Determinants of physical quality of life in patients with chronic thromboembolic pulmonary hypertension after treatment: Insights from invasive exercise stress test.

BACKGROUND: Impaired quality of life (QoL) is prevalent among patients with chronic thromboembolic pulmonary hypertension (CTEPH) despite improved survival due to medical advances. We clarified the physical QoL of patients with CTEPH with mildly elevated pulmonary hemodynamics and evaluated its determinants using a database of patients with CTEPH evaluated for hemodynamics during exercise. METHODS: The QoL was measured in 144 patients with CTEPH (age, 66 (58-73) years; men/women, 48/96) with mildly elevated mean pulmonary artery pressure (<30 mm Hg) at rest after treatment with balloon pulmonary angioplasty and/or pulmonary endarterectomy using the Short-Form 36 (SF-36) questionnaire. The enrolled patients were divided into 2 groups: physical component summary (PCS) scores in the SF-36 over 50 as PCS-good and those under 50 as PCS-poor. RESULTS: The median PCS in SF-36 score was 43.4 (IQR 32.4-49.5) points. The PCS-poor group (n = 110) was older and had lower exercise capacity and SaO2 during exercise. PCS scores were correlated with 6-minute walk distance (rs=0.40, p < 0.001), quadriceps strength (rs=0.34, p < 0.001), peak VO2 (rs=0.31, p < 0.001), SaO2 at rest (rs=0.35, p < 0.001) and peak exercise (rs=0.33, p < 0.001), home oxygen therapy usage (rs=-0.28, p = 0.001), and pulmonary vascular resistance at peak exercise (rs=-0.26, p = 0.002). CONCLUSIONS: The impairment of physical QoL was common in patients with CTEPH with improved hemodynamics; exercise capacity, hypoxemia, and hemodynamic status during exercise were related to the physical QoL.

A case of atrial septal defect with right-to-left shunting without pulmonary hypertension.

We report a case of a 54-year-old man with atrial septal defect who presented with oxygen desaturation on pulse oximetry. Cardiac magnetic resonance imaging and transesophageal echocardiography showed right-to-left shunting through an atrial septal defect, which was confirmed by superior vena cavography and suggested Eisenmenger syndrome. However, cardiac catheterization revealed a normal pulmonary arterial pressure. Simultaneous measurement of interatrial pressure identified two transient interatrial pressure gradient points, where the right atrial pressure was higher than the left atrial pressure. The patient was finally diagnosed with atrial septal defect without pulmonary hypertension. Right-to-left shunting was primarily caused by a transient interatrial pressure gradient due to a time delay in both initial atrial contraction and completion of passive ventricular filling between the right and left sides of the heart. Surgical closure of the atrial septal defect was performed, and hypoxemia improved. This is the first report of right-to-left shunting without pulmonary hypertension caused by a transient interatrial pressure gradient due to a time delay between the right and left cardiac cycles. Precise assessment of the simultaneous interatrial pressure in addition to diagnostic imaging played a pivotal role in clarifying the etiology of this rare condition. Learning objective: Atrial septal defect with right-to-left shunting without Eisenmenger syndrome is a rare condition. We identified transient interatrial pressure gradients associated with a time delay in both initial atrial contraction and completion of the passive ventricular filling phase, which we considered as the primary mechanism underpinning right-to-left shunting. Simultaneous measurement of interatrial pressure played a pivotal role in elucidating the hemodynamics and abnormal shunt flow mechanism.

Essential thrombocythemia complicated with simultaneous two-vessel acute myocardial infarction in the subacute phase of takotsubo cardiomyopathy: A case report.

We report the case of a 79-year-old woman with essential thrombocythemia who presented with simultaneous two-vessel acute myocardial infarction (AMI) in the subacute phase of takotsubo cardiomyopathy. Despite sufficient anticoagulation therapy with warfarin to prevent thrombus formation in the left ventricle, the patient developed simultaneous two-vessel AMI in the right and left circumflex coronary arteries 16 days after the onset of takotsubo cardiomyopathy. Thromboembolism from the left ventricle associated with takotsubo cardiomyopathy was considered a potential cause of this event. However, macroscopic and pathological findings of the aspirated thrombi revealed that the primary cause of AMI was non-organized white platelet thrombi associated with essential thrombocythemia. In addition to oral anticoagulation therapy with warfarin, low-dose aspirin was started. The patient was discharged without any symptoms, and the clinical course has been uneventful for >5 years. This case highlights the potential risk of fatal complications associated with essential thrombocythemia, including simultaneous multivessel AMI. Additionally, pathological findings of the thrombi may play a crucial role in clarifying the etiology in such complicated cases. Appropriate antithrombotic therapy should be selected according to the pathogenesis of the condition. Learning objective: We describe a 79-year-old woman with essential thrombocythemia complicated with simultaneous two-vessel acute myocardial infarction (AMI) in the subacute phase of takotsubo cardiomyopathy. Although patients with essential thrombocythemia are highly predisposed to thrombotic events including AMI, the appropriate antithrombotic regimen remains controversial. The macroscopic and pathological findings of the thrombi play a pivotal role in clarifying the etiology, which may lead to the appropriate antithrombotic therapy.