scholarly journals Cutaneous Iontophoresis of Vasoactive Medications in Patients with Scleroderma- Associated Pulmonary Arterial Hypertension

2021 ◽  
Author(s):  
Sami Al Ampnti ◽  
Allaa Almoushref ◽  
Tawfeq Naal ◽  
Celia Melillo ◽  
Kulwant S. Aulak ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Reactive Hyperemia ◽  
Stroke Volume Index ◽  
Screening Tests ◽  
Volume Index ◽  
Heart Catheterization ◽  
Doppler Flowmetry ◽  
Microvascular Response ◽  
Pulmonary Arterial

Abstract Background:It remains unknown whether the cutaneous microvascular responses are different between patients with scleroderma-associated pulmonary arterial hypertension (SSc-PAH) and SSc without pulmonary hypertension (PH).Methods:We included 59 patients with SSc between March 2013 and September 2019. We divided patients into 4 groups: a) no PH by right heart catheterization (RHC) (n=8), b) no PH by noninvasive screening tests (n=16), c) treatment naïve PAH (n=16) and d) PAH under treatment (n=19). Microvascular studies using laser Doppler flowmetry (LDF) were done immediately after RHC or at the time of an outpatient clinic visit (group b). Results:The median (IQR) age was 59 (54-68) years, and 90% were females. The responses to thermal and post occlusive reactive hyperemia, acetylcholine, and sodium nitroprusside iontophoresis were similar among groups. The microvascular response to treprostinil was more pronounced in SSc patients without PH by screening tests (% change: 340 (214-781)) compared with SSc-PAH (naïve + treatment) (Perfusion Units (PU) % change: 153 (94-255) % [p=0.01]). The response to A-350619 (a sGC activator) was significantly higher in patients with SSc without PH by screening tests (PU % change: 168 (46-1,296)) than those with SSc-PAH (PU % change: 22 (15-57) % [p=0.006]). The % change in PU with A350619 was directly associated with cardiac index and stroke volume index (R: 0.36, p=0.03 and 0.39, p=0.02, respectively). Conclusions:Patients with SSc-PAH have a lower cutaneous microvascular response to a prostacyclin analog treprostinil and the sGC activator A-350619 when compared with patients with SSc and no evidence of PH on screening tests.

Pulmonary Arterial Hypertension in Systemic Sclerosis Is Associated with Profound Impairment of Microvascular Endothelium-dependent Vasodilatation

2011 ◽  
Vol 39 (1) ◽  
pp. 100-105 ◽  
Author(s):  
HERMAN M.A. HOFSTEE ◽  
ALEXANDRE E. VOSKUYL ◽  
ANTON VONK NOORDEGRAAF ◽  
YVO M. SMULDERS ◽  
PIET E. POSTMUS ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Heart Catheterization ◽  
Microvascular Function ◽  
Heart Catheterization ◽  
Microvascular Endothelium ◽  
Right Heart ◽  
Doppler Flowmetry ◽  
Pulmonary Arterial

Objective.Impaired microvascular function may contribute to organ complications in patients with systemic sclerosis (SSc). We investigated whether SSc patients with and without pulmonary arterial hypertension (PAH) show a graded impairment of microvascular function compared to healthy controls.Methods.Twenty-two patients with SSc and 22 controls were studied. All patients underwent right heart catheterization; 6 had no PAH (SSc-nonPAH) and 16 had PAH (SSc-PAH). Acetylcholine (ACh)-mediated endothelium-dependent vasodilatation and sodium nitroprusside (SNP)-mediated endothelium-independent vasodilatation were assessed by iontophoresis combined with laser Doppler flowmetry.Results.Compared to sex- and age-matched controls, ACh-mediated vasodilatation was reduced in SSc-PAH (340.4% vs 79.5%, respectively; p < 0.01), but not in SSc-nonPAH (340.4% vs 397.9%; p = 0.90). No significant differences were present between the groups in SNP-mediated vasodilatation.Conclusion.Systemic microvascular endothelium-dependent vasodilatation is markedly reduced in SSc complicated by PAH.

scholarly journals Nitric Oxide Stroke Volume Index as a New Hemodynamic Prognostic Parameter for Patients with Pulmonary Arterial Hypertension

2020 ◽  
Vol 9 (9) ◽  
pp. 2939
Author(s):  
Karolina Barańska-Pawełczak ◽  
Celina Wojciechowska ◽  
Mariusz Opara ◽  
Wojciech Jacheć
Keyword(s):  
Nitric Oxide ◽  
Pulmonary Arterial Hypertension ◽  
Confidence Interval ◽  
Stroke Volume ◽  
Arterial Hypertension ◽  
Lung Transplantation ◽  
Stroke Volume Index ◽  
Volume Index ◽  
Pulmonary Arterial

The aim of the study was to determine the prognostic value of hemodynamic parameters measured during initial diagnostic right heart catheterization (RHC) in standard conditions and using a nitric oxide reversibility test. A retrospective observational study of 62 patients with pulmonary arterial hypertension (PAH) was performed. Clinical, biochemical, echocardiographic, and hemodynamic data obtained at the time of the PAH diagnosis were precisely analyzed. Patients were followed for five years. Death or lung transplantation was considered as a primary endpoint. The mean follow-up period was 1090 ± 703 days and the median age was 46.84 years. In the studied group, 25 patients survived, 36 patients died, and one underwent a lung transplantation. From all the examined parameters, only stroke volume index during reversibility test with iNO (SVI(NO test)) (HR = 0.910; 95% confidence interval 0.878–0.944; p < 0.001) and initial arterial oxygen saturation (SaO2) (HR = 0.910; 95% confidence interval 0.843–0.982; p = 0.015) have been established as independent predictors of death or lung transplantation in the five-year follow–up. An SVI(NO test) value above 39.86 mL/m2 was associated with 100% five-year survival rate (AUC = 0.956; 95% confidence interval 0.899–1.000; p < 0.001; specificity/sensitivity: 100/84%). The results of the analysis suggest that the SVI(NO test) measured during the initial diagnostic RHC could be a very valuable prognostic factor in the PAH patients.

scholarly journals Exercise cardiac MRI unmasks right ventricular dysfunction in acute hypoxia and chronic pulmonary arterial hypertension

2018 ◽  
Vol 315 (4) ◽  
pp. H950-H957 ◽  
Author(s):  
Shareen Jaijee ◽  
Marina Quinlan ◽  
Pawel Tokarczuk ◽  
Matthew Clemence ◽  
Luke S.G.E. Howard ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Ejection Fraction ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Stroke Volume Index ◽  
Volume Index ◽  
Contractile Reserve ◽  
Control Subjects ◽  
Healthy Control ◽  
Pulmonary Arterial

Coupling of right ventricular (RV) contractility to afterload is maintained at rest in the early stages of pulmonary arterial hypertension (PAH), but exercise may unmask depleted contractile reserves. We assessed whether elevated afterload reduces RV contractile reserve despite compensated resting function using noninvasive exercise imaging. Fourteen patients with PAH (mean age: 39.1 yr, 10 women and 4 men) and 34 healthy control subjects (mean ageL 35.6 yr, 17 women and 17 men) completed real-time cardiac magnetic resonance imaging during submaximal exercise breathing room air. Control subjects were then also exercised during acute normobaric hypoxia (fraction of inspired O2: 12%). RV contractile reserve was assessed by the effect of exercise on ejection fraction. In control subjects, the increase in RV ejection fraction on exercise was less during hypoxia ( P = 0.017), but the response of left ventricular ejection fraction to exercise did not change. Patients with PAH had an impaired RV reserve, with half demonstrating a fall in RV ejection fraction on exercise despite comparable resting function to controls (PAH: rest 53.6 ± 4.3% vs. exercise 51.4 ± 10.7%; controls: rest 57.1 ± 5.2% vs. exercise 69.6 ± 6.1%, P < 0.0001). In control subjects, the increase in stroke volume index on exercise was driven by reduced RV end-systolic volume, whereas patients with PAH did not augment the stroke volume index, with increases in both end-diastolic and end-systolic volumes. From baseline hemodynamic and exercise capacity variables, only the minute ventilation-to-CO2 output ratio was an independent predictor of RV functional reserve ( P = 0.021). In conclusion, noninvasive cardiac imaging during exercise unmasks depleted RV contractile reserves in healthy adults under hypoxic conditions and patients with PAH under normoxic conditions despite preserved ejection fraction at rest. NEW & NOTEWORTHY Right ventricular (RV) reserve was assessed using real-time cardiac magnetic resonance imaging in patients with pulmonary arterial hypertension and in healthy control subjects under normobaric hypoxia, which has been previously associated with acute pulmonary hypertension. Hypoxia caused a mild reduction in RV reserve, whereas chronic pulmonary arterial hypertension was associated with a marked reduction in RV reserve.

scholarly journals PROGNOSTIC VALUE OF STROKE VOLUME INDEX IN PATIENTS WITH PULMONARY ARTERIAL HYPERTENSION AT INTERMEDIATE RISK

2020 ◽  
Vol 75 (11) ◽  
pp. 2098
Author(s):  
Fabio Dardi ◽  
Massimiliano Palazzini ◽  
Elisa Zuffa ◽  
Daniele Guarino ◽  
Alessandro De Lorenzis ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Stroke Volume ◽  
Arterial Hypertension ◽  
Prognostic Value ◽  
Stroke Volume Index ◽  
Volume Index ◽  
Intermediate Risk ◽  
Pulmonary Arterial

scholarly journals SAT0240 THE PROGNOSIS OF TWO DISTINCT CLINICAL PHENOTYPES OF SLE-PAH

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1063.2-1063
Author(s):  
J. Wang ◽  
Y. Feng ◽  
Y. Lei ◽  
X. Zhang
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lupus Erythematosus ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Systemic Lupus ◽  
Clinical Phenotypes ◽  
Weighted Score ◽  
Pulmonary Arterial

Background:Based on the characteristics of systemic lupus erythematosus-associated pulmonary arterial hypertension (SLE-PAH), Sunet alhas put forward a scoring system to distinguish two clinical phenotypes as vasculitic and vasculopathic subtypes[1]. A weighted score ≥2 suggested a vasculitic subtype by combining two factors: The time interval between SLE and PAH diagnosis <2 years and ≥2 years were 1 and 0 point; SLE Disease Activity Index (SLEDAI) >9, 5-9 and <5 were 2, 1, 0 point, respectively. While the vasculitic subtype seemed to have poorer prognosis in Sun’s research, other study has shown controversial result[2].Objectives:To find out the prognosis of two distinct clinical phenotypes of SLE-PAH.Methods:Between 2008 and 2019, a SLE-PAH cohort confirmed by right heart catheterization (RHC) from Guangdong Provincial People’s Hospital was included. Other groups of pulmonary hypertension were excluded. Based on the scoring system, patients were divided into vasculitic (weighted score≥2) and vasculopathic subtypes (weighted score<2). The endpoint was PAH-related mortality. Survival status were confirmed by clinic follow-up data or phone call.Results:A total of 53 SLE-PAH patients were enrolled. The cases of vasculitic and vasculopathic subtype were 14 and 39, respectively. Ten endpoint events occurred. Eight attributed to PAH and the cause could not be traced in two which were still included in study. The pooled 1-, 3-, 5-year survival rates were 85.7%, 78.6%, 65.5% in vasculitic subtype, and 93.9%, 87.5%, 87.5% in vasculopathic subtype, respectively. Kaplan-Meier analysis showed vasculitic subtype tended to have a poorer prognosis than vasculopathic subtype (p=0.16, HR 2.4, 95%CI 0.5-13.8, figure 1).Figure 1.Survival curves for patients with systemic lupus erythematosus-pulmonary arterial hypertension (SLE-PAH) in two distinct subtypes. RHC, Right Heart Catheterization.Conclusion:The prognosis of the two phenotypes of SLE-PAH was statistically indifferent while the vasculitic subtype showed a trend of worse prognosis. Further studies are needed.References:[1]F. Sun, Y. Lei, W. Wu, L. Guo, K. Wang, Z. Chen, W. Xu, X. Wang, T. Li, X. Zhang, S. Ye, Two distinct clinical phenotypes of pulmonary arterial hypertension secondary to systemic lupus erythematosus, Ann Rheum Dis 78(1) (2019) 148-150.[2]J. Qian, M. Li, J. Zhao, Q. Wang, Z. Tian, X. Zeng, Inflammation in SLE-PAH: good news or not?, Ann Rheum Dis (2018).0:1–2. doi:10.1136/annrheumdis-2018-214605Disclosure of Interests:None declared

Abstract 16667: Computed Tomography-Based Pulmonary Vascular Tortuosity as a Marker in Resting and Exercise Pulmonary Arterial Hypertension

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Eileen M Harder ◽  
Pietro Nardelli ◽  
Gonzalo Sanchez-Ferrero ◽  
James Ross ◽  
Sam Y Ash ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Early Form ◽  
Arterial Tortuosity ◽  
Vascular Tortuosity ◽  
Measure Analysis ◽  
Pulmonary Arterial

Introduction: Increased vascular tortuosity has been proposed as a marker of pulmonary arterial hypertension (PAH). In this analysis, we compared arterial and venous vascular tortuosity between controls and subjects with resting PAH. Furthermore, we examined if abnormalities could be detected in exercise PAH (EPAH), thought to be an early form of PAH. Methods: From an institutional registry, 388 patients with both right heart catheterization and computed tomography angiography (CTA) data were selected. Within this cohort, three distinct groups were identified: 1) controls, who had no cardiopulmonary disease and normal resting and exercise hemodynamics; 2) EPAH, with normal resting hemodynamics but age-adjusted pre-capillary pulmonary hypertension on exertion, and 3) PAH, defined as resting mPAP >20mmHg, pulmonary vascular resistance >3 Wood Units, and pulmonary capillary wedge pressure <15mmHg. Tortuosity was defined as the actual path length of a vessel divided by the linear distance between the two farthest endpoints of the vessel segment on CTA. AV>10% was defined as the number of arterial segments with tortuosity >10% divided by the same venous measure. Analysis was performed with Wilcoxon rank sum tests in R 3.5. Results: There were 99 patients in the final cohort, including 47 (47.4%) with PAH, 12 (12.1%) with EPAH, and 40 (40.4%) without disease. Compared to controls, median arterial tortuosity was increased in PAH (3.3 ± 0.1% vs. 3.4 ± 0.1%, p=0.0009; Figure 1) but not in EPAH (3.3 ± 0.1%, p=0.82). Median venous tortuosity did not differ between groups. AV>10% was increased in EPAH (vs. controls, 1.86 ± 0.38 vs. 1.56 ± 0.44, p=0.03) and resting PAH (2.0 ± 1.2 p=2e-6). Conclusions: Increased arterial tortuosity on CTA is a biomarker of resting PAH. When corrected for venous tortuosity, arterial tortuosity also appears to be abnormal in EPAH. Figure 1 . Arterial vessels in PAH, EPAH, and control subjects. Red segments have tortuosity > 10%.

Transition from IV epoprostenol to oral treprostinil in a patient with group 1 pulmonary arterial hypertension

2021 ◽  
Author(s):  
Preeyaporn Sarangarm ◽  
Kirsten Elwood
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Altered Mental Status ◽  
Direct Conversion ◽  
Central Line ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Final Titration ◽  
Rapid Transition ◽  
Pulmonary Arterial

Abstract Purpose Epoprostenol and treprostinil are prostacyclins indicated for the treatment of pulmonary arterial hypertension (PAH). Although there is literature describing the conversion of intravenous (IV) epoprostenol to IV treprostinil or IV treprostinil to oral treprostinil, there is little data on the direct conversion of IV epoprostenol to oral treprostinil. In this case, we describe the direct conversion of IV epoprostenol to oral treprostinil without an intermediary conversion to IV treprostinil. Summary A 39 year-old female with PAH was admitted for altered mental status and self-removal of her peripherally inserted central catheter (PICC) used for IV epoprostenol. Given the unplanned hospitalization, absence of a dedicated central line for IV prostacyclin therapy, and concern the patient may remove a future subcutaneous line, the patient was transitioned to oral treprostinil. Of note, despite triple PAH therapy, the patient was unable to reach a low risk group based on her prognostic risk assessment. A right heart catheterization four months prior found severe PAH with a pulmonary arterial pressure of 79/32 mmHg (mean, 49 mmHg) and pulmonary vascular resistance of 10.6 Wood units. To expedite the transition, the patient was directly converted from IV epoprostenol to oral treprostinil without an intermediary conversion to IV treprostinil. A target oral treprostinil dose of 5 mg TID was calculated based on 110% of the IV epoprostenol dose (19 ng/kg/min) utilizing the conversion recommended by the medication manufacturer. Every 8 hours, IV epoprostenol was decreased by 2 ng/kg/min and oral treprostinil was increased by 0.5 mg. The target oral treprostinil dose of 5 mg TID was reached 72 hours after conversion initiation. Three hours after the final titration, the patient was discharged home on room air. Conclusion In this case, rapid transition from IV epoprostenol to oral treprostinil was achieved in 72 hours without reported adverse effects.

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