BACKGROUND: Polyangiitis overlap syndrome (POS),a systemic vasculitis not classifiable into well-defined syndromes, is  diagnosed based on combined characteristics of two or more primary systemic vasculitides, such as Takayasu arteritis  and anti-neutrophil cytoplasmic antibody (ANCA)-associated  vasculitis  (AAV), but not two AAVs. Our objective is to   present such,   with associated  multiple  infections.
CASE: A  25-year-old  Filipino  female  had  six  years  of  recurrent  purpura,  debilitating  joint  pains,  hemoptysis,  rhinorrhea, epistaxis, eye redness, dyspnea,  and  abdominal  cramps. She was cachectic, had oral ulcers, bibasal  crackles,  polyarthritis,  and  generalized  purpura.   Work-up   showed   anemia,   eosinophilia  excluding   parasitism,   elevated inflammatory markers, and  positive cytoplasmic (c)-ANCA and anti-proteinase 3. There   was   chronic sinusitis on rigid rhinoscopy, middle to lower lung field  interstitial   infiltrates   on   radiographs, pulmonary vasculitis   on  chest   CT,   moderate pulmonary hypertension on  echocardiography,  but  normal  spirometry.  Skin  biopsy  showed  leukocytoclastic vasculitis without  necrotizing  granulomas.Serology showed chronic hepatitis B infection.   Pseudomonas  aeruginosa and  methicillin-resistant Staphylococcus  aureus  grew  from  bronchial  washings and   nasal   swabs,   respectively. ASO titer was high. Human  immunodeficiency  virus  infection  was  ruled  out.  She   was   diagnosed with POS,with features of two AAVs- limited granulomatosis   with   polyangiitis   (GPA)   and   Churg-Strauss   syndrome  (CSS), associated with   multiple  infections,   and  was  given naproxen and culture-guided   antibiotics  (ciprofloxacin, clindamycin, penicillin),with partial resolution of symptoms.  Prednisone   10  mg once  a  day (OD)  was   started  two weeks   after hepatitis B reactivation prophylaxis   with   lamivudine 100mg OD. Complete   resolution of skin lesions and upper airway symptoms and    inflammatory  marker  improvement  were observed after 12  weeks. She is  maintained on prednisone   5.0   mg   daily.
CONCLUSION AND RECOMMENDATIONS: This   is   the   first   reported   case   of   POS   involving   two   AAVs   -   limited   GPA   and   incomplete   CSS,   associated   with   multiple   pathogens.Identifying  POS  is  important;    inability to  classify patients  into well-recognized  vasculitic  syndromes  delays  treatment.  The  infections likely  perpetuated   the   vasculitis,  and  both antimicrobials  and  immunosuppression were  necessary  to  induce   disease   remission

Human ; Female ; Adult ; Churg-strauss Syndrome ; Methicillin-resistant Staphylococcus Aureus ; Pseudomonas Aeruginosa ; Hepatitis B, Chronic ; Takayasu Arteritis ; Granulomatosis With Polyangiitis ; Wells Syndrome

INTRODUCTION: Cardiac hemangiomas are rare benign primary tumors.We present the successful management of a patient with a hemangioma causing significant right ventricular outflow tract (RVOT) obstruction.
CLINICAL PRESENTATION: A 54-year-old  female  with  no  co-morbidities  presented  with  progressive  right-sided  heart failure  symptoms.Examination  revealed  a  prominent right  ventricular  heave,  irregular  cardiac  rhythm,murmurs  consistent  with  pulmonic  stenosis  and  tricuspid regurgitation,  ascites,  and  bipedal  edema.  Given the echo features of the mass and the patient's clinical course,we  favored  a  benign  cardiac  tumor  over  malignancy.Differentials included  myxoma,  fibroma,  and  papillary fibroelastoma. Medical management included enoxaparin and carvedilol as anticoagulation and rate control for the atrial flutter, respectively. Surgical treatment consisted of tumor excision and tricuspid valve annuloplasty.
RESULTS: Intra-operatively, the stalk was indeed attached to the RV free wall. Histopathology was consistent with primary cardiac hemangioma. The patient's post-operative course was complicated by pneumonia, acute kidney injury, and tracheostomy, but she was eventually discharged improved after a month in the ICU.
SIGNIFICANCE: This report highlights a rare primary cardiac tumor in an unusual location. While there have been several reports in the world literature on cardiac hemangiomas, less than ten cases have been shown to have significant RVOT obstruction as in our patient.
CONCLUSION: Cardiac hemangiomas should be part of the differential diagnosis for an intracardiac mass in the right ventricle.  Meticulous  echocardiography  can  be  a  non-invasive and inexpensive aid to diagnosis and pre-operative planning.

Human ; Female ; Middle Aged ; Tricuspid Valve ; Carvedilol ; Enoxaparin ; Tricuspid Valve Insufficiency ; Heart Ventricles ; Tracheostomy ; Myxoma ; Heart Neoplasms ; Propanolamines ; Hemangioma ; Pulmonary Valve Stenosis

INTRODUCTION: Anthracycline is a cornerstone in the treatment of various cancers. One major limitation to its use is cardiotoxicity. Renin angiotensin system (RAS) inhibitors have been shown to attenuate myocardial injury, initial data is promising in its use as prophylaxis for anthracyclineinduced cardiotoxicity. The aim of the study is to determine effectiveness of prophylactic RAS inhibitors in preventing anthracycline-induced cardiotoxicity and adverse cardiac events among adult cancer patients

METHODS: Systematic search of databases PUBMED, MEDLINE, EMBASE, and CENTRAL was done. Selection criteria were: 1) randomized controlled trials (RCT) 2) adult cancer patients with normal ejection fraction and without heart failure symptoms 3) RAS inhibitors as prophylaxis versus placebo 4) development of cardiac events, all-cause mortality and left ventricular ejection fraction (LVEF) reduction as outcomes. Two reviewers independently assessed the trials. Disagreements were resolved with a third reviewer. Test for effect of intervention, heterogeneity, trial quality and risk of bias were assessed using the Cochrane Review Manager Software version 5.3.

RESULTS: Five RCTs involving 530 adult patients, with average age of 50± two years old, and average follow-up from six months to three years were included. Combined clinical outcomes of heart failure, cardiac events and all-cause mortality showed an RR of 0.27[95%CI 0.18, 0.40],p<0.00001, in favor of RAS inhibitors. There is same benefit in LVEF preservation with mean difference of 4.37%[95%CI 1.20, 7.55;p=0.007]. Exploratory subgroup analysis showed significant benefit in LVEF preservation with combined RAS inhibitor and beta-blocker, with mean difference of 2.45%[95%CI 1.27, 3.63]. There is overall significant heterogeneity (I2=95%). Excluding one article with high-risk population, after sensitivity analysis, showed same benefit but reduced heterogeneity.

CONCLUSION: Renin angiotensin system (RAS) inhibitors may be used as prophylaxis for cardiotoxicity. As prophylaxis, it reduced the clinical outcome of cardiac events, heart failure, and all-cause mortality among cancer patients needing anthracycline. Combined RAS inhibitor and betablocker limits LVEF reduction.

Human ; Male ; Female ; Cardiotoxicity ; Renin-angiotensin System ; Medline ; Stroke Volume ; Patient Selection ; Follow-up Studies ; Anthracyclines ; Pubmed ; Heart Failure ; Adrenergic Beta-antagonists ; Neoplasms

INTRODUCTION: Patients with acute coronary syndrome (ACS) exhibit a wide spectrum of early risk of death (one to 10 percent). High platelet counts may indicate a propensity for platelet-rich thrombi. Lymphocyte counts drop during ACS due to stress-induced cortisol release. Combining these two markers, recent studies have found that the platelet-tolymphocyte ratio (PLR) is associated with adverse cardiac events among patients with ACS, but local data is limited. The objective of this study is to determine if an elevated PLR taken on admission is associated with higher rates of adverse cardiac events.

METHODS: A retrospective cohort of adult patients with ACS admitted at the UP-Philippine General Hospital was analyzed. Leukocyte and platelet counts were measured by an automated hematology analyzer. The PLR values of these patients were computed, and they were stratified into two groups after determining the optimal cut-off from the receiver operating characteristic curve (ROC) curve. The primary outcome was in-hospital mortality. Secondary outcomes included development of heart failure, cardiogenic shock, reinfarction, and significant arrhythmias.

RESULTS: A total of 174 Filipinos with ACS were included. In-hospital mortality occurred in 30 patients (17%). These patients had a higher PLR compared to those who were discharged alive (p-value <0.0001). The optimal cutoff value of PLR to predict in-hospital mortality is 165, with a sensitivity of 77% and specificity of 70% (area under the ROC curve of 0.766). On multiple logistic regression analysis, a high PLR was an independent predictor of in-hospital mortality (RR 8.52; p 0.003) after controlling for the effect of other variables. The development of the predetermined secondary outcomes did not correlate with PLR on multivariate analysis.

CONCLUSION: Among Filipino patients with ACS, an elevated PLR taken within 24 hours of admission is a useful marker to predict in-hospital mortality, thus providing vital information for risk stratification and more aggressive management strategies.

Human ; Male ; Female ; Blood Platelets ; Acute Coronary Syndrome ; Shock, Cardiogenic ; Hospital Mortality ; Hydrocortisone ; Hospitals, General ; Philippines ; Lymphocyte Count ; Heart Failure ; Arrhythmias, Cardiac ; Leukocytes ; Hematology

Introduction. Coronary artery disease (CAD) remains a significant public health problem worldwide and in the Philippines. Adherence to guideline-directed therapy improves the quality of care (QOC).

Objective. We aimed to evaluate the QOC initially received by ST-Elevation Myocardial Infarction (STEMI) patients at our Emergency Room (ER), based on compliance to 2014 PHA CAD guidelines recommendations.

Methods. We reviewed the charts of adult patients with STEMI admitted at the ER who were not previously managed in a different hospital. The primary outcome was QOC assessed through quality indicators (QI) based on class I and IIa recommendations in the PHA CAD guidelines.

Results. Of the 29 patients included, all had ECG done upon admission, but only four were done within 10 minutes (QI: 13.79%). All eligible patients received antiplatelets (QI: 100%). Six eligible patients (QI: 100%) received nitrates, and four eligible patients (QI: 100%) received morphine. Of 16 eligible patients, only six were reperfused within the recommended 12 hours of ischemia (QI: 37.5%), two by thrombolysis and four by the primary percutaneous coronary intervention (PCI).

Conclusion. The timely performance of initial ECG and reperfusion need improvement. Suitable performance measures for the provision of nitrates and morphine to eligible patients were met. Investigating intrinsic and extrinsic factors that lead to the time delays observed are also recommended.

ST Elevation Myocardial Infarction ; Emergency Service, Hospital

Introduction: Takayasu’s arteritis (TA), a large vessel vasculitis has various initial presenting manifestations; making it difficult to diagnose. Hence, the number of those with the disease in the population is underestimated. The study intends to update local data and to describe different presentations of the disease to enhance awareness for TA. Methods: This is a retrospective study done in a tertiary government hospital. Twenty-two out of twenty three charts of patients diagnosed with TA based on the 1990 ACR criteria were reviewed. Demographic profile, initial clinical manifestations, imaging, treatment and outcomes were collected. Descriptive statistics was applied. Institutional Review Board approval was obtained prior to study initiation. Results: Majority (90.1%) were female; mean age at onset of symptoms and at diagnosis were 30.4 (+12.3)years and 33.2 (+12.0)years respectively. The common reasons for consult were hypertension (26.3%), claudication (21.1%) and abdominal pain (11%). Laboratories showed elevated erythrocyte sedimentation rate (87.5%), leukocytosis (43.8%), anemia (31%) and thrombocytosis (4.5%). Common imaging findings were cardiomegaly (27.3%), aortic regurgitation (27.3%) and carotid stenosis (18.2%). CT angiogram in 90% of cases demonstrated arterial wall narrowing. Other findings were aneurysm (31.8%), contour irregularities (13.6%) and femoral artery occlusion (4.5%). Treatment for active disease were glucocorticoids alone (44%) and combined glucocorticoids and other immunosuppressants (56%). Of the 22 records reviewed, six patients (27%) had stroke. Four (18.2 %) had different surgical procedures; ray amputation of toe for digital ischemia, embolectomy for digital gangrene, balloon angioplasty of the renal artery and renal angioplasty for stenosis. Two (9.1%) who had pregnancies after TA diagnosis had premature deliveries without neonatal complications. No mortality was recorded over the mean follow-up of 49.33 patient-years. Conclusion Clinicians should be aware of the different initial presenting signs and symptoms of TA since development of collateral circulation may mask other symptoms. Thus, thorough history and physical assessment are essential tools in the diagnosis of TA.

Objective. Several studies showed that genetic factors affect responsiveness to statins among different populations. This study investigated the associations of candidate genetic variants with poor response to statins among Filipinos.

Methods. In this unmatched case-control study, dyslipidemic participants were grouped into statin responders and poor responders based on the degree of reduction in LDL-c from baseline. DNA from blood samples were genotyped and analyzed. The association of candidate variants with statin response was determined using chi-square and logistic regression analysis.

Results. We included 162 adults on statins (30 poor responders as cases, 132 good responders as controls). The following variants are nominally associated with poor response to statin among Filipinos at a per-comparison error rate of 0.05: rs173539 near CETP (OR=3.05, p=0.015), rs1800591 in MTTP (OR=3.07, p=0.021), and rs1558861 near the BUD13-ZPR1-APOA5 region (OR=5.08, p=0.004).

Conclusion. Genetic variants near CETP, MTTP and the BUD13-ZPR1-APOA5 region are associated with poor response to statins among Filipinos. Further study is recommended to test the external validity of the study in the general Filipino population.

Lipids ; Hydroxymethylglutaryl-CoA Reductase Inhibitors

Background. Low levels of high-density lipoprotein cholesterol (HDL-c) is a well-recognized risk factor in the development of cardiovascular diseases. Associated gene variants for low HDL-c have already been demonstrated in various populations. Such associations have yet to be established among Filipinos who reportedly have a much higher prevalence of low HDL-c levels compared to other races.

Objective. To determine the association of selected genetic variants and clinical factors with low HDL-c phenotype in Filipinos.

Methods. An age- and sex-matched case-control study was conducted among adult Filipino participants with serum HDL-c concentration less than 35 mg/dL (n=61) and those with HDL-c levels of more than 40 mg/dL (n=116). Genotyping was done using DNA obtained from blood samples. Candidate variants were correlated with the low HDL-c phenotype using chi-squared test and conditional logistic regression analysis.

Results. Twelve single nucleotide polymorphisms (SNPs) were associated with low HDL-c phenotype among Filipinos with univariate regression analysis. The variant rs1260326 of glucokinase regulator (GCKR) (CT genotype: adjusted OR=5.17; p-value=0.007; TT genotype: adjusted OR=6.28; p-value=0.027) remained associated with low HDL-c phenotype, together with hypertension and elevated body mass index, after multiple regression analysis.

Conclusion. The variant rs1260326 near GCKR is associated with low HDL-c phenotype among Filipinos. Its role in the expression of low HDL-c phenotype should be further investigated prior to the development of possible clinical applications.

Cardiovascular Diseases ; Dyslipidemias ; Genetics ; Polymorphism, Single Nucleotide