scholarly journals Changes in pulmonary exercise haemodynamics in scleroderma: a 4-year prospective study

2017 ◽  
Vol 50 (1) ◽  
pp. 1601708 ◽  
Author(s):  
Gabor Kovacs ◽  
Alexander Avian ◽  
Nora Wutte ◽  
Franz Hafner ◽  
Florentine Moazedi-Fürst ◽  
...  
Keyword(s):  
Cardiopulmonary Exercise Testing ◽  
Peak Oxygen Uptake ◽  
Pulmonary Vascular Disease ◽  
Significant Deterioration ◽  
End Point ◽  
Complete Dataset ◽  
Systolic Pulmonary Arterial Pressure ◽  
Right Heart Catheter ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is a feared complication of systemic sclerosis. In this prospective cohort study, we monitored the changes in resting and exercise pulmonary haemodynamics of scleroderma patients without initial PAH over a mean follow-up period of ∼4 years.All patients underwent exercise echocardiography and cardiopulmonary exercise testing at baseline and follow-up. A subgroup underwent exercise right heart catheter (RHC) investigations. The primary end-point was the echocardiographic systolic pulmonary arterial pressure at 50 W exercise (sPAP50).We included 99 patients, of whom 58 had a complete dataset. Three out of 99 patients developed RHC-confirmed PAH (0.75 cases per 100 patient-years). sPAP50 increased (p<0.001) and peak oxygen uptake (secondary end-point) decreased significantly (p=0.001) during follow-up, but there was no significant change in resting sPAP (p=0.38). In the RHC subgroup (n=28), mean (m)PAP and pulmonary vascular resistance at 50 W increased significantly (p=0.02 and p=0.002, respectively), but resting mPAP was unchanged.Scleroderma patients without PAH develop a mild but significant deterioration of pulmonary exercise haemodynamics and exercise capacity over a 4-year follow-up period, indicating a progression of pulmonary vascular disease. The manifestation rate of RHC-confirmed PAH was 0.75 cases per 100 patient-years.

scholarly journals Insights from Cardiopulmonary Exercise Testing in Pediatric Patients with Hypertrophic Cardiomyopathy

Biomolecules ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 376
Author(s):  
Giovanna Gallo ◽  
Vittoria Mastromarino ◽  
Giuseppe Limongelli ◽  
Giulio Calcagni ◽  
Antonello Maruotti ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Functional Capacity ◽  
Univariate Analysis ◽  
Cardiopulmonary Exercise Testing ◽  
Heart Association ◽  
Peak Oxygen Uptake ◽  
Cardiopulmonary Exercise Test ◽  
Cardiopulmonary Exercise ◽  
End Point

The usefulness of cardiopulmonary exercise test (CPET) in adult hypertrophic cardiomyopathy (HCM) patients is well-known, whereas its role in pediatric HCM patients has not yet been explored. The present study investigates possible insights from a CPET assessment in a cohort of pediatric HCM outpatients in terms of functional and prognostic assessment. Sixty consecutive pediatric HCM outpatients aged <18 years old were enrolled, each of them undergoing a full clinical assessment including a CPET; a group of 60 healthy subjects served as controls. A unique composite end-point of heart failure (HF) related and sudden cardiac death (SCD) or SCD-equivalent events was also explored. During a median follow-up of 53 months (25th–75th: 13–84 months), a total of 13 HF- and 7 SCD-related first events were collected. Compared to controls, HCM patients showed an impaired functional capacity with most of them showing peak oxygen uptake (pVO2) values of <80% of the predicted, clearly discrepant with functional New York Heart Association class assessment. The composite end-point occurred more frequently in patients with the worst CPETs’ profiles. At the univariate analysis, pVO2% was the variable with the strongest association with adverse events at follow-up (C-index = 0.72, p = 0.025) and a cut-off value equal to 60% was the most accurate in identifying those patients at the highest risk. In a pediatric HCM subset, the CPET assessment allows a true functional capacity estimation and it might be helpful in identifying early those patients at high risk of events.

The pulmonary vasculature in a neonatal porcine model with increased pulmonary blood flow and pressure

2001 ◽  
Vol 11 (4) ◽  
pp. 420-430 ◽  
Author(s):  
Elisabeth V. Stenbøg ◽  
Daniel A. Steinbrüchel ◽  
Anne Bloch Thomsen ◽  
Ulrik Baandrup ◽  
Lene Heickendorff ◽  
...  
Keyword(s):  
Blood Flow ◽  
Pulmonary Artery ◽  
Vascular Disease ◽  
Pulmonary Blood Flow ◽  
Left Pulmonary Artery ◽  
Pulmonary Vasculature ◽  
Pulmonary Vascular Disease ◽  
Systolic Pulmonary Arterial Pressure ◽  
Pulmonary Arterial ◽  
Circulating Levels

Introduction: Hypertension and hyperperfusion of the pulmonary vascular bed in the setting of congenital cardiac malformations may lead to progressive pulmonary vascular disease. To improve the understanding of the basic mechanisms of this disease, there is a need for clinically relevant animal models which reflect the disease process. Material and Results: We randomly allocated 45 newborn pigs, at the age of 48 hrs, to groups in which there was either construction of a 3 mm central aorto-pulmonary shunt, undertaken in 9, or ligation of the left pulmonary artery, achieved in 13. Controls included sham operations in 13, or no operations in 10 pigs. Follow-up was continued for three months. The interventions were compatible with survival in most pigs. The shunts resulted in an acute 85% increase in systolic pulmonary arterial pressure, and a more than twofold increase in pulmonary blood flow. By three months of age, nearly all shunts had closed spontaneously, and haemodynamics were normal. Ligation of the left pulmonary artery resulted in a normal total pulmonary blood flow, despite only the right lung being perfused, and a 33% increase in systolic pulmonary arterial pressure. These haemodynamic changes were maintained throughout the period of study. In both groups, histomorphometry revealed markedly increased muscularity of the intra-acinar pulmonary arteries. Circulating levels of endothelin were normal in the shunted animals, and elevated in those with ligation of the left pulmonary artery. Conclusion: In neonatal porcine models of pulmonary vascular disease, created by construction of 3 mm central aorto-pulmonary shunts and ligation of one pulmonary artery, we observed histopathological changes of the pulmonary vasculature similar to early hypertensive pulmonary vascular disease in humans. Elevated circulating levels of endothelin were associated with abnormal haemodynamics rather than abnormal pathology. These findings could be valuable for future studies on the pathogenesis of hypertensive pulmonary vascular disease associated with congenital cardiac malformations.

scholarly journals P1261DIAGNOSTIC VALUE OF TEMPORARY OCCLUSION OF ARTERIO-VENOUS HIGH FLOW FISTULA DURING RIGHT HEART CATHETERISATION IN PATIENTS WITH PULMONARY HYPERTENSION

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Eugene Zeltyn-Abramov ◽  
RUSTAM ISKHAKOV ◽  
NATALYA BELAVINA ◽  
NATALIYA KLOCHKOVA ◽  
NADIA FROLOVA
Keyword(s):  
Arterial Pressure ◽  
Pulmonary Arterial Pressure ◽  
High Flow ◽  
Right Atrial ◽  
Volume Index ◽  
Atrial Volume ◽  
Right Heart ◽  
Systolic Pulmonary Arterial Pressure ◽  
Pulmonary Arterial

Abstract Background and Aims Pulmonary hypertension (PH) is prevalent in patients with functioning high-flow arterio-venous fistula (HFAVF) and associated with congestive heart failure (CHF). The real incidence and possible causes of this phenomenon is a matter of debate. Hemodynamic effects of HFAVF is considering as one of the reasons for PH formation. The subject of study was the impact of HFAVF on selected parameters of central hemodynamics. In particular, the diagnostic relevance of test of temporary HFAVF occlusion (TTO) was evaluated during right heart catheterization (RHC). Method A total of 13 patients were enrolled: 8 - after kidney transplantation (KT) with preserved allograft function and 5 - on maintenance hemodialysis (HD). All of them demonstrated clinical presentation of CHF III-IV class (NYHA). Severe PH and diastolic disfunction (DD) were observed at a baseline: echocardiographic systolic pulmonary arterial pressure sPAP (mmHg): M=59 (SD 13), ratio of mitral early diastolic inflow velocity (E) (pulsed wave Doppler) to average of septal and lateral mitral annular early-diastolic peak velocity (e′) (tissue Doppler imaging) E/e′ M=18 (SD 5). The ones who have comorbid conditions that cause PH were excluded. All patients bore an upper arm HFAVF, flow of the AVF (Qa) measured by Doppler ultrasonography was markedly high (Qa): M = 3,8 l/min (SD 1,2), the cardio-pulmonary recirculation (Qa/CO): M = 51% (SD 13). All patients underwent RHC and TTO AVF. Echocardiography (Echo) was performed initially and on the follow up (8 weeks after AVF closure/flow reduction). Statistical analysis was performed using the STATISTICA 13 software (Wilcoxon, T-test). Results The results of RHC and Echo data are presented in tables 1, 2. As can be seen from the table data, all cases demonstrated instrumental features of high output CHF (HO CHF) in accordance with patient’s clinical status. TTO of HFAVF resulted in statistically significant decrease in CO and CI values, but no changes in PAP parameters were observed. Taking into account clinical and instrumental features of advanced CHF, HF AVF was closed in 8 patients after KT and in 2 patients on HD. 3 patients on HD underwent AVF flow reduction up to Qa not exceeding 1,1 l/min. Follow-up demonstrated complete resolution of CHF and dramatic improvement of DD, reduction in CO, CI, sPAP, volume parameters. CO, cardiac output; CI, cardiac index; sPAP, systolic pulmonary arterial pressure; meanPAP, mean pulmonary arterial pressure; RAP, mean right atrial pressure; PCWP, pulmonary capillary wedge pressure; LV EDVi, left ventricular end-diastolic volume index; LAVi, left atrial volume index; RAVi, right atrial volume index; Conclusion PH is a component of AVF-induced HO CHF and could be classified as postcapillary one. TTO confirms significant HFAVF contribution to specific changes of parameters of central hemodynamics due to HO CHF. TTO does not impact on PAP values and therefore is not valid to clarify PH genesis per se.

scholarly journals 150 Incremental value of cardiopulmonary exercise testing in intermediate-risk pulmonary arterial hypertension

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Roberto Badagliacca ◽  
Franz Rischard ◽  
Francesco Lo Giudice ◽  
Luke Howard ◽  
Silvia Papa ◽  
...  
Keyword(s):  
Risk Assessment ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Exercise Testing ◽  
Prognostic Model ◽  
Cardiopulmonary Exercise Testing ◽  
Intermediate Risk ◽  
Cardiopulmonary Exercise ◽  
Pulmonary Arterial

Abstract Aims Risk assessment in pulmonary arterial hypertension (PAH) is essential for prognostication. However, the majority of patients end-up in an intermediate risk status despite targeted-therapy, offering insufficient guidance in clinical practice. The added value of cardiopulmonary exercise testing (CPET) in this setting remains undefined. Methods and results Two independent cohorts with idiopathic PAH at intermediate risk were used to develop (n = 124) and externally validate (n = 143) the prognostic model. Risk assessment was based on the simplified version of the ESC/ERS guidelines score. The same definition of clinical worsening (CW) was used for both cohorts. Discrimination and calibration were assessed. Seventy-four derivation cohort patients experienced CW (51.2%) during a median of 34 months. Stroke volume index (SVI) and 6-min walk-distance (6MWD) were independent predictors of CW. With addition of CPET variables, SVI and VO2 peak independently improved the power of the prognostic model, determined by the integrated discrimination integral (IDI) index. ROC-derived cut-off values for SVI and VO2 peak were 34 and 14 ml/kg/min, respectively. Forty-eight validation cohort patients experienced CW (33.5%) during a median of 27 months follow-up. Different combinations of cut-off values of SVI and VO2 peak defined three meaningful groups showing good discrimination and calibration. The event-free survival rates at 1, 2, and 3 years were, respectively, 96%, 89%, and 89% for high SVI/high VO2 peak combination; 85%, 73%, and 61% for high SVI/low VO2 peak; and 80%, 70%, and 56% for low SVI/low VO2 peak. Conclusions Combinations of VO2 peak and SVI during follow-up is important in the prognostication of intermediate-risk prevalent patients with idiopathic PAH.

Abstract 17438: Misclassification of the Cause of Dyspnea by Resting Right Heart Catheterization: the Impact of Invasive Cardiopulmonary Exercise Testing

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Mário Santos ◽  
Aaron B Waxman ◽  
Julie Tracy ◽  
Fariha Khalid ◽  
Alexander R Opotowsky ◽  
...  
Keyword(s):  
Exercise Testing ◽  
Cardiopulmonary Exercise Testing ◽  
Right Heart Catheterization ◽  
Peak Exercise ◽  
Pulmonary Vascular Disease ◽  
Heart Catheterization ◽  
Right Heart ◽  
Cardiopulmonary Exercise ◽  
Pulmonary Arterial ◽  
The Impact

Introduction: Supine resting right heart catheterization (srRHC) is the standard method to differentiate pulmonary arterial hypertension (PAH) and heart failure with preserved ejection fraction (HFpEF), but most such patients complain of symptoms during exercise. We hypothesized that the upright invasive exercise phenotype of patients with unexplained exertional intolerance provides a distinct and additive perspective compared with supine resting RHC in the diagnosis of PAH and HFpEF. Methods: We reviewed results of consecutive patients with unexplained effort intolerance who underwent same day sequential srRHC and invasive cardiopulmonary exercise testing (iCPET) between March 2011 and October 2013. At rest, patients were classified with PAH if mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg and pulmonary arterial wedge pressure (PAWP) ≤ 15 mmHg; as HFpEF if PAWP > 15 mmHg; and as normal if none of the above hemodynamic criteria were met. At peak exercise, patients were categorized as exercise-induced PAH (eiPAH), exercise HFpEF (eHFpEF), normal (eNormal), or peripheral limitation according to the criteria displayed in the table. Results: Of 255 patients, 212 (83%) had normal srRHC. Of these, 46 (22%) had an abnormal iCPET result: eiPAH (n=24), eHFpEF (n=22). A resting mPAP > 18 mmHg discriminated eiPAH reasonably well (ROC AUC: 0.75; 95%CI: 0.67-0.83). Of those with abnormal srRHC, iCPET reclassified diagnosis for 16/43 (37%). Of the 30 patients who had HFpEF by srRHC, 12 (40%) had a normal cardiac hemodynamic profile during upright maximum exercise. 4 (31%) of the 13 patients with PAH at rest had no suggestion of intrinsic pulmonary vascular disease during exercise (3 eHFpEF and 1 non-cardiac limitation) with iCPET. Conclusions: In patients with exertional intolerance, iCPET reveals hemodynamic abnormalities which are overlooked with resting RHC and reclassifies a significant subset of apparent PAH and HFpEF patients by srRHC.

scholarly journals Exercise physiology in pulmonary hypertension patients with and without congenital heart disease

2018 ◽  
Vol 26 (1) ◽  
pp. 86-93 ◽  
Author(s):  
Francesca M Righini ◽  
Anna Apostolo ◽  
Pinar B Heck ◽  
Stefania Farina ◽  
Alfred Hager ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Adult Congenital Heart Disease ◽  
Cardiopulmonary Exercise Testing ◽  
Peak Oxygen Uptake ◽  
Carbon Dioxide Elimination ◽  
Pulmonary Pressure ◽  
Adult Congenital ◽  
Pulmonary Arterial

Background Cardiopulmonary exercise testing allows the assessment of integrative cardiopulmonary response to exercise. Aims The aim of the study was to better understand the exercise physiology in pulmonary arterial hypertension related to adult congenital heart disease compared to non-adult congenital heart disease patients by means of cardiopulmonary exercise testing parameters. Methods The present is a multicentre retrospective study which includes pulmonary hypertension group 1 and group 4 patients. All subjects underwent full clinical and instrumental evaluation, including cardiopulmonary exercise testing and right heart catheterization. Results One hundred and sixty-seven pulmonary hypertension patients (93 women and 74 men, 57 adult congenital heart disease and 110 non-adult congenital heart disease) were enrolled. Adult congenital heart disease patients had higher pulmonary pressure (mean pulmonary arterial pressure: 59.8 ± 19.5 mmHg vs 44.6 ± 16.5 mmHg, p < 0.001) and lower pulmonary blood flow (pulmonary blood flow: 3.3 (2.1–4.3) l/min vs 4.5 (3.8–5.4) l/min, p < 0.001). At cardiopulmonary exercise testing they had lower peak oxygen uptake/kg (12.8 ± 3.8 ml/kg/min vs 15.5 ± 4.2 ml/kg/min, p < 0.001) and higher ventilation/carbon dioxide elimination slope (53.2 (43.3–64.8) vs 44.0 (34.6–51.6), p < 0.001). When patients were paired for gender and peak oxygen uptake ( ± 1 ml/kg/min), obtaining 44 pairs, adult congenital heart disease patients had higher pulmonary pressure (mean pulmonary arterial pressure: 58.4 ± 20.2 mmHg vs 42.8 ± 16.8 mmHg, p < 0.001) and ventilation/carbon dioxide elimination slope (51.2 (43.4–63.6) vs 44.9 (35.4–55.1), p = 0.033). Conclusions In pulmonary arterial hypertension-adult congenital heart disease patients, pulmonary pressure and ventilation/carbon dioxide elimination slope are higher compared to non-adult congenital heart disease pulmonary hypertension patients, while pulmonary blood flow and peak oxygen uptake are lower. After matching patients for gender and peak oxygen uptake, pulmonary pressure and ventilation/carbon dioxide elimination remain higher in adult congenital heart disease patients suggesting that the long-term adaptation to high pulmonary pressure, hypoxia and low pulmonary blood flow, as well as a persisting shunt has, at least partially, preserved exercise performance of pulmonary arterial hypertension-adult congenital heart disease patients.

Morphologic and hemodynamic results of staged repair of pulmonary atresia with ventricular septal defect in the presence of hypoplastic pulmonary arteries and systemic-to-pulmonary collateral arteries

1993 ◽  
Vol 3 (2) ◽  
pp. 98-103
Author(s):  
James L. Wilkinson ◽  
Yin Ming Ng ◽  
Krishnan S. Iyer ◽  
Roger B. B. Mee
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Ventricular Pressure ◽  
Pulmonary Vascular Disease ◽  
Time Interval ◽  
Collateral Arteries ◽  
Pulmonary Arterial

AbstractFollow-up cardiac catheterization and angiography was performed in 23 patients after complete, staged repair of pulmonary atresia with ventricular septal defect and multiple systemic-to-pulmonary collateral arteries. Time interval from repair varied from 0.2 to 89 months (mean 26 months). Major residual anomalies of arborization of the pulmonary arteries, with or without pulmonary vascular disease, were present in six patients, two of whom had die patch on the ventricular septal defect perforated because of systemic or suprasystemic right ventricular pressure in the early postoperative period after repair. Both of these children later died, while the other four patients were found to have moderately to severely elevated ventricular pressure ratios and pulmonary arterial pressure measurements, though they are all alive at follow-up intervals from 20 months to 6.8 years. The remaining 17 patients all had hemodynamically satisfactory or good results, with pulmonary arterial mean pressures ranging from 8 to 30 mm Hg (mean 22 mm Hg) and pressure ratios between the ventricles from 0.24 to 0.75 (mean 0.43)—the higher ratios generally reflecting gradients across the conduit valve. Angiography demonstrated that the hypoplastic pulmonary arteries increased in size to normal dimensions, during staging, in most patients, though abnormalities of arborization persisted in several. Analysis of the data from the entire cohort of 58 patients, who were accepted for staging towards repair, showed a significant association between early age at entry to the program and likelihood of complete repair being achievable.

Surgical Strategy in Patients with Atrial Septal Defect and Severe Pulmonary Hypertension

2012 ◽  
Vol 15 (2) ◽  
pp. 111 ◽  
Author(s):  
Yang Hyun Cho ◽  
Tae-Gook Jun ◽  
Ji-Hyuk Yang ◽  
Pyo Won Park ◽  
June Huh ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Atrial Septal Defect ◽  
Tricuspid Regurgitation ◽  
Septal Defect ◽  
Maze Procedure ◽  
Severe Pulmonary Hypertension ◽  
Tricuspid Annuloplasty ◽  
Pulmonary Arterial ◽  
Asd Closure

The aim of the study was to review our experience with atrial septal defect (ASD) closure with a fenestrated patch in patients with severe pulmonary hypertension. Between July 2004 and February 2009, 16 patients with isolated ASD underwent closure with a fenestrated patch. All patients had a secundum type ASD and severe pulmonary hypertension. Patients ranged in age from 6 to 57 years (mean � SD, 34.9 � 13.5 years). The follow-up period was 9 to 59 months (mean, 34.5 � 13.1 months). The ranges of preoperative systolic and pulmonary arterial pressures were 63 to 119 mm Hg (mean, 83.8 � 13.9 mm Hg) and 37 to 77 mm Hg (mean, 51.1 � 10.1 mm Hg). The ranges of preoperative values for the ratio of the pulmonary flow to the systemic flow and for pulmonary arterial resistance were 1.1 to 2.7 (mean, 1.95 � 0.5) and 3.9 to 16.7 Wood units (mean, 9.8 � 2.9 Wood units), respectively. There was no early or late mortality. Tricuspid annuloplasty was performed in 14 patients (87.5%). The peak tricuspid regurgitation gradient and the ratio of the systolic pulmonary artery pressure to the systemic arterial pressure were decreased in all patients. The New York Heart Association class and the grade of tricuspid regurgitation were improved in 13 patients (81.2%) and 15 patients (93.7%), respectively. ASD closure in patients with severe pulmonary hypertension can be performed safely if we create fenestration. Tricuspid annuloplasty and a Cox maze procedure may improve the clinical result. Close observation and follow-up will be needed to validate the long-term benefits.

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