MK-5475, an inhaled soluble guanylate cyclase stimulator, for treatment of pulmonary arterial hypertension: the INSIGNIA-PAH study.

Background: MK-5475 is an investigational inhaled soluble guanylate cyclase stimulator hypothesised to avoid most side-effects of systemic vasodilation.

Methods: The phase 2 INSIGNIA-PAH (NCT04732221) trial randomised adults with pulmonary arterial hypertension (PAH) on stable background therapy 1:1:1:1 to once-daily dosing with placebo, MK-5475 32 µg, 100 µg or 380 µg dry powder inhalation for 12 weeks.

Objectives: The objectives were to evaluate pulmonary vascular resistance (PVR; primary), 6-min walk distance (6MWD; secondary), additional selected haemodynamic parameters, and safety and tolerability in participants with PAH.

Underuse of reperfusion therapy with systemic thrombolysis in high-risk acute pulmonary embolism in a Portuguese center.

Introduction: Reperfusion therapy is generally recommended in acute high-risk pulmonary embolism (HR-PE), but several population-based studies report that it is underused. Data on epidemiology, management and outcomes of HR-PE in Portugal are scarce.

Objective: To determine the reperfusion rate in HR-PE patients, the reasons for non-reperfusion, and how it influences outcomes.

Blueprint for developing an effective pulmonary embolism response network.

Introduction: Acute pulmonary embolism (PE) is a major cause of morbidity and mortality in Portugal. It is the third most common cause of cardiovascular death after stroke and myocardial infarction. However, the management of acute PE remains poorly standardized, and there is a lack of access to mechanical reperfusion when indicated.

Severe and Apparently Irreversible Pulmonary Arterial Hypertension in a Patient with Ostium Secundum Atrial Septal Defect - A Successful Case of Treat and Close Strategy.

Irreversible pulmonary arterial hypertension is considered a contraindication for surgical or percutaneous closure of atrial septal defects (ASD) due to risk of right heart failure. We present a case of 37 years-old woman who was referred to our center due to progressive worsening fatigue and high probability of pulmonary hypertension on a transthoracic echocardiogram. The diagnostic work-up revealed the presence of an ostium secundum atrial septal defect and severe pre-capillary pulmonary hypertension on right heart cathetherization (RHC).

Continuous Aspiration Mechanical Thrombectomy for the management of intermediate- and high-risk pulmonary embolism: Data from the first cohort in Portugal.

Introduction: This study describes the experience of a reference center using continuous aspiration mechanical thrombectomy for acute high- and intermediate-high-risk pulmonary embolism (PE).

Methods: Twenty-nine consecutive patients with acute central PE (48.3% high-risk PE; 82.

Safety and efficacy of balloon pulmonary angioplasty in a Portuguese pulmonary hypertension expert center.

Introduction: Balloon pulmonary angioplasty (BPA) is an alternative therapy in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) or residual/recurrent pulmonary hypertension (PH) after surgery. The aim of this study was to assess the short-term efficacy and safety of a BPA program.

Methods: This prospective single-center study included all BPA sessions performed in CTEPH patients between 2017 and 2019.

Safety and efficacy of balloon pulmonary angioplasty in a Portuguese pulmonary hypertension expert center.

Introduction: Balloon pulmonary angioplasty (BPA) is an alternative therapy in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) or residual/recurrent pulmonary hypertension (PH) after surgery. The aim of this study was to assess the short-term efficacy and safety of a BPA program.

Methods: This prospective single-center study included all BPA sessions performed in CTEPH patients between 2017 and 2019.

Neurofibromatosis-associated pulmonary hypertension: an ominous duo.

The authors report a rare clinical case of a patient with neurofibromatosis type 1 (NF1) complicated by pulmonary hypertension (PH), which presents with rapid progression. An exhaustive investigation was performed to identify the main aetiology of the PH. It was concluded that the PH could be associated with NF1, and so belonged in group 5 of the clinical classification of PH.

Chronic thromboembolic pulmonary hypertension secondary to implantable cardioverter defibrillator lead thrombus in a patient with Brugada syndrome: a rare complication requiring a multidisciplinary approach.

We report the case of a 57-year-old male patient with prior syncope associated with sustained ventricular tachycardia in the setting of Brugada syndrome, who was submitted to implantation of a cardioverter defibrillator for secondary prevention. During follow-up, he presented a significant increase in lead impedance, and a transthoracic echocardiogram showed a mass attached to the lead. He was started on oral anticoagulation after infective endocarditis was excluded but nevertheless suffered repeated episodes of pulmonary embolism that led to severe chronic thromboembolic pulmonary hypertension.

Dyspnea in antiphospholipid syndrome: Beyond pulmonary embolism.

Pulmonary embolism due to primary antiphospholipid syndrome is rarely associated with chronic thromboembolic pulmonary hypertension, and therefore according to the latest guidelines on pulmonary hypertension, routine screening is not recommended. We describe a young patient with a late diagnosis of chronic thromboembolic pulmonary hypertension in the context of pulmonary embolism, primary antiphospholipid syndrome and suboptimal anticoagulation. Of note, mild cardiopulmonary symptoms were consistently misattributed to a depressive disorder because physical examination was normal, serial Doppler echocardiography failed to show pulmonary hypertension, and all other diagnostic tests were normal.

Pulmonary veno-occlusive disease: a probably underdiagnosed cause of pulmonary hypertension in systemic sclerosis.

Pulmonary hypertension is a serious complication of systemic sclerosis and remains one of the leading causes of mortality. Pulmonary veno-occlusive disease (PVOD), recently reclassified as pulmonary arterial hypertension (PAH) with overt features of venous/capillaries involvement, is a subgroup of group 1 pulmonary hypertension, which has been rarely reported in systemic sclerosis patients. It is symptomatically indistinguishable from idiopathic pulmonary arterial hypertension and should be suspected in those with manifestations of pulmonary arterial hypertension who have evidence of pulmonary venous congestion in the absence of left-sided heart disease.

A pregnant woman with a mechanical prosthetic valve.

A 24-year-old woman who was 11 weeks pregnant and had a mechanical mitral valve presented at the emergency department with ischemic stroke. Although treatment with aspirin and unfractionated heparin was initially successful, subsequent management was almost impossible, as she eventually suffered a right hemispheric stroke, requiring percutaneous intervention and an emergency cesarian; prosthetic valve thrombosis with hemodynamic instability; and multiple bleeding complications. This case demonstrates how difficult it can be to select the appropriate treatment strategy for prosthetic valve thrombosis.

Three-vessel myocardial bridging: A possible cause of myocardial stunning.

The authors report a rare clinical case of myocardial bridging of the three major coronary arteries, which manifested in an unusual way with severe biventricular dysfunction in the context of tachycardia. For the diagnosis, the authors relied on non-invasive multimodality cardiac imaging, including cardiac magnetic resonance, computed tomography angiography and myocardial perfusion scintigraphy. The implementation of targeted medical and neurohormonal therapy resulted in the recovery of ventricular function and clinical improvement.

Portuguese Society of Cardiothoracic and Vascular Surgery/Portuguese Society of Cardiology recommendations for waiting times for cardiac surgery.

Appointed jointly by the Portuguese Society of Cardiothoracic and Vascular Surgery (SPCCTV) and the Portuguese Society of Cardiology (SPC), the Working Group on Waiting Times for Cardiac Surgery was established with the aim of developing practical recommendations for clinically acceptable waiting times for the three critical phases of the care of adults with heart disease who require surgery or other cardiological intervention: cardiology appointments; the diagnostic process; and invasive treatment. Cardiac surgery has specific characteristics that are not comparable to other surgical specialties. It is important to reduce maximum waiting times and to increase the efficacy of systems for patient monitoring and tracking.

Oral anticoagulation for pulmonary arterial hypertension: systematic review and meta-analysis.

Background: Uncertainty exists about the benefit of oral anticoagulation in the treatment of pulmonary arterial hypertension (PAH), which is a lethal disease. We aimed to review and quantify the effect of oral anticoagulants in overall survival of PAH patients.

Methods: We searched for randomized and observational studies that evaluated oral anticoagulants in PAH in the electronic databases MEDLINE, CENTRAL and ISI Web of Knowledge (December 2013).

Echocardiographic assessment of right ventricular contractile reserve in patients with pulmonary hypertension.

Introduction And Aim: Right ventricular function is a major determinant of prognosis in pulmonary hypertension. The aim of this study was to assess and compare right ventricular contractile reserve in healthy subjects (controls) and in subjects with pulmonary hypertension (cases).

Methods: In this prospective study of seven cases and seven controls undergoing treadmill stress echocardiography, right ventricular S-wave velocity, tricuspid annular plane systolic excursion (TAPSE), right ventricular fractional area change (RVFAC) and stroke volume index were assessed at rest and with exercise.

[SPCCTV and SPC Recommendations Related to the Waiting Times for Cardiac Surgery].

Appointed jointly by the Portuguese Society for Cardiothoracic and Vascular Surgery (SPCCTV) and by the Portuguese Society of Cardiology (SPC), the Working Group related to the Waiting Times for Cardiac Surgery was created with the aim of developing practical recommendations about clinically acceptable waiting times for the three critical phases of the care of adults with a cardiac disease that require surgery or an intervention: cardiology appointments; diagnostic process and invasive therapy. Cardiac surgery has its own characteristics, not comparable to other surgical specialties and, therefore, it is important to reduce its maximum waiting times and, also, increase the efficacy of the systems which are responsible to monitor and trace the patient. The information given in this document was based, mostly, in available clinical information.

Clinical applications of exercise stress echocardiography in the treadmill with upright evaluation during and after exercise.

Exercise stress echocardiography is the most frequently used stress test in our laboratory. Exercise echocardiography is used mainly in the study of patients with coronary artery disease. However, the technique is increasingly being used to study other diseases.

[Takotsubo cardiomyopathy in a cardiology department].

Takotsubo cardiomyopathy, also known as transient left ventricular apical ballooning syndrome, stress-induced cardiomyopathy and broken heart syndrome, is characterized by transient left ventricular dysfunction in the absence of obstructive coronary artery disease. It was first described in 1990 in Japan, and gained worldwide recognition following the publication of several series of case reports. Its prevalence is estimated to be 1.