Pulmonary Artery Pressure In Children Research Articles

Non Invasive Assessment of Pulmonary Artery Pressure in Children with Portal Hypertension

Abstract Background Pulmonary artery hypertension is a complication of liver cirrhosis with Portal Hypertension (PHT). It has been mentioned in case reports or small series of children. Fewinformation known on its prevalence and long-term survival in childhood liver disease. This study will assess the Pulmonary Atery Pressure (PAP) in children with PHT in Pediatric Hepatologyclinic, Ain Shams University Children Hospital in order to identify clinical characteristics of Porto PH and its prevalence among those children. Aim of the Work To assess the PAP using Echocardiography in children with PHT who attend Pediatric Hepatology clinic, Ain Shams University Children Hospital. Patients and Methods This case-control study was conducted on thirty patients enrolled from Pediatric Hepatology clinic, Ain Shams University Children Hospital in a period of one year from June 2021 to June 2022 according to inclusion criteria : history of hematemesis and melena, clinical examination: Splenomegaly, dilated anterior abdominal wall vessels, ascites ± firm hepatomegaly and imaging: Ultrasonography (US) /color Doppler-US (CDUS): Splenomegaly, presence of Porto collateral vessels and ascites. This information will be completed with data on liver size, echo texture, and margins which can suggest underlying cirrhosis, portal vein diameter, portal blood flow velocity, direction of blood flow in portal vein with age ranging from 1 to 16 years with median (IQR) of 8 (4.5-12) years; they were 11 females (36.7%) and 19 males (63.3%) and thirty healthy matched age and sex as a control group. Results In our study, MPAP was 9.79 ± 2.21 mmHg (7.2-18.2 mmHg). Statistically, there was statistically significant increase in the level of TR velocity, TR PG, RVSP and MPAP in patients with PHT than control group with p-value <0.001, 0.001; 0.001 and <0.001; respectively. Also, there was statistically significant increase in the percentage of patients with abnormal TR PG and patients with elevated systolic PAP in patients with PHT (20.0%) than control group (0.0%) with p-value = 0.010 and 0.010; respectively. Also, there was statistically significant decrease in the level of PA acceleration time (ms) in patients with PHT than control group with p-value <0.001. Also, there was statistically significant increase in the level of PAEDP (mmHg) and LA (cm) in patients with PHT than control group with p-value <0.001 and 0.014; respectively. In our studied group of patients we did not found any of them has PH despite the higher parameters of their Echo studies and that needs follow up of those patients. So the prevalence of PH in those patients is 0%. Conclusion PHT is an important risk factor of development of PH in children. Transthoracic echocardiography can be reliably used as an initial non-invasive, safe and reliable modality for the assessment of pulmonary artery hypertension in children, and can obviate the need of right heart catheterization in some patients, especially those with mild PHT.

Explanatory deep learning to predict elevated pulmonary artery pressure in children with ventricular septal defects using standard chest x-rays: a novel approach.

Early risk assessment of pulmonary arterial hypertension (PAH) in patients with congenital heart disease (CHD) is crucial to ensure timely treatment. We hypothesize that applying artificial intelligence (AI) to chest x-rays (CXRs) could identify the future risk of PAH in patients with ventricular septal defect (VSD). A total of 831 VSD patients (161 PAH-VSD, 670 nonPAH-VSD) was retrospectively included. A residual neural networks (ResNet) was trained for classify VSD patients with different outcomes based on chest radiographs. The endpoint of this study was the occurrence of PAH in VSD children before or after surgery. In the validation set, the AI algorithm achieved an area under the curve (AUC) of 0.82. In an independent test set, the AI algorithm significantly outperformed human observers in terms of AUC (0.81 vs. 0.65). Class Activation Mapping (CAM) images demonstrated the model's attention focused on the pulmonary artery segment. The preliminary findings of this study suggest that the application of artificial intelligence to chest x-rays in VSD patients can effectively identify the risk of PAH.

Evaluation of Pre and Post-operative Pulmonary Artery Pressures in Children with Adenotonsillar Hypertrophy and Correlation with Chronicity: A Prospective Study on 50 Children.

Children with grade III or IV of adenotonsillar hypertrophy especially of a longer duration, may show cardiovascular changes due to mouth breathing. These changes can be detected on doppler echocardiocardiography which can be used as a screening tool. Fifty paediatric patients of grade 3 and 4 adenotonsillar hypertrophy underwent adenotonsillar resection after a pre-operative work up which included doppler echocardiography to measure pulmonary artery pressures. A post-operative doppler echocardiography was done after three months. 11 children (22%) showed deranged pulmonary artery pressure values with increased mean pulmonary artery pressure (MPAP) and pulmonary artery systolic pressure (PSAP) on preoperative doppler echocardiography, and 2 out of these 11 children had Pulmonary Artery Hypertension. A significant (p < 0.001) improvement was noted in pulmonary artery pressure values (MPAP and PSAP returned to normal) after adenotonsillectomy in 3-month postoperative echocardiography. Also, a statistically significant correlation was obtained between chronicity of symptoms and raised pulmonary artery pressures. Our study concluded that these children with chronic untreated upper airway obstructive symptoms get predisposed to potential cardiac complications. And these grave consequences can be easily prevented by a timely intervention.

Role of Highly Sensitive Cardiac Troponin I in the Prediction of Pediatric Pulmonary Hypertension due to Congenital Heart Disease

Background: CHD is a major health problem in pediatric population. It is associated with abnormal hemodynamic load and neurohormonal activation. Which leads to pulmonary hypertension causing myocardial injury. So, it needs timely intervention. Cardiac troponins are specific for myocardial injury. HscT I enhances accuracy for diagnosis of myocardial injury even in early stage of Pulmonary Hypertension. In recent years role of HscT I suggested to be useful in evaluation of PAH due to CHD in children. Objectives: To analyze the role of HscT I as a diagnostic tool for PAH due to CHD in Children. Methods: This prospective cross-sectional study was conducted in Pediatric cardiology department, BSMMU over a period of 1 year. The study included total 54 patients with moderate to large VSD/PDA. Patients were selected according to inclusion and exclusion criteria. Results: Total 54 cases was divided into two group PAH group (n=27) and non-PAH (n=27) group. Among PAH group 20 patient (74.1%) had VSD and 7 patient (25.9%) had PDA. In non PAH group 15 had VSD (55.6%) and 12 had PDA (44.4%). The mean troponin I level was significantly higher in PAH group than non-PAH group (0.03052±0.0511 vs. 0.0087±0.0075, p=0.03). Conclusion: In this study, there was significant difference between HscT I level of PAH group and non-PAH group and significant positive correlation between HscT I level and mean PAP. As a marker of myocardial injury, HscT I can be a predictor of raised pulmonary arterial pressure in children with post tricuspid left to right shunt.

Hemodynamic and prognostic impact of the diastolic pulmonary arterial pressure in children with pulmonary arterial hypertension-a registry-based analysis.

Diastolic pulmonary arterial pressure (dPAP) is regarded to be less sensitive to flow metrics as compared to mean PAP (mPAP), and was therefore proposed for the assessment of a precapillary component in patients with postcapillary pulmonary hypertension (PH). To analyze the diagnostic and prognostic impact of dPAP in patients with pure precapillary PH, we purposed to compare the correlation between dPAP and mPAP, as well as hemodynamically-derived calculations [ratio of PAP to systemic arterial pressure (PAP/SAP), pulmonary vascular resistance index (PVRI), transpulmonary gradient (TPG)], using both dPAP and mPAP, at rest and during acute vasoreactivity testing (AVT) in children with idiopathic or heritable pulmonary arterial hypertension (IPAH/HPAH). Furthermore, we aimed to assess the association of these metrics (at baseline and changes after AVT) with transplant-free survival. We conducted a retrospective analysis of the TOPP (Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension) registry including 246 IPAH/HPAH patients. Of these, 45 children (18.3%) died, and 13 (5.3%) received lung transplantation during the observation period. dPAP and mPAP-derived variables showed almost linear relationship. Higher mPAP/mSAP, and dPAP-/mPAP-derived PVRI at rest was associated with time to death/transplantation. At maximum AVT-response, the decrease of dPAP and mPAP, diastolic pulmonary gradient (DPG) and TPG, as well as dPAP/dSAP and mPAP/mSAP was associated with time to death/transplantation, showing higher significance than corresponding baseline values. Remarkably, no predictive value was found for PVRI-reduction during AVT, neither dPAP- nor mPAP-derived. There is a strong relationship between dPAP and mPAP-derived variables. According to our results, hemodynamics during AVT (irrespectively of dPAP- or mPAP-derived) may have more prognostic implications than resting hemodynamics in children with IPAH/HPAH, except for PVRI.

Ventricular Function and Pulmonary Artery Pressure in Children with Mild and Moderate Bronchial Asthma

Background: Bronchial asthma is a chronic airway disease. Cardiac affection in children with bronchial asthma was reported mainly in cases with severe asthma and during acute exacerbations. Aim of the Work: To study cardiac affection in children with mild and moderate bronchial asthma using conventional and tissue Doppler imaging [TDI] echocardiography. Patients and Methods: This was a prospective case control study. Study involved 160 children aged 6-14 years old who were following in outpatient pediatric clinic, Al-Hussein hospital, Al-Azhar University, Cairo, Egypt, during the period from January 4, 2016, till February 15, 2017. All children underwent peak expiratory flow rate [PEFR] assessment. Echocardiography was done for all to assess systolic and diastolic functions of right ventricle [RV] and left ventricle [LV] using conventional and tissue Doppler imaging [TDI] echocardiography. Results: TDI parameters showed evidence of RV diastolic dysfunction in patients with moderate asthma in the form of significantly lower tricuspid valve [TV] e’ [12.1 ± 2.7 cm/s], lower TV e’/a’ ratio [1.28 ± 0.18], prolonged RV isovolumetric relaxation time [IVRT] [71.8 ± 2.4 ms], and higher RV myocardial performance index [0.49 ± 0.04] compared to control group. Also, patients with mild asthma had impaired RV diastolic function in the form of significantly lower TV e’/a’ [1.51 ± 0.13] and longer RV IVRT [65 ± 2.9 ms] compared to control group. Pulmonary artery pressure was significantly elevated in moderate asthma group [18.4 ± 9.2 mmHg]. RV IVRT was the only parameter showing a significant negative correlation to PEFR Conclusion: Pediatric patients with mild and moderate bronchial asthma may have RV diastolic dysfunction. TDI is a good screening tool to detect early changes in cardiac function in such patients. RV IVRT measured by TDI is a sensitive tool to detect early diastolic impairment.

The direct comparison of inhaled versus intravenous levosimendan in children with pulmonary hypertension undergoing on-cardiopulmonary bypass cardiac surgery: A randomized, controlled, non-inferiority study

Study objectivePulmonary arterial hypertension is commonly seen in children with left to right intracardiac shunts and affects the outcomes of cardiac surgery. Our study aimed to compare the efficacy of inhaled levosimendan (LS) versus intravenous LS in reducing elevated pulmonary artery pressure (PAP) in children scheduled for cardiac surgery. DesignNon-inferiority, prospective, randomized, blinded, controlled study. SettingOperative room and intensive care unit (ICU), institutional children's hospital of Mansoura Faculty of Medicine, Egypt. Patients50 patients of either sex, aged 1 to 5 years undergoing surgical repair of intracardiac left to right shunt complicated by pulmonary hypertension were recruited for the study. InterventionsIn the intravenous LS group, patients received intravenous infusion of LS a rate of 0.1 μg/kg/min and in the inhaled LS group, LS (36 μg/kg/6 h) was delivered by nebulization. MeasurementsThe primary endpoint was systolic PAP, while the secondary endpoints were the heart rate, mean arterial blood pressure, dose of norepinephrine, time to extubation and ICU length of stay. Main resultsBoth intravenous and inhaled routes of LS similarly reduced the high systolic PAP over all time points of measurement and intravenous LS was associated with higher heart rate, lower arterial pressure and the need for a higher dose of norepinephrine than the inhaled LS. ConclusionInhalation of LS is non-inferior to intravenous LS in reducing high PAP in children who underwent on-pump cardiac surgery and it is associated with less tachycardia and hypotension with reduced need for vasoactive drugs.

Effect of acute lower respiratory tract infection on pulmonary artery pressure in children with post-tricuspid left-to-right shunt.

We sought to examine the influence of clinically severe lower respiratory tract infection on pulmonary artery pressure in children having CHD with post-tricuspid left-to-right shunt, as it may have physiological and clinical implications. In a prospective single-centre observational study, 45 children with post-tricuspid left-to-right shunt and clinically severe lower respiratory tract infection were evaluated during the illness and 2 weeks after its resolution. Pulmonary artery systolic pressure was estimated non-invasively using shunt gradient by echocardiography and systolic blood pressure measured non-invasively.Median pulmonary artery systolic pressure during lower respiratory tract infection was only mildly (although statistically significantly) elevated during lower respiratory tract infection [60 (42-74) versus 53 (40-73) mmHg, (p < 0.0001)]. However, clinically significant change in pulmonary artery systolic pressure defined as the increase of >10 mmHg was present in only 9 (20%) patients. In the absence of hypoxia or acidosis, only a small minority (9%, n = 4) showed significant pulmonary artery systolic pressure rise >10 mmHg. In the absence of hypoxia or acidosis, severe lower respiratory tract infection in patients with acyanotic CHD results in only mild elevation of pulmonary artery systolic pressure in most of the patients.

Pulmonary Vasodilator Therapy in Children with Single Ventricle Physiology: Effects on Saturation and Pulmonary Arterial Pressure

In children with single ventricle physiology, increased pulmonary vascular resistance may impede surgical progression or result in failing single ventricle physiology. The use of pulmonary vasodilators has been suggested as a potential therapy. However, knowledge on indication, dosage, and effect is limited. A retrospective case notes review of all (n = 36) children with single ventricle physiology, treated with pulmonary vasodilators by the UK Pulmonary Hypertension Service for Children 2004–2017. Therapy was initiated in Stage 1 (n = 12), Glenn (n = 8), or TCPC (n = 16). Treatment indications were high mean pulmonary arterial pressure, cyanosis, reduced exercise tolerance, protein-losing enteropathy, ascites, or plastic bronchitis. Average dose of sildenafil was 2.0 mg/kg/day and bosentan was 3.3 mg/kg/day. 56% had combination therapy. Therapy was associated with a reduction of the mean pulmonary arterial pressure from 19 to 14 mmHg (n = 17, p < 0.01). Initial therapy with one or two vasodilators was associated with an increase in the mean saturation from 80 to 85%, (n = 16, p < 0.01). Adding a second vasodilator did not give significant additional effect. 5 of 12 patients progressed from Stage 1 to Glenn, Kawashima, or TCPC, and 2 of 8 from Glenn to TCPC during a mean follow-up time of 4.7 years (0–12.8). Bosentan was discontinued in 57% and sildenafil in 14% of treated patients and saturations remained stable. Pulmonary vasodilator therapy was well tolerated and associated with improvements in saturation and mean pulmonary arterial pressure in children with single ventricle physiology. It appears safe to discontinue when no clear benefit is observed.

Prevalence and Predictors of Elevated Pulmonary Artery Pressure in Nigerian Children with Sickle Cell Anaemia

Background: Sickle Cell Anaemia (SCA) is the most common inherited disorder in Nigeria. Pulmonary Hypertension (PH) is a known complication of SCA that commences from childhood and progresses as they grow older.&#x0D; Aim: To determine the prevalence and predictors of elevated pulmonary artery pressure (PAP) in children with SCA.&#x0D; Study Design: This is an analytical cross-sectional study.&#x0D; Methods: Three hundred subjects with SCA in steady state and 300 HbAA controls, aged 6 months – 15 years, were enrolled into the study. Children with SCA were recruited by simple random sampling, from the Paediatric Sickle Cell Clinic, Usmanu Danfodiyo University Teaching Hospital, Sokoto. All the children had clinical evaluation, echocardiography, full blood count and reticulocyte count. Pulmonary artery systolic pressure (PASP) and mean pulmonary artery pressure (MPAP) were determined by measurement of tricuspid regurgitant velocity (TRV) and mean gradient (MG) respectively. &#x0D; Results: Seventy-two children with SCA (24.0%) had elevated PAP (defined as TRV ≥ 2.5 m /s) while only 9 (3.0%) had pulmonary hypertension (defined as MPAP ≥ 25 mmHg). Older age (O.R = 1.03, p &lt; 0.001), high systolic blood pressure (O.R = 1.2, p = 0.01), and high reticulocyte count (O.R = 1.33, p = 0.04) were weakly associated with increased likelihood of elevated PAP. Normal pulse oxygen saturation (SPO2 ≥ 95%, O.R = 0.4, p = 0.002) and normal heart sounds on auscultation (O.R = 0.2, p = 0.04) were associated with a reduced likelihood of elevated PAP. Presence of left parasternal heave (O.R = 8.4, p = 0.001) significantly predicted elevated PAP in children with SCA.&#x0D; Conclusion: PH occurs as a complication in children with SCA and certain clinical and laboratory parameters do predict this complication in children.

Effect of dexmedetomidine on pulmonary artery pressure in children with congenital heart disease and pulmonary hypertension.

Background:This study was undertaken to determine the effects of dexmedetomidine on pulmonary artery pressure (PAP) in children with congenital heart disease (CHD) and pulmonary hypertension (PH) undergoing cardiac catheterization with and without a planned intervention during monitored anesthetic care using midazolam and ketamine.Materials and Methods:Children (<18 years) with known CHD and PH who were scheduled for cardiac catheterization and interventional procedures were included in the study. The procedures were performed under monitored anesthesia. After obtaining baseline PAPs, an intravenous (IV) infusion of dexmedetomidine (1 μg/kg) was given for over 10 min. During infusion, heart rate (HR), blood pressure (BP), respiratory rate (RR), and peripheral arterial oxygen saturation (SPO2) were recorded every 2 min until completion of dexmedetomidine infusion, 15 min later, and when the procedure was completed. In addition, pulmonary artery systolic and diastolic pressures, and mean pulmonary artery pressure (MPAP) were recorded and the pulmonary artery systolic pressure (PASP)/systolic blood pressure (BP) ratio was calculated.Results:All children tolerated the procedure without adverse events. The HR decreased significantly over time during dexmedetomidine infusion. The changes in systemic systolic BP and PAPs were not significantly different from the baseline value at all points of measurement as was the ratio between the systolic pulmonary artery and systolic systemic BPs.Conclusions:Administration of dexmedetomidine in a dose of 1 μg/kg over 10 min did not significantly alter the PAP in children with CHD and PH. There was a decrease in the HR that was not clinically significant. The children tolerated dexmedetomidine without adverse events.

Effects of HIV Infection on Pulmonary Artery Pressure in Children

BackgroundPulmonary hypertension may complicate human immunodeficiency virus (HIV) infection and result in right ventricular (RV) failure and premature death. There are limited data of the effects of childhood HIV infection or antiretroviral therapy (ART) on pulmonary artery pressure (PAP). ObjectivesTo establish if there is an association between childhood HIV infection or its treatment and pulmonary artery pressure. MethodsThe study conducted a cross-sectional study of 102 HIV-infected (48 ART-naïve, 54 ART-exposed) and 51 HIV-uninfected children in Jakarta, Indonesia, to estimate PAP using echocardiography parameters: tricuspid regurgitation peak velocity (TRV), left ventricular systolic index and diastolic eccentricity index (EI), and RV systolic function, assessed by tricuspid annulus plane systolic excursion. The association between either ART-naive or ART-exposed HIV and PAP was explored using general linear modelling adjusted for potential confounders. ResultsART-exposed HIV-infected children had higher TRV (adjusted difference: 0.36 m/s; 95% confidence interval [CI]: 0.12 to 0.60; p = 0.003) and diastolic EI (adjusted difference 0.06; 95% CI: 0.01 to 0.11; p = 0.02) than did uninfected children. The EI in ART-exposed children was significantly higher than normal. ART-naive HIV-infected children had a lower tricuspid annulus plane systolic excursion (adjusted difference: –2.2 mm; 95% CI: –3.73 to –0.71; p = 0.004), despite no difference in TRV (adjusted difference: 0.18 m/s; 95% CI: –0.06 to 0.43 m/s; p = 0.14). Seven (13%) ART-exposed and 4 (8.3%) ART-naïve HIV-infected children had pulmonary hypertension. Within-HIV group comparisons showed that accounting for lower respiratory tract infections attenuated the lower RV systolic function in ART-naïve children but not in ART-exposed children (difference: –1.1 mm; 95% CI:–2.8 to 0.7 mm; p = 0.22), but not the higher left ventricular eccentricity indexes in the ART-exposed children (systolic difference: 0.07; 95% CI: 0.02 to 0.12; p = 0.007; diastolic difference: 0.08; 95% CI: 0.02 to 0.14; p = 0.006). ConclusionsART-exposed HIV infection is associated with higher estimated PAP. Reduced RV systolic function is seen in ART-naïve HIV infection. Lower respiratory tract infection partly explains lower systolic RV function in ART-naïve relative to ART-exposed HIV infection.

Doppler echocardiographic assessment of pulmonary artery pressure in children with sickle cell anaemia.

Pulmonary artery hypertension (PAH) is a known complication of patients with sickle cell disease (SCD). The prevalence of PAH has been reported to be high in children with SCD in developed countries. The mortality rate of patients with SCD who develop PAH is said to be 40% at 24 to 40 months after diagnosis. In Sub-Saharan Africa, particularly Nigeria, where the prevalence of SCD is high, there is a dearth of data on the prevalence of PAH in children with SCD. PAH may be a likely contributor to the unacceptably high mortality rate of children with SCD in Africa. The present study aimed to determine the pattern of pulmonary artery pressure in children with sickle cell anaemia (SCA) aged 1 to 12 years in their steady state using Doppler echocardiography in our environment. Analytical, comparative and cross-sectional study. It was carried out at Lagos State University Teaching Hospital (LASUTH) over a period of seven months (31st August 2015 through 31st March 2016). A total of 400 children, aged 1 to 12 years were recruited following parental consent and assent. Two hundred children with SCA in steady state and 200 age and sex matched children with haemoglobin genotype AA who served as controls were studied. All subjects and controls had Doppler echocardiography performed on them by the author. PAH was defined as a pulmonary artery systolic pressure (PASP) of ≥30 mmHg at rest derived from the tricuspid regurgitant velocity (TRV) measured from Doppler echocardiography. This corresponds with TRV value of ≥2.5 m/s. The prevalence of PAH in children with SCA was 8% (n=16). None of the controls had PAH. The youngest subject with PAH was aged 2 years. The overall mean pulmonary artery pressure of children with SCA was significantly higher than that of controls (18.54±5.81 vs. 13.76±5.71 mmHg, P=0.000). The prevalence of PAH in children with SCA demonstrated an increase in trend with age. The prevalence of PAH in children with SCA in steady state is high. This complication was noticed as early as the second year of life. It is recommended that all children with SCA should have periodic Doppler echocardiography for early detection of PAH.

The effects of adenotonsillar hypertrophy corrective surgery on left ventricular functions and pulmonary artery pressure in children

ObjectiveComparison of left ventricular functions in preoperative and postoperative periods of children with adenotonsillar hypertrophy (ATH) who have findings of upper airway obstruction (UAO), using echocardiographic parameters. MethodsThirty children who were diagnosed with UAO due to ATH, and who have undergone adenoidectomy/adenotonsillectomy and 30 healthy children, between 2 and 11 years of age, were included in the study. Patient group was evaluated by the pulsed wave tissue Doppler echocardiography, as well as with conventional echocardiography, before and 6 months after the operation. ResultsOf 30 children in study group, 18 (60%) had adenotonsillectomy and 12 (40%) had adenoidectomy. The differences between groups regarding myocardial performance index (MPI) was not statistically significant (p = 0.847). There was not any statistically significant difference between groups in terms of mitral isovolemic acceleration (MIVA) (2.28 ± 0.67, 2.24 ± 0.55, 2.23 ± 0.49; p = 0.943, respectively). Interventricular septum diameter (IVSD) was significantly higher in preoperative group than postoperative and control groups (3.68 ± 0.52, 3.50 ± 0.40, 3.38 ± 0.60; p = 0.028, respectively). Pulmonary acceleration time (PAcT) was found to be significantly lower in preoperative group compared to postoperative and control groups (107.64 ± 16.60, 119.52 ± 15.95, 120.47 ± 16.19; p = 0.004, respectively). Mean pulmonary arterial pressure (mPAP) was significantly higher in preoperative group than postoperative and control groups (30.58 ± 8.11, 25.23 ± 9.07, 25.00 ± 6.52; p = 0.002, respectively). In postoperative group mPAP was found to be similar to the control group. ConclusionsClinical or subclinical left ventricle (LV) dysfunction in children with ATH who have findings of UAO was not determined while mean pulmonary arterial pressure was significantly higher compared with the control cases. Besides early adenotonsillectomy is a beneficial treatment option for these patients.

Association of aortic stiffness to brain natriuretic peptide in children before and after device closure of patent ductus arteriosus

ObjectivesWe evaluated the influence of device closure for patent ductus arteriosus (PDA) on the aortic stiffness index (ASI) and brain natriuretic peptide (BNP) and their association with cardiac function. Patients and methodsASI and echocardiography assessment before and after treatment (16±9months) in 48 children with PDA (mean age 10±4.5) and 52 control children (mean age 9.7±4.6). BNP level was measured pre-closure for all children, and was measured six months after closure only for children with PDA. ResultsASI was higher in PDA patients than in controls (P<0.001). ASI correlated with age (P<0.05), LVEF% (P<0.01), E/E′ (<0.03), pulmonary artery pressure (P<0.001), and BNP (P<0.001). ASI and BNP significantly decreased after closure (P<0.001). ASI and BNP were independent predictors for post-closure systolic dysfunction (P<0.001and <0.005, respectively). Receiver operating curve (ROC) analysis showed that ASI⩾13.5, BNP level ⩾75pg/ml and basal mean pulmonary artery pressure (PAP)⩾23 were powerful predictors for post-closure systolic function. ConclusionASI is significantly associated with BNP and basal PAP in children with PDA. After device closure, aortic distensibility improved significantly and was associated with significant improvement in both systolic and diastolic functions. ASI can be used for monitoring the course of patients with PDA, and may give opportunities for early intervention.

Monitoring pulmonary artery pressure in children undergoing the Fontan procedure

To compare external and internal jugular venous pressures to monitor pulmonary artery pressure during the Fontan procedure. During the Fontan procedure, the internal jugular vein is used to assess pulmonary artery pressure. The risk of venous thrombosis in the low-pressure Fontan circuit may compromise the long-term success of cavopulmonary anastomosis. This study compared external and internal jugular venous pressures, femoral venous pressure, and actual pulmonary artery pressure during the Fontan procedure. 32 children undergoing a single-stage Fontan on cardiopulmonary bypass were monitored for mean external and internal jugular venous pressures and femoral venous pressure pre- and post-cardiopulmonary bypass. Pulmonary artery pressure was also recorded directly after cardiopulmonary bypass. The pressure data were analyzed using analysis of variance, and the coefficient of correlation was found. The differences between external jugular, internal jugular, and femoral venous pressure pre-cardiopulmonary bypass, and between the 3 venous pressures and the pulmonary artery pressure after cardiopulmonary bypass were found to be non-significant. The coefficient of correlation of external jugular venous pressure and internal jugular venous pressure was 0.8163 (p < 0.0001) pre-cardiopulmonary bypass, and 0.6465 (p = 0.0001) post-cardiopulmonary bypass. The external jugular venous pressure correlates well with both internal jugular venous pressure and femoral venous pressure as a marker of pulmonary artery pressure in children undergoing the Fontan procedure. The use of external jugular venous pressure may also preclude the risk of thrombosis.

Assessment of Pulmonary Arterial Pressure by Velocity-Encoded Cine Magnetic Resonance Imaging in Children With Congenital Heart Disease

Velocity-encoded cine magnetic resonance imaging (VEC-MRI) has recently been reported as effective for assessing not only pulmonary blood flow (Qp) but also pulmonary arterial pressure (PAP) in adults. However, there have been few reports on the usefulness of VEC-MRI for assessing PAP in children with congenital heart disease (CHD). We evaluated 34 children with CHD. Qp and systemic blood flows (Qs) were determined by cardiac catheterization and VEC-MRI. The right-to-left Qp ratio (R/L) was measured by pulmonary perfusion scintigraphy and VEC-MRI. The pulmonary-to-systemic blood pressure ratio (Pp/Ps) was determined by cardiac catheterization. The acceleration time (AcT), ejection time (ET), peak velocity (PV), acceleration volume (AcV), and maximal change in flow rate during ejection (MCFR) in the pulmonary arteries, which were standardized by body surface area, were determined by VEC-MRI. The children were divided into 2 groups according to Pp/Ps. The Qs, R/L ratio and Qp/Qs obtained by VEC-MRI strongly correlated with those obtained by catheterization and scintigraphy. No significant differences in AcT, ET, AcT/ET, PV, or AcV were observed between the 2 groups. However, a significant difference was observed in MCFR. Furthermore, a significant correlation was observed between the MCFR and Pp/Ps. This study clearly demonstrated that VEC-MRI is useful for assessing not only blood flow, but also PAP, by referring to MCFR in children.

Doppler Tissue Imaging Provides an Estimate of Pulmonary Arterial Pressure in Children with Pulmonary Hypertension Due to Congenital Intracardiac Shunts

The aim of this study is to determine the relationship between the cardiac catheterization findings and pulsed-wave (PW) Doppler and Doppler tissue imaging (DTI) in pulmonary arterial hypertension patients with congenital heart disease with intracardiac shunts. The present study aims to determine the relationship between the cardiac catheterization findings and PW Doppler and Doppler tissue imaging (DTI) in patients who have pulmonary arterial hypertension patients due to congenital heart disease with intracardiac shunts. Echocardiographic measurements were performed at the catheter angiography laboratory with concurrent catheterization. Left and right ventricle inflow velocities were recorded with PW Doppler and DTI studies. Maximum tricuspid regurgitation velocity (TS) was recorded in cases with measurable levels by continuous-wave Doppler. Moreover, the correlations among the echocardiographic values and invasive hemodynamic measures such as systolic pulmonary arterial pressure (PAPsystolic), mean pulmonary arterial pressure (PAPmean), diastolic pulmonary arterial pressure (PAPdiastolic) and pulmonary vascular resistance index (PVRI) were evaluated. A negative correlation was found between TE'/TA' and PAPsystolic, PAPdiastolic and PAPmean (P = 0.008, r = -0.480; P = 0.001, r = -0.584; P = 0.001, r = -0.567, respectively). ME/ME' was also found to be negatively correlated with PAPdiastolic, PAPmean and PVRI (P = 0.002, r = -0.556; P = 0.005, r = -0.502; P = 0.027, r = -0.411, respectively). The concurrent use of TE'/TA' (cut-off value <2.6) and TS had a sensitivity of 79% and a specificity of 93% for distinguishing between patients with healthy controls. When used in conjunction with conventional methods, TE'/TA' has the highest sensitivity and specificity in distinguishing between patients and healthy controls.

Effect of Hydroxyurea on Elevated Pulmonary Artery Pressures in Children with Sickle Cell Disease

Abstract 4841 Background:Pulmonary hypertension (PHT) is a significant complication of sickle cell disease. Studies in children report a 16–30% prevalence of elevated pulmonary artery pressures, as estimated by measurement of tricuspid regurgitant jet velocity (TRV) on echocardiography. The pathogenesis of elevated pulmonary artery pressures is multifactorial, with hemolysis induced endothelial dysfunction playing a major role. More recent studies highlight the role of inflammation in the pathogenesis. Hydroxyurea is a well established treatment for sickle cell disease. It acts primarily by induction of fetal hemoglobin, thereby reducing hemolysis, with possible additional effects on vascular and endothelial function. The aim of this study was to determine if early detection and treatment with hydroxyurea will decrease elevated pulmonary artery pressures in children with sickle cell disease. Methods:The study was conducted at 3 centers. Children with Hb SS and Hb Sb0 thalassemia between the ages of 5–21 years, with a screening echocardiogram showing a TRV ≥ 2.5 m/sec were identified. An echocardiogram was repeated to confirm elevated TRV. Subjects with persistent elevation of TRV ≥ 2.5 m/sec on repeat echocardiogram, were consented and started on hydroxyurea at 20 mg/kg/d with escalation to a maximum tolerated dose or a total daily dose of 30 mg/kg/d. Laboratory data and echocardiograms were repeated at 6 and 12 months to measure effect of hydroxyurea on TRV. Additionally blood and urine samples were also collected pre treatment, at 6 and 12 months post treatment for biomarker analysis, which will be performed later. Baseline and 6 month laboratory and echocardiogram data were compared using paired t test. Results:Twelve patients were enrolled. Mean age was 12.25 years (range 6–19 years) with a M:F ratio of 2:1. Average follow up is 11 months. Patients tolerated hydroxyurea well, and in 90% of patients the dose was escalated to 30 mg/kg/d. 1 patient achieved MTD at 20 mg/kg/d. Two patients went off study at 4 and 5 months respectively.As shown in Table 1, six months after starting hydroxyurea there was a significant increase in mean oxygen saturation, hemoglobin, mean corpuscular volume and fetal hemoglobin. There was a significant decrease in mean reticulocyte count, LDH and white blood cell count. There was no significant change in TRV six months after treatment with hydroxyurea.Table 1Patient Characteristics pre and post treatment with hydroxyureaBaseline Mean ± SD6 month Follow up Mean ± SDP valueO2 saturation94.88 ±2.0298.33 ±1.320.001Hemoglobin7.3 ± 0.728.3 ± 0.920.017Retic count14.78 ± 5.165.14 ± 1.910.005MCV81.9 ± 7.5699.63 ± 12.450.0002LDH704.5 ± 227.75411.75 ± 140.080.017WBC12.33 ± 3.337.91 ± 4.950.03Platelete count445.88 ± 69.87342.55 ± 150.170.1Fetal hemoglobin %5.76 ± 2.4516.43 ± 9.600.01TRV (m/sec)2.67 ± 0.152.79 ± 0.370.37 Conclusion:Hydroxyurea significantly decreased measures of hemolysis in children with sickle cell disease. Six months after treatment with hydroxyurea, there was no significant change in estimated pulmonary artery pressures measured on echocardiography. The study is ongoing to see if hydroxyurea affects pulmonary artery pressures with a longer duration of treatment. Disclosures:No relevant conflicts of interest to declare.

The effect of adenotonsillectomy on right ventricle function and pulmonary artery pressure in children with adenotonsillar hypertrophy

Objectives Adenotonsillar hypertrophy (ATH) is the most common cause of upper airway obstruction in children. Severe upper airway obstruction may have an effect on chronic alveolar hypoventilation, which consequently may lead to right ventricle (RV) dysfunction induced by hypoxemic pulmonary vasoconstriction. The investigators aimed to study RV function and mean pulmonary artery pressure (mPAP) in patients with ATH who were undergoing adenotonsillectomy by using tissue Doppler echocardiography (TDE). Methods The study examined 27 children with ATH who had a mean age of 8 ± 2 years. The subjects were comprised 17 (63%) males and 10 (37%) females. Hypertrophy of the tonsils was graded according to the Brodsky scale. Children having either grade 3 or 4 hypertrophied adenotonsils were recruited for the study. Adenotonsillectomy was performed on all subjects in the study group and echocardiographic examination was repeated 3 months postoperatively. Results Tricuspid Em significantly increased after adenotonsillectomy (17.7 ± 3.6 vs. 19.1 ± 5.5, p = 0.04). The RV myocardial performance index (MPI) and mPAP significantly decreased after adenotonsillectomy (RV MPI: 0.57 ± 0.13 vs. 0.40 ± 0.12, p < 0.001 and mPAP (mm Hg): 31 ± 9 vs. 25 ± 7, p = 0.001). Conclusion The results of this study, evaluated with the results of previous studies, demonstrated that adenotonsillectomy improved RV performance and reduced mPAP in children with ATH.