Pulmonary arterial hypertension: a clot in question.

Pulmonary arterial hypertension (PAH) is a group of disorders characterized by a progressive increase in pulmonary vascular resistance leading to right heart failure and premature death. We present an unusual case of PAH diagnosed initially as Idiopathic PAH (IPAH) after secondary causes were excluded which was successfully managed for a number of years with vasodilators and anticoagulation. Over the months after stopping anticoagulation (because of recurring small bowel hemorrhaging) patient developed progressive findings of right heart failure, which failed to respond to escalating doses of prostacyclin. The patient died and an autopsy revealed the surprising finding of extensive organized central pulmonary artery thrombi as is seen in patients with chronic thromboembolic pulmonary hypertension (CTEPH). We discuss the question of whether these thrombi are generally embolic or develop in situ and recommend that clinicians have a high index of suspicion for central thrombi in patients with IPAH were anticoagulation is contraindicated.

Brugada pattern mimicking acute coronary syndrome in a febrile state.

The Brugada type pattern is characterized by a coved or saddleback shaped ST-segment elevation in the right precordial leads (V1-V3) on a surface 12 lead electrocardiogram (ECG). This pattern can be seen spontaneously, induced by sodium channel blocking drugs or rarely by hyperthermia. The mechanism is secondary to an alteration in the sodium channels induced by a febrile state. Such ECG's could easily be mistaken for acute ST segment elevation myocardial infarction and thus pose a unique clinical challenge to emergency room physicians. We report such a case of fever induced Brugada pattern and discuss the underlying mechanisms.

Pineal germinoma.

Germinomas are gonadal neoplasms that rarely occur extragonadally in the midline structures of the human body. Newly diagnosed adult cases of pineal gland germinomas are very rare since most of the cases are diagnosed in the mid teens. The estimated incidence of this tumor in western countries is between 0.4-3.4%. Typically, this tumor is diagnosed by its characteristic radiological appearance alone, supported by tumor marker(s) or by stereotactic biopsy of the tumor. We are reporting a very unique case of pineal germinoma diagnosed in an adult at our institute by cerebrospinal fluid cytology with literature review. We analyzed case reports, literature reviews, and therapy and diagnostic articles about pineal germinoma in the English literature from 1983 to 2009 through the national library of medicine, Pubmed, and OVID search engines. We used key words "pineal germinoma," "pineal gland tumor," "CNS germinoma," and "extragonodal germinomas" to search for our articles.

Hemorrhagic Cardiac Tamponade Associated with Apixaban: A Case Report and Systematic Review of Literature.

BACKGROUND: Hemorrhagic cardiac tamponade (HCT) is characterized by rapid accumulation of blood in the pericardium causing hemodynamic collapse. We report a case of HCT due to Apixaban use in a patient with renal cell carcinoma, supplemented with a systematic review of pericardial tamponade associated with the use of direct oral anticoagulants (DOACs). CASE REPORT: A 62-year-old African American male with a history of metastatic renal cell carcinoma presented with dyspnea while taking Apixaban. He was diagnosed with pericardial tamponade and 800 ml of hemorrhagic effusion was drained. The pericardial fluid analysis was negative for malignancy and suggestive of HCT. He had a complicated hospital course and died several days later. METHODS: We searched MEDLINE, EMBASE and other sources for published cases of pericardial tamponade associated with DOACs. Our outcomes of interest included patient characteristics, risk factors, timing from the start of anticoagulation to tamponade, treatment and mortality. Simple descriptive statistics using percentages for categorical variables were used to describe the included cases. RESULTS: A total of 26 cases were included in the final systematic review after searching MEDLINE, EMBASE and other sources. The mean age was 70 years (range 43-88) with 19 (73%) males. Twelve cases (46%) were associated with Rivaroxaban, 9 (37%) with Dabigatran and 5(19%) with Apixaban. Sixteen cases had elevated INR and 15 had elevated creatinine. Only 2 patients died but 24 had to undergo pericardiocentesis. CONCLUSION: Cardiac tamponade is rarely associated with DOACs and elderly male patients with renal and coagulation abnormalities appear to have the highest risk.

Obesity, brain natriuretic peptide levels and mortality in patients hospitalized with heart failure and preserved left ventricular systolic function.

BACKGROUND: An inverse relationship between brain natriuretic peptide (BNP) levels and body mass index (BMI) has been described for patients with left ventricular (LV) systolic dysfunction. In this study, the association of BMI, BNP levels and mortality in patients hospitalized for heart failure with preserved LV systolic function (HFpLVF) was investigated. METHODS: One hundred fifty consecutive patients (98% men) who were hospitalized with HFpLVF and had BNP levels measured on admission were analyzed. Patients were divided into categories of BMI: normal (BMI < 25 kg/m), overweight (BMI 25-29.9 kg/m) and obese (BMI ≥ 30 kg/m). Relevant clinical and echocardiographic characteristics and all-cause mortality were obtained through chart review. RESULTS: BNP levels were significantly lower in obese (median = 227 pg/mL) and overweight (median = 396 pg/mL) patients compared with those with normal BMI (median = 608 pg/mL, P = 0.003). Higher BMI predicted BNP levels of <100 pg/mL. Compared with patients with normal BMI, overweight and obese patients had a significantly lower risk of total mortality, even after adjusting for other clinical characteristics, including log-transformed BNP levels, atrial fibrillation, the use of beta-blockers at discharge, age, hemoglobin levels and the presence of pulmonary congestion on admission. Higher BNP levels also independently predicted mortality. CONCLUSIONS: An inverse relationship between BMI and BNP levels exists in patients hospitalized with HFpLVF. Higher BMI is associated with lower mortality, whereas higher BNP levels predict higher mortality in male patients with HFpLVF. These findings should be confirmed in a larger multicenter setting.

Is risk factor control and guideline-based medical therapy optimal in patients with nonobstructive coronary artery disease? A Veterans Affairs study.

BACKGROUND: Aggressive risk factor modification using evidence-based secondary prevention strategies is recommended in coronary artery disease (CAD). Utilization of such strategies was compared in patients with nonobstructive CAD (NOCAD) and obstructive CAD (OCAD). METHODS: Patients undergoing coronary angiography (excluding normal coronary angiograms), between January 2006 and June 2006, at the Veterans Affairs Medical Center were included. Demographic, clinical and treatment data were compared between the groups at baseline and 1 year. RESULTS: Of the 354 patients who underwent coronary angiography, 222 (63%) had follow-up data available at 12 ± 2 months. The mean age in the NOCAD (n = 119) and OCAD (n = 103) groups was similar. There was a lower prevalence of hypertension and heart failure (P < 0.05) in the NOCAD group. Compared with the OCAD group, aspirin use was similar but statin use was lower in the NOCAD group (P = 0.008). At 1 year, statin use (P = 0001) and angiotensin-converting enzyme inhibitor/angiotensin receptor blocker use (P = 0.001) were significantly lower, whereas the use of aspirin was numerically lower (P = 0.06) in the NOCAD group. Mean low-density lipoprotein cholesterol levels were at goal (<100 mg/dL) in the NOCAD group at baseline and 1 year, whereas the same slightly worsened in the OCAD group at 1 year. CONCLUSIONS: The use of evidence-based medical therapy is lower in patients with NOCAD compared with those with OCAD. Improved awareness among health care providers and a unified effort to implement secondary prevention strategies may help correct such deficiencies.

Primary pericardial sarcoma: a case report and a brief review.

There are very few cases of primary pericardial sarcomas reported in the English literature. Pericardial tumors, like other cardiac tumors, are most likely to be metastatic in nature and are an extension of primary tumors from the surrounding structures. Sarcomas are the most common primary pericardial tumors. Surgical eradication of the tumor is considered to be the treatment of choice. We are presenting a case of a primary pericardial, high-grade pleomorphic undifferentiated sarcoma that was diagnosed at our institution. We discuss the available diagnostic modalities and also shed light on alternative therapies when patients are not ideal surgical candidates.

Papillary renal cell carcinoma with cutaneous metastases.

Cutaneous metastasis is a rare form of presentation of renal cell carcinoma (RCC). Case reports and case series of RCC with skin metastasis in the English literature from 1972 to 2008 were identified by searching the keywords "renal cell cancer," "papillary renal cell tumor," "skin metastasis," and "cutaneous metastasis" in the National Library of Medicine, PUBMED, OVID, and EMBASE search engines. Although a few cases of RCC with skin metastasis are reported in the literature, almost all are clear cell histologic type. We are reporting a unique case of papillary RCC with cutaneous metastases diagnosed at our institution along with a comprehensive literature review of papillary RCC etiology, clinical presentation, prognosis, diagnosis, and the treatment options that are currently available.