Advances in Diagnosis and Therapeutics in Recurrent Autoimmune Pericarditis in Pregnancy.

Pericarditis in pregnancy is uncommon, and there is a paucity of data regarding the safety and efficacy of conventional therapy. We describe a complex case of recurrent pericarditis in the setting of pregnancy and newly diagnosed systemic lupus erythematosus and discuss the challenges in managing this subset of patients.

Pericardial mesothelioma presenting as constrictive pericarditis.

A 46-year-old woman underwent pericardiocentesis and pericardial window for recurrent pericardial effusion. She presented 17 months later with signs and symptoms consistent with constrictive pericarditis. Cardiac magnetic resonance imaging revealed an infiltrative mass surrounding the pericardium. A transcutaneous core needle biopsy of the pericardium confirmed the diagnosis of pericardial mesothelioma.

A case report of infective endocarditis in a 52-year-old female with unrepaired tricuspid atresia and d-transposition of the great arteries.

Background: d-Transposition of the great arteries (d-TGA) is a congenital cardiac defect that is typically fatal. Those patients who survive without surgical repair and who are rare in number, need adequate intracardiac shunting and will suffer from chronic cyanosis. Here, we present a rare case of an adult with unrepaired cyanotic congenital heart disease (CHD) who developed infective endocarditis (IE) and also our approach to the medical decision-making process in this uncommonly encountered dilemma. Case summary: A 52-year-old female with unrepaired d-TGA with tricuspid atresia, hypoplastic right ventricle, unrestricted atrial septal defect, ventricular septal defect, and sub-valvular as well as valvular pulmonic stenosis with a hypoplastic, bicuspid pulmonary valve presented with abdominal pain and hypoxia and was found to have an acute renal infarct. Transthoracic echocardiogram (TTE) revealed a large mobile mass on the mitral valve. Blood cultures grew Streptococcus mitis-oralis and she was diagnosed with streptococcal native mitral valve IE complicated by a renal embolus. Her large left-sided vegetation and embolic phenomenon favoured surgery. However, a right heart catheterization showed normal intracardiac pressures, likely a result of multi-level obstruction relating to sub-valvular and valvular pulmonary stenosis protecting the pulmonary vasculature from over-circulation and pulmonary hypertension. Cardiac surgery posed a significant risk of destabilizing her delicately balanced haemodynamics. Hence, she was treated with ceftriaxone for 4 weeks. A repeat TTE 8 weeks later showed a resolution of the vegetation. Discussion: A decision for surgery vs. medical treatment for IE in adult patients with compensated CHD should be made following a multi-disciplinary assessment of each patient's unique cardiac haemodynamics and after shared decision-making with the patient.

Hyperlipidemia and Cardiovascular Disease in People with Type 1 Diabetes: Review of Current Guidelines and Evidence.

PURPOSE OF REVIEW: In this review, we discuss the prevalence of cardiovascular disease in people with type 1 diabetes. We outline key risk factors associated with increased cardiovascular event rates and discuss the prevalence and mechanisms underlying hyperlipidemia in people with type 1 diabetes. Finally, we summarize the evidence to support early and more aggressive lipid-lowering therapy in people with type 1 diabetes and review current guideline recommendations. RECENT FINDINGS: Comprehensive treatment of hyperglycemia, hypertension, and hyperlipidemia reduces adverse cardiovascular outcomes in people with type 2 diabetes. In contrast, evidence to support a comparable benefit of intensive cardiovascular risk factor management in people with type 1 diabetes is lacking from prospective, randomized trials and has only been shown in registries. Therefore, current treatment guidelines extrapolate prospective clinical trial evidence obtained in people with type 2 diabetes to provide similar treatment recommendations for people with type 1 and type 2 diabetes. Evidence supports the more aggressive treatment of cardiovascular risk factors in people with type 1 diabetes, who would likely benefit from early risk stratification and comprehensive risk factor management, including aggressive lipid-lowering therapy.

Recurrent Pericardial Effusions and Pericarditis Due to Yellow Nail Syndrome.

A 79-year-old woman presents with recurrent pericardial and pleural effusions for several years. She was noted to have exudative pleural effusions and bilateral nailbed discoloration. The constellation of her presenting symptoms and existing physical examination findings revealed a diagnosis of yellow nail syndrome, a rare cause of recurrent pericardial effusions. (Level of Difficulty: Advanced.).

Ross procedure after prosthetic valve thrombosis in a patient with antiphospholipid syndrome and recurrent bleeding.

The Ross procedure allows replacement of a diseased aortic valve with pulmonary root autograft, possibly avoiding the highly thrombotic mechanical valves and immunologic deterioration of tissue valves in antiphospholipid syndrome (APS). Here, we present the use of the Ross procedure in a 42-year-old woman with mild intellectual disability, APS, and a complex anticoagulation history after she presented with thrombosis of her mechanical On-X aortic valve previously implanted for non-bacterial thrombotic endocarditis.

Dupilumab-Associated Eosinophilic Granulomatosis With Polyangiitis.

A 58-year-old Caucasian male developed generalized weakness, bilateral upper and lower extremity arthralgias, left pedal edema, and a new rash over two weeks after receiving one dose of dupilumab for chronic sinusitis. He was found to be positive for perinuclear anti-neutrophil cytoplasmic antibodies and myeloperoxidase, with evidence of interstitial lung disease on CT imaging. A skin biopsy showed eosinophilic vasculitis and a kidney biopsy showed pauci-immune glomerulonephritis. He was diagnosed with eosinophilic granulomatosis with polyangiitis due to dupilumab exposure.

Hypertensive emergency and seizures in a 30-year-old man with anti-glomerular basement membrane disease.

Anti-glomerular basement membrane disease can rapidly lead to renal failure and blood pressure dysregulation. A rare complication is hypertensive encephalopathy in the form of seizures. Patients who have a negative initial seizure workup should have an MRI. These patients need tight blood pressure control and monitoring to prevent future seizures.

A rare case of nitrofurantoin-induced acute lung injury.

Nitrofurantoin is a common treatment for urinary tract infections. Acute lung injury resulting from nitrofurantoin is a rare, life-threatening complication with women being at greater risk. Symptoms include respiratory distress with fevers, rash, eosinophilia, and new-onset atrial fibrillation. Treatment includes discontinuing the drug and possibly glucocorticoids for persistent oxygen demand.

Fifty-eight-year-old female with abdominal migraine: A rare cause of episodic gastrointestinal disturbance in adults.

Abdominal migraine (AM) is a predominantly pediatric condition characterized by erratic episodes of abdominal pain, nausea, and vomiting with spontaneous periodic relief. It should be considered as a differential diagnosis in symptomatic adults.

Differences in microRNA-29 and Pro-fibrotic Gene Expression in Mouse and Human Hypertrophic Cardiomyopathy.

Background: Hypertrophic cardiomyopathy (HCM) is characterized by myocyte hypertrophy and fibrosis. Studies in two mouse models (R92W-TnT/R403Q-MyHC) at early HCM stage revealed upregulation of endothelin (ET1) signaling in both mutants, but TGFß signaling only in TnT mutants. Dysregulation of miR-29 expression has been implicated in cardiac fibrosis. But it is unknown whether expression of miR-29a/b/c and profibrotic genes is commonly regulated in mouse and human HCM. Methods: In order to understand mechanisms underlying fibrosis in HCM, and examine similarities/differences in expression of miR-29a/b/c and several profibrotic genes in mouse and human HCM, we performed parallel studies in rat cardiac myocyte/fibroblast cultures, examined gene expression in two mouse models of (non-obstructive) HCM (R92W-TnT, R403Q-MyHC)/controls at early (5 weeks) and established (24 weeks) disease stage, and analyzed publicly available mRNA/miRNA expression data from obstructive-HCM patients undergoing septal myectomy/controls (unused donor hearts). Results: Myocyte cultures: ET1 increased superoxide/H2O2, stimulated TGFß expression/secretion, and suppressed miR-29a expression in myocytes. The effect of ET1 on miR-29 and TGFß expression/secretion was antagonized by N-acetyl-cysteine, a reactive oxygen species scavenger. Fibroblast cultures: ET1 had no effect on pro-fibrotic gene expression in fibroblasts. TGFß1/TGFß2 suppressed miR-29a and increased collagen expression, which was abolished by miR-29a overexpression. Mouse and human HCM: Expression of miR-29a/b/c was lower, and TGFB1/collagen gene expression was higher in TnT mutant-LV at 5 and 24 weeks; no difference was observed in expression of these genes in MyHC mutant-LV and in human myectomy tissue. TGFB2 expression was higher in LV of both mutant mice and human myectomy tissue. ACE2, a negative regulator of the renin-angiotensin-aldosterone system, was the most upregulated transcript in human myectomy tissue. Pathway analysis predicted upregulation of the anti-hypertrophic/anti-fibrotic liver X receptor/retinoid X receptor (LXR/RXR) pathway only in human myectomy tissue. Conclusions: Our in vitro studies suggest that activation of ET1 signaling in cardiac myocytes increases reactive oxygen species and stimulates TGFß secretion, which downregulates miR-29a and increases collagen in fibroblasts, thus contributing to fibrosis. Our gene expression studies in mouse and human HCM reveal allele-specific differences in miR-29 family/profibrotic gene expression in mouse HCM, and activation of anti-hypertrophic/anti-fibrotic genes and pathways in human HCM.