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

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
A Constantine ◽  
K Dimopoulos ◽  
R Condliffe ◽  
P Clift ◽  
G Chaplin ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Free Survival ◽  
Paediatric Cohort ◽  
Group A ◽  
Pulmonary Arterial ◽  
Group B ◽  
Group D ◽  
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. FUNDunding 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 1 Figure 2

scholarly journals Diagnosis of Neonatal Congenital Heart Disease: A Combination of Heart Murmur, SpO2 Abnormality, Tachypnea, and Extracardiac Malformations

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Kai Chen ◽  
Jiao Wang ◽  
Huihui Zhou ◽  
Xiang Huang
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Heart Murmur ◽  
Heart Murmurs ◽  
Group A ◽  
Postnatal Outcome ◽  
Group B ◽  
Group D ◽  
Youden’S Index

Congenital heart disease (CHD) is one of the commonest congenital malformations that are mostly asymptomatic at birth, which challenges the diagnosis of neonatal CHD. An early accurate prenatal diagnosis will give parents a choice, as well as the opportunity to plan the delivery and improve the postnatal outcome. The purpose of the study is to evaluate the value of heart murmurs, SpO2 abnormalities, tachypnea, and extracardiac malformations in screening neonatal CHD. All 4500 newborns in the obstetrics department of our hospital from January 2019 to January 2020 are selected as study subjects. Newborns were grouped according with the presence of heart murmurs, tachypnea, transdermal SpO 2 < 95 % , and extracardiac malformations alone or in combination (≥3). Patients with murmur, tachypnea, and abnormal SpO2 were assigned into group A, those with murmur, tachypnea, and extracardiac malformations into group B, those with murmurs, SpO2, and extracardiac malformations into group C, those with SpO2, tachypnea, and extracardiac malformations into group D, and those with all four into group E. The color echocardiography identified 65 children with CHD (1.4%) among the included 4,500 newborns. When murmur, tachypnea, abnormal SpO2, and extracardiac malformation were independently used to diagnose CHD, the sensitivity ranged from 30.68% to 51.26%, with specificity ranging from 47.36% to 82.65% and Youden’s index (YI) ranging from 0.13 to 0.36. When murmur, tachypnea, abnormal SpO2, extracardiac malformation were together used to diagnose CHD, 91.23% sensitivity, 95.26 specificity, and 0.91 YI were observed. In conclusion, a combination of four indicators, murmur, tachypnea, abnormal SpO2, and extracardiac malformation yielded good performance in diagnosing neonatal CHD.

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

2020 ◽  
Vol 48 (11) ◽  
pp. 030006052096437
Author(s):  
Yang Zi-yang ◽  
Zhao Kaixun ◽  
Luo Dongling ◽  
Yin Zhou ◽  
Zhou Chengbin ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Carcinoembryonic Antigen ◽  
Congenital Heart ◽  
Pulmonary Artery Hypertension ◽  
Group A ◽  
Serum Cea ◽  
Pulmonary Arterial ◽  
Group B

Objective Pulmonary 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. Methods Seventy-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. Results Mean 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. Conclusion CEA levels are increased with PO and decreased with PVR in CHD-PH.

scholarly journals COMPARISON OF INTRANASAL DEXMEDETOMIDINE V/S MIDAZOLAM AS A PREMEDICATION IN CHIDREN WITH CONGENITAL HEART DISEASE UNDERGOING CARDIAC SURGERY

2021 ◽  
Vol 9 (01) ◽  
pp. 814-822
Author(s):  
Sanjay Kumar Morwal ◽  
Ajit Singh ◽  
C.K. Vyas ◽  
Neelu Sharma
Keyword(s):  
Congenital Heart Disease ◽  
Cardiac Surgery ◽  
Heart Disease ◽  
Congenital Heart ◽  
Arterial Blood ◽  
Sedative Effects ◽  
Group A ◽  
Child Separation ◽  
Group B ◽  
Intranasal Midazolam

Background: The intranasal route is a reliable way to administer preanaesthetics and sedatives to children. The aim of present study was to compare the anxiolytic and sedative effects of intranasal dexemedetomidine and midazolam as a premedication in children with congenital heart disease undergoing cardiac surgery.. Patients and Methods: Fifty chidrens 2-12 years of age group of either sex with congenital heart disease undergoing cardiac surgery were randomly allocated into two groups. Group A-(n=25) - received intranasal dexmedetomidine as premedication.(0.1 µg/kg diluted in 2ml NS ) Group B- (n=25)- received intranasal midazolam as premedication. (0.2 mg/kg diluted in 2ml NS) Heart rate, mean arterial blood pressure and oxygen saturation were monitored upto30 minute after drug administration. The sedation score, anxiety score and child separation score were recorded until the child taken to the OR. The postoperative agitation score was also observed. Results and Conclusion: Premedication with intranasal dexmedetomidine attained significant and satisfactory sedation with better parental separation and lower anxiety levels without any adverse effects as compared with intranasal midazolam in children with congenital heart disease undergoing cardiac surgery.

scholarly journals Efficacy of citrulline supplementation to decrease the risk of pulmonary hypertension after congenital heart disease surgery. A local experience.

2020 ◽  
Vol 77 (4) ◽  
pp. 249-253
Author(s):  
Silene Silvera Ruiz ◽  
Carola L Grosso ◽  
Margot Tablada ◽  
Marcelo Cabrera ◽  
Raquel Dodelson de Kremer ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Pediatric Patients ◽  
Reference Level ◽  
Plasma Citrulline ◽  
Post Surgery ◽  
Group A ◽  
Group B

Pulmonary hypertension (PH) is a major cause of morbi-mortality among patients with congenital heart disease (CHD) and also a potentially severe complication after surgical repair. Oral citrulline, a precursor to NO synthesis, is safe and efficacious for decreasing the risk of postoperative PH. Objective: The aim of the present study was to investigate in pediatric patients the changes of plasma citrulline, arginine, homocysteine and nitric oxide (NO) metabolites and pulmonary artery pressures (PAP) pre-post cardiac surgery in order to describe our population status with regard to the risk of pulmonary hypertension and look for potential biomarkers for early detection and treatment. Main results/Discussion: 16 Argentine pediatric patients with CHD undergoing cardiopulmonary bypass were randomized in two groups: (A) with and (B) without perioperative citrulline supplementation. We found that plasma citrulline median levels before surgery were lower in both groups respect to referential values, probably due to the poor nutritional status of our patients; only group A surpassed post-surgery the minimum recommended level to avoid PH. Furthermore, none of the patients in group A showed mean PAP higher than 20 mmHg, whereas in group B, 67% of the measurements were ≥ than the reference level. Conclusions: We reaffirm that citrulline supplementation it is effective in reducing postoperative pulmonary hypertension and biomarkers could evidence patient status as a translational medicine application.

scholarly journals Delayed Onset of Alcuronium Effect in Children with Cyanotic Congenital Heart Disease

1993 ◽  
Vol 21 (2) ◽  
pp. 197-200 ◽  
Author(s):  
J. P. Keneally ◽  
P. H. Goonetilleke ◽  
I. M. Ramzan
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Plasma Concentrations ◽  
Cyanotic Congenital Heart Disease ◽  
Disease Group ◽  
Normal Children ◽  
Significant Difference ◽  
Group A ◽  
Group B

The effect of alcuronium dichloride (0.3 mg/kg) was studied in seven normal children (Group A), nine with acyanotic congenital heart disease (Group B) and eight with cyanotic disease (Group C). The onset of action was recorded using an integrated electromyograph and blood samples taken for later estimation of plasma concentrations of the drug. The mean time (SD) taken to 75% suppression of twitch height was 1.3(0.8), 1.7(1.0) and 3.8(2.8) minutes, respectively, in each of the three groups. This was significantly slower in Group C compared with both other groups (P<0.05). While six of the Group A patients and seven from Group B reached 95 % paralysis within ten minutes, only two of the cyanosed children achieved this level of relaxation. However, if times to 95% relaxation were extrapolated, there was no significant difference between the groups at 4.5(3.9), 5.8(5.7) and 10.9(6.5) minutes respectively. There was a weak but statistically significant relationship between haematocrit and time to 75% blockade. Maximum twitch depression was similar in all three groups with plasma concentrations at this time being 1.6(0.7), 1.8(0.5) and 2.3(1.4) μg/ml respectively. Again, there was no statistically significant difference between these values. These results confirm that the clinical onset of relaxation is delayed in children with cyanotic congenital heart disease, possibly because of delayed distribution of alcuronium.

scholarly journals Universal implantation of temporary epicardial pacing wires after surgery for congenital heart disease: necessity or luxury?

2019 ◽  
Author(s):  
Gopinath Perumal ◽  
Supreet Prakash Marathe ◽  
Kim S Betts ◽  
Jessica Suna ◽  
Jim Morwood ◽  
...  
Keyword(s):  
Risk Factors ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Primary Outcome ◽  
Postoperative Arrhythmia ◽  
Propensity Matching ◽  
Group A ◽  
Epicardial Pacing ◽  
Group B

AbstractOBJECTIVESRoutine implantation of temporary epicardial pacing wires after surgery for congenital heart disease (CHD) has recently been questioned. We evaluated the incidence of arrhythmias, arrhythmias causing haemodynamic compromise and the safety of a strategy of selective implantation of pacing wires in our unit.METHODSAll patients who underwent surgery for CHD using cardiopulmonary bypass between September 2015 and December 2016 were retrospectively enrolled in the study (n = 313). Patients were stratified into group A (universal implantation) and group B (selective implantation). Group B received pacing wires only when postoperative rhythm disturbances were anticipated based on the operating surgeon’s judgement. The primary outcome was arrhythmia causing haemodynamic compromise. Outcomes were compared between unmatched and propensity matched groups.RESULTSForty-eight patients experienced an arrhythmia causing haemodynamic compromise (15.3%). Twenty-three patients (7.3%) experienced an arrhythmia causing haemodynamic compromise that required the use of pacing wires for therapeutic purposes (group A n = 13, group B n = 10, P = 0.34). There were no pacing wire related complications in either group. All patients in group A and 90% in group B had pacing wires when needed (P = 0.435). In group A, 89% of patients had pacing wires which were not used compared with 13% in group B (P < 0.001). Results were unchanged when repeated using propensity matching (81 pairs).CONCLUSIONSThe probability of developing a postoperative arrhythmia requiring therapeutic pacing can be predicted using the risk factors identified in our study. The routine implantation of pacing wires after surgery for CHD is not necessary. A measured reduction from universal implantation is safe.

scholarly journals Angiostatic Peptide, Endostatin, Predicts Severity in Pediatric Congenital Heart Disease–Associated Pulmonary Hypertension

2021 ◽  
Author(s):  
Caroline M. Daly ◽  
Megan Griffiths ◽  
Catherine E. Simpson ◽  
Jun Yang ◽  
Rachel L. Damico ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Artery ◽  
Risk Stratification ◽  
Disease Severity ◽  
Congenital Heart ◽  
Adverse Outcomes ◽  
Right Atrial ◽  
Free Survival ◽  
Over Time

Background Endostatin, an angiogenic inhibitor, is associated with worse pulmonary arterial hypertension (PAH) outcomes in adults and poor lung growth in children. This study sought to assess whether endostatin is associated with disease severity and outcomes in pediatric PAH. Methods and Results Serum endostatin was measured in cross‐sectional (N=160) and longitudinal cohorts (N=64) of pediatric subjects with PAH, healthy pediatric controls and pediatric controls with congenital heart disease (CHD) (N=54, N=15), and adults with CHD associated PAH (APAH‐CHD, N=185). Outcomes, assessed by regression and Kaplan‐Meier analysis, included hemodynamics, change in endostatin over time, and transplant‐free survival. Endostatin secretion was evaluated in pulmonary artery endothelial and smooth muscle cells. Endostatin was higher in those with PAH compared with healthy controls and controls with CHD and was highest in those with APAH‐CHD. In APAH‐CHD, endostatin was associated with a shorter 6‐minute walk distance and increased mean right atrial pressure. Over time, endostatin was associated with higher pulmonary artery pressure and pulmonary vascular resistance index, right ventricular dilation, and dysfunction. Endostatin decreased with improved hemodynamics over time. Endostatin was associated with worse transplant‐free survival. Addition of endostatin to an NT‐proBNP (N‐terminal pro‐B‐type natriuretic peptide) based survival analysis improved risk stratification, reclassifying subjects with adverse outcomes. Endostatin was secreted primarily by pulmonary artery endothelial cells. Conclusions Endostatin is associated with disease severity, disease improvement, and worse survival in APAH‐CHD. Endostatin with NT‐proBNP improves risk stratification, better predicting adverse outcomes. The association of elevated endostatin with shunt lesions suggests that endostatin could be driven by both pulmonary artery flow and pressure. Endostatin could be studied as a noninvasive prognostic marker, particularly in APAH‐CHD.

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