Complication Of Congenital Heart Disease Research Articles

Pulmonary hypertension in congenital heart disease of children

Background: Pulmonary arterial hypertension is the dreaded complication of congenital heart disease with left-right shunt. The aim of this study was to describe the epidemio-clinical aspects of PAH associated with congenital heart disease. Methods: This was a retrospective descriptive multicentre study over a 2-year period (1 January 2020 to 31 December 2021) conducted in three University Hospitals in Antananarivo. Hospitalized children with congenital left-right shunt heart disease were included. Results: A total of 74 cases were recruited. The sex ratio was 0.7. The mean age of the children was 10.9 (SD=18.2) months. The mean age of onset of heart disease was 8 (SD=13.6) months. Heart disease was dominated by ventricular septal defect (51.3%), patent ductus arteriosus (39.2%), and atrioventricular canal (8.1%). PAH was encountered in 49 cases (66.2%). The majority of children with PAH were under 12 months of age (71.4%) and malnourished (70.2%). According to the type of heart disease, PAH was encountered in all complete CAVs, in 76.3% of CIVs and in 55.2% of PCAs. Eseinmenger's syndrome was present in 4% of cases, and the death rate was 20.3%. Conclusions: Improved management of children with congenital heart disease is needed, both in terms of diagnosis and surgical treatment, to prevent the onset of PAH.

PULMONARY ARTERIAL HYPERTENSION ASSOCIATED WITH CONGENITAL HEART DISEASE: RATIONALE FOR MANAGEMENT ALGORITHM BASED ON A LITERATURE REVIEW

The substantial progress achieved in cardiology and paediatric cardiac surgery has led to a considerable increase of life expectancy of patients with congenital heart disease (CHD), and at the same time to growing number of adult patients developing pulmonary arterial hypertension (PAH) associated with congenital heart defects. Nowadays guidelines were developed and updated for PAH, but no expert recommendations exist for effective management of PAH associated with CHD. Thus, it is important to develop an algorithm for managing these patients, based on international standards and a comprehensive understanding of prognostic markers of worsening the course of pulmonary arterial hypertension (PAH), considering local cardiac tertiary hospitals' capabilities.
 The aim. To highlight the pathophysiology and clinical characteristics of PAH-CHD patients, identify prognostic markers for its onset and progression, and develop a management algorithm for patients.
 Results. The article presents the pathophysiology of PAH-CHD, the mechanisms of right ventricle adaptation to pressure overload, which determine the functional abilities of patients and prognosis, PAH formation, and progression after defects correction. The accurate identification of stages in PAH-CHD (volume overload, mixed, stage of pulmonary vascular disease) is essential for the timely determination of surgical intervention, selecting conservative treatment strategies, and predicting the outcomes of surgical defect correction. The type of defect, its size, and the volume of blood flow through the defect influence the onset and progression of PH-CHD. We review the pathophysiology and clinical features of PAH-CHD, as well as the reasons for the late diagnosis of heart failure at the stage of severe decompensation and irreversible remodelling of the lung vessels when diuretic therapy is not effective enough and inotropic support, circulatory mechanical support and heart / lung complex transplantation are required. The necessity of developing new scales for stratifying the risk of PAH-CHD is justified considering the particularities of the disease course and cardiac hemodynamic in different anatomical types of the defects. Based on this literature review, at the Municipal Enterprise «Dnipropetrovsk Regional Clinical Center for Diagnostics and Treatment» of the Dnipropetrovsk Regional Council» the algorithm for managing patients with PAH-CHD was devised and implemented into clinical practice.
 Conclusions. PAH is a common complication of CHD. The prevalence of PAH-CHD is substantial and it can manifest and progress even in patients with previously corrected heart defects. To effectively manage patients with PAH-CHD, an algorithm has been devised and implemented in clinical practice. It includes factors that have demonstrated associations with poor prognosis which are more than two hospitalizations for HF in the last year, pulmonary vascular resistance (PVR) (or index of PVR), mean pressure in the pulmonary artery, pulmonary capillary wedge pressure, left ventricular ejection fraction, heart failure with a reduced ejection fraction, right ventricle heart failure, NYHA FC, high risk of atrial fibrillation or flutter, 6-minute walking test distance, NTproBNP, highly sensitive C-reactive protein, B-lines on lung ultrasound, glomerular filtration rate, haemoglobin, platelet count, sodium, Model for End-Stage Liver Disease scale.

Efficacy of atrioventricular valve regurgitation in the first trimester for the diagnosis of atrioventricular septal defect.

To investigate the efficacy of atrioventricular valve regurgitation (AVVR) for predicting atrioventricular septal defect (AVSD) in the first trimester. We performed a prospective observational study, screening for complicated congenital heart diseases and AVVR in fetuses at 11 to 13+6 weeks of gestation by advanced dynamic flow in four-chamber view and three-vessel-trachea view. 43 549 fetuses at 11 to 13+6 weeks of gestation were screened by echocardiography, of which 37 cases were diagnosed with AVSD, including complete AVSD (31 cases), intermediate AVSD (3 cases) and partial AVSD (1 cases), undiagnosed intermediate AVSD (2 cases), and misdiagnosed case (2 cases). AVVR was observed in 34 cases (34/37) in the first trimester, 59. 46% (22/37) nuchal translucency greater than 95th percentile, 29. 73% (11/37) absence of nasal bone, 32. 43% (12/37) ductus venosus A wave inversion, and 40. 54% (15/37) had tricuspid regurgitation. The sensitivity of common AVVR in predicting AVSD is better than other ultrasonic indexes. AVVR can be used as an ultrasonic indicator to predict AVSD in the first trimester, which is beneficial to detect AVSD.

Preoperative Sildenafil in Pediatric Patients Undergoing Congenital Heart Surgeries: A Systematic Review and Meta-Analysis.

Background. Pulmonary hypertension (PH) is a common complication of congenital heart disease (CHD). With early surgical intervention, outcomes have improved over the last two decades. Persistent PH, however, may still occur following surgery. Sildenafil has been shown to be beneficial for postoperative pulmonary hypertensive crises. The role of preoperative sildenafil in controlling postoperative PH is poorly elucidated. This study aimed to evaluate the effect of preoperative sildenafil on pediatric patients undergoing congenital heart surgeries. Methods. A comprehensive literature search was conducted in scientific databases. We included randomized controlled trials which assessed the effect of preoperative sildenafil in pediatric patients with CHD undergoing repair surgeries. Primary outcomes were pre- and postoperative differences in mean pulmonary arterial pressure (mPAP) and mean pulmonary artery/aortic pressure ratio (PA/Ao ratio). Results. Four studies (n = 233) were retained for the final analysis. Dose of sildenafil ranged from .3 to .5mg/kg every 4-6hours via oral/nasogastric route, with timing of administration varied from 1 to 2weeks before surgery. Compared to controls, preoperative sildenafil was associated with greater reduction in postoperative mPAP (MD -5.02; 95% CI [-8.91, -1.13]) and mean PA/Ao ratio (MD -.11; 95% CI [-.17, -.06]). Shorter CPB time, ICU length of stay, and duration of mechanical ventilation were also observed in the sildenafil group. Conclusion. Preoperative sildenafil is beneficial in reducing PAP, thereby reducing the risk of postoperative PH crisis. Further studies are warranted to identify the optimal dosage and timing of administration of sildenafil in CHD patients prior to surgery.

Pattern of Congenital Heart Diseases among Less than 5 Years Old Children of Bangladesh-An Observational Study from a Pediatric Cardiac Center

Introduction: Congenital Heart Disease (CHD) is the most common congenital problems. CHD is about 25% of all congenital malformations and is the most common type of heart disease among children. It may present in different ages from birth to adolescence. Worldwide, CHD is relatively common with a prevalence ranging from 1.0-6.6 per 1000 live births. Aim of the Study: The aim of this study was to find out the pattern of congenital heart diseases among less than 5 years old Children of a pediatric cardiac center. Methods: This was a retrospective observational study and conducted in the Department of pediatric cardiology of Combined Military Hospital Dhaka, Bangladesh during the period from July, 2018 to July, 2020. A total of 1000 patients from outdoor and indoor patient registry who were diagnosed and treated as a case of congenital heart disease within the mentioned period were included in the study. Result: Majority of our patients was male (58%) compared to female (42%). Among all patients, 29.80% had VSD, followed by 20.4% & 14.9% had ASD & TOF respectively and 72% & 28% of our patients had Acyanotic & cyanotic CHD respectively.VSD & TOF was the most common in patients with acyanotic & cyanotic CHD respectively. Recurrent RTI, pneumonia, heart failure, infective endocarditis, and Cyanotic spell are common complications of congenital heart disease. Down syndrome and congenital rubella syndrome are the main syndromic association in congenital heart diseases. The majority (97%) of our patients improved, and 3% died. Conclusion: Our study focuses on the congenital heart diseases of children of Bangladesh. Among the acyanotic congenital heart disease, VSD is the maximum & among the cyanotic congenital heart disease TOF is the highest in number. With early diagnosis, treatment and regular follow up, mortality rate is very low and the prognosis is excellent.

Doppler Echocardiography Combined with NTproBNP/BNP in the Diagnosis of Pulmonary Artery Hypertension Associated with Congenital Heart Disease.

Pulmonary artery hypertension (PAH) is a common complication of congenital heart disease (CHD) and is associated with worse outcomes and increased mortality. The Doppler echocardiography (DE) is a commonly used imaging tool for both diagnosis and follow-up examination of PAH. Here is to evaluate the diagnostic performance of DE combined with NTproBNP/BNP as screening strategy in PAH patients with CHD. A retrospective study in 64 patients with CHD has been carried out to compare estimate pulmonary artery systolic pressure (PASP) measured with DE to that measured with right heart catheterization (RHC). The Pearson correlation analyses were used to calculate the correlation coefficients between RHC and DE. The Bland-Altman analyses were carried out to assess the agreement between the two methods. ROC analyses were used to evaluate the diagnostic performance of DE, NTproBNP/BNP, and DE combined with NTproBNP/BNP. Our data have demonstrated that a mild correlation (r = 0.4401, P < 0.01) was observed between PASP (78.1 ± 29.0 mmHg) measured during RHC and PASP (74.9 ± 19.7 mmHg) as estimated using DE. The Bland-Altman analysis demonstrated that the bias for DE PASP estimates was 3.2 mmHg with 95% limits of agreement ranging from -49.53 to 55.90 mmHg. The results of DE showed an AUC of 0.848 (95% CI = 0.666-1; P < 0.001), the sensitivity of which was 98.3% and the specificity was 77.8%. The AUC of NTproBNP/BNP for the identification of PAH was 0.804 (95% CI = 0.651-0.956; P < 0.001), the sensitivity of which was 81.4% and the specificity was 87.5%. The AUC of DE combined with NTproBNP/BNP was 0.857 (95% CI = 0.676-1; P < 0.001), of which sensitivity was 100% and specificity was 77.8%. The positive predictive value (PPV) and negative predictive value (NPV) were 96.6% and 100%, respectively. Our study shows that the Doppler echocardiography combined with NTproBNP/BNP has better diagnostic performance in pulmonary artery hypertension associated with congenital heart disease, especially when DE negative screening in PAH patients.

Analysis on echocardiographic data of fetal heart defects at high altitude in Yunnan province and surrounding high altitude areas

Objective: To explore the current situation of fetal heart defects in Yunnan Province and surrounding high altitude areas and the social factors affecting pregnancy outcome. Methods: This is a retrospective study. Pregnant woman who underwent fetal echocardiography and diagnosed as fetal cardiac defects in Yunnan Fuwai Cardiovascular Hospital from June 2017 to January 2021 were included. According to the clinical prognostic risk scoring system and grading criteria of fetal cardiac birth defects, the cases were divided into grade Ⅰ to Ⅳ. The disease distribution and proportion of each prognostic grade, pregnancy outcomes were analyzed and compared. The cases were divided into continued pregnancy group and terminated pregnancy group according to pregnancy outcome. The social factors that may affect the selection of pregnancy outcomes were analyzed by multivariate logistic regression analysis. Results: A total of 4 929 fetal echocardiography examination data were collected, and 4 464 cases (90.57%) were from Yunnan Province and surrounding high altitude areas. 2 166 cases of heart defects were finally analyzed, including 998 cases of congenital heart disease (CHD), 93 cases of cardiac tumors, cardiomyopathy and arrhythmia, 1 075 cases of foramen ovale, ductus arteriosus abnormalities and normal variations. The pregnant women were (29.2±5.0) years old with (25.6±3.8) gestational weeks. The number of cases with prognostic grade from Ⅰ to Ⅳ was 1 037 (47.88%), 620 (28.62%), 314 (14.50%), and 44 (2.03%), respectively. And 151 cases (6.97%) were not classified. The cases of normal variation and thin aortic arch development accounted for 42.66% (924/2 166), 5.22% (113/2 166), respectively. The top 3 diseases of grade Ⅱ were ventricular septal defect, coarctation of aorta and mild-moderate pulmonary stenosis, respectively, and their distribution was 11.63% (252/2 166), 3.92% (85/2 166) and 2.35% (51/2 166) respectively in all cases of heart defects, and 25.25% (252/998), 8.52% (85/998) and 5.11% (51/998) respectively in cases of CHD. Among the cases rated as grade Ⅲ and Ⅳ, most of them were complicated congenital heart disease, and the disease types are scattered. The more common cases in grade Ⅲ were complete transposition of great arteries (accounting for 2.40% (52/2 166) of all cases with heart defects, 5.21% (52/998) of all cases with CHD) and pulmonary artery occlusion (type Ⅰ to Ⅲ) with ventricular septal defect (accounting for 2.17% (47/2 166) of all cases with heart defects, and 4.71% (47/998) of all cases with CHD). In grade Ⅳ, single ventricle (0.74% (16/2 166) of all cases with heart defects, 1.60% (16/998) of all cases with CHD) and left ventricular dysplasia syndrome (0.65% (14/2 166) of all cases with heart defects, 1.40% (14/998) of all cases with CHD) are more common. A total of 1 084 cases were successfully followed up, and 675 cases were born, 392 cases were terminated, spontaneous abortion occurred in 17 cases. The proportion of terminated pregnancy cases was significantly increased from grade Ⅰ to Ⅳ, accounting for 5.24% (21/401), 27.78% (70/252), 89.54% (214/239) and 95.56% (43/45), respectively. Among the terminated pregnancy cases, those with grade Ⅲ accounted for the highest proportion (54.59% (214/392)). The distribution of terminated pregnancy cases was mainly complex congenital malformations or diseases with very poor prognosis (pregnancy outcome grade Ⅲ and Ⅳ), and proportion of terminated pregnancy with pregnancy outcome grade Ⅰ and Ⅱ cases (normal variation or good prognosis) accounted for 5.36% (21/392) and 17.86% (70/392), respectively. The results of multivariate logistic regression analysis showed that pregnant women with low education (high school and below: OR=2.73, 95%CI 1.26-5.93, P<0.001; illiteracy: OR=3.27, 95%CI 1.29-7.10, P<0.001) and low family income (Annual income<100 000 yuan: OR=2.47, 95%CI 1.69-5.12, P<0.001) were more likely to choose termination of pregnancy in case of fetal heart defect. Conclusion: In Yunnan province and the surrounding high altitude areas, the disease distribution of fetal heart defect is mainly simple or low-risk disease, but the complex malformation, especially the disease with poor pregnancy outcome, accounts for a relative high proportion. Pregnancy termination also occurs in some cases with good pregnancy outcome. The education level and family income of pregnant women may affect their choice of pregnancy outcome in case of fetal heart defect.

Quality of life in adults with congenital heart disease with and without pulmonary hypertension: a comparative study.

Pulmonary vascular disease and pulmonary hypertension (PH) belong to the most relevant complications of congenital heart disease (CHD) in the long-term course. Although PH might lead to a severely impaired quality of life (QOL), there are no current studies comparing QOL in adults with CHD (ACHD) with and without PH. Therefore, this study aimed to systematically examine QOL in ACHD with and without PH in order to generate a more differentiated understanding of their overall health-status and to employ newly gained findings into general care of this particular patient population. In this comparative cross-sectional study, a representative sample of 803 adults with various forms of CHD with or without PH was analyzed. Data collection took place between September 2017 until February 2018 in a tertiary care center for ACHD. Medical data was retrieved from medical records. QOL was assessed using the EQ-5D-5L questionnaire. Descriptive methods, chi-square and t-tests were used to compare QOL of patients with and without PH. Of 803 patients, 752 patients had no PH [93.6%; 47.3% female; mean age: 34.9±11.83 (range, 18-86) years], 51 were identified with manifest PH [6.4%; 55.8% female; mean age: 41.9±12.17 (range, 21-69) years]. PH patients showed significantly worse overall QOL [mean no PH: 86.78±13.30 (8.2-100) vs. mean PH: 81.79±12.77 (43.6-100); 95% confidence interval (CI): 1.43 to 9.46; P=0.013], worse crosswalk index [mean no PH: 93.29±12.86 (-11, 100) vs. mean PH: 89.73±11.77 (47-100); 95% CI: 0.08 to 7.56; P=0.043], and worse VAS [mean no PH: 80.12±16.50 (15-100) vs. mean PH: 72.56±16.80 (40-100); 95% CI: 3.29 to 12.17; P=0.004]. However, after adjusting for age, only the VAS stayed significant (P=0.039). The QOL within the dimensions mobility (P<0.001), self-care (P=0.002), and usual activities (P=0.007), were significantly decreased in PH patients. Overall, anxiety and depression (11.08%) as well as pain and discomfort (11.03%) were the most impaired dimensions in both patient groups. Since QOL is a significant predictor of outcome, PH-CHD patients need an early provision of psychosocial, health promoting support in addition to dedicated care and targeted PH treatment. It is therefore pivotal to timely identify unique psychosocial impairments in order to enhance quantity and QOL in this particularly vulnerable patient population.

The Ventricular Hypokinesis at the Apical Pacing Site due to the Early Shortening and Rebound Stretch in a Case with Fontan Procedure.

The left ventricular (LV) apex is recommended as the first choice for positioning the epicardial pacing. We encountered a patient with congenital heart disease (CHD) showing hypokinesis of the LV apical pacing site after implantation of a pacemaker with epicardial leads. This phenomenon was revealed by the early shortening and systolic rebound stretch of the same lesion on two-dimensional speckle tracking echocardiography, which developed in the intraventricular dyssynchrony between the LV apex and base. Cardiac resynchronization therapy provided an excellent result around the hypokinetic lesion. It is wise to arrange detailed evaluations in each patient with complicated CHD, aiming at a successful treatment to enable ventricular synchronicity.

Paediatric pulmonary arterial hypertension following congenital heart defect repair: enhanced risk stratification and outcomes in a national cohort

Abstract Introduction Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD). PAH following CHD repair (repaired PAH-CHD) is an emerging population with increased morbidity and mortality [1–3]. Risk scores that are widely used in the adult PAH population have not been validated and may not be applicable in young children with repaired PAH-CHD. Purpose To characterize the 20-year cohort of the UK National Paediatric PH Service, focusing on peri-operative characteristics and outcomes of children with repaired PAH-CHD, and develop a tailored risk stratification tool. Methods We included consecutive children presenting to our specialist service between 2001 and 2021 with a diagnosis of repaired PAH-CHD. Patients with univentricular physiology, segmental pulmonary hypertension (PH), and PH primarily due to abnormal development of the pulmonary vasculature were excluded. Univariable Cox regression analysis was performed in children with PAH present ≥3 months after CHD repair, with an outcome of death or transplantation. Time-dependent ROC analysis was used to evaluate the risk model. Results Overall, 178 patients were included (age 3.2 [1.3–7.9] years, 58.4% female). Most children (73.0%) were referred following CHD repair. Complex CHD was present in 61.2%, and 48.9% had combined pre- and post-tricuspid shunts. Down syndrome was present in 33.1%. At the first post-operative PH assessment, 53.1% of patients had symptoms, mainly breathlessness. On echocardiographic evaluation, 30.9% had moderate-severe right ventricular dilatation and 23.7% had right ventricular systolic impairment. Over a median follow-up of 6.0 [2.4–11.0] years, 30 (19.2%) patients died and 5 (3.2%) patients underwent lung transplantation. Transpant-free survival at 1, 5, and 10 years was 94.7% (95% CI: 91.2–98.3%), 85.9% (95% CI: 80.3–91.9%), and 80.1% (95% CI: 73.1–87.7%), respectively. Cox analysis identified several clinical variables associated with death or transplantation, of which the following variables assessed post-repair were used to develop the risk score: absence of pre-operative PH, breathlessness, right ventricular dysfunction, and pulmonary vascular resistance index &amp;gt;14 WU m2 or lesion at high risk of causing PH (Figure 1). This novel, simple risk score performed well (AUC &amp;gt;80% at 1, 3, 5 and 10 years) and was well-calibrated in our cohort (Figure 2, D'Agostino-Nam p=0.76). Conclusions In this national cohort of children with repaired PAH-CHD, mortality is significant. This novel, simple risk score performed well in our population and could be used in clinical practice at the time of post-operative assessment to predict outcome and direct management but requires further validation. Funding Acknowledgement Type of funding sources: None.

The patient with Down syndrome and pulmonary arterial hypertension associated with congenital heart disease: from childhood to adulthood

Abstract Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD) that significantly affects its prognosis. Down syndrome is a common comorbidity among patients with PAH associated with CHD. Children born in recent years with CHD have benefited from advances in pediatric cardiac surgery that have modified their evolution compared to adults with the same pathologies. Our objective was to know and compare the characteristics of the adult and pediatric population with DS and PAH associated with CHD. REHAP and REHIPED are Spanish, voluntary, multicenter registries that include patients with PAH associated with CHD. REHAP started in 2007 and includes patients over 18 years of age. Patients diagnosed with PAH after this time were prospectively included, until 2021. Patients who were followed in the participating centers and diagnosed after January 1998 were retrospectively included in the registry. REHIPED started in 2009 and collects patients between 2 months and 18 years of age. Patients diagnosed with PAH after this time were prospectively included, until 2021. Patients who were followed and diagnosed after January 1998 were retrospectively included. 131 adults and 47 children with DS and PAH associated with CHD were analyzed. Their baseline characteristics, hemodynamics, treatment, and 10-year survival were compared. Adult patients with DS presented more frequently complete atrioventricular canal defect as underlying heart disease and were mostly in Eisenmenger's situation (84%). In pediatric patients the largest group was post-repair PAH (50%). Functional class was similar in both groups, although adults presented significantly greater hemodynamic severity. Regarding medical treatment, no differences were observed between both groups: there was a very low use of prostanoids both at baseline and at follow-up, and a majority use of oral monotherapy. There were no significant differences in survival. Corrective surgery in patients with DS has become more frequent over the years: adults are mostly in Eisenmenger's situation, while post-repair PAH is the most numerous group in children. Although adults show greater hemodynamic severity, survival is similar in both groups. Medical treatment, which showed no differences between pediatric and adult age, is very conservative, with most patients on oral monotherapy and rare use of prostanoids. Funding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Janssen MSD

Psychological needs of parents of children with complicated congenital heart disease after admitting to pediatric intensive care unit: A questionnaire study.

BACKGROUNDParents of children with complicated congenital heart disease (CHD) have different needs after surgery. Little literature reports the impact factors for psychological needs of parents of children with complicated CHD.AIMTo investigate the status quo of the needs of parents of children after surgery for complex CHD, and analyze the influencing factors, in order to provide a theoretical basis for formulating corresponding nursing countermeasures.METHODSA modified Chinese version of the Critical Care Family Needs Inventory (M-CCFNI) was used to select 200 parents of children with complex CHD after surgery within 72 h after admission to the intensive care unit in our hospital to conduct an online questionnaire survey. The aim was to understand the needs of parents in relation to the following five aspects: The support from medical staff, comfort of the parents themselves, the acquisition of information, their closeness to the children, and assurance of the child’s condition.RESULTSParents of children with complex CHD had a higher degree of demand, especially in terms of condition assurance, acquisition of information, and closeness to the children. The age, education level, and residence of the parents were related to the five dimensions of the needs of parents of children with complex CHD who had undergone surgery.CONCLUSIONIn practice, nurses should formulate corresponding nursing strategies based on the different cultural and social backgrounds of parents of children after complex CHD surgery to meet their different needs, and improve satisfaction. These findings provide a theoretical basis for constructing a family participatory nursing model for children in the intensive care unit in the future.

Congenital Heart Disease and Neurodevelopmental Outcomes in Infants and Adolescents

Abstract: Patients with complicated congenital heart disease are at risk of neurodevelopmental disability. This study aimed to study the correlation between brain development and congenital heart disease in infants. Studies have shown that brain maturation is delayed, and injury may be caused in the white matter or brain volume after the interventions like surgery for congenital heart disease. Secondary research was conducted to find the results of the effects of congenital heart disease on brain development. It was found that white matter has more injury than grey matter. The total brain volume is lower than in control patients. Amongst six different types of congenital heart disease (TOF, DORV, TGA, AoOb, HLHS, HRHS) studied Tetralogy of Fallot showed the most reduced brain volume compared to others. Adolescents with cyanotic heart disease lost more brain volume than those without, particularly in the white matter. In conclusion, it was found that patients with congenital heart disease have smaller brain volumes, smaller cerebellar volumes, and injury in the white matter

Clinical Applications of 4D Flow MR Imaging in Aortic Valvular and Congenital Heart Disease.

4D flow MRI allows time-resolved 3D velocity-encoded phase-contrast imaging for 3D visualization and quantification of aortic and intracardiac flow. Radiologists should be familiar with the principles of 4D flow MRI and methods for evaluating blood flow qualitatively and quantitatively. The most substantial benefits of 4D flow MRI are that it enables the simultaneous comprehensive assessment of different vessels, and that retrospective analysis can be achieved in all vessels in any direction in the field of view, which is especially beneficial for patients with complicated congenital heart disease (CHD). For aortic valvular diseases, new parameters such as wall shear stress and energy loss may provide new prognostic values for 4D flow MRI. In this review, we introduce the clinical applications of 4D flow MRI for the visualization of blood flow and quantification of hemodynamic metrics in the setting of aortic valvular disease and CHD, including intracardiac shunt and coronary artery anomaly.

C80. Pulmonary Embolism in Congenital Heart Disease with Pulmonary Arterial Hypertension: a Case Series

Abstract Background Pulmonary arterial hypertension (PAH) is a frequent complication of congenital heart disease (CHD), notably in patients with left-to-right shunts. Patients with severe PAH due to CHD, Eisenmenger syndrome (ES), represent a high-risk group of pulmonary artery (PA) thrombosis patients. We present serial adult cases of CHD with PAH complicated by pulmonary embolism (PE). Case Description We found 3 cases of pulmonary embolism on adult congenital heart disease in our center. All of the patients were female, with an age range of 36 to 61 years old. The presenting symptoms were shortness of breath, WHO classification class II-III. All patients were hemodynamically stable. CHD and estimated RV systolic pressure were evaluated with echocardiography. CT-PA was performed in all patients, which findings were supporting PE. All patients were treated with anticoagulation. After three months, right heart catheterization was conducted in two patients, and one patient was refused. Discussion The prevalence of PE among adult patient with CHD and PAH range from 17 to 21%. PE was frequently found in older patients, women, and patients with lower oxygen saturation. To date, no uniformity regarding routine prophylaxis anticoagulation therapy in ES due to the high bleeding risk in this population despite being at risk of PA thrombosis. We conclude that risk stratification for PA thrombosis in adult CHD-PAH patients is essential, and further research is needed to prevent PE in this population.

OR14. Cardiac Index by Right Heart Catheterization was an Independent Predictor of Mortality in Adult Pulmonary Arterial Hypertension-associated with Congenital Heart Disease with Shunt

Abstract Background and Aims Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD). There were several variables predictor of mortality in PAH-CHD from previous studies, but study predictor of mortality in PAH-CHD with shunts has never been done, especially in Indonesia. This study aims to identify independent predictors of mortality among adult PAH-CHD with shunt in Dr. Sardjito Hospital, Yogyakarta, Indonesia. Method and Results We conducted a retrospective cohort study that included adult PAH-CHD patients with shunts from COHARD-PH (The Congenital HeARt Disease in adult and Pulmonary Hypertension) registry who were recruited from July 2012 - October 2020. Twelve variables were selected as potential predictors of mortality. Predictor variables were obtained from clinical (WHO-functional class, SaO2, 6-min walking distance), laboratory (haemoglobin, NT-pro BNP), echocardiographic (pericardial effusion, defect size, TAPSE), and hemodynamic (RA pressure, cardiac index, SvO2, flow ratio) data at the time of diagnosis of PAH. Baseline characteristics showed significantly lower systolic blood pressure, higher incidence of Eisenmenger syndrome, higher NT-pro BNP, and lower cardiac output in subjects who died as compared to those survived. Multivariate analysis showed cardiac index as an independent predictor of mortality (OR 7.69; p = 0.011). Systolic blood pressure was a significant confounder of cardiac index in affecting mortality (OR 10.56; p = 0.001). Conclusion Cardiac index was an independent predictor of mortality of PAH-CHD with shunts. Systolic blood pressure was a significant confounder of cardiac index on mortality.

C80. Pulmonary Embolism in Congenital Heart Disease with Pulmonary Arterial Hypertension: a Case Series

Abstract Background Pulmonary arterial hypertension (PAH) is a frequent complication of congenital heart disease (CHD), notably in patients with left-to-right shunts. Patients with severe PAH due to CHD, Eisenmenger syndrome (ES), represent a high-risk group of pulmonary artery (PA) thrombosis patients. We present serial adult cases of CHD with PAH complicated by pulmonary embolism (PE). Case Description We found 3 cases of pulmonary embolism on adult congenital heart disease in our center. All of the patients were female, with an age range of 36 to 61 years old. The presenting symptoms were shortness of breath, WHO classification class II-III. All patients were hemodynamically stable. CHD and estimated RV systolic pressure were evaluated with echocardiography. CT-PA was performed in all patients, which findings were supporting PE. All patients were treated with anticoagulation. After three months, right heart catheterization was conducted in two patients, and one patient was refused. Discussion The prevalence of PE among adult patient with CHD and PAH range from 17 to 21%. PE was frequently found in older patients, women, and patients with lower oxygen saturation. To date, no uniformity regarding routine prophylaxis anticoagulation therapy in ES due to the high bleeding risk in this population despite being at risk of PA thrombosis. We conclude that risk stratification for PA thrombosis in adult CHD-PAH patients is essential, and further research is needed to prevent PE in this population.

Abstract 13798: Pulmonary Arterial Hypertension Associated With Repaired Congenital Heart Disease in Children: Comparable Survival to Eisenmenger Syndrome

Introduction: Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD). Advances in management have altered the demographics of PAH-CHD, with fewer cases of Eisenmenger syndrome (ES) and more patients with PAH after CHD repair, who have worse outcomes than ES patients and an idiopathic PAH (IPAH)-like phenotype. However, pediatric data are limited. Methods: Patients under active follow-up in the National Paediatric Pulmonary Hypertension service UK between 2001 and 2021 were identified. Incident patients were included in survival analysis. Results: Overall, 164 patients with repaired PAH-CHD were included (57.9% female, 53.7% trisomy 21, 3.7[0.1-16.8] years at diagnosis), along with 95 patients with ES and 146 with IPAH. Most repaired PAH-CHD patients had an isolated post-tricuspid (59%), followed by a combined pre/post-tricuspid (28%) and isolated pre-tricuspid shunt (13.4%). Median age at repair was 13.6[5.6-51] months. PAH was diagnosed prior to repair in 24.5%. Symptoms were present in 53.7%, whereas 46.3% were identified during surveillance. PAH therapy was started in 86%; at last follow-up, repaired PAH-CHD patients were less likely to be on combination therapy than IPAH patients (32.9% vs 70.5%, p&lt;0.0001). Resolution of PH occurred in 8.5% patients at 6.4[3.1-9.4] months from diagnosis. Transplant-free survival in repaired PAH-CHD was 91.9% (95%CI: 87.6-96.4%) at 1 year, 86.1% (80.4-92.2%) at 5 years, and 77% (68.2-86.9%) at 10 years. Repaired PAH-CHD diagnosed in infancy had a significantly worse survival ( Fig 1A ). There was no difference in survival between repaired PAH-CHD and ES (p=0.68). IPAH had a worse survival than both groups (HR vs repaired 1.97, 95%CI:1.22-3.2, p=0.006, Fig 1B ). Conclusions: In this national paediatric cohort, patients with PAH after CHD repair had a better prognosis than patients with IPAH and similar to ES, albeit with a 23% mortality at 10 years, worse in patients diagnosed as infants.

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&amp;lt;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&amp;lt;0.0001). The proportion of group B PAH-CHD patients increased (9.4% vs. 33.3%, p&amp;lt;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.