scholarly journals Right Heart Size and Right Ventricular Reserve in Pulmonary Hypertension: Impact on Management and Prognosis

Diagnostics ◽  
2020 ◽  
Vol 10 (12) ◽  
pp. 1110
Author(s):  
Ekkehard Grünig ◽  
Christina A. Eichstaedt ◽  
Rebekka Seeger ◽  
Nicola Benjamin

Various parameters reflecting right heart size, right ventricular function and capacitance have been shown to be prognostically important in patients with pulmonary hypertension (PH). In the advanced disease, patients suffer from right heart failure, which is a main reason for an impaired prognosis. Right heart size has shown to be associated with right ventricular function and reserve and is correlated with prognosis in patients with PH. Right ventricular reserve, defined as the ability of the ventricle to adjust to exercise or pharmacologic stress, is expressed by various parameters, which may be determined invasively by right heart catheterization or by stress-Doppler-echocardiography as a noninvasive approach. As the term “right ventricular contractile reserve” may be misleading, “right ventricular output reserve” seems desirable as a preferred term of increase in cardiac output during exercise. Both right heart size and right ventricular reserve have been shown to be of prognostic importance and may therefore be useful for risk assessment in patients with pulmonary hypertension. In this article we aim to display different aspects of right heart size and right ventricular reserve and their prognostic role in PH.

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
T Iwanaga ◽  
T Aoki ◽  
T Ogo ◽  
A Tsuji ◽  
J Ueda ◽  
...  

Abstract Background Although pulmonary endarterectomy (PEA) is an established surgical treatment for chronic thromboembolic pulmonary hypertension (CTEPH), a part of patients after PEA show residual pulmonary hypertension, leading to limited exercise capacity. Recently, several studies have indicated that balloon pulmonary angioplasty (BPA) improves hemodynamics, exercise capacity and prognosis in inoperable CTEPH patients. However, the effects of BPA in patients with residual pulmonary hypertension after PEA remain to be elucidated. Aim In the present study, we investigated comprehensive efficacy of BPA on hemodynamics, exercise capacity and right ventricular function in those with residual pulmonary hypertension after PEA. Methods From October 2010 to February 2019, 227 patients with CTEPH underwent PEA in our institution. Right heart catheterization after PEA (median follow up period from PEA to right heart catheterization 39 [10.5, 90] months) showed that 55 patients showed residual PH (mean pulmonary artery pressure (mPAP)≥25mmHg), and 38 of them referred to BPA (mean age 57 years old, male 8 (21%)) due to residual symptoms. In 29 out of 38 patients (76%) who completed BPA and underwent follow-up right heart catheterization, we examined hemodynamics, exercise capacity and right ventricular function before and after BPA. Follow-up examination was performed 3 months after last BPA session. Results In this study population (N=29), PEA significantly improved mPAP (47±7 to 38±10 mmHg), pulmonary vascular resistance (PVR, 14.6±4.6 to 9.2±4.6 WU) and right ventricular ejection fraction measured by magnetic resonance imaging (26.6±11.3 to 38.4±6.8%) (Figure). Median period from PEA to first BPA procedure was 42 [13.5, 94] months. Total session number during study period was 160 sessions, and mean session number of BPA was 5.5±1.5 per patient. Follow-up study revealed that BPA additionally improved mPAP (38±10 to 27±8 mmHg) and PVR (9.2±4.6 to 5.1±2.2 WU) (Figure). Similarly, 6-minute walk distance (393±125 to 452±125 m) and peak VO2 (16.4±3.8 to 18.1±4.6 ml/min/kg, p<0.05) were increased, and WHO functional class also significantly improved by BPA (I/II/III/IV, 0/21/8/ 0 to 1/27/1/0, p<0.01). In addition, right ventricular ejection fraction (38.4±6.8 to 44.2±7.1%) was increased after BPA (Figure). There were no procedure-related deaths and major lung injuries requiring oral intubation during study period. 3-year survival in patients after BPA was 100% (median follow-up period after last BPA session, 32 [18, 46] months). Conclusion In CTEPH patients with residual pulmonary hypertension after PEA, additional BPA significantly improved hemodynamics, right ventricular function, exercise capacity and residual symptoms without severe complications, leading to good prognosis. These results suggest that combination therapy of PEA and BPA could be an effective therapeutic option for post PEA patients with residual symptoms and exercise limitation. Figure 1 Funding Acknowledgement Type of funding source: None


2020 ◽  
Vol 10 (4) ◽  
pp. 204589402094411
Author(s):  
Bashar N. Alzghoul ◽  
Robert Hamburger ◽  
Thomas Lewandowski ◽  
Brandon Janssen ◽  
Daniel Grey ◽  
...  

Pulmonary hypertension in interstitial lung diseases is associated with increased mortality and hospitalizations and reduced exercise capacity. Interstitial pneumonia with autoimmune features (IPAF) is a recently described interstitial lung disease. The characteristics of pulmonary hypertension in IPAF patients are unknown. We sought to characterize patients with IPAF based on their echocardiographic probability of pulmonary hypertension and compare patients with and without pulmonary hypertension identified by right heart catheterization. We conducted a retrospective study of patients seen in the interstitial lung disease clinic from 2015 to 2018. Forty-seven patients with IPAF were identified. Patients were classified into low, intermediate and high echocardiographic pulmonary hypertension probabilities. A sub-group analysis of patients with pulmonary hypertension and without pulmonary hypertension (IPAF-PH vs. IPAF-no PH) identified by right heart catheterization was also performed. Linear regression analysis was performed to study the association between 6-min-walk-distance (6MWD) and pulmonary vascular resistance (PVR) while adjusting for age and body mass index. Right ventricular hypertrophy (>5 mm), right ventricular enlargement (>41 mm) and right ventricular systolic dysfunction defined as fractional area change% ≤35 was present in 76%, 24%, and 39% of patients, respectively. Pulmonary hypertension was identified in 12.7% of patients. IPAF-PH patients had higher mean pulmonary artery pressure and lower cardiac output compared to the IPAF-no PH group (34 mmHg vs. 19 mmHg, p = 0.002 and 4.0 vs. 5.7 L/min, p = 0.023, respectively). Lower 6MWD was associated with higher PVR on regression analysis ( p = 0.002). Pulmonologists should be aware that a significant number of IPAF patients may develop pulmonary hypertension. Reduced 6MWD may suggest the presence of pulmonary hypertension in IPAF patients.


2020 ◽  
Vol 10 (3) ◽  
pp. 204589402093528 ◽  
Author(s):  
Dana Kay ◽  
Ferdous Kadri ◽  
Garrett Fitzpatrick ◽  
Hassan Alnuaimat ◽  
Raju Reddy ◽  
...  

Pulmonary arterial hypertension has been reported with a prevalence of 7.9% in patients with anti-synthetase syndrome; however, anti-synthetase syndrome associated with pulmonary veno-occlusive disease (PVOD) has never before been described in the literature. We present a novel case of anti-synthetase syndrome-associated PVOD in a patient who presented with hypoxic respiratory failure associated with right heart failure and was diagnosed with anti-synthetase syndrome based on his autoimmune serology and pre-capillary pulmonary hypertension on right heart catheterization. He was initiated on pulmonary arterial hypertension therapy, but with escalating dose of parenteral epoprostenol, experienced acute clinical worsening with chest imaging concerning for PVOD that was confirmed on autopsy. Anti-synthetase syndrome can be associated with PVOD, and it should be suspected in patients who have evidence of pre-capillary pulmonary hypertension and who deteriorate with the initiation of pulmonary hypertension-specific therapy.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Vineet A Agrawal ◽  
Amelia Muhs ◽  
Tufik Assad ◽  
Ivan M Robbins ◽  
John Newman ◽  
...  

Introduction: Fluid challenge during right heart catheterization (RHC) identifies occult left heart diastolic dysfunction (Occ-DD) in 10-20% of patients initially diagnosed with pre-capillary pulmonary hypertension (PH). The prognostic implications of the response to fluid challenge are not known. Abnormal right ventricular (RV) function is a poor prognostic indicator in all forms of PH. Hypothesis: We hypothesized that fluid challenge during RHC identifies patients with worse RV function and survival. Methods: Consecutive patients referred for RHC with fluid challenge from 2009-2016 were studied. Occ-DD was defined by a resting pulmonary arterial wedge pressure (PAWP) < 15 mmHg, and a PAWP > 18 mmHg after 500cc fluid challenge. Resting DD was defined by resting PAWP > 15 mmHg. RV function was assessed by pulmonary artery pulsatility index (PAPi) or RV stroke work index (RVSWI). Statistical analysis was conducted using Kruskal Wallis test. Survival analysis was conducted by log rank test. Results: Of the 310 patients identified, 83% were female with a mean age of 54 + 14 years. The cohort consisted of 204 (66%) patients without DD, 30 (10%) with Occ-DD, and 76 (24%) with resting DD. Patients with Occ-DD were older (59 + 12 vs 51 + 14, p < 0.0001) with greater BMI (31 + 6 vs 28 + 7, p < 0.0001), but had similar prevalence of obesity, hypertension, diabetes mellitus, and coronary disease to all groups. Patients with Occ-DD had lower PAPi (7.8 + 0.4 vs 8.5 + 0.4, p < 0.0001), lower RVSWI (9.9 + 0.8 vs 17.8 + 0.7 mmHg*ml/m2, p < 0.0001) (Figure), and decreased survival (3.7 + 3.5 vs 4.9 + 3.5 years, p < 0.0001) compared to those without DD. After adjustment for RV function, survival was no longer different between groups. Conclusions: Patients with Occ-DD after fluid challenge during RHC have decreased survival due to worse RV function compared to patients without DD. These findings suggest important prognostic implications for fluid challenge in the evaluation of patients with PH.


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