Normal Pulmonary Artery Pressure Research Articles

Echocardiographic estimation of pulmonary arterial and right atrial pressures in children with congenital heart disease: a comprehensive prospective study and introduction of novel equations

BackgroundPediatric pulmonary hypertension (PH) is characterized by a mean pulmonary arterial pressure exceeding 20 mmHg. There is limited research on the suitability of adult-based methods for estimating PH in pediatric populations. Using established formulas for adults, this study aimed to evaluate the correlation between echocardiographic estimates of systolic, diastolic, and mean pulmonary arterial pressures, and mean right atrial pressures in children with congenital heart disease (CHD).MethodsA prospective study was conducted involving children with CHD undergoing cardiac catheterization without prior cardiac surgery. We used echocardiography to estimate pulmonary and right atrial pressures and compared these with invasively measured values. Four reliable regression equations were developed to estimate systolic, diastolic, and mean pulmonary arterial pressures, and mean right atrial pressures. Cutoff values were determined to predict the occurrence of PH. Linear regression, Bland–Altman analysis, and receiver operating characteristic curve analysis were performed to assess the accuracy of echocardiography and establish diagnostic thresholds for PH.ResultsThe study involved 55 children (23 with normal pulmonary arterial pressure and 32 with PH) with acyanotic CHD aged 1 to 192 months. Four equations were developed to detect high pulmonary arterial pressures, with cutoff values of 32.9 for systolic pulmonary arterial pressure, 14.95 for diastolic pulmonary arterial pressure, and 20.7 for mean pulmonary arterial pressure. The results showed high sensitivity and moderate specificity but a tendency to underestimate systolic and mean pulmonary arterial pressures at higher pressures.ConclusionsThe study provides valuable insights into the use of adult-based echocardiographic formulas for estimating PH in pediatric patients with acyanotic CHD.

Mid-term outcomes of minimally invasive surgery for partial atrioventricular septal defect in children

Objective: This study aims to describe the mid-term outcomes of minimally invasive surgery for partial atrioventricular septal defect in children. Summary: Partial atrioventricular septal defect is one of the three types of atrioventricular septal defect, accounting for about 3.5% of congenital heart disease. Traditionally, complete repair has been performed via sternotomy. However, with the advancement of minimally invasive surgical techniques in treating congenital heart conditions, we conducted this study to assess the feasibility and mid-term outcomes of this approach. Subjects and methods: This descriptive study spanned from January 2018 to December 2023, involving 30 patients diagnosed with partial atrioventricular septal defect who underwent minimally invasive complete repair. The cohort comprised 12 males and 18 females, with an average age of 18 months and average weight of 10.3 kg. The mean diameter of the primary atrial septal defect was 15 mm. Eight patients (26.7%) exhibited moderate to severe mitral regurgitation, and the average pulmonary artery pressure was 37 mmHg. Results: The average durations of cardiopulmonary bypass and aortic cross-clamp were 82 minutes and 58 minutes, respectively. None of the patients required sternotomy. The average time of artificial ventilator post-surgery was 6.9 hours, with a mean hospital stay of 7.6 days. Postoperative echocardiography showed promising results: complete closure of atrial septal defect, with 90% of patients having absent or mild mitral valve regurgitation, only 10% experienced moderate mitral valve regurgitation. The average follow-up period was 38 months, during which there were no postoperative fatalities. One patient required reoperation due to severe mitral regurgitation from an anterior cleft. Conclusion: Minimally invasive surgery for partial atrioventricular septal defect in children can be safely performed, yielding favorable mid-term outcomes during follow-up.

Clinical Effects of Sildenafil in the Treatment of Congenital Heart Disease with Pulmonary Hypertension

Objective: To analyze the improvement of mean pulmonary arterial pressure and oxygenation index as well as the therapeutic effect of sildenafil treatment in patients with congenital heart disease and pulmonary arterial hypertension. Methods: A total of 104 patients with congenital heart disease and pulmonary arterial hypertension were recruited from February 2023 to January 2024 and grouped into study and control groups using a randomized numerical table, with 52 patients in each group. The study group received sildenafil treatment, while the control group received conventional treatment. The therapeutic effects of both groups were analyzed and compared. Results: The study group had an average pulmonary arterial pressure of 26.23 ± 2.16 mmHg, an oxygenation index of 241.63 ± 4.86, and a total effective treatment rate of 98.08%, which was significantly better than the control group (P < 0.05). Conclusion: By implementing sildenafil treatment for patients with congenital heart disease and pulmonary hypertension, the mean pulmonary artery pressure and oxygenation index of the patients improved significantly, and the effective rate of treatment increased significantly.

Current Position and Future Perspectives of Melatonin and Its Supplements in Pulmonary Hypertension

Melatonin, which is secreted principally by the pineal gland at night, affects several cardiovascular conditions including arterial hemodynamics, right ventricle functions, and pulmonary artery functions. Pulmonary hypertension is a hemodynamic and pathophysiological condition defined as an increase of average pulmonary artery pressure exceeding 20 mmHg at rest. In the light of the literature data, as discussed in this study, melatonin and its supplements may play a role in the pathogenesis and treatment of pulmonary hypertension.

Frequency of Pulmonary Hypertension and Its Associated Risk Factors in End-Stage Renal Disease (ESRD) Patients on Maintenance Hemodialysis.

Introduction Pulmonary hypertension (PH) is a recognized complication in patients with end-stage renal disease (ESRD undergoing maintenance hemodialysis (MHD).PHiscommonly foundin patients with chronic kidney disease (CKD) and ESRD.PH is associated with increased morbidity and mortality in patients with CKD. Methodology This cross-sectional study aimed to assess the prevalence of PH and its associated risk factors in MHD patients. A total of 220 ESRD patients on MHD patients at The Kidney Center, Karachi, Pakistan, aged 18-70 were included. Patients with chronic obstructive lung disease, valvular heart disease, and obstructive sleep apnea were excluded, as these conditions can be responsible for PH. PH was evaluated by echocardiography (ECHO), which was performed by a cardiologist. Results The mean age was 50.65 ± 14.4 years, with 131 (59.5%) males and 89 (40.5%) females. The average duration on hemodialysis was 5.3 ± 2.8 years. Hypertension (89.5%) and ischemic heart disease (24.1%) were prominent comorbidities. Hypertensive nephropathy (42.7%) was the leading cause of ESRD. Left ventricular hypertrophy was mild in most cases (85.5%), whereas regional wall motion abnormality (RWMA) was common (67.3%). The average pulmonary artery pressure was 35.2 ± 15.3 mmHg. Out of 220 patients, 109 patients (49.8%) of them had mild PH, nine patients (4.1%) had severe PH, and 72 patients (32.7%) had moderate PH. Associations between PH and various factors were examined. RWMA, left ventricular hypertrophy, and left ventricular ejection fraction were significantly associated with PH (p < 0.001). Serum calcium and albumin levels were also associated with PH severity (p < 0.05). Other demographic and laboratory parameters did not show a significant association. Conclusion This study highlights the prevalence of PH in MHD patients and identifies associated risk factors. Understanding these associations can aid in better managing PH in ESRD patients.

Modifiers of the Association between E/e' Ratio and Survival among Patients with No Apparent Structural or Functional Cardiac Abnormality

BackgroundThe ratio between early mitral flow wave to early diastolic mitral annulus velocity (E/e’ ratio) varies according to age and sex and is associated with mortality in heart failure. We sought to describe the association between E/e’ and mortality in patients with no apparent structural or functional cardiac abnormality and explore possible modifiers of this association. MethodsA retrospective study of 104,315 patients who underwent echocardiographic evaluation during 2009-2021 in the largest tertiary center in Israel. Patients with cancer, ventricular dysfunction, significant valvular or structural heart disease, or evidence of pulmonary hypertension were excluded. ResultsThe final analysis included 32,836 patients with a median age of 56 (43-66) years, and 13,547 (41%) were female. The median E/e’ was 8.3 (6.8-10.3), and 9,306 (28%) had an E/e’ >10. During a median follow-up of 5.7 (3.3-8.5) years, 2,396 (7.3%) individuals died. E/e’ >10 was associated with mortality (adjusted hazard ratio [HR] 1.16, 95% confidence interval [CI] 1.07-1.27, p<0.001). The mortality risk associated with E/e’ >10 was significantly higher in those aged ≤70 (HR 1.26, 95% CI 1.12-1.42, p<0.001), males (HR 1.34, 95% CI 1.19-1.49, p<0.001), a normal left ventricular mass (HR 1.13, 95% CI 1.02-1.24, p = 0.017), and pulmonary artery pressure <30 mmHg (HR 1.18, 95% CI 1.06-1.30, p = 0.003). ConclusionAn elevated E/e’ is associated with mortality, specifically in younger individuals, males, and those with a normal left ventricular mass and lower pulmonary artery pressure. This suggests that an elevated E/e’ might be a marker of subclinical risk in these subgroups. Further studies are needed to identify whether an elevated E/e' is useful in shared decision-making regarding the management of cardiovascular risk factors.

Frequency of RV Diastolic Dysfunction in Chronic Heart Failure Patients

Objectives: In this cross-sectional study we aim to assess the frequency of RVDD in chronic heart failure patients with reduced EF who visited as outpatient in a tertiary care hospital.&#x0D; Methodology: We included consecutive patients with chronic heart failure who had reduced EF. We assessed these patients for RVDD if they had normal RV systolic function and pulmonary artery pressures. The RVDD was categorized as normal, impaired relaxation, pseudonormal filling and restricted filling according to ASE guideline. RVDD grades were compared with LV systolic dysfunction and diastolic dysfunction grades.&#x0D; Results: Out of 199 patients evaluated, 66.3% (132) were male will with the mean age of 56.8 ± 12 years. The majority, 176, 88.4% of patients were with diagnosis if ICMP. The RVDD was noted in 112 (56.3%) patients out of which 30 (26.8%) had stage 1 (impaired relaxation) 78 (69.6%) had stage 2 (pseudonormal) and 4 (3.6%) patients had grade 3 (restrictive filling) RVDD. When compared a statistically significant association (P &lt; 0.001) was observed between LV and RV diastolic dysfunction.&#x0D; Conclusion: RVDD is under diagnosed entity of neglected ventricle which is significantly associated with LV systolic and diastolic dysfunction even if the RV systolic function is normal. The prognostic significance of this diastolic ventricular interaction needs to be further investigated.

Abstract 14822: Portopulmonary Hypertension Unveiled: Effects of Transjugular Intrahepatic Portopulmonary Shunt Placement

Introduction: Transjugular intrahepatic portosystemic shunts (TIPS) treat portal hypertension by connecting the portal vein to the hepatic vein in the liver. The resultant shunting of portal venous flow to the systemic circulation helps reduce the portosystemic gradient and is used to treat complications of portal hypertension. Right heart failure and the development of pulmonary hypertension after TIPS are known complications; however, portopulmonary hypertension (POPH) is rare. Description of Case: A 61-year-old male presents to the clinic for six months of exertional dyspnea, weight gain, and lower extremity edema. He has a history of coronary artery disease and nonalcoholic steatohepatitis cirrhosis, with TIPS placement four years prior for esophageal variceal bleeding. An echocardiogram revealed right ventricular (RV) dilatation with moderately reduced function, a flattened left ventricular septum, and an estimated pulmonary artery systolic pressure of 91 mmHg. An echocardiogram and right heart catheterization (RHC) performed before his TIPS showed normal RV function and pulmonary artery pressures (PAP). Decision Making: The patient was promptly started on furosemide and spironolactone, and after adequate diuresis, a repeat RHC was obtained, which showed significantly elevated PAP, and normal left-sided filling pressures and cardiac output. Other causes of pulmonary hypertension were ruled out, and the patient was started on tadalafil monotherapy for severe POPH, with significant improvement in his PAP (Table 1). Conclusion: POPH after TIPS is a rare complication that should be suspected in patients presenting with signs of right-sided volume overload. The mechanism by which TIPS leads to POPH is not entirely understood. The degree of RV dysfunction correlates with mortality, and prompt treatment is warranted.

The association of Pulmonary Hypertension and right ventricular systolic function – updates in diagnosis and treatment

Right ventricular (RV) systolic function is an essential but neglected component in cardiac evaluation, and its importance to the contribution to overall cardiac function is undermined. It is not only sensitive to the effect of left heart valve disease but is also more sensitive to changes in pressure overload than the left ventricle. Pulmonary Hypertension is the common and well-recognized complication of RV systolic dysfunction. It is also the leading cause of pulmonary valve disease and right ventricular dysfunction. Patients with a high pulmonary artery pressure (PAP) and a low RV ejection fraction have a seven-fold higher risk of death than heart failure patients with a normal PAP and RV ejection fraction. Furthermore, it is an independent predictor of survival in these patients. In this review, we examine the association of right ventricular systolic function with Pulmonary Hypertension by focusing on various pathological and clinical manifestations while assessing their impact. We also explore new 2022 ESC/ERS guidelines for diagnosing and treating right ventricular dysfunction in Pulmonary Hypertension.

Monocrotaline model of Pulmonary hypertension in immature rats from the perspective of Serotonergic regulation

Pulmonary hypertension (PH) is a multifactorial disease characterized by an average pulmonary artery pressure of 25mmHg or higher. Although PH is generally classified into five distinct groups, pulmonary arterial hypertension (PAH), especially the idiopathic subgroup (IPAH) or congenital heart disease (CHD) subgroup, has been the focus of most of our research from a serotonergic regulatory perspective. There are many models of pulmonary hypertension in experimental practice. Each model aim at certain goals and is based on various mechanisms. The monocrotaline model of pulmonary hypertension is one of the best models for studying pulmonary hypertension due to endothelial dysfunction. However, in experimental practice, this model is used only for adulthood. At present, the influence of the serotonergic system is not taken into account in the treatment of children with pulmonary hypertension. We have modified the monocrotaline model of pulmonary hypertension for immature rats. A positive correlation was found between the concentration of serotonin metabolites in the urine and the degree of pulmonary hypertension, which can become a potential marker of pulmonary hypertension.

Evaluating the role of serum uric acid in the risk stratification and therapeutic response of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD).

Background: Pulmonary arterial hypertension (PAH) is a malignant pulmonary vascular disease that negatively impacts quality of life, exercise capacity, and mortality. This study sought to investigate the relationship between serum uric acid (UA) level and the disease severity and treatment response of patients with PAH and congenital heart disease (PAH-CHD). Methods: This study included 225 CHD patients and 40 healthy subjects. Serum UA was measured in all patients, and UA levels and haemodynamic parameters were re-evaluated in 20 patients who had received PAH-specific drug treatment for at least 7 ± 1month. Results: Serum UA levels were significantly higher in PAH-CHD patients than in CHD patients with a normal pulmonary artery pressure and normal subjects (347.7 ± 105.7μmol/L vs. 278.3 ± 84.6μmol/L; 347.7 ± 105.7μmol/L vs. 255.7 ± 44.5μmol/L, p < 0.05). UA levels in the intermediate and high risk groups were significantly higher than those in the low-risk group (365.6 ± 107.8μmol/L vs. 311.2 ± 82.8μmol/L; 451.6 ± 117.6μmol/L vs. 311.2 ± 82.8μmol/L, p < 0.05). Serum UA levels positively correlated with mean pulmonary arterial pressure, WHO functional class, pulmonary vascular resistance, and NT-proBNP (r = 0.343, 0.357, 0.406, 0.398; p < 0.001), and negatively with mixed venous oxygen saturation (SvO2) and arterial oxygen saturation (SaO2) (r = -0.293, -0.329; p < 0.001). UA significantly decreased from 352.7 ± 97.5 to 294.4 ± 56.8μmol/L (p = 0.001) after PAH-specific drug treatment for at least 6months, along with significant decreases in mean pulmonary arterial pressure and pulmonary vascular resistance and increases in cardiac index and mixed SvO2. Conclusion: Serum UA can be used as a practical and economic biomarker for risk stratification and the evaluation of PAH-specific drug treatment effects for patients with PAH-CHD.

Right Ventricle Systolic Dysfunction: A Rare Cause of Right-To-Left Interatrial Shunt with Normal Pulmonary Artery Pressure

There is little information about right-to-left shunting with normal pulmonary artery pressure, which is produced as a consequence of right ventricle systolic dysfunction. We presented a case of unexpected transesophageal echocardiography findings of right-to-left shunting through an atrial septal defect despite normal pulmonary artery pressure, related to right ventricle systolic dysfunction in a 27-year-old young woman. Transesophageal echocardiography imaging revealed right ventricular systolic dysfunction and normal pulmonary artery pressure.

Clinical characteristics of patients with a risk of pulmonary artery hypertension secondary to ARDS in a high-altitude area

BackgroundHypoxaemia plays an important role in the development of pulmonary artery hypertension (PAH). Patients with acute respiratory distress syndrome (ARDS) in a high-altitude area have different pathophysiological characteristics from those...

Resting pulmonary function and artery pressure and cardiopulmonary exercise testing in chronic heart failure patients in Taiwan − a prospective observational cross-sectional study comparing healthy subjects and interstitial lung disease patients

Background Restrictive ventilatory defects and elevated pulmonary artery pressure (PAP) are common in patients with chronic heart failure (CHF) and those with interstitial lung disease (ILD). However, as oxyhemoglobin desaturation seldom occurs in stable CHF patients at peak exercise, we hypothesized that the pathophysiology may be different between them. This study aimed to investigate: (1) PAP and lung function at rest, (2) pulmonary gas exchange (PGX) and breathing patterns at peak exercise, (3) mechanisms of dyspnea at peak exercise in patients with CHF compared to healthy subjects and ILD patients. Methods We consecutively enrolled 83 participants (27 with CHF, 23 with ILD, and 33 healthy controls). The CHF and ILD groups had similar functional status. Lung function and cardiopulmonary exercise tests with Borg Dyspnea Score were performed. PAP was estimated using echocardiography. Resting lung function, PAP and peak exercise data in the CHF group were compared to the healthy and ILD groups. Correlation analysis was performed to elucidate the mechanisms of dyspnea in the CHF and ILD groups. Results Compared to the healthy group, the CHF group had normal lung function, PAP at rest, and normal dyspnea score and PGX at peak exercise, whereas the ILD group had abnormal values compared to the CHF group. Dyspnea score was positively correlated with pressure gradient, lung expansion capabilities, and expiratory tidal flow in the CHF group (all p < 0.05), but inversely correlated with inspiratory time-related variables in the ILD group (all p < 0.05). Conclusion Normal lung function and PAP at rest, and dyspnea scores and PGX at peak exercise indicated that pulmonary hypertension and fibrosis were insignificant in the patients with CHF. The factors affecting dyspnea at peak exercise were different between the CHF and ILD groups. As the sample size in this study was small, large-scale studies are warranted to confirm our findings.

A Patient with Right-to-Left Shunt Atrial Septal Defect Without Pulmonary Hypertension

We present a case of atrial septal defect (ASD) in a 27-year-old young woman who presented with a right-to-left shunt despite normal pulmonary artery pressure without anomalous pulmonary venous drainage and only mild tricuspid regurgitation (TR), but it was associated with right ventricular dysfunction. Considering the dysfunction of the right heart and significant dilatation of the tricuspid annulus, the surgeon still decided to repair ASD and perform tricuspid valvuloplasty. The surgery lasted approximately three hours, and the whole procedure was smooth and uneventful. Her postoperative recovery was uneventful and discharged in good condition. The occurrence of right-to-left (R-L) shunting in patients with ASD is uncommon and is usually an ominous sign, heralding irreversible pulmonary hypertension (PH). There is little information about similar shunts for patients without PH and tricuspid regurgitation (TR), and the majority of these patients have an anatomic anomaly that favors this type of shunt. ASD with R-L shunt without Eisenmenger physiology is rarely reported and may be underestimated due to difficulty in its diagnosis. Intraoperative transesophageal echocardiography (TEE) examination should not only focus on cardiac structure but also the evaluation of ventricular function. Right-to-left shunt is possible in ASD patients even with normal pulmonary arterial pressure.

PO-03-023 OUTCOMES OF LEFT BUNDLE BRANCH AREA PACING AND ATRIOVENTRICULAR NODE ABLATION IN PATIENTS WITH GROUP III PULMONARY HYPERTENSION AND ATRIAL FIBRILLATION IN REDUCING RECURRENT HOSPITALIZATION

Data on the feasibility of left bundle branch area pacing (LBBAP) and atrioventricular node (AVN) ablation in patients with pulmonary hypertension (PH) are limited. This study investigated the characteristics and outcomes of LBBAP and AVN ablation in patients with PH. This is a retrospective study of patients with PH and AF who underwent LBBAP and AVN ablation at the Cleveland Clinic between January 1, 2019 and August 1, 2022. Pre-procedural, post-procedural, and follow-up patient data were obtained. A total of 11 patients (mean age 74±10 years old, 100% female) with PH and AF who underwent concomitant LBBAP and AVN ablation were included. The etiology of PH was Group III PH in all patients (55% had COPD and 91% had restrictive lung disease), with the average pulmonary arterial systolic pressure (PASP) of 42 mmHg by echocardiogram, FEV1 of 61.9±17.4% predicted, and FVC of 59.9±7.8% predicted. Baseline ejection fraction was 57±10%, QRS duration was 96±20 ms. Concomitant LBBAP and AVN ablation was successful in all patients. Final implant characteristics included: QRS duration was 124±8 ms, left ventricular activation time was 73.7±5.4 ms; threshold 0.6±0.2 mV @ 0.4 ms and was 0.7±0.2 mV @ 0.4 ms on follow-up; impedance was 618±119 ohms and was 545±145 ohms on follow-up. Prior to the LBBAP and AVN ablation procedure, the average number of AF or heart failure (HF) hospitalizations 24 months prior to the procedure was 4.9±5.3, 64% of patients were on antiarrhythmic drugs, and 100% were on either beta-blockers or calcium channel blockers. During a mean follow-up of 21.9±15.3 months after the LBBAP and AVN ablation procedure, the average AF or HF hospitalization was 0.4±0.9, and all patients were no longer on any antiarrhythmic drugs, beta-blockers, or calcium channel blockers. There were no cases of lead dislodgement. In patients with elevated PA systolic pressure, successful LBBAP and AVN ablation with good two-year outcomes are achievable.Tabled 1Pre LBBAP and AVN ablation*Post LBBAP and AVN ablation**P-valueAF or HF hospitalizations4.9 ± 5.30.4 ± 0.9P<0.01On antiarrhythmic drugs7 (63.6%)0 (0%)P<0.01On rate control drugs11 (100%)0 (0%)NALead dislodgement-0 (0%)NALead capture threshold, mV-0.7 ± 0.2NAValues are mean ± SD or n (%) *24 months prior to LBBAP and AVN ablation **Average follow-up of 22±15 months after LBBAP and AVN ablation Open table in a new tab

Mean pulmonary artery pressure estimated by echocardiogram rapidly exceeds 20mmHg from the normal range in patients with connective tissue disease.

Earlier intervention for pulmonary hypertension (PH) has been reported to improve the prognosis of patients with connective tissue disease (CTD). However, it is not fully elucidated how rapidly PH develops in patients showing normal mean pulmonary arterial pressure (mPAP) at the index investigation. We evaluated 191 CTD patients with normal mPAP retrospectively. The mPAP was estimated by the formerly defined method employing echocardiography (mPAPecho). We investigated predictive factors that predict increasing mPAPecho at follow-up transthoracic echocardiography (TTE) using uni- and multi variable analysis. The mean age was 61.5years old, and 160 patients were female. The percentage of patients in whom mPAPecho exceeded 20mmHg at follow-up TTE was 38%. Multivariable analysis revealed that acceleration time/ejection time (AcT/ET) measured at the right ventricular outflow tract at initial TTE was independently associated with the consequent increase of mPAPecho at the follow-up TTE. When using 0.43 of best cutoff value in AcT/ET calculated by receiver operating characteristic analysis, the change in mPAPecho in patients with low AcT/ET was significantly higher than in those with high AcT/ET (3.05mmHg in patients with AcT/ET < 0.43 and 1.00mmHg in patients with AcT/ET ≥ 0.43, p < 0.001). Thirty-eight percent of CTD patients who show the normal estimated mPAP by TTE develop gradual elevation of mPAP to the level to consider early intervention within 2years. AcT/ET at initial TTE can predict increasing mPAP at follow-up TTE.

Procedural Sedation in Congenital Heart Disease

AbstractProcedural sedation in patients with congenital heart disease (CHD) is associated with significant morbidity and mortality. It is vital for the practitioner to fully understand the complexity of lesions, their hemodynamics, and the impact of medications commonly used for procedural sedation on the stability of systemic vascular resistance and pulmonary flow. According to the literature, we explain the interaction of the systemic vascular resistance and pulmonary flow in such lesions and divide them into five categories outlined in this article: (1) CHDs with left-to-right shunt with normal pulmonary arterial pressure and resistance, (2) CHD with left-to-right shunt and moderate to severe elevation of pulmonary arterial pressure with near-normal pulmonary vascular resistance, (3) CHD with pulmonary flow dependent on systemic vascular resistance, (4) patients with congenital coronary stenosis and coronary anomalies, and 5) aortic obstructive lesions.

Echocardiographic Markers of Mild Pulmonary Hypertension are not Correlated with Worse Respiratory Outcomes in Infants with Bronchiolitis.

Pulmonary hypertension has been reported as a crucial factor in the pathophysiology of severe bronchiolitis. The aim of this study was to evaluate pulmonary artery pressure (PAP) in patients with bronchiolitis and to analyze their correlation with clinical outcomes. This prospective cohort study examined children admitted for bronchiolitis. PAP was assessed by right ventricle (RV) acceleration/ejection time ratio (AT/ET), isovolumic relaxation time, eccentricity index, and the presence of a pulmonary systolic notch. Pulmonary hypertension (PH) was considered if at least two altered parameters were present. Severity of clinical course was established by higher N-terminal (NT)-prohormone BNP (NT-proBNP) values, the need for positive pressure respiratory support (PPRS), and the duration of hospital admission. One hundred sixty-nine children were included in analysis. Sixty-eight patients (40%) required PPRS, and those patients had increased NT-proBNP values and worse tricuspid annular systolic excursion (TAPSE) compared to mild cases (p < 0.001and p < 0.001, respectively). Twenty-two (13%) cases had at least two altered parameters of PAP and met criteria for presumed PH, with no differences in NT-proBNP values, TAPSE, need for PPRS or hospital length of stay compared to normal PAP group (p = 0.98, p = 0.07, p = 0.94 and p = 0.64, respectively). We found no correlation between altered RV AT/ET and worse cardiac function, NT-proBNP values or hospital length of stay. In our cohort, the presence of echocardiographic findings of PH were not associated with worse clinical outcomes. Patients with severe bronchiolitis had higher values ​​of NT-proBNP but, interestingly, no clear association with PH.

Pulmonary arterial compliance in patients of CHD with increased pulmonary blood flow.

Pulmonary arterial compliance, the dynamic component of pulmonary vasculature, remains inadequately studied in patients with left to right shunts. We sought to study the pulmonary arterial compliance in patients with left to right shunt lesions and its utility in clinical decision-making. In this single-centre retrospective study, we reviewed cardiac catheterisation data of consecutive patients of left to right shunt lesions catheterised over one year. In addition to the various other parameters, pulmonary arterial compliance was calculated, as indexed pulmonary flow (Qpi) / (Heart rate × pulse pressure in the pulmonary artery). RC time was also calculated, as the product of pulmonary arterial compliance and pulmonary vascular resistance index. Patients were divided into "operable," "borderline," and "inoperable" based on the decision of the treating team, and the pulmonary arterial compliance values were evaluated in these groups to study if it can be utilised to refine the operability decision. 298 patients (Median age 16 years, 56% <18 years) with various acyanotic shunt lesions were included. Overall, the pulmonary arterial compliance varied with Qpi, pulmonary artery mean pressure, and pulmonary vascular resistance index, but did not vary with age, type of lesion, or transpulmonary gradients. The median pulmonary arterial compliance in patients with normal pulmonary artery pressure (Mean pulmonary artery pressure less than 20 mmHg) was 4.1 ml/mmHg/m2 (IQR 3.2). The median pulmonary arterial compliance for operable patients was 2.67 ml/mmHg/m2 (IQR 2.2). Median pulmonary arterial compliance was significantly lower in both inoperable (0.52 ml/mmHg/m2, IQR 0.34) and borderline (0.80 ml/mmHg/m2, IQR 0.36) groups when compared to operable patients (p < 0.001). A pulmonary arterial compliance value lower than 1.18 ml/mmHg/m2 identified inoperable patients with high sensitivity and specificity (95%, AUC 0.99). However, in borderline cases, assessment by this value did not agree with empirical clinical assessment.The median RC time for the entire study population was 0.47 S (IQR 0.30). RC time in operable patients was significantly lower than that in the inoperable patients (Median 0.40 IQR 0.23 in operable, 0.73 0.25 in inoperable patients (p < 0.001). Addition of pulmonary arterial compliance to the routine haemodynamic assessment of patients with shunt lesions may improve our understanding of the pulmonary circulation and may have clinical utility.