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Cardiogenic shock (CS) is a heterogeneous clinical syndrome characterized by low cardiac output leading to end-organ hypoperfusion. Organ dysoxia ranging from transient organ injury to irreversible organ failure and death occurs across all CS etiologies but differing by incidence and type. Herein, we review the recognition and management of respiratory, renal and hepatic failure complicating CS. We also discuss unmet needs in the CS care pathway and future research priorities for generating evidence-based best practices for the management of extra-cardiac sequelae. The complexity of CS admitted to the contemporary cardiac intensive care unit demands a workforce skilled to care for these extra-cardiac critical illness complications with an appreciation for how cardio-systemic interactions influence critical illness outcomes in afflicted patients.
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Unidades de Terapia Intensiva , Choque Cardiogênico , Humanos , Choque Cardiogênico/terapia , Choque Cardiogênico/etiologia , Cuidados Críticos/métodos , Insuficiência Respiratória/terapia , Insuficiência Respiratória/etiologiaRESUMO
Pulmonary hypertension in sickle cell disease (SCD) is a complex phenomenon resulting from multiple overlapping etiologies, including pulmonary vasoconstriction in the setting of chronic hemolytic anemia, diastolic dysfunction, and chronic thromboembolic disease. The presence of pulmonary hypertension of any cause in SCD confers a significant increase in mortality risk. Evidence to guide the management of patients with sickle cell disease and chronic thromboembolic pulmonary hypertension (CTEPH) is scant and largely the realm of case reports and small case series. Centered on a discussion of a complex young patient with hemoglobin hemoglobin SC who ultimately underwent treatment with pulmonary thromboendarterectomy, we review the available literature to guide management and discuss and overview of treatment of CTEPH in SCD, considering the unique considerations and challenges facing patients suffering from this multisystem disease.
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Background: Pelvic venous obstruction (PVO), defined as greater than 50% stenosis or occlusion of pelvic veins, is a known risk factor for deep vein thrombosis (DVT). DVT is a known risk factor for chronic thromboembolic pulmonary hypertension (CTEPH), but the prevalence of PVO in CTEPH is unknown. Methods: This cross-sectional study at Temple University's tertiary referral center for Pulmonary Hypertension, Right Heart Failure, and CTEPH sought to identify the presence of PVO in patients with CTEPH who underwent cardiac catheterization, pulmonary angiography, and venography. Results: A total of 193 CTEPH patients were referred for pulmonary angiography, and among these, 148 underwent venography. PVO was identified in 65 (44%) patients. Lower extremity (LE) DVT was associated with PVO (p = 0.004). The severity of pulmonary hypertension was similar with and without PVO (mean pulmonary artery pressure 43.0 ± 10.3 mm Hg vs. 43.8 ± 12.4 mm Hg, p = 0.70), as was the need for pulmonary thromboendarterectomy (69.2% vs. 61.4%, p = 0.32). Conclusions: Pelvic vein obstruction is common and a novel clinical association in patients with CTEPH, particularly in patients with a history of LE DVT. PVO and its role in CTEPH warrants further study, including the potential role of revascularization to mitigate further risk.
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Pulmonary hypertension (PH) is a disorder involving a heterogeneous group of medical conditions encompassing several cardiopulmonary illnesses. Implementing new diagnostic criteria for PH in conjunction with multimodality diagnostic tools is crucial for accurate and early recognition of this life-threatening form of right heart failure. This should streamline early referrals to accredited PH centers, with a goal to rapidly institute targeted therapy in order to optimize prognosis.
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Objectives: We sought to evaluate the efficacy and safety of refined balloon pulmonary angioplasty (BPA) in the treatment of patients with chronic thromboembolic pulmonary hypertension (CTEPH). Background: BPA is rapidly evolving therapeutic option for patients with nonsurgical CTEPH. There are few US studies that have reported on the outcomes of this novel therapeutic option. Methods: This is a retrospective study of CTEPH patients that underwent BPA at Temple University Hospital. The primary efficacy endpoint was the change in pulmonary vascular resistance (PVR) after BPA as compared to baseline and the primary safety endpoint was the rate of hemoptysis within 24 hours. Secondary endpoints included death, WHO functional class, and 6-minute walk distance (6MWD). We used logistic regression to evaluate factors associated with a hemodynamic and functional response. Results: A total of 211 BPA sessions were performed on 77 patients (average 2.7 ± 1.7 sessions/patient). After BPA the mean PVR improved by 26% (P<0.001) while the mean 6MWD improved by 71.7 meters (P <0.001) and WHO functional class improved by one functional class (P <0.001). Ten sessions (4.7%) were complicated by hemoptysis. The independent factors associated with a improved functional and hemodynamic response included the pre-procedural use of riociguat, reduce baseline PA compliance and > 3 BPA sessions per patient. Conclusion: This single center study from the US showed that BPA with refined techniques in patients with CTEPH was safe and was associated with significant improvements in pulmonary hemodynamics and functional capacity.
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PURPOSE OF REVIEW: The purpose of this review is to provide an overview of assessment of right ventricular function in the context of pulmonary hypertension and pulmonary arterial hypertension (PAH). We will review unique features of right ventricular anatomy, delineation of cause of pulmonary hypertension through careful right ventricular assessment, echocardiographic and hemodynamic evaluation, and the importance of this assessment in prognosis. RECENT FINDINGS: The importance of performance in prognosis and risk assessment in patients with pulmonary hypertension has been continually emphasized in ongoing research. Representative parameters of right ventricular function have been shown to be predictive of prognosis in patients with pulmonary hypertension. Further, the importance of serial right ventricular assessment in risk assessment and prognosis has remained an emerging theme. SUMMARY: Careful evaluation of right ventricular function is paramount in assessing the cause of pulmonary hypertension and severity of disease. Further, it has prognostic significance, as many representative parameters of right ventricular function have been linked with mortality. In our opinion, right ventricular function should be assessed serially throughout the course of treatment in pulmonary hypertension, and baseline parameters in addition to dynamic changes should be incorporated into risk assessment. Achieving normal or near-normal right ventricular performance may serve as a principal goal in the treatment of pulmonary hypertension.
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Hipertensão Pulmonar , Hipertensão Arterial Pulmonar , Disfunção Ventricular Direita , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/etiologia , Prognóstico , Hipertensão Pulmonar Primária Familiar , Ecocardiografia , Disfunção Ventricular Direita/diagnóstico por imagem , Disfunção Ventricular Direita/etiologiaRESUMO
Pulmonary arterial hypertension (PAH) is often a progressive, fatal disease. Because of nonspecificity of symptoms and limited awareness of PAH, patients are often diagnosed and referred late to accredited pulmonary hypertension (PH) centers, contributing to worsening survival and overall prognosis. The objective of the present study was to determine if the virtual echocardiography screening tool (VEST), a simple scoring system using routinely reported echocardiographic metrics, could capture earlier diagnoses of PAH before clinical recognition and referral to expert PH centers. This study is a retrospective analysis of 132 patients with PAH evaluated consecutively at 2 accredited referral PH centers. VEST scores and time to evaluation at PH center were quantified based on the first available echocardiogram before referral. Clinical risk assessment was calculated at initial evaluation by the PH center using the REVEAL (Registry to Evaluate Early and Long-term PAH Disease Management) 2.0 calculator. An overwhelming majority (93%) of the study participants had markedly abnormal VEST scores predictive of PAH before evaluation at a PH referral center. The median delay from VEST to evaluation was >6 months at 206 days (quartile 1, quartile 3: 55, 757). At initial evaluation, 72% were intermediate or high-risk based on REVEAL 2.0 risk assessment. In conclusion, we propose that VEST is a powerful yet simple scoring tool that can capture high-risk patients with PAH, prompting earlier diagnosis and referrals to accredited PH centers, and allowing for earlier expert implementation of PH medical therapies.
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Hipertensão Pulmonar , Hipertensão Arterial Pulmonar , Humanos , Hipertensão Arterial Pulmonar/diagnóstico por imagem , Hipertensão Arterial Pulmonar/complicações , Estudos Retrospectivos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/tratamento farmacológico , Prognóstico , EcocardiografiaRESUMO
Introduction: Right ventricular (RV) function is a major determinant of outcome in patients with precapillary pulmonary hypertension (PH). We studied the effect of ranolazine on RV function over 6 months using multi-modality imaging and biochemical markers in patients with precapillary PH (groups I, III, and IV) and RV dysfunction [CMR imaging ejection fraction (EF) < 45%] in a longitudinal, randomized, double-blinded, placebo-controlled, multicenter study of ranolazine treatment. Methods: Enrolled patients were assessed using cardiac magnetic resonance (CMR) imaging, 11C-acetate and 18-F-FDG positron emission tomography (PET), and plasma metabolomic profiling, at baseline and at the end of treatment. Results: Twenty-two patients were enrolled, and 15 patients completed all follow-up studies with 9 in the ranolazine arm and 6 in the placebo arm. RVEF and RV/Left ventricle (LV) mean glucose uptake were significantly improved after 6 months of treatment in the ranolazine arm. Metabolomic changes in aromatic amino acid metabolism, redox homeostasis, and bile acid metabolism were observed after ranolazine treatment, and several changes significantly correlated with changes in PET and CMR-derived fluid dynamic measurements. Discussion: Ranolazine may improve RV function by altering RV metabolism in patients with precapillary PH. Larger studies are needed to confirm the beneficial effects of ranolazine.
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Pulmonary 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.
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Hipertensão Pulmonar , Humanos , Idoso , American Heart Association , Medição de Risco , Pressão Sanguínea , Artéria PulmonarRESUMO
PURPOSE OF REVIEW: Although pregnancy in pulmonary arterial hypertension (PAH) is considered high risk and contraindicated, the incidence is rising. It is paramount to understand the pathophysiology and effective management strategies to ensure optimal outcomes for maternal and fetal survival. RECENT FINDINGS: In this review, we highlight the outcomes of recent case series of PAH patients in pregnancy, with a focus on proper risk assessment and target goals of PAH therapy. These findings support the notion that the pillars of PAH management, including pulmonary vascular resistance reduction resulting in right heart functional improvement, and widening of the cardiopulmonary reserve, should serve as a blueprint for PAH management in pregnancy. SUMMARY: Multidisciplinary and tailored management of PAH in pregnancy, with emphasis on optimizing right heart function prior to delivery, can result in excellent clinical outcomes in a referral pulmonary hypertension center.
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Hipertensão Pulmonar , Hipertensão Arterial Pulmonar , Gravidez , Feminino , Humanos , Hipertensão Arterial Pulmonar/terapia , Hipertensão Pulmonar/terapia , Medição de Risco , CoraçãoRESUMO
Background: COVID-19 has affected individuals across the globe, and those with cardiac implantable electronic devices (CIEDs) likely represent a high-risk group. These devices can be interrogated to reveal information about the patient activity, heart rate parameters, and respiratory rate. Case summary: Four patients with CIEDs and left ventricular dysfunction were admitted to a single institution for COVID-19 infection. Each patient survived hospitalization, and none required intensive care. Retrospectively, CIED interrogation revealed each patient had decreased activity level prior to their reporting COVID-19 symptoms. Similarly, respiratory rate increased before symptom onset for three of the patients, while one did not have these data available. Of the three patients with heart rate variability (HRV) available, two had decreased HRV before they developed symptoms. After hospital discharge, these parameters returned to their baseline. Discussion: This case series suggests physiologic changes identifiable through interrogation of CIEDs may occur prior to the reported onset of COVID-19 symptoms. These data may provide objective evidence on which to base more sensitive assessments of infectious risk when performing contact tracing in communities.
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In this systematic review, we seek to clarify the impact of treatment of hyperthyroidism on pulmonary hypertension in patients with both these conditions. We included 39 of 709 articles retrieved, that studied patients with hyperthyroidism and pulmonary hypertension (PH). From these, those with a documented pre-treatment Pulmonary Artery Systolic Pressure (PASP) > 35 mmHg and complete follow up were analyzed, yielding 3 case series and 22 case reports with a total of 81 cases. A significant improvement in PASP was noted with achieving euthyroidism in the 3 case series. The case reports showed a significant reduction in mean PASP from 60.5 ± 13.2 mmHg to 37.5 ± 10.1 mmHg (p < 0.001) in patients with Grave's disease with achieving euthyroidism. No deaths were reported during the follow up period. Achievement of a euthyroid state in patients with hyperthyroidism is associated with statistically significant reductions in PASP.
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Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary hypertension (PH) arising from pulmonary vascular obstruction at varying levels within the pulmonary vascular bed, due to chronic pulmonary emboli. The cornerstone of treatment for CTEPH is pulmonary thromboendarterectomy (PTE), a specialized surgery to remove the chronic vascular obstruction. At experienced centers, PTE leads to marked hemodynamic improvement and, in many cases, normalization of cardiopulmonary hemodynamics. However, increasing evidence supports the fact that a significant percentage of patients will have persistent PH after PTE. No consensus exists on the optimal approach to post-PTE patient assessment, and often the most experienced CTEPH centers have little experience in the direct follow-up care of the CTEPH patient post PTE. In this article, we will discuss a practical approach to patient assessment after PTE to help guide clinicians on how to recognize significant PH following PTE. In doing so, we identify the true phenotype of persistent PH post PTE so that appropriate patients can be further helped with the evolving therapies for the management of CTEPH.
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(1) Background: In pulmonary arterial hypertension (PAH), pregnancy is regarded a contraindication due to high maternal and fetal morbidity and mortality. We report our experience in the management of pregnancies in PAH. (2) Methods: retrospective observational study in a nationally accredited pulmonary hypertension (PH) center from 2013 to 2021. (3) Results: seven pregnancies in six women with PAH, ranging from low to high risk and 21 to 37 years old. Half had known pre-existing PAH before pregnancy. One had a multifetal gestation, and one was pregnant twice under our care. PH medical therapy and serial clinical assessment throughout pregnancy were implemented with focused attention on optimizing right heart function. Delivery was planned by a multidisciplinary team involving PH cardiology, maternal fetal medicine, and obstetric anesthesiology. Patients delivered between 31 and 40 weeks of gestation; five of the seven were via cesarean section. All received regional anesthesia and were monitored in the PH intermediate step-down unit after delivery until discharge. In all cases, delivery was without complications with excellent outcomes for the mother and child. (4) Conclusions: Multidisciplinary and tailored management of PAH in pregnancy, emphasizing optimized right heart function prior to delivery, can result in excellent clinical outcomes in a referral PH center.
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To curb transmission of SARS-CoV-2 and preserve hospital resources, elective procedures were postponed in the United States, affecting patients previously scheduled for electrophysiology (EP) procedures. We aimed to understand patients' perceptions related to procedural postponements during the first wave of the SARS-CoV-2 pandemic. We performed a telephone survey between May 1-15 2020, of consecutive patients who experienced procedural postponement from March-April. Of 112 patients, 20% may have been lost to follow up and 12% lost interest in having their procedures done. The level of anxiety related to postponement was moderate to high in more than two thirds of patients.
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OBJECTIVES: The aim of this study was to investigate the incidence of May-Thurner anatomy (MTA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and identify its predictors. BACKGROUND: MTA is an anatomical variant characterized by compression of left common iliac vein by the overlying right iliac artery. Over time, this leads to venous intimal scarring, blood flow stasis, and the development of deep vein thrombosis (DVT). DVT is a known risk factor for the development of CTEPH. The prevalence of this anatomical variation in patients with CTEPH is unknown. METHODS: A retrospective chart review was conducted in patients referred to Temple University Hospital's cardiac catheterization laboratory for the evaluation of CTEPH between January 2016 and June 2020. Among these patients, those who underwent invasive venography were evaluated for the presence of angiographic MTA. Multivariate regression was used to identify factors associated with presence of MTA. RESULTS: A total of 193 patients with CTEPH were referred for pulmonary angiography, of whom 148 patients underwent invasive venography. MTA was identified in 44 patients (29.7%). Factors associated with the presence of MTA were lower extremity DVT (odds ratio: 3.5; 95% confidence interval: 1.58-7.8; P = 0.002), and left lower extremity post-thrombotic syndrome (odds ratio: 2.0; 95% confidence interval: 0.98-4.1; P = 0.05). Patients with MTA were more likely to undergo pulmonary thromboendarterectomy than patients without MTA (79.5% vs 58.7%; P = 0.015). CONCLUSIONS: MTA is very common in patients with CTEPH. History of lower extremity DVT and or left lower extremity post-thrombotic syndrome was associated with the presence of MTA.
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Hipertensão Pulmonar , Síndrome de May-Thurner , Embolia Pulmonar , Trombose Venosa , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/epidemiologia , Veia Ilíaca/diagnóstico por imagem , Embolia Pulmonar/diagnóstico por imagem , Embolia Pulmonar/epidemiologia , Estudos Retrospectivos , Resultado do Tratamento , Trombose Venosa/diagnóstico por imagem , Trombose Venosa/epidemiologiaRESUMO
Tumoral obstruction is a small, but broadly defined, category of pulmonary hypertension that encompasses microvascular tumor emboli, tumor thrombotic microangiopathy, and macrovascular tumor obstruction within the pulmonary circulation. We present 4 patients with solid tumors, severe pre-capillary pulmonary hypertension, right ventricular failure, and pulmonary veno-occlusive-like disease. (Level of Difficulty: Advanced.).
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A 66-year-old man with a medical history significant for multiple pulmonary embolism and morbid obesity was evaluated for chronic thromboembolic pulmonary hypertension. Echocardiogram and right heart catheterisation were significant for severe pulmonary hypertension. Therefore, he was started on pulmonary hypertension medical therapy with riociguat and ambrisentan, in addition to anticoagulation. He experienced a dramatic clinical response to medical therapy. Despite haemodynamic improvement, the patient remained symptomatic with significant fatigue, exertional dyspnea and poor functional status as highlighted by a 6 min walk distance of only 128 m. Patient was referred for bariatric surgery with a gastric sleeve, after which he successfully lost 95 lbs in 6 months. Postoperative right heart catheterisation demonstrated normal pulmonary vascular resistance and cardiac output. His echocardiogram revealed normal right ventricular size and function. His 6 min walk distance also nearly quadrupled from 128 to 512 m, consistent with WHO Functional Class I.
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Cirurgia Bariátrica , Hipertensão Pulmonar , Embolia Pulmonar , Idoso , Doença Crônica , Ecocardiografia , Humanos , Hipertensão Pulmonar/tratamento farmacológico , Masculino , Embolia Pulmonar/complicações , Embolia Pulmonar/diagnóstico por imagem , Embolia Pulmonar/tratamento farmacológico , Resultado do Tratamento , Resistência Vascular , CaminhadaRESUMO
INTRODUCTION: A major outcome determinant in patients with precapillary pulmonary hypertension (PH) is right ventricular (RV) function. We studied the effect of ranolazine on RV function over 6 months using cardiovascular magnetic resonance (CMR) imaging in patients with precapillary PH (groups I, III, and IV). METHODS AND RESULTS: We enrolled patients with PH and RV dysfunction (CMR imaging ejection fraction [EF] of <45%) in a longitudinal, randomized, double-blinded, placebo controlled, multicenter study of ranolazine treatment. All enrolled patients were on stable PH-specific therapy. Enrolled patients were assessed using CMR imaging, New York Heart Association functional class, N-terminal pro brain natriuretic peptide, 6-minute walk test, and quality of life health outcomes at baseline and repeated at the end of treatment. The primary outcome was change in RVEF after 6 months of treatment. Analysis of covariance was used to analyze the longitudinal changes taking into account baseline values, age, and sex, based on per protocol population. Twenty-two patients were enrolled, and 9 patients completed follow-up CMR imaging after ranolazine treatment and 6 completed placebo treatment. There was significant increase in RVEF at end of treatment compared with baseline in the ranolazine group adjusted for baseline values, age, and sex. There were no statistically significant changes in secondary outcomes such as changes in New York Heart Association functional class, 6-minute walk distance, N-terminal pro brain natriuretic peptide, or quality of life measures. Ranolazine treated patients experienced a higher number of adverse events, but only one was discontinued owing to side effects. CONCLUSIONS: Ranolazine may improve RV function in patients with precapillary PH. Larger studies are needed to confirm the beneficial effects of ranolazine.