD) is the leading cause of pediatric mortality, with many cases affecting the right ventricular outflow tract (RVOT) or pulmonary valve (PV). Understanding the mechanics of the disease condition can provide insight into development of durable repair techniques and bioengineered replacement devices. This work presents a mechanical and structural analysis of the pulmonary valve of two pediatric cases. Methods Two PV tissues were excised as part of the operative procedure. One PV was obtained from a 9-month-old with Noonan syndrome (Patient 1) and the other from a 6-month-old with tricuspid atresia (Patient 2). The leaflets were subjected to planar biaxial tensile testing and second harmonic generation (SHG) imaging for mechanical and structural evaluation. Results and discussion Patient 1 exhibited a more anisotropic mechanical response than Patient 2, with sample stiffness on par with that of adult PV tissue. Additionally, both samples showed radial and circumferential alignment of collagen fibers on the ventricularis and fibrosa sides of the leaflets, respectively. Collagen fibers on the fibrosa side were also more crimped than on the ventricularis side. [ABSTRACT FROM AUTHOR]

PA/IVS is a rare, heterogenous congenital heart defect anatomically defined by complete obstruction to the right ventricular outflow tract with varying degrees of hypoplasia of the right ventricle and tricuspid valve. This lesion can have associated coronary artery anomalies and, in some cases, right ventricular-dependent coronary circulation. Due to the wide spectrum of presenting anatomic and clinical features, the treatment options are often dictated by the degree of development of the tricuspid valve and right ventricle. The purpose of this review is to discuss the spectrum of pulmonary atresia with intact ventricular septum morphologies and to evaluate the surgical decision-making process and approaches to surgical repair with respect to the impact of hypoplastic right-sided cardiac features. [ABSTRACT FROM AUTHOR]

Abstract Background Periventricular leukomalacia is a common white-matter injury after neonatal cardiac surgery; however, its potential cellular mechanism remains uncertain. There is limited study regarding periventricular leukomalacia treatment. Methods A neonatal rat brain slice perfusion model was used for reproducing the condition of cardiopulmonary bypass, and oxygen glucose deprivation simulated circulatory arrest. Seven-day-old Sprague–Dawley rats were randomly divided into 7 groups: (1) control group with 36°C; (2) 60 minutes of oxygen glucose deprivation group on 15°C, 25°C, 36°C, respectively; and (3) 60 minutes of oxygen glucose deprivation group on 15°C, 25°C, 36°C, plus minocycline (10 μmol/L), respectively. Immunohistochemistry, Western blot, and inflammatory mediators were compared after the perfusion procedures in the different groups. Results This neonatal rat brain slice perfusion with oxygen glucose deprivation model could replicate the pathophysiologic process and injury after cardiopulmonary bypass and hypothermic circulatory arrest. With the increase of oxygen glucose deprivation perfusion temperature, we found that both microglia activation and preoligodendrocyte loss increased. The application of minocycline can significantly inhibit microglial activation and preoligodendrocyte cells loss in the normothermic (36°C) and moderate hypothermia (25°C) oxygen glucose deprivation groups (P <.05), with accompanying significant decreasing microglial inflammatory productions; however, no significant improvement was found in the deep hypothermia (15°C) group. Conclusions The microglial activation may play a key role in preoligodendrocyte injury in the ex vivo neonatal rat brain slice perfusion and circulatory arrest model. Inhibition of microglial activation with minocycline may be an attractive target for white-matter protection during cardiopulmonary bypass and hypothermic circulatory arrest. [ABSTRACT FROM AUTHOR]

Although patent ductus arteriosus is essential in fetal life, interventions to close or minimize the adverse hemodynamic effects associated with the left-to-right shunt are often needed after birth, especially in extremely premature infants. However, there are clinical conditions where maintaining patency of the ductus is essential for survival. In this article we discuss use of prostaglandin E1 in the management of congenital heart defects, pulmonary hypertension and left ventricular failure in early neonatal period. [ABSTRACT FROM AUTHOR]

Background Heart failure results in significant morbidity and mortality in young children with hypoplastic left heart syndrome (HLHS) after the Norwood procedure. Methods We studied subjects enrolled in the prospective Single Ventricle Reconstruction (SVR) Trial who survived to hospital discharge after a Norwood operation and were followed up to age 6 years. The primary outcome was heart failure, defined as heart transplant listing after Norwood hospitalization, death attributable to heart failure, or symptomatic heart failure (New York Heart Association [NYHA] Class IV). Multivariate modeling was undertaken using Cox regression methodology to determine variables associated with heart failure. Results Of the 461 subjects discharged home following a Norwood procedure, 66 (14.3%) met the criteria for heart failure. Among these, 15 died from heart failure, 39 were listed for transplant (22 had a transplant, 12 died after listing, and 5 were alive and not yet transplanted), and 12 had NYHA Class IV heart failure but were never listed. The median age at heart failure identification was 1.28 (interquartile range 0.30 to 4.69) years. Factors associated with early heart failure included post-Norwood lower fractional area change, need for extracorporeal membrane oxygenation, non-Hispanic ethnicity, Norwood perfusion type, and total support time ( p < 0.05). Conclusions By 6 years of age, heart failure developed in nearly 15% of children after the Norwood procedure. Although transplant listing was common, many patients died from heart failure before receiving a transplant or without being listed. Shunt type did not impact the risk of developing heart failure. [ABSTRACT FROM AUTHOR]

Congenital heart defect (CHD) is one of the most common birth defects and is the leading cause of neonatal death. Currently, there are no biomarkers available for prenatal diagnosis of CHD. Clinical strategies to diagnose CHD mostly depend on fetal echocardiography. Recent advances in “omics” techniques have opened up new possibilities for biomarker discoveries. In this review, we discuss recent advances in prenatal detection of CHD using biomarkers obtained by “omics” approaches, including genomics, proteomics, metabolomics, and others. There is great potential in obtaining various kinds of parameters using “omics” studies to facilitate early and accurate diagnosis of CHD. [ABSTRACT FROM AUTHOR]