Complex Heart Defects Research Articles

Variability in patient-reported outcomes among different congenital heart defects: a comparative analysis of 7,498 patients from 32 countries

Abstract Background Patient-reported outcomes (PROs) may not always correlate with the complexity of congenital heart defects (CHDs), as significant variation has been observed between CHDs within complexity levels. Accurate comparisons across various types of CHDs necessitate large sample sizes. Purpose The objective of this study was to compare PROs across different CHDs in a large international sample. Methods ‘Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease – International Study II’ (APPROACH-IS II) was an international cross-sectional investigation, in which 8,415 patients from 32 countries in 6 continents were enrolled (20 high; 7 upper-middle; 4 lower-middle; and 1 low income). For the comparison across different CHDs, conditions with at least 50 patients were included in the analysis. Hence, the sample consisted of 7,498 patients (median age=33y; 53.1% women). Patients completed questionnaires to measure perceived health status (RAND-12 Health Survey; EuroQOL-5D Visual Analog Scale); depressive symptoms (Patient Health Questionnaire-8); anxiety (General Anxiety Disorder-7) and quality of life (Linear Analog Scale). Generalized linear mixed models (GLMMs) were performed with center as random effect. Results Cyanotic heart defects were associated with the lowest scores for physical functioning, mental health, self-rated health, and quality of life (see Figure). Other CHDs found to be associated with poor PRO scores encompassed unrepaired secundum atrial septal defect, Ebstein anomaly, single ventricle, and truncus arteriosus. Conversely, the most favorable scores for all PROs, except for anxiety, were observed with isolated pulmonic stenosis. Other CHDs with favorable PRO scores comprised coarctation of the aorta, isolated small ventricular septal defect, congenital aortic valve disease, and repaired ventricular septal defect. Adjusted for sociodemographic and physiological factors, GLMMs revealed significant differences across defects, using the best score for each PRO as the reference (see Figure). Conclusion Substantial variation in PROs was observed among different types of CHDs. Interestingly, some complex heart defects demonstrated relatively good PRO scores, comparable to some simple defects. On the other hand, unrepaired simple defects or those with residual lesions, were associated with worse PROs.

Cardiac anatomic digital twins: findings from a single national centre

Abstract Aims New three-dimensional cardiac visualization technologies are increasingly employed for anatomic digital twins in pre-operative planning. However, the role and influence of extended reality (virtual, augmented, or mixed) within heart team settings remain unclear. We aimed to assess the impact of mixed reality visualization of the intracardiac anatomy on surgical decision-making in patients with complex heart defects. Methods and results Between September 2020 and December 2022, we recruited 50 patients and generated anatomic digital twins and visualized them in mixed reality. These anatomic digital twins were presented to the heart team after initial decisions were made using standard visualization methods. Changes in the surgical strategy were recorded. Additionally, heart team members rated their mixed reality experience through a questionnaire, and post-operative outcomes were registered. Anatomic digital twins changed the initially decided upon surgical strategies for 68% of cases. While artificial intelligence facilitated the rapid creation of digital anatomic twins, manual corrections were always necessary. Conclusion In conclusion, mixed reality anatomic digital twins added information to standard visualization methods and significantly influenced surgical planning, with evidence that these strategies can be implemented safely without additional risk.

High-Dimensional Single-Cell Mass Cytometry Demonstrates Differential Platelet Functional Phenotypes in Infants With Congenital Heart Disease.

Congenital heart disease (CHD) is a group of complex heart defects associated with hematologic abnormalities, including increased risk of thrombotic and bleeding events. Past studies have observed evidence of platelet hyperreactivity, while other studies showed decreased platelet activation in patients with CHD. The goal of this study was to develop a mass spectrometry approach to characterize single platelets in infants with CHD and identify potential etiology for such discrepant results. We enrolled 19 infants with CHD along with 21 non-CHD controls at Yale New Haven Children's Heart Center. A single-cell high-dimensional mass cytometry method was developed to quantitatively interrogate platelet surface markers in whole blood. Additionally, plasma cytokine analysis was performed through a multiplexed panel of 52 vascular and inflammatory markers to assess for platelet releasates. We found that infants with CHD had significant differences in platelet activation and functional markers by mass cytometry compared with non-CHD controls. Based on cell surface markers, we classified the platelets into 8 subpopulations (P0 to P7). Distinct subpopulations of platelets (P1, P4, and P5) exhibiting decreased aggregatory phenotype but altered secretory phenotypes were also identified and found to be more abundant in the blood of infants with CHD. Electron microscopy identified increased proportion of hypogranular platelets in CHD. Moreover, cytokine analysis demonstrated an overall increase in plasma cytokines and biomarkers in CHD, including IL (interleukin)-6, IL-8, IL-27, RANTES, and VWF (von Willebrand factor), which are expressed in platelet granules and can be released upon activation. We developed a robust mass cytometry approach to identify platelet phenotypic heterogeneity. Infants with CHD had alterations in distinct subpopulations of platelets with overall reduced aggregatory phenotype and secretory dysfunction. These findings suggest that platelets in infants with CHD may be exhausted due to persistent stimulation and may explain both bleeding and thrombotic vascular complications associated with CHD.

Percutaneous pulmonary valve implantation in a patient with congenitally corrected transposition of the great arteries: a case report

BackgroundPercutaneous pulmonary valve implantation has become an attractive method of dysfunctional right ventricle outflow tract treatment.Case presentationWe describe a unique case of a 20-year-old Caucasian male patient with a complex cyanotic heart defect, namely pulmonary atresia, with congenitally corrected transposition of the great arteries and ventricular septal defect after Rastelli-like surgery at the age of 5 years with homograft use. At the age of 20 years, the patient needed percutaneous pulmonary valve implantation owing to homograft dysfunction. Despite unusual course of the coronary arteries, balloon testing in the landing zone of the right ventricle outflow tract excluded potential coronary artery compression. Then, after presentation, a Melody valve was implanted successfully in the pulmonary valve position. The 8-year follow-up was uneventful.ConclusionThis is likely the first description of a percutaneous pulmonary valve implantation in such anatomy. Such a procedure is feasible; however, it requires exceptional caution owing to the anomalous coronary arteries course, which can be the reason for their compression.

Congenital Heart Disease In Preterm Infants

Background: Congenital heart defects and preterm birth are two important causes of neonatal and infant mortality. However, the relationship between them has not yet been fully clarified. Objective: To find the association of congenital heart disease with premature delivery. Patients and Methods: The study is an observational cross sectional study, done in Al-Batool teaching hospital for maternity and children from July 2022 to January 2023. A self-prepared questionnaire was used to gather data. Gestational age was calculated mainly on postnatal gestational assessment by Dubowitz/Ballard method by a pediatrician. Echocardiography was done by Echocardiography specialist doctor to identify the presence and types of congenital heart defects. SPSS version 25 was used for statistical analysis. Results: Total number of births (term and preterm) was 1616, congenital heart diseases were detected in 60(3.71%) of them, most of them were isolated heart defect. In preterm babies, the congenital heart diseases were detected in 8% (p value was <0.001), they were of different types as followings: patent ductus arteriosus (n=10,26%), atrial septal defect (n=8,21%), ventricular septal defect (n=7,18%), transposition of the great arteries (n=4,10%), tetralogy of Fallot (n=2,5%), and complex of congenital heart disease (n=7,18%), while in full term babies, the rate of congenital heart diseases was only 1.9%. Conclusion: The incidence of congenital heart disease was higher in preterm than full term delivered babies, mostly in the group of transposition of the great arteries, ventricular septal defect, complex heart defects, followed by atrial septal defect. Keywords: Preterm, delivery, congenital heart disease.

Congenital Heart Disease In Preterm Infants

Background: Congenital heart defects and preterm birth are two important causes of neonatal and infant mortality. However, the relationship between them has not yet been fully clarified.
 Objective: To find the association of congenital heart disease with premature delivery.
 Patients and Methods: The study is an observational cross sectional study, done in Al-Batool teaching hospital for maternity and children from July 2022 to January 2023. A self-prepared questionnaire was used to gather data. Gestational age was calculated mainly on postnatal gestational assessment by Dubowitz/Ballard method by a pediatrician. Echocardiography was done by Echocardiography specialist doctor to identify the presence and types of congenital heart defects. SPSS version 25 was used for statistical analysis.
 Results: Total number of births (term and preterm) was 1616, congenital heart diseases were detected in 60(3.71%) of them, most of them were isolated heart defect. In preterm babies, the congenital heart diseases were detected in 8% (p value was <0.001), they were of different types as followings: patent ductus arteriosus (n=10,26%), atrial septal defect (n=8,21%), ventricular septal defect (n=7,18%), transposition of the great arteries (n=4,10%), tetralogy of Fallot (n=2,5%), and complex of congenital heart disease (n=7,18%), while in full term babies, the rate of congenital heart diseases was only 1.9%.
 Conclusion: The incidence of congenital heart disease was higher in preterm than full term delivered babies, mostly in the group of transposition of the great arteries, ventricular septal defect, complex heart defects, followed by atrial septal defect.

Risk stratification of non-cardiac surgeries in adult congenital heart disease

Abstract Background Adults with congenital heart disease (ACHD) face multiple surgeries throughout their lifetime. Data on the specific risk of undergoing non-cardiac surgery and impact of severity of ACHD on outcome are limited. We aimed to assess frequency and outcome of non-cardiac surgery and to identify ACHD patients specifically at risk. Methods Based on data including all hospital admissions in Germany from 2011 to 2018, we identified all ACHD cases that were admitted for non-cardiac surgery. Non-cardiac surgery was categorized in low-, medium and high-risk based on a clinically implemented risk score validated for non-congenital cardiac patients. The analyzed data contained information on patient demographics, diagnoses, in-hospital complications, and mortality. The primary endpoints of the study were major adverse cardiovascular events (MACE), major infection, major bleeding, thromboembolism and in-hospital death. Outcomes of ACHD were compared to a propensity score matched cohort of non-ACHD. Results Overall, 13,041 ACHD inpatient cases were included in the analysis (simple n=9,333 (72%), moderate n=2,741 (21%), and complex heart defects n=966 (7%)). 23.0% simple ACHD underwent low-risk surgery, whilst 31.1% were intermediate and 45.0% high-risk procedures. In the medium complexity ACHD cohort, 35.8% received low-risk, 42.3% intermediate risk and 21.8% high-risk surgery. In complex ACHD, 42.4% underwent low-risk, 41.2% intermediate risk and 16.4% high-risk surgery. Simple ACHD more frequently presented with typical cardiovascular risk factors such as dyslipidemia, arterial hypertension and diabetes mellitus compared to moderately and severely complex ACHD at time of non-cardiac surgery (p<0.001 for all). Compared to a propensity score matched cohort of non-ACHD, congenital cardiac patients more often met the endpoints with an increasing frequency depending on complexity of disease and pre-defined risk of surgery. After adjustment on multivariable logistic regression analysis, moderate and severe complexity of the congenital cardiac defect was associated with an adverse outcome. Risk for death was increased in moderately complex (OR 1.44, 95% CI 1.15 - 1.81, p=0.001) and severely complex ACHD (OR 2.28, 95% CI 1.63 - 3.20, p<0.001) compared to non-ACHD. Compared to low-risk surgeries, patients were at increased risk for death when undergoing mid- (OR 7.66, 95% CI 5.18 - 11.34, p<0.001) or high-risk surgeries (OR 8.03, 95% CI 5.42 - 11.90, p<0.001). Conclusions ACHD patients are at increased risk of adverse complications when undergoing non-cardiac surgery. Risk for adverse outcomes increased with complexity of disease and was generally higher than in non-ACHD individuals. The presented data show that surgical risk classifications implemented for non-congenital cardiac patients were also associated with a much higher adverse outcome in cases undergoing high- versus low-risk procedures.

Profile of cardiac lesions among laboratory confirmed congenital rubella syndrome (CRS) infants: a nationwide sentinel surveillance, India, 2016-22.

The phenotypical profile of cardiovascular malformations in patients with congenital rubella syndrome (CRS) is varied. We aimed to describe the profile of cardiac defects among CRS patients detected in the sentinel CRS surveillance in India during 2016-22. Sentinel sites enrolled infants with suspected CRS based on presence of cardiac defects, hearing impairment, eye signs, or maternal history of febrile rash illness. Suspected CRS cases underwent detailed systemic examination, including echocardiography and serological investigation for rubella. Cardiac defects were categorized as 'Simple' or 'Complex' as per the National Heart, Lung, and Blood Institute classification. We compared the distribution of cardiac defects among laboratory confirmed CRS cases and seronegative discarded cases. Of the 4578 suspected CRS cases enrolled by 14 sites, 558 (12.2%) were laboratory confirmed. 419 (75.1%) laboratory confirmed cases had structural heart defects (simple defects: n=273, 65.2%, complex defects: n=144, 34.4%), with ventricular septal defect (42.7%), atrial septal defect (39.4%), patent ductus arteriosus (36.5%), and tetralogy of Fallot as the commonest defects (4.5%). Laboratory confirmed CRS cases had higher odds of left to right shunt lesions (OR=1.58, 95% CI: 1.15-2.17). This was mainly on account of a significant association of PDA with CRS (OR=1.77, 95% CI: 1.42-2.21). Mortality was higher among CRS patients with complex heart defects (HR=2.04, 95% CI: 1.26-3.30). Three-fourths of the laboratory confirmed CRS cases had structural heart defects. CRS patients with complex cardiac defects had higher mortality. Detecting CRS infection early and providing timely intervention for cardiovascular defects is critical for the management of CRS patients. Ministry of Health and Family Welfare, Govt of India, through Gavi, the Vaccine Alliance.

Influence of Congenital Heart Defect Types over Cardiopulmonary Bypass, Aortic Cross-Clamping, and Intensive Care Unit Length of Stay and Their Association with Genetic Factors

Aim: Surgical correction of congenital heart defects (CHD) often requires interruption of blood flow through cardiopulmonary bypass (CPB) and aortic cross-clamping (ACC), for which duration(s) are considered to be prognostic factors, along with intensive care unit (ICU) length of stay (ICULOS). The aim of this study was to evaluate these surgical prognostic factors in pediatric patients with different types of CHD regarding their type of lesion and associated genetic factors.
 Study Design: Cross-sectional cohort study with 307 pediatric patients.
 Place and Duration of Study: Pediatric Intensive Care Unit (ICU) of Hospital da Criança Santo Antônio, in Porto Alegre/RS, Brazil, from 2006-2009 (3 years)
 Methodology: After inclusion criteria, we studied 266 pediatric patients admitted for the first time in a reference cardiac pediatric ICU from Southern Brazil following cardiac surgery. Intraoperative prognostic factors such as duration of CPB, ACC and ICULOS, in addition to dysmorphological and cytogenetic examinations were compiled and analyzed. P-values of <0.05 were considered significant.
 Results: CPB time was associated to four outflow tract defects (Tetralogy of Fallot [ToF], transposition of the great arteries [TGA], double outlet right ventricle, and truncus arteriosus [TA]), atrioventricular septal defect, and hypoplastic left heart syndrome (HLHS) (P < 0.001). ACC duration was associated with three outflow tract defects (ToF, TGA, and TA) and HLHS (P < 0.001). Moreover, CPB and ACC times showed an association with cyanotic and complex heart defects, as well as prolonged ICULOS (P < 0.001). There was no relationship between these prognostic factors and syndromic aspects or cytogenetic findings.
 Conclusions: CHD type has an impact over CPB and ACC duration and ICULOS, whereas genetic factors are not associated with those prognostic factors.

The Role of CT Angiography Above Echocardiography in Evaluations of Congenital Heart Disease in TCH (2013-2018)

Back ground: Congenital heart disease (CHD) one of the most commonly occurring congenital anomaly, affecting 4-10/1000 live births, Echocardiography is the mainstay of diagnosis, the recent developments in CT techniques increasing role of CT in the evaluation of congenital heart disease that minimize the radiation exposure particularly for children. Objective: This study is performed to assess and high light the role of multi-detector computed tomography (CT angiography) in the evaluation of cardiovascular abnormalities as (Cono- truncal defects) in children and the Clinical indications above trans-thoracic echocardiography. Method: This study included 70 patients, they were referred or following in pediatric cardiology clinic at TCH from January 2013 till October 2018. All patients presented with history or clinical suspicion of Cono- truncal defect were included in the study, for all patient's full history and clinical examination were obtained. An echocardiography examination was done. CT angiography was performed by Philips machine (16 segment). Results: Pulmonary anomalies represent the majority of detected congenital defects in our study, represent 37 cases (53 %) and the commonest pulmonary anomaly is pulmonary stenosis. The majority of patient shows the same finding in echocardiography & CT angiography which represent 47 cases (67.1%), while in 12 cases (17%) CT angiography add more details in regards to the size of the great vessels and the peripheral structure (peripheral pulmonary arteries), while CT angiography rule-out the echocardiography finding in only 11 cases (15 %). Out of 23 cases of patients with Aortic anomaly, 19 cases (82.6%) have the same finding in echocardiography while in 3 cases (13%) the result of CT rolled out the echo finding, and 29 (78.4%) cases out of 37 cases with pulmonary anomalies have same finding in both echocardiography and CT angiography. Conclusion: We conclude that CT angiography is considered a non-invasive diagnostic tool complementary to the cardiac echocardiography in the diagnosis of complex heart defects especially in pulmonary anomalies. Recommendation: Similar study need to be conducted with large sample to confirm the extent of this finding.

The use of a modified blelock taussig anastomosis in the surgical treatment of complex congenital heart defects with univentricular hemodynamics and depleted pulmonary blood flow

Aim . To study the five-year experience of using a modified subclavian-pulmonary anastomosis according to BlelokTaussig in the surgical treatment of complex congenital heart disease in patients with univentricular hemodynamics and depleted pulmonary blood flow. Material and methods . From 2012 to 2020, 32 children with complex CHD, with duct-dependent blood flow, underwent operations to impose a modified subclavian-pulmonary Blelock-Taussig anastomosis (MBTA). The average age of the children was 13±6.7 months, and the average weight was 8.8± 5.2 kg. Results and discussion . Fatal outcome in the early postoperative period was observed in 3.13% of cases. Bleeding in the early postoperative period was observed in 6.25% of patients. In 28.1% of patients with Fallo’s tetrada thereafter radical correction of the defect was performed. Bidirectional cavopulmonary anastomosis was performed in 40.6% of patients with a single ventricle. Postoperative follow-up results were studied in 96.8% of patients. Conclusion . The use of a modified left subclavian-pulmonary anastomosis according to Blalock-Taussig is the optimal palliative procedure in children with depleted pulmonary blood flow. This surgery is performed to prepare the patient for subsequent correction of a complex heart defect and allows the right pulmonary artery to be preserved intact for future use during radical or hemodynamic correction of the defect.

The phenotypic spectrum of terminal and subterminal 6p deletions based on a social media-derived cohort and literature review

BackgroundTerminal 6p deletions are rare, and information on their clinical consequences is scarce, which impedes optimal management and follow-up by clinicians. The parent-driven Chromosome 6 Project collaborates with families of affected children worldwide to better understand the clinical effects of chromosome 6 aberrations and to support clinical guidance. A microarray report is required for participation, and detailed phenotype information is collected directly from parents through a multilingual web-based questionnaire. Information collected from parents is then combined with case data from literature reports. Here, we present our findings on 13 newly identified patients and 46 literature cases with genotypically well-characterised terminal and subterminal 6p deletions. We provide phenotype descriptions for both the whole group and for subgroups based on deletion size and HI gene content.ResultsThe total group shared a common phenotype characterised by ocular anterior segment dysgenesis, vision problems, brain malformations, congenital defects of the cardiac septa and valves, mild to moderate hearing impairment, eye movement abnormalities, hypotonia, mild developmental delay and dysmorphic features. These characteristics were observed in all subgroups where FOXC1 was included in the deletion, confirming a dominant role for this gene. Additional characteristics were seen in individuals with terminal deletions exceeding 4.02 Mb, namely complex heart defects, corpus callosum abnormalities, kidney abnormalities and orofacial clefting. Some of these additional features may be related to the loss of other genes in the terminal 6p region, such as RREB1 for the cardiac phenotypes and TUBB2A and TUBB2B for the cerebral phenotypes. In the newly identified patients, we observed previously unreported features including gastrointestinal problems, neurological abnormalities, balance problems and sleep disturbances.ConclusionsWe present an overview of the phenotypic characteristics observed in terminal and subterminal 6p deletions. This reveals a common phenotype that can be highly attributable to haploinsufficiency of FOXC1, with a possible additional effect of other genes in the 6p25 region. We also delineate the developmental abilities of affected individuals and report on previously unrecognised features, showing the added benefit of collecting information directly from parents. Based on our overview, we provide recommendations for clinical surveillance to support clinicians, patients and families.

Definitive repair of pulmonary atresia with ventricular septal defect using valved conduit for low body weight infant

Pulmonary atresia with ventricular septal defect is a complex congenital heart defect whose management varies depending on the anatomy of pulmonary circulation and surgeon’s preferences. The surgical outcomes of complete repair in small infants and neonates in Vietnam have been seldomly reported. We reported our medium-term results of complete repair of this complex lesion using valved conduit in infants and neonates at our institution. The study included 25 patients with the body weight of less than 5kg who underwent definitive repair using valved conduit between January 2016 and December 2021. The median age at surgery was 66 days (IQR 45 – 116 days), and the mean body weight was 4 kg (3 – 5). Most (96%) attended all follow-up visits, and the average follow-up time was 37.4 months (8.4 – 69.3). There was 1 in-hospital death and 2 late mortalities. The 30-days and 5 years survival rate were 96% and 83%, respectively. One patient required conduit replacement at 3 years post operative because of conduit failure. The proportion of patients with freedom from conduit replacement at 5 years was 95%. Three cases required balloon dilatation of the left pulmonary artery due to stenosis. Freedom from all reinterventions and reoperations at 5 years was 52%. Definitive repair of this type of complex heart defect in small infants can be performed safely and resulted in good surgical outcomes.

Anticoagulation treatment in adult patients with congenital heart defects and bleeding-related mortality

BackgroundCongenital heart disease (CHD) is the most common type of birth defect. Survival, especially for patients with complex heart defects, has increased dramatically and today 97 % of those born with heart defects reach adulthood. As a late effect of both the congenital malformations and various types of interventions, these patients generally have an increased risk of atrial fibrillation, which is strongly associated with stroke. Additionally, implantation of foreign materials such as mechanical heart valves is also associated with higher risk for embolization. Therefore, adult patient with congenital heart disease (ACHD) are at a substantially increased risk of thromboembolism which necessitates anticoagulant therapy. However, the impact and risk of bleeding and bleeding-related mortality, particularly in young ACHD patients, during long-term anticoagulation treatment is unclear. MethodsIn this retrospective cohort study, 117 deceased ACHD patients with anticoagulant treatment were identified by using the Swedish registry of congenital heart disease between 2005 and 2021. Data from medical records were collected and analyzed. Variables obtained were main diagnosis, indication and type of anticoagulation treatment, complications, and cause of death. ResultsA total of 17 ACHD patients (14.5 %) experienced bleeding events. Ten ACHD patients (8.5 %) experienced major bleeding, including five patients (4.3 %) who died of a hemorrhagic stroke. Another seven ACHD patients had minor bleeding (6 %). ConclusionDespite the occurrence of bleeding-related complications, the proportion of serious, fatal bleeding was low among deceased ACHD patients with anticoagulation treatment.

Cord Blood Cardiovascular Biomarkers in Left-Sided Congenital Heart Disease.

Fetal echocardiography has limited prognostic ability in the evaluation of left-sided congenital heart defects (left heart defects). Cord blood cardiovascular biomarkers could improve the prognostic evaluation of left heart defects. A multicenter prospective cohort (2013−2019) including fetuses with left heart defects (aortic coarctation, aortic stenosis, hypoplastic left heart, and multilevel obstruction (complex left heart defects) subdivided according to their outcome (favorable vs. poor), and control fetuses were evaluated in the third trimester of pregnancy at three referral centers in Spain. Poor outcome was defined as univentricular palliation, heart transplant, or death. Cord blood concentrations of N-terminal precursor of B-type natriuretic peptide, Troponin I, transforming growth factor β, placental growth factor, and soluble fms-like tyrosine kinase-1 were determined. A total of 45 fetuses with left heart defects (29 favorable and 16 poor outcomes) and 35 normal fetuses were included, with a median follow-up of 3.1 years (interquartile range 1.4−3.9). Left heart defects with favorable outcome showed markedly increased cord blood transforming growth factor β (normal heart median 15.5 ng/mL (6.8−21.4) vs. favorable outcome 51.7 ng/mL (13.8−73.9) vs. poor outcome 25.1 ng/mL (6.9−39.0), p = 0.001) and decreased placental growth factor concentrations (normal heart 17.9 pg/mL (13.8−23.9) vs. favorable outcome 12.8 pg/mL (11.7−13.6) vs. poor outcome 11.0 pg/mL (8.8−15.4), p < 0.001). Poor outcome left heart defects had higher N-terminal precursor of B-type natriuretic peptide (normal heart 508.0 pg/mL (287.5−776.3) vs. favorable outcome 617.0 pg/mL (389.8−1087.8) vs. poor outcome 1450.0 pg/mL (919.0−1645.0), p = 0.001) and drastically reduced soluble fms-like tyrosine kinase-1 concentrations (normal heart 1929.7 pg/mL (1364.3−2715.8) vs. favorable outcome (1848.3 pg/mL (646.9−2313.6) vs. poor outcome 259.0 pg/mL (182.0−606.0), p < 0.001). Results showed that fetuses with left heart defects present a distinct cord blood biomarker profile according to their outcome.

Taking a closer look at occupational/career development in young adults with a complex congenital heart defect: A secondary analysis

This study re-analyzed a detailed aspect of the emerging adult developmental period among a small sample of participants living with a complex (chronic) heart defect. The purpose of this analysis was to explore narratives specific to work and career variables among people with chronic heart disease. A qualitative secondary analysis was conducted to extract work and career variables from existing qualitative telephone semi-structured interviews (N=7). Three themes were identified from the analysis: (a) different from my peers’, (b) restructure and reframe’, and (c) ‘trying to find my niche in life. Congenital heart disease makes one reframe to approach life, ‘disrupts’ achieving developmental tasks. That would impact adult role expectations.

Health Supervision for Children and Adolescents With Down Syndrome.

Health Supervision for Children and Adolescents With Down Syndrome.

2q37.3 Deletion with Complex Heart Defects Suggesting Interruption of Early Ventricular Looping

A maternally inherited 828 kb microdeletion of 2q37.3 manifested in a 3-week-old premature boy as left juxtaposition of the atrial appendages associated with tricuspid atresia, double outlet infundibulum, subvalvar pulmonary atresia, large secundum atrial septal defect, and right aortic arch with mirror-image branching, consistent with developmental arrest early in heart looping. To the best of our knowledge, no previous 2q37 deletion syndrome has been reported with such a severe cardiac dysmorphology. Hence, this case adds to the cardiac phenotypes identified in 2q37 deletion syndrome.

Academician Evgeniy Nikolaevich Meshalkin (1916–1997) (on the 105th anniversary of his birth)

The outstanding cardiologist and cardiac surgeon Evgeny Nikolaevich Meshalkin was born on February 25, 1916 in the city of Yekaterinoslav, now Dnepropetrovsk. In 1918, the Meshalkin family moved to Rostov-on-Don, and in 1928 to Moscow, where Evgeny graduated from school in 1930, and then studied at the factory school at the Sickle and Hammer factory. In 1941, Evgeny Nikolaevich graduated from the 2nd Moscow Medical Institute and from August 1941 to May 1945 was a participant of the Great Patriotic War permanently in the field army. After demobilization from the army, E. N. Meshalkin worked from 1946 to 1956 at the department and at the clinic of Faculty Surgery of the 2nd Moscow State Medical Institute, headed by Academician of the USSR Academy of Medical Sciences A. N. Bakulev. In 1950, he defended his PhD thesis «Intubation anesthesia», and in 1953, his first monograph «Intubation anesthesia Technique» was published. Evgeny Nikolaevich is rightfully considered one of the founders of the Russian anesthesiology. In 1953, E. N. Meshalkin defended his doctoral dissertation «Probing and contrast study of the heart and major vessels». In conditions of moderate nonperfusion hypothermia, Yevgeny Nikolaevich successfully operated on complex heart defects, performed the insertion of mechanical prostheses for mitral and aortic insufficiency. From January 1956 to 1960, he was the head of the Department of Thoracic Surgery and Anesthesiology of the Central Institute of Advanced Medical Training (now the Russian Medical Academy of Postgraduate Education). E.N.Meshalkin owns 47 copyright certificates and patents, which are implemented not only in the Research Institute of Circulatory Pathology, but also in other cardiac surgery centers in Russia. Honorary citizen of Novosibirsk Evgeny Nikolaevich Meshalkin passed away on March 8, 1997 and was buried in Novosibirsk at the Southern Cemetery. In memory of the outstanding scientist, the Novosibirsk Research Institute of Circulatory Pathology was named after Academician E. N. Meshalkin.

Frequency, Mortality, and Predictors of Adverse Outcomes for Endocarditis in Patients with Congenital Heart Disease: Results of a Nationwide Analysis including 2512 Endocarditis Cases.

Background: Infective endocarditis (IE) represents a major complication in patients with congenital heart disease (CHD) and is associated with high morbidity and mortality. The aim of this study was to analyse the frequency and outcome of IE in contemporary CHD patients based on all IE hospital admissions in Germany over a 10-year period. Methods: Based on data of all hospital admissions in Germany from 2009 to 2018, we identified all CHD cases with a diagnosis of IE. The data contained information on patient demographics, diagnoses, surgical procedures, and mortality. The primary endpoint of the study was endocarditis-associated mortality as well as major adverse events (defined as death or myocardial infarction, stroke, pulmonary embolism, sepsis, renal dialysis, resuscitation, or intubation). Results: Overall, 309,245 CHD inpatient cases were included in the analysis (underlying heart defects of simple complexity 55%, moderate complexity 23%, and complex heart defects 22%, respectively). Of those, 2512 (0.8% of all inpatient cases) were treated for IE. The mortality rate of IE inpatient cases was 6% with a major adverse events rate of 46%, and 41.5% of cases required surgical intervention. The overall IE associated mortality was lower in adult CHD cases compared to the 153,242 in adult IE cases without CHD (7.1% vs. 16.1%, p < 0.001). After adjustments using multivariable logistic regression analysis, the presence or complexity of CHD was not associated with the outcomes. Meanwhile, age, male sex, and co-morbidities emerged as significant predictors of adverse outcomes. Conclusions: IE accounts for a minority of CHD related hospitalizations but remains a deadly disease, and major adverse events are common in this setting. Due to different demographic and co-morbidity spectrums, adult CHD patients tend to have better survival prospects when compared to non-CHD IE patients. Acquired co-morbidities emerged as the main predictors of adverse outcomes.