Curvularia Pneumonia Presenting as a Mass-Like Lesion.

Curvularia species of fungi are ubiquitous and mostly comprise plant or soil residents. Rarely pathogens, they are found in tropical and subtropical climates. On rare occasions, these fungi can be of clinical significance and lead to a variety of disease processes, mainly in immunocompromised individuals. Most infections are limited to allergic fungal rhinosinusitis; however, disseminated disease and invasive infections have been increasingly reported. There exist no therapeutic guidelines for invasive Curvularia infections currently, but amphotericin and various azoles have been used with varying degrees of success. We present a unique case of an immunocompetent 44-year-old female who presented with symptoms concerning for pneumonia and was found to have a mass-like lesion in the lung concerning for malignancy. Biopsy and histopathology of the lesion were consistent with invasive Curvularia pulmonary infection. We reviewed this case in the setting of reported literature concerning Curvularia with an emphasis on the epidemiology, pathology, diagnosis, and emerging management protocols of invasive Curvularia infections.

Racial-ethnic Differences in Reasons for Misuse of Prescription Medications Among US Adults.

OBJECTIVES: This study aims to investigate racial-ethnic differences in reasons for misuse of prescription medications among a nationally representative sample of US adults. METHODS: We analyzed data from the 2015-2019 National Surveys on Drug Use and Health. The study population includes US adults (18-49 years old) who reported misuse of 3 types of prescription drugs (stimulants [n = 6139], sedatives and tranquilizers [n = 5643], and pain relievers [n = 8780]) for 3 reasons: medical-only (eg, to help with pain), recreational-only (eg, to get high), or combined medical and recreational reasons. Multinomial logistic regressions assessed the association between reasons of misuse of prescription medications and self-identified race-ethnicity. RESULTS: Misuse of the 4 types of prescription medications was primarily motivated by medical reasons (63%-80%). Compared to non-Hispanic Whites, non-Hispanic Blacks (nHB), and Hispanics (H) were less likely to report misuse of pain relievers for combined (nHB: adjusted relative risk ratio [aRRR] = 0.6, 95% confidence interval [CI]: 0.4, 0.7; H; aRRR = 0.7, 95% CI: 0.5, 0.9) or recreational reasons (nHB: aRRR = 0.8, 95% CI: 0.6, 1.0; H; aRRR = 0.7, 95% CI: 0.6, 0.9) rather than medical-only reasons. The odds of misuse of sedatives and tranquilizers for recreational-only reasons as opposed to medical-only reasons were higher among nHB (aRRR = 1.9, 95% CI: 1.3, 2.7) and H (aRRR = 1.9, 95% CI: 1.4, 2.4) than among non-Hispanic Whites. CONCLUSIONS: The increased misuse of prescription pain relievers for medical reasons among racial-ethnic minority groups demonstrates a continued need to investigate underlying structural factors driving these behaviors. The higher odds of sedative and tranquilizer misuse for recreational purposes among racial-ethnic minority groups warrant further investigation.

Patient-related factors may influence nursing perception of sleep in the Intensive Care Unit.

OBJECTIVE: There exist conflicting data regarding the accuracy of ICU nurses accurately assessing patient sleep using validated questionnaires. Using the Richards-Campbell Sleep Questionnaire (RSQ), we hypothesize that patient factors might influence nursing perception of their sleep. METHODS: Patients in the ICU who met the inclusion criteria were asked to complete the sleep questionnaire, as were their nurses and intraclass correlation analysis was done. RESULTS: 38 paired patient-nurse surveys were included for analysis. The mean difference in total average score of the RSQ was not significantly different between patients and nurses. There was fair intraclass correlation by patient age, black race, and admission for respiratory illnesses. A good intraclass correlation existed for non-blacks and admission for non-respiratory reasons. Most striking was the intraclass correlation by sex, with poor intraclass correlation for women compared to an excellent correlation for men. CONCLUSION: The results of our study confirm that patients in our ICU have poor sleep with a fair intraclass correlation. When examined by patient related factor, the greatest divergence between patient and nursing perception of sleep in the ICU using the RCSQ was patient female sex. More research is needed in this area to better understand the divergence and improve sleep in the ICU.

Pulmonary Function after Proton Therapy for Hodgkin Lymphoma.

PURPOSE: Acute and late toxicity from chemotherapy, targeted therapy, and radiation therapy can cause significant morbidity among survivors of Hodgkin lymphoma (HL), including pulmonary dysfunction. Improved dosimetry may influence pulmonary function tests (PFTs), an objective and clinically significant measure of pulmonary toxicity. The present study investigates the impact of proton therapy on PFTs among HL survivors. PATIENTS AND METHODS: We monitored 15 patients with mediastinal HL who were enrolled in an institutional HL trial. All patients were treated with combination chemotherapy plus involved-node proton therapy. All patients were to undergo PFTs before starting treatment and at approximately 6 and 12 months after completing proton therapy. RESULTS: Twelve patients were included in the analysis and 3 excluded. The mean forced vital capacity (FVC) was 96.2% ± 16.5% (mean ± SD) predicted at baseline and 98.2% ± 19.4% predicted at 12 months. The mean forced expiratory volume in 1 second (FEV1) was 96.7% ± 17.2% predicted at baseline and 97% ± 15.1% predicted at 12 months. The mean FEV1/FVC ratio was 99.5 ± 8.29 at baseline and 97.8 ± 8.02 at 12 months. The mean diffusing capacity of the lung for carbon monoxide was 81.4% ± 18.4% predicted at baseline and 95.7% ± 23.5% predicted at 12 months. CONCLUSION: No unexpected changes were observed to the lungs as illustrated through follow-up PFTs. Long-term follow-up and validation in a larger cohort are needed.

A case-report of successful use of thromboelastography to manage anticoagulation therapy in a patient with concomitant catastrophic antiphospholipid syndrome and heparin-induced thrombocytopenia.

: Catastrophic antiphospholipid syndrome (CAPS) is a severe but rare form of antiphospholipid syndrome (APS) that results in multiple thrombosis of multiple organs within a week . Similarly, heparin-induced thrombocytopenia (HIT) has been associated with severe and life-threatening thrombosis, both conditions mediated by an autoimmune disorder resulting in a highly thrombotic state . Both conditions requiring aggressive therapeutic anticoagulation when coinciding CAPS complicated by HIT presents a therapeutic challenge. Current recommendations advocate for the use of anti-Xa activity monitoring in the setting of APS because of the common laboratory interaction with commercially available tests, such as prothrombin and activated partial thromboplastin times . With the recommendations to utilize direct thrombin inhibitors (DTI) in the presence of HIT this precludes the possibility of anti-Xa monitoring making for a monitoring predicament. The current case presents a patient where thromboelastography (TEG) was utilized to direct anticoagulation in the setting of concurrent CAPS and HIT without complication.

Impact of unfavorable factors on outcomes among inoperable stage II-IV Nonsmall cell lung cancer patients treated with proton therapy.

PURPOSE: To investigate the impact of unfavorable risk factors among patients with locally advanced nonsmall cell lung cancer (LA-NSCLC) treated with proton therapy (PT). MATERIAL AND METHODS: From May 2008 through July 2015, 90 consecutive patients with unresectable stage II-IV (oligometastatic) NSCLC were treated with PT. Unfavorable factors including age ≥80 years, stage IV, weight loss >10% in 3 months, performance status (PS) ≥2, FEV1 < 1.0 or O2 dependency, prior lung cancer, prior lung surgery, prior 2nd cancer in the past 3 years, and prior chest irradiation were evaluated. All patients received standard fractionation of 1.8-2 Gy(RBE) (median dose, 70 Gy[RBE]). Overall survival (OS) and progression-free survival (PFS) were calculated with the Kaplan-Meier method. The impact of unfavorable factors was analyzed in Cox regression models. RESULTS: Twenty-six percent were favorable-risk, while 42%, 22%, and 10% had 1-, 2-, or ≥3 unfavorable factors. The 2-year OS was 52% and 45% (p = .8522), and 2-year PFS was 21% and 44% (p = .0207), for favorable and unfavorable risk patients, respectively. Among patients with stage III-IV, only PS ≥2 adversely impacted OS (p = .0015). CONCLUSION: Most patients treated with PT for LA-NSCLC have unfavorable risk factors. These patients had similar outcomes to favorable-risk patients. Enrollment in future clinical trials may improve if eligibility is less restrictive.

An unusual case of hypercapnic respiratory failure.

Asphyxiating thoracic dystrophy (ATD also known as Jeune syndrome) is a very rare disorder with an incidence in the United States of 1 case per 100,000-130,000 live births. Chronic alveolar hypoventilation leading to concurrent hypoxia is the main cause of morbidity and mortality in these patients due to its complications. A 22-year-old male with past medical history of ATD and severe kyphoscoliosis presented with progressively worsening shortness of breath for several days. Past surgical history was significant for multiple reconstructive sternal surgeries, his first surgery was at the age of two. His chest exam was without wheezing and was notable for symmetrically decreased breath sounds. Arterial blood gas showed PH 7.17, PCO2 155, PO2 95 and O2 saturation of 97% on 2 L nasal cannulae. PA and lateral chest xrays showed a long and severely narrow thoracic cavity. 2D echocardiography showed left ventricular ejection fraction of 55% and evidence of severe pulmonary hypertension. CT chest angiography showed severe dilatation of the pulmonary artery in comparison to ascending aorta and significant right ventricular enlargement. Right heart catheterization confirmed these findings with a mean pulmonary arterial pressure of 61 mmHg. Within several days of hospitalization, patient developed acute worsening of his chronic hypercapnic respiratory failure thought to be due to worsening of pulmonary arterial hypertension with right heart failure. This in return was attributed to underlying ventilatory failure secondary to severe thoracic dystrophy. ATD is an autosomal recessive genetic disorder. Mutations in the IFT80 gene, which encode for an intraflagellar protein, cause this protein to be defective. Clinically, ATD is characterized by a small, narrow chest and variable limb shortness. While ATD is compatible with life, respiratory failure and infections are often fatal during infancy. Patients that survive past the age of 2 have seen respiratory complications resolve due to less pronounced thoracic malformations, but in our case the main cause of worsening pulmonary function was the degree of pulmonary arterial hypertension and right heart failure. Patients with ATD usually develop progressive hypercapnic respiratory failure due to an abnormally small thorax. Surgical options include lateral thoracic expansion or sternal and chest wall reconstruction. However, these surgeries only add a few years to survival without a definitive cure.

Diagnostic accuracy of echocardiography combined with chest CT in pulmonary hypertension.

BACKGROUND: Transthoracic echocardiography (TTE) and computerized axial tomography (CT) are complementary imaging techniques. It is possible that a combination of the two may offer a better way of identifying pulmonary hypertension (PH) than either one alone. OBJECTIVES: To determine the diagnostic accuracy of TTE combined with chest CT in pulmonary hypertension. METHODS: We performed a retrospective review of consecutive patients who had undergone TTE, CT and right heart catheterization (RHC) between 7/1/2008 and 6/30/2012. PH was defined as systolic pulmonary artery pressure >40 mm Hg or tricuspid regurgitant (TR) jet velocity >2.8m/s on TTE, ratio of diameter of pulmonary artery to ascending aorta (rPA) >1 or diameter of PA (dPA) >30 mm on CT, and mean PAP (mPAP) >25 mm Hg on RHC. RESULTS: There was a total of 87 patients. The mean ± SD age was 54.3 ± 15.9 years and 69 (79%) were female. The prevalence of PH was 75%. The mean ± SD mPAP was 35.8 ± 14.2 mm Hg. The majority of the patients belonged to World Health Organization group I PH. Fifty per cent of the CT scans were done with intravenous contrast dye. The combination of TR jet velocity and rPA provided the best combination of sensitivity (98%) and specificity (70%) with an ROC area under the curve of 0.84. CONCLUSION: The combination of TTE and chest CT is better than either imaging technique alone in identifying patients with PH in a heterogeneous population and may exclude PH.

A case of worsening pulmonary arterial hypertension and pleural effusions by bosutinib after prior treatment with dasatinib.

A 52-year-old man with a past medical history of chronic myeloid leukemia (CML) in remission developed progressive shortness of breath over a two-month period. He was initially treated with dasatinib for four years, until developing pulmonary arterial hypertension (PAH) with pleural effusions. His symptoms improved after stopping dasatinib. He was then switched to bosutinib for approximately one year, which was then stopped before admission due to worsening shortness of breath. His initial workup showed bilateral pleural effusions with severe PAH and cor pulmonale. He had symptomatic improvement with PAH-specific therapy following discontinuation of the bosutinib. The life expectancy of CML patients has increased in the era of the tyrosine kinase inhibitors (TKIs), and managing adverse events (AEs) of the TKIs and improving quality of life are becoming more important. Pulmonary hypertension (PH) and pleural effusions are rarely reported AEs of bosutinib. More reports with PH and pleural effusions arising after bosutinib use in patients previously treated with dasatinib is furthermore concerning. In this era with novel chemotherapeutic agents, physicians ought to be weary of the significant morbidity implicated by these agents in the lives of patients.

Association between aspirin use and deep venous thrombosis in mechanically ventilated ICU patients.

Deep venous thrombosis (DVT) is common in intensive care unit (ICU) patients. It is often silent and may be complicated by pulmonary embolism and death. Thromboprophylaxis with heparin does not always prevent venous thromboembolism (VTE). Aspirin (ASA) reduces the risk of VTE in surgical and high-risk medical patients but it is unknown if ASA may prevent DVT in mechanically ventilated ICU patients. We performed a retrospective chart review of critically ill patients who received mechanical ventilation for >72 h and underwent venous ultrasonography for suspected DVT between Jan 2012 and Dec 2013. We excluded patients who were on therapeutic doses of anticoagulation or had coagulopathy. We used multivariable logistic regression to evaluate association between aspirin use and DVT during hospitalization. There were 193 patients. The mean ± SD age was 58 ± 15.7 years. Half were male. DVT was found in 49 (25.4%). DVT was found in the first 15 days of hospitalization in 67.3% of the patients. The majority (82.8%) received thromboprophylaxis with unfractionated or low molecular weight heparin. Fifty-six (29%) were on ASA. On multivariable regression analysis, ASA use was associated with a significant reduction in the odds of finding DVT (OR 0.39, 95% CI 0.16-0.94; p = 0.036). DVT is common in mechanically ventilated ICU patients despite the use of thromboprophylaxis. Aspirin may prevent DVT in such patients.

Operator dependent factors implicated in failure of non-invasive positive pressure ventilation (NIPPV) for respiratory failure.

BACKGROUND: Non-invasive Positive Pressure Ventilation (NIPPV) is employed for the management of acute respiratory failure and studies have shown that it can prevent the need for endotracheal intubation, mechanical ventilation and associated complications. Given limited studies evaluating the factors, other than those related patient or underlying disease severity, that may lead to NIPPV failure, we performed this study to gain insight into current practices in terms of utilization of NIPPV and operator dependent factors that may possibly contribute to failure of NIPPV. METHOD: After institutional board review approval a retrospective chart review was performed of consecutive patients who were initiated on and failed NIPPV between January 2009 and December 2009. Data was recorded regarding baseline demographics, admission diagnosis, indications for NIPPV, presence of contraindications, type of NIPPV and initial settings, ABG analysis before and after initiation, whether a titration of the settings was performed or not, operator related factors that may have contributed to failure of NIPPV and clinical outcomes. RESULTS: Among 1095 patients screened, 111 failed NIPPV. The mean age was 60 years with 59% males. The most frequent indication for initiating NIPPV was COPD exacerbation (N = 27) followed by pneumonia (N = 26). CPAP was used in 5(6%) patients. Median inspiratory positive airway pressure (IPAP) and expiratory positive airway pressure (EPAP) setting were 10 and 5 cm of H2 O respectively. Three most common reasons for failure were an inappropriate indication (33%), Progression of underlying disease (30%) and lack of titration (23%). Overall mortality was 22%. Mortality was higher when NIPPV failure was seen among patients with an inappropriate indication or an overlooked contraindication compared to those with an appropriate indication (27% vs 17%). CONCLUSIONS: Excluding progression of underlying disease, operator dependent factors linked to NIPPV failure are; inappropriate indication, lack of adequate titration and an overlooked contraindication. Inappropriate utilization of NIPPV in respiratory failure is associated with higher mortality.

Skin rash in the intensive care unit: Stevens-Johnson syndrome, toxic epidermal necrolysis, or a rare manifestation of a hidden cutaneous malignancy: A case report.

Skin rashes are infrequently encountered in the intensive care units, either as a result or as a cause of admission. The entities of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) form a spectrum of desquamating skin diseases that have multiple etiologies, the most common being drug-related reactions; very rarely, the cause may be cutaneous malignancies. We herein present a unique case of a 54-year-old male patient with psoriasis treated with methotrexate, who presented with a cellulitis-like clinical picture, then developed a severe progressive systemic inflammatory response syndrome, and progressed clinically to SJS, then TEN even after discontinuing the antibiotics and methotrexate. A skin biopsy demonstrated an aggressive and rapidly-progressing T-cell lymphoma. The present case highlights the necessity of skin biopsy when encountering SJS and TEN in the ICU in order to identify potentially treatable/controllable causes. Although it appeared reasonable to correlate TEN solely to medications, the skin biopsies clearly demonstrated an aggressive T-cell skin lymphoma. In a patient with a better general condition it may have been helpful to treat this malignancy. TEN is a life-threatening condition and skin biopsy is the cornerstone of diagnosis, despite the presence of multiple risk factors and the typical physical findings of a drug-induced reaction.

A Phase 2 Trial of Concurrent Chemotherapy and Proton Therapy for Stage III Non-Small Cell Lung Cancer: Results and Reflections Following Early Closure of a Single-Institution Study.

PURPOSE: Proton therapy has been shown to reduce radiation dose to organs at risk (OAR) and could be used to safely escalate the radiation dose. We analyzed outcomes in a group of phase 2 study patients treated with dose-escalated proton therapy with concurrent chemotherapy for stage 3 non-small cell lung cancer (NSCLC). METHODS AND MATERIALS: From 2009 through 2013, LU02, a phase 2 trial of proton therapy delivering 74 to 80 Gy at 2 Gy/fraction with concurrent chemotherapy for stage 3 NSCLC, was opened to accrual at our institution. Due to slow accrual and competing trials, the study was closed after just 14 patients (stage IIIA, 9 patients; stage IIIB, 5 patients) were accrued over 4 years. During that same time period, 55 additional stage III patients were treated with high-dose proton therapy, including 7 in multi-institutional proton clinical trials, 4 not enrolled due to physician preference, and 44 who were ineligible based on strict entry criteria. An unknown number of patients were ineligible for enrollment due to insurance coverage issues and thus were treated with photon radiation. Median follow-up of surviving patients was 52 months. RESULTS: Two-year overall survival and progression-free survival rates were 57% and 25%, respectively. Median lengths of overall survival and progression-free survival were 33 months and 14 months, respectively. There were no acute grade 3 toxicities related to proton therapy. Late grade 3 gastrointestinal toxicity and pulmonary toxicity each occurred in 1 patient. CONCLUSIONS: Dose-escalated proton therapy with concurrent chemotherapy was well tolerated with encouraging results among a small cohort of patients. Unfortunately, single-institution proton studies may be difficult to accrue and consideration for pragmatic and/or multicenter trial design should be considered when developing future proton clinical trials.

A Large Pleural Effusion following Abdominal Aortic Surgery.

Chylous ascites and coexistent chylothorax is a rare but important complication following retroperitoneal abdominal surgery. We report a 70-year-old male who developed gradual abdominal distension, chest tightness, and dyspnea five months after having an uncomplicated aortobifemoral bypass performed. Physical examination was consistent with a large right sided effusion and ascites which were confirmed by computed tomography. Thoracentesis yielded an opaque milky fluid with analysis consistent with a chylothorax with a paracentesis revealing fluid that was similar in both appearance and biochemistry. The patient failed initial conservative management so a chest tube was placed followed by chemical pleurodesis. We review the literature of the pathophysiology and treatment approach to such a pleural effusion.

Severe Hyponatremia Due to Valproic Acid Toxicity.

Hyponatremia is a very commonly encountered clinical entity with potentially dangerous effects and for which many precipitating factors have been identified. We present a case of valproic acid (VPA) overdose causing profound hyponatremia, with one of the lowest serum sodium levels ever documented in literature. A 54-year-old woman with hypothyroidism, hypertension and bipolar disorder presented with somnolence after intentionally ingesting 7,500 mg VPA. She was drowsy but easily arousable with no hemodynamic compromise and an unremarkable physical exam. There was no clinical suspicion for organic neurological or pulmonary disease, adrenal insufficiency or volume depletion. She was found to have a serum sodium of 99 mEq/L, low plasma osmolality (211 mOsm/kg H2O), and high urine osmolality (115 mOsm/kg H2O). Her urine sodium was 18 mEq/L. She was euthyroid (TSH: 3.06 mIU/L) and compliant with thyroxine replacement. She was admitted to the intensive care unit for close monitoring and VPA was withheld. Over 36 hours her VPA level fell from 59.3 mg/L to 22.8 mg/L, serum sodium steadily rose to 125 mEq/L and there was concomitant improvement in her mental status. At 72 hours, she was transferred for an inpatient psychiatric evaluation and her sodium level was 135 mEq/L. She luckily did not experience any seizures or decline in neurological function. The clinical presentation in this patient is consistent with the syndrome of inappropriate antidiuretic hormone secretion (SIADH) leading to a dramatic fall in sodium to a level of 99 mEq/L. Chronic VPA use has been associated with SIADH and chronic hyponatremia. Review of records in this patient from 1 year prior revealed that her last measured sodium level was 127 mEq/L. It is therefore most likely that our case is one of acute on chronic hyponatremia provoked by VPA overdose in the setting of chronic VPA use. Whilst our patient's course was relatively benign, this case illustrates a rare consequence of VPA toxicity, which if unnoticed in another patient may be tragic.

Broncholithiasis presenting as bronchiectasis and recurrent pneumonias.

A broncholith is defined as the presence of calcified material within a bronchus or within a cavity communicating with a bronchus. It is most frequently caused by Histoplasmosis or tuberculosis (TB) spp. Bronchial distortion, irritation and erosion by broncholiths can cause bronchiectasis, recurrent pneumonias and haemoptysis. We present a case of recurrent pneumonia due to a broncholith, which resolved conservatively with antibiotics. Owing to recurrent fevers and post obstructive pneumonias, a lobectomy or rigid bronchoscopic removal were considered but the patient was deemed not to be a candidate for general anaesthesia due to her comorbidities. Broncholiths are an uncommon cause of bronchiectasis and recurrent pneumonias. However, the wide range of symptoms and low clinical suspicion are the main reasons why a diagnosis can be delayed. Various treatment options are available and the choice of therapy should be made depending on the broncholith's size, mobility, location and local surgical expertise.

Is There Any Association between PEEP and Upper Extremity DVT?

Background. We hypothesized that positive end-exploratory pressure (PEEP) may promote venous stasis in the upper extremities and predispose to upper extremity deep vein thrombosis (UEDVT). Methods. We performed a retrospective case control study of medical intensive care unit patients who required mechanical ventilation (MV) for >72 hours and underwent duplex ultrasound of their upper veins for suspected DVT between January 2011 and December 2013. Results. UEDVT was found in 32 (28.5%) of 112 patients. Nineteen (67.8%) had a central venous catheter on the same side. The mean ± SD duration of MV was 13.2 ± 9.5 days. Average PEEP was 7.13 ± 2.97 cm H2O. Average PEEP was ≥10 cm H2O in 23 (20.5%) patients. Congestive heart failure (CHF) significantly increased the odds of UEDVT (OR 4.53, 95% CI 1.13-18.11; P = 0.03) whereas longer duration of MV (≥13 vs. <13 days) significantly reduced it (OR 0.29, 95% CI 0.11-0.8; P = 0.02). Morbid obesity showed a trend towards significance (OR 3.82, 95% CI 0.95-15.4; P = 0.06). Neither PEEP nor any of the other analyzed predictors was associated with UEDVT. Conclusions. There is no association between PEEP and UEDVT. CHF may predispose to UEDVT whereas the risk of UEDVT declines with longer duration of MV.

An unusual case of postpartum dyspnea.

A 40-year-old South Asian woman was admitted in active labor at 38 weeks' gestation. She had an unremarkable medical history with routine prenatal care, negative HIV testing results, and an uneventful pregnancy. She received a Bacillus Calmette-Guérin vaccine during childhood and reportedly had a subsequent positive purified protein-derivative test result 1 year prior to conception. She never smoked and had seven normal term pregnancies.

Use of pulmonary arterial hypertension-specific therapy in non-WHO group I pulmonary hypertension.

OBJECTIVES: The development of pulmonary hypertension (PH) in non-World Health Organization group I PH adversely affects exercise capacity. It is unclear whether pulmonary artery hypertension (PAH)-specific drugs improve pulmonary hemodynamics and exercise capacity in such patients. METHODS: We performed a retrospective chart review of consecutive patients with non-World Health Organization group I PH treated with PAH-specific therapy. RESULTS: We identified 24 patients. The mean (standard deviation) age was 48 (14.8) years. Seventeen (71%) patients were women. The 6-minute walk distance improved significantly for the whole group in an initial follow-up period of 4.6 (2.3) months; however, the improvement was seen only in patients with obstructive sleep apnea (OSA) or severe PH and it was not sustained during a longer follow-up period of 11.5 (4.1) months, except in patients with OSA. PH was treated with a variety of PAH-specific drugs, including combination therapy in five patients. CONCLUSIONS: The use of PAH-specific therapy in selected patients with PH secondary to lung diseases, OSA, or sarcoidosis may result in significant improvement in 6-minute walk distance, particularly in patients with OSA or severe PH.

Predicting the Need for Upfront Combination Therapy in Pulmonary Arterial Hypertension.

BACKGROUND: Combination therapy is commonly used for pulmonary arterial hypertension (PAH) treatment. We aimed to identify factors that may predict the need for future combination therapy. METHODS: We conducted a retrospective chart review of consecutive patients with PAH in an aim to describe baseline clinical, echocardiogram, and hemodynamic characteristics of patients who eventually required combination therapy during the course of their disease and compared them to the ones who were maintained on monotherapy. RESULTS: The monotherapy group was followed for an average of 31.8 ± 18.8 months and the combination therapy group was followed for an average of 28.7 ± 13.6 months. Among the 71 patients analyzed, a significantly higher number of patients who eventually required combination therapy belonged to World Health Organization functional class 3 (45% vs 37%) and 4 (23% vs 0) at baseline, compared with those on monotherapy (P < .05). Combination group also had a higher Registry to Evaluate Early And Long-term PAH Disease Management (REVEAL) PAH risk score at presentation. End of 6-minute walk test (6MWT), oxygen saturation (Spo 2) was also lower in the combination therapy group, 86% ± 8% versus 91% ± 7% (P < .05). Patients who eventually required combination therapy were more frequently noticed to have right ventricular enlargement, right atrial enlargement, and had a higher resting estimated right ventricular systolic pressure (RVSP). Right heart catheterization-derived hemodynamics data at baseline showed that the combination therapy group had a higher mean pulmonary artery (PA) pressure, lower pulmonary capillary wedge pressure, lower cardiac output, and higher pulmonary vascular resistance (PVR). On univariate analysis, only PVR ≥300 dyne·s/cm(5), mean PA pressure of ≥40 mm Hg, estimated RVSP ≥ 60 mm Hg, PAH risk score ≥ 10, and end of 6MWT saturation of ≤ 90% were of significance. CONCLUSION: Patients with PAH who require combination therapy in the course of their disease have worse hemodynamics, PAH risk score, functional class, and end of 6MWT oxygen saturation at the time of presentation compared to patients maintained on monotherapy.