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Pulmonary Vasodilator Therapy in Severe Pulmonary Hypertension Due to Chronic Obstructive Pulmonary Disease (Severe PH-COPD): A Systematic Review and Meta-Analysis

Elkhapery, Ahmed; Hammami, M Bakri; Sulica, Roxana; Boppana, Hemanth; Abdalla, Zeinab; Iyer, Charoo; Taifour, Hazem; Niu, Chengu; Deshwal, Himanshu
PMID: 38132665
ISSN: 2308-3425
CID: 5612232

Pulmonary Vasodilator Therapy in Severe Pulmonary Hypertension Due to Chronic Obstructive Pulmonary Disease (Severe PH-COPD): A Systematic Review and Meta-Analysis

Elkhapery, Ahmed; Hammami, M. Bakri; Sulica, Roxana; Boppana, Hemanth; Abdalla, Zeinab; Iyer, Charoo; Taifour, Hazem; Niu, Chengu; Deshwal, Himanshu
Background: Chronic obstructive pulmonary disease-associated pulmonary hypertension (PH-COPD) results in a significant impact on symptoms, quality of life, and survival. There is scant and conflicting evidence about the use of pulmonary hypertension (PH) specific therapy in patients with PH-COPD. Study Design and Methods: PubMed, OVID, CINAHL, Cochrane, Embase, and Web of Science were searched using various MESH terms to identify randomized controlled trials (RCTs) or observational studies investigating PH-specific therapies in patients with severe PH-COPD, defined by mean pulmonary artery pressure (mPAP) of more than 35 mm Hg or pulmonary vascular resistance (PVR) of more than 5 woods units on right heart catheterization. The primary outcome was a change in mPAP and PVR. Secondary outcomes were changes in six-minute walk distance (6MWD), changes in the brain-natriuretic peptide (BNP), New York Heart Association (NYHA) functional class, oxygenation, and survival. Results: Thirteen studies satisfied the inclusion criteria, including a total of 328 patients with severe PH-COPD. Out of these, 308 patients received some type of specific therapy for PH. There was a significant reduction in mPAP (mean difference (MD) −3.68, 95% CI [−2.03, −5.32], p < 0.0001) and PVR (MD −1.40 Wood units, 95% CI [−1.97, −0.82], p < 0.00001). There was a significant increase in the cardiac index as well (MD 0.26 L/min/m2, 95% CI [0.14, 0.39], p < 0.0001). There were fewer patients who had NYHA class III/lV symptoms, with an odds ratio of 0.55 (95% CI [0.30, 1.01], p = 0.05). There was no significant difference in the 6MWD (12.62 m, 95% CI [−8.55, 33.79], p = 0.24), PaO2 (MD −2.20 mm Hg, 95% CI [−4.62, 0.22], p = 0.08), or BNP or NT-proBNP therapy (MD −0.15, 95% CI [−0.46, 0.17], p = 0.36). Conclusion: The use of PH-specific therapies in severe PH-COPD resulted in a significant reduction in mPAP and PVR and increased CI, with fewer patients remaining in NYHA functional class III/IV. However, no significant difference in the 6MWD, biomarkers of right ventricular dysfunction, or oxygenation was identified, demonstrating a lack of hypoxemia worsening with treatment. Further studies are needed to investigate the use of PH medications in patients with severe PH-COPD.
ISSN: 2308-3425
CID: 5630632

Approach to the hospitalized patient with pulmonary arterial hypertension

Deshwal, Himanshu; Sulica, Roxana
PURPOSE OF REVIEW/OBJECTIVE:Hospitalization in pulmonary arterial hypertension (PAH) patients is an important clinical worsening event significantly associated with subsequent mortality. Furthermore, irrespective of the cause of hospitalization, the overall outcome is closely related to the severity of the right ventricular (RV) dysfunction. Therefore, understanding the pathophysiology of pulmonary hypertension and RV failure is paramount in successfully managing PAH patients requiring hospitalization. This review highlights diagnostic and therapeutic approaches in various clinical scenarios that might be encountered during hospitalization of the World Health Organization group I PAH patient. RECENT FINDINGS/RESULTS:This article covers recent literature describing risk factors, predictors of outcome and state-of the art management approach to a hospitalized PAH patients with a special focus on management of RV failure and common complications in PAH requiring hospitalization. SUMMARY/CONCLUSIONS:The review highlights the importance of multidisciplinary approach to a hospitalized PAH patient and highlight important implications in clinical practice and knowledge gaps for potential future research.
PMID: 37435683
ISSN: 1531-6971
CID: 5537592

Undiagnosed ventricular septal defect with resultant Eisenmenger syndrome presenting with diplopia [Case Report]

Duncan, Ellen; Small, Adam; Sulica, Roxana; Halpern, Dan
Ventricular septal defect (VSD) is the most common congenital heart lesion among children. In most cases, however, it is identified and corrected in childhood, before long-term sequelae such as pulmonary hypertension develop. In this case report, we present a young man with an undiagnosed VSD with consequent Eisenmenger syndrome who initially presented to medical attention with diplopia found to be caused by cerebral infarcts.
PMID: 36456362
ISSN: 1532-8171
CID: 5374132

Long-term impact of add-on sequential triple combination therapy in pulmonary arterial hypertension: real world experience

Deshwal, Himanshu; Weinstein, Tatiana; Salyer, Rachel; Thompson, Jesse; Cefali, Frank; Fenton, Rebecca; Bondarsky, Eric; Sulica, Roxana
BACKGROUND:Sequential triple combination therapy is recommended for pulmonary arterial hypertension (PAH) patients who are not at therapeutic goal on dual therapy, but long-term data on efficacy and safety is scarce. OBJECTIVE:To assess the long-term impact of sequential triple combination therapy in patients with PAH who are not at goal on dual combination therapy. STUDY DESIGN AND METHODS:We performed a retrospective observational study in a racially/ethnically diverse cohort of consecutive PAH patients on a stable dual therapy regimen who remained in intermediate- or high-risk category and were subsequently initiated on sequential triple combination therapy. We studied interval change in functional, echocardiographic, and hemodynamic parameters, REVEAL 2.0 risk category and ERS/ESC 2022 simplified four-strata risk category. Multivariate logistic regression analysis was performed to identify independent predictors of successful risk reduction (achievement or maintenance of REVEAL 2.0 low-risk category). Kaplan-Meier survival curves were created to assess the effect of risk reduction on survival. RESULTS: < 0.001), six-minute walk distance (35 m) with 61.8% of patients achieving low-risk status by REVEAL 2.0, and a 28% of patients' improvement in pulmonary vascular resistance. Female gender was identified as a strong predictor of successful risk reduction, whereas Hispanic ethnicity estimated right atrial pressure on echocardiogram and pericardial effusion predicted lower probability of risk reduction. Patients who achieved or maintained low-risk status had significantly improved survival. CONCLUSION:Add-on sequential triple combination therapy significantly increased functional, echocardiographic, and hemodynamic parameters with improvement in risk category and survival.
PMID: 37795626
ISSN: 1753-4666
CID: 5634842

Delphi consensus recommendation for optimization of pulmonary hypertension therapy focusing on switching from a phosphodiesterase 5 inhibitor to riociguat

Rahaghi, Franck F; Balasubramanian, Vijay P; Bourge, Robert C; Burger, Charles D; Chakinala, Murali M; Eggert, Michael S; Elwing, Jean M; Feldman, Jeremy; King, Christopher; Klinger, James R; Mathai, Stephen C; McConnell, John Wesley; Palevsky, Harold I; Restrepo-Jaramillo, Ricardo; Safdar, Zeenat; Sager, Jeffrey S; Sood, Namita; Sulica, Roxana; White, R James; Hill, Nicholas S
Dual combination therapy with a phosphodiesterase-5 inhibitor (PDE5i) and endothelin receptor antagonist is recommended for most patients with intermediate-risk pulmonary arterial hypertension (PAH). The RESPITE and REPLACE studies suggest that switching from a PDE5i to a soluble guanylate cyclase (sGC) activator may provide clinical improvement in this situation. The optimal approach to escalation or transition of therapy in this or other scenarios is not well defined. We developed an expert consensus statement on the transition to sGC and other treatment escalations and transitions in PAH using a modified Delphi process. The Delphi process used a panel of 20 physicians with expertise in PAH. Panelists answered three questionnaires on the management of treatment escalations and transitions in PAH. The initial questionnaire included open-ended questions. Later questionnaires consolidated the responses into statements that panelists rated on a Likert scale from -5 (strongly disagree) to +5 (strongly agree) to determine consensus. The Delphi process produced several consensus recommendations. Escalation should be considered for patients who are at high risk or not achieving treatment goals, by adding an agent from a new class, switching from oral to parenteral prostacyclins, or increasing the dose. Switching to a new class or within a class should be considered if tolerability or other considerations unrelated to efficacy are affecting adherence. Switching from a PDE5i to an SGC activator may benefit patients with intermediate risk who are not improving on their present therapy. These consensus-based recommendations may be helpful to clinicians and beneficial for patients when evidence-based guidance is unavailable.
PMID: 35514769
ISSN: 2045-8932
CID: 5216412

Advances in the management of pulmonary arterial hypertension

Deshwal, Himanshu; Weinstein, Tatiana; Sulica, Roxana
The management of pulmonary arterial hypertension (PAH) has significantly evolved over the last decades in the wake of more sensitive diagnostics and specialized clinical programs that can provide focused medical care. In the current era of PAH care, 1-year survival rates have increased to 86%-90% from 65% in the 1980s, and average long-term survival has increased to 6 years from 2.8 years. The heterogeneity in the etiology and disease course has opened doors to focusing research in phenotyping the disease and understanding the pathophysiology at a cellular and genetic level. This may eventually lead to precision medicine and the development of medications that may prevent or reverse pulmonary vascular remodeling. With more insight, clinical trial designs and primary end-points may change to identify the true survival benefit of pharmacotherapy. Identifying responders from non-responders to therapy may help provide individualized patient-centered care rather than an algorithm-based approach. The purpose of this review is to highlight the latest advances in screening, diagnosis, and management of PAH.
PMID: 34580123
ISSN: 1708-8267
CID: 5061602

Cor Pulmonale from Concomitant Human Immunodeficiency Virus Infection and Methamphetamine Use [Case Report]

Maidman, Samuel D; Sulica, Roxana; Freedberg, Robin S; Bamira, Daniel; Vainrib, Alan F; Ro, Richard; Latson, Larry A; Saric, Muhamed
PMID: 34430775
ISSN: 2468-6441
CID: 5148322

COVID-19 in Pulmonary Artery Hypertension (PAH) Patients: Observations from a Large PAH Center in New York City

Sulica, Roxana; Cefali, Frank; Motschwiller, Caroline; Fenton, Rebecca; Barroso, Anabela; Sterman, Daniel
Information on outcomes of COVID-19 in pulmonary arterial hypertension (PAH) patients is limited to a few case series and surveys. Here, we describe our experience at a large Pulmonary Hypertension Center in New York City at the height of the pandemic. We performed a retrospective chart review of eleven consecutive PAH patients who were diagnosed with SARS-CoV-2 infection. We analyzed demographics, PAH severity, risk factors for COVID-19, and COVID-19 severity and outcomes. We found in our sample that 63.6% of patients required intensive care, and there was a 45.45% overall mortality. Most patients had a known COVID-19 contact and mean duration of symptoms prior to presentation was 12 days. Only 4/11 (36%) patients presented to a center with pulmonary hypertension expertise, all of whom survived. Most patients had at least moderate pulmonary hypertension with an average REVEAL score of 7.81 despite double or triple PAH therapy. Our cases series underscores the gravity of SARS-CoV-2 infection in patients with PAH. It also suggests possible interventions to prevent unfavorable outcomes such as preserving social distancing, PAH management optimization, and early and preferential presentation to a center with specialized expertise in PAH.
PMID: 33467533
ISSN: 2075-4418
CID: 4760522

Response [Letter]

Altschul, Erica; Remy-Jardin, Martine; Machnicki, Stephen; Sulica, Roxana; Raoof, Suhail
PMID: 32386642
ISSN: 1931-3543
CID: 4437892