Browsing by Subject "Hypertension, Pulmonary"
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Item Open Access A Multifaceted Approach to Pulmonary Hypertension in Adults With Congenital Heart Disease.(Progress in cardiovascular diseases, 2018-09) Fathallah, Mouhammad; Krasuski, Richard AAdvances in the management of congenital heart disease (CHD) in children have resulted in growing numbers of adults with CHD. Pulmonary arterial hypertension related to CHD (PAH-CHD) is a common complication, affecting up to 10% of patients; and can arise even after successful and complete defect repair, with severe and potentially fatal consequences. Careful work-up in these patients is essential, particularly hemodynamic assessment, and can help define the most appropriate therapeutic approach. Management can be challenging, but the therapeutic armamentarium is continually expanding and now includes surgical, transcatheter and medical options. Timely correction of defects along with early treatment with advanced medical therapies appears to improve quality of life and possible even improve survival. Interestingly most studies of PAH-CHD have focused on its most severely afflicted patients, those with Eisenmenger Syndrome, making it less certain how to manage PAH-CHD of milder degrees. This review summarizes our current understanding of PAH-CHD and emphasizes the need for close follow-up in specialized centers of care where close collaboration is common practice.Item Open Access Association of anemia and long-term survival in patients with pulmonary hypertension.(Int J Cardiol, 2011-08-04) Krasuski, Richard A; Hart, Stephen A; Smith, Brad; Wang, Andrew; Harrison, J Kevin; Bashore, Thomas MBACKGROUND: Anemia is a marker of worsened clinical outcome in patients with heart failure from left ventricular dysfunction. Pulmonary hypertension often results in right ventricular dysfunction. Accordingly we sought to examine the association of hemoglobin levels and long-term all-cause mortality in a cohort of patients with pulmonary hypertension. METHODS: Baseline demographic information, clinical characteristics and fasting blood work were obtained in a cohort of 145 patients with pulmonary hypertension referred for pulmonary vasodilator testing. Data was retrospectively analyzed with Cox-proportional hazards analysis. RESULTS: Baseline characteristics of the cohort included age (mean±SD) 55.8±14.6 years, 75% women, 50% with idiopathic pulmonary hypertension, mean pulmonary artery pressure 46.1±14.2 mm Hg and arterial O(2) saturation 91±6 %. The most commonly utilized pulmonary hypertension specific therapeutic agents in descending order of frequency were epoprostenol (27%), sildenafil (21%), bosentan (17%), and treprostinil (6%). Over a median follow-up of 2.1 years, there were 39 deaths (26.9%). Patients who died had significantly lower hemoglobin levels than those survived (12.2±2.3 vs. 13.7±2.0, p<0.001). After adjustment for known predictors of death and pulmonary hypertension etiology, anemic patients were 3.3 times more likely to die than non-anemic patients (95% CI [1.43-7.51], p=0.005). CONCLUSIONS: Hemoglobin levels closely parallel survival in pulmonary hypertension. Modification of anemia in this disorder could alter the clinical course and calls for further research in this area.Item Open Access Congenital heart disease and pulmonary hypertension.(Heart Fail Clin, 2012-07) Gupta, Vedant; Tonelli, Adriano R; Krasuski, Richard AMany patients with congenital heart disease and systemic-to-pulmonary shunts develop pulmonary arterial hypertension (PAH), particularly if the cardiac defect is left unrepaired. A persistent increase in pulmonary blood flow may lead to obstructive arteriopathy and increased pulmonary vascular resistance, a condition that can lead to reversal of shunt and cyanosis (Eisenmenger syndrome). Cardiac catheterization is crucial to confirm diagnosis and facilitate treatment. Bosentan is the only medication to date to be compared with placebo in a randomized controlled trial specifically targeting congenital heart disease-associated PAH. Lung transplantation with repair of the cardiac defect or combined heart-lung transplantation is reserved for recalcitrant cases.Item Open Access Effect of obesity on B-type natriuretic peptide levels in patients with pulmonary arterial hypertension.(Am J Cardiol, 2012-09-15) Batal, Omar; Faulx, Michael; Krasuski, Richard A; Khatib, Omar F; Hammel, Jeff P; Hussein, Ayman A; Minai, Omar A; Dweik, Raed ABrain natriuretic peptide (BNP) levels are lower in obese patients with left ventricular failure than in their comparably ill, leaner counterparts. The effect of obesity on BNP in patients with pulmonary arterial hypertension (PAH) is unknown. We reviewed our prospective PAH registry data collected from November 2001 to December 2007 for patients undergoing right heart catheterization who met the criteria for PAH and had the BNP level and body mass index determined at baseline. The median BNP level for the lean, overweight, and obese patients was 285 pg/ml (interquartile range 131 to 548), 315 pg/ml (interquartile range 88 to 531), and 117 pg/ml (interquartile range 58 to 270), respectively (p = 0.029). A greater body mass index was associated with a lower BNP level, adjusted for age, gender, New York Heart Association functional class, hypertension, coronary artery disease, and mean right atrial and pulmonary arterial pressures (p <0.001). No statistically significant differences were found among the groups in age, race, medical co-morbidities, underlying etiology of PAH, use of vasoactive medications, New York Heart Association functional class, echocardiographic parameters, or pulmonary function. Obese patients had greater right atrial and pulmonary artery pressures. Increased BNP was associated with worse survival in the lean and overweight patients only. In conclusion, the BNP levels are attenuated in obese patients with PAH despite similar or worse hemodynamics or functional class compared to lean or overweight patients and should therefore be interpreted with caution.Item Open Access Evaluation and Management of Pulmonary Hypertension in Noncardiac Surgery: A Scientific Statement From the American Heart Association.(Circulation, 2023-04) Rajagopal, Sudarshan; Ruetzler, Kurt; Ghadimi, Kamrouz; Horn, Evelyn M; Kelava, Marta; Kudelko, Kristina T; Moreno-Duarte, Ingrid; Preston, Ioana; Rose Bovino, Leonie L; Smilowitz, Nathaniel R; Vaidya, Anjali; American Heart Association Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation, and the Council on Cardiovascular and Stroke NursingPulmonary hypertension, defined as an elevation in blood pressure in the pulmonary arteries, is associated with an increased risk of death. The prevalence of pulmonary hypertension is increasing, with an aging population, a rising prevalence of heart and lung disease, and improved pulmonary hypertension survival with targeted therapies. Patients with pulmonary hypertension frequently require noncardiac surgery, although pulmonary hypertension is associated with excess perioperative morbidity and death. This scientific statement provides guidance on the evaluation and management of pulmonary hypertension in patients undergoing noncardiac surgery. We advocate for a multistep process focused on (1) classification of pulmonary hypertension group to define the underlying pathology; (2) preoperative risk assessment that will guide surgical decision-making; (3) pulmonary hypertension optimization before surgery to reduce perioperative risk; (4) intraoperative management of pulmonary hypertension to avoid right ventricular dysfunction and to maintain cardiac output; and (5) postoperative management of pulmonary hypertension to ensure recovery from surgery. Last, this scientific statement highlights the paucity of evidence to support perioperative pulmonary hypertension management and identifies areas of uncertainty and opportunities for future investigation.Item Open Access In-hospital outcomes of premature infants with severe bronchopulmonary dysplasia.(Journal of perinatology : official journal of the California Perinatal Association, 2017-07) Jackson, W; Hornik, CP; Messina, JA; Guglielmo, K; Watwe, A; Delancy, G; Valdez, A; MacArthur, T; Peter-Wohl, S; Smith, PB; Tolia, VN; Laughon, MMOBJECTIVE:To characterize in-hospital outcomes of premature infants diagnosed with severe bronchopulmonary dysplasia (BPD). STUDY DESIGN:Retrospective cohort study including premature infants with severe BPD discharged from 348 Pediatrix Medical Group neonatal intensive care units from 1997 to 2015. RESULTS:There were 10 752 infants with severe BPD, and 549/10 752 (5%) died before discharge. Infants who died were more likely to be male, small for gestational age, have received more medical interventions and more frequently diagnosed with surgical necrotizing enterocolitis, culture-proven sepsis and pulmonary hypertension following 36 weeks of postmenstrual age compared with survivors. Approximately 70% of infants with severe BPD were discharged by 44 weeks of postmenstrual age, and 86% were discharged by 48 weeks of postmenstrual age. CONCLUSIONS:A majority of infants diagnosed with severe BPD were discharged home by 44 weeks of postmenstrual age. These results may inform discussions with families regarding the expected hospital course of infants diagnosed with severe BPD.Item Open Access Low-density lipoprotein cholesterol and survival in pulmonary arterial hypertension.(Scientific reports, 2017-02-15) Kopeć, Grzegorz; Waligóra, Marcin; Tyrka, Anna; Jonas, Kamil; Pencina, Michael J; Zdrojewski, Tomasz; Moertl, Deddo; Stokwiszewski, Jakub; Zagożdżon, Paweł; Podolec, PiotrLow-density lipoprotein cholesterol(LDL-C) is a well established metabolic marker of cardiovascular risk, however, its role in pulmonary arterial hypertension (PAH) has not been determined. Therefore we assessed whether LDL-C levels are altered in PAH patients, if they are associated with survival in this group and whether pulmonary hypertension (PH) reversal can influence LDL-C levels. Consecutive 46 PAH males and 94 females were age matched with a representative sample of 1168 males and 1245 females, respectively. Cox regression models were used to assess the association between LDL-C and mortality. The effect of PH reversal on LDL-C levels was assessed in 34 patients with chronic thromboembolic pulmonary hypertension (CTEPH) undergoing invasive treatment. LDL-C was lower in both PAH (2.6 ± 0.8 mmol/l) and CTEPH (2.7 ± 0.7 mmol/l) patients when compared to controls (3.2 ± 1.1 mmol/l, p < 0.001). In PAH patients lower LDL-C significantly predicted death (HR:0.44/1 mmol/l, 95%CI:0.26-0.74, p = 0.002) after a median follow-up time of 33(21-36) months. In the CTEPH group, LDL-C increased (from 2.6[2.1-3.2] to 4.0[2.8-4.9]mmol/l, p = 0.01) in patients with PH reversal but remained unchanged in other patients (2.4[2.2-2.7] vs 2.3[2.1-2.5]mmol/l, p = 0.51). We concluded that LDL-C level is low in patients with PAH and is associated with an increased risk of death. Reversal of PH increases LDL-C levels.Item Open Access Mesenchymal Stem Cell-derived Extracellular Vesicles Prevent Experimental Bronchopulmonary Dysplasia Complicated By Pulmonary Hypertension.(Stem cells translational medicine, 2022-08) Sharma, Mayank; Bellio, Michael A; Benny, Merline; Kulandavelu, Shathiyah; Chen, Pingping; Janjindamai, Chawisa; Han, Chenxu; Chang, Liming; Sterling, Shanique; Williams, Kevin; Damianos, Andreas; Batlahally, Sunil; Kelly, Kaitlyn; Aguilar-Caballero, Daniela; Zambrano, Ronald; Chen, Shaoyi; Huang, Jian; Wu, Shu; Hare, Joshua M; Schmidt, Augusto; Khan, Aisha; Young, KarenMesenchymal stem cell (MSC) extracellular vesicles (EVs) have beneficial effects in preclinical bronchopulmonary dysplasia and pulmonary hypertension (BPD-PH) models. The optimal source, dosing, route, and duration of effects are however unknown. The objectives of this study were to (a) compare the efficacy of GMP-grade EVs obtained from Wharton's Jelly MSCs (WJ-MSCs) and bone marrow (BM-MSCs), (b) determine the optimal dosing and route of administration, (c) evaluate its long-term effects, and (d) determine how MSC EVs alter the lung transcriptome. Newborn rats exposed to normoxia or hyperoxia (85% O2) from postnatal day (P)1-P14 were given (a) intra-tracheal (IT) BM or WJ-MSC EVs or placebo, (b) varying doses of IT WJ-MSC EVs, or (c) IT or intravenous (IV) WJ-MSC EVs on P3. Rats were evaluated at P14 or 3 months. Early administration of IT BM-MSC or WJ-MSC EVs had similar beneficial effects on lung structure and PH in hyperoxia-exposed rats. WJ-MSC EVs however had superior effects on cardiac remodeling. Low, medium, and high dose WJ-MSC EVs had similar cardiopulmonary regenerative effects. IT and IV WJ-MSC EVs similarly improved vascular density and reduced PH in hyperoxic rats. Gene-set enrichment analysis of transcripts differentially expressed in WJ-MSC EV-treated rats showed that induced transcripts were associated with angiogenesis. Long-term studies demonstrated that a single early MSC EV dose has pulmonary vascular protective effects 3 months after administration. Together, our findings have significant translational implications as it provides critical insight into the optimal source, dosing, route, mechanisms of action, and duration of effects of MSC-EVs for BPD-PH.Item Open Access Partial anomalous pulmonary venous connection and pulmonary arterial hypertension.(Respirology, 2012-08) Sahay, Sandeep; Krasuski, Richard A; Tonelli, Adriano RBACKGROUND AND OBJECTIVE: Isolated partial anomalous pulmonary venous connection (PAPVC) has been implicated as a cause of pulmonary arterial hypertension (PAH); however this condition is often overlooked in the diagnostic work up of patients with PH. We studied the prevalence of PAH both in patients with isolated PAPVC or associated with other congenital heart diseases (CHD) such as atrial septal defect (ASD). We also aimed to identify factors related to the presence of PAH in these patients. METHODS: We retrospectively analyzed data from the Adult CHD database at the Cleveland Clinic, U.S.A. between October 2005-2010. We included all patients diagnosed with PAPVC with or without other CHD. We excluded all patients with previous corrective surgeries. RESULTS: We identified 14 (2.5%) patients with PAPVC. Group I included patients with PAPVC (with or without patent foramen ovale (PFO)). Group II included patients with PAPVC associated with other CHD. PAH was seen in six (6/14, 42.8%) patients, two (2/7, 28.5%) in group I and four (4/7, 57.1%) in group II (P = 0.3). The mean pulmonary artery pressure in all patients (n = 14) was 29.5 ± 13.8 mm Hg. group I had a mean PAP of 23.6 ± 6.6 mm Hg as compared to 33.7 ± 16.5 mm Hg for group II (P = 0.34). The two patients in group I with PAH had either two anomalous pulmonary veins or a condition (sickle cell disease) that could potentially explain the haemodynamic findings. CONCLUSIONS: Patients with PAPVC (with or without PFO) in the absence of other CHD had normal pulmonary arterial pressure (PAP) unless they have two pulmonary veins with anomalous return or associated conditions known to cause PAH.Item Open Access Pharmacologic management of perioperative pulmonary hypertension.(J Cardiovasc Pharmacol, 2014-04) Cheng, Julie W; Tonelli, Adriano R; Pettersson, Gosta; Krasuski, Richard APerioperative pulmonary hypertension can originate from an established disease or acutely develop within the surgical setting. Patients with increased pulmonary vascular resistance are consequently at greater risk for complications. Despite the various specific therapies available, the ideal therapeutic approach in this patient population is not currently clear. This article describes the basic principles of perioperative pulmonary hypertension and reviews the different classes of agents used to promote pulmonary vasodilation in the surgical setting.Item Open Access Pulmonary hypertension and elevated transpulmonary gradient in patients with mitral stenosis.(J Heart Valve Dis, 2010-11) Hart, Stephen A; Krasuski, Richard A; Wang, Andrew; Kisslo, Katherine; Harrison, J Kevin; Bashore, Thomas MBACKGROUND AND AIM OF THE STUDY: Pulmonary hypertension frequently complicates mitral stenosis, with a subset of these patients exhibiting pressures well in excess of their mitral valve hemodynamics. The prevalence of this condition and its impact on clinical outcome following percutaneous balloon mitral commissurotomy (PBMC) is unknown. METHODS: The transpulmonary gradient (TPG) was measured in 317 patients undergoing PBMC; patients were subsequently defined as having either an appropriate or excessive TPG (< or =15 mmHg or >15 mmHg, respectively). Twenty-two patients were excluded due to valvuloplasty-related significant mitral regurgitation. The remaining 295 patients (250 females, 45 males; mean age 52 +/- 13 years) were prospectively followed up, with each patient underwent serial echocardiography. RESULTS: Among the patients, 214 (73%) had pulmonary hypertension (pulmonary artery pressure >25 mmHg) and 55 (19%) also had an elevated TPG. Females were almost fivefold more likely than males to have an elevated TPG (p = 0.003). Patients with an elevated TPG had a worse mean NYHA functional class than those with a normal TPG (3.0 +/- 0.5 versus 2.7 +/- 0.6, p = 0.01), while the mitral valve area (MVA) was slightly smaller in patients with an elevated TPG (1.0 +/- 0.2 versus 1.1 +/- 0.2 cm2, p = 0.003). All patients demonstrated a significant increase in MVA after commissurotomy (final MVA 1.7 +/- 0.6 cm2, p < 0.001 for elevated TPG; 1.8 +/- 0.4 cm2, p < 0.001 for normal TPG), and the NYHA class at six months was improved for all patients (2.8 +/- 0.6 versus 1.6 +/- 0.7, p < 0.001). The improvements in NYHA class, TPG and MVA were sustained at 36 months. CONCLUSION: Pulmonary hypertension with elevated TPG occurs in patients with mitral stenosis, and is significantly more common in females. Despite worse symptoms and higher right-sided pressures, PBMC is equally successful in patients with a normal TPG, and provides sustained benefit for up to 36 months after the procedure.Item Open Access Response to inhaled nitric oxide predicts survival in patients with pulmonary hypertension.(J Card Fail, 2011-04) Krasuski, Richard A; Devendra, Ganesh P; Hart, Stephen A; Wang, Andrew; Harrison, J Kevin; Bashore, Thomas MOBJECTIVE: To examine the ability of vasodilator response to predict survival in a diverse cohort of patients with pulmonary hypertension (PH). PATIENTS & METHODS: A total of 214 consecutive treatment-naive patients referred for invasive PH evaluation were enrolled between November 1998 and December 2008. Vasoreactivity was assessed during inhalation of 40 parts per million nitric oxide (iNO) and vasodilator responders were defined as those participants who achieved a mean pulmonary artery pressure (PAP) of ≤ 40 mm Hg and a drop in mean PAP ≥ the median for the cohort (13%). Kaplan-Meier analysis and Cox proportional hazards modeling were used to identify predictors of survival. RESULTS: There were 51 deaths (25.9%) over a mean follow-up period of 2.3 years. Kaplan-Meier analysis demonstrated that vasodilator responders had significantly improved survival (P < .01). Vasodilator responders had improved survival regardless of whether or not they had idiopathic or nonidiopathic PH (P = .02, P < .01) or whether or not they had Dana Point class 1 or non-Dana Point class 1 PH (P < .01, P = .01). In multivariate modeling, advanced age, elevated right atrial pressure, elevated serum creatinine, and worsened functional class significantly predicted shorter survival (P = .01, P = .01, P = .01, P < .01), whereas vasodilator response predicted improved survival (P = .01). CONCLUSIONS: Vasodilator responsiveness to iNO is an important method of risk stratifying PH patients, with results that apply regardless of clinical etiology.Item Open Access Right-sided heart catheterization: de rigueur in sarcoidosis?(Chest, 2010-11) Culver, Daniel A; Krasuski, Richard AItem Open Access Treatment-related biomarkers in pulmonary hypertension.(Am J Respir Cell Mol Biol, 2015-06) Swaminathan, Aparna C; Dusek, Alex C; McMahon, Tim JSignificant advances in the treatment of pulmonary arterial hypertension (PAH) over the last two decades have led to the introduction of multiple classes of oral therapy, but the disease remains devastating for many patients. Disease progression, in spite of oral monotherapy, is a major problem, and alternative therapy, such as infusion of prostacyclins, is cumbersome and carries considerable potential morbidity. Use of combination oral therapy, including drugs from both the endothelin receptor antagonist and phosphodiesterase-5 inhibitor classes, has increased, and there is some evidence to support this approach. Given the multiple options now available in pulmonary hypertension (PH) therapy, biomarkers to guide treatment decisions could be helpful. Here, we review the evidence for and against the clinical use of molecular biomarkers relevant to PH pathogenesis, emphasizing assayable markers that may also inform more rational selection of agents that influence pathways targeted by treatment. We emphasize the interactive nature of changes in mediators and messengers, such as endothelin-1, prostacyclin, brain natriuretic peptide (which has demonstrated biomarker utility), nitric oxide derivatives, and cyclic guanosine monophosphate, which play important roles in processes central to progression of PAH, such as vascular remodeling, vasoconstriction, and maladaptive right ventricular changes, and are relevant to its therapy. Accordingly, we propose that the identification and use of a molecular biomarker panel that assays these molecules in parallel and serially might, if validated, better inform unique patient phenotypes, prognosis, and the rational selection and titration of combination oral and other therapy in individual patients with PH/PAH.Item Open Access Two Cases of Late Shone Syndrome With Pulmonary Hypertension: Heart-Lung Transplant or Valve Surgery?(World J Pediatr Congenit Heart Surg, 2016-01) Robich, Michael P; Stewart, Robert D; Zahka, Kenneth G; Krasuski, Richard A; Hanna, Mazen; Blackstone, Eugene H; Pettersson, Gosta BTwo cases of Shone syndrome with severe mitral and aortic valve problems and pulmonary hypertension were referred for heart-lung transplantation. Severely elevated pulmonary vascular resistance (PVR) was confirmed as was severe periprosthetic mitral and aortic regurgitation. Based on the severity of the valve lesions in both patients, surgery was decided upon and undertaken. Both experienced early pulmonary hypertensive crises, one more than the other, that gradually subsided, followed by excellent recovery and reversal of pulmonary hypertension and PVR. These cases illustrate Braunwald's concept that pulmonary hypertension secondary to left-sided valve disease is reversible.