Atrioventricular Septal Defect Research Articles

The Timing and Mechanisms of AV Valve Failure in Children and Adults With Unbalanced Atrioventricular Septal Defect

Atrioventricular valve regurgitation (AVVR) is common in children with unbalanced atrioventricular septal defect (uAVSD) undergoing single ventricle palliation and is associated with increased mortality and morbidity. The time to onset and mechanisms of significant AVVR in uAVSD have not been well characterised. We aimed to define the time to onset and mechanisms of ≥ moderate AVVR in uAVSD.

Congenital heart defects in molecularly confirmed KBG syndrome patients.

Congenital heart defects (CHDs) are known to occur in 9%-25% of patients with KBG syndrome. In this study we analyzed the prevalence and anatomic types of CHDs in 46 personal patients with KBG syndrome, carrying pathogenetic variants in ANKRD11 or 16q24.3 deletion, and reviewed CHDs in patients with molecular diagnosis of KBG syndrome from the literature. CHD was diagnosed in 15/40 (38%) patients with ANKRD11 variant, and in one patient with 16q24.3 deletion. Left ventricular outflow tract obstructions have been diagnosed in 9/15 (60%), subaortic or muscular ventricular septal defect in 5/15 (33%), dextrocardia in 1/15 (8%). The single patient with 16q24.3 deletion and CHD had complete atrioventricular septal defect (AVSD) with aortic coarctation. Review of KBG patients from the literature and present series showed that septal defects have been diagnosed in 44% (27/61) of the cases, left ventricular tract obstructions in 31% (19/61), AVSD in 18% (11/61). Septal defects have been diagnosed in 78% of total patients with 16q24.3 deletion. Valvar anomalies are frequently diagnosed, prevalently involving the left side of the heart. A distinctive association with AVSD is identifiable and could represent a marker to suggest the diagnosis in younger patients. In conclusion, after precise molecular diagnosis and systematic cardiological screening the prevalence of CHD in KBG syndrome seems to be higher than previously reported in clinical articles. In addition to septal defects, left-sided anomalies and AVSD should be considered. Clinical management of KBG syndrome should include accurate and detailed echocardiogram at time of diagnosis.

Anaesthetic Management of a Pregnant Patient with Repaired Ventricular Septal Defect: A Case Report

Congenital heart disease (CHD) is the most common congenital anomaly. Pregnancy is not always well tolerated in women with congenital heart disease (CHD) such as atrio-ventricular septal defect (AVSD), predominantly due to heart failure deterioration and increasing pulmonary hypertension (PH). Management of those patients are challenging, especially during third trimester and after delivery care. Decision about time of termination, mode of delivery and anaesthetic management are also debatable.

In-Hospital Outcomes in Fontan Completion Surgery According to Age

Actual timing of the Fontan operation is variable. Our aim was to evaluate the impact of age at the time of Fontan operation on mortality and clinical outcome and characterize patients with worse outcomes. We conducted a retrospective, cross-sectional study on the Fontan operation using nationally representative databases from 2003 to 2016 and categorized the patient into 1 of 5 groups according to their age at the time of surgery: <2, 2, 3, 4, and ≥5years. Survey-weighted logistic regression models were used to compare the outcomes of the different age groups. A total of 6,647 children underwent the Fontan completion procedure during the study period with median age 3 (interquartile range 2 to 4) years. The in-hospital mortality was 2.1%. In logistic regression models, in-hospital mortality, respiratory failure, acute kidney injury, chylothorax, arrhythmias, and sudden cardiac arrest were similar among the 5 age groups. Compared with children >2 years, those <2 years were less likely to be admitted for surgery on an elective basis (73.5% vs 90.4%, p <0.001), more likely to have chromosomal anomalies (2.7% vs 1.7%), and more likely to have repair of atrioventricular valves (8.5% vs 6.0%, p=0.027). Mortality was higher in those with an underlying atrioventricular septal defect (AVSD) adjusted odds ratio 4.3 (2.4 to 7.9, p <0.001). Repair of AV valves was more common in the AVSD group compared with those in non-AVSD (14.3% vs 5.5%, p <0.001). In conclusion, age at Fontan completion does not adversely affect the in-hospital outcomes. Our focus should be on optimizing essential factors that are crucial for successful Fontan completion.

Common Arterial Trunk Associated with Functionally Univentricular Heart: Anatomical Study and Review of the Literature.

Common arterial trunk (CAT) is a rare congenital heart disease that is commonly included into the spectrum of conotruncal heart defects. CAT is rarely associated with functionally univentricular hearts, and only few cases have been described so far. Here, we describe the anatomical characteristics of CAT associated with a univentricular heart diagnosed in children and fetuses referred to our institution, and we completed the anatomical description of this rare condition through an extensive review of the literature. The complete cohort ultimately gathered 32 cases described in the literature completed by seven cases from our unit (seven fetuses and one child). Four types of univentricular hearts associated with CAT were observed: tricuspid atresia or hypoplastic right ventricle in 16 cases, mitral atresia or hypoplastic left ventricle in 12 cases, double-inlet left ventricle in 2 cases, and unbalanced atrioventricular septal defect in 9 cases. Our study questions the diagnosis of CAT as the exclusive consequence of an anomaly of the wedging process, following the convergence between the embryonic atrioventricular canal and the common outflow tract. We confirm that some forms of CAT can be considered to be due to an arrest of cardiac development at the stages preceding the convergence.

Unbalanced atrioventricular septal defect with dominant right ventricle: diagnostic criteria, indications for biventricular correction, and results. A clinical observation series

Relevance: Surgical treatment for congenital heart disease (CHD) with borderline left ventricle (LV) dimensions is one of the most challenging issues in current pediatric cardiac surgery. Despite the prevailing general trend to the expansion of indications for biventricular repair of an unbalanced atrioventricular septal defect (AVSD) with a dominant right ventricle (RV), the diagnostic criteria for imbalance, feasibility of radical repair and its functional results are intensively discussed in the literature.Aim: To present quantitative criteria for the assessment of the LV size which could serve as an indication for biventricular repair of the right dominant unbalanced AVSD.Materials and methods: We present a series of 4 patients with AVSD and dominant RV, who underwent radical repair of the defect. The mean age of the patients was 2.4 years, and 3 of 4 had concomitant CHD: tetralogy of Fallot, double outlet right ventricle with pulmonary artery stenosis, and moderate hypoplasia of the aortic arch with subaortic stenosis. Three of 4 patients had previously undergone palliative interventions: two of them – pulmonary bending, one, the first stage of univentricular repair (atrioseptostomy, cavopulmonary anastomosis). Two children had been diagnosed with Down syndrome. The mean left ventricular end diastolic diameter (LVEDD) at baseline was 17.9 mm, corresponding to Z score of -5.4.Results: All four patients underwent biventricular repair of the defect with a two-patch technique. In one case, it was supplemented with placement of the pulmonary trunk homograft prosthesis, in the other with pulmonary valve commissurotomy and cavopulmonary anastomosis due to moderate tricuspid valve stenosis and in the third case the mitral valve was replaced. In 3 patients pacemaker implantation was performed. The criterion for feasibility of complete septation was the ratio of the longitudinal dimensions of the left and right ventricles (LAR &gt; 0.7), confirmed by computed tomography performed before the surgery. The mean LVEDD after surgery was 28.3 mm, corresponding to Z score of -0.8. At the time of the hospital discharge, the left and right atrioventricular valves insufficiency did not exceed 2 and 1 degrees, respectively.Conclusion: Computed tomography allows for accurate measurement and comparison of the longitudinal dimensions of the heart ventricles with LAR index as a tool for assessment of the LV size before the biventricular repair. An additional prospective study is required to objectify the data obtained and identify predictors of an unfavorable outcome of radical repair in patients with right dominant unbalanced AVSD.

Hypoplastic Left Heart Syndrome: Diagnostic Difficulties in Prenatal Period

The article discusses the possible diagnostic errors in the hypoplastic left heart syndrome (HLHS) with mitral atresia and intact interventricular septum. “Atrioventricular canal defects” can be commonly and mistakenly diagnosed the prenatal period in such cases. The aspects and possibilities of differential diagnosis of these defects in the prenatal period are discussed. Ultrasound sign is presented in the article, we have named it “hockey stick with puck” that characterizes the retrograde blood flow in the aortic arch. This sign is diagnosed by color Doppler mapping of the sagittal section of the ductus arteriosus and can serve as a marker of mitral/aortic atresia.

A surgical approach of an unusual variant of complete atrioventricular defect; A case report

BackgroundComplete atrioventricular canal is a congenital heart defect that is characterized by an atrial septal defect, ventricular septal defect, and a common atrioventricular valve. Standard surgical techniques for repairing complete atrioventricular canal defect mainly includes repairing the defect with a single patch, a double patch, or with the modified single patch technique.Case presentationThis paper presents a novel surgical repair technique of an unusual anatomical presentation for a complete atrioventricular canal defect in a patient with Down syndrome.ConclusionsUnusual anatomical variant for congenital heart defects occurs frequently, which gives surgeons real opportunities to innovate surgical approaches. This patient was an example of an unusual anatomical presentation for complete atrioventricular canal, and the surgical technique used for this patient was novel. Follow up for these patients is mandatory for long term results.

Maternal use of cough medications during early pregnancy and selected birth defects: a US multisite, case–control study

ObjectiveTo examine associations between maternal use of cough medications containing dextromethorphan (DM) without guaifenesin (glyceryl guaiacolate (GG)) (‘DM alone’), GG without DM (‘GG alone’) or DM +GG and major birth...

Long-Term Outcome Up To 40 Years after Single Patch Repair of Complete Atrioventricular Septal Defect in Infancy or Childhood.

Objectives Patients with repaired complete atrioventricular septal defect (CAVSD) represent an increasing portion of grown-ups with congenital heart disease. For repair of CAVSD, the single-patch technique has been employed first. This technique requires division of the bridging leaflets, thus, among other issues, long-term function of the atrioventricular valves is of particular concern. Methods Between 1978 and 2001, 100 consecutive patients with isolated CAVSD underwent single-patch repair in our institution. Hospital mortality was 11%. Primary endpoints were clinical status, atrioventricular valve function, and freedom from reoperation in long term. Follow-up was obtained contacting the patient and/or caregiver, and the referring cardiologist. Results Eighty-three patients were eligible for long-term follow-up (21.0 ± 8.7, mean ± standard deviation [21.5; 2.1–40.0, median; min–max] years after surgical repair). Actual long-term mortality was 3.4%. Quality of life (QoL; self- or caregiver-reported in patients with Down syndrome) was excellent or good in 81%, mild congestive heart failure was present in 16%, moderate in 3.6% as estimated by New York Heart Association classification. Echocardiography revealed normal systolic left ventricular function in all cases. Regurgitation of the right atrioventricular valve was mild in 48%, mild–moderate in 3.6%, and moderate in 1.2%. The left atrioventricular valve was mildly stenotic in 15% and mild to moderately stenotic in 2%; regurgitation was mild in 54%, mild to moderate in 13%, and moderate in 15% of patients. Freedom from left atrioventricular-valve-related reoperation was 95.3, 92.7, and 89.3% after 5, 10, and 30 years, respectively. Permanent pacemaker therapy, as an immediate result of CAVSD repair ( n = 7) or as a result of late-onset sick sinus syndrome ( n = 5), required up to six reoperations in single patients. Freedom from pacemaker-related reoperation was 91.4, 84.4, and 51.5% after 5, 10, and 30 years, respectively. Conclusion Up to 40 years after single-patch repair of CAVSD, clinical status and functional results are promising, particularly, in terms of atrioventricular valve function. Permanent pacemaker therapy results in a life-long need for surgical reinterventions.

C57. Left Main Coronary Artery Compression by A Dilatated Pulmonary Artery in Patients with Pulmonary Arterial Hypertension: What Should We Do?

Abstract Background Pulmonary artery hypertension (PAH) is often associated with angina and extrinsic compression of left main coronary artery (LMCA) by a dilatated pulmonary artery is recognized as the cause. The optimal management for pulmonary arterial hypertension with extrinsic LMCA compression has not been clearly established. Case Summary We report on 31-year-old female with secundum atrial septal defect and 44 -year-old female with incomplete atrioventricular septal defect complain of progressive typical angina that aggravated with exertion. The electrocardiogram showed right heart strain and chest radiograph showed cardiomegaly involving the right cavities with enlargement of the pulmonary arch. Right heart catheterization showed pulmonary arterial hypertension with the mean pulmonary artery pressure of 61 mmHg and 65 mmHg respectively. Cardiac CT demonstrated compression of proximal LMCA by dilatated pulmonary artery. Although with optimal phosphodiesterase-5 (PDE5) inhibitor and prostacyclin analog therapy, patients still complain of angina. Discussion LMCA compression may be associated with additional complications of severe myocardial ischemia, including myocardial infarction, left ventricle dysfunction, arrhythmia, and eventually, sudden death. It may occur in patients with PAH with severely dilated PA and can be identified with either computed tomographic coronary angiography or selective coronary angiography. Furthermore, there are no guidelines recommending optimal management of this disease entity. Optimalization of pharmacologic treatment of PAH may reduce compression-related symptoms. A stent implantation is a promising alternative option for the treatment of LMCA compression and provides an optimal risk-to-benefit ratio, both for symptom relief and for long-term outcomes.

C57. Left Main Coronary Artery Compression by A Dilatated Pulmonary Artery in Patients with Pulmonary Arterial Hypertension: What Should We Do?

Abstract Background Pulmonary artery hypertension (PAH) is often associated with angina and extrinsic compression of left main coronary artery (LMCA) by a dilatated pulmonary artery is recognized as the cause. The optimal management for pulmonary arterial hypertension with extrinsic LMCA compression has not been clearly established. Case Summary We report on 31-year-old female with secundum atrial septal defect and 44 -year-old female with incomplete atrioventricular septal defect complain of progressive typical angina that aggravated with exertion. The electrocardiogram showed right heart strain and chest radiograph showed cardiomegaly involving the right cavities with enlargement of the pulmonary arch. Right heart catheterization showed pulmonary arterial hypertension with the mean pulmonary artery pressure of 61 mmHg and 65 mmHg respectively. Cardiac CT demonstrated compression of proximal LMCA by dilatated pulmonary artery. Although with optimal phosphodiesterase-5 (PDE5) inhibitor and prostacyclin analog therapy, patients still complain of angina. Discussion LMCA compression may be associated with additional complications of severe myocardial ischemia, including myocardial infarction, left ventricle dysfunction, arrhythmia, and eventually, sudden death. It may occur in patients with PAH with severely dilated PA and can be identified with either computed tomographic coronary angiography or selective coronary angiography. Furthermore, there are no guidelines recommending optimal management of this disease entity. Optimalization of pharmacologic treatment of PAH may reduce compression-related symptoms. A stent implantation is a promising alternative option for the treatment of LMCA compression and provides an optimal risk-to-benefit ratio, both for symptom relief and for long-term outcomes.

Abstract 9074: Cardiovascular Phenotypes Profiling for Congenitally Corrected Transposition of the Great Arteries and Its Prognosis Implication

Introduction: Congenitally corrected transposition of the great arteries (ccTGA) is a rare and complex congenital heart disease with the characteristics of double discordance. Enormous accompanied anomalies are the culprit of difficult prognosis evaluation and clinical decisions. Hypothesis: To delineate a novel ccTGA clustering modality under HPO instruction and elucidate the relationship between phenotypes and prognosis in ccTGA patients. Methods: A retrospective review of 270 patients diagnosed with ccTGA in Fuwai hospital from 2009 to 2020 and cross-sectional follow-up was performed. Human Phenotype Ontology (HPO)-instructed clustering method was administered in ccTGA classification. Kaplan-Meier survival, Landmark analysis, and cox regression analysis were used to investigate the difference of outcomes between clusters with the combination of surgeries. Results: The median follow-up time was 4.29 (2.07-7.37) years. A total of three distinct phenotypic clusters were obtained after HPO-instructed classification with 21 in cluster 1, 136 in cluster 2, and 113 in cluster 3. Landmark analysis revealed significantly worse mid-term outcomes in all-cause mortality (p=0.042) and composite endpoints (p=0.008) of cluster 3 in comparison with cluster 1 and cluster 2. Multivariate analysis indicated that pulmonary arterial hypertension (PAH), atrioventricular septal defect (AVSD), and arrhythmia were risk factors for composite endpoints. Moreover, the surgical treatment was significantly different among the three groups (p&lt;0.001) and surgical strategies had different effects on the prognosis of the different phenotypic clusters. Conclusions: HPO-instructed clustering method delineates a novel image of ccTGA classification that might improve prognosis prediction and clinical decision.

Abstract 12610: Larger Papillary Muscles and Thicker Chords Were Associated With Post-Operative Left Atrioventricular Valve Regurgitation in Partial AVSD, a Quantitative Three-Dimensional Echocardiography Study

Background: Up to 15% of patients with partial Atrioventricular Septal Defects ( p AVSD) require re-repair of the Left AtrioVentricular Valve (LAVV), with 30% having significant regurgitation. Previous qualitative studies suggest abnormalities in papillary muscles, shortened chordae and thick chords as features associated with LAVVR. We aim to quantitatively assess the sub valvar apparatus in pAVSD using three-Dimensional Echocardiography (3DE) for its association with LAVVR. Hypothesis: We hypothesize that larger papillary muscle and chordal thickness in patients with p AVSD is associated with post-operative LAVVR. Methods: This is a prospective study between 2006 and 2018, of pAVSD aged 0-18 years. All underwent 3DE under sedation using X7-2 probe on a Philips iE33 ultrasound system. Utilizing offline QLAB multi-planar reconstruction (v.10.8.5) we measured anterolateral (APM) and posteromedial papillary (PPM) muscle length; cross sectional area and circumference; chordal length and thickness in mid systole. We measured post-operative vena contracta and LAVV diameter in Apical 4 chamber using 2D color Doppler. Cohort was divided into patients with significant post-operative LAVVR vs. controls based on LAVV vena contracta/LAVV annulus ratio cut off &gt;0.25 (greatest one-third of cohort), LAVVR (n=22) and Controls (n=44). All variables are indexed to Body Surface Area, expressed as median [interquartile range] or mean (standard deviation). Results: LAVVR group were younger at initial surgery, had larger papillary muscles and thicker chords. Table 1 summarizes the variables for Control and LAVVR. Eighteen patients had LAVV re-repair, 14 (63%) were in LAVVR group and 4 (9%) in Controls. Median follow up was 1.5 years. Median time to re-repair was 1.3 years. Conclusion: p AVSD patients with a greater risk of post-operative LAVVR and subsequently re-repair were younger at first repair and had larger papillary muscles length, circumference and thicker chords.

Abstract 13689: The Center-Volume Effect on Congenital Heart Surgery Revised: Is It Time to Consider Regionalization of Care?

Introduction: Regionalization of congenital heart surgery (CHS) has been debated in the U.S. and other countries. Previous reports demonstrated a variable effect of a center’s volume on in-hospital postoperative mortality. However, the volume-outcome relationship for CHS remains incompletely understood without considering outcomes beyond hospital discharge. Hypothesis: We sought to determine, whether procedure-specific center volume predicts postoperative mortality for infants undergoing three complex benchmark procedures (Norwood procedure, arterial switch operation and complete atrioventricular canal repair) and used ventricular septal defect (VSD) closure as a simple benchmark procedure comparison. Methods: We used data from the Pediatric Cardiac Care Consortium, a multi-center U.S.-based clinical registry of CHS, enriched with post-discharge death data after linkage with the National Death Index. We used generalized estimating equations to assess the association between procedure-specific volume and mortality from the time of CHS and up to three years after CHS. Models were adjusted for patient age and weight at CHS, presence of a chromosomal abnormality, and surgical era and accounted for clustering at the center-level. Results: Analysis included 6,656 infants operated at 42 centers between 1982 and 2003. We found an absolute reduction in post-operative risk of death ranging from 6-12% for every additional surgery performed in the same center for Norwood, ASO, and CAVC repair and at all time points examined (Table). There was no statistically significant association between VSD center volume and mortality at any time point. Conclusions: In-hospital and longer-term survival after infantile CHS is improved with increased center case volume for complex CHS. These findings support the regionalization of complex CHS in the U.S.. Decision-makers should consider feasibility of such regionalization to improve outcomes after complex CHS.

Incidence and management of the left ventricular outflow obstruction in patients with atrioventricular septal defects.

OBJECTIVESLeft ventricular outflow tract obstruction (LVOTO) is a recognized complication after complete repair of atrioventricular septal defect (AVSD). This study reviewed the incidence and management of LVOTO following AVSD repair at a single institution.METHODSFrom 1975 to 2019, 24 patients (3.3%, 24/730) underwent reoperation due to LVOTO following partial AVSD (pAVSD) and complete AVSD (cAVSD) repair. The data were retrospectively reviewed.RESULTSThe incidence of LVOTO following pAVSD and cAVSD repair was 4.4% (12/275) and 2.6% (12/455). Freedom from LVOTO reoperation following pAVSD and cAVSD repair at 25 years was 94.3% [95% confidence interval (CI); 89.7–96.7] and 95% (95% CI; 91.1–97.3). The median time from complete repair of pAVSD and cAVSD to LVOTO reoperation was 4.4 years [interquartile range (IQR): 3.4–6.7] and 2.6 years (IQR: 2.2–4.7). Freedom from second LVOTO reoperation at 5, 10 and 15 years was 83.7% (95% CI; 57.2–98.2), 59.2% (95% CI; 28.7, 80.3) and 39.5% (95% CI; 13.2–65.3). The median time between the first and the second LVOTO reoperation in the groups of pAVSD and cAVSD was 6.1 years (IQR: 3.4–8.9) and 8.6 years (IQR: 5.7–9.8). There was no significant difference regarding the first (P = 0.7406) and subsequent LVOTO (P = 0.7153) following complete repair of pAVSD and cAVSD. Combined access to the left ventricular outflow tract was not protective regarding LVOTO reoccurrence. Survival for both groups after LVOTO reoperation at 15 years was 95.6% (95% CI 99.4–72.9).CONCLUSIONSIncidence of LVOTO after AVSD repair is low but the reoccurrence rate is high. Standard subaortic resection does not always provide definitive LVOTO relief. The survival after LVOTO reoperation is excellent.

Congenital heart defects in the recurrent 2q13 deletion syndrome

The recurrent 2q13 deletion syndrome is a rare genetic disorder associated with developmental delay, cardiac and urogenital malformations, and minor facial anomalies. Congenital heart defects (CHDs) are the most frequent malformations associated with del2q13. Experimental studies in zebrafish suggest that two genes mapping within the 2q13 critical region (FBLN7 and TMEM87B) could confer susceptibility to congenital heart defects in affected individuals. We reviewed the cardiac characteristics in four patients with 2q13 deletion admitted to our hospitals, and in published patients. Two of our patients had congenital heart defects, consisting in partial anomalous pulmonary venous connection, ostium secundum atrial septal defect ostium secundum, and small muscular ventricular septal defect in one of them, and aortic valve insufficiency with partial fusion of two commissures (incomplete bicuspid aortic valve) and mitral valve insufficiency due to trivial mitral valve prolapse in the other. The anatomic types of CHD in del2q13 syndrome are highly variable and distributed widely, including laterality defects, complex atrioventricular septal defect, septal anomalies, and cardiomyopathies. Cardiac evaluation should be part of the clinical workup at diagnosis of 2q13 deletion.

Parental consanguinity and congenital heart defects in Afghan children with Down’s syndrome

Abstract&#x0D; Background: It seems that parents’ consanguinity status effects the pattern of congenital heart defects (CHDs). In this study the CHD pattern in children with Down’s syndrome (DS) was determined in Afghanistan’s children population with a high consanguineous marriage rate and compared with historical cohorts from populations with low prevalence of parental consanguinity.&#x0D; Objective: This analytic cross sectional study aimed to determine the frequency and distribution of CHDs in Afghanistan children with Down’s Syndrome as a group in a community with a high consanguineous marriage rate and comparing these with different global studies and populations with low prevalence of consanguinity.&#x0D; Methods: This analytic cross sectional study was conducted in a pediatric teaching hospital in Kabul city - Afghanistan, named Maiwand Hospital. The subjects were all children with DS proven by clinical and cytogenetic study and referred to the pediatric cardiology service from September 2018–September 2020. Parents’ consanguinity was documented and 2D echocardiography and Doppler studies were performed by two experienced pediatric cardiologists after physical examination, ECG, and chest X-ray for each patient.&#x0D; Results: During the two-year study period, 120 DS patients were identified, 78 (65%) of whom had CHDs. The prevalence of isolated and multiple CHD in the 78 children with DS were 35% (42 patients) and 30% (36 patients), respectively. Ventricular septal defect (20.5%) and atrial septal defect (15.3%) were the most common isolated defects. The combination of VSD and PDA (20.5%) were the most frequent multiple CHDs. The most com­mon associations of CHD were VSD + PDA (20.5%) and VSD + ASD (10.2%). Consanguinity was found in 69.2 % of all parents.&#x0D; Conclusions: A higher frequency of CHD was documented in DS children from this population with a high consanguineous marriage rate. The frequencies of specific lesions were almost similar to those reported locally and internationally; VSD was the most frequently detected in this study. Interesting was the predominance of left-right shunt lesions and the relative rarity of cyanotic and complex CHD in this DS population.&#x0D; key words: congenital heart disease, atrioventricular septal defect, Down’s syndrome, trisomy 21, Afghanistan

2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients: Executive Summary.

2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients: Executive Summary.

Cohort study of intervened functionally univentricular heart in England and Wales (2000–2018)

ObjectiveGiven the paucity of long-term outcome data for complex congenital heart disease (CHD), we aimed to describe the treatment pathways and survival for patients who started interventions for functionally univentricular...