Hector Cajigas

Pulmonary Hypertension Care Center Network: Improving Care and Outcomes in Pulmonary Hypertension

&NA; Pulmonary hypertension (PH) is a chronic, progressive, life‐threatening disease that requires expert multidisciplinary care. To facilitate this level of care, the Pulmonary Hypertension Association established across the United States a network of pulmonary hypertension care centers (PHCCs) with special expertise in PH, particularly pulmonary arterial hypertension, to raise the overall quality of care and outcomes for patients with this life‐threatening disease. Since the inception of PHCCs in September 2014, to date 35 centers have been accredited in the United States. This model of care brings together physicians and specialists from other disciplines to provide care, facilitate basic and clinical research, and educate the next generation of providers. PHCCs also offer additional opportunities for improvements in PH care. The patient registry offered through the PHCCs is an organized system by which data are collected to evaluate the outcomes of patients with PH. This registry helps in detecting variations in outcomes across centers, thus identifying opportunities for improvement. Multiple tactics were undertaken to implement the strategic plan, training, and tools throughout the PHCC network. In addition, strategies to foster collaboration between care center staff and individuals with PH and their families are the cornerstone of the PHCCs. The Pulmonary Vascular Network of the American College of Chest Physicians believes this to be a positive step that will improve the quality of care delivered in the United States to patients with PH.

Inhaled Iloprost for Chronic Thromboembolic Pulmonary Hypertension (CTEPH) During Pregnancy: A Case Report.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a subset of pulmonary hypertension caused by acute and recurrent pulmonary emboli. Pulmonary thromboendarterectomy is the treatment of choice, but 10–50% of patients are ineligible for this procedure. We describe the case of a 25‐year‐old, morbidly obese (228‐kg, body mass index 83.5 kg/m2) pregnant woman (G3P2) who presented at 24 weeks’ gestation; bilateral pulmonary angiography revealed filling defects and confirmed the diagnosis of CTEPH. The patient was evaluated and deemed to present too high of a risk for pulmonary thromboendarterectomy, so a multidisciplinary team initiated medical therapy. Sildenafil 20 mg orally 3 times/day was started at week 24 of gestation, and inhaled iloprost was added at 26 weeks and titrated to 5 µg inhaled every 2 hrs in order to optimize hemodynamic status prior to a cesarean section delivery scheduled to be performed 6 weeks later. At 32 weeks of gestation, the patients pulmonary arterial systolic pressure was 77 mm Hg, right atrial pressure was 15 mm Hg, and pulmonary capillary wedge pressure of 16 mm Hg, and a healthy 1741‐g male infant was delivered by cesarean section. The patient was transferred back to the medical intensive care unit in stable condition and discharged home 9 days following the procedure. Pharmacotherapeutic strategies for patients with CTEPH who become pregnant are limited to phosphodiesterase type 5 inhibitors and prostacyclin analog therapies due to the teratogenicity of the other drug classes used to treat the disorder (endothelin receptor antagonists and soluble guanylate cyclase stimulators). To our knowledge, this is the first case report of inhaled iloprost use in addition to oral sildenafil to improve patient symptomatology and hemodynamics during the peripartum period of a young pregnant patient with inoperable CTEPH. This drug therapy was used safely, with no noted adverse effects to the newborn or to the patient.

Classification and diagnosis of pulmonary hypertension

AbstractPulmonary hypertension has been classified into five major subgroups in order to better understand and apply knowledge from the areas of molecular biology, pathophysiology and clinical science. Accurate classification of the patient not only optimizes diagnostic approach but also confers the best benefit, as therapeutic approaches are applied accurately. Current diagnostic strategies begin with a detailed history and physical, which are directed to elucidate symptoms that may increase the degree of suspicion. Subsequent application of a logical approach to progress through the diagnostic algorithm , with understanding of the complexity of this process, allows for best possible outcomes. Proper diagnosis and classification will eventually increase the potential for appropriate research and progress toward of a possible cure for this fatal disease.

Pulmonary Hypertension Care Center Network Improving care and outcomes in PH.

&NA; Pulmonary hypertension (PH) is a chronic, progressive, life‐threatening disease that requires expert multidisciplinary care. To facilitate this level of care, the Pulmonary Hypertension Association established across the United States a network of pulmonary hypertension care centers (PHCCs) with special expertise in PH, particularly pulmonary arterial hypertension, to raise the overall quality of care and outcomes for patients with this life‐threatening disease. Since the inception of PHCCs in September 2014, to date 35 centers have been accredited in the United States. This model of care brings together physicians and specialists from other disciplines to provide care, facilitate basic and clinical research, and educate the next generation of providers. PHCCs also offer additional opportunities for improvements in PH care. The patient registry offered through the PHCCs is an organized system by which data are collected to evaluate the outcomes of patients with PH. This registry helps in detecting variations in outcomes across centers, thus identifying opportunities for improvement. Multiple tactics were undertaken to implement the strategic plan, training, and tools throughout the PHCC network. In addition, strategies to foster collaboration between care center staff and individuals with PH and their families are the cornerstone of the PHCCs. The Pulmonary Vascular Network of the American College of Chest Physicians believes this to be a positive step that will improve the quality of care delivered in the United States to patients with PH.

Spontaneous Cerebral Hemorrhage and Sudden Biventricular Failure After Lung Transplantation.

A woman in her mid-60s with end-stage lung disease secondary to systemic sclerosis and pulmonary hypertension underwent bilateral sequential lung transplantation without cardiopulmonary bypass through a clamshell thoracotomy. The immediate postoperative course was uneventful, and she was weaned off oxygen support. On postoperative day 10, she developed seizures when walking and was immediately intubated. Brain imaging revealed a new intracerebral hemorrhage in the right frontal and left occipital lobes, with narrowing of the internal carotid, basilar, posterior cerebral, and right posterior communicating arteries, suggestive of vasospasm or vasculopathy. Subsequently, she developed hypoxemia and hypotension associated with acute pulmonary edema and new cardiomegaly (Figure 1A). An electrocardiogram showed nonspecific ST changes with T-wave inversion (Figure 1B), and serum levels of troponin and creatine phosphokinase were negative. Transesophageal echocardiography revealed new dilation of the left ventricle (LV), with an ejection fraction of 10%, functional mitral regurgitation, anterior and apical akinesis, and basal hypokinesis (Video 1, Video 2, and Video 3). The right ventricle (RV) was distended, with moderate tricuspid regurgitation. Recent pretransplantation coronary angiography did not reveal atherosclerosis. Her cardiopulmonary status rapidly worsened, with cardiogenic shock and refractory hypoxemia (arterial blood pH, 7.12; arterial partial pressure of oxygen, 43 mm Hg; and partial pressure of carbon dioxide, 56 mm Hg), despite mechanical ventilation (rate, 24/min; tidal volume, 6 mL/kg; fraction of inspired oxygen, 100%; and positive end-expiratory pressure, 15 cm H2O), neuromuscular blockade, and administration of inotropes. Video Chest radiographs A

Current Perspectives on Lung Allocation Schemes in the USA and Europe

Over the past three decades, lung transplantation has evolved as a recognized treatment for patients with advanced lung disease. However, lung transplantation has remained limited by the shortage of acceptable donor lung organs. Previously, allocation of donor lungs in the USA was based primarily upon a recipient’s wait list time. However, in 2005, under the mandate of the US Department of Health and Human Services, the lung allocation system was changed in order to incorporate medical urgency and to ensure equitable and efficient allocation for all transplant candidates. Clinical criteria that predicted wait list mortality and 1-year post-transplant survival were incorporated into the lung allocation score (LAS). The LAS has been associated with an increased number of wait list candidates, decreased wait list time, decreased wait list mortality, and a change in the clinical characteristics of the lung transplant candidates. Given the success of the LAS in the USA, several European transplant organizations are considering adopting a similar lung allocation schema. Continued refinement of the donor lung allocation schema in the USA and Europe will optimize lung transplant outcomes.