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BACKGROUND: Little research is available to provide practical guidance to health-care providers for exercise preparticipation screening and referral of patients with interstitial lung diseases (ILDs), including lymphangioleiomyomatosis (LAM), to participate in remote, unsupervised exercise programs. RESEARCH QUESTION: What exercise preparticipation screening steps are essential to determine whether a patient with LAM is medically appropriate to participate in a remote, unsupervised exercise program? STUDY DESIGN AND METHODS: Sixteen experts in LAM and ILD participated in a two-round modified Delphi study, ranking their level of agreement for 10 statements related to unsupervised exercise training in LAM, with an apriori definition of consensus. Additionally, 60 patients with LAM completed a survey of the perceived risks and benefits of remote exercise training in LAM. RESULTS: Seven of the 10 statements reached consensus among experts. Experts agreed that an in-person clinical exercise test is indicated to screen for exercise-induced hypoxemia and prescribe supplemental oxygen therapy as indicated prior to initiating a remote exercise program. Patients with recent pneumothorax should wait to start an exercise program for at least 4 weeks until after resolution of pneumothorax and clearance by a physician. Patients with high cardiovascular risk for event during exercise, severe resting pulmonary hypertension, or risk for falls may be more appropriate for referral to a rehabilitation center. A LAM-specific remote exercise preparticipation screening tool was developed from the consensus statements and agreed upon by the panelists. INTERPRETATION: A modified Delphi study approach was useful to develop disease-specific recommendations for safety and preparticipation screening prior to unsupervised, remotely administered exercise in LAM. The primary product of this study is a clinical decision aid for providers to use when medically screening patients prior to participation in the newly launched LAMFit remote exercise program.
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Introduction: Up to 50% of pulmonary embolism (PE) patients have perfusion defects or residual vascular obstruction during follow-up despite adequate anticoagulant treatment, and a similar percentage experience chronic functional limitations and/or dyspnoea post-PE. We aimed to evaluate the association between pulmonary perfusion defects or residual vascular obstruction and functional recovery after PE. Methods: We performed a systematic review and meta-analysis including studies assessing both the presence of perfusion defects or residual vascular obstruction and functional recovery (i.e. persistent symptoms, quality of life, exercise endurance). An odds ratio was pooled for perfusion defects or residual vascular obstruction and persistent symptoms using a random-effect model. Results: 12 studies were included totalling 1888 PE patients; at a median of 6â months after PE (range 2-72 months), 34% had perfusion defects or residual vascular obstruction and 37% reported persistent symptoms. Among patients with perfusion defects or residual vascular obstruction, 48% (95% CI 37-60%, I2=82%) remained symptomatic during follow-up, compared to 34% (95% CI 20-51%, I2=96%) of patients without such defects. Presence of perfusion defects or residual vascular obstruction was associated with persistent symptoms (OR 2.15, 95% CI 1.66-2.78; I2=0%, τ=0). Notably, there was no association between these defects and quality of life or cardiopulmonary exercise test parameters. Conclusion: While the odds of having persistent symptoms was higher in patients with perfusion defects or residual vascular obstruction after acute PE, a significant proportion of these patients reported no limitations. A possible causality between perfusion defects or residual vascular obstruction and residual functional limitation therefore remains to be proven.
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Right ventricular (RV) dysfunction in pulmonary arterial hypertension (PAH) is associated with poor outcomes. Cardiac magnetic resonance imaging (cMRI) is the gold standard for volumetric assessment, and few reports have correlated 6-min walk distance (6MWD) and cMRI parameters in PAH. Cardiac Effort, (the number of heart beats used during 6-min walk test)/(6MWD), incorporates physiologic changes into walk distance and has been associated with stroke volume (SV) measured by nuclear imaging and indirect Fick. Here, we aimed to interrogate the relationship of Cardiac Effort and 6MWD with SV measured by the gold standard, cMRI. This was a single-center, observational, prospective study in Group 1 PAH patients. Subjects completed 6-min walk with heart rate monitoring (Cardiac Effort) and cMRI within 24 h. cMRI was correlated to Cardiac Effort and 6MWD using Spearman Correlation Coefficient. Twenty-five participants with a wide range of RV function completed both cMRI and Cardiac Effort. There was a strong correlation between left ventricle SV index and both Cardiac Effort (r = -0.70, p = 0.0001) and 6MWD (r = 0.67, p = 0.0002). Cardiac Effort and 6MWD were statistically separated in patients at prognostically significant thresholds of left ventricle SV index (>31 ml/m2), RV Ejection Fraction (>35%), and SV/End Systolic Volume ( > 0.53). Cardiac Effort and 6MWD are noninvasive ways to gain insight into those with impaired SV. 6MWD may correlate better with SV than previously thought and heart rate monitoring provides physiologic context to the walk distance obtained.
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Pulmonary arterial hypertension (PAH) patients have low activity. Activity intensity or duration could be a measure of clinical status or improvement. We aimed to determine whether standard or novel actigraphy measures could detect increases in activity after adding therapy. This was a prospective, single-center observational study evaluating activity after adding therapy in Group 1 PAH; we also report a validation cohort. For our study, two different accelerometers were used, a wrist (ActiGraph) and chest (MC10) device. Patients were analyzed in two groups, Treatment Intensification (TI, adding therapy) or Stable. Both groups had baseline monitoring periods of 7 days; the TI group had follow-up at 3 months, while Stables had follow-up within 4 weeks to assess stability. Activity time and steps were reported from both devices' proprietary algorithms. In ActiGraph only, steps in 1-min intervals throughout the day were ranked (not necessarily contiguous). Average values for each week were calculated and compared using nonparametric testing. Thirty patients had paired data (11 Stable and 19 TI). There was no between-group difference at baseline; we did not observe therapy-associated changes on average daily steps or activity time/intensity. The top 5 min of steps (capacity) increased after adding therapy; there was no difference in the stable group. This key finding was validated in a previously reported randomized trial studying a behavioral intervention to increase exercise. Total daily activity metrics are influenced by both disease and non-disease factors, making therapy-associated change difficult to detect. Peak minute steps were a treatment-responsive marker in both a pharmacologic and training intervention.
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Pulmonary hypertension (PH) is a leading cause of morbidity and mortality in systemic sclerosis (SSc). PH is a heterogenous condition and several different forms of PH are associated with SSc, including pulmonary arterial hypertension (PAH) resulting from a pulmonary arterial vasculopathy, PH due to interstitial lung disease, PH due to left heart disease, and PH due to thromboembolic disease. Extensive research has led to an improved understanding of the mediators involved in the pathogenesis of SSc-PH. Initial combination therapy is the preferred treatment approach for SSc-PAH and requires coordinated care with a multidisciplinary team including rheumatology, pulmonology, and cardiology.
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Hipertensión Pulmonar , Esclerodermia Sistémica , Humanos , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/terapia , Esclerodermia Sistémica/complicaciones , Esclerodermia Sistémica/terapia , FenotipoRESUMEN
BACKGROUND: There is a paucity of literature describing the recovery trajectory after surgery for upper extremity ischemia. Using Patient-Reported Outcomes Measurement Information System (PROMIS) Physical Function (PF), Upper Extremity (UE), Pain Interference (PI), and Depression domains, we aimed to describe the postoperative recovery of such patients. METHODS: We queried our PROMIS database for patients undergoing surgery for vasospastic or occlusive disease over a 4.5-year period. Inclusion criteria were preoperative, early (average 3 weeks) and late (average 6 months) postoperative PROMIS PF and/or UE, PI, and Depression scores. The change in PROMIS scores was calculated for each time point. Changes in PROMIS scores were compared with minimal clinically important difference estimates. RESULTS: We identified 13 patients undergoing 13 surgical interventions that met inclusion criteria. More than one-half of our patients were men (n = 7 [54%]), and more than one-half of the surgeries (n = 7 [54%]) were for isolated occlusive diagnoses, with the remainder for vasospastic disease. At short-term postoperative follow-up, the change in PROMIS PF, UE, PI, and Depression scores was -6.34 (SD: 9.13), -6.81 (SD: 9.61), 3.16 (SD: 5.78), and -3.05 (SD: 8.37), respectively. At mid-term postoperative follow-up, the change in PROMIS PF, UE, PI, and Depression scores was 4.45 (SD: 10.33), 8.04 (SD: 13.84), -7.03 (SD: 7.06), and -12.27 (SD: 10.85), respectively. CONCLUSIONS: Our findings suggest patients undergoing surgical treatment for upper extremity ischemia experience a worsening of functional symptoms initially, as expected, followed by notable improvement.
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Medición de Resultados Informados por el Paciente , Extremidad Superior , Masculino , Humanos , Femenino , Extremidad Superior/cirugía , Diferencia Mínima Clínicamente Importante , Depresión , Isquemia/etiología , Isquemia/cirugíaRESUMEN
BACKGROUND: The SARS-CoV-2 pandemic has limited objective physiologic assessments. A standardized remote alternative is not currently available. "Cardiac effort" (CE), that is, the total number of heart beats divided by the 6-min walk test (6MWT) distance (beats/m), has improved reproducibility in the 6MWT and correlated with right ventricular function in pulmonary arterial hypertension. RESEARCH QUESTION: Can a chest-based accelerometer estimate 6MWT distance remotely? Is remote cardiac effort more reproducible than 6MWT distance when compared with clinic assessment? STUDY DESIGN AND METHODS: This was a single-center, prospective observational study, with institutional review board approval, completed between October 2020 and April 2021. Group 1 subjects with pulmonary arterial hypertension, receiving stable therapy for > 90 days, completed four to six total 6MWTs during a 2-week period to assess reproducibility. The first and last 6MWTs were performed in the clinic; two to four remote 6MWTs were completed at each participant's discretion. Masks were not worn. BioStamp nPoint sensors (MC10) were worn on the chest to measure heart rate and accelerometry. Two blinded readers counted laps, using accelerometry data obtained on the clinic or user-defined course. Averages of clinic variables and remote variables were used for Wilcoxon matched-pairs signed rank tests, Bland-Altman plots, or Spearman correlation coefficients. RESULTS: Estimated 6MWT distance, using the MC10, correlated strongly with directly measured 6MWT distance (r = 0.99; P < .0001; in 20 subjects). Remote 6MWT distances were shorter than clinic 6MWT distances: 405 m (330-464 m) vs 389 m (312-430 m) (P = .002). There was no difference between in-clinic and remote CE: 1.75 beats/m (1.48-2.20 beats/m) vs 1.86 beats/m (1.57-2.14 beats/m) (P = .14). INTERPRETATION: Remote 6MWT was feasible on a user-defined course; 6MWT distance was shorter than clinic distance. CE calculated by chest heart rate and accelerometer-estimated distance provides a reproducible remote assessment of exercise tolerance, comparable to the clinic-measured value.
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COVID-19 , Hipertensión Arterial Pulmonar , Humanos , Prueba de Paso , Reproducibilidad de los Resultados , Caminata/fisiología , SARS-CoV-2 , Tolerancia al Ejercicio/fisiología , Hipertensión Pulmonar Primaria Familiar , Prueba de EsfuerzoRESUMEN
There are no prospective studies or guidelines describing transition between selexipag and oral treprostinil. We present two different transition strategies from selexipag to oral treprostinil, one started inpatient and then completed at home, and one completely under outpatient settings. Neither patient experienced worsening prostacyclin-type adverse effects; both were rigorous in their attention to a 7-8 hour administration schedule for oral treprostinil, and both experienced objective clinical benefit at follow-up. Prospective studies are needed to help guide clinical decisions when patients remain intermediate risk after a trial of either drug.
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Aims: Activity trackers for clinical trials and remote monitoring are appealing as they provide objective data outside of the clinic setting. Algorithms determine physical activity intensity and count steps. Multiple studies show physical inactivity in pulmonary arterial hypertension (PAH). There are no studies comparing different activity trackers worn on different parts of the body in PAH. We had patients with PAH simultaneously wear two different accelerometers, compared measures between the two devices, and correlated the measures with standard clinical metrics in PAH. Methods and results: This was a single-centre, prospective observational study. Daily physical activity and daily total steps were measured using Actigraph GT9X Link and MC10 Biostamp nPoint for 5-10 days. Actigraph was worn on the non-dominant hand and the MC10 Biostamp nPoint was worn on the chest and leg with disposable adhesives. Twenty-two participants wore both accelerometers >12 h/day for an average 7.8 days. The average activity time measured by Actigraph was significantly higher than that measured by MC10 (251 ± 25 min vs. 113 ± 18 min, P = 0.0001). Actigraph's algorithm reported more time in light activity than moderate (190 ± 62 min vs. 60 ± 56 min, P = 0.0001). REVEAL 2.0 scores correlated highly with activity time measured using either device. Invasively measured haemodynamics within 7 days did not correlate with activity time or daily steps. Conclusion: Different activity trackers yield discordant results in PAH patients. Further studies are needed in determining the best device, optimal wear time, and different thresholds for activities in chronic diseases.
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INTRODUCTION: Response to anticoagulation varies during management of acute hospitalized pulmonary embolism. We aimed to study thrombus histology in pulmonary embolism samples removed during acute surgical embolectomy to evaluate whether thrombus morphology was similar between patients and whether there was an association with duration of symptoms and/or resolution on follow up imaging. METHODS: This was a retrospective observational single center study at the University of Rochester Medical Center. We evaluated patients that underwent acute surgical embolectomy and followed up in our clinic 2 - 4 months after the event with Ventilation/Perfusion (V/Q) scan obtained for all regardless of symptoms. Thromboemboli were formalin fixed and processed for light microscopy in the hospital histopathology laboratory. Four-micron thick sections were stained with hematoxylin and eosin, Masson trichrome, and Verhoeff elastic tissue stains. Immunohistochemistry was performed using anti-CD31 and anti-CD68. Slides were independently evaluated for time-dependent microscopic changes using Irniger's classification by two blinded pathologists. RESULTS: Sixteen patients underwent embolectomy with fifteen having V/Q imaging at follow up. The majority of patients were female. Samples showed a generally similar overall architecture that included a central core composed primarily of red blood cells and fibrin and an outer layer of platelets and monocytes. Two samples had evidence of fibrosis and recanalization. CONCLUSIONS: We found heterogeneous histopathology in samples obtained during acute embolectomy. Further prospective studies should systematically characterize clot morphology and evaluate treatment response and outcomes. Careful thrombus specimen measurement and consistent sampling for sections will be required to draw firm conclusions.
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Embolia Pulmonar , Trombosis , Embolectomía , Femenino , Humanos , Masculino , Estudios Prospectivos , Resultado del TratamientoRESUMEN
BACKGROUND AND OBJECTIVES: Pulmonary hypertension is commonly seen in end stage kidney disease and is most commonly due to elevated left heart pressures. There is limited data about vasodilator use during the management of Group 1 pulmonary arterial hypertension in the context of those who also have or later developed end stage kidney disease. The objective of this study was to determine safety and efficacy of vasodilator therapy in precapillary pulmonary hypertension requiring renal replacement therapy. DESIGN: This was a single-center retrospective case series. Patients were identified from our Pulmonary Hypertension Clinic using a historical roster from 2012 to 2020. Patients were included if they >18 years of age, had Group 1 or Group 4 (precapillary) pulmonary hypertension on right heart catheterization, and also had end stage kidney disease requiring either intermittent hemodialysis or peritoneal dialysis. RESULTS: 18 patients were identified with invasively confirmed Group 1 or Group 4 pulmonary hypertension and end stage kidney disease on renal replacement therapy. Scleroderma was the most common etiology for renal failure. 17 patients were treated with vasodilator therapy. Fifteen patients had paired right heart catheterizations that showed a significant decrease in mean pulmonary artery pressure and pulmonary vascular resistance. Therapy was relatively well tolerated but hypotension was common and midodrine was often helpful. Two patients had successful renal transplantation after starting vasodilator therapy. CONCLUSION: We found vasodilator therapy was reasonably well tolerated and associated with a drop in mean pressure and pulmonary vascular resistance in patients with end stage kidney disease on dialysis.
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Hipertensión Pulmonar/tratamiento farmacológico , Hipertensión Pulmonar/etiología , Fallo Renal Crónico/complicaciones , Vasodilatadores/uso terapéutico , Adulto , Anciano , Femenino , Humanos , Fallo Renal Crónico/terapia , Trasplante de Riñón , Masculino , Persona de Mediana Edad , Diálisis Renal , Estudios RetrospectivosRESUMEN
BACKGROUND: Pulmonary embolism (PE) response teams (PERT) have been developed to improve in-hospital mortality. Identifying intermediate risk PE patients that will progress despite anticoagulation is difficult, especially because outcomes with modern anticoagulation are quite good. OBJECTIVE: The primary aim of this study was to evaluate the rate of anticoagulation failure (new deep vein thrombosis or PE, right ventricular failure resulting in shock, cardiac arrest, or PE-attributable death) in intermediate risk PE patients managed by PERT. The secondary objective was to determine whether there was a significant decrease in heart rate 24 h after initiation of anticoagulation in intermediate risk PE. METHODS: This was a retrospective observational study of patients treated for acute intermediate risk PE at the University of Rochester Medical Center who also had outpatient followup between November 2016-June 2019. RESULTS: Ninety-two patients presented as intermediate-risk PE and had outpatient followup. Seventy-four patients were initially treated with anticoagulation. None of these patients failed anticoagulation. Of the eighteen intermediate risk patients that underwent advanced intervention, none failed anticoagulation first. There was significant decrease in resting heart rate 24 h after starting therapeutic anticoagulation, 107 beats/min vs 89 beats/min, p = 0.0001. CONCLUSION: We did not observe anticoagulation failure in the management of acute, intermediate risk PE. Reductions in heart rate may reflect improvements in right ventricular function; we hypothesize that those whose heart rate does not fall may be optimal candidates for advanced intervention.
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Anticoagulantes/administración & dosificación , Frecuencia Cardíaca , Embolia Pulmonar/diagnóstico , Embolia Pulmonar/tratamiento farmacológico , Descanso/fisiología , Anciano , Femenino , Estudios de Seguimiento , Mortalidad Hospitalaria , Humanos , Masculino , Persona de Mediana Edad , Embolia Pulmonar/mortalidad , Embolia Pulmonar/fisiopatología , Estudios Retrospectivos , Riesgo , Medición de Riesgo , Insuficiencia del Tratamiento , Resultado del Tratamiento , Función Ventricular DerechaRESUMEN
Necrotizing autoimmune myopathy (NAM) is a rare inflammatory process characterized by bilateral proximal muscle weakness and elevated creatinine kinase levels. It is one of the idiopathic inflammatory myopathies. It can be associated with anti-signal recognition particle (SRP) antibody which is commonly seen in middle-aged females. Classic findings on muscle biopsy include muscle fiber necrosis without inflammation. Pulmonary manifestations associated with anti-SRP NAM is rare, and often a challenging correlation to make as our understanding of inflammatory myopathies and interstitial lung disease is still evolving. There have been some associations of Anti SRP NAM with NSIP which responds to corticosteroids. We present a 29 year old male with asymptomatic NAM who presented with a combination of NSIP and pulmonary arterial hypertension (PAH). His PAH was responsive to oral vasodilator therapy however his interstitial lung disease (ILD) rapidly progressed to usual interstitial pneumonia (UIP) requiring lung transplantation. This case highlights 1) an extremely rare presentation of rapidly progressive NAM associated ILD in a young man, in which pulmonary manifestations occurred in the absence of myopathy, 2) The importance of doing a complete work up for interstitial lung disease, including diligent examination for myopathic features and obtaining CK levels, 3) Identifying that interstitial lung diseases can progress despite control of the underlying etiology with corticosteroids and immunosuppressives, 4) Recognition of pre capillary PAH in patients with disproportionally elevated pressures relative to their pulmonary findings, 5) The first report of treatment responsive pulmonary vascular disease associated with NAM, and 6) The importance of early lung transplantation evaluation.
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There is little reported on the efficacy and safety of direct oral anticoagulants (DOACs) in morbid obesity after venous thromboembolism (VTE). In this observational study, patients were followed up after intermediate- or high-risk pulmonary embolism (PE) at the University of Rochester Pulmonary Hypertension Clinic 2-4â months after the initial event. All patients had echocardiography and V/Q imaging regardless of symptoms. Outcomes of interest were the rates of recurrent VTE, thrombus resolution and development of chronic thromboembolic pulmonary hypertension (CTEPH) in patients with morbid obesity treated with a DOAC compared to treatment with vitamin K antagonists and to non-morbidly obese patients after PE. Using the electronic medical record, recurrent events were assessed up to 12â months after the event. 107 patients (body mass index (BMI)>40â kg·m-2, n=32; BMI 30-39.9â kg·m-2, n=39; BMI<30â kg·m-2, n=36) attended follow-up appointments after treatment for PE. A DOAC was used in 70 patients (BMI>40â kg·m-2, n=19; BMI 30-39.9 kg·m-2, n=27; BMI<30â kg·m-2, n=24). There were no recurrent events within the first 12â months of initial diagnosis based on symptoms and imaging in any patient. There was no difference in rate of residual unmatched perfusion defect with DOACs or conventional anticoagulation (49% versus 49%). This finding remained in the subset of morbidly obese patients (47% versus 50%). For the overall cohort, there was no difference in the rate of CTEPH development based on anticoagulation with a DOAC (5% versus 8% with warfarin). There were no major bleeding complications with a DOAC. DOAC therapy appears to be effective and safe in morbid obesity even after intermediate- or high-risk PE. â.
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Right ventricular (RV) function is a predictor of outcomes in pulmonary arterial hypertension (PAH). The 6-min walk test (6MWT) is likely an indirect measure of RV function during exercise, but changes in absolute walk distance can also be influenced by factors like effort and musculoskeletal disease. Paired 6MWT with continuous electrocardiogram monitoring was performed in stable PAH patients, patients adding PAH therapies, and healthy controls. Heart rate expenditure (HRE) was calculated (integrating pulse during 6MWT) and then divided by walk distance (HRE/d). We also evaluated changes in peak heart rate, time above age-adjusted maximum predicted heart rate, and heart rate at 6 min. HRE/d was compared to invasive hemodynamic measures in patients who had right heart catheterization performed within seven days, WHO functional class assessment, and Emphasis 10 questionnaire. We measured two 6MWT in 15 stable PAH patients, 13 treatment intensification patients, and 8 healthy controls. HRE/d was reproducible in the stable PAH group (median difference, -0.79%), while it decreased (median difference, 23%, p = 0.0001) after adding vasodilator therapy. In 11 patients with right heart catheterization, HRE/d correlated strongly with stroke volume, r = -0.72, p = 0.01. Peak heart rate decreased after adding vasodilator therapy. HRE/d also correlated with WHO functional class and Emphasis 10 score. Continuous heart rate monitoring during 6MWT provides valuable physiologic data accounting for effort. HRE/d appears to enhance test reproducibility in stable patients while detecting change after adding therapy as compared to walk distance alone.
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Long-term outcomes after acute pulmonary embolism vary from complete resolution to chronic thromboembolic pulmonary hypertension (CTEPH). Guidelines after acute pulmonary embolism are generally limited to anticoagulation duration. We assessed patients with estimated prognosis >1 year in our pulmonary hypertension clinic 2-4 months after treatment for intermediate- or high-risk acute pulmonary embolism. At follow-up, ventilation-perfusion scan and echocardiogram were offered. The aim of this study was to assess for recurrent symptomatic disease, residual imaging defects or right ventricular dysfunction, and functional disability after acute management of pulmonary embolism. After treatment for acute intermediate- or high-risk pulmonary embolism, 104 patients followed up in pulmonary hypertension clinic. Of those, 55% of patients had self-reported limitation in activity. No patients had symptomatic recurrence of pulmonary embolism. Forty-eight percent of patients had residual perfusion defects on perfusion imaging, while 91% of patients had either normal or only mildly enlarged right ventricles. We identified heart failure preserved ejection fraction, iron deficiency, and obstructive sleep apnea as significant contributors to breathlessness. Treatment of these conditions was associated with improvement. Surprisingly, we diagnosed CTEPH in nine patients; for some, chronic thrombus may already have been present at the time of index evaluation. Our findings suggest that follow-up in a dedicated pulmonary hypertension clinic 2-4 months after acute intermediate- or high-risk pulmonary embolism may add value to patient care. We identified treatable comorbidities that could be contributing to post-pulmonary embolism syndrome as well as CTEPH.