Fenstad, Eric R.

Introduction: Creation of an atrial septal defect via atrial septostomy (AS) is a therapeutic option traditionally utilized for PAH in settings where medical therapy was unavailable. Its utility in the context of progressive disease despite medical therapy remains incompletely understood. Hypothesis: AS may be a useful option in patients with symptomatic PAH despite medical therapy and may improve functional class. Methods: Single center retrospective study of all adult PAH patients receiving AS at a tertiary PAH referral center. Results: Eleven atrial septostomy procedures were performed in 8 patients who were refractory to maximal medical management. All patients were WHO functional class III or IV and average patient age was 40 ± 13 years with a female predominance (89%). Mean RA pressure was 18 ± 4 mm Hg with cardiac index of 2.28 ± 0.82 L/min/m 2 prior to AS. Mean pulmonary artery pressure was 63 mm Hg with a pulmonary artery saturation of 48% and average systolic blood pressure of 97 mm Hg. Baseline NT-proBNP was 5573 ± 4589 pg/ml with a mean 6 minute walk distance (6MWD) of 311 ± 131 meters. Five of the 11 procedures resulted in functional class improvement. Functional class change did not relate to change in 6MWD or NT-pro BNP level. Three patients had repeat AS due to spontaneous narrowing of the original septostomy, with a mean duration of 220 ± 135 days between procedures. One patient died within 30 days of the procedure and another at day 88 while one patient received a heart/lung transplant at day 42 post AS. Mean follow up was 243 ± 183 days. The REVEAL risk scores ranged from 10 to 15, with predicted 1 year survival from pre-septostomy clinical data ranging from 22% to 80% (mean predicted survival of 57%). Actual transplant free survival at 1 year was 63%. Conclusions: Selected patients at high risk for 1 year mortality may receive symptomatic benefit from atrial septostomy. One year mortality remains high, and not significantly different from that predicted by the REVEAL score. Atrial septostomy may be a palliative treatment option for patients who are refractory to maximal medical therapy but further long term investigation is necessary.

Objective. To determine the prevalence of monoclonal gammopathy of undetermined significance (MGUS) in patients with PH as well as precapillary PH. Methods. Olmsted County residents with PH, diagnosed between 1/1/1995 and 9/30/2017, were identified, and age and sex were matched to a normal control group. The PH group and normal control group were then cross-referenced with the Mayo Clinic MGUS database. Charts were reviewed to verify MGUS and PH. Heart catheterization data were then analyzed in these patients for reference to the gold standard for diagnosis. Results. There were 3419 patients diagnosed with PH by echocardiography between 1995 and 2017 in Olmsted County that met the criteria of our study. When the PH group (N = 3313) was matched to a normal control group (3313), a diagnosis of MGUS was significantly associated with PH 10.2% (OR = l.84 [95% CI 1.5–2.2], p < 0.001 ), compared with controls 5.8% based on echo diagnosis. Using heart catheterization data (484 patients), a diagnosis of MGUS was associated with PH 13.0% (OR = 3.94 [95% CI 2.28–6.82], p < 0.001 ). For pulmonary artery hypertension (N = 222), a diagnosis of MGUS was associated with PH at similar 12.2% (OR = 4.50 [95%CI 1.86–10.90], p < 0.001 . Conclusions. There is a higher prevalence of MGUS in patients with PH and precapillary PH compared with normal controls. This association cannot be explained fully by other underlying diagnoses associated with PH. Assessing for this in patients with PH of unclear etiology may be reasonable in the workup of patients found to have PH.

Introduction: Pericardial decompression syndrome (PDS) has been reported as a spectrum of cardiac decompensation following pericardiocentesis including ventricular failure, cardiogenic shock and pulmonary edema. PDS has been reported in patients undergoing drainage ≥450 mL of pericardial fluid. Hence judicious drainage of large volume effusions to avoid complication has been recommended Hypothesis: PDS complicating undergoing transthoracic echocardiography (TTE) guided pericardiocentesis is uncommen Methods: Consecutive patients undergoing TTE guided pericardiocentesis at our center from 1/2007 to 12/2016 were reviewed. Procedures were performed in a standard fashion under moderate conscious sedation with TTE determining the location of aspiration and determining adequate resolution of pericardial fluid post procedure. All fluid was removed at time of centesis regardless of the size of effusion, except for patients with severe pulmonary hypertension (n=9), who had staged removal of 100cc every 10 minutes. Post procedure, a pigtail catheter was left in the pericardial space for ongoing intermittent drainage and removed after the total net output was <50 mL/24 hours. Patients were monitored in an intermediate level care unit. All patients underwent repeat TTE at the time of catheter removal. Large volume was defined as ≥450 mL of fluid. Patients in whom pericardiocentesis was technically unsuccessful were excluded Results: Over a 10-year period a total of 1067 patients (57% male, mean age 62±16 years) underwent 1164 technically successful pericardiocentesis procedures. Of these, 561 patients had large volume (≥450 mL) aspirated (mean 750±345 mL aspirated, range 450 - 4300 mL). Patients had a pericardial catheter in place for a mean 2.9±2 days. One patient with pre-existing severe biventricular dysfunction after aspiration of 400cc developed hypotension that improved with reversal of sedation, but proceeded to have PEA arrest and die. No other patient developed clinical events of acute left or right heart failure, ventricular dysfunction, unexplained hypotension, cardiogenic shock or other clinical events that could relate to PDS Conclusions: At most one episode of PDS was observed after technically successful percutaneous pericardiocentesis in 1164 cases (561 were large volume), although whether this case was PDS is unclear. PDS appears to be very rare, and staging pericardial decompression over time may be unnecessary, other than, perhaps, in patients with severe ventricular dysfunction or severe pulmonary hypertension

Section editor's note: Welcome to a new section in Advances titled “Pulmonary Hypertension Grand Rounds” (PHGR), which we are introducing in this issue. With this addition, Advances offers a unique opportunity for trainees and junior faculty interested in pulmonary hypertension (PH). This section will highlight cases with literature reviews written by young clinicians and researchers in PH. PHGR was created to use patient cases to integrate individual clinical expertise with the best available evidence from the literature to enable the best decisions for patients. As with the rest of the journal, our audience continues to be physicians, researchers, nurses, students, residents, and fellows interested in both the care of individual patients and the advances in our field. We welcome comments about the section and inquiries regarding submitting a case for publication. Please direct inquiries to advancesgrandrounds@PHAssociation.org

Summary: Pulmonary hypertension (PH) can be associated with a high level of symptom burden from the disease as well as its treatment. Involvement of palliative care (PC) services may help facilitate discussion regarding goals of care, prognostic planning, and treatment options focused on improving quality of life (QOL). Background: PC is active total care of a patient whose disease is not fully responsive to curative therapies, with symptom control as the top priority. After a life-limiting diagnosis is made, health care teams and patients determine prognosis, whether cure is attainable or reasonable, what treatment options are available, risks and benefits of associated treatments, and how treatment or nontreatment will impact QOL and survival. QOL is often the focus of palliative interventions, with the goal to minimize symptoms and empower patients with accurate information to help affirm life and meet objectives of care. Implications for clinicians: PC can begin at the onset of symptoms in a disease that cannot be cured. Early PC may help facilitate discussion regarding goals of care when patient expectations are discordant with prognosis. While PC is a responsibility of all clinicians, subspecialist assistance can be helpful when a clinical decline occurs, in the setting of uncertainty, when patients are removed from the transplant list, or when long-term QOL issues are present. Conclusion: Communication with patients who have PH can be delicate and requires an understanding of the disease's process, trajectory, and prognosis. PC teams possess communication skills that may benefit patients and providers with QOL optimization, delivery of difficult news, advanced care planning, and shared decision-making.

PurposeTo evaluate with magnetic resonance elastography (MRE) whether patients with constrictive pericarditis (CP) have increased hepatic stiffness. CP results in reduced pericardial compliance, ventricular interdependence, and right heart failure. Patients with untreated CP may develop liver fibrosis and ultimately cirrhosis due to chronic venous congestion. Chronic venous congestion ± fibrosis may lead to increased liver stiffness.Materials and MethodsProspectively, patients with suspected CP underwent 2D transthoracic echocardiography, cardiac MRI, and liver MRE. An automated method was used to draw regions of interest (ROIs) on the stiffness maps to calculate the mean liver stiffness in kilopascals (kPa). A t‐test with α = 0.05 was performed between stiffness values of patients with positive and negative CP findings based on previously published echocardiography criteria.ResultsNineteen patients met inclusion criteria with a mean ± standard deviation (SD) age of 51 ± 16 years. Nine patients (47%) had CP. Mean liver stiffness trended higher in patients with CP compared to those without CP (4.04 kPa vs. 2.46; P = 0.045). Liver stiffness correlated with MRI septal bounce (P = 0.04), inferior vena cava size (P = 0.003), echo abnormal septal motion (P = 0.04), and echo mitral inflow variation >25% (P = 0.02). Only MRI septal bounce predicted CP by echocardiography (P < 0.001).ConclusionCP was associated with increased liver stiffness. The increased stiffness is most likely secondary to chronic hepatic venous congestion and/or fibrosis. MRE may be useful for noninvasive liver stiffness assessment in CP. J. Magn. Reson. Imaging 2016;44:81–88.

An association between pulmonary hypertension (PH) and POEMS syndrome (characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) as well as other plasma cell dyscrasias, including multiple myeloma (MM), has been shown to exist. Recent case reports have identified a reversible form of PH that occurs outside of previously identified etiologies. We report two cases of PH in the setting of smoldering MM (SMM) that resolved with chemotherapy and stem cell transplantation. Although other features were individualized among the cases (dermatomyositis, scleromyxedema), treatment of MM and SMM resulted in a normalization of right ventricular systolic pressure and improvement in right ventricular dysfunction that was previously unresponsive to PH therapies. The magnitude and sustained nature of reversibility in these four cases could offer clues about the pathophysiology and treatment of PH.

Pulmonary arterial hypertension (PAH) is a chronic, symptomatic, life‐threatening illness; however, it is complex, with variable expression regarding impact on quality of life (QOL). This study investigated attitudes and comfort of physicians regarding palliative care (PC) for patients with PAH and explored potential barriers to PC in PAH. An internet‐based, mixed‐methods survey was distributed to Pulmonary Hypertension Clinicians and Researchers, a professional organization within the Pulmonary Hypertension Association. Only responses from physicians involved in clinical care of patients with PAH were analyzed. Of 355 clinicians/researchers, 79 (22%) returned surveys, including 76 (21%) providers involved in clinical care. Responding physicians were mainly pulmonologists (67%), practiced in university/academic medical centers (89%), had been in practice a mean of 12 ± 7 years, cared for a median of 100 PAH patients per year, and reported a high level of confidence in managing PAH (87%), advanced PAH‐specific pharmacologic interventions (95%), and end‐of‐life care (88%). Smaller proportions were comfortable managing pain (62%) and QOL issues (78%). Most physicians (91%) reported utilizing PC consultation at least once in the prior year, primarily in the setting of end‐of‐life/active dying (59%), hospice referral (46%), or symptomatic dyspnea/impaired QOL (40%). The most frequent reasons for not referring patients to PC included nonapproval by the patient/family (51%) and concern that PC is “giving up hope” (43%). PAH may result in symptoms that impair QOL despite optimal PAH therapy; however, PC awareness and utilization for PAH providers is low. Opportunities may exist to integrate PC into care for PAH patients.