1.Pulmonary Arterial Aneurysm with Thrombus Formation and Atrial Septal Defect with Pulmonary Hypertension
Sime Raymond B. Fernandez ; Jessie F. Orcasitas
Philippine Journal of Internal Medicine 2019;57(1):46-50
Introduction:
Pulmonary arterial aneurysm (PAA) is a rare condition with varying symptomatology. Patients are often referred due to incidental finding of intrathoracic mass on chest radiograph. Common causes include structural cardiac and vascular abnormality, pulmonary hypertension, infection, vasculitis, connective tissue disease, and trauma.
Case presentation:
A 37-year-old female came in due to shortness of breath associated with easy fatigability, exertional dyspnea, orthopnea, and occasional palpitations. Workup revealed 2D echocardiography findings of atrial septal defect (ASD) with left to right shunt; dilated right atrium and right ventricle; severe pulmonary hypertension; dilated main pulmonary artery. Contrast-enhanced computed tomography (CT) angiography showed PAA in the right main trunk, left and right pulmonary artery with thrombus formation. Cardiac catheterization and hemodynamic study were intended prior to definitive surgical treatment however patient did not opt to proceed. She was managed with warfarin, bisoprolol, digoxin, and trimetazidine. In spite of symptom relief, the patient still needs regular follow-up for reassessment to monitor disease progression.
Discussion:
Pulmonary arterial aneurysm (PAA) is a rare complication of ASD, a common congenital heart disease. In this case, it presented as a mediastinal mass with nonspecific symptoms diagnosed after routine chest x-ray, verified through echocardiogram. Contrast-enhanced CT angiography ruled out aortic aneurysm and provided additional information of the extent of thrombus formation. However, pulmonary angiography still remains the diagnostic gold standard. Due to the rarity of the disease, optimal treatment of PAA is still uncertain. It is reasonable, however, to treat conservatively for asymptomatic patient with no significant pulmonary hypertension. A more aggressive surgical approach has been advocated for patients with complications.
Conclusion
It is important to emphasize that PAA can present with non-specific manifestations thus high index of suspicion is critical for the diagnosis. It is also vital for early detection of the aneurysm and correction of the etiology to prevent disease morbidity and mortality.
Thrombosis
;
Heart Septal Defects, Atrial
2.The Correlation of Ankle Brachial Index and the severity of Acute Ischemic Stroke in a Tertiary Hospital in Davao City, Philippines: A Cross-Sectional Study
Sime Raymond B. Fernandez ; Annabelle Y. Lao-Reyes
Philippine Journal of Internal Medicine 2020;59(2):89-93
INTRODUCTION: Peripheral arterial disease (PAD) had been shown to have a higher likelihood of developing cardiovascular events as well as cerebrovascular accidents particularly acute ischemic stroke. However, there are limited data on the association between ankle brachial index (ABI) values and the severity of ischemic stroke. This study aimed to determine the correlation of ABI values and the severity of acute ischemic stroke in Southern Philippines Medical Center.
METHODS: A prospective cross-sectional study with 112 patients diagnosed with acute ischemic stroke from June to October 2017. The ABI ratio of the subjects were obtained and correlated with the severity of stroke using National Institutes of Health Stroke Scale (NIHSS). Data analyses utilized chi-square test for categorical variables while ANOVA test for continuous variables. Spearman rho was used to determine the association between ABI and NIHSS.
RESULTS: Majority of patients with acute ischemic stroke had PAD with ABI ratio of ≤ 0.9 (51.8%). Using t-test, the NIHSS was significantly higher among patients with PAD having a mean score of 12.43 ± 5.29 compared to patients with normal ABI ratio having a mean score of 5.13 ± 4.09 (p= < 0.001). Furthermore, using Spearman’s rho statistics, ABI ratio was negatively correlated with NIHSS score (p < 0.001).
CONCLUSION: Our results confirmed that there is a correlation between low ABI value and the severity of acute ischemic stroke. Routine ABI screening may help physicians intensify treatment strategies for those high-risk patients to prevent future events.
Ankle Brachial Index
;
Peripheral Arterial Disease
;
Stroke
3.Diffuse cutaneous systemic sclerosis: A case report.
Janice Natasha C. NG ; Sime Raymond B. FERNANDEZ ; Victoria P. GUILLANO ; Bryan Edgar K. GUEVARA
Philippine Journal of Internal Medicine 2017;55(2):1-4
BACKGROUND: Systemic sclerosis (SSc) is a rare, connective tissue disease with multisystem involvement.This is due to immunological processes,vascular endothelial cell injury and extensive activation of fibrolast that commonly affects the skin and other internal organs such as the esophagus, lungs, heart, and kidneys. SSc has one of the highest mortality among the autoimmune rheumatic diseases, hence the emphasis on the early recognition and management to prevent significant progression of the disease.
CASE: A 22-year-old female presented with a one-year history of multiple hard and hypopigmented patches on the face, neck, trunk and upper extremities. Further examination revealed mask-like facies, microstomia, frenulum sclerosis, Raynaud's phenomenon, pitted scars on the digital pulp of hands and sclerodactyly.Baseline blood chemistry,chest radiograph and electrocardiography were all negative for systemic involvement. Autoantibodies were positive for dsDNA, SS-A/Ro and Scl-70. Skin biopsy revealed sclerosing dermatitis, which was consistent with SSc.
OUTCOME: The patient was initially started with oral prednisone 0.5 mg/kg/day and was increased to 0.75 mg/kg/day for eight weeks. Prednisone was slowly tapered to 5.0 mg/day and methotrexate 15.0 mg/week was included in the management for eight weeks which resulted in decreased joint pains, halted the progression of skin induration, decreased in pruritus and palmar edema.
CONCLUSION: The characteristic dermatological findings of SSc are not only important signs to dermatologists, but these serves as diagnostic clues for clinicians from other disciplines as well. In our case, the presence of the autoantibody Scl-70 indicated the potential risk of pulmonary fibrosis and pulmonary arterial hypertension that accounts with high mortality.Hence,physicians should be aware of the possible risk of organ damage,even when asymptomatic because there is a high risk of disease progression. The importance of early recognition and a multidisciplinary approach lead to the good outcome in this case.
Human ; Female ; Adult ; Autoantibodies ; Prednisone ; Methotrexate ; Cicatrix ; Microstomia ; Sclerosis ; Raynaud Disease ; Pulmonary Fibrosis ; Hypertension, Pulmonary ; Edema ; Rheumatic Diseases ; Scleroderma, Systemic ; Scleroderma, Diffuse