Complication Of Congenital Heart Disease Research Articles

Pulmonary arterial hypertension associated with congenital heart disease in children: clinical characterisation, outcomes and changes in demographics over time

Abstract Background/Introduction Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD) in children and is associated with significant morbidity and mortality. The impact of different phenotypes on management and survival remains unclear. Purpose To examine the clinical features, management and outcomes of paediatric PAH-CHD patients based on the 20-year experience of the UK National Paediatric Pulmonary Hypertension (PH) Service. Methods Consecutive PAH-CHD patients entering the service between January 2001 and January 2021 were included and classified into: Eisenmenger syndrome (ES, group A), PAH related to a significant systemic-pulmonary shunt (group B), PAH with small or co-incidental CHD (group C) and PAH following defect repair (group D). Incident patients (without pre-existing PAH) were included in survival analysis. Results Of the overall PH paediatric cohort of 1104 patients, 819 (74.2%) had co-existing CHD and 354 (32.1%) patients received a diagnosis of PAH-CHD: 57.1% female, median [IQR] 4.6 [1.7–10.9] years. Group D PAH-CHD was the commonest subgroup, accounting for 36%, while the least frequent subtype was group C (14%). Group A and group B PAH-CHD represented 26% and 24%, respectively. Down syndrome was present in over one third (122, 34.5%) of PAH-CHD patients and was more commonly associated with ES (p=0.02). PAH therapy was started in 79.9% of PAH-CHD patients. At the end of follow-up, patients with group C PAH-CHD were more likely to be on combination therapy than any other group (64.6% vs. 28.4%, p<0.0001). Prostanoid therapy was used in a minority (11%) of patients. The subgroup distribution of PAH-CHD at diagnosis changed from the early (2000–2005) to late (2015–2020) period (Figure 1). The proportion of ES patients decreased from 43.4% to 14.6% of PAH-CHD (p<0.0001). The proportion of group B PAH-CHD patients increased (9.4% vs. 33.3%, p<0.0001), with the majority (59.3%) deemed “operable” on specialist assessment. There was a trend for an increase in repaired PAH-CHD patients between the early and late era (31.1% vs. 43.8%, p=0.09). Transplant-free survival in PAH-CHD was 90.9% (95% CI: 87.8–94%) at 1 year, 77.9% (95% CI: 73.1–83.1%) at 5 years, and 74.9% (95% CI: 69.6–80.7%) at 10 years (Figure 2). Group C PAH-CHD had a lower transplant-free survival than the other 3 groups (HR 2.54, 95% CI: 1.51–4.28, p=0.0005). There was no difference in outcome between group A and group D PAH-CHD (HR 1.19, 95% CI: 0.62–2.28, p=0.6). Conclusions Repaired PAH-CHD, not ES, was the most common subtype in this large paediatric cohort. Over time, there were fewer ES patients and more “operable” patients with left-right shunts, suggesting an improvement in early diagnosis and management. Despite widespread use of PAH therapy, PAH-CHD remains a life-limiting disease with the poorest outcomes in PAH with co-incidental CHD. Funding Acknowledgement Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): Dr Constantine received a personal PhD fellowship grant (CHAMPION PhD Fellowship) Figure 1Figure 2

Correlation between Pulmonary Artery Hypertension Caused by Acyanotic CHD with Left to Right Shunt and Increased Level of Endothelin-1

Background: Pulmonary hypertension is defined as a resting mean pulmonary artery pressure (mPAP) of 25 mmHg or above. PAH is a frequent complication of congenital heart disease (CHD), particularly in patients with left-to-right (systemic-to-pulmonary) shunts. Objective: The present study aimed to investigate changes in the blood level of endothelin-1 (ET-1) in patients of PAH complicated by acyanotic CHD and to detect possible correlation with different hemodynamic parameters. Patient and methods: 48 children were recruited in Pediatric Cardiology Unit, Pediatrics Department in Zagazig University Hospitals after obtaining the required permissions and informed consent from the their caregivers. They were divided into Group (A), which included 24 children with acyanotic CHD with left to right shunt with PAH and Group B included 24 children with acyanotic CHD with left to right shunt but no PAH. All children were subjected to clinical and radiological examination, echocardiography and measurement of endothelin-1 level as well as correlation between the studied parameters were performed. Results: the present results showed that Group A was significantly associated with previous need of hospital admission and insignificantly associated with ICU need. Endothelin-1 was significantly negatively correlated with weight and oxygen saturation (SPO2) but significantly positively correlated with systolic blood pressure (SBP), heart rate (HR), respiratory rate (RR) and pulmonary blood pressure. Significant area under the curve (AUC) with cutoff value >1.55 with sensitivity 85.05 and specificity 91.1%. Conclusion: Pulmonary hypertension (PAH) complicated by CHD can be predicted by estimation of endothelin-1 (ET-1) as an indicator of endothelial injury.

Respiratory morbidity in children with congenital heart disease

ObjectiveTo evaluate the respiratory outcome in children with congenital heart disease (CHD), considering recent management procedures and the CHD pathophysiology. Design and SettingClinical and functional respiratory outcome were evaluated in 8-year-old children with isolated CHD followed up from birth in the prospective population-based EPICARD cohort. PatientsChildren were assigned to two groups, based on the pathophysiology of the CHD: CHDs with left-to-right shunt (n = 212) and CHDs with right outflow tract obstruction (n = 113). ResultsCurrent wheezing episodes were observed in 15% of the children with isolated CHD and left-to-right shunt, and 11% of the children with isolated CHD and right outflow tract obstruction (not significant). Total lung capacity (TLC) was the only respiratory function parameter that significantly differed between the two groups. It was lower in children with left-to-right shunt (88.72 ± 0.65% predicted) than in those with right outflow tract obstruction (91.84 ± 0.96, p = 0.006). In multivariate analysis, CHD with left-to-right shunt (coeff. [95% CI]: -3.17 [-5.45; -0.89]) and surgery before the age of 2 months (-6.52 [-10.90; -2.15]) were identified as independent factors associated with significantly lower TLC values. ConclusionLower TLC remains a long-term complication in CHD, particularly in cases with left-to-right shunt and in patients requiring early repair. These findings suggest that an increase in pulmonary blood flow may directly impair lung development.

Clinical Profile and Outcome of Children with Congenital Heart Disease in First Year of Life

Diagnosis of congenital heart disease (CHD) which is a structural abnormality of the heart or intra thoracic great vessels in the earliest possible time is very important. Identifying the various modes of presentation, early referral and appropriate intervention can save lives and reduce risk of complications. The objectives of this study were to determine the clinical profile, complication and immediate outcome of children with congenital heart disease in first year of life. This cross-sectional observational study was conducted from October 2011 to March 2012 among 50 children from zero to one year of age who was diagnosed as CHD by echocardiography admitted in Department of Paediatrics of ShaheedSuhrawardy Medical College, Dhaka and Department of Paediatric Cardiology of National Institute of Cardiovascular Diseases, Dhaka, Bangladesh. Among 50 infants acyanotic CHD was detected in 70% and cyanotic in 30% infants. Major structural defects were venticularseptal defect 34%, patent ductusarteriosus 30%, tetralogy of fallot’s 14%, transposition of great arteries 12%, atrial septal defect 6%. Presenting features were cough 82%, dyspnea 80%, poor weight gain 70%, feeding difficulty 68% and fever 58%. Frequently observed complications were failure to thrive, pulmonary hypertension and heart failure. Among the outcome of CHD 8% cases closed spontaneously, case fatality rate was 8% and the rest were advised accordingly for surgery, intervention and medical management among which 14% had device closure within the study period. High index of suspicion, early diagnosis, close monitoring and timely intervention can reduce complication of CHD.
 Bangladesh Med J. 2020 Sept; 49(3) : 17-21

Neurodevelopmental outcomes in congenital heart disease through the lens of single ventricle patients.

This review aims to summarize and organize the current body of literature on this contemporary topic, alongside a more general discussion of neurodevelopmental complications of congenital heart disease. It is theorized that the causes of the neurodevelopment disabilities are multifactorial resulting from structural central nervous system abnormalities, haemodynamic alterations and/or biochemical changes. It is therefore imperative that all patients with single ventricle anatomy and physiology receive long-term neurologic and developmental assessments in addition to their cardiac monitoring. Advancements in surgical techniques and medical management have improved survivorship of these medically complex patients. Neurodevelopmental sequelae are one of the most common comorbidities affecting this patient population leading to long-term challenges in motor, language, social and cognitive skills.

Obstetric Patients Characteristics Labor in Dr. M. Djamil General Hospital Padang December 2019-February 2020 Period

Introduction : Maternal death in Indonesia was still high therefore efforts was needed to suppress this maternal mortality rate.Objective : To determine risk factors of maternal mortality and morbidity.Methods : This was a descriptive study using cross sectional design with study sample including all laboring mother in Obstetric Department of Dr. M. Djamil General Hospital Padang from December 2019 to February 2020 period. Sample were collected using total sampling technique with total of 119 samples included in this study. Data was collected from patients’ medical record, including age, gestational age, gravidity, history of labor, diagnosis, method of delivery, maternal condition, and neonates condition at birth.Results : Mean of age was 30.6 ± 5.4 years, mean of maternal age was 36.1 ± 3.8 weeks, about 77 patients (64.7%) were multipara, 49 patients (41.2%) had history of spontaneous labor, most frequent diagnosis were severe preeclampsia in 20 patients (16.8%), impending eclampsia in 9 patients (7.6%), history of cesarean section 8 patients (6.7%), twin pregnancy 7 patients (5.9%), and pregnancy with positive HBsAg in 7 patients (5,9%). Total 32 patients (26.9%) were referred patients from various general hospital in West Sumatra. A total of 105 patients (88.2%) had cesarean section. Mean birth weight of neonates was 2363.4 ± 964.7 gram, mean birth length was 44.1 ± 5.9 cm, mean apgar score in the first 1 minutes was 5,9 ± 2,2, and mean apgar score in the first 5 minutes 7.4 ± 2.3. During 3 months of study, total maternal deaths were found in 3 patients due to complicated eclampsia, severe preeclampsia, and maternal congenital heart disease, while neonatal death was found in 8 babies, where 6 (75%) had IUFD, and 2 (25%) had very low birth weight dying in the first 24 hours of life due to respiratory failure.Conclusion : Most of labored mother were multiparas with history of spontaneous birth, and mostly diagnosed as severe preeclampsia with method of delivery of cesarean section, and maternal and neonatal death were still occurred.Keywords: Death, Maternal, Neonatal

Is Down Syndrome Related to Pulmonary Arterial Hypertension? A Comparative Study

Down syndrome (DS) is a genetic impairment associated with comorbidities such as Congenital Heart Disease (CHD). Pulmonary Arterial Hypertension (PAH) is a complication of CHD in most patients. Due to insufficient documents about the prevalence of PAH in DS with CHDs compared to non-DS (NDS)+CHD patients, this study aimed to compare the prevalence of PAH between DS-CHD and NDS-CHD patients. This is a cross-sectional study conducted on DS-CHD patients referred to the Pediatric and Congenital Cardiology Division at Imam Reza training hospital in Mashhad, Iran, between April 2015 and February 2016. The comparison group included NDS-CHD children matched in terms of age and gender. A comprehensive Echocardiography was run for all patients to determine the types of CHD and pulmonary arterial pressure. Seventy-seven patients were enrolled in the study (47 in the DS-CHD group and 30 in the NDS-CHD group). 48.9% of the DS-CHD patients and 23.3% of the NDS-CHD group developed PAH, which revealed a significantly higher rate of PH among DS-CHD patients (P=0.025). Our findings denote a higher prevalence of PAH among DS-CHD patients compared to NDS-CHD patients. Such an observation is a meaningful warning for DS patients to take early necessary medical or corrective therapies for CHD in order to prevent complications and irreversible pulmonary vascular disease.

Survival of Patients with High-Risk Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

Background: Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD) with uncorrected left-to-right shunts. Currently, no consensus guideline exists on the management of PAH-CHD in children, especially those who do not meet operability criteria. Objective: To compare survival between three groups of high-risk PAH-CHD, group 1: total correction including both surgical and percutaneous intervention, group 2: palliative treatment, and group 3: conservative with medical treatment group. Materials and Methods: All pediatric patients with PAH-CHD that underwent cardiac catheterization between January 1, 2008 and December 31, 2017 were retrospectively reviewed. Inclusion criteria were high risk PAH-CHD patients who had pulmonary vascular resistance (PVR) greater than 6 Wood unit·m² and PVR-to-SVR ratio greater than 0.3 evaluated in room air. Exclusion criteria were younger than three months of age, severe left side heart disease with pulmonary capillary wedge pressure greater than 15 mmHg, obstructive total pulmonary venous return, and single ventricle physiology. The Kaplan-Meier analysis was performed from the date of PAH diagnosis to the date of all-cause mortality or to censored date at last follow-up. Results: Seventy-six patients with a median age at diagnosis of 27.5 months (IQR 14.5 to 69.0 months) were included in this study. The patients were divided into three subgroups and included 38 patients (50.0%) in group 1, six patients (7.9%) in group 2, and 32 patients (42.1%) in group 3. The median follow-up time was 554 days (IQR 103 to 2,133 days). The overall mortality was 21.7%. One-year survival in patients with simple lesion in group 1 and 3 were 79.5% and 87.5% and patients with complex lesions in group 1, 2, and 3 were 93.8%, 83.3%, and 73.1%, respectively. The results showed that most mortalities occurred in the first year. There were no statistically significant differences in survival among difference types of treatment (log rank test, p=0.522). Conclusion: The mortality of high-risk PAH-CHD patients were not different among those who underwent corrective surgery, palliative, or conservative treatment. The mortality was high in the first year after PAH diagnosis and remain stable afterward. Management decision for an individual with high-risk PAH-CHD patients requires comprehensive clinical assessment to balance the risks and benefits before making individualized clinical judgment. Keywords: Pulmonary hypertension; Congenital heart disease; High-risk patients

Reversibility of heart block after congenital heart surgeries in pediatrics, is it predictable?

Abstract Funding Acknowledgements Type of funding sources: None. Background AV block is a serious complication of congenital heart diseases(CHD) repair surgeries.it is associated with an increased mortality and morbidity. Purpose : Identifying the incidence of Complete Heart Block (CHB) after heart surgeries for CHD, analyzing the patients` specifications and procedural data to evaluate the potential correlations that contribute the HB postoperatively. Patients and methods : We enrolled all patients who underwent CHD surgeries from September 2014 till February 2020 and identified the patients who developed postoperative CHB, those patients were divided into two groups, (a group who developed transient CHB and those who developed permanent CHB which required implantation of permanent pacemaker (PPM). We defined the transient HB as the recovery from an advanced second or third degree HB after the surgery within 10days . And the presence of advanced second or third degree HB persisting for more than 10 days after cardiac surgery is considered a permanent HB and indicated for PPM implantation according to current guidelines . Results : Our cohort includes 2377 CHD surgeries, 46 % female patients, median weight was 12.06 kg at the time of the operation, median height was 85 cm. Mean age at the time of the operation was 2.7 years in the cohort with transient HB while it was 2.6 years in the cohort who received PPM. Incidence of HB following those surgeries was 3 %(72) patients, 1.8 %of the patients (43) had transient HB and 1.2% of the patients (21 ) received a PPM. Recovery from HB was within 7days in 35 patients (82%),Two out of the 43 patients who had transient HB ended up with late onset CHB requiring pacing representing 4.6 % of that cohort, one after one month of recovery and the other one after 11 years from the surgery despite they restored their sinus rhythm within 6 days and 10 days respectively. We found the highest incidence of postoperative HB associated with VSDs surgeries either isolated or with other complex CHD diseases followed by TOF with no significant difference between transient and permanent groups according to the type of surgery. The duration of preoperative PR interval suggested the incidence of permanent CHB postoperatively with a P value of 0.005 We found that gender, age, weight and height didn`t affect the outcome, preoperative heart rate and preoperative medications didn`t alter the prognosis, the duration of CBP time and the clamping time were of no statistical significance between the 2 groups with P value of ( 0.38 and 0.33 ). Conclusion : Incidence of CHB post CHD surgeries in our center is 3 %, only 1.2 % required PPM. Various clinical data , ECG criteria , procedural time and duration of the HB except for the preoperative PR interval failed to prove a significant correlation with the incidence of CHB necessitating further investigation to identify the specific risk factors for such complication. Abstract Figure. Incidence of CHB with various surgeries

Dexmedetomidine Protects Human Cardiomyocytes Against Ischemia-Reperfusion Injury Through α2-Adrenergic Receptor/AMPK-Dependent Autophagy.

Background: Ischemia-reperfusion injury (I/R) strongly affects the prognosis of children with complicated congenital heart diseases (CHDs) who undergo long-term cardiac surgical processes. Recently, the α2-adrenergic receptor agonist Dexmedetomidine (Dex) has been reported to protect cardiomyocytes (CMs) from I/R in cellular models and adult rodent models. However, whether and how Dex may protect human CMs in young children remains largely unknown. Methods and Results: Human ventricular tissue from tetralogy of Fallot (TOF) patients and CMs derived from human-induced pluripotent stem cells (iPSC-CMs) were used to assess whether and how Dex protects human CMs from I/R. The results showed that when pretreated with Dex, the apoptosis marker-TUNEL and cleaved caspase 3 in the ventricular tissue were significantly reduced. In addition, the autophagy marker LC3II was significantly increased compared with that of the control group. When exposed to the hypoxia/reoxygenation process, iPSC-CMs pretreated with Dex also showed reduced TUNEL and cleaved caspase 3 and increased LC3II. When the autophagy inhibitor (3-methyladenine, 3-MA) was applied to the iPSC-CMs, the protective effect of Dex on the CMs was largely blocked. In addition, when the fusion of autophagosomes with lysosomes was blocked by Bafilomycin A1, the degradation of p62 induced by Dex during the autophagy process was suspended. Moreover, when pretreated with Dex, both the human ventricle and the iPSC-CMs expressed more AMP-activated protein kinase (AMPK) and phospho AMPK (pAMPK) during the I/R process. After AMPK knockout or the use of an α2-adrenergic receptor antagonist-yohimbine, the protection of Dex and its enhancement of autophagy were inhibited. Conclusion: Dex protects young human CMs from I/R injury, and α2-adrenergic receptor/AMPK-dependent autophagy plays an important role during this process. Dex may have a therapeutic effect for children with CHD who undergo long-term cardiac surgical processes.

Is surgery necessary for adults with isolated interrupted aortic arch?: Case series with literature review.

Interrupted aortic arch (IAA) is defined as a complete interruption of aortic lumen between the ascending and descending aorta. It is an uncommon and complicated congenital heart disease. It is rare for patients with isolated IAA to survive to adulthood without operation. Here we present three rare cases with isolated IAA together with a review of the literature of IAA. Besides, we reviewed reported adult cases with isolated IAA in the last 20 years and summarized the relevant data of the isolated type. We retrospectively searched the hospital databases for adult patients with isolated IAA diagnosed at the Wuhan Union Hospital over the past 10 years. Cases related to adult isolated IAA published in last 20 years were identified by searching Pubmed. Three adult patients with isolated IAA were identified. Two were referred to us for hypertension management and were diagnosed with IAA. They declined surgical treatments and took antihypertensive medications. One patient was referred to our hospital for further treatment options after diagnosed with IAA at another hospital. He received an extra-anatomic bypass surgery. But his hypertension did not well resolve after surgery, and was subsequently managed by anti-hypertensives medications. 25 published adult patients with isolated IAA were identified in Pubmed and relevant details were summarized. Adult patients with isolated IAA usually have extensive collateral vessels joining the descending aorta. Anti-hypertensives medical management with long-term follow-up appears to be a reasonable treatment option for these patients, although surgical intervention is a good choice.

CYLD mediates human pulmonary artery smooth muscle cell dysfunction in congenital heart disease-associated pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD). Deubiquitinase cylindromatosis (CYLD) has been reported to significantly aggravate vascular smooth muscle cell (VSMC) phenotypic transformation, proliferation, and migration. Here, we aimed to further investigate its roles and underlying mechanisms in the CHD-PAH development. The expression of CYLD in the lung tissues from CHD-PAH patients and monocrotaline (MCT) plus aortocaval (AV)-induced PAH rats, pulmonary artery smooth muscle cells (PASMCs) from MCT-AV-induced PAH rats, and human PASMCs (HPASMCs) was evaluated. After infection with CYLD siRNA or pcNDA3.1-CYLD, the proliferation, migration, and apoptosis of HPASMCs were measured using an EdU assay, transwell and scratch wound healing assays, and flow cytometric assay, respectively. An adeno-associated virus (AAV) vector encoding CYLD was used to suppress CYLD expression by being intratracheally instilled in rats 7 days before MCT-AV treatment. The results showed that CYLD was increased in the lung tissues from CHD-PAH patients and MCT-AV-induced PAH rats, and in PASMCs from MCT-AV-induced PAH rats. The contractile-type HPASMCs expressed low levels of CYLD, while the proliferative synthetic-type HPASMCs expressed high levels of CYLD. In addition, CYLD could mediate HPASMC dysfunction, which regulated HPASMC phenotypic transformation and proliferation via the modulation of p38 and ERK activation, while CYLD regulated HPASMC migration via the modulation of p38 activation. In vivo results demonstrated that the local suppression of CYLD expression could attenuate the increased levels of PAH and its associated pulmonary vascular remodeling in MCT-AV-induced PAH rats. Collectively, these results indicated that CYLD might be a potential novel therapeutic target for the prevention of PAH and pulmonary vascular remodeling in CHD-PAH through the modulation of HPASMC dysfunction.

Evaluation of neonatal acute kidney injury after critical congenital heart disease surgery.

Acute kidney injury (AKI) is a common complication of congenital heart diseases (CHDs) after cardiac surgery. This study aimed to define the frequency and critical course, risk factors and short-term outcomes of AKI in postoperative CHD neonates. Postoperatively followed term CHD newborn infants were enrolled in the study. Infants with congenital anomalies of the urinary tract and other major congenital anomalies were excluded. Neonatal modified KDIGO criteria were used to assess AKI. A total of 199 postoperatively followed newborn infants were included in the study. Acute kidney injury was detected in 71 (35.6%) patients. Of these patients, 24 (33.8%) were in stage 1, 14 (19.7%) in stage 2, and 33 (46.5%) in stage 3. Acute kidney injury occurred within the first week (median 1 day [IQR 1-2 days]) of cardiac surgery in 93% of the patients. The duration of invasive respiratory support and extracorporeal membrane oxygenation (ECMO) and mortality were significantly higher in stage 3 patients. Higher vasoactive-inotropic score (OR, 1.02; 95% CI, 1.0-1.04; p = 0.008) and receiving ECMO (OR, 7.9; 95% CI, 2.6-24.4; p = 0.001) were associated with risk for the development of AKI. The mortality rate was 52.1% in the AKI (+) patients, and having AKI (OR 7.1; 95% CI, 3.5-14.18) was significantly associated with mortality. Acute kidney injury, a common early complication after critical neonatal CHD cardiac surgery, is associated with increased morbidity and mortality. Stage 3 AKI is associated with significantly higher mortality rates.

Doğuştan Kalp Hastalıklı Çocuklarda Optik Koherens Tomografi ile Koroid Kalınlığının Değerlendirilmesi

Objective: The main reason for complications in congenital heart diseases (CHD) is decreased blood oxygen saturation and polycythemia which are typical for cyanosis. These parameters may promote the damage of the retina because haemodynamic regulation is essential for the structural and functional integrity of the macular subfields. The aim of this study was to evaluate choroidal thickness (CT) measurements in children with CHD using spectral domain optical coherence tomography (OCT). Methods: This prospective study compared 30 CHD and 30 healthy control children. CT was examined with spectralis spectral-domain OCT (Retinascan RS-3000; Nidek). CT was obtained at the subfovea, 500 μm and 1000 μm nasal to the fovea (N500, N1000) and 500 μm and 1000 μm temporal to the fovea (T500, T1000). Only the right eye values were used for statistical comparisons between the groups. The domain cardiac lesions were divided physiologically into two categories: volume overload and cyanotic. Results: Mean age was 11.0±3.5 years in CHD childrens and 10.9±3.6 years in the control group (p=0.971). Children with CHD had no statistically significant CT measurements compared with healthy controls (p>0.05). Conclusion: Although in high haematocrit, low oxygen saturation or the presence of the volume overload in the history of CHD patients, our data suggests that patients with CHD show normal CT. The reason may be medical and surgical treatment of hypoxia, erythrocytosis and volume overload in CHD patients.

Chapter 2 - Neurologic complications of congenital heart disease in adults

Congenital heart disease (CHD) is a heterogeneous group of structural abnormalities of the cardiovascular system that are present at birth. Advances in childhood medical and surgical treatment have led to increasing numbers of adults with CHD. Neurological complications of CHD in adults are varied and can include an increased risk of stroke not only related to the underlying congenital defect and its surgical management but also due to atherosclerotic disease associated with advancing age. In addition to cerebrovascular events, CHD in adults is also associated with an increased risk of neurodevelopmental disorders, cognitive impairment, psychiatric disease, and epilepsy. Collaborative multidisciplinary care with contributions from neurologists and cardiologists with expertise in adult CHD is necessary to provide optimal long-term care for this complex and rapidly evolving population.

ATS Core Curriculum 2020. Pediatric Pulmonary Medicine.

The American Thoracic Society Core Curriculum updates clinicians annually in adult and pediatric pulmonary disease, medical critical care, and sleep medicine, in a 3- to 4-year recurring cycle of topics. These topics will be presented at the 2020 International Conference. Below is the pediatric pulmonary medicine core, including pediatric hypoxemic respiratory failure; modalities in noninvasive management of chronic respiratory failure in childhood; surgical and nonsurgical management of congenital lung malformations; an update on smoke inhalation lung injury; an update on vaporizers, e-cigarettes, and other electronic delivery systems; pulmonary complications of sarcoidosis; pulmonary complications of congenital heart disease; and updates on the management of congenital diaphragmatic hernia.

Carcinoembryonic antigen levels are increased with pulmonary output in pulmonary hypertension due to congenital heart disease.

ObjectivePulmonary artery hypertension (PAH) is a severe complication of congenital heart disease (CHD). Monitoring of pulmonary arterial pressure (PAP) and pulmonary vascular resistance (PVR) is essential during follow-up. This retrospective study aimed to examine carcinoembryonic antigen (CEA) as an additional marker for evaluation by investigating the correlation between CEA levels and hemodynamics in CHD-PAH.MethodsSeventy-six patients with CHD-PAH (mean PAP [mPAP] >25 mmHg and PVR >3 Wood units, group A), 71 patients with CHD and pulmonary hypertension (CHD-PH, mPAP >25 mmHg and PVR ≤3 Wood units, group B), and 102 patients with CHD without PH (mPAP ≤25 mmHg, group C) were enrolled. Serum CEA levels and the relationships between CEA levels and hemodynamic data were assessed.ResultsMean serum CEA levels were 1.99±1.61, 2.44±1.82, and 1.58±1.07 ng/mL, mPAP was 58.66±20.21, 30.2±4.83, and 17.31±4.51 mmHg, and PVR was 10.12±7.01, 2.19±0.56, and 2.2±1.1 Wood units in groups A, B, and C, respectively. Mean pulmonary output (PO) was 7.24±3.07, 15.79±5.49, 10.18±4.72 L/minute, respectively. CEA levels were positively correlated with PO and negatively correlated with PVR in all of the patients.ConclusionCEA levels are increased with PO and decreased with PVR in CHD-PH.

Adults With Mild-to-Moderate Congenital Heart Disease Demonstrate Measurable Neurocognitive Deficits.

BackgroundNeurocognitive impairment is a common complication of congenital heart disease (CHD) as well as acquired cardiovascular disease. Data are limited on neurocognitive function in adults with CHD (ACHD).Methods and ResultsA total of 1020 individuals with mild‐to‐moderate ACHD and 497 987 individuals without ACHD from the volunteer‐based UK Biobank study underwent neurocognitive tests for fluid intelligence, reaction time, numeric memory, symbol‐digit substitution, and trail making at enrollment and follow‐up. Performance scores were compared before and after exclusion of preexisting stroke or coronary artery disease as measures of cerebro‐ and cardiovascular disease. Individuals with ACHD had significantly poorer performance on alpha‐numeric trail making, a measure of visual attention and cognitive flexibility, spending 6.4 seconds longer on alpha‐numeric trail making (95% CI, 3.0–9.9 seconds, P=0.002) and 2.5 seconds longer on numeric trail making (95% CI, 0.5–4.6 seconds, P=0.034), a measure of visual attention and processing speed. The ACHD cohort had modestly lower performance on symbol‐digit substitution, a measure of processing speed, with 0.9 fewer correct substitutions (95% CI, − 1.5 to − 0.2 substitutions, P=0.021). After excluding preexisting stroke or coronary artery disease, individuals with ACHD continued to show poorer performance in all 6 domains (P=NS).ConclusionsIndividuals with mild‐to‐moderate ACHD had poorer neurocognitive performance, most significantly in tests of cognitive flexibility, analogous to deficits in children with CHD. These differences appear to be driven by increased burden of cerebro‐ and cardiovascular disease among individuals with ACHD.

3-Dimensional Speckle-Tracking Echocardiography-Derived Interventricular Activation Imaging in a Patient With Repaired Tetralogy of Fallot

Using 3-dimensional speckle-tracking echocardiography-derived activation imaging system, we visualized interventricular dyssynchrony in a repaired tetralogy of Fallot case with pacing-induced left ventricular dysfunction. The activation imaging system visualized interventricular dyssynchrony and resynchronization after cardiac resynchronization therapy and may be useful to assess electromechanical disturbance in complicated congenital heart diseases. (Level of Difficulty: Intermediate.)

A case of giant main pulmonary artery aneurysm associated with complicated congenital heart disease and simultaneous chronic pulmonary artery dissection

BackgroundPulmonary artery aneurysm (PAA), usually associated with congenital heart disease (CHD), is a very rare clinical condition. Pulmonary artery dissection (PAD) is considered the most life-threatening complication of PAA, and patients can progress from being asymptomatic to sudden death. We report the following case of PAA associated with complicated congenital heart disease and simultaneous chronic PAD. To our knowledge, few such complicated cases have ever been reported.Case presentationA 36-year-old male presented to our hospital with a 10-year history of heart fatigue after activities and aggravated for 2 years. Computed tomography angiography (CTA) and echocardiogram showed a giant main pulmonary artery aneurysm (14 cm) with complicated congenital heart disease (a small patent ductus arteriosus, ventricular septal defects, aortic coarctation). Chronic PAD, which was mistaken for a pulmonary valve before operation, was detected during surgery.ConclusionsPAA associated with complicated CHD and simultaneous PAD is very rare, and its clinical symptoms are varied. Radiologists and surgeons should pay attention to determining whether this patient simultaneously had PAD when PAA was detected on preoperative CTA imaging.